Perivascular epithelioid cell neoplasms: pathology and pathogenesis

Folpe, Andrew L.; Kwiatkowski, David J.

doi:10.1016/j.humpath.2009.05.011

Cited by 342 publications

(321 citation statements)

References 108 publications

(111 reference statements)

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“…The differential diagnosis of an epithelioid PEComa includes malignant melanoma, clear cell sarcoma, paraganglioma, alveolar soft part sarcoma (ASPS) and clear cell carcinoma [19]. The nasal cavity is the most common site for head and neck mucosal melanomas [20].…”

Section: Discussionmentioning

confidence: 99%

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PEComa of the Nose: Report of a Case with Immunohistochemical and Ultrustructural Studies and a Review of the Literature

Afrogheh

Schneider

Bezuidenhout

et al. 2013

Head and Neck Pathol

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Section: Discussionmentioning

confidence: 99%

“…Provisional criteria for malignany have been proposed based on worrisome features of large size ([5 cm), high nuclear grade and cellularity, necrosis and [1 mitotic figures/50 HPF [19]. The development of solid criteria for malignancy may require evaluation of a reasonable number of cases with adequate follow up information.…”

Section: Discussionmentioning

confidence: 99%

PEComa of the Nose: Report of a Case with Immunohistochemical and Ultrustructural Studies and a Review of the Literature

Afrogheh

Schneider

Bezuidenhout

et al. 2013

Head and Neck Pathol

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“…However inactivation of TSC1 and/or TSC2 genes, [30,31] with subsequent activation of mammalian target of rapamycin (mTOR) pathway has been associated with the pathogenesis of both syndromic and sporadic PEComas [32]. Recent studies highlighting genetic events, including chromosome losses (1p, 17p, 18p, 19p) and gains (2q, 3q, 5q, 12q and X) have been indicated in the development of some PEComas [32].…”

Section: Discussionmentioning

confidence: 99%

Perivascular Epithelioid Cell Tumors (PEComas) of the Head and Neck: Report of Three Cases and Review of the Literature

Bandhlish

Barnes

Rabban

et al. 2011

Head and Neck Pathol

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PEComas are a family of neoplastic lesions that share overlapping morphology, immunohistochemistry, and ultrastructure that include angiomyolipoma, lymphangioleiomyomatosis, clear cell ''sugar'' tumor of the lung as well as similar tumors occurring in a variety of visceral, cutaneous and soft tissue sites throughout the body. The defining histopathological features are epithelioid cells with a perivascular distribution containing clear to pale eosinophilic granular cytoplasm and a round-to-oval centrally located nucleus with an inconspicuous nucleolus. Immunohistochemically, coexpression of melanocytic (HMB-45 and/or Melan-A) and myoid markers are characteristic. In the present study, we describe three PEComas occurring in the head and neck (nasal cavity and larynx) and discuss the behavior of these distinctive tumors and review the literature of head and neck PEComas. The importance of recognizing this entity will ensure its consideration in the differential diagnosis of tumors of the head and neck with a similar morphology. The histogenesis of PEComas still remains elusive and additional cases with a prolonged follow up remain important to accurately determine the behavior of these distinctive tumors. Complete surgical excision still remains the treatment of choice for histologically benign PEComas.

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“…11,12 Lesions in this general category include angiomyolipoma (and its so-called epithelioid variant), clear cell ('sugar') tumor and lymphangioleiomyomatosis. It has been recognized in recent years that histologically comparable lesions may arise at a wide variety of sites [12][13][14] including intraabdominal and somatic soft tissues and a variety of organs, including most notably the uterus and gastrointestinal tract. Most cases arise in adults and there is a very marked predominance in females.…”

Section: Pecomamentioning

confidence: 99%

Recently characterized soft tissue tumors that bring biologic insight

Fletcher

2014

Modern Pathology

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Previously unrecognized but clinicopathologically (and often molecularly) distinct types of soft tissue tumor continue to be characterized, allowing wider recognition, more consistent application of diagnostic criteria, more reliable prediction of tumor behavior and enhancement of existing classification schemes. Examples of such 'entities' that have become much better understood over the past decade or so include deep 'benign' fibrous histiocytoma, hemosiderotic fibrolipomatous tumor, PEComa, spindle cell liposarcoma, myoepithelial tumors of soft tissue and spindle cell/sclerosing rhabdomyosarcoma. These tumor types, as well as the insights which they have engendered, are briefly reviewed here.

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Perivascular epithelioid cell neoplasms: pathology and pathogenesis

Cited by 342 publications

References 108 publications

PEComa of the Nose: Report of a Case with Immunohistochemical and Ultrustructural Studies and a Review of the Literature

PEComa of the Nose: Report of a Case with Immunohistochemical and Ultrustructural Studies and a Review of the Literature

Perivascular Epithelioid Cell Tumors (PEComas) of the Head and Neck: Report of Three Cases and Review of the Literature

Recently characterized soft tissue tumors that bring biologic insight

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