Difference between revisions of "Testicular adrenal rest tumour"

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*Benign.<ref name=pmid25485724>{{Cite journal  | last1 = Smeets | first1 = EE. | last2 = Span | first2 = PN. | last3 = van Herwaarden | first3 = AE. | last4 = Wevers | first4 = RA. | last5 = Hermus | first5 = AR. | last6 = Sweep | first6 = FC. | last7 = Claahsen-van der Grinten | first7 = HL. | title = Molecular characterization of testicular adrenal rest tumors in congenital adrenal hyperplasia: lesions with both adrenocortical and Leydig cell features. | journal = J Clin Endocrinol Metab | volume = 100 | issue = 3 | pages = E524-30 | month = Mar | year = 2015 | doi = 10.1210/jc.2014-2036 | PMID = 25485724 }}</ref>
*Benign.<ref name=pmid25485724>{{Cite journal  | last1 = Smeets | first1 = EE. | last2 = Span | first2 = PN. | last3 = van Herwaarden | first3 = AE. | last4 = Wevers | first4 = RA. | last5 = Hermus | first5 = AR. | last6 = Sweep | first6 = FC. | last7 = Claahsen-van der Grinten | first7 = HL. | title = Molecular characterization of testicular adrenal rest tumors in congenital adrenal hyperplasia: lesions with both adrenocortical and Leydig cell features. | journal = J Clin Endocrinol Metab | volume = 100 | issue = 3 | pages = E524-30 | month = Mar | year = 2015 | doi = 10.1210/jc.2014-2036 | PMID = 25485724 }}</ref>
*May overlap with [[Leydig cell tumour]] - see ''molecular'' section.
*May overlap with [[Leydig cell tumour]] - see ''molecular'' section.
*Associated with ''congenital adrenal hyperplasia''.
*Associated with ''congenital adrenal hyperplasia'' (abbreviated ''CAH'').
**Due to mutation in CYP21A2.<ref name=pmid19531083>{{Cite journal  | last1 = Mouritsen | first1 = A. | last2 = Jørgensen | first2 = N. | last3 = Main | first3 = KM. | last4 = Schwartz | first4 = M. | last5 = Juul | first5 = A. | title = Testicular adrenal rest tumours in boys, adolescents and adult men with congenital adrenal hyperplasia may be associated with the CYP21A2 mutation. | journal = Int J Androl | volume = 33 | issue = 3 | pages = 521-7 | month = Jun | year = 2010 | doi = 10.1111/j.1365-2605.2009.00967.x | PMID = 19531083 }}</ref>
**Due to mutation in CYP21A2.<ref name=pmid19531083>{{Cite journal  | last1 = Mouritsen | first1 = A. | last2 = Jørgensen | first2 = N. | last3 = Main | first3 = KM. | last4 = Schwartz | first4 = M. | last5 = Juul | first5 = A. | title = Testicular adrenal rest tumours in boys, adolescents and adult men with congenital adrenal hyperplasia may be associated with the CYP21A2 mutation. | journal = Int J Androl | volume = 33 | issue = 3 | pages = 521-7 | month = Jun | year = 2010 | doi = 10.1111/j.1365-2605.2009.00967.x | PMID = 19531083 }}</ref>
**TART prevalence increases with age - one study suggests moderate prevalance at age 10, increasing to 100% of individuals over 16 years of age.<ref>{{Cite journal  | last1 = Claahsen-van der Grinten | first1 = HL. | last2 = Dehzad | first2 = F. | last3 = Kamphuis-van Ulzen | first3 = K. | last4 = de Korte | first4 = CL. | title = Increased prevalence of testicular adrenal rest tumours during adolescence in congenital adrenal hyperplasia. | journal = Horm Res Paediatr | volume = 82 | issue = 4 | pages = 238-44 | month =  | year = 2014 | doi = 10.1159/000365570 | PMID = 25195868 }}</ref>
**TART prevalence increases with age - one study suggests moderate prevalance at age 10, increasing to 100% of individuals with CAH over 16 years of age.<ref>{{Cite journal  | last1 = Claahsen-van der Grinten | first1 = HL. | last2 = Dehzad | first2 = F. | last3 = Kamphuis-van Ulzen | first3 = K. | last4 = de Korte | first4 = CL. | title = Increased prevalence of testicular adrenal rest tumours during adolescence in congenital adrenal hyperplasia. | journal = Horm Res Paediatr | volume = 82 | issue = 4 | pages = 238-44 | month =  | year = 2014 | doi = 10.1159/000365570 | PMID = 25195868 }}</ref>


Clinical:
Clinical:

Revision as of 04:12, 15 July 2015

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
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).
    • Due to mutation in CYP21A2.[3]
    • TART prevalence increases with age - one study suggests moderate prevalance at age 10, increasing to 100% of individuals with CAH over 16 years of age.[4]

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.[5]

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. ↑ 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.
  4. ↑ 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.
  5. ↑ 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.
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