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adrenal cortical carcinoma

Tuesday 16 March 2004

adrenal cortical carcinomas; adrenocortical carcinoma

Digital case

- JRC:14921 : Lung metastasis of adrenal cortical carcinoma.
- JRC:14613 : Adrenal cortical carcinoma (with Cushing syndrome).
- JRC:18771 : Adrenal cortical carcinoma

Microscopic prognostic parameters

- Weiss score (21997290)

Differential diagnosis

- islet cell carcinoma
- pulmonary large cell undifferentiated carcinoma
- hepatocellular carcinoma (HCC)
- renal cell carcinoma
- bronchogenic carcinoma
- pheochromocytoma and paraganglioma
- alveolar soft part sarcoma

Variants

- oncocytic adrenocortical carcinoma
- virilizing adrenocortical carcinoma

Immunochemistry

- melan-A+ (MLANA+) (9422316)
- alpha-inhibin+ (INHA+)

Predisposition

- Beckwith-Wiedemann syndrome
- germ-line P53 mutations

  • Li-Fraumeni syndrome
  • inherited R337H p53 mutation without Li-Fraumeni syndrome (11481490)

- Carney complex
- multiple endocrine neoplasia type 1
- congenital adrenal hyperplasia
- McCune-Albright syndrome

Associations

- ganglioneuroblastoma (P53 germline mutations) (9598730)

Cytogenetics

- CGH: 10514385

Molecular biology

> Multistep adrenocortical tumorigenesis (12629075)

- TP53 (17p13)

  • TP53 mutations (30-50%) (11454518)
  • TP53-associated adrenocortical carcinoma

- APC mutations

  • APC-associated adrenocortical carcinoma

- Locus 11p15.5

- LOH at 11q13: significantly higher in adrenocortical carcinomas (60%) than in benign lesions (11%) (9935177)

- high expression of cyclin E and G1 CDK
- altered expression of novH (11502835)

- allelotype: 9596277

CGH

Losses

- 1p21-31
- 2q
- 3p
- 3q
- 6q
- 9p
- 11q14-qter

Gains and amplifications

- 5q12
- 9q34
- 12q
- 17q
- 17p
- 20q

LOH

- 1p (22%)
- 3p (22%)
- 9p (26%)

References

- The weiss score and beyond—histopathology for adrenocortical carcinoma. Papotti M, Libè R, Duregon E, Volante M, Bertherat J, Tissier F. Horm Cancer. 2011 Dec;2(6):333-40. PMID: 21997290

Reviews

- Differences in the molecular mechanisms of adrenocortical tumorigenesis between children and adults. Faria AM, Almeida MQ. Mol Cell Endocrinol. 2012 Mar 31;351(1):52-7. PMID: 22019901

- Stratakis CA. Genetics of adrenocortical tumors: gatekeepers, landscapers and conductors in symphony. Trends Endocrinol Metab. 2003 Nov;14(9):404-10. PMID: 14580759

- Reincke M, Beuschlein F, Slawik M, Borm K. Molecular adrenocortical tumourigenesis. Eur J Clin Invest. 2000 Dec;30 Suppl 3:63-8. PMID: 11281372

Portfolio

  • Adrenocortical carcinoma