Bladder Perivascular Epithelioid Cell Tumor: Dynamic CT and MRI Presentation of 2 Cases With 2-year Follow-up and Review of the Literature

Xiang-ke, DU; Guo, Hong; Zhang, Weiguo

doi:10.1016/j.clgc.2019.06.016

Cited by 9 publications

(16 citation statements)

References 28 publications

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“…Tumours are more common in the females. The sizes of tumours reported in the literature range from 1.5-18 cm, with the median size being between 4-5 cm [1,6,7].…”

Section: Discussionmentioning

confidence: 99%

“…Non-contrast MRI in patients with PEComa has shown hypointense masses in T1-weighted imaging and hyperintense masses in T2-weighted imaging [1,4,8]. PEComa tumours are usually described as solid masses with occasional cystic changes [2,4,7]; however, available studies suggest that PEComa can also take the form of a multicystic mass with fluid-fluid levels [6]. Most tumours were well demarcated and characterized by regular shape and heterogeneous contrast enhancement with late washout.…”

Section: Discussionmentioning

confidence: 99%

“…It was characterized by heterogeneous post-contrast enhancement. Internal septa of varying thickness may be present within the tumour mass, as well as enhancing areas of necrosis in the centre of the lesion [7]. In some cases of PEComas, the presence of high intensity signal of fat in T1-weighted images has been described [6].…”

Section: Discussionmentioning

confidence: 99%

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Radiological features of perivascular epithelioid cell tumours (PEComas) in a paediatric patient – Case report

Karska¹,

Kozioł²,

Leśniewska³

et al. 2022

J Pre Clin Clin Res.

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Introduction. Perivascular epithelioid cell tumour (PEComa) is a rare family of mesenchymal tumours composed of epithelioid cells. Due to its very rare occurrence, little information is available on the imaging characteristics of this type of lesion. Case Report. A 16-year-old patient was admitted for diagnosis of an incidentally found lesion of unclear origin on MRI of the lumbar spine. Targeted MRI revealed a pathological solid-cystic mass within the pelis, in the left rectovesical pouch, infiltrating the bladder wall. A biopsy of the tumour was performed. On histopathological examination, a PEComa tumour was diagnosed. Control examinations were performed. The tumour was treated with embolization, surgical resection and Sirolimus therapy. Conclusions. The tumour showed the characteristic features of PEC-oma on imaging studies reported in the literature. Radiological diagnosis is not fully specific, therefore histopathological examination is necessary for a definitive diagnosis.

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“…Tumours are more common in the females. The sizes of tumours reported in the literature range from 1.5-18 cm, with the median size being between 4-5 cm [1,6,7].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Radiological features of perivascular epithelioid cell tumours (PEComas) in a paediatric patient – Case report

Karska¹,

Kozioł²,

Leśniewska³

et al. 2022

J Pre Clin Clin Res.

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“… 1 Overall, PEComas show a female predilection; earlier work suggested bladder PEComas were more common in males; 6 however, a recent review describes a slight female predominance, with a median age of 32.1 years (range = 16–65). 7 PEComas may present with non-specific local symptoms but are frequently incidental findings. Bladder PEComas may be associated with abdominopelvic discomfort, lower urinary tract symptoms and haematuria, 6 at times mimicking urothelial carcinoma clinically.…”

Section: Discussionmentioning

confidence: 99%

Bladder perivascular epithelioid cell neoplasm: The importance of immunohistochemistry in the diagnosis of an unusual neoplasm

Noon

Ayres

Patel

et al. 2021

SAGE Open Medical Case Reports

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Perivascular epithelioid cell neoplasms represent a group of uncommon mesenchymal tumours with as yet undiscovered benign counterpart. Although perivascular epithelioid cell neoplasms have been described arising in most organ systems as well as in soft tissue and bone, only a small number of perivascular epithelioid cell neoplasms have been reported in the bladder. To date, there is no agreed system for predicting the behaviour of these tumours. We describe a case of a perivascular epithelioid cell neoplasm of the bladder arising in a 57-year-old male and initially diagnosed on biopsy and present a review of the literature focussing on the pathological differential diagnosis and the importance of key histological features in conjunction with a broad immunohistochemical panel. This case report highlights the key features of bladder perivascular epithelioid cell neoplasms that distinguishes these rare neoplasms from other bladder lesions.

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“…PEComas are known to occur in the genitourinary tract 9-14 including the urinary bladder; however, there are only a few reported cases of primary PEComa of the urinary bladder in the literature. 15-39 Despite improvements in diagnostic modalities, PEComa remains a diagnostic challenge for radiologists as well as pathologists. Additionally, due to the limited number of cases, prognostic assessment is also difficult.…”

Section: Introductionmentioning

confidence: 99%

Perivascular Epithelioid Cell Tumor of the Urinary Bladder: A Systematic Review

Hamza

Guo

2019

Int J Surg Pathol

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Perivascular epithelioid cell tumor (PEComa) of the urinary bladder is a rare neoplasm showing distinct melanocytic and smooth muscle differentiation. We aimed to review the clinicopathologic features of bladder PEComa using all the available cases in the literature, along with 2 new cases from our database. The patients included 15 females and 15 males with a mean age of 39.2 ± 15.3 years. Painless hematuria was the most common clinical presentation. The tumors were usually well circumscribed with a mean tumor size of 4.4 ± 2.7 cm. Bladder PEComas demonstrated nests, trabeculae, or sheets of epithelioid cells with intermixed spindled cells and numerous thin-walled vessels. Immunohistochemical studies showed that the tumors were positive for HMB45 (27/27), cathepsin (4/4), SMA (20/22), and caldesmon (3/3) and were negative for pan cytokeratin (0/18) and EMA (0/4). Molecular studies revealed that PEComa was associated with the TFE3 (n = 3) and EWSR1 (n = 1) gene rearrangements. Treatment included partial cystectomy (n = 18), transurethral resection (n = 8), and radical cystectomy (n = 4). Twenty patients had no evidence of disease during a mean follow-up time of 19.4 ± 17.2 months. Two patients had recurrence, and 1 patient died of metastatic disease. In conclusion, bladder PEComas demonstrate distinct morphologic and immunohistochemical features. Although most tumors follow a benign course, a small subset may develop metastasis and cause death.

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Bladder Perivascular Epithelioid Cell Tumor: Dynamic CT and MRI Presentation of 2 Cases With 2-year Follow-up and Review of the Literature

Cited by 9 publications

References 28 publications

Radiological features of perivascular epithelioid cell tumours (PEComas) in a paediatric patient – Case report

Radiological features of perivascular epithelioid cell tumours (PEComas) in a paediatric patient – Case report

Bladder perivascular epithelioid cell neoplasm: The importance of immunohistochemistry in the diagnosis of an unusual neoplasm

Perivascular Epithelioid Cell Tumor of the Urinary Bladder: A Systematic Review

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