Week 2: Pathology of Adrenals Flashcards
embryogenesis of adrenal glands
- cortex from coelomic mesoderm near developing gonad and kidney
- medulla from neural crest
- medulla only in the head and body of the adrenals and does not extend to the tail
Enzymatic defects -adrenal pathology
- Congenital adrenal hyperplasia
- autosomal recessive enzyme defects in cortisol synthetic pathway
- insufficient cortisol and increased ACTH. Causes cortical hyperplasia - C21 hydroxylase deficiency: leads to excess 17-a-hydroxyprogesterone->excess testosterone
- >virilization (masculization)
- symptoms vary, may lead to pseudohermaphroditism in females, or macrogenitosomia in male children
Waterhouse-Friedrichsen Syndrome
- acute hemorrhagic destruction of adrenal glands
- causes: gram neg. sepsis, usually meningococcemia, occasionally overwhelming sepsis due to other organisms. possibly severe stress (burns, shock), patients on anti coagulant therapy
- acute onset, with febrile illness. DIC and extensive hemorrhage
- adrenals are enlarged, distorted, massive hemorrhage, replacement of glands by hematonas
Addison Disease
- Primary: hyperpigmentation and vitiligo (due to hypothalamic and/or pituitary lesions). Electrolyte disturbances, hypotension.
- Secondary: no major electrolyte disturbances. low ACTH.
- causes: autoimmune (with thyroiditis is called Schmidt syndrome), Tb, other infections, metastatic carcinoma, amyloidosis
Hyperplasia of adrenal cortex
- usual cause is hyper secretion of ACTH by pituitary adenoma or ectopic secretion of ACTH by lung carcinoma
- thickened zona reticularis particularly, but also fasciculate
Myelolipoma of adrenals
- benign mesenchymal tumor of adrenal cortex
- asymptomatic mostly
- incidental finding on CT scan or at autopsy
- sharply circumscribed, unencapsulated, pale yellow.
- micro: fat cells mixed with myeloid cells and lymphocytes
Adenoma of Adrenal cortex
-circumscribed, encapsulated tumors
-must be differentiated from accessory cortical nodules (small, orderly layering, like normal cortex)
SYNDROMES
1. Primary aldosteronism (Conn syndrome)
-hyperaldosteronism
2. Cushing syndrome
3. Adult adrenal virilism: usually women
-hirsutism and virilism
-high urinary 17-KS present
-brownish cut surface
4. feminizing tumor: men
5. Non functioning adenoma
-freq. seen at autopsy or incidentally during surgery
Carcinoma of the adrenal cortex
- larger than adenomas
- bulky, yellow tumor
- necrosis, calcification, hemorrhage
- Micro: pleomorphic cells, hyperchromatism, mitoses, vascular invasion
- opposite adrenal may be atrophic
- Syndromes: aldosteronism, cushing syndrome, adrenal virilism, feminization
- may be associated with Li Fraumeni syndrome (p53), Beckwith-Wiederman syndrome (Wilm’s tumor)
Hyperplasia of adrenal medulla
-enlargement of medulla with extension into the tail of the gland
Associated with
1. MEN 2A
2. sporadic syndrome with paroxysmal HTN, elevated urinary catecholamines
Neuroblastoma of adrenals
- commonest extracranial solid tumor in infancy and childhood
- usually bilateral, aggressive, highly malignant
- n-myc oncogene associated with poor prognosis
- Gross: large bulky white mass, calcification, necrosis
- micro: small undifferentiated round cells, laying in fibrillary storm, may be in rosettes
- differentation to ganglioneuroblastoma or mature ganglioneuroma may occur
- also may regress spontaneously, leaving calcification behind
pathology of pheochromacytoma
- proxysmal hypertension and/or hyperglycemia may be presenting symptoms
- 10% tumor: 10% bilateral, extra-adrenal, malignant, familial, occur in children
- Associated with MEN 2A/B, Neurofibromatosis, Sturge-weber, Von Hippel-Lindau disease (RCC, chrome3)
- gross: circumscribed and encapsulated. Brownish, pale red. positive chromatin reaction
- micro: large cells, abundant cytoplasm. cords and nests separated by vascular spaces.
- capsular and vascular invasion may occur in benign tumors, malignancy based on metastases
- Labs: increased urinary excretion of free catecholamines and metabolites
