病例報告:罕見膀胱血管周圍上皮細胞瘤
蔡佳宏1、沈元琦1、潘怡平2
高雄長庚紀念醫院 泌尿科1 ，解剖病理部2
case report: a RARE CASE - PEComa of bladder
Chia-Hung Tsai1, Yuan-Chi Shen1, Yi-Ping Pan2
Department of Urology1 and Pathology2, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan
Purpose:
Perivascular epithelioid cell tumor (PEComa) is a rare group of mesenchymal tumor. PEComa from bladder is extremely rare and the previous report is limited. We present a case of bladder PEComa.
Case presentation:
This 60-year-old woman had underlying disease of hypertension and uterine myoma. She suffered from right flank pain for a month and discomfort progressed recently. She also had poor appetite and body weight loss. She denied fever, hematuria or lower urinary tract symptoms. Abdomen CT revealed right hydronephrosis, right pelvis mass (4.5 x 4.0 x 3.3 cm) with low ureter and bladder compression. We arranged cystoscopy at first and a right lateral wall bulging mass by extravesical external compression noted. No intravesical mucosa lesion was noted. Right ureteroscopy showed patent right ureter with external compression. Then we shifted to open surgery for tumor total excision with right Gibson incision. A right pelvis solid mass was found with right distal ureter adhesion and bladder wall detrusor muscle involvement. The tumor was free from uterus. Tumor was removed completely combined with right distal ureterectomy, partial cystectomy and ureter reimplantation. Patient recovered well after operation and discharged from ward after 10 days.
Pathologic report revealed tumor tissue composing of nests and sheets of spindle to epithelioid cells bearing clear or granular pinkish cytoplasm separated by delicate fibrous septa. No infiltrative growth, cytologic atypia, necrosis, or increased mitotic activity is found. Immunohistochemical stain revealed SMA and HMB45 positive, consistent with bladder PEComa
Discussion:
PEComa may be originated from uterus, ovary, cervix or other soft tissue. Some patients with PEComa are related to tuberous sclerosis complex (TSC). In this patient, this tumor is not originated from uterus. However, we found that tumor was originated from serosal layer and detrusor muscle layer of bladder wall. This is a rare anatomical site of PEComa. She didn’t reveal other sign of TSC. According to previous literature review, less than 30 cases of bladder PEComa were reported and non of them are related with TSC. We performed a complete excision and recontrstruction for her and patient’s discomfort was relieved. Further follow up is necessary to determine the tumor behavior.