A Case of Clear Cell Adenocarcinoma of the Müllerian Duct in Persistent Müllerian Duct Syndrome

Abstract

We report a case of a 67-year-old man with clear cell adenocarcinoma of the remnant uterus in persistent Müllerian duct syndrome. He had a normal penis, urethra, and scrotum, and there was also a vagina and uterus. He died in a traffic accident, and clear cell adenocarcinoma was discovered incidentally at autopsy. Clear cell adenocarcinoma of the remnant uterus metastasized to the retroperitoneal lymph nodes and bilateral lungs. Persistent Müllerian duct syndrome is characterized by the persistence of Müllerian derivatives in otherwise normally virilized males. A variety of germ cell tumors of the testis have been reported in association with persistent Müllerian duct syndrome. However, no malignant change of the persistent Müllerian duct structures has been reported. This represents the first reported case of malignant change of the persistent Müllerian duct structures in persistent Müllerian duct syndrome.

Focal Müllerian duct retention in male mice with constitutively activated β-catenin expression in the Müllerian duct mesenchyme

“…In human patients, because surgical removal of the MD derivatives is often carried out when surgery in boys is also necessary for cryptorchid, nonpalpable testes and the vast majority of PMDS patients have mutations in MIS signaling components (8), later consequences of MD retention due to dysregulated Wnt/β-catenin signaling will be difficult to discern (37,38). However, there is one reported case of uterine adenosarcoma (39) and another of clear cell adenocarcinoma (40) in human patients with PMDS. We did not observe evidence of neoplastic transformation in the retained MD tissue of these mice by 3 mo.…”

Indirect inguinal hernia with uterine tissue in a male: A case of persistent Mullerian duct syndrome and literature review

“…9 However, more recent reports have demonstrated rare malignancies, usually adenocarcinomas of the Mullerian duct, associated with retained Mullerian structures. [20][21][22][23][24][25][26] If resection is attempted, great care must be taken to avoid damage to the vascular supply to the testis, or vas deferens, which run in close proximity to the remnant uterus and proximal vagina. 9 Interestingly, laparoscopic management of PMDS has now also been successfully reported.…”

Bilateral Seminoma Arising in Transverse Testicular Ectopia with Persistent Müllerian Duct Syndrome and its Metastasis to Rudimentary Uterus: Case Report

“…2 PMDS is al so a ra re con di ti on in which Mül le ri an de ri va ti ves are pre sent in a ma le with a nor mal ma le phe noty pe. 1,3 The syndro me is an au to so mal or X-lin ked re ces sive di sor der ca u sed by in suf fi ci ent Mül le ri an duct in hi bi ting fac tor or un res pon si ve ness of the end organ. 1,6 FI GU RE 1: A hu ge right tes ti cu lar tu mor re semb ling a le i om yo ma to us ute rus has a blind en ding va gi na-li ke struc tu re on one si de (ar row) and the left ecto pic tes ti cu lar tu mor is bo und to the hu ge mass with a sper ma tic cord li ke struc tu re on the ot her si de (ar row he ad).…”

“…Tıbbi Patoloji le ri an struc tu res is a very ra re event; only thre e cases ha ve be en re por ted to da te. [3][4][5] Ho we ver no metas ta sis to the Mül le ri an duct struc tu res has be en re por ted yet. Furt her mo re, alt ho ugh so me ar tic les do cu ment tes ti cu lar ma lig nancy as so ci a ted with PMDS and TTE, no ne of the se ca ses had bi la te ral tes ti cu lar ma lig nancy.…”