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adrenal cortical carcinoma

Etiology: - rare cases associated with a) Li-Fraumeni syndrome b) Beckwith-Wiedemann syndrome c) congenital adrenal hyperplasia. Epidemiology: - ~ 0.2% of all malignancies. - bimodal age distribution: a) small peak in first two decades b) larger peak in fifth decade. Pathology: - large tumors weighing > 100 gms in adults (some benign tumors associated with adrenogenital syndrome can weigh considerably more than 100 gms) - nodular - foci of necrosis, hemorrhage & calcification common - secrete cortisol &/or androgens Microscopic pathology: - histologic criteria for diagnosis, vs adenoma - venous invasion - necrosis - pleomorphism/high nuclear grade (Fuhrman, renal cell carcinoma) - mitoses/atypical mitoses - diffuse architecture (> 30% of area of tumor) - capsular invasion - broad fibrous bands Genetics: - associated with defects in TP53 Clinical manifestations: - nausea [3] - abdominal pain - palpable abdominal mass (up to 30%) - symptoms from mass effect - back pain [3] - hypocalcemia/hypercalcemia - Cushing's syndrome - adrenogenital syndrome - hyperaldosteronism (rare) Laboratory: - Immunocytochemistry: - inhibin: positive - A103 (melan A): positive - vimentin: positive - cytokeratin: -/+ - epithelial membrane antigen: negative - chromogranin: negative - CEA: negative Radiology: - contrast-enhanced computed tomography - generally > 4 cm with irregular margins* - calcifications, necrosis - heterogenous enhancement* - density > 10-20 HU* - contrast washout < 50% in 10 minutes* - magnetic resonance imaging (MRI) - hyperintense of T2-weighted images* * compare with adrenal cortical adenoma Differential diagnosis: - adrenal cortical adenoma (distinguishing CT characteristics) - pheochromocytoma - hepatocellular carcinoma - renal cell carcinoma - metastatic adenocarcinoma Management: 1) open surgical excision followed with mitotane [3] 2) mitotane if inoperable, recurrent &/or metastatic [3] 3) radiation therapy may be option [3]

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General

adrenal cancer carcinoma

References

  1. Diagnostic Surgical Pathology. Sternberg ed. Lippincott, Williams & Wilkins, 3rd ed. 1999
  2. Dabbs. Diagnostic Immunohistochemistry. Churchill-Livingstone, 2002. pages 223-4, 475.
  3. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018, 2022.
  4. Berruti A, Baudin E, Gelderblom H et al Adrenal cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012 Oct;23 Suppl 7:vii131-8. PMID: 22997446 Free Article
  5. Cavlan D, Bharwani N, Grossman A. Androgen- and estrogen-secreting adrenal cancers. Semin Oncol. 2010 Dec;37(6):638-48. PMID: 21167382
  6. Fassnacht M, Dekkers O, Else T, et al. European Society of Endocrinology Clinical Practice Guidelines on the Management of Adrenocortical Carcinoma in Adults, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2018 Jul 24. PMID: 30042120 http://www.eje-online.org/content/early/2018/07/24/EJE-18-0608.long
  7. Adrenocortical Carcinoma (PDQ): Treatment http://www.nci.nih.gov/cancertopics/pdq/treatment/adrenocortical/HealthProfessional

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