Malignancies Flashcards
16 y/o male
weight loss over the past 3 mos
body malaise, (+) night sweats and intermittent febrile episodes
dry cough, no dyspnea, occasional episodes of epistaxis
(+) episode of flu 2 wks ago
pale palpebral conjunctiva, pale buccal mucosa
(+) multiple nontender cervical and supraclavicular lymphadenopathy
Hodgkin lymphoma
MC solid tumor in children
brain tumor
2nd MC malignancy after leukemia
The MC astrocytoma in children
pilocytic astrocytoma
Classic site of occurrence of astrocytoma
cerebellum
optic pathway region
MC posterior fossa tumor
cerebellar astrocytoma
Classic but not exclusive neuroradiologic finding in pilocytic astrocytoma
the presence of a contrast-enhancing nodule within the wall of a cystic mass
Condensed masses of glial filaments occurring in compact areas
Rosenthal fibers
2nd most common astrocytoma
consists of a group of tumors characterized by a pattern of diffuse infiltration of tumor cells amidst normal neural tissue
diffuse astrocytoma (DA)
predilection to supratentorial locations
Occurs most commonly in the HYPOTHALAMIC/OPTIC CHIASMIC REGION and carries a high risk of local as well as cerebrospinal spread
affects young children and infants
WHO grade II tumor
Pilomyxoid astrocytoma
Manifestations of Astrocytoma
headache, seizure, weakness, memory loss, motor weakness, visual symptoms, personality changes
Management of Astrocytoma
surgical resection
radiotherapy and chemotherapy
The MC group of MALIGNANT CNS tumors of childhood, accounting for approximately 20% of pediatric CNS tumors
Embryonal tumors or primitive neuroectodermal tumors (PNETs)
Accounts for 90% of embryonal CNS tumors
Cerebellar tumor occurring predominantly in MALES and at a median age of 5-7 yr
Medulloblastoma
2nd MC posterior fossa tumor of childhood
Manifestations of Medulloblastoma
obstruction of the 4th ventricle –> increased ICP - headache, vomiting, nausea, altered mental status, HPN
cerebellar dysfunction - gait, ataxia, dysmetria, poor balance
Management of Medulloblastoma
surgical resection
radiotherapy and chemotherapy
Common tumor of childhood, accounting for 7–10% of all childhood tumors
Solid or solid-cystic tumors from remnants of Rathke pouch of the pituitary stalk in the SUPRASELLAR region
Craniopharyngioma
adamantinomatous CP
papillary CP
Manifestations of Craniopharyngioma
visual deficits that can be secondary to compression of optic chiasm
Management of Craniopharyngioma
surgical resection
radiotherapy
The MC primary malignant bone tumor in children
and adolescents
Osteosarcoma
METAPHYSIS
lungs and bone - common site of metastases
Familial cancer syndrome associated with germline mutations of the P53 gene
Li-Fraumeni syndrome
Rare condition associated with short stature, skin telangiectasia, small hands and feet, hypoplasticity or absence of the thumbs, and a high risk of osteosarcoma
Rothmund-Thomson syndrome
4 pathologic subtypes of conventional high-grade osteosarcoma
osteoblastic
fibroblastic
chondroblastic
telangiectatic
The MC presenting manifestations of osteosarcoma
pain, limp, and swelling
The MC presenting manifestations of osteosarcoma
pain, limp, and swelling
initial complaints may be attributed to a sports injury or sprain
any bone or joint pain not responding to conservative therapy within a reasonable time should be investigated thoroughly
Classic radiographic appearance of osteosarcoma
sunburst pattern
Differential diagnosis of a lytic bone lesion
histiocytosis
Ewing sarcoma
lymphoma
bone cyst
Treatment of osteosarcoma
MAP - the standard chemotherapy regimen for osteosarcoma
METHOTREXATE
DOXORUBICIN
CISPLATIN
treat patients with preoperative chemotherapy in an attempt to facilitate limb salvage operations and to treat micrometastatic disease immediately
chemotherapy and surgery, 5 yr disease-free survival of patients with nonmetastatic extremity osteosarcoma is 65–75%
long-term follow-up of patients with osteosarcoma is important to monitor for late effects of chemotherapy,
such as cardiotoxicity from anthracycline and hearing loss from cisplatin
2nd MC primary malignant bone tumor
undifferentiated sarcoma of bone, also may arise from soft tissue
Ewing Sarcoma
DIAPHYSIS
lungs and bone - common site of metastases
Primary tumors arising in the chest wall
Askin tumors
Manifestations of Ewing sarcoma
pain, swelling, limitation of motion, and tenderness over the involved bone or soft tissue
often associated with systemic manifestations, such as fever and weight loss, and may be accompanied
by elevated inflammatory markers
Radiographic appearance of a primarily lytic bone lesion with periosteal reaction in Ewing sarcoma
onion-skinning
Treatment of Ewing sarcoma
Vincristine Doxorubicin Cyclophosphamide Etoposide Ifosfamide
irradiation or surgery
The MC extracranial solid tumor in children
The most commonly diagnosed malignancy in infants
Neuroblastoma
Median age of children at diagnosis of neuroblastoma
22 mo
90% of cases - diagnosed by 5 yr of age
Derived from primordial neural crest cells, form a spectrum with variable degrees of neural differentiation, ranging from tumors with primarily undifferentiated small round cells
(neuroblastoma) to tumors consisting of mature and maturing schwannian stroma with ganglion cells (ganglioneuroblastoma or ganglioneuroma)
Neuroblastoma
Massive involvement of the liver with metastatic disease, with or without respiratory distress
Pepper syndrome
Unilateral ptosis, myosis, and anhidrosis associated with a thoracic or cervical primary tumor.
Symptoms do not resolve with tumor resection
Horner syndrome
Approximately half of neuroblastoma tumors arise
ADRENAL GLANDS
most of the remainder originate in the paraspinal sympathetic ganglia
Metastatic spread - more common in children >1 yr
old at diagnosis
local invasion or distant hematogenous or lymphatic routes
Manifestations of neuroblastoma
abdominal mass that crosses the midline
abdominal pain
proptosis
periorbital ecchymosis - raccoon eyes
Horner syndrome - unilateral ptosis, myosis and anhidrosis
subcutaneous tumor nodules
opsoclonus/myoclonus (dancing eyes, dancing feet)
MC sites of metastases (neuroblastoma)
long bones and skull BM liver lymph nodes skin
Diagnosis of Neuroblastoma
abdominal CT scan - hemorrhage and calcification
24 hr urine catecholamine metabolites homovanillic acid (HVA) and vanillylmandelic acid (VMA) - elevated in ~95% of patients and help to confirm the diagnosis
Diagnosis of Neuroblastoma
abdominal CT scan - hemorrhage and calcification
24 hr urine catecholamine metabolites homovanillic acid (HVA) and vanillylmandelic acid (VMA) - elevated in ~95% of patients and help to confirm the diagnosis
bone marrow - myelophthisic marrow
biopsy
International Neuroblastoma Risk Group (INRG) Staging System (INSS)
L1 tumors (previously classified as INSS stage 1) - localized and confined to 1 body compartment without any IDRFs
L2 tumors (previously classified as INSS stages 2 and 3) - localized tumors with the presence of IDRFs.
M (previously classified as INSS stage 4) - Disseminated tumors with metastases
to bones, bone marrow, liver, distant lymph nodes, and other organs
Stage MS (previously stage 4S) - refers to neuroblastoma in children <18 mo old with dissemination to liver, skin, or bone marrow without bone involvement and with a primary tumor that would otherwise be staged as L1 or L2
2 y/o male abdominal swelling occasional jerky movements abdominal mass cross the midline bluish subcutaneous nodules palpated
Neuroblastoma
Treatment for children with low-risk neuroblastoma
SURGERY for stages L1 and L2
observation for asymptomatic stage MS, with cure rates generally >90% without further therapy
Treatment of intermediate-risk neuroblastoma
Surgery, chemotherapy, and in some cases, radiation therapy
CHEMOTHERAPY cisplatin or carboplatin cyclophosphamide etoposide doxorubicin
High-risk neuroblastoma
poor long-term survival rates between 25% and 35% with treatment that consisted of intensive chemotherapy, high-dose chemotherapy with autologous stem cell rescue, surgery, radiation, and 13-cis-retinoic acid (isotretinoin, Accutane)
The MC primary malignant RENAL TUMOR of childhood
The 2nd MC malignant abdominal tumor in childhood after neuroblastoma
Wilms tumor (nephroblastoma)
2-5 y/o
Wilms tumor is associated w/
neurofibromatosis
Beckwith-Wiedemann syndrome - hemihypertrophy, visceromegaly, macroglossia)
WAGR syndrome - Wilms tumor, aniridia, GU abnormalities, mental retardation)
Manifestations of Wilms tumor
painless abdominal enlargement w/ flank mass that does NOT cross the midline
hematuria
HPN
Diagnostics of Wilms tumor
abdominal UTZ, CT scan or MRI
electrolytes, renal function tests
chest x-ray or CT scan
Treatment of Wilms tumor
COG - advocates upfront surgery prior to initiating
treatment
SIOP - recommends preoperative chemotherapy
asymptomatic 2 y/o
abdominal mass on the L flank
IVP - enlarged kidney w/ distorted pelvis and calyces
Wilms tumor
