Testicular adrenal rest tumour
| 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, adrenal cortical rest |
| IHC | melan A +ve |
| Site | testis |
|
| |
| Associated Dx | congenital adrenal hyperplasia |
| Signs | testicular masses (bilateral) |
| Prevalence | extremely rare |
| Blood work | serum ACTH elevated |
| Prognosis | benign |
| Clin. DDx | other testicular masses |
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
- Very rare.
- Benign.[2]
- May overlap with Leydig cell tumour - see molecular section.
- Associated with congenital adrenal hyperplasia (abbreviated CAH).
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:
- Leydig cell tumour - history different.
- Adrenal cortical rest - architecture different, not a sizable mass lesion and not bilateral.[citation needed]
Images
IHC
- Melan A +ve.[5]
Molecular
- Molecular characteristics are in keeping with adrenal tissue; however, some Leydig cell markers active.[2]
- Mutation in CYP21A2 causative of congenital adrenal hyperplasia.[3]
See also
References
- ↑ 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.
- ↑ 2.0 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.
- ↑ 3.0 3.1 Mouritsen, A.; Jørgensen, N.; Main, KM.; Schwartz, M.; Juul, A. (Jun 2010). "Testicular adrenal rest tumours in boys, adolescents and adult men with congenital adrenal hyperplasia may be associated with the CYP21A2 mutation.". Int J Androl 33 (3): 521-7. doi:10.1111/j.1365-2605.2009.00967.x. PMID 19531083.
- ↑ Claahsen-van der Grinten, HL.; Dehzad, F.; Kamphuis-van Ulzen, K.; de Korte, CL. (2014). "Increased prevalence of testicular adrenal rest tumours during adolescence in congenital adrenal hyperplasia.". Horm Res Paediatr 82 (4): 238-44. doi:10.1159/000365570. PMID 25195868.
- ↑ 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.