ENDOCRINE PATHOLOGY

CHAPTER 4 ENDOCRINE PATHOLOGY





Congenital adrenal hyperplasia

Adrenal ‘cysts’

Heterotopic tissue in adrenals

Adrenal cortical adenoma / carcinoma

Phaeochromocytoma

Predisposing conditions associated with phaeochromocytoma and other endocrine tumors
Multiple endocrine neoplasia type 2 (men2)

Multiple endocrine neoplasia type 1 (men1)

Hyperparathyroidism–jaw tumor syndrome

Von hippel–lindau syndrome

Neurofibromatosis (nf) type 1

Carney syndrome



CONGENITAL ADRENAL HYPERPLASIA



• Most 21-hydroxylase deficiency
image present with virilization

• Diffuse bilateral cortical enlargement
image esp zone reticularis


ADRENAL ‘CYSTS’



• Pseudocysts due to previous hemorrhage

• Endothelial lined vascular / lymphatic cyst

• Cystic degeneration of adrenal tumor including NTs

• True epithelial lined cysts
image Beckwith–Wiedemann syndrome

• V rare ectopic bronchogenic adrenal cysts


HETEROTOPIC TISSUE IN ADRENALS



• Well reported including:
image liver

image thyroid

image ovary


ADRENAL CORTICAL ADENOMA / CARCINOMA



Epidemiology



• Mainly older adults

• Well reported smaller peak in frequency in early childhood:
image most are sporadic

image may be associated with Li–Fraumeni or Beckwith–Wiedemann syndrome

• Children have overall better prognosis than adults


Clinical features



• Mass effect

• Excess hormone production
image androgens – virilization

image glucocorticoids – Cushing’s syndrome

image mineralocorticoids – hypertension

• 30–50% metastases

• May invade renal vein and IVC

• Stage important predictor of outcome


Histopathological features (Figs 4.1–4.7)



• Sheets and trabeculae of cells morphologically resembling normal adrenal cortex
image clear and eosinophilic cytoplasm
– variable nuclear atypia and mitotic rate

• Main features suggesting malignant behavior (carcinoma vs adenoma):
image diameter >5cm

image fibrous bands

image necrosis

image capsular invasion

image vascular invasion

image greater mitotic rate

image

Fig. 4.1


image

Figs 4.1–4.2 Photographs of a resected adrenocortical carcinoma, measuring more than 10cm in maximum extent, the cut surface showing a solid tan yellow appearance with areas of necrosis.


image

Fig. 4.3


image

Fig. 4.4


image

Fig. 4.5


image

Fig. 4.6


image

Figs 4.3–4.7 Photomicrographs of a case of adrenocortical carcinoma in a child, demonstrating focal necrosis and marked nuclear pleomorphism. There was also capsular and vascular invasion and local recurrence appeared soon after surgery.



Immunohistochemical staining



• Alpha inhibin+

• SF1+

• Chromogranin negative (cf medullary tumors)

• May be only weakly positive for cytokeratin and EMA

• CEA negative


Differential diagnoses and pitfalls



• Smaller lesions difficult to predict clinical behavior from histological features

• May be related to germ-line point mutation of p53 even with no positive family history, acting in a tissue-specific manner (Ribeiro et al 2001)


PHAEOCHROMOCYTOMA



• Tumor of chromaffin cells of adrenal medulla
image paraganglioma if affecting other sites

• Benign (90%)

• Malignant (10%: strictly defined by metastases)


Epidemiology



• Mainly sporadic

• >10% associated with NF-1, MEN2, VHL and/or familial cases
image these more likely to present at younger age

Related posts:

  1. BONE PATHOLOGY
  2. HEAD AND NECK PATHOLOGY
  3. RENAL PATHOLOGY
  4. TUMORS AND TUMOR-LIKE LESIONS

Stay updated, free articles. Join our Telegram channel
Tags:
Jun 18, 2016 | Posted by in PEDIATRICS | Comments Off on ENDOCRINE PATHOLOGY

Full access? Get Clinical Tree
Get Clinical Tree app for offline access