Robbins Ch 10

Question 1

What is the most common extracranial solid tumor of childhood and most frequently diagnosed tumor in infancy?

Answer 1

Neuroblastoma

Question 2

What germline mutations are associated with neuroblastoma?

Answer 2

Anaplastic lymphoma kinase (ALK)

Question 3

40% of neuroblastomas arise in the adrenal medulla with the rest developing along the sympathetic chain, especially in the?

Answer 3

Paravertebral region and posterior mediastinum

Question 4

Rosettes (Homer-Wright pseudorosettes) can be found in neuroblastomas which the tumor cells are concentrically arranged about a central space filled with?

Answer 4

Neuropil

Question 5

Neuroblastomas have positive immunohistochemical reactions for?

Answer 5

Neuron-specific enolase

Question 6

Disseminated neuroblastomas may present with multiple cutaneous metastases that cause?

Answer 6

Blueberry muffin baby

Question 7

About 90% of neuroblastomas cause secretion of?

Answer 7

Catecholamines

Question 8

What is currently the most important genetic abnormality used in risk stratification of neuroblastoma?

Answer 8

MYCN oncogene amplification

Question 9

What is the most common primary renal tumor of childhood and the 4th most common pediatric malignancy in the US?

Answer 9

Wilms tumor

Question 10

What are putative precursor lesions of Wilms tumors and are seen in the renal parenchyma adjacent to approximately 25% to 40% of unilateral tumors, nearly 100% in bilateral tumors?

Answer 10

Nephrogenic rests

Question 11

It is important to document the presence of nephrogenic rests in the resected specimen because these patients are at an increased risk of?

Answer 11

Developing Wilms tumor in the contralateral kidney

Question 12

What genetic deletion is more at risk for Wilms tumor?

Answer 12

WT1 deletion

Question 13

What syndrome is characterized by Wilms tumor, gonadal dysgenesis (male pseudohermaphroditism), and early-onset nephropathy leading to renal failure?

Answer 13

Denys-Drash syndrome

Question 14

What syndrome is characterized by Wilms tumor, macroglossia, hemihypertrophy, omphalocele, and abnormal large cells in the adrenal cortex?

Answer 14

Beckwith-Wiedemann syndrome

Question 15

Wilms tumor has what classic triphasic combination?

Answer 15

1) Blastemal – small blue cells in sheets with few distinctive features
2) Stromal – usually fibrotic or myxoid in nature
3) Epithelial – abortive tubules or glomeruli