Testicular adrenal rest tumour

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Testicular adrenal rest tumour
Diagnosis in short

LM nests of eosinophilic cells interspersed with thin bands of fibrous tissue, mild (endocrine) nuclear atypia
LM DDx Leydig cell tumour
IHC melan A +ve
Site testis

Associated Dx congenital adrenal hyperplasia
Signs testicular masses (bilateral)
Prevalence extremely rare
Blood work serum ACTH elevated

Testicular adrenal rest tumour, abbreviated TART, is a rare tumour associated with increased adrenocorticotropic hormone (ACTH), typically seen in the context of congenital adrenal hyperplasia.[1]

General

Clinical:

  • Serum ACTH elevated.

Gross

  • Bilateral testicular masses.

Microscopic

Features:

  • Nests of eosinophilic cells interspersed with thin bands of fibrous tissue.
  • Mild (endocrine) nuclear atypia.

DDx:

Images

IHC

  • Melan A +ve.[3]

Molecular

  • Molecular characteristics are in keeping with adrenal tissue; however, some Leydig cell markers active.[2]

See also

References

  1. Olpin, JD.; Witt, B. (Feb 2014). "Testicular adrenal rest tumors in a patient with congenital adrenal hyperplasia.". J Radiol Case Rep 8 (2): 46-53. doi:10.3941/jrcr.v8i2.1489. PMID 24967019.
  2. 2.0 2.1 Smeets, EE.; Span, PN.; van Herwaarden, AE.; Wevers, RA.; Hermus, AR.; Sweep, FC.; Claahsen-van der Grinten, HL. (Mar 2015). "Molecular characterization of testicular adrenal rest tumors in congenital adrenal hyperplasia: lesions with both adrenocortical and Leydig cell features.". J Clin Endocrinol Metab 100 (3): E524-30. doi:10.1210/jc.2014-2036. PMID 25485724.
  3. Mizukami, H.; Hamamatsu, A.; Mori, S.; Hara, S.; Kuroda, M.; Nagai, T.; Fukunaga, T. (Mar 2011). "Autopsy and genetic diagnosis of 21-hydroxylase deficiency with bilateral testicular tumors in a case under no medication for over one year.". Forensic Sci Int 206 (1-3): e71-5. doi:10.1016/j.forsciint.2010.09.017. PMID 20951518.