Persistent müllerian duct syndrome and prostate cancer

Persistent Mullerian duct syndrome (PMDS) is a rare type of male pseudohermaphroditism characterized by the presence of Mullerian derivatives (uterus, fallopian tubes and upper vagina), cryptorchidism either unilateral or bilateral along with inguinal hernia in a phenotypically and genotypically male person. PMDS is rarely associated with malignancies, the most common being testicular germ cell tumours. Occasional case of Adenocarcinoma arising in the PMD remnant structure is reported. We report an unusual case of male type of PMDS with squamous cell carcinoma (SCC) arising from PMD derivatives and presented with hematuria Also, this patient had karyotype of 47XYY (Jacob syndrome). Both these are hitherto unreported in English literature to the best of our knowledge.

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“…(also called as Jacob syndrome) Associat ion with this karyotype has not been reported in cases of PMDS. [4] This was also a rare association.…”

Section: Discussionmentioning

confidence: 84%

Squamous Cell Carcinoma Arising from Remnant of Mullerian Duct With 47XYY Karyotype – Rare Case

Padmanabhan¹,

Kadam²,

Gadgil³

2022

Ann of Pathol and Lab Med

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“…The association of prostatic cancer with PMDS is quite rare as only two cases of prostate cancer among PMDS patient have been reported in the literature so far. [ 10 12 ] This case represents the third case of prostate cancer among a patient with underlying PMDS. We demonstrate radiological-pathological correlation for this case for an opportunity to further the existing knowledge of this extremely rare medical diagnosis.…”

Section: Discussionmentioning

confidence: 98%

“…[ 1 ] Cryptorchidism and diminished testosterone levels in postpubertal life in patients with PMDS play a protective role against prostate cancer. [ 7 9 10 ]…”

Section: Introductionmentioning

confidence: 99%

Prostatic adenocarcinoma in a patient with persistent Müllerian duct syndrome

et al. 2022

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“…En revanche cette technique conservatrice exposerait au risque d'une compression extrinseque, d'une cancerisation testiculaire qui serait liee plutSt a I'ectopie testiculaire mal corrigee ou meme bien faite [14,16,19] et & la degenerescence carcinomateuse de ces derives [19,20]. Ces derniers ont ete Iongtemps consideres comme denues de tout potentiel malin.…”

Section: Discussionunclassified