Difference between revisions of "Testicular adrenal rest tumour"

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      =  
| Image      = Testicular adrenal rest tumour -- intermed. mag.jpg
| Width      =
| Width      =
| Caption    =  
| Caption    = Testicular adrenal rest tumour. [[H&E stain]].
| Synonyms  =
| Synonyms  =
| Micro      = nests of eosinophilic cells interspersed with thin bands of fibrous tissue, mild (endocrine) [[nuclear atypia]]
| Micro      = nests of eosinophilic cells interspersed with thin bands of fibrous tissue, mild (endocrine) [[nuclear atypia]]
Line 53: Line 53:
DDx:
DDx:
*[[Leydig cell tumour]] - history different.
*[[Leydig cell tumour]] - history different.
*[[Adrenal cortical rest]] - not a sizable mass lesion and not bilateral.{{fact}}
*[[Adrenal cortical rest]] - architecture different, not a sizable mass lesion and not bilateral.{{fact}}


===Images===
===Images===
====www====
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4037253/figure/f4-jrcr-8-2-46/ TART (nih.gov)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4037253/figure/f4-jrcr-8-2-46/ TART (nih.gov)].
====Case====
<gallery>
Image: Testicular adrenal rest tumour -- low mag.jpg | TART - low mag. (WC)
Image: Testicular adrenal rest tumour -- intermed. mag.jpg | Image: Testicular adrenal rest tumour -- high mag.jpg | TART - high mag. (WC)
Image: Testicular adrenal rest tumour - alt -- intermed. mag.jpg | TART - intermed. mag. (WC)
Image: Testicular adrenal rest tumour -- high mag.jpg | TART - high mag. (WC)
Image: Testicular adrenal rest tumour -- very high mag.jpg | TART - very high mag. (WC)
Image: Testicular adrenal rest tumour - alt -- high mag.jpg | TART - high mag. (WC)
Image: Testicular adrenal rest tumour - alt -- very high mag.jpg | TART - very high mag. (WC)
</gallery>


==IHC==
==IHC==
*Melan A +ve.<ref name=pmid20951518>{{Cite journal  | last1 = Mizukami | first1 = H. | last2 = Hamamatsu | first2 = A. | last3 = Mori | first3 = S. | last4 = Hara | first4 = S. | last5 = Kuroda | first5 = M. | last6 = Nagai | first6 = T. | last7 = Fukunaga | first7 = T. | title = Autopsy and genetic diagnosis of 21-hydroxylase deficiency with bilateral testicular tumors in a case under no medication for over one year. | journal = Forensic Sci Int | volume = 206 | issue = 1-3 | pages = e71-5 | month = Mar | year = 2011 | doi = 10.1016/j.forsciint.2010.09.017 | PMID = 20951518 }}</ref>
*Melan A +ve.<ref name=pmid20951518>{{Cite journal  | last1 = Mizukami | first1 = H. | last2 = Hamamatsu | first2 = A. | last3 = Mori | first3 = S. | last4 = Hara | first4 = S. | last5 = Kuroda | first5 = M. | last6 = Nagai | first6 = T. | last7 = Fukunaga | first7 = T. | title = Autopsy and genetic diagnosis of 21-hydroxylase deficiency with bilateral testicular tumors in a case under no medication for over one year. | journal = Forensic Sci Int | volume = 206 | issue = 1-3 | pages = e71-5 | month = Mar | year = 2011 | doi = 10.1016/j.forsciint.2010.09.017 | PMID = 20951518 }}</ref>
*CD56 +ve.<ref name=pmid23984262>{{Cite journal  | last1 = Ali | first1 = HH. | last2 = Samkari | first2 = A. | last3 = Arabi | first3 = H. | title = Testicular adrenal rest "tumor" or Leydig cell tumor? A report of a challenging case with literature review. | journal = Avicenna J Med | volume = 3 | issue = 1 | pages = 15-9 | month = Jan | year = 2013 | doi = 10.4103/2231-0770.112789 | PMID = 23984262 }}</ref>
*AR -ve.<ref name=pmid23984262/>


==Molecular==
==Molecular==

Latest revision as of 21:51, 25 May 2016

Testicular adrenal rest tumour
Diagnosis in short

Testicular adrenal rest tumour. H&E stain.
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).
    • 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

www

Case

  • TART - low mag. (WC) 

  • TART - high mag. (WC)

  • TART - intermed. mag. (WC)

  • TART - high mag. (WC)

  • TART - very high mag. (WC)

  • TART - high mag. (WC)

  • TART - very high mag. (WC)

IHC

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

  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. ↑ 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.
  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.
  6. ↑ 6.0 6.1 Ali, HH.; Samkari, A.; Arabi, H. (Jan 2013). "Testicular adrenal rest "tumor" or Leydig cell tumor? A report of a challenging case with literature review.". Avicenna J Med 3 (1): 15-9. doi:10.4103/2231-0770.112789. PMID 23984262.
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