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General Pathology > Exam #3: Diseases of Childhood & Infancy III > Flashcards

Exam #3: Diseases of Childhood & Infancy III Flashcards

1
Q

What are the four different types of fibrous tumors in infants?

A
  • Infantile myofibromatosis
  • Aggressive infantile fibromatosis
  • Infantile digital fibroma
  • Congenital infantile fibrosarcoma
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2
Q

What is an infantile myofibromatosis?

A

Benign fibrous tumor in which cells express muscle-specific actin

*****This is the most common fibrous tumor in infants

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3
Q

What is an aggressive infantile fibromatosis?

A

Myofibroblast tumor that infiltrates skeletal muscle

**Note that despite infiltration, this is a benign tumor b/c it does not metastasize

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4
Q

What is a fibrosarcoma?

A

Malignant fibrous tumor in infants

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5
Q

What is a teratoma?

A

Germ cell neoplasm

  • Most are sacrococcygeal
  • Contain multiple different types of tissue
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6
Q

What is the most common solid tumor in the newbown? What sex is it more common in?

A
  • Teratoma
  • Girls

*****Benign mostly, ~10 % are malignant

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7
Q

What is the most common SOLID congenital MALIGNANCY?

A

Congenital neuroblastoma

*****Note this is vs. Teratoma, which is the most common benign solid tumor

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8
Q

Describe the microscopic appearance of a teratoma.

A

Contain multiple germ layers i.e.

  • Epithelial cells
  • Fibroblasts
  • Cartilage
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9
Q

What is the second leading cause of death in kids 5-14?

A

Malignancy

**Note that accidents are the leading cause of death in kids from 5-14

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10
Q

What is a neuroblastoma?

A

Malignant tumor of primitive sympathetic cells (derived from primordial neural crest cells)

  • Mainly from adrenal medulla & sympathetic ganglia
  • 10 % of childhood cancer

**This is the most common malignant solid tumor in kids

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11
Q

Describe the clinical presentation of a neuroblastoma.

A

1) Abdominal mass
- 40% are tumors of the adrenal medulla
- 25% paravertebral sympathetic chain in the abdomen
2) Weight loss
3) Respiratory distress
4) Proptosis
5) Periorbital ecchymosis

**Note that proptosis & periorbital ecchymosis are a fxn of the periorbital region being a common metastatic site

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12
Q

What are the three major sources of childhood malignancies?

A

1) Hematopoietic
2) Nervous
3) Renal
4) Adrenal

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13
Q

What is “blueberry muffin baby” pathognomonic for?

A

Neuroblastoma–this is most commonly seen in neonates where disseminated neuroblastomas have invaded the skin causing a deep blue discoloration

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14
Q

What is proptosis?

A

Bulging eye

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15
Q

What is the most common primary malignant tumor of the kidney in children?

A

Wilms tumor

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16
Q

How is neuroblastoma diagnosed?

A
  • Increased catecholamines in blood or catecholamine metabolites in urine
  • Blood neuron-specific enolase (NSE)
  • Tumor/ bone marrow biopsy for NSE staining
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17
Q

What are the catecholamine metabolites found in urine?

A
  • Vanillylamandelic acid (VMA)

- Homovanillic acid (HVA)

18
Q

What is “blueberry muffin baby” pathognomonic for?

A

Neuroblastoma

19
Q

What are the different syndromes associated with Wilms tumors?

A

WAGR syndrome

  • Aniridia (no iris)
  • Genital anomalies
  • Retardation

Denys-Drash Syndrome

  • Nephropathy leading to renal failure
  • Gonadal dysgenesis (male pseudohermaphroditism)
  • Gonadoblastoma
20
Q

What are the hallmarks of neuroblastoma on microscopy?

A

1) Small blue round cells
2) Rosette structure
3) Dense core neurosecretory granules*

*EM

21
Q

What is the most common primary malignant tumor of the kidney in children?

A

Wilms tumor

22
Q

What is the mutation associated with Denys-Drash Syndrome? What percent have Wilms Tumor?

A

WT1 (Dominant negative)

90%

23
Q

What is the prognosis of Wilms Tumor?

A

Very good w/ nephrectomy & chemotherapy

24
Q

What are the clinical manifestations of a Wilms Tumor?

A

Abdominal mass
Hematuria
Fever
HTN

25
Q

What are the two most common tumors that present as a mass in a child? What are the clinical implications?

A

Neuroblastoma
Wilms tumor

*Must differentiate with imaging or biopsy & need to evaluate function of contralateral kidney

26
Q

Describe the microscopic appearnace of a Wilms tumor. How does it differ from neuroblastoma?

A

Tightly packed blue cells with blastemal component & interspersed primitive tubules

**NOT ROSETTE

27
Q

What is the mutation associated with WAGR Syndrome? What percent have Wilms Tumor?

A

Del 11p13 WT1

  • 33%
28
Q

What is the mutation associated with Denys-Drash Syndrome? What percent have Wilms Tumor?

A

WT1

90%

29
Q

What is the prognosis of WIlms Tumor?

A

Very good w/ nephrectomy & chemotherapy

30
Q

What is a Rhabdomyosarcoma?

A

Most common sarcoma of childhood

31
Q

Describe the microscopic appearance of Embryonal Rhabdomyosarcoma.

A

Malignant cells ranging from primitive & round to spindled eosinophilic

32
Q

What are the three subtypes of Rhabdomyosarcoma? Prognosis?

A 
Embryonal Rhabdomyosarcoma (60%)
- Sarcoma botryoides--best prognosis

Aleolar Rhabdomyosarcoma (20%)

Pleomprphic Rhabdomyosarcoma (20%)
- worst prognosis
33
Q

Describe the microscopic appearance of Embryonal Rhabdomyosarcoma.

A

Malignant cells ranging from primitie & round to spindled eosinophilic

34
Q

How is rhabdomyoblastic differentation confirmed?

A

IHC staining for myogenin

35
Q

Describe the microscopic appearance of Aleolar Rhabdomyosarcoma.

A

Tumor is tavered by a network of fibrous septae that divide the cells into clusters or aggregates creating the appearnce of a pulmonary alveoli

36
Q

Describe the microscopic appearance of Pleomprphic Rhabdomyosarcoma.

A

Numerous large bizarre eosinophilic tumor cells

37
Q

What are the features of Fetal Alcohol Syndrome?

A
  • Growth Retardation
  • Microcephaly
  • Short palpebral fissures
  • Maxillary hypoplasia
  • Atrial septal defect
38
Q

What is a NTD?

A

Neural tube defect= opening in the spinal cord or brain early in development

39
Q

What dietary supplement is associated with reducing NTD?

A

Folic acid supplementation

40
Q

How do you screen for a NTD?

A

Maternal AFP

41
Q

What is the indicator for fetal lung maturity?

A

Surfactat

42
Q

What do you test to tell fetal lung maturity?

A

Fetal amniotic fluid–L/S ratio i.e. Lecithin-sphingomyelin ratio

General Pathology (33 decks)

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