Perivascular epithelioid cell neoplasm of the urinary bladder in an adolescent: a case report and review of the literature

Liu, Yin; Bu, Hong; Chen, Min; Yu, Jianqun; Zhuang, Hua; Chen, Jie; Zhang, Hongying

doi:10.1186/1746-1596-7-183

Cited by 22 publications

(19 citation statements)

References 30 publications

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“…Some patients present with abdominal pain, as was the case with this patient, while others have clinical signs and symptoms mimicking urothelial carcinoma, such as haematuria 6 7 9–11. The sole paediatric case presented with vague abdominal discomfort and urinary frequency without haematuria 8. A number of reports have also described patients with lower urinary tract symptoms (including dysuria, frequency and urgency),6 8 10 12 which is also similar to this case.…”

Section: Discussionsupporting

confidence: 69%

“…The sole paediatric case presented with vague abdominal discomfort and urinary frequency without haematuria 8. A number of reports have also described patients with lower urinary tract symptoms (including dysuria, frequency and urgency),6 8 10 12 which is also similar to this case. This lesion may also present as an asymptomatic palpable mass or as an incidental finding on workup for another condition 7 13.…”

Section: Discussionsupporting

confidence: 69%

“…Based on prior case reports, the mean age at diagnosis for bladder PEComa is 36, with most cases presenting between the third and fifth decade 7. There is, however, a single report of bladder PEComa in a paediatric patient—a 16-year-old Chinese girl with no known syndrome 8. Although PEComas of other anatomic sites are often associated with tuberous sclerosis, there are no reports of bladder PEComas associated with tuberous sclerosis 4–21.…”

Section: Discussionmentioning

confidence: 99%

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Perivascular epithelioid cell tumour of the bladder

et al. 2014

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SUMMARYA 39-year-old woman presented with a long history of pelvic pain and urinary urgency. Prior workup by her primary care doctor had been negative. The patient's gynaecologist ultimately referred her to a urologist following an ultrasound that revealed a possible bladder mass. MRI of the abdomen and pelvis demonstrated a 4 cm soft tissue lesion arising from the bladder. Cystoscopy showed an atypical mass on the anterior bladder wall, and pathological examination of the TURBT (transurethral resection of the bladder tumour) specimen revealed a perivascular epithelioid cell tumour (PEComa) with involvement of the detrusor muscle. The patient underwent a robotically assisted laparoscopic partial cystectomy. Final pathology confirmed a PEComa with negative margins. The patient had an uncomplicated postoperative course and is doing well following surgery. A surveillance cystoscopy at 6 months showed no evidence of recurrence. This case underscores the variability of clinical presentation of PEComas while proposing an appropriate method of surgical management. BACKGROUND

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Section: Discussionsupporting

confidence: 69%

Section: Discussionsupporting

confidence: 69%

Section: Discussionmentioning

confidence: 99%

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Perivascular epithelioid cell tumour of the bladder

et al. 2014

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“…5 In 2003, Pan and colleagues 6 reported the first prostatic PEComa. The tumour was composed of a variable percentage of epithelioid and spindle cells with clear to granular cyto plasm arranged in nests separated by a vascular stroma.…”

Section: Discussionmentioning

confidence: 99%

Primary perivascular epithelioid cell tumour (PEComa) of the prostate

Eken

Sağlıcan

2014

CUAJ

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Perivascular epithelioid cell tumours (PEComas) are a family of rare mesenchymal tumours arising in various anatomic locations. PEComas are defined by the presence of perivascular epithelioid cells that coexpress muscle and melanotic markers, especially HMB-45. They have unpredictable biological behaviour and are mostly benign, but some tumours can become unresectable or metastatic. Surgical resection, when possible, is the best treatment option. Radiation therapy, cytotoxic chemotherapy or immunotherapy have been reported as treatment options, either alone or in combination therapy. Prostatic PEComa is extremely rare, with only 1 malignant case reported. We report the first case of prostatic PEComa, which was benign and treated with transurethral resection.

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“…2 Perivascular epithelioid cell tumors have been previously reported in a variety of sites including the heart, 4 kidney, 5 urinary bladder, 6 prostate, esophagus, 7 intestines, 8 pancreas, 3 liver, uterus, 9 ovary, vulva, lung, palate, bone, 10 falciform ligament, retroperitoneal soft tissue, thigh, groin, and skin. 1,2,11 Perivascular epithelioid cell tumors typically display nested architecture and are composed of epithelioid cells with a radial arrangement around the vascular lumen and a clear to granular, lightly eosinophilic cytoplasm.…”

Section: Microscopic Examinationmentioning

confidence: 99%

Malignant Perivascular Epithelioid Cell Tumor of the Gallbladder: A Case Report and Review of Literature

Zhao

Anders

2014

Archives of Pathology &Amp; Laboratory Medicine

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Immunohistochemically, the perivascular epithelioid cells show positivity for melanocytic markers, such as HMB-45 and Melan-A, as well as smooth muscle markers, such as actin and myosin.1 Here, we report the first case of a malignant PEComa arising in the gallbladder. REPORT OF A CASEThe patient was a 46-year-old woman who was diagnosed with high-grade invasive ductal carcinoma of the left breast showing left sentinel node metastasis in 2010. Both estrogen and progesterone receptor status were negative by immunohistochemistry. She received 4 cycles of preoperative chemotherapy, followed by left mastectomy and left axillary lymph node dissection. She then received adjuvant chemotherapy, as well as regional radiotherapy. In September 2012, the patient presented with right upper quadrant pain. An ultrasound of the abdomen suggested a primary gallbladder wall malignancy with regional nodal metastasis and possible hepatic invasion. A computed tomography scan of the abdomen and pelvis revealed a gallbladder mass extending into the pericholecystic fat and inseparable from the liver, with an enlarged portacaval lymph node measuring up to 3.3 cm with mild compression of the inferior vena cava (Figure 1, A). A computed tomography scan of the chest showed no evidence of distant metastatic disease. She subsequently underwent an open cholecystectomy in October 2012. Macroscopic ExaminationThe cholecystectomy specimen weighed 62.2 g and consisted of a previously opened gallbladder resected en bloc with its underlying 6.5 3 4.0 3 2.5-cm liver bed. The fixed gallbladder measured up to 11.0 cm. There was a large tumor mass within the lumen of the gallbladder that measured 5.5 3 4.5 3 3.3 cm (Figure 1, B). The tumor was arising in the fundic portion of the gallbladder and grossly did not extend closer than 4.0 cm from the cystic duct resection margin. The tumor invaded the wall of the gallbladder. Aside from this large tumor the gallbladder mucosa was otherwise normal. No calculi were identified. Microscopic ExaminationThe tumor infiltrated through the full thickness of the gallbladder wall. It abutted the serosa, but the tumor cells were separated from the serosal surface by a thin strand of connective tissue. The neoplastic cells were epithelioid and characterized by clear to granular, lightly eosinophilic cytoplasm and small, centrally located, normochromatic, round to oval nuclei (Figure 2, A and B). The nuclei showed slight variation, and most demonstrated small nucleoli. The cells had fairly distinct cell borders and were arranged as nests and sheets. Scattered mitotic figures were noted. There was no evidence of necrosis or hemorrhage. Clearly defined lymphovascular space invasion was present. Aside from the fundic tumor, the remaining gallbladder mucosa, including cystic duct sections, was benign. There were small nodules of tumor in the liver with similar morphology, underlying the gallbladder, which did not involve the resected margin of the liver. Contiguous spread between the liver tumor and the gallbladder tumor was...

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Perivascular epithelioid cell neoplasm of the urinary bladder in an adolescent: a case report and review of the literature

Cited by 22 publications

References 30 publications

Perivascular epithelioid cell tumour of the bladder

Perivascular epithelioid cell tumour of the bladder

Primary perivascular epithelioid cell tumour (PEComa) of the prostate

Malignant Perivascular Epithelioid Cell Tumor of the Gallbladder: A Case Report and Review of Literature

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