Clear cell myomelanocytic tumour: minimally invasive treatment of a rare bladder tumour

Abstract

Clear cell myomelanocytic tumours are extremely rare neoplastic growths considered to be members of the family of perivascular epithelioid cell tumours (PEComas), which have in common the coexpression of melanocytic and smooth muscle immunohistochemical markers. These tumours are known to be ubiquitous with uncertain tumour biology and to have unpredictable clinical behaviour. They have been reported in the genitourinary tract, including the kidney and prostate. There are only 3 reported cases of clear cell myomelanocytic tumours originating in the urinary bladder. We report a case of a 24-year-old woman with chronic pelvic pain who underwent laparoscopic partial cystectomy and total excision of a bladder mass. Pathological examination revealed primary PEComa of the urinary bladder. Subsequent follow-up procedures, including cystoscopy and imaging, have not revealed any evidence of disease recurrence. The patient remains clinically free of disease 3 months after surgery.

Bladder Perivascular Epithelioid Cell Tumor (PEComa): A Case Report and Literature Review

“…Again histology showed PEComa. The patient was clinically free of disease 3 months after surgery (9).…”

Perivascular epithelioid cell tumour of the bladder

“…A few patients were managed with TURBT alone and did not require further surgical intervention 7 8 11. There are several reports of PEComas of the bladder treated successfully with partial cystectomy 7 10 12 13 17 18 21. In two other cases, patients underwent radical cystectomy for metastatic tumours exhibiting aggressive clinical behaviour 9 14.…”

“…In two other cases, patients underwent radical cystectomy for metastatic tumours exhibiting aggressive clinical behaviour 9 14. Pianezza et al 21 described in detail a laparoscopic partial cystectomy for resection of a CCMMT. While the use of robotics for the management of common bladder lesions such as urothelial carcinoma has become routine, a robotic approach to partial cystectomy for the treatment of PEComa of the bladder has not been described in prior literature.…”

Bladder Perivascular Epithelioid Cell Tumor: A Novel Rare Neoplasm

“…Another case of primary PEComa of the bladder has been described with the patient being alive 4 years after partial cystectomy and adjuvant interferon-␣ immunotherapy [8] . In 2008, Pianezza et al [9] reported the 4th documented case of bladder PEComa in an 24-year-old woman with chronic pelvic pain who underwent laparoscopic partial cystectomy of a bladder PEComa. More recently, Sukov et al [10] reported 3 cases of bladder PEComa in 2 men in their 4th decade and 1 woman in her 3rd decade.…”