A Rare Case of Clear Cell Carcinoma, Müllerian Type in the Renal Pelvis of a 21-Year-Old Woman

Abstract

Clear Cell Carcinomas of Müllerian origin are extremely rare within the upper urinary system. Their morphology is identical to that of the Clear Cell Carcinomas of the female genital tract. When they arise in the urinary tract, it is thought to be due to ectopic Müllerian embryogenesis. Here, we present a case of a 21-year-old woman with a Clear Cell Carcinoma, Müllerian type, arising from the renal pelvis. Histologically, it consisted of tubulopapillary architecture with associated foamy macrophages and a mucinous background. The neoplastic cells exhibited variably sized round nuclei with prominent nucleoli, eosinophilic to vacuolated cytoplasm with occasional intracytoplasmic mucin vacuoles, and a hobnail appearance. Immunohistochemical stains showed that the neoplastic cells were positive for Pax-8, p53, CK7, HMWK 903, and INI-1 and focally positive for p504s (AMACR). The neoplastic cells were negative for GATA-3, CK5/CK6, p63, CK20, and CDX-2 immunostains, ruling out urothelial or enteric phenotype. Additional immunostains performed by an outside institution showed that the neoplastic cells were positive for HNF-1β. The overall morphology and immunophenotype were consistent with Clear Cell Carcinoma of Müllerian origin arising from the renal pelvis. Follow-up revealed no metastasis or other tumor sites, supporting that this was the primary location.

Clear cell carcinoma of Mullerian origin in the urinary bladder: A rare entity

“…Mullerian neoplasms of the urinary system are rare, and are classified into CCC and endometrioid carcinoma. 2 CCC in the urinary system is rare and was originally referred to as "mesonephric carcinoma" owing to its origin from the mesonephric duct. As per recent World Health Organization classification, these tumors have been classified as CCC, Mullerian type.…”

“…However, lesions in a virgin abdomen or lesions containing multiple cell types are difficult to explain with this theory. 2,4 Oliva et al reviewed 13 patients with CCC of the urinary bladder in order to study the origin, and could not identify histopathological or immunohistochemical markers to distinguish Mullerian or transitional cell origin. 5 Our patient had a history of previous surgery, and so the theory of implantation might be a plausible explanation.…”

“…1 A Mullerian neoplasm in the urinary bladder could be endometrioid carcinoma or clear cell carcinoma (CCC). 2 The identification of the Mullerian neoplasm is crucial for patient management owing to its etiology, natural history, and prognosis. Here, we present the case of a 42-year-old female with primary urinary bladder CCC of Mullerian origin presenting with suprapubic pain and dysmenorrhea and managed with a total abdominal hysterectomy and partial cystectomy.…”

Müllerian-Type Clear Cell Carcinoma of Donor Origin in a Male Patient with a Kidney Transplant: Ascertained by Molecular Testing

“…Conversely, CCC of the Müllerian type very rarely arises in the male genitourinary (GU) tract, presumably due to ectopic Müllerian embryogenesis [4]. In the majority of such cases, the CCC occurs in the bladder and urethra, whereas CCC of the upper urinary tract is exceedingly rare, with less than five cases reported to date [5]. Although a case of conventional urothelial carcinoma with divergent Müllerian-type CCC differentiation arising from the renal pelvis of a kidney graft in a male patient was recently reported [6], CCC of the Müllerian type arising from transplanted organs has not been described to date.…”