Perivascular epithelioid cell tumor of the ascending colon mesentery in a child: case report and review of the literature

Gross, Eitan; Vernea, Fiona; Weintraub, Michael; Koplewitz, Benjamin Z.

doi:10.1016/j.jpedsurg.2010.01.015

Cited by 27 publications

(27 citation statements)

References 12 publications

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“…The ratio of PEComa incidenceinwomenandmenis7:1,indicatingitsmarkedpredominanceinwomen,withapproximately40%ofthetumors The clinicopathological features of the mesenteric PEComa cases included in this study are summarized in table 1 [4,8,9]. Inclusive of the present case, 4 mesenteric PEComashavebeenreported:2inwomen,1inanadultmale, and1inayoungboy.Thetumorsrangedfrom4to27cmin size.Duringfollow-up,2patients(cases1and2)showedno recurrence,whereastheremaining2patients(cases3and4) with at least 3 high-risk features showed local recurrences.…”

Section: Discussionmentioning

confidence: 70%

“…Gastrointestinal PEComas arerarewithapproximately20casesdescribedinthelitera-turesofar [4,[8][9][10].MostreportedgastrointestinalPEComas areofthecolon,andonly3casesofmesentericPEComahave beendescribedintheliterature [4,8,9].…”

Section: Disclosure Statementmentioning

confidence: 99%

“…PEComas usually show positive immunohistochemical staining for both melanocytic markers, HMB-45 and melan-A, and the smooth muscle markersactinanddesmin [4,5,9,11]whichhelpindifferentiatingbetweengastrointestinalstromaltumors,clearcellcarcinomas, and smooth cell tumors of the gastrointestinal tract. Inourcase,thetumortestedpositiveforHMB-45,melan-A, andactin,whichclearlyconfirmedthediagnosisofPEComa.…”

Section: Disclosure Statementmentioning

confidence: 99%

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

Lai

Hsu

et al. 2012

Onkologie

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Background: Perivascular epithelioid cell tumors (PEComas) are very rare mesenchymal neoplasms, and have been found in various organs such as the liver, kidney, falciform ligament, uterus, uterine cervix, and both the small and large bowel. However, only 3 cases of mesenteric PEComa have been described in the literature so far. The treatment and prognosis of malignant mesenteric PEComas are discussed. Case Report: We report the case of a 59-year-old man diagnosed with PEComa. He underwent segmental resection of the jejunum and tumor resection. Malignant mesenteric PEComa was confirmed on the basis of clinicopathological features. Tumor resection was followed by concurrent chemoradiotherapy. Conclusion: Besides surgery, no effective treatment for malignant PEComa has been established thus far because of the rarity of this tumor. Here, we report our experience of treating a malignant mesenteric PEComa using surgery and subsequent adjuvant therapy, which effectively controlled disease progression and prevented local recurrence.

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Section: Discussionmentioning

confidence: 70%

Section: Disclosure Statementmentioning

confidence: 99%

Section: Disclosure Statementmentioning

confidence: 99%

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

Lai

Hsu

et al. 2012

Onkologie

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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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Polyps and Tumors of the Gastrointestinal Tract in Childhood

Pawel

2024

Pathology of Pediatric Gastrointestinal and Liver Disease

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Perivascular epithelioid cell tumor of the ascending colon mesentery in a child: case report and review of the literature

Cited by 27 publications

References 12 publications

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

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

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

Polyps and Tumors of the Gastrointestinal Tract in Childhood

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