Abdominopelvic Perivascular Epithelioid Cell Tumor with Overt Malignancy: A Case Report

Chen, I-Yu; Yang, Sheau-Fang; Chai, Chee‐Yin; Chen, Fang‐Ming

doi:10.1016/s1607-551x(09)70201-3

Cited by 12 publications

(10 citation statements)

References 20 publications

(19 reference statements)

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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: 69%

“…[3] hypothesized that PECs are capable of modulating their morphology and immunophenotype. PEComas have been referred to by various names reflecting the diversity of the pathologicalfindings.ThePEComafamilyoftumorsincludes angiomyolipoma, lymphangioleiomyomatosis, abdominopelvic sarcoma of PECs, clear cell sugar tumor, clear cell myomelanocytictumor,pulmonarysugartumor,andprimary extrapulmonarysugartumor [4,5].…”

Section: Discussionmentioning

confidence: 99%

“…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: 69%

Section: Discussionmentioning

confidence: 99%

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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“…Sporadic cases have been treated with chemotherapy with hardly any effect. [8][9][10] In this patient, we had to deal with an additional clinical problem: in addition to the rare pathology, chyle was leaking until 2 months postoperatively. With our conservative treatment, this problem was adequately solved; however, the possible malignant fashion of the tumor has no further been explored.…”

Section: Discussionmentioning

confidence: 99%

Perivascular Epithelioid Cell Tumor of the Retroperitoneum in a Young Woman Resulting in an Abdominal Chyloma

Lans

Ramshorst

Hermans

et al. 2009

Journal of Gastrointestinal Surgery

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Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm which appears to have predominancy for young, frequently Asian, women. The neoplasm is composed chiefly of HMB-45-positive epithelioid cells with clear to granular cytoplasm and usually showing a perivascular distribution. These tumors have been reported in various organs under a variety of designations. Malignant PEComas exist but are very rare. The difficulty in determining optimal therapy, owing to the sparse literature available, led us to present this case. We report a retroperitoneal PEComa discovered during emergency surgery for abdominal pain in a 28-year-old Asian woman. The postoperative period was complicated by chylous ascites that was initially controlled by a wait-and-see policy with total parenteral nutrition. However, the chyle production gradually increased to more than 4 l per day. The development of a bacterial peritonitis resulted in cessation of production of abdominal fluid permitting normal nutrition without chylous leakage. Effective treatment for this rare complication of PEComa is not yet known; therefore, we have chosen to engage in long-term clinical follow-up.

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Perivascular epithelioid cell neoplasms (PEComas): four malignant cases expanding the histopathological spectrum and a description of a unique finding

et al. 2007

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Four cases of malignant PEComas were stained with smooth muscle actin, muscle specific actin, desmin, human melanoma black-45, melan-A, microphthalmia transcription factor, S100 and cyclin D1. One case was studied by electron microscopy (EM). Tumour locations were the thigh, elbow, retroperitoneum and bladder in association with a urachal cyst. There were two men and two women; the average age was 51.3 years, and the size ranged from 5.0-23.0 cm. In three cases, 50-95% of the tumour was composed of pleomorphic sarcomatous areas. All cases had at least focal clear-cell areas. One case showed a continuous single layer of perivascular clear cells remote from the tumour, transitioning to invasive nests and to PEComa. EM demonstrated these cells in apposition to and in direct contact with the abluminal surface of the basal lamina of the capillaries. We suggest the term "pecosis" for these areas. All cases were positive for two or more melanocytic markers and for at least one actin. S100 and desmin were focally positive in one case. Cyclin D1 was positive in 3:4 cases. Four cases of malignant PEComa are described with the existence of a unique lesion (pecosis) in one case. These tumours may manifest largely as sarcomas appearing to be undifferentiated and should be considered in their differential diagnosis.

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Abdominopelvic Perivascular Epithelioid Cell Tumor with Overt Malignancy: A Case Report

Cited by 12 publications

References 20 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

Perivascular Epithelioid Cell Tumor of the Retroperitoneum in a Young Woman Resulting in an Abdominal Chyloma

Perivascular epithelioid cell neoplasms (PEComas): four malignant cases expanding the histopathological spectrum and a description of a unique finding

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