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

Lai, C Z; Hsu, Kuo-Feng; Yu, Jyh-Cherng; Chen, Cheng‐Jueng; Hsieh, Chung-Bao; Chan, De‐Chuan; Li, Heng-Sheng; Hsu, Hung-Ming

doi:10.1159/000336826

Cited by 13 publications

(11 citation statements)

References 4 publications

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“…Multifocal PEComas (PEComatosis) have been described in the literature [18]. Mesenteric PEComas are rare with only 6 cases described in the literature so far [3-5] (Table 1). The ratio of mesenteric PEComa incidence in women and men is 2:1.…”

Section: Discussionmentioning

confidence: 99%

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Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: a case report and review of literature

Jiang

et al. 2013

Diagn Pathol

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Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1309992178882788Perivascular epithelioid cell tumor (PEComa) is a rare but distinct mesenchymal neoplasm composed of histologically and immunohistochemically unique perivascular epithelioid cells. Due to its relative rarity, little is known about the histogenesis and prognostic factors of this tumor. We describe a case of unusual mesenteric PEComa in a 38-year-old female patient with regional lymph node involvement. Histologically, the tumor was composed of sheet of epithelioid cells with abundant clear or eosinophillic cytoplasms. Extensive coagulative necrosis and a few mitotic figures (2/50 high power field) could be found in tumor. The epithelioid tumor cells were diffusely positive for HMB-45, Melan-A, and focally positive for calponin. One of enlarged mesenteric lymph nodes was observed to be involved by tumor. A diagnosis of malignant mesenteric PEComa with lymph node involvement was made. The patient received chemotherapy after total resection of tumor and segmental resection of involved jejunum. There was no sign of recurrence of tumor found in period of 6-month regular follow-up after chemotherapy. To our knowledge, this is the first case of malignant PEComa in mesentery accompanied with regional lymph node involvement. The literature on this rare tumor is reviewed and diagnostic criteria of malignant PEComa are discussed.

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Section: Discussionmentioning

confidence: 99%

“…PEComas other than AML, CCST or LAM are rare. Although increasingly reported over the past decade, PEComas occurring in mesentery are exceedingly rare, with only 6 cases described in the literature so far [3-5]. The clinical behavior of PEComa is not predictable, and there are no strict histologic criteria for malignancy.…”

Section: Introductionmentioning

confidence: 99%

Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: a case report and review of literature

Jiang

et al. 2013

Diagn Pathol

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“…The clinical behaviour of pecomas is generally benign. However, pecomas can display aggressive clinical behaviour, and some reported cases resulted in death 10,11 .…”

Section: Discussionmentioning

confidence: 99%

“…Perivascular epithelioid cell tumours of the mesentery are remarkably rare, and fewer than 10 cases are currently reported in the literature 4,[9][10][11][12] . To the best of our knowledge, the literature current contains no reports describing a pecoma of the terminal ileum mesentery as a secondary tumour in a survivor of childhood cancer.…”

Section: Introductionmentioning

confidence: 99%

PEComa of the Terminal Ileum Mesentery as a Secondary Tumour in an Adult Survivor of Embryonal Rhabdomyosarcoma

et al. 2015

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Perivascular epithelioid cell tumours (pecomas) are rare mesenchymal tumours that are characterized by perivascular epithelioid cell differentiation and immunoreactivity to myogenic and melanocytic markers. These tumours can be classified as benign, uncertain malignant potential, or malignant. Because of the rarity of pecomas, their cause and clinical prognosis remain unclear.To the best of our knowledge, no reports in the literature describe a pecoma of the terminal ileum mesentery as a secondary tumour in an adult survivor of childhood embryonal rhabdomyosarcoma, let alone any childhood cancer. Here, we present the case of a 27-year-old man with a pecoma involving the mesentery of the terminal ileum. At the age of 5, he had been treated with a combination of chemotherapy and high-dose pelvic radiation therapy for embryonal rhabdomyosarcoma, most likely arising from the posterior bladder wall.During routine follow-up 22 years after this patient's initial treatment, computed tomography imaging revealed a mass within the terminal ileum mesentery. The tumour was successfully treated with surgical resection, and pathology examination determined the mass to be a pecoma with uncertain malignant potential.This first case of a pecoma of the terminal ileum mesentery arising within a high-dose radiation therapy field as a secondary tumour in an adult survivor of childhood cancer highlights the importance of screening and surveillance in high-risk childhood cancer survivors treated with high-dose radiation therapy. Further research to build a better understanding of this remarkably rare tumour is warranted.

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“…7 However, locally advanced or metastatic dis ease portends a poor prognosis, and strategies incorporat ing chemotherapy (dacarbazine, vincristine and imatinib mesylate), radiation and immunotherapy have been reported. 4,8,9 Since PEComa can be aggressive, careful follow-up is recommend- Primary PEComa of the prostate ed. In the future, new targeted therapies may emerge as definitive treatments for localized aggressive and metastatic PEComa.…”

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

Cited by 13 publications

References 4 publications

Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: a case report and review of literature

Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: a case report and review of literature

PEComa of the Terminal Ileum Mesentery as a Secondary Tumour in an Adult Survivor of Embryonal Rhabdomyosarcoma

Primary perivascular epithelioid cell tumour (PEComa) of the prostate

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