Adrenocortical carcinoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Raviteja Guddeti, M.B.B.S. [2]Shivali Marketkar, M.B.B.S. [3]

Pathophysiology

Adrenocortical carcinoma may be linked to a cancer syndrome that is passed down through families (inherited).

This tumor can produce the hormones cortisol, aldosterone, estrogen, or testosterone, as well as other hormones. In women the tumor often releases these hormones, which can lead to male characteristics.

Studies suggest that mutations of the tumor suppressor genes TP53 and TP57 might also be possible contributing factors for the development of Adrenocortical carcinoma.

Gross Pathology

Grossly, adrenocortical carcinomas are often large, with a tan-yellow cut surface, and areas of hemorrhage and necrosis. On microscopic examination, the tumor usually displays sheets of atypical cells with some resemblance to the cells of the normal adrenal cortex. The presence of invasion and mitotic activity help differentiate small cancers from adrenocortical adenomas.[1]There are several relatively rare variants of adrenal cortical carcinoma: Oncocytic adrenal cortical carcinoma, Myxoid adrenal cortical carcinoma, Carcinosarcoma, Adenosquamous adrenocortical carcinoma, Clear cell adrenal cortical carcinoma. Shown below is the gross appearance of adrenocortical carcinoma.This large adrenal cortical carcinoma was resected from a 27-year-old female. The tumor measured 17 cm in diameter and invaded kidney and spleen which necessitated en bloc removal of these organs with tumor. Patient had evidence of virilization. Adrenal cortical carcinoma.JPG

Microscopic Pathology

Shown below is a micrograph of an adrenocortical carcinoma (left of image - dark blue) and the adrenal cortex it arose from (right-top of image - pink/light blue). Benign adrenal medulla is present (right-middle of image - gray/blue). H&E stain.

800px-Adrenal cortical carcinoma - low mag.jpg

Video

Shown below is a video explaining the histology of adrenocortical carcinoma

Genetics

The main etiologic factor of adrenocortical cancer is unknown. Families with Li-Fraumeni syndrome have increased risk. The p53, retinoblastoma protein (RB) tumor suppressor genes located on chromosomes 17p, 13q respectively, may be changed. The genes h19, insulin-like growth factor II (IGF-II), p57kip2 are important for fetal growth and development. They are located on chromosome 11p. Expression of the h19 gene is markedly reduced in both nonfunctioning and functioning adrenal cortical carcinomas, especially in tumors producing cortisol and aldosterone. There is also a loss of activity of the p57kip2 gene product in virilizing adenomas and adrenal cortical carcinomas. In contrast, IGF-II gene expression has been shown to be high in adrenal cortical carcinomas. Finally, c-myc gene expression is relatively high in neoplasms, and it is often linked to poor prognosis.[2]

References

  1. Richard Cote, Saul Suster, Lawrence Weiss, Noel Weidner (Editor). Modern Surgical Pathology (2 Volume Set). London: W B Saunders. ISBN 0-7216-7253-1. 
  2. Kufe D (2000). in Benedict RC, Holland JF: Cancer medicine, 5th, Hamilton, Ont: B.C. Decker. ISBN 1-55009-113-1. OCLC 156944448. 

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