Perivascular Epithelioid Cell Tumor of the Orbit

Guthoff, Rainer; Guthoff, Tanja; Mueller-Hermelink, Hans Konrad; Sold-Darseff, Johanna; Geissinger, Eva

doi:10.1001/archopht.126.7.1009

Cited by 21 publications

(28 citation statements)

References 6 publications

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“…Two of the 4 cases originated from the orbit and 1 from the eyelid [3,4,5]. The remaining case, a 7-year-old boy who underwent biopsy first and iridocyclectomy 1 year later, was the only case of PEComa identified as intraocular tumor [5].…”

Section: Discussionmentioning

confidence: 99%

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Perivascular Epithelioid Cell Tumor Arising from Ciliary Body Treated by Local Resection

2014

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Aims: Perivascular epithelioid cell tumor (PEComa) is a mesenchymal neoplasm originating from perivascular myoid cells. We report a case of PEComa arising from the ciliary body. Methods: Case report. Results: A 13-year-old girl was referred to our department with a clinical diagnosis of ciliary body melanoma in her right eye. Her visual acuity was 20/600 OD. Slit-lamp examination revealed a brown tumor behind the iris. The ocular fundus could not be observed due to a cataract. Ultrasonography depicted an oval mass approximately 10 mm in diameter at the ciliary body. The tumor was successfully treated by local resection, and the patient's visual acuity improved to 20/20. Histopathological and immunohistochemical findings of the excised tumor were compatible with the diagnosis of PEComa of the ciliary body. No local recurrence of the tumor was observed for over 4 years after surgery. Conclusion: A very rare case of PEComa of the ciliary body was successfully treated by local resection, with favorable visual outcome and no recurrence for several years. PEComa can be differentiated from other ciliary body tumors by immunohistochemical study. © 2014 S. Karger AG, Basel

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Section: Discussionmentioning

confidence: 99%

“…PEComa can arise in unusual sites including the head and neck, but most of these cases were reported as small case series. Only 4 cases of PEComa have been reported in the ophthalmic literature [3,4,5]. Furusato et al [5] described a case of intraocular PEComa, but did not provide sufficient clinical data and outcome after treatment.…”

Section: Introductionmentioning

confidence: 99%

Perivascular Epithelioid Cell Tumor Arising from Ciliary Body Treated by Local Resection

2014

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“…Other than a mass effect, no other specific signs or symptoms were noted. PEComas are rare in the head and neck [7][8][9][10][11][12][13][14][15][16][17], and those arising in the nasal cavity and larynx are even more unusual [7,12]. The common morphologic features observed in all three tumors included: large polygonal to epithelioid cells with clear or eosinophilic granular cytoplasm, centrally located nuclei with vesicular chromatin, and occasional nucleoli; a predominantly nested growth pattern associated with a delicate vascular stroma, similar to the vessels seen in clear cell renal cell carcinomas and paragangliomas; and a zone of subepithelial sparing.…”

Section: Discussionmentioning

confidence: 99%

“…Based on our review of the English literature, we identified 13 cases [7][8][9][10][11][12][13][14][15][16][17] of primary head and neck PEComas (excluding examples described as AML). Including our three cases, this number now consists of 16 tumors (Table 1).…”

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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“…Perivascular epithelioid cell tumors (PEComas) are uncommon mesenchymal neoplasms that are believed to have originated from perivascular myoid cells [1, 2]. The term “PEComa” was first used by Bonetti et al [3] to describe a cell type that was immunoreactive to melanocytic markers and had an epithelioid appearance, clear-acidophilic cytoplasm, and perivascular distribution.…”

Section: Introductionmentioning

confidence: 99%

Giant Perivascular Epithelioid Cell Tumor of the Orbit: A Clinicopathological Analysis and Review of the Literature

et al. 2018

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Aim: To describe and review the clinical, radiological, and histopathological characteristics of an orbital perivascular epithelioid cell tumor (PEComa). Methods: A systematic review of clinical records, radiological investigations, microscopic features, and immunohistochemical characteristics was done. Results: A 9-year-old female child presented with a year-long history of a large orbital mass associated with painless, progressive proptosis of the right eye. Radiologically, a well-defined orbital mass was seen with no intracranial extension. Excision was performed and histopathological examination showed uniform epithelioid cells in nests separated by thin fibrovascular septae. The tumor cells stained positively for Human Melanoma Black-45, but negatively for desmin, S-100, smooth muscle actin, MyoD1, microphthalmia-associated transcription factor, vimentin, CD10, CD31, and CD34 with a low proliferation index of 5–7%. Based on the tumor’s morphological and immunohistochemical characteristics, a diagnosis of giant orbital PEComa was made. No recurrence was seen at the last follow-up. Conclusions: PEComas are uncommon mesenchymal neoplasms that have typical histological features, with an immunohistochemical profile of negativity for epithelial markers and positivity for melanocytic markers. For benign PEComas, complete excision is advised. However, since PEComas elsewhere in the body have been known to be malignant, a close follow-up of such cases is recommended.

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Perivascular Epithelioid Cell Tumor of the Orbit

Cited by 21 publications

References 6 publications

Perivascular Epithelioid Cell Tumor Arising from Ciliary Body Treated by Local Resection

Perivascular Epithelioid Cell Tumor Arising from Ciliary Body Treated by Local Resection

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

Giant Perivascular Epithelioid Cell Tumor of the Orbit: A Clinicopathological Analysis and Review of the Literature

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