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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2012  |  Volume : 23  |  Issue : 5  |  Page : 1032-1034
PEComa of the urinary bladder

1 Department of Urology, Apollo Hospitals, Chennai, Tamil Nadu, India
2 Department of Pathology, Apollo Hospitals, Chennai, Tamil Nadu, India

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Date of Web Publication13-Sep-2012


Perivascular epithelioid cell tumors are a family of related mesenchymal neo­plasms that include angiomyolipoma, lymphangiomyomatosis, clear cell sugar tumor of the lung and a group of rare morphologically and immunophenotypically similar lesions that show immunoreactivity for both melanocytic (HMB-45 and/or melan-A) and smooth muscle (actin and/or desmin) markers. This case is presented for its rare occurrence and characteristic mor­phologic and immunohistochemical features.

How to cite this article:
Shringarpure SS, Thachil JV, Maya M. PEComa of the urinary bladder. Saudi J Kidney Dis Transpl 2012;23:1032-4

How to cite this URL:
Shringarpure SS, Thachil JV, Maya M. PEComa of the urinary bladder. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2021 Jun 24];23:1032-4. Available from: https://www.sjkdt.org/text.asp?2012/23/5/1032/100943

   Introduction Top

Perivascular epithelioid cell tumors (PEComas) are rare tumors that share a distinctive cell type - perivascular epitheloid cell "PEC" (which has no tissue counterpart). They are a family of related mesenchymal neoplasms that include angiomyolipoma, lymphangiomyomatosis and clear cell sugar tumor of the lung. PEComas are composed of nests and sheets of usually epitheloid but occasionally spindled cells with clear to granular cytoplasm and focal asso­ciation with blood vessels. Here, we present a rare case of PEComa of urinary bladder who presented with painless hematuria.

   Case Report Top

A 39-year-old male presented with history of painless terminal hematuria for the past one month. Ultrasound abdomen showed a 3 cm mass near the left vesicoureteric junction. All other blood investigations were normal. Cystoscopy revealed a solid sessile 3 cm × 3 cm tumor superior and medially to the left ureteric orifice. The tumor was resected transurethrally. Final histopathology of the tumor showed spindled and epithelioid clear cell neoplasm [Figure 1]. Immunohistochemistry was posi­tive for Vimentin, smooth muscle actin, S 100, MSA and HMB45 and negative for cytokeratin and Desmin [Figure 2]. Thus, a diagnosis of PEComa of the urinary bladder was made. Retrospective detailed his­tory and physical examination failed to reveal features of tuberous sclerosis. Because of the benign nature of the tumor, the patient was followed-up by active surveillance. Repeat check cystoscopy after three months showed no re­currence of the tumor.
Figure 1: Tumor cells were mainly epithelioid with abundant eosinophilic and granular cytoplasm. Occasional nuclear inclusions were present (hematoxylin– eosin, original magnification ×400).

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Figure 2: Tumor cells demonstrated strong and diffuse cytoplasmic positivity for HMB45 and Melan-A (Melan-A immunoperoxidase, original magnification ×400).

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   Discussion Top

Bonetti et al [1] first described PEComas. All these tumors all share a distinctive cell type - PEC (which has no tissue counterpart). PEComas are composed of nests and sheets of usually epitheloid but occasionally spindled cells with clear to granular cytoplasm and fo­cal association with blood vessels. Nearly all PEComas show immunoreactivity for both melanocytic (HMB-45 and or melan-A) and smooth muscle actin and/or desmin) markers. A subset of PEComas behaves in a malignant fashion.

PEComas have been reported in a variety of locations - uterus, falciform ligament, vulva and prostate. [2] Primary PEComa of urinary bladder was reported by Parfitt et al, [3] who described a 48-year-old male with a bladder tumor with enterovesical fistula who under­went partial cystectomy with resection and small bowel anastomosis, who was treated with interferon (IFN)-alfa therapy.

Pan et al [4] reported a case of PEComa occur­ring in the deep detrusor muscle of the bladder in a 33-year-old woman, which was an inci­dental discovery during work-up for dysmenorrhea. Parmeswaran et al [5] described a case of PEComa of urinary bladder in a 19-year-old female that was excised transurethrally.

Sukov et al reported three cases of PEComa arising in the urinary bladder in two men in their fourth decade and one woman in her third decade. [6] All three tumors showed histologic features characteristic of PEComa, including spindled and epithelioid cell morphology with variable clear cell change, and all co-expressed melanocytic- and smooth muscle-associated markers by immunohistochemistry. Follow-up demonstrated an indolent course for two pa­tients with no evidence of disease at ten and 21 months, respectively, and the third case was recently diagnosed.

Folpe et al in their study of PEComas of soft tissue and gynecologic origin showed that recurrence and/or metastasis was strongly associated with tumor size > median size (8 cm), mitotic activity greater than 1/50 HPF and necrosis. Small PEComas without any worrisome histologic features are most likely benign. PEComas with nuclear pleomorphism alone ("symplastic") and large PEComas with­out other worrisome features have uncertain malignant potential. PEComas with two or more worrisome histologic features should be considered malignant. Occasional PEComas express unusual markers, such as S-100 pro­tein, desmin and, rarely, CK. The role of Trans­cription Factor E3 TFE3 in PEComas should be further studied. [7] Optimal treatment for PEComas is not known at this time. Primary excision is usually cura­tive, as most tumors are benign. However, lo­cally advanced or metastatic disease tends to have a poor prognosis and strategies incorpo­rating chemotherapy, radiation and immunotherapy have been reported. Further studies are needed to clarify the clinical and pathological effects of IFN-α therapy in patients with PEComa and the risks of IFN therapy should be weighed against the potential benefits, if any, in patient lacking detectable residual tumor. Partial, complete and absent responses have been noted for dacarbazine, vincristine and imatinib mesylate, a tyrosine-kinase inhibitor.

   References Top

1.Bonetti F, Pea M, Martignoni G. The perivascular epitheloid cell and related lesions. Adv Anat Pathol 1997;4:343-58.  Back to cited text no. 1
2.Folpe AL, Goodman ZD, Ishak KG. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: A novel member of theperivascular epithelioid clear cell family of tumors with a predilection for children and young adults. Am J Surg Pathol 2000;24:1239-46.  Back to cited text no. 2
3.Parfitt JR, Bella AJ, Wehrli BM, Izawa JI. Primary PEComa of the bladder treated with primary excision and adjuvant interferon-alpha immunotherapy: A case report. BMC Urol 2006;6:20.  Back to cited text no. 3
4.Pan CC, Yu IT, Yang AH, Chiang H. Clear cell myomelanocytic tumor of the urinary bladder. Am J Surg Pathol 2003;27:689-92.  Back to cited text no. 4
5.Kalyanasundaram K, Parameswaran A, Mani R. Perivascular epithelioid tumor of urinary bladder and vagina. Ann Diagn Pathol 2005; 9:275-8.  Back to cited text no. 5
6.Sukov WR, Cheville JC, Amin MB, Gupta R, Folpe AL. Perivascular Epithelioid Cell Tumor (PEComa) of the Urinary Bladder: Report of 3 Cases and Review of the Literature. Am J Surg Pathol 2009;33:304-8.  Back to cited text no. 6
7.Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol 2005;29:1558-75.  Back to cited text no. 7

Correspondence Address:
Sanish S Shringarpure
Department of Urology, Apollo Hospitals, Chennai 600006, Tamil Nadu
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DOI: 10.4103/1319-2442.100943

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