153 - adrenal medulla path

Question 1

describe adrenal medulla embryology

Answer 1

it is derived from ectoderm. invade the adrenal cortex at seventh week of development. made of small, dark staining neuroblasts and large light staining pheochromoblasts. neuroblasts predominate until birth

Question 2

what cells make up the adrenal medulla?

Answer 2

1) pheochromocytes - secrete catecholamines, large size, basophillic cytoplasm and salt and pepper nuclei
2) sustentacular cells - spindle shaped support cells
3) nerves and ganglion cells - stimulate release of catecholamines

Question 3

how do you stain for sustentacular cells?

Answer 3

s100 protein immunohistochemical stain

Question 4

describe pheochromocytoma

Answer 4

rare. most common in 50s. affects men and women equally. causes htn, headache, palpitations, nausea. rule of 10%s: (bilateral, extradrenal, children, malignant, familial)

Question 5

what is the classic growth pattern in pheochromocytomas?

Answer 5

ball of cells called zellballen

Question 6

what is a malignant feature of malignant pheochromocytoma?

Answer 6

tumor cell spindling, vascular and capsular invasion

Question 7

describe ganglioneuroma

Answer 7

benign tumor or neuroblasts composed of mature ganglion cells. not hormonally active.

Question 8

describe neuroblastoma

Answer 8

malignant tumor of neuroblasts. most common congenital neoplasm in children. Small round blue cell tumor. homer-wright pseudo rosettes (neuroblasts surrounding a central core of neuropil)

Question 9

what is blueberry muffin baby?

Answer 9

stage 4s of neuroblastoma (favorable prognosis)

Question 10

who more commonly gets adrenal medulla cysts

Answer 10

females

Question 11

what are favorable prognoses for neuroblastoma?

Answer 11

low MKI (mitosis-karyorrhexis index) <200/5000 cells. age less than one year. stage 1, 2a, 2b, 4s