SM153 Adrenal Path

Question 1

Adrenal medulla development: embryonic tissue type? Cell type?

Answer 1

Ectoderm

Neural crest cells

Question 2

Two sets of medullary progeny cells formed from neural crest cells? What do they become? Which type is more numerous when?

Answer 2

Neuroblasts: small, dark nuclei, no cytoplasm. Become nerves, sustentacular cells, ganglion cells.

Pheochromoblasts: big, light nuclei, lots of cytoplasm. Become mature chromaffin cells.

Neuroblasts are most numerous during gestation, pheochromoblasts become more numerous after birth

Question 3

What shape is the right adrenal gland, what shape is the left?

Answer 3

Right is triangular, left is semilunar

Question 4

Normal weight of adrenal glands

Answer 4

4-5 g each or 8-10 g total (6+ and 12+ are abnormal)

Question 5

Chromaffin cell histology

Answer 5

Main cell type. Synthesize and secrete catecholamines. Relatively large size, basophilic cytoplasm, salt and pepper nuclei, arranged in clusters

Question 6

Sustentacular cell histology

Answer 6

Spindle-shaped support cells, image with S100 IHC

Question 7

What is the paraganglion system?

Answer 7

Cell nests widely distributed throughout the body that contain neuroendocrine cells closely related to adrenal chromaffin cells and the ANS

Question 8

Pheochromocytoma definition, cell type

Answer 8

Most common medullary tumor in adults.

Catecholamine-secreting tumor arising from the medulla, composed of chromaffin cells

Question 9

Paraganglioma definition

Answer 9

Catecholamine-secreting tumor (identical to pheochromocytoma) outside of the medulla

Question 10

Pheochromocytoma clinical signs, labs

Answer 10

Clinical: 5 P’s all from hyperadrenergic state: pressure (HTN), palpitations, pain (headache), perspiration, pallor

Labs: elevated catecholamines and VMA/metanephrine in blood and urine

Question 11

Pheochromocytoma rule of 10’s

Answer 11

10% malignant, 10% bilateral, 10% extra-adrenal (paraganglioma), 10% children, 10% calcify, 10% familial

Question 12

Pheochromocytoma gene associations

Answer 12

MEN2A, MEN2B, neurofibromatosis type I

Question 13

Pheochromocytoma gross and microscopic pathology

Answer 13

Gross: solitary, unilateral masses (exception: familial cases are bilateral and multiple)

Microscopic: zellballen nests of cells, some elongated spindle cells, otherwise resemble normal chromaffin cells

Question 14

Pheochromocytoma vs. adrenal cortical adenoma

Answer 14

Pheo: brown/red, heterogeneous cut surface, surround by an intact cortex, purple cytoplasm with sustentacular cells present

Cortical adenoma: yellow, homogenous cut surface, contiguous with cortex, pink cytoplasm

Question 15

Pheochromocytoma: malignant or benign most often? What is the definitive criteria for malignancy?

Answer 15

Benign

Metastasis

Question 16

Ganglioneuroma: definition, cell type, gross and microscopic pathology

Answer 16

Benign tumor of neuroblast cells, composed of mature ganglion and schwannian cells

Gross: intact cortex, very homogenous white/yellow cut surface. Microscopic: mixture of mature schwannian stroma with interspersed ganglion cells

Question 17

Neuroblastoma definition, cell type

Answer 17

Most common medullary tumor in kids

Malignant tumor of neuroblastic origin

Question 18

Neuroblastoma clinical signs, labs

Answer 18

Palpable abdominal mass with or without pain

Elevated catecholamine metabolites in urine (VMA and HMA). No HTN (unlike pheo)

Question 19

Neuroblastoma gross and microscopic pathology

Answer 19

Gross: large, firm, grey/white, often with hemorrhage, necrosis, and cysts

Microscopic: small round blue cells, neuropil (pink fibrillary material in the background), Homer-Wright pseudo-rosettes (neuroblasts surrounding a central core of neuropil)

Question 20

What do you see in stage IV-S of neuroblastoma, and is this a good or bad prognostic indicator?

Answer 20

Blueberry muffin baby

Favorable indicator

Question 21

What is the MKI index for neuroblastomas?

Answer 21

Mitosis-karyorrhexis index, involves counting the number of mitoses and karyorrhexis, high index is bad

Question 22

Adrenal cortex development: embryonic tissue type?

Answer 22

Mesoderm