Neck masses, thyroid nodules, sialorrhea Flashcards

Question 1

Q

List a complete differential for a lateral pediatric neck mass

Answer

A

A. CONGENITAL
1. Branchial cleft anomalies
2. Laryngocele
3. Thymic cyst
4. Pseudotumor of infancy
5. Heterotopic cervical salivary gland tissue
6. Preauricular pit/sinus/cyst
7. Bronchogenic cyst
8. Epidermal inclusion cyst
9. Pilomatrixoma
10. Accessory tragus
11. Cervical rib
12. Choristoma (a histologically normal tissue proliferation or nodule of a soft tissue type not normally found in the anatomic site of proliferation)
13. Foregut duplication cyst

B. INFECTIOUS
1. Lymphadenitis
- Acute viral
- Acute bacterial
- Mycobacterial
- Cat-scratch disease
- Fungal, parasitic, opportunitic infection
- PFAPA
- HIV, EBV
2. Infectious presentation of congenital mass
3. Anatomical site of infection
- PTA
- Retropharyngeal/parapharyngeal space
- Prevertebral space/danger space
- Mandibular space/submandibular sialadenitis
- Masticator space
- Buccal space
- Parotid space/parotitis

C. INFLAMMATORY
1. Sarcoidosis
2. Kawasaki Disease
3. Kikuchi-Fujimoto disease
4. Rosai-Dorfman disease
5. Castleman disease
6. Systemic lupus erythematosus
7. Juvenile idiopathic arthritis
8. Dermatomyositis
9. Serum sickness
10. Churg-Strauss syndrome (eGPA)

D. NEOPLASTIC BENIGN
1. Teratoma
2. Neurofibroma
3. Lipoma
4. Hibernoma
5. Lipoblastoma
6. Paraganglioma
7. Goiter
8. Benign mixed tumor
9. Osteoblastoma
10. Fibromatosis
11. Nodular fasciitis
13. Schwannoma
14. Pleomorphic adenoma
15. Neuroma
16. Rhabdomyoma
17. Neurofibroma

E. NEOPLASTIC MALIGNANT
1. Lymphoma
2. Metastatic carcinoma
3. Rhabdomyosarcoma
4. Neuroblastoma
5. Fibrous histiocytoma
6. Langerhans cell histiocytosis
7. Chloroma
8. Malignant peripheral nerve sheath tumor
9. Myoepithelial carcinoma

F. VASCULAR
1. Hemangioma
2. Lymphatic
3. capillary
4. Venous
5. Arterial
6. Mixed

Question 2

Q

List a complete differential for a midline pediatric neck mass

Answer

A

A. CONGENITAL
1. Thyroglossal duct cyst
2. Dermoid cyst
3. Plunging ranula
4. Teratoma
5. Thymic cyst or ectopic thymus
6. Ectopic thyroid

B. INFECTIOUS/INFLAMMATORY
1. Lymphadenitis - from infections arising from oral or nasal cavity
2. Thyroiditis
3. Infectious presentation of congenital mass

C. NEOPLASTIC
1. Benign
- Thyroid adenoma
2. Malignant
- Thyroid carcinoma
- Malignant thyroglossal duct cyst

Question 3

Q

What is the most common congenital cervical neck mass?

Answer

A

Branchial cleft anomalies

Question 4

Q

What is the difference between a cyst, sinus, and fistula?

Answer

A

Cyst = no communication with the body surface (fully enclosed, encapsulated, entrapped sac). No internal/external openings.

Sinus = communicates with a single body surface opening - either skin or pharynx (one blind end). Tract from mesoderm to skin (no internal opening)

Fistula - Communicates with two body surfaces (communicating tract).

Question 5

Q

What is the most common arch anomaly?

Answer

A

Second branchial arch anomaly

Question 6

Q

What can isolated pharyngeal arch remnants present as? List two presentations

Answer

A

Accessory Auricle
Subcutaneous cartilaginous tags along the anterior border of the SCM

Question 7

Q

Regarding first branchial cleft anomalies, discuss:

What are the two main different types? How do they differ in content and in the position? Describe the position of each
Describe the general clinical presentation of each type

Answer

A

Rare, occur below the level of the tragus

“WORK” TYPES OF FIRST BRANCHIAL CLEFT ANOMALIES:

Work Type 1:
- Duplication of the ectodermal EAC (ectodermal elements only!) - presents parallel to EAC
- Cyst tends to be pre- or peri-auricular – may go into parotid
- Exists anterior to/within normal EAC
- Lateral to CNVII (and typically superficial)
- Typically ends in a blind sac/cul-de-sac or bony plate near/at the level of the mesotympanum
- Treatment: Surgical excision
Work Type 2 (more common):
- Duplication that involves both ectodermal and mesodermal elements of the canal, including the cartilaginous portion of the canal
- Cyst tends to be near angle of mandible (always superior to hyoid) - inferior to ear, superior neck
- Position is variable near CNVII (often medial to/underlying VII - be careful!) – more often than Type 1; but can also be lateral
- Typically ends near or in the EAC, by the bony-cartilaginous junction
- Often involves parotid

CLINICAL PRESENTATION
1. May present as recurrent inflammatory lesions in the EAC or periauricular region
1. Punctum anterior to root of the helix
2. Mostly asymptomatic
3. Occasionally infected

Notability Pediatric neck masses lecture

Question 8

Q

How are first branchial cleft anomalies different from preauricular cysts?

Answer

A

DIFFERENCE BETWEEN PREAURICULAR CYSTS/SINUSES:
- Preauricular cysts/sinuses occur anterior to the EAC, usually superior to tragus
- Typically inclusion cysts related to incomplete fusion of the ectodermal hillocks of His from the first/second branchial arches during formation of the auricle
- Other theory is they are a localizede folding of ectoderm during auricular development

Question 9

Q

What is the treatment of 1st branchial arch anomalies?

Answer

A

Treatment of Work Type 1:
1. If infected: antibiotics ± needle drainage (avoid lancing), wait 6 weeks after infection prior to OR
2. OR: Trace tract to the root of the helix, incise a wedge of perichondrium ± cartilage
3. Facial nerve not at risk here (compared to Type 1 Branchial cleft anomaly CNVII at risk)

Treatment of Work Type 2:
- Surgical excision, superficial parotidectomy
- Trace back to origin (typically the bony/cartilaginous junction of EAC)
- Cartilage resection
- CNVII must be identified! Tract relation to CNVII variable

Question 10

Q

Describe the typical course of a Work Type I and Work Type II Branchial Cleft Cysts

Answer

A

Type I: ectodermal elements only; duplicated EAC; typically begins periauricularly → passes lateral (superior) to CN VII → parallels the EAC → ends as a blind sac near the mesotympanum

Type II: more common; ectodermal and mesodermal elements; duplicated membranous EAC and pinna; presents near angle of mandible→ passes lateral or medial to CN VII → ends near or in the EAC

Usman lecture congenital neck masses

Question 11

Q

Regarding second branchial anomalies, discuss:
1. What is the frequency?
2. Where is the typical location and course?
3. What might the treatment include?

Answer

A

FREQUENCY:
1. Most common, >90%
2. 10% are bilateral

LOCATION/COURSE:
1. Exits anterior to SCM, below angle of mandible, in upper 1/3 neck
2. Cysts may lie anterior to SCM
3. Fistulas may extend from the superficial area of the neck to the palatine tonsil superiorly
- Starts with external opening in the mid-lower neck at the anterior border of the SCM
- Penetrates the platysma muscle
- Courses along carotid sheath
- Heads superiorly between the internal and external carotid arteries
- Passes DEEP to CNVII, and SUPERFICIAL to glossopharyngeal nerve and hypoglossal, and below the stylohyoid ligament
- Ends in the palatine tonsillar fossa or middle constrictors

DEEP TO: 2nd Arch structures
- ECA
- Stylohyoid muscle
- Posterior belly of digastric
- CNVII

SUPERFICIAL TO: 3rd arch derivations
- ICA
- CNIX
- CNXII

TREATMENT:
1. Tonsillectomy

Question 12

Q

Regarding third branchial anomalies, discuss:
1. What is the frequency?
2. Where is the typical location and course?
3. What is the common clinical presentation - 3 features

Answer

A

Frequency: Rare

LOCATION/COURSE:
- Starts at the medial edge of the SCM in the lower 2/3 of the neck, lateral to the common carotid
- Superficial to CNX and common carotid, and CNXII
- Travels medial and posterior to the internal carotid
- Travels over the hypoglossal
- Travels deep under glossopharyngeal
- May also run through the thyroid gland
- Punctures through the thyrohyoid membrane into the base of the piriform sinus, over the superior laryngeal nerve

DEEP TO: Third arch derivatives
- Internal Carotid Artery
- CN IX

SUPERFICIAL TO: Fourth arch derivatives and 12:
- CNX
- Common carotid
- CNXII
- Superior laryngeal nerve

CLINICAL PRESENTATION:
1. Typically recurrent suppurative thyroiditis (may need to take some parts of thyroid with excision)
2. Left side more common (90%)
3. Lesions may be associated with parathyroid, thymic, or thyroid tissue

TREATMENT:
- Surgical approach: must visualize recurrent laryngeal nerves (thyroidectomy incision)

Question 13

Q

Regarding fourth branchial arch anomalies, discuss:
1. What is the frequency?
2. Where is the typical location and course?
3. What is the common clinical presentation?
4. What is the treatment?

Answer

A

Frequency:
- Very rare
- 60 reported cases, almost all on the left side

LOCATION/COURSE:
- Start medial to the SCM in the lower neck
- Travel inferiorly around the subclavian artery on the right; around the arch of the aorta on the left (follows RLN)
- Posterior to common carotid artery
- Moves lateral to hypoglossal and loops around the hypoglossal
- Posterior to thyroid gland to tracheoesophageal groove
- Emerges near the cricothyroid joint
- Pierces cricothyroid membrane Inferior to SLN, superior/superficial to RLN
- End in apex of the piriform sinus or cervical esophagus

CLINICAL PRESENTATION:
- Can present as recurrent thyroiditis or lower neck abscess

TREATMENT:
- Surgical excision: inspection of the pyriform sinus should precede surgical exploration in third and fourth BAs
- Recurrence ~22%
- I+D abscess first, external approach
- Endoscopic electrocautery or laser excision possible

Question 14

Q

A kid with recurrent thyroiditis, or acute suppurative thyroiditis/thyroid cyst/abscess - what should be ruled out?
How should this be worked up?
What is the treatment?

Answer

A

Rule out: 3rd/4th branchial anomy fistula

Workup:
1. US or CT
2. Laryngeal endoscopy: look for sinus opening in piriform apex

Treatment options:
1. Obliterate sinus opening
2. Hemithyroidectomy to include cyst, then track the sinus back to the piriform

Question 15

Q

Regarding branchial arch anomalies in general, discuss:
1. What is the most common?
2. How often are they bilateral?
3. What syndromes are commonly associated?
4. What is the general treatment? Is there specific treatment for 1st branchial arch anomalies?

Answer

A

Most common = 2nd arch

Bilateral ~5%

Syndromes associated:
1. 1st/2nd: Treacher collins, BOR, Goldenhar
2. 3rd/4th: 22q11

TREATMENT:
1. Surgical excision complete of the cyst wall and fistula tracts to avoid recurrence
2. Acute infections: antibiotics, I+D for abscess
3. Definitive surgical excision should be deferred until resolution of acute inflammation

1ST BRANCHIAL TREATMENT:
1. Excision via parotidectomy incision, retrograde dissection to EAC, with care to identify and preserve the facial nerve, which may be displaced inferiorly by the mass
2. Cartilage resection - ensure excision of base of tract

2ND BRANCHIAL TREATMENT:
- Surgical excision of the entire tract to tonsillar fossa
- Step-ladder incisions may be needed

3RD/4TH BRANCHIAL TREATMENT:
1. DL may be needed to inspect piriform sinus for fistula
2. Cannulating fistula to inject dye may be useful to identify tract
- Can also use monopolar flexible electrode to endoscopically cauterize the tract
- 15% partial thyroidectomy - then track upwards to the piriform

Question 16

Q

Most common cause of chronic benign pediatric lymphadenopathy?

Answer

A

Cat Scratch Disease (Bartonella Henselae)
- Serologic testing
- Intracellular gram-negative bacillus on Warthin-Starry stain
- Treatment: Self-limiting, or Azithromycin
- Avoid incision/drainage (draining tract)

Question 17

Q

What is a condition that can occur in immunocompromised patients with Bartonella Henselae infection?

Answer

A

Bacillary Angiomatosis - vascular, proliferative form of Bartonella infection

Question 18

Q

What is the most common infectious organism in pediatric bacterial cervical lymphadenitis?

Answer

A

Staphylococcus aureus
Streptococcus pyogenes

Question 19

Q

Regarding thyroglossal duct cysts, discuss:
1. What is the common clinical presentation? Where exactly do they present?
2. How are they typically examined?
3. What is the workup and why?
4. What is the rate of malignancy?
5. What is the histology seen of thyroglossal duct epithelium? 3
7. What is the management? What is the risk of recurrence?

Answer

A

Clinical presentation:
1. Midline neck mass just above thyroid lamina below hyoid - passes through hyoid (hyoid grows around tract in utero)
2. Prone to infection, especially following URTI

Examination:
1. Midline neck mass anywhere from BOT to thyroid - 80% occur at or near hyoid bone
2. Mass moves superiorly in the neck when tongue is protruded

Workup:
1. U/S: confirm diagnosis and presence of normal thyroid gland (sometimes thyroid gland fails to form and this is the only normal thyroid gland)
2. Preop TFTs and nuclear medicine not required
- If only thyroid is within cyst, need to have careful consideration prior to surgical excision

Malignancy:
- Differentiated thyroid cancers within TGDCs very low rate, indolent nature
- If malignancy found in TGDC, associated 6.6% risk of microcarcinoma in the normal-appearing thyroid
- Thyroidectomy not required for a malignant TGDC, but gland needs to be evaluated closely and monitored for life

Histology:
1. Squamous
2. Respiratory
3. Thyroid follicles and colloid

Management:
1. Surgery - Sistrunk procedure
- Removal of the central hyoid and a core of muscle from the genioglossus extending to the foramen cecum
- Modification: Do not need pharyngotomy at the foramen cecum to be removed too
- TGDC Simple Excision without hyoid = 46% recurrence
- Sistunk - 5.8% recurrence

Question 20

Q

Where are the common locations of a thyroglossal duct cyst? Name 4

Answer

A

Location of TGDCs:
1. Infrahyoid - 65%
2. Suprahyoid - 20%
3. Intrahyoid - 15%
4. Lingual - 2%
5. Suprasternal - rare
6. Laryngeal - care reports

Question 21

Q

What is the differential diagnosis for a pediatric posterior tongue mass? 7

Answer

A

Lingual thyroid (undescended)
Thyroglossal duct cyst (lingual, undescended)
Lingual tonsils
Granular cell tumor (epulis is a type on the alveolus, not tongue)
Vascular anomaly (venous, lymphatic)
Dermoid tumor
Teratoma

Question 22

Q

What are the investigations for a posterior tongue mass? 3

Answer

A

CT scan to define margins of the mass
TSH, T3/T4 establish thyroid function
I-123 scan or ultrasound to rule out lingual thyroid, identify other foci of functioning thyroid tissue

Question 23

Q

What is the frequency of head and neck malignancy in kids?

What are the most common head and neck malignancies in children, based on different varying age groups?

Answer

A

Frequency = 12% of all pediatric malignancies occur in H/N

Overall incidence in kids:
1. Lymphomas - 27% (Hodgkin 17%, NHL 10%)
2. Neural tumors (23%; retinoblastoma, neuroblastoma)
3. Thyroid malignancies (21%, PTC most common)
4. Soft tissue sarcomas (12% rhabdo; 8% non-rhabdo soft tissue sarcomas)

0-1 YEARS OLD:
1. Neuroblastoma
2. Rhabdomyosarcoma
3. Lymphoma
4. NRSTS (Nonrhabdomyosarcoma soft tissue sarcoma)

1-5 YEARS OLD
1. Rhabdomyosarcoma
2. Lymphoma
3. Neuroblastoma
4. NRSTS

6-10 YEARS OLD
1. Lymphoma
2. Rhabdomyosarcoma
3. NRSTS
4. Thyroid malignancies

11-18 YEARS OLD
1. Lymphoma
2. Thyroid malignancies
3. Nasopharyngeal malignancies
4. Salivary gland neoplasms

Question 24

Q

What are red flags on history/exam to suggest malignancy for children with neck masses? List 7

Answer

A

Age < 12 months
Lymph node is non-tender and hard
Diameter >3cm
Supraclavicular location
Persistent generalized lymphadenopathy
Mediastinal or abdominal mass
Persistently unexplained: pruritus, fever, weight loss, pallor, fatigue, petechiae, hemorrhagic lesions, or hepatosplenomegaly

Cummings Box 204.1 (Ch 204)

Question 25

Q

Is ionizing radiation a risk factor for cancer in children? What types of malignancies? (4)

What other agents predispose patients to certain cancers?

Answer

A

Ionizing radiation is a risk factor for: “TABO”
1. Thyroid cancer
2. Acute lymphoblastic leukemia
3. Brain tumors
4. Osteosarcomas

Imaging principle = ionizing dose as low as reasonably achievable

Chemotherapeutic agents predispose to acute myelocytic leukemia and osteosarcoma

Question 26

Q

NOT IN VANCOUVERS

What syndromes are associated with pediatric head and neck malignancies? What types of malignancies are they associated with?

Answer

A

Down Syndrome (Leukemia)
Neurofibromatosis Type 1 (Leukemia, gliomas, rhabdomyosarcoma, pheochromocytoma, astrocytoma)
Neurofibromatosis Type 2 (Astrocytoma, melanoma, meningioma)
Li-Fraumeni Syndrome (Osteosarcoma, Rhabdomyosarcoma, leukemia, lymphoma, breast) - pretty much all cancers; autosomal dominant disorder that is usually associated with abnormalities in the tumor suppressor protein P53 gene (TP53) located on chromosome 17p13
Gorlin syndrome (Basal cell carcinoma, medulloblastoma)
Multiple endocrine neoplasia Type 1 (parathyroid, pancreas, gastrinomas, insulinomas, carcinoid tumor)
Multiple endocrine neoplasia Type 2a (Medullary thyroid carcinoma, pheo, parathyroid adenomas)
Multiple endocrine neoplasia Type 2b (Medullary thyroid carcinoma, pheo, mucosal neuromas, and ganglioneuromas
Peutz-Jeghers syndrome (Stomach, small intestine, colon, pancreas, uterine, breast)
Beckwith-Wiedemann syndrome (Rhabdomyosarcoma, neuroblastoma, Wilm’s tumor, hepatoblastoma)
Werner syndrome (thyroid, leukemia, melanoma, osteosarcoma)
Ataxia telangiectasia (Lymphoma, leukemia)
Wiskott-Aldrich syndrome - primary immunodeficiency syndromes (Lymphoma - Non-Hidgkin)
Gardner Syndrome (aka. Familial Adenomatous Polyposis; FAP) - Papillary thyroid carcinoma, colon cancer
X-linked immunodeficiency

Cummings Table 204.2 (Ch 204)

Question 27

Q

What are the risk factors for pediatric malignancy?

Answer

A

A. Genetic (see separate card, but generally):
1. Down’s - leukemia
2. MEN 2A/2B - Medullary thyroid carcinoma
3. NF 1/2 - astrocytoma
4. Beckwith-Weidemann - Rhabdo, Wilm’s
5. Gorlin - skin cancer
6. Gardner syndrome - PTC, colon cancer
7. Li-Fraumeni - all cancers
8. Primary immunodeficiency syndroems

B. Environmental
1. XRT
2. Infectious (e.g. EBV)

C. Immunodeficiency
1. Post-transplant
2. Chemotherapy
3. HIV+
4. Autoimmune disease
5. Immunosuppressive drugs

Question 28

Q

What are main differences between pediatric vs. adult malignancies?

Answer

A

Adults = epithelial origin predominantely

Pediatrics = Mesenchymal, neuroectodermal origin predominantely

Question 29

Q

For pediatric tumors, how should they be managed when biopsying?

Answer

A

Always send FRESH!
- Most are secondary to genetic translocation/syndromes therefore can do karyotyping

Question 30

Q

What is the frequency and epidemiology of pediatric lymphoma? Which type of lymphoma is most common?

Answer

A

Most common pediatric malignancy of the head/neck.

Third most common malignancy in children = lymphoma (after leukemia and brain tumors)

Hodgkin lymphoma:
- 95% Classic hodgkin lymphoma in USA
- NLPHL less common in USA
- Classic Hodgkin Lymphoma bimodal distribution between 15-40, and 60
– Childhood form (± 14 years) – M>F
– Young adult form (15-34 years)
– Older adult (55-74 years)

Non Hodgkin Lymphoma
- Incidence higher in younger children, rare in infants

Question 31

Q

What are the risk factors for head and neck lymphoma?

Answer

A

Immunosuppression
HIV
EBV
HTLV (Human T-Lymphocytic virus)
Sjogren’s
RA
Hepatitis C
Hashimoto’s

Question 32

Q

What is the classification of pediatric lymphoma types?

Answer

A

HODGKIN LYMPHOMA
NON-HODGKIN LYMPHOMA (60%)

Hodgkin is more common in the head/neck

A. HODGKIN Lymphoma Classification:
1. Nodular Lymphocyte-predominant Hodgkin lymphoma (NLPHL)
2. Classic Hodgkin Lymphoma (CHL) - Rye Classification (based on ratio of B cells to RS cells)
- Nodular sclerosis subtype (80%)
- Mixed cellularity subtype (15%)
- Lymphocyte-rich subtype
- Lymphocyte-depleted subtype

B. NON-HODGKIN Lymphoma WHO Classification
90% High grade B-cell, aggressive

Lymphoblastic lymphomas (precursor lymphoid neoplasms)
- T-lymphoblastic lymphoma (15-20%)
- B-lymphoblastic lymphoma (3%)
Mature B-cell lymphomas
- Burkitt lymphoma (35-40%)
- Diffuse large B-cell lymphoma (15-20%) - most common in head/neck
- Primary mediastinal B-cell lymphoma (1-2%)
- Pediatric follicular lymphoma (Rare)
- Pediatric nodal marginal zone lymphoma (rare)
Mature T-cell Neoplasms
- Anaplastic large cell lymphomas, ALK positive (15-20%)
- Peripheral T-cell lymphoma NOS (Rare)

Other:
1. Mucosa-associated lymphoid tissue (MALT) lymphoma

Question 33

Q

Discuss the clinical presentation of lymphoma - 5 findings.

What are the differences between the clinical presentation of Hodgkin’s Lymphoma vs. Non-Hodgkin’s lymphoma? Name 5 differences

Answer

A

General presentation (in OTOHNS):
1. Painless supraclavicular or cervical mass
2. Lymph node is firmer than reactive node, “rubbery”
3. Rapid enlargement may be tender node
4. B symptoms: Fever > 38 x 3 days consecutive; unexplained weight loss > 10% body weight in 6 months; drenching night sweats
5. Airway symptoms - cough, stridor, respiratory compromise - should prompt heightened concern for mediastinal disease

HODGKIN’S LYMPHOMA
1. More often localized to a single axial group of nodes - e.g. cervical, mediastinal, para-aortic (90% presents as enlarged nodal group above diaphragm, and mediastinal adenopathy common
2. Orderly spread by contiguity
3. Mesenteric nodes and Waldeyer ring rarely involved
4. Extranodal presentation rare
5. More common in adolescents and 60s (bimodal distribution)
6. More common in males 2:1
6. B-symptoms common (e.g. weight loss, fever, night sweats)

NON-HODGKIN’S LYMPHOMA
1. More frequent involvement of multiple peripheral nodes
2. Non-contiguous spread
3. Waldeyer ring and mesengeric nodes commonly involved
4. Extranodal presentation common (e.g. Waldeyer ring, salivary, larynx, sinus, orbit, scalp, etc.)
5. More common in males, increases with age
6. B-symptoms rare
7. Rapidly progressive
8. More likely to present initially with distant metastasis or direct extension to the CNS associated with neurologic impairment
9. Other risk factors: AIDS, immunosuppressive therapy, chemotherapy, Congenital immunodeficiency syndromes (e.g. Wiskott-Aldrich syndrome, Ataxia-telangiectasia, X-linked lymphoproliferative disorders)

Question 34

Q

Discuss the Ann Arbor Staging for Hodgkin and Non-Hodgkin Lymphoma

Answer

A

STAGE 1:
1. (I) Involvement of single lymph node region; OR
2. (Ie) Single extralymphatic organ/site

STAGE 2:
- (II) Involvement of 2 or more lymph node regions on same side of diaphragm; OR
- (IIe) Localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm

STAGE 3:
- (III) Involvement of lymph node regions on both sides of the diaphragm; ± S, E, or SE (below)
- (IIIs) May be accompanied by involvement of the spleen; OR
- (IIIe) May be accompanied by localized involvement of an extralymphatic organ or site (IIIe); OR
- (IIIse) Both

STAGE 4:
- Diffuse or disseminated involvement of one or more extralymphatic organs or tissues with or without associated lymph node involvement

Subclassifiers:
A: No B-symptoms
B: B-symptoms
E: Extranodal involvement (e.g. organ)
X: Bulky disease (>10cm or mediastinal mass ratio > 0.33)

Question 35

Q

What is the mediastinal mass ratio?

Answer

A

Mediastinal Mass ratio = maximum width of mass/maximum intrathoracic diameter

Question 36

Q

NOT IN VANCOUVER

Describe the Pediatric Non-Hodgkin Lymphoma St. Jude Staging System

Answer

A

STAGE I:
1. Single tumor (Extranodal) or single anatomic area (nodal) with an exclusion of mediastinal or abdomen involvement

STAGE II:
1. A single tumor (extranodal with regional node involvement
2. Two or more nodal areas on the same side of the diaphragm
3. Two single (Extranodal) tumors with or without regional node involvement on the same side of the diaphragm
4. A primary GI tumor usually in the ileocecal area with or without involvement of associated mesenteric nodes only, grossly completely resected

STAGE III:
1. Two single tumors (extranodal) on opposite sides of the diaphragm
2. Two or more nodal areas above and below the diaphragm
3. All primary intrathoracic tumors (mediastinal, pleural, thymic)
4. All extensive primary intraabdominal disease
5. All paraspinal or epidural tumors regardless of other tumor site(s)

STAGE IV:
1. Any of the aforementioned with initial central nervous system and/or bone marrow involvement

Question 37

Q

Describe the workup for a patient with suspected lymphoma.

What are 3 must-haves, and what are 5 additional investigations to be considered?

Answer

A

Summary of Must haves:
1. Labs - CBC, ESR, CRP, ALP, LDH
2. Imaging: CXR, CT Neck/Chest, MRI abdo
3. Open nodal incisional biopsy/excisional biopsy (avoid steroids, must go fresh for flow cytometry, cytology, and IHC)

Summary of Additional Considerations:
1. Bone marrow biopsy (if B symptoms or + stage 3) –> abnormal if > 25% blasts
2. Lumbar puncture if CNS concern (or MRI head)
3. Barium swallow if Waldeyer’s ring - 10% may have GI lesion)
4. Bone scan or dedicated CT scan for site of suspected bony involvement
5. PET scan controversial for staging

DETAILS BELOW ——

History and physical exam, including FNL
Labs: CBC, ESR, Serum Copper, ferritin, ALP, CRP, LDH
Imaging: CXR, CT Neck + Chest, MRI (or CT) Abdomen - MRI more useful for abdomen in pediatrics because of low volume retroperitoneal fat and minimum radiation
Lumbar Puncture for CNS involvement (can also consider MRI head)
Barium swallow (3-11% of patients with Waldeyer’s ring lymphoma will have associated GI lesion
CT any site of suspected bony involvement (or Bone Scan)
PET scan controversial: some use for initial staging and follow up; others state it leads to upstaging of disease and more aggressive treatments with increased side effects and morbidity without improving outcomes (needs more study in pediatric population)
Bone marrow Biopsy (if +B symptoms or Stage 3+) –> abnormal if >25% blasts
Open nodal incisional/excisional biopsy; considerations:
- Avoid systemic corticosteroids prior to biopsy (delays or inability to diagnosis, inability to stage)
- Must go fresh for flow cytometry, cytology, H&E stain, ± immunohistochemistry

Cummings Chapter 204

Question 38

Q

What is the prognosis of lymphoma and long term survival?

What are the prognostic factors for patients with lymphoma? 8

Which are unfavourable? 9

Answer

A

90% Long term survival

Prognostic factors:
1. Ann arbor classification
2. Mediastinal mass ratio
3. Tumor size
4. Proliferation rate
5. Extranodal sites involvement
6. Serum LDH
7. Patient’s performance status
8. CNS involvement

POOR PROGNOSTIC FACTORS:
1. Presence of B-symptoms
2. Bulky mediastinal disease
3. Extranodal sites > 1
4. Advanced stage at presentation (3 or 4)
5. Mediastinal mass ratio > 0.33
6. CNS involvement (very bad)
7. ECOG > 2
8. LDH > 1
9. Age > 60

Question 39

Q

Compare and contrast endemic vs. sporadic Burkitt’s lymphoma with respect to the following categories:

Clinical presentation
Most common distribution of disease
Annual incidence
Common tumor sites
Histopathological features
Presence of EBV DNA in tumor cells
Presence of t(8;14), t(2;8), or t(8;22) translocations
Chromosome 8 break points

Answer

A

ENDEMIC BURKITT’S LYMPHOMA
1. Clinical presentation - 6-10 years old, M>F
2. Most common distribution of disease - Equatorial Africa, New Guinea, Amazonian Brazil, Turkey
3. Annual incidence - 10 in 100000
4. Common tumor sites - Jaw, abdomen, CNS, CSF
5. Histopathological features - CD20+, usually IgM, κ or λ CD10+, BCL2-, starry sky pattern of phagocytic histiocytes and tumor cells
6. Presence of EBV DNA in tumor cells - 95%
7. Presence of t(8;14), t(2;8), or t(8;22) translocations - Yes
8. Chromosome 8 break points - Upstream of cMYC gene

SPORADIC BURKITT’S LYMPHOMA
1. Clinical presentation - 6=12 years old, M>F
2. Most common distribution of disease - North American, Europe
3. Annual incidence - 0.2 in 100000
4. Common tumor sites - Abdomen, marrow, lymph nodes, ovaries
5. Histopathological features - CD20+, usually IgM, κ or λ CD10+, BCL2-, starry sky pattern of phagocytic histiocytes and tumor cells
6. Presence of EBV DNA in tumor cells - 15%
7. Presence of t(8;14), t(2;8), or t(8;22) translocations - Yes
8. Chromosome 8 break points - within cMYC gene

Question 40

Q

What is Richter’s Transformation?

Answer

A

Low grade to high grade Non-Hodgkin’s lymphoma transformation
- Survival < 1 year

Question 41

Q

Discuss the management for Hodgkin’s lymphoma in pediatric lymphoma.

What are the common treatment side effects?

Answer

A

Combined chemotherapy + radiation modalities now favoured to reduce treatment side effects associated with Rads or Chemo alone

Radiation dose = 15-25Gy, combined with chemo regimens such as:

MOPP therapy (+ XRT = 97% local control)
- Mechlorethamine (nitrogen mustard)
- Oncovin (Vincristine)
- Procarbazine
- Prednisone
ABVD therapy
- Adriamycin (Doxorubicin)
- Bleomycin
- Vinblastine
- Dacarbazine
Stanford V
- Doxorubicin
- Vinblastine
- Mustard
- Bleomycin
- Vincristine
- Etoposide
- Prednisone

Response rates (with combination treatment):
1. Earlier stage disease - 8 year OS 98%
2. Advanced stage - 4 year event free survival 87%, OS 90%

Side effects:
1. Post-treatment hypothyroid in 2/3

Kevan/Ashley notes/Vancouver notes - Early lymphocyte predominant/very early, localized disease:
1. XRT only
- 10 year survival 90%, 10 year relapse free survival 75-80%

Cummings Chapter 204

Question 42

Q

Discuss the management for Non-Hodgkin’s lymphoma in pediatric lymphoma

Answer

A

Treatment is different based on subtypes and stages of disease

Chemotherapy (mainstay due to occult micrometastases)
- CHOP: Cyclophosphamide, Hydroxydaunomycin, Vincristine, Prednisone); or
- R-CHOP: R = Rituximab
- Others (if extensive disease): Ifosfamide, Methotrexate, Cytarabine, Doxorubicin, Etoposide
- Intrathecal chemotherapy for CNS disease

Duration: 4-7 day dose-intensive regimen to maximuze tumor cell kill rates

Radiotherapy:
- Less commonly used in NHL
- Used when pharmaceutic therapy (prednisone, cyclophosphamide) has been insufficient, especially for significant mediastinal disease causing airway compression
- Other options - CNS involvement (rarely used)

Other treatment considerations:
- Avoid GA where possible (given risk of bulky mediastinal disease and airway compression) – trach not effective for mediastinal obstruction

Question 43

Q

What are the International Prognostic indicators of Non-Hodgkin’s lymphoma? What has worse prognosis?

Answer

A

WORSE OUTCOMES IN:
1. Age > 60
2. Stage 3 or 4
3. Extranodal sites > 1
4. LDH > 1
5. ECOG > 2

5-year survival:
1. Stage 1 = 75%
2. Stage 2 = 50%
3. Stage 3 = 40%
4. Stage 4-5 = 25%

Question 44

Q

Describe the Histopathology of Hodgkin’s Lymphoma

Answer

A

Cells:
1. Hodgkin Cell
- Mononuclear and have basophilic cytoplasm
2. Pathognomonic Reed-Sternberg (RS) cells
- Large cells with abundant slightly basophilic cytoplasm, and must be multinuclear with at least two nuclei in separates lobes (“owl eyes”)
- Nucleolus tend to be prominent and eosinophilic
3. A reactive infiltrate of non-neoplastic cells is bulk of lesion

Other features:
1. High EBV + titres, and EBV-associated antigens

Stains:
1. CD15
2. CD20
3. CD30
4. LCA (leukocyte common antigen)

Cummings ch 204; Kevan Peds Page 125

Question 45

Q

NOT IN VANCOUVER

What are the histologic subtypes of HL?

Answer

A

Histologic Subtypes of HL features:
1. Nodular
- Neoplastic and inflammatory cells, which may develop into nodules that can be seen on gross specimens
- Fibrosis very pronounced
2. Mixed cellularity
- More common in patients younger than 10
- Tends to have minimal fibrosis
3. Lymphocyte-depleted CHL
- Have many more RS cells amid few lymphocytes
- Common in patients with HIV, rare in children
4. Lymphocyte-rich CHL
- Background of many small B-lymphocytes with an overall nodular or diffuse pattern
5. Nodular Lymphocyte-predominant Hodgkin lymphoma
- Mononuclear malignant cells “popcorn cells” because of lobulated appearanace
- Few RS cells; must immunophenotypically distinguish from lymphocyte-rich CHL

Question 46

Q

Describe the histopathology for Non-Hodgkin’s lymphoma

Answer

A

General features:
1. Diffuse, rather than follicular or nodular, pattern of growth

Burkitt’s Lymphoma:
1. Monomorphic, medium-sized cells with basophilic cytoplasm, round to ovoid nuclei, multiple nuclei
2. “Starry sky” appearance = ingested apoptotic cells
3. 80% Translocation of cMYC gene on chromosome 8, adjacent to enhancers that increase gene expression
4. EBV+ associated with break points outside of c-myc (negative EBV associated with break points within the gene)
- EBV viral gene products have been associated with induction of B-cell lymphomas and have been found to be essential to B-cell transformation

https://www.researchgate.net/profile/Hind-Alkatan/publication/258523927/figure/fig6/AS:669647351324687@1536667778860/The-classic-starry-sky-histologic-appearance-in-a-case-of-Burkitts-lymphoma.png

Question 47

Q

For a patient/child with suspected lymphoma and orthopnea, you are asked to do an open biopsy. What must you first consider/do?

Answer

A

Get CXR to rule out mediastinal involvement!

Might have a chest full of mets/effusion requiring special anesthetic considerations (or no GA, biopsy under local instead)

Question 48

Q

Define these chemotherapy protocols:
1. CHOP
2. R-CHOP
3. MOPP
4. ABVD

Answer

A

CHOP:
1. Cyclophosphamide
2. Hydroxydaunomycin
3. Vincristine (Oncovin)
4. Prednisone

R-CHOP:
1. Rituximab
2. CHOP

MOPP:
1. Mechlorethamine (nitrogen mustard)
2. Oncovin (Vincristine)
3. Procarbazine
4. Prednisone

ABVD:
1. Adriamycin (Doxorubicin)
2. Bleomycin
3. Vinblastine
4. Dacarbazine

Question 49

Q

Regarding tumor lysis syndrome, discuss:
1. What is it?
2. What causes it?
3. What are the risk factors?
4. What are the typical features? 6
5. What is the treatment? 5

Answer

A

TUMOR LYSIS SYNDROME: Severe metabolic complications seen soon after initiation of anti-lymphoma/leukemia treatment

CAUSE: Massive lysis of tumor cells, releasing flood of metabolically active ions and enzymes

RISK FACTORS:
1. Large, bulky tumors

FEATURES (Hyper KUP):
1. Hyperkalemia
2. Hyperuricemia
3. Hyperphosphatemia
4. HyPOcalcemia
5. Renal failure
6. Can progress to death by arrhythmia

TREATMENT:
1. IV fluids (dilution, enhances renal function)
2. Bicarbonate IV (to alkalize urine, enhance uric acid excretion)
3. Allopurinol (treats uricemia)
4. Electrolyte correction
5. Hemodialysis

Question 50

Q

What is the differential diagnosis for small round blue cell tumors?

Answer

A

MR SSSLEEP MEN

Melanoma
Rhabdomyosarcoma
Sinonasal undifferentiated carcinoma (SNUC)
Small cell cancer
Sarcoma
Lymphoma
Ewing’s Sarcoma
Esthesioneuroblastoma
Primitive Neuroectodermal Tumor (PNET)
Merkel Cell Carcinoma
Extramedullary plasmacytoma
Neuroblastoma

OTHER/”Extra’s”:
1. Desmoplastic small round cell tumor
2. Clear cell sarcoma
3. Extrarenal monophasic Wilm’s tumor
4. Extrarenal rhabdoid tumor
5. Extraskeletal myxoid chondrosarcoma

Question 51

Q

What are the most common epithelial malignancies in children’s parotid gland?

Answer

A

MEC
Acinic cell carcinoma
Rhabdomyosarcoma
Lymphoma
Adenocarcinoma, Adenoid cystic

“MARLA”

MALIGNANT:
1. Mucoepidermoid carcinoma (50% most common)
2. Acinic cell
3. Adenocarcinoma
4. Undifferentiated carcinoma
5. Adenoid cystic

Question 52

Q

Regarding rhabdomyosarcoma, discuss:
1. What is the epidemiology? What population does it commonly occur in?
2. What are the subtypes of rhabdomyosarcoma? Briefly describe how each one appears and if there are any associated genetics. What is the prognosis of each?

Answer

A

EPIDEMIOLOGY:
1. Bimodal peaks: Young kids (2-6 years) and young adults (10-18 years)
2. 28% head and neck

TYPES:
1. EMBRYONAL (60%)
- Young kids and infants
- Genetics: Not clear, 11p15 locus –> IGF-2 Gene
- Better prognosis
- Subtypes: Botryoid, Spindle cell

1a. EMBRYONAL BOTRYOID (6%)
- Appears as a cluster of grapes
- Exclusively in infants
- Arise under the mucosal surface of body orifices such as the vagina, bladder, nasopharynx

1b. EMBRYONAL SPINDLE (3%)
- Usually involves paratesticular sites

ALVEOLAR (20%)
- Adolescents
- Common in the extremities and trunk
- Genetics: PAX-3/FKHR fusion –> powerful transcription activator
- Worst prognosis
PLEMORPHIC/ANAPLASTIC (< 20% - Vancouver notes 5%) - undifferentiated/anaplastic
- Young to older adults
- Extremities & Trunk
- Can be seen in patients with Li-Fraumeni syndrome
- Poor prognosis
- Some consider this a subtype of
embryonal RS in peds and a form of
malignant fibrous histiocytoma in adults
and deleted it as a type on its own in the
new classifications

Question 53

Q

What are the most common H&N sites? Which sites are considered more or less favourable with prognosis?
What are the most common metastatic sitese for head and neck rhabdo?

Answer

A

MOST COMMON HEAD/NECK SITES (35-40% of all cases)
1. Orbit (25-30%)
2. Nasopharynx
3. Neck/face musculature
4. Temporal bone/middle ear
5. Sinonasal cavity

A parameningeal site = an area next to the membranes covering the brain, such as the nasal passages and nearby sinuses, middle ear, or the uppermost part of the throat

Favourable Sites:
1. Orbit (most common 25%)
2. Non-parameningeal H/N
3. Biliary

Unfavourable sites:
Parameningeal H/N sites (IMMNNPPP) - close to skull base and increased likelihood of intracranial extension
1. Infratemporal fossa
2. Middle ear
3. Mastoid region
4. Nasal cavity
5. Nasopharynx
6. Paranasal sinus
7. Pterygopalatine fossa
8. ± Parapharyngeal region (sometimes included)
9. Any site not “favourable”

MOST COMMON METASTATIC SITES:
1. Lung
2. Bone
3. Bone marrow

Question 54

Q

Describe the histopathology of rhabdomyosarcoma.

What is the immunohistochemical stains used?

Answer

A

General:
- Spindle cells in myxoid stroma
- Stains: actin, desmin, myoglobin, vimentin

Alveolar:
- Small round cells in a dense cluster configuration with fibrous septae around spaces that resemble pulmonary alveoli
- Pattern occurs in >50% of the tumor (if < 50% = embryonal)

Embryonal:
- Small round or spindle shaped malignant cells (“strap cells”) with interspersed large rhabdomyoblasts and eosinophilic cytoplasm (blue)

STAINS:
1. Myogenin
2. Muscle specific actin
3. Desmin

Figure 204.2 and 204.3 Cummings Ch 204

Question 55

Q

Describe the genetics of rhabdomyosarcoma

Answer

A

Alveolar associated with:
- Translocation between long arms of Chromosome 2 and 13, or 1 + 13 (less common)
- Leads to juxtaposition of FOXO1 gene with either PAX3 or PAX7 genes
- Fusion gene product = worse prognosis

Question 56

Q

What are the clinical features of rhabdomyosarcoma? 3
Percent of LN and mets on presentation?

Workup: List 8 things to be done

Answer

A

CLINICAL PRESENTATION:
1. Firm, asymptomatic mass
2. Local mass effect (non specific) - e.g. nasal obstruction, rhinorrhea, sinusitis, otitis media, epistaxis, aural polyps, otorrhea, hoarseness, and/or epiphora
3. Cranial nerve deficits from skull base erosion
4. 12% regional spread to LN at presentation
5. 5-15% distant metastasis at presentation (lung, bones)

WORKUP:
1. Full head/neck examination including cranial nerves and scope
2. Biopsy
3. Labs: CBC, lytes, renal function testing, liver enzymes, coagulation studies
4. Bone marrow aspirate and lumbar puncture for CSF cytology (staging)
5. CT of primary site, and for regional or distant metastasis (CT Chest, MRI brain, bone scan)
6. PET/CT sensitive and specific to evaluate for macroscope metastasis
7. Echo (prior to chemo)
8. Genetics for PAX-3 / FKHR mutation

Question 57

Q

Describe the TNM & AJCC pre-treatment staging for Head and Neck Rhabdomyosarcoma. What is the % survival for each AJCC stage?

What is the prognosis if patients require salvage surgery?

Answer

A

Pre-treatment staging is the TNMM stage. Referred to as “Stage”

TUMOR

T1: Tumor confined to organ or tissue of origin (primary site)
a. ≤ 5cm
b. >5cm
T2: Invasive tumors beyond organ or tissue of origin (primary site)
a. ≤ 5cm
b. >5cm

NODE

NX: Regional nodes not exained
N0: No regional LN involvement
N1: Any regional LN involvement

METASTASIS

M0: No distant metastasis
M1: Any distant metastasis

AJCC PRE-TREATMENT STAGING

STAGE I:
- Favourable site
- Any T, Any N, M0
- Survival 90%

STAGE II:
- Unfavourable site
- Any T ≤ 5cm, NX or N0, M0
- Survival 85%

STAGE III:
- Unfavourable site
- A: Any T ≤5cm, N1
- B: Any T >5cm, Any N
- M0
- Survival 70%

STAGE IV:
- Any site
- Any T, Any N, M1
- Survival 30%

50% who undergo salvage surgery die from disease

“Five Tiny New Mice”

Question 58

Q

Describe the Surgical Pathologic Grouping System for Pediatric Rhabdomyosarcoma

Answer

A

This staging is often used as a “post-operative” staging. Referred to as “Group”.

GROUP I: Localized tumor, completely removed, microscopically cleared margins and no LN involvement

GROUP II: Localized tumor, grossly removed with either:
(a) Microscopic residual disease
(b) Regional disease with involved, grossly removed regional lymph nodes
(c) Regional disease with grossly removed but microscopic residual and/or histologic involvement of the most distal node from the primary

GROUP III: Localized tumor, incompletely removed with gross, residual disease (macroscopic residual) - either after biopsy or subtotal resection

GROUP IV: Distant metastases present at diagnosis

Question 59

Q

NOT IN VANCOUVER

Describe the Prognostic stratification for Rhabdomyosarcoma

Answer

A

Based on PAX3:FOXO1 or PAX7:FOXO1 gene fusion status

VERY LOW RISK:
- Fusion Negative: MYOD1 wild-type, TP53 wild-type
- Stage I
- Group I

LOW RISK:
- Fusion Negative: MYOD1 wild-type, TP53 wild-type
- Stage I with Group II or III (Orbit only)
- Group II with Group I or II

INTERMEDIATE RISK:
- Fusion positive; Stage I-III, Group I-III
- Fusion negative; Stage I Group III (non-orbit); Stage II-III Group III; Stage III Group I-II; Stage IV Group IV (age < 10 years)

HIGH RISK:
- Fusion positive, Stage IV, Group IV
- Fusion negative, Stage IV, Group IV (Age ≥ 10 years)

Table 6: Soft tissue sarcoma committee of the Children’s Oncology Group https://www.cancer.gov/types/soft-tissue-sarcoma/hp/rhabdomyosarcoma-treatment-pdq#_128

Question 60

Q

Describe the management of Rhabdomyosarcoma. What is the prognosis?

Answer

A

SURGICAL RESECTION:
- 5mm adequate margins
- 1mm acceptable in anatomically sensitive areas
- Treatment of choice for non-parameningeal tumors
- Should only be recommended if there is a strong likelihood that clear margins will be achieved (debulking not recommended)
- Prophylactic neck dissections not recommended, but address clinically evidence LNs (N+ only)
- Some suggest SLNB (especially alveolar RMS)

CHEMOTHERAPY: Typically combination recommended, tailored by prognostic group
- VAC regimen: Vincristine, actinomycin/adriamycin, Cyclophosphamide

RADIATION (40-50Gy)
- Used for patients with persistent local disease
- Based on the volume of disease remaining after surgery and initial chemotherapy, location of tumor, structures in proximity, tumor histology
- Important for microscopic + gross residual primary disease, and for control of nodal disease
- Neck is typically not treated unless there is N+ disease

PROTOCOLS:
1. Resectable (with acceptable morbidity): Surgery + Chemo
2. Unresectable: Chemo (VAC) + XRT
- Restage 6-8 weeks post-treatment; then consider salvage treatment

Note on treatment based on location:
1. Orbit: Most common, treated with CRT (no surgery)
2. Parameningeal: worse prognosis due to SB/intracranial involvement 65-80%, treated with CRT ± surgery if resectable
3. Non-parameningeal: If superficial, more amendable to surgical excision

POST-TREATMENT PROTOCOL:
- CT, MRI or PET after completion of adjuvant therapy to evaluate treatment response
- Biopsy of unusual tissue or contrast enhancement at primary tumor site to rule out persistent disease
- Failure to respond to initial treatment is poor prognosis
- Prognosis based on staging; 50% who undergo salvage surgery die from disease

Question 61

Q

What are the prognostic factors for head and neck pediatric rhabdomyosarcoma?

Answer

A

Subtype (embryonal best)
Size (>5cm bad)
Subsite (favourable vs. unfavourable sites)
N+
M+
Gross margins positive (Grade 3 or 4)
Age > 10 years (typically get the bad subtype)
Fusion gene positive (PAX3 / PAX 7 : FOXO1 fusion gene positive is worse prognosis)

Question 62

Q

Regarding post-transplantation lymphoproliferative disorder (PTLD), discuss:
1. What is this?
2. What is the pathophysiology?
3. What is the clinical presentation? 3

Answer

A

PTLD: What is it?
- Umbrella term for all abnormal proliferations of lymphoid tissue in a transplant recipient
- Ranges from lymphoid hyperplasia to non-hodgkin’s lymphoma

PATHOPHYSIOLOGY:
- Usually manifests with B-cell proliferation induced by EBV
- Proliferation is left unopposed by the pharmacologically suppressed T-cell system

CLINICAL PRESENTATION:
1. Similar to mononucleosis
2. Tonsillar hypertrophy
3. Adenoid enlargement
4. Cervical adenopathy (common in H/N)

Question 63

Q

Risk factors for post-transplant lymphoproliferative disease? 5

Answer

A

RISK FACTORS:
1. Degree and type of immunosuppressive
2. EBV seronegative status at time of transplant
3. Young age
4. Donor-recipient mismatch & Graft V Host disease
5. T-cell depletion or use of anti-T-cell monoclonal antibodies

Question 64

Q

MGX of post-transplant lymphoproliferative disease? 9

Answer

A

MANAGEMENT:
1. Biopsy of tissue to diagnose and exclude lymphoma
2. Excision of obstructing lymphoid tissue (T+A) can be curative
3. Reduction or cessation of immunosuppression
4. Anti-viral treatment with Acyclovir or Ganciclovir to control Epstein-Barr virus (EBV) replication
5. Interferon alpha
6. IV immunoglobulin
7. Immunotherapy with donor T-lymphocyte infusion
8. Chemotherapy ± RT for lymphoma cases
9. Surgery to remove the transplanted organ (last line)

Answer 65

A

Mucous retention cyst = True cysts with an epithelial lining; typically results from ductal obstruction involving the minor salivary glands. Commonly seen on lips, buccal mucosa, tongue

Mucoceles = Represent mucous extravasation into the surroudning soft tissue; are not true cysts as they lack an epithelial lining

Answer 66

A

Ranula = mucocele (pseudocyst) that originates in most cases from obstruction or injury of the sublingual gland ducts

May be an initial and early manifestation of HIV infection

Classification
1. Congenital or acquired
2. Primary or recurrent

TYPES:
1. Simple ranula: Limited to floor of mouth
2. Plunging ranula: Extends through the mylohyoid muscle through a dehiscent mylohyoid into the neck/submental space

DIAGNOSIS:
1. FNA - Presence of amylase in aspirated fluid

TREATMENT OPTIONS:
1. Observation
2. Incision and drainage
3. Marsupialization
4. Sclerotherapy
5. Definitive: Resection of ipsilateral sublingual gland along the drainage of the ranula (reduces risk of recurrence)
- Complete excision of pseudocyst wall is not necessary
- Intraoral drainage vs. cervical incision (if large or revision)

Answer 67

A

CASTLE (CArcinoma Showing Thymic-Like differentiation)
SETTLE (Spindle-cell Epithelial Tumor with Thymic-Like differentiation) tumors

Answer 68

A

Left sided neck mass

Pathology: Hassall’s Corpuscles (epithelial rests within thymic medulla lymphoid tissue)

https://zoomify.luc.edu/lymphoid/dms117/02.gif

Answer 69

A

Pseudotumor of infancy = SCM tumor of infancy = congenital torticollis = fibromatosis coli

DEFINITION:
- Benign congenital inflammation of the SCM, presenting as a lateral neck mass

CLINICAL PRESENTATION:
- Painless, firm, fibrous mass in the substance of the sternocleidomastoid muscle
- Sternal head and the distal third of the muscle

EPIDEMIOLOGY:
- Age 2-4 weeks
- Occurs in 0.4% of all newborns

DIAGNOSIS:
- U/S
- Rule out all other worrisome causes

TREATMENT:
1. Conservative
- Controlled manual massage and stretching up to age 1 year will resolve majority of cases
- Physiotherapy with positioning exercises
2. Enlarges over 2-3 months, then resolves over 4-8 months
3. < 10% cases = Surgical SCM resection or Tenotomy/Tenomyotomy (Indications: Persistent after 12 months, develops facial asymmetry/plagiocephaly)
4. ± Botox

NATURAL HISTORY:
- Usually increases in size until 1 month
- Remains static for 2-3 months, then gradually diminishes in size and disappears over 4-8 months
- The resulting fibrosis of the SCM may lead to congenital muscular torticollis in 10-20%

PROGNOSIS:
1. 80% resolve with conservative management
2. 20% progress to torticollis requiring release
3. 10% associated hip dyplasia

Answer 70

A

Chronic granulomatous disease that involves the lymphatic network in and surrounding the salivary glands lead to salivary gland involvement
- Direct infiltration of adjacent gladnular parenchyma occurs in fulminant cases

Features:
- Asymptomatic gradual enlargement of nodule within the gland

Examples:
1. Tuberculous mycobacterial diseases
2. Nontuberculous mycobacterial disease
3. Actinomycosis
4. Cat-scratch disease (CSD)
5. Toxoplasmosis
6. Sarcoidosis

Answer 71

A

CAUSATIVE ORGANISM: Mycobacterium tuberculosis

EPIDEMIOLOGY:
- Increased frequency of resistant strains due to immigration from endemic nations + HIV
- 20% of TB is extrapulmonary
- Primary salivary TB is rare - involves the parotid gland
- Most common in older children and adults

TRANSMISSION:
1. Direct person-to-person contact

PATHOPHYSIOLOGY:
1. Primary salivary gland infection: Infection of a focus in the tonsil or gingivobuccal sulcus –> Ascends to the glands by way of the ducts –> salivary gland infection –> May spread to cervical nodes through lymphatic drainage
2. Primary cervicolymphadenitis –> lymphogenous spread to salivary gland –> hematogenous spread to distant focus
3. Mycobacteria are encapsulated in intraglandular lymph nodes –> might be reactivated many years after acute pulmonary infection

Submandibular gland more commonly involved gland after systemic TB infection

CLINICAL PRESENTATION:
Two different forms:
1. Acute inflammatory lesion with diffuse glandular edema (may be confused with sialadenitis or abscess)
2. Chronic, tumorous lesion seen as a discrete slow-growing mass that mimics neoplasm

Symptoms:
- Fever
- Night sweats
- Weight loss
- Involvement of facial nerve rare

DIAGNOSIS:
1. CXR (commonly negative; possible healed granulomatous disease)
2. CT of H/N TB demonstrates 3 different patterns
a. Early disease: Involved LN with nonspecific homogeneous enhancement
b. Nodal mass with central lucecy and thick rims of enhancement, minimally effaced fascial planes
c. Fibrocalcified nodes, usually in treated TB
3. Positive TB test
4. FNA biopsy (lower risk of draining fistula than incisional biopsy) - culture (6 weeks) and acid-fast smears

TREATMENT:
1. Triple drug therapy for 4-6 months: Isoniazid plus Rifampin, Pyrazinamide, Ethambutol
2. Surgical excision for resistant or unclear cases

Answer 72

A

CAUSATIVE ORGANISMS:
1. M. avium intracellular complex (MAC)
2. M. Scrofulaceum
3. M. Kansaii
4. M. Bovis (more common with unpasteurized milk)

EPIDEMIOLOGY:
1. Children < 5 years old more common
2. Immunocompromised

TRANSMISSION:
- Soil, water, domestic and wild animals, milk, other food items
- Incubation: weekends to months
- Symptomatic: weekends to months
- Not infectious

CLINICAL PRESENTATION:
- Rapidly enlarging and persistent parotid or neck mass failing to respond to antibiotics
- Skin adherent to surrounding tissues and develops characteristic violaceous discoloration
- May progress to fluctuation and draining sinus in advance stages
- Few systemic symptoms, generally well child
- Cervical lymphadenopathy commonly unilateral and high jugular nodes or preauricular areas

DIAGNOSIS:
1. Clinical
2. Fine needle aspiration - but risk of fistula formation
3. Mantoux test weakly positive - 5-9mm elevation (10mm diagnostic)
4. PCR
5. Culture - may take 6 weeks, frequently negative
6. U/S: Necrotic LN, calcifications in mature lesions

TREATMENT OPTIONS:
1. OBSERVATION: Will self-resolve after months, but may result in chronic draining fistula and scarring
2. MEDICAL: Biaxin (Clarithromycin) + Rifampin (but +++ long treatment course required, still may fistulize)
3. SURGICAL: Excision or I+D/currettage –> best for long-term cosmesis

MEDICAL OPTIONS:
1. Clarithromycin 15mg/kg/day divided BID, max 500mg per dose
- Side effects: GI symptoms, QL prolongation, ototoxicity, anterior uveitis
2. Azithromycin 5-12mg/kg/day (max 500mg per dose)
- GI symptoms, pruritus, prolonged QT
3. Ethambutol 15-25mg/kg/dose once daily; monitor ocular toxicity
- GI symptoms, optic neuritis (usually reversible), red-green color blindness
4. Rifabutin 10-20mg/kg/day (max 300mg)
- GI, orange discoloration of tears/urine/stool, hepatitis, renal failure, granulocytopenia, anterior uveitis

Answer 73

A

Systemic symptoms
- TB: Single large node with fevers and malaise in TB
- NTB: Rare
Lung disease
- TB: Often
- NTB: Rare
Violaceous skin changes
- TB: Not seen
- NTB: Seen often
PPD (Purified protein derivative)
- TB: Strongly positive
- NTB: Weak or negative
Treatment
- TB: Medical
- NTB: Often resistant to medical, therefore often require surgical excision

Answer 74

A

ETIOLOGY:
1. Gram-positive anaerobic bacilli
2. Mostly actinomyces israelii and A. Gerencseriae

EPIDEMIOLOGY:
1. Cervicofacial 55%
2. Abdominopelvis 20%
3. Pulmonothoracic 15%

CLINICAL PRESENTATION:
1. Slow-growing, nontender mass with multiple sinus tracts or advanced stage with pain and trismus associated with fever
2. Poor oral hygiene combined with trauma to the mucosa permits invasion of the organism
3. History of recent dental disease and manipultation common (common case: dental disease and dental extraction previously and then develops like a sinus tract with atypical mycobacterium)
4. Constitutional symptoms typically absent

DIAGNOSIS:
1. FNA histology - presence of sulfur granules and pathologic organisms; firm fibrous encasement of multiloculated abscess
2. CT / MRI for bone involvement

TREATMENT:
1. 6 week of antibiotics followed by 6 months of oral antibiotics for complete eradication
2. Penicillin is antimicrobial of choice (not resistant)
3. Other options: Clindamycin, Doxycycline, Erythromycin
4. Surgical excision for removal of extensive fibrosis and sinus tracts if poorly responsive to antibiotics

Answer 75

A

CAUSATIVE ORGANISMS:
1. Bartonella Henselae (gram-negative cellular bacillus)

EPIDEMIOLOGY
1. Most commonly inoculation caused by scratch trauma from domestic cat
2. 90% exposure to cat history, 75% cat scratch or bite
3. 5% dogs history
4. Head and neck second most common site after upper extremity

CLINICAL PRESENTATION
1. Papule or pustule at a scratch/bite site, followed in 1-2 weeks by development of LN in region of inoculation
2. Nodes enlarge for 1-2 weeks, may not resolve for 2-3 months (overall self-limiting)
3. Erythema, pain, spontaneous suppuration in 10-30%
4. Fever and mild systemic symptoms 30%
5. Common sites: submandibular, cervical, preauricular

DIAGNOSIS:
1. Antibodies to Bartonella Henselae - FISH and enzyme immunoassay
2. BIopsy - Histology demonstrating B. Henselae with use of Warthin-Starry silver lining

TREATMENT
1. Usually none - self-limiting within 2-4 months
2. Antibiotics for systemically ill or highly symptomatic - Rifampin, Erythromycin, Gentamycin, Azithromycin, Ciprofloxacin most effective (beta lactams not effective)

Answer 76

A

ATYPICAL PRESENTATIONS:
1. Parinaud oculoglandular syndrome (unilateral granulomatous conjunctivitis with preauricular/submandibular lymph node)
2. Vertebral osteomyelitis encephalitis
3. Granulomatous hepatitis
4. Optic neuritis
5. Baciliary angiomatosis (cutaneous proliferation of vascular lesions) - immunocompromised patients

POV BAG

Answer 77

A

CAUSATIVE ORGANISM:
Toxoplasma Gondii (may be trophozoite, cyst, and oocyst forms)

TRANSMISSION:
Host usually domestic cat (feline vector)
Human transmission via ingested undercooked meats, or cat feces

CLINICAL PRESENTATION:
1. Disseminated form (immunocompromised): Myalgia, lethargy, anorexia, hepatosplenomegaly, pericarditis, and myocarditis
2. Lymphadenopathic form: isolated cervical LN

DIAGNOSIS:
1. Histology - lymph node architecture preserved, hyperplastic follicles and germinal centres showing abundant mitoses and necrotic nuclear debris.
2. Epitheliod cells with abundant pale eosinophilic cytoplasm occur singly or in groups and are foudn in cortical and paracortical zones and sinuses
3. Serologic testing

TREATMENT:
1. Chemotherapy for progressive infections, or pregnant/immunocompromised individuals (Pyrimethamine, Trisulfapyrmidines)

Answer 78

A

DEFINITION: Formation of immune granulomas in various organs, especially lungs and lymphatic organs

EPIDEMIOLOGY:
1. Age 20-40s
2. Usually triggered by environmental or infectious event
3. Women > Men 1.3:1
4. Black > White ethnicity

CLINICAL PRESENTATION
1. Persistent dry cough
2. Eye or skin manifestations
3. Peripheral LN enlargement
4. Fatigue, weight loss, fever or night sweats
5. Erythema nodosum

SALIVARY INVOLVEMENT 5-10%; 3 PATTNERS OF PRESENTATION:
1. Most common: Major salivary gland swelling with xerostomia
2. Non-caseating granulomas on biopsy of minor salivary glands
3. Uvoparotid fever (Heerfordt syndrome) - chronic, febrile, enlargment of parotid glands, anterior uveitis and facial nerve palsy

DIAGNOSIS
1. Labs: Hypercalcemia, abnormal vitamin D3 metabolism within granulomatous lesions
2. 90% abnormal CXR - LN and pulmonary infiltrates
3. Biopsy (lip mucosa - minor salivary gland biopsy) - noncaseating granuloma, periductal amyloid deposits, anti-AA amyloid antibody immunostaining reveals amyloid deposits around acini - ??
4. Bronchoalveolar lavage: CD4/CD8 T-lymphocyte ratio > 3.5
5. Soluble angiotensin-converting enzyme level more than twice the normal value

TREATMENT:
1. Corticosteroids (lowest effective dosage)
2. May resolve spontaneously
3. Methotrexate

Answer 79

A

DEFINITION:
- Infantile acute febrile mucocutaneous lymph node syndrome
- Multisystem autoimmune acute vasculitis in infants, involvement mucous membranes, skin, lymph nodes, cardiac system, and neurologic system
- Common affects kids < 5 years old
- Etiology unknown

CLINICAL PRESENTATION: CRASH & BURN - Must have fever > 5 days and 4/5 of the main clinical fevers
1. Fever > 5 days
2. Conjunctivitis - bilateral, nonexudative, painless bulbar conjunctival injection
3. Rash - polymorphous generalized rash
4. Erythema of palms and soles, followed by membranous desquamation of the finger and toe tips
5. Adenitis - acute non-purulent cervical LN with diameter > 1.5cm, usually unilateral
6. Oropharyngeal/Mucosal changes (strawberry tongue) - erythema, fissuring, crusting of lips, strawberry tongue

Other features:
1. Facial paralysis (marker for increased severity)
2. Aspetic meningitis and irritability
3. Coronary aneurysms 15-20%
4. Acalculous cholecystitis

INVESTIGATIONS:
1. Clinical diagnosis
2. ECHO to r/o coronary aneurysms
3. ESR + CRP
4. Abdominal U/S (for cholecystitis)

TREATMENT:
1. Supportive care
2. High dose ASA for coronary aneurysms
3. IVIg
4. Others: Steroids, methotrexate, cyclophosphamide, anticoagulants in aneurysm patients

Answer 80

A

RISK FACTORS:
1. XRT - exposure to 20-29Gy (especially CNS tumors)
2. Family history - genetic testing for suspected familial cases
3. MEN 2a/b
4. Gardner syndrome (FAP) - colonic polyps, osteomas, PTC
5. Cowden’s syndrome - PTC, trichelimommas, hamartomas
6. History of leukemia or Hodgkin’s lymphoma

EPIDEMIOLOGY:
- 10x more common in teens compared to younger children
- F:M 5:1
- Nodules in 2% of pediatrics, 50% malignancy if clinically significant
- More likely to have multifocal and bilateral disease

GENETICS:
- BRAF is RARE in pediatrics
- More common: RET/PTC

CLINICAL FEATURES:
- 85% Papillary thyroid cancer (classic, solid, follicular, diffuse, sclerosing)
- 25% hematogenous spread of PTC
- 25% of solitary nodules are malignant (vs. 5% in adults)
- 70% present with nodal metastases (vs. rare in adults)
- 20% present with distant metastases (rare in adults)
- Good prognosis despite more aggressive initial presentation - long term survival over 90%

WORKUP:
1. FNA biopsy (ultrasound guided)
2. Thyroid function testing
3. Calcitonin & CEA levels - rule out medullary thyroid cancer
4. Genetics: RET oncogene - rule out MEN 2A/B

Answer 81

A

ULTRASOUND FINDINGS: Concerning for malignancy if:
1. Size >4cm
2. Microcalcifications
3. Irregular or infiltrative margins
4. Hypoechogenicity
5. Subcapsular localization
6. Increased intranodular vascularity
7. Enlarged regional lymph nodes

Answer 82

A

Biopsy if ≥1cm and suspicious ultrasound features
Nodules < 1cm generally not biopsied unless specific concerning features on ultrasound

Indications for removal of benign nodules:
1. Suspicious Biopsy Results:
2. Rapid Growth
3. Compressive symptoms
4. Genetic Syndromes/Family History:
5. Large (>4cm) or Multiple Nodules:
6. Patient/Family Preferences

TREATMENT:
1. Benign cytology - manage with serial US and repeat FNAB for enlargement or concerning change in appearance
2. Non-diagnostic, indeterminate, or suspicious cytology - repeat FNAb, molecular genetic testing, or surgery
3. Diagnostic hemithyroidectomy - completion if positive
4. Therapeutic total thyroidectomy (generally if high risk cancer, large, or genetic disease) with central ND for positive; +/- lateral ND

Answer 83

A

ROLE OF POST-OPERATIVE RAI (improves DFS, but controversial for small Stage I lesions):
- Distant metastases
- Large tumors (T3 or greater)
- Extensive regional nodal involvement

Notes:
- No role in medullary thyroid cancer (does not uptake iodine)
- Dosage for residual disease based on body weight: 1-1.5 mCi/Kg; 37-56Mbq/kg
- Risks: gonadal damage, xerostomia, pulmonary fibrosis, menstruation abnormalities, decreased sperm count (avoid conception at least 4 months, sperm banking considered if cumulative activity > 400mCi), secondary cancers including leukemia

Secondary Malignancies (increased RR 1.16 with I-131 treatment, increased mortality compared to general population):
1. Leukemia
2. Stomach cancer
3. Bladder cancer
4. Colon cancer
5. Salivary gland cancer
6. Breast cancer

POST-OPERATIVE CARE:
1. Thyroid hormone replacement
2. Serum thyroglobulin levels to monitor for tumor recurrence
3. Imaging if abnormal thyroglobulin or nodal masses noted
4. PTC can recur up to 40 years later, so these patients should be followed for life!

Answer 84

A

All are Stage 1, unless they have distant mets (M1)

Answer 85

A

MEN1 (PPP):
1. Parathyroid adenomas
2. Pituitary adenomas
3. Pancreatic adenomas

MEN2A (PPM):
1. Pheochromocytoma
2. Parathyroid adenoma
3. Medullary thyroid carcinoma
4. Lichenoid amyloidosis

MEN2B (PMM):
1. Pheochromocytoma
2. Medullary thyroid carcinoma
3. Mucosal neuromas/Marfanoid features

Answer 86

A

Rare entity
Lack of data - strong recommendations regarding therapy cannot be made for who would benefit from thyroid surgery vs. I-131 therapy

Special considerations:
- In all children, consideration should be given to genetic counselling and genetic testing for PTEN mutations, particularly with macrocephaly or a family history suggestive of the PTEN hamartoma tumor syndrome (higher risk of follicular thyroid carcinoma)

Answer 87

A

MEN2A: Total thyroidectomy at age < 5 years

MEN2B: Total thyroidectomy at age < 1 years

Answer 88

A

Most common extracerebral solid tumors of infancy/childhood
White > Nonwhite children
2-5% are in the head/neck region (most common site is adrenal medulla or adjacent retroperitoneum)
50% under age 1, 80% under age 5

Answer 89

A

Develops wherever sympathetic nervous tissue is found (embryonal tumors arising from primitive neuroectodermal cells of neural crest origin)
Primary tumors occur paravertebrally along the sympathetic chain
Symptoms depend on the location of the primary tumor
Unusual characteristic of this tumor is spontaneous regression (typically in infants with small primary tumors, liver involvement, subcutaneous nodules, and patchy bone marrow infiltration without major replacement of hematopoietic cells)

Answer 90

A

Types of presentations: “PHILO”
Paraplegia
Heterchromia
Ipsilateral ptosis
Laryngotracheal
Orbital neuroblastoma

Laryngotracheal or pharyngeal compression from cervical masses –> airway obstruction, dysphagia, aspiration
Ipsilateral ptosis if superior cervical ganglion involved
Orbital neuroblastima –> periorbital ecchymosis “panda eyes” and ocular muscle paresis
Heterchromia irides - sympathetic nervous system is intemiately associated with development and maintenace of eye color
Paraplegia if neuroblastoma extends through adjacent intervertebral foramina into spinal cord (common with mediastinal and retroperitoneal tumors)

Answer 91

A

STAGE 1: Localized tumor with complete gross resection, with or without microscopic residual disease; representative ipsilateral lymph nodes negative for tumor microscopically (nodes attached to and removed with the primary tumor may be positive)

STAGE 2A: Localized tumor with incomplete gross excision; representative ipsilateral nonadherent lymph nodes negative for tumor microscopically

STAGE 2B: Localized tumor with or without complete gross excision with ipsilateral nonadherent nodes positive for tumor (enlarged contralateral lymph nodes negative microscopically)

STAGE 3: Unresectable unilateral tumor infiltrating across the midline, with or without regional LN involvement; or localized unilateral tumor with contralateral regional LN involvement, or midline tumor with bilateral extension by infiltration (unresectable) or by LN involvement

STAGE 4: Any primary tumor with dissemination to distant LN, bone, bone marrow, liver, skin, or other organs (except as defined for 4S)

STAGE 4S: Localized primary tumor (defined for stages 1, 2A, or 2B) with dissemination to skin, liver, or bone marrow (limited to infants < 1 years old)

Answer 92

A

Neuroendocrine tumor
Small round blue cell tumor
Arises from neural crest cells
Stains (for all neural crest cell tumors - paraganglioma, medullary thyroid cancer)
a. Chromogranin A
b. Synaptophysin
c. NSE (Neuron specific enolase)

Answer 93

A

90% secrete homovanillic acid (HVA) or vanillylmandelic acid (VMA) - catecholamines
MYCN oncogene (25%) - most aggressive
ALK - familial cases
Hirschsprung’s disease

Answer 94

A

24 hour urine catecholamines (to detect HVA/VMA)
CT and MRI - Neck/Chest/Abdo/Pelvis - rule out mets
Bone scan
Bone marrow aspirate and biopsy - r/o mets
MIBG Scintigraphy (I-123 metaiodobenzylguanidine) - r/o mets
Biopsy - genetic subtyping

Answer 95

A

Age at diagnosis (older age)
INSS stage
Tumor histopathology
DNA content of tumor
MYCN amplification
Genetics subtypes

Overall survival: 90%
High risk with intensive treatment: 35%

Answer 96

A

Determined by stage of disease and sites of involvement

LOW RISK/STAGE I: Surgical removal (or observation - may spontaneously regress)

INTERMEDIATE RISK: Surgery + chemotherapy

HIGH RISK: Induction chemo, surgery, adjuvant chemo/XRT, bone marrow rescue

Surgery involves:
- Excisional biopsy Stage II-IV
- Resection of involved cervical sympathetic ganglion
- Salvage resection after chemotherapy can be considered if initial resection cannot resect fully without acceptable morbidity

Answer 97

A

BENIGN:
1. Hemangioma (most common)
2. Pleomorphic adenoma
3. Lymphangioma
4. Neurogenic

MALIGNANT:
1. Mucoepidermoid carcinoma (50% most common)
2. Acinic cell
3. Lymphoma

Other:
4. Adenocarcinoma
4. Undifferentiated carcinoma
5. Adenoid cystic

Most common location = parotid gland

Answer 98

A

A. BENIGN
- Slow-growing asymptomatic firm mass

Mesenchymal Origin
1. Hemangiomas
2. Lymphatic vascular malformations

Epithelial Origin
1. Pleomorphic adenoma
2. Warthin’s tumor
3. Oncocytoma
4. Monomorphic adenoma

Other
1. Plexiform neurofibroma
2. Embryoma
3. Lipoma
4. Teratoma
5. Sialoblastoma

B. MALIGNANT
- Older children and adolescents
- Usually occurs as an asymptomatic firm mass
- Low incidence of facial paralysis and metastasis

Mesenchymal Origin
1. Rhabdomyosarcoma

Epithelial Origin
1. Mucoepidermoid carcinoma
2. Acinic cell carcinoma
3. Adenocarcinoma
4. Undifferentiated carcinoma
5. Adenoid cystic carcinoma
6. Squamous cell carcinoma
7. Carcinoma ex pleomorphic

C. OTHER
1. Pyogenic granuloma

Characteristics:
- May occur at any age
- Typically well demarcated
- Reactive to stimulus

Answer 99

A

Epidemiology:
- 86% parotid (most submandibular and minor salivary gland tumors are benign)
- 50% of pediatric salivary neoplasms are malignant
- 86% moderate to well differentiated

MOST COMMON:
1. Mucoepidermoid (40%)
2. Acinic cell carcinoma (37%)
3. LYmphoma

RISK FACTORS:
- Kids radiated for other malignancies have higher risk of secondary mucoepidermoid

Answer 100

A

DEFINITION:
- Histiocytoses = group of diseases defined by pathologic behaviour of cells regularly involved in phagocytosis and antigen presentation
- Uncontrolled proliferation of histiocytes (monocytes & macrophages)
- Aka. Langerhans cell histiocytosis
- Aka. Reticuloendotheliosis

CLINICAL PRESENTATION (varied, but in general)
1. Skin rash
2. Painful lytic bone lesion (most common skull) - can also be painless
3. Fever, weight loss, diarrhea, edema, dyspnea, polydipsia, polyuria

OLD CLASSIFICATION
1. MONOSTOTIC / UNIFOCAL = Eosinophilic Granuloma (ages 5-15yo)
- Localized lesion, responds to topical/injected steroids
- Limited to bone (could be monoostotic or polyostotic within bone), cutaneous granulomas
- Slowly progressive, spontaneous remission
2. POLYOSTOTIC / MULTIFOCAL = Hand-Schuller-Christian Disease (ages 2-10yo); Triad of:
- Lytic skull lesions (scalp, EAC, otic capsule, etc.)
- Exophthalmos (secondary to orbital bone involvement)
- Diabetes Insipidus (secondary to pituitary stalk involvement)
3. FULMINANT (Multifocal, multisystem) = Letterer-Siwe Disease (age < 2 yo)
- Infantile, rapidly progressive, 50% fatal
- Diffuse organ/tissue involvement –> purpuric rash

WHO CLASSIFICATION
1. CLASS I: Langerhan Cell Histiocytosis (LHC)
- Presentation: Focal lesion
- Histology: Langerhan cells with cleaved nuclei, Birbeck granules
- Stains: S-100, CD1a surface antigen
- Treatment: Excision, curettage, topical steroid, intralesional steroid, Low dose XRT, immunosuppressives (methotrexate, cyclophosphamide) if persistent

CLASS II: Hemophagocytic Lymphohistiocytosis (HLH)
- Overwhelming erythrophagocytosis by macrophages
- Rapidly fulminant & fatal despite chemo
CLASS III: Malignant Histiocytosis
- Histiocytic lymphoma/leukemia
- Uncontrolled proliferation of monocyte precursors
- Treatment: chemo (vincristine + prednisone; or cladribine)

DIAGNOSTIC FINDINGS:
1. Biopsy: Birbeck granules
2. Stains: S100, CD1a positive

PROGNOSIS
- Usually limited, very treatable
- 15% progress to fulminant, fatal disease

TREATMENT:
1. Systemic steroids
2. Methotrexate, Thalidomide, Chemotherapy, XRT
3. Local curettage of bone OK, but no role for aggressive surgery

Answer 101

A

ACUTE:
1. Mucositis
2. Otitis externa
3. Alopecia
4. Transverse myositis

LONG-TERM:
1. 2nd primary cancer (usually 15-20 years later): < 5%
2. Growth retardation: 15-30%
3. Dental: caries, arrested growth, malocclusion
4. Thyroid dysfunction, endocrinopathies
5. SNHL

Answer 102

A

VITAMIN C

A. INFLAMMATORY - ACUTE
1. Bacterial: lymphadenitis or suppurative adenitis (Staph, Strep viridans, Anaerobes)
2. Viral: Mumps, EBV, HIV, Coxsackivirus A, Echovirus, CMV, parainfluenza, Adenovirus

B. INFLAMMATORY - CHRONIC
1. Obstructive
- Mucocele/ranula
- Sialolithiasis
- Sialectasis
2. Granulomatous
- Atypical mycobacteria
- Cat-scratch disease
- Actinomycosis
- Sarcoidosis
- Toxoplasmosis
- Histoplasmosis

C. CONGENITAL
1. Agenesis
2. Branchial cleft cyst or fistula
3. Dermoid
4. Ductal cyst
5. Ranula (can also be congnital)

D. NEOPLASTIC - BENIGN
1. Pleomorphic adenoma
2. Warthin’s tumor
3. Lipoma
4. Monomorphic adenoma
5. Neurofibroma

E. NEOPLASTIC - MALIGNANT
1. Primary
2. Mucoepidermoid carcinoma
3. Acinic cell carcinoma
4. Adenocarcinoma
5. Adenoid cystic carcinoma
6. Lymphoma
7. Rhabdomyosarcoma
8. Metastatic

F. VASCULAR MALFORMATIONS
1. Hemangioma
2. Lymphatic malformation
3. Venous anomaly
4. Arteriovenous malformation

G. AUTOIMMUNE
1. Sjogren syndrome
2. Benign lymphoepithelial disease
3. Wegener Granulomatosis
4. Sarcoidosis

H. TRAUMATIC
1. Blunt
2. Penetrating
3. Radiation induced

Answer 103

A

Viral parotitis (including mumps)
Obstructive sialadenitis due to stones
Juvenile recurrent parotitis

Answer 104

A

ETIOLOGY:
1. Inflammatory idiopathic non-obstructive sialectasia (dilation of salivary ducts)

ORGANISMS:
1. Staph aureus
2. Strep Viridans
3. Note however: Classifically thought to be inflammatory not infectious (no pus clinically)

NATURAL COURSE:
- Onset ~ 4 years old
- 3-4 attacks/year, usually self-limiting
- Usually resolves completely by puberty
- Non-purulent secretions from duct (compared to purulent in bacterial parotitis)

HISTOLOGY:
1. Lymphocytic infiltrate
2. Ductal dilation
3. Ductal epithelial hyperplasia and metaplasia
4. Fibrosis

SIALOGRAPHY FINDINGS (X-ray of salivary ducts and glands)
1. Punctate Sialectasis (proximal duct dilation with cystic cavities)
2. Distal duct stricturing
3. Extravasation of secretions into parenchyma
4. No clear obstruction

INDICATIONS FOR SIALOENDOSCOPY:
1. 2 episodes in 6 months or 2 in 1 year

SIALENDOSCOPY FINDINGS:
- White appearance of the ductal layer without visible layer of healthy blood vessels
- May have debris within duct

Answer 105

A

TREATMENT (No consensus)
1. Symptomatic
- Analgesics
- Massage
- Sialagogues
- Warm compresses
- Hydration
2. Medical
- Clavulin or Penicillin
- Antibiotics controversial as its commonly felt to have inflammatory etiology, not infectious
3. Sialendoscopy with duct irrigation with saline ± corticosteroids (eliminate or drastically reduces number of recurrences of acute episodes)
4. Surgical (extreme cases, or persisting in adulthood)
- Parotidectomy
- Tympanic neurectomy (eliminates parasympathetic activity in parotid gland, for treating cases of persistent parotid fistulae)
- Can also just irrigate the duct in the OR

Answer 106

A

Benign, self-limited inflammatory salivary gland lesion caused by Ischemic changes of minor salivary gland acini
- Common at the junction of the hard and soft palate
- Painless ulcerated lesion or nodular swelling
- May look like a malignancy
- Heals spontaneously in 2-3 months

https://www.mdpi.com/medicina/medicina-56-00188/article_deploy/html/images/medicina-56-00188-g001.png

Answer 107

A

See Cummings Chapter 205 Figure 205.1 and 205.2

Or Kevan Pediatrics Question 136

Answer 108

A

DEFINITION:
- Systemic autoimmune disease characterized by infiltration of glandular tissue by predominantly CD4+ T-lymphocytes and B-cell activation with autoantibody production

CLINICAL PRESENTATION
- Recurrent swelling of the salivary glands (most common = parotid)
- Keratoconjunctivitis sicca and xerostomia

DIAGNOSIS:
1. Minor salivary gland biopsy
2. Ultrasound
3. Lab work: Elevated ESR, Autoantibodies to anti-Ro/SS-A and Anta-La/SS-B antigens, antinuclear antibody, rheumatoid factor, hypergammaglobulinemia

TREATMENT:
1. Symptomatic
2. Botulinum toxin injection

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