Case Number : Case 774 - 5th June Posted By: Guest

Mixed tumour of skin

Agree mixed tumour of the skin

Trichoblastoma

Cribriform trichoblastoma.
I found the sieve-like nodules of basaloid cells embedded in a specialized stroma with plump fibroblasts.
This patient should be investigated for Brooke-Spiegler syndrome.

I will diagnose this also as cribriform trichoblastoma..

I agree with Cribriform trichoblastoma. The specialized stroma is a nice clue. To say sometinhg differente, only for differential diagnosis purpose, the last two pictures have some similarities with Adenoid cystic carcinoma.

Agree with trichoblastoma

[quote name='Juan Carlos Garcés, Ecuador' timestamp='1370437604']
Agree with trichoblastoma
[/quote]
¡Saludos, amigo Garcés! Estoy contento que un gran dermatopatólogo como tú está de acuerdo con mi diagnóstico.

With history of precocious puberty and limited clinical information, caution would dictate adding metastatic granulosa cell tumor to the differential. These can have bland cytology, and trabecular vs. solid polycystic architecture with [url="http://www.webpathology.com/image.asp?n=12&Case=533"]Call-Exner bodies[/url] (round glandular spaces with sparse eosinophilic granular content, lined by poorly palisading granulosa cells) similar to those seen above.

Nice example of a trichoblastoma (trichoepithelioma) for me. Cribriform and adenoid areas. Agree very uncommon at this age so consider BSS.

Speaking out my thoughts: precocious puberty can be seen in certain conditions of centrally or peripherally origin. Gonadal tumors like germ cell tumors, granulosa cell tumors and sertoli-leydig neoplasms may be causes. McCunne-Albright and Angelman syndromes do not show this kind of cutaneous component. Brooke-Spiegel syndrome is associated with spiradenomas, trichoepitheliomas and cylindromas, but I do not know of precocious puberty. Applying the Occam razor, I would think of a metastatic gonadal or endocrine tumor, favoring the first one over the second, mostly a granulosa cell tumor juvenile type or an arrhenoblastoma. Having said that, I am lining up with Dr. Davie.

Clinical onset of Brooke-Spiegler syndrome generally happens during puberty. So I think that precocious puberty in our 10-year-old patient probably have triggered the beggining of the syndrome menifestation.

I called this trichoblastoma.