Perivascular Epithelioid Cell Tumor of the Heart in a Child

Tai, Yoshiaki; Wei, Lixin; Shi, Huaiyin

doi:10.2350/09-10-0726-cr.1

Cited by 10 publications

(12 citation statements)

References 9 publications

(10 reference statements)

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“…PEComas have been found in various organs and have a tendency to affect women with approximately 40% of tumors originated from the uterus [2], although some unusual sites can be also involved occasionally, such as vagina [11], orbit [12], small and large bowel [13], heart [14] prostate [15], bladder [16] and endometrium [17]. Multifocal PEComas (PEComatosis) have been described in the literature [18].…”

Section: Discussionmentioning

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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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%

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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“…To date, only 5 cases of cardiac PEComas were reported [10, 14–17]. The clinicopathologic features of those cases were summarized in Table 4.…”

Section: Discussionmentioning

confidence: 99%

Cardiac Epithelioid PEComa: Report of Two Cases and Review of the Literature

Niu

Wang

Zhang

et al. 2012

Case Reports in Medicine

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Cardiac PEComa is very rare. We reported two cases of epithelioid PEComas, one in an adult and one in a 2-year-old child. Both tumors were composed of sheets of epithelioid cells with coagulation necrosis. In addition, the adult case showed marked nuclear atypia and high mitotic activity with atypical mitosis and the pediatric case showed unusual clear cell features. Immunohistochemically, both tumors were positive for HMB-45 and SMA and negative for S100 and cytokeratin. Electron microscopy was performed in the pediatric case and showed premelanosomes. The adult patient developed extensive metastasis indicating malignant behavior. Prior to the two cases, only 5 other cases of cardiac PEComa were reported and the literatures are reviewed.

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“…Focal and occasionally remarkable expression of S-100 protein, SMA, desmin, CD117 (c-KIT) and CD 34 has been noted in these tumours [16, 17]. PEComas have a widespread distribution, with reports of cases occurring in sites such as gastro-intestinal tract, soft tissues and female genital organs, but also in the heart [18], laryngopharyx [19] and pancreas [17]. …”

Section: Discussionmentioning

confidence: 99%

“…S-100 expression has been reported in both PEComas [18] and spindle cell GISTs [20, 24] (telocytes are also positive for S-100) [5]. …”

Section: Discussionmentioning

confidence: 99%

“…(3) SMA is expressed in GISTs [23], as well as in the vast majority of PEComas [16] (telocytes have positive expression of ␣SMA) [9]. (4) S-100 expression has been reported in both PEComas [18] and spindle cell GISTs [20,24] (telocytes are also positive for S-100) [5]. (5) PEComas as well as GISTs are occasionally CD34-positive [17,23] (telocytes have positive expression for CD34) [5,9,25,26].…”

Section: ) Cd117 (C-kit) Expression Is Proper Of the Vast Majority (mentioning

confidence: 99%

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Telocytes are the common cell of origin of both PEComas and GISTs: an evidence-supported hypothesis

Ardeleanu

Bussolati

2011

Journal of Cellular and Molecular Medicine

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We advance the hypothesis that the telocyte might be the cell of origin of both PEComas (perivascular epithelioid cell tumours) and GISTs (gastro-intestinal and extra-gastrointestinal stromal tumours). The hypothesis is supported by data from the literature reporting that both PEComas and GISTs, as well as telocytes, share the expression of several markers. These data were supplemented by original immunohistochemical tests on selected series. Specifically: (1) Melanoma markers (Melan A, MiTF) typical of PEComas are expressed by a substantial fraction of GISTs. A fraction of GISTs was also found positive for CD63, a tetraspanin protein originally described in melanomas and marking exosomes. (2) c-KIT (CD117), proper of the vast majority of GISTs, can be expressed by PEComas (as well as by telocytes). (3) Markers described in telocytes (CD34, S-100, smooth muscle actin and vascular endothelial growth factor) have been reported as positive in cases of PEComas and GISTs. Telocytes show distinctive ultrastructural features with thin, extended, telopodes and are likely involved in inter-cellular signalling via paracrine secretion as well as by shed vesicles and exosomes. These cells have been described in many locations (cavitary and non-cavitary organs) and might display potentialities of a wide spectrum of differentiation (and function). In conclusion we propose that telocytes could be the common cells of origin for both PEComas and GISTs.

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Perivascular Epithelioid Cell Tumor of the Heart in a Child

Cited by 10 publications

References 9 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

Cardiac Epithelioid PEComa: Report of Two Cases and Review of the Literature

Telocytes are the common cell of origin of both PEComas and GISTs: an evidence-supported hypothesis

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