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]Ahmad Al Maradni, M.D. [4]

Overview

On gross pathology, a large tan-yellow surface with areas of hemorrhage and necrosis is a characteristic finding of adrenocortical carcinoma. On microscopic histopathological analysis, sheets of atypical cells with some resemblance to the cells of the normal adrenal cortex are a characteristic finding of adrenocortical carcinoma.

Pathophysiology

Genetics

Gross Pathology

On gross pathology, adrenocortical carcinomas are often large, with a tan-yellow cut surface and areas of hemorrhage and necrosis.

Shown above is a large adrenal cortical carcinoma resected from a 27-year-old woman. 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.

Microscopic Pathology

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 helps differentiate small cancers from adrenocortical adenomas.[1]

The Weiss criteria of adrenocortical malignancy comprise the most reliable histopathological scoring system differentiating ACC from ACA 9–11

ACC can be diagnosed by the presence of at least 3 of the 9 Weiss criteria:

  • Three relate to cytological features (nuclear grade, mitoses and atypical mitoses)
  • Three refer to tumor structure (clear cells, diffuse architecture, and confluent necrosis)
  • Three relate to invasion (venous invasion, sinusoidal invasion, and capsular infiltration)

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.

Video

Shown below is a video explaining the histology of adrenocortical carcinoma

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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.

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