Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): an immunohistochemical study and review of the literature

Greene, Laura; Mount, Sharon L.; Schned, Alan R.; Cooper, Kumarasen

doi:10.1016/s0090-8258(03)00325-1

Cited by 55 publications

(55 citation statements)

References 10 publications

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“…In agreement with others [3,16] the tumor cells in our case were S-100 negative. Contrary to us and to most reports, some authors reported patchy S-100 staining of PEComa cells [6,11]. In the present study the immunoexpression of CD-10 was negative in the tumor cells, although in some cases of PEComas positivity for CD-10 was noted.…”

Section: Discussioncontrasting

confidence: 99%

“…In the present case immunohistochemical staining confirmed the melanocytic and myoid differentiation of the tumor cells. Similarly as in the study of Gunia et al [5], epithelioid cells were focally immunoreactive for HMB-45, whereas Greene et al reported this immunostaining as diffuse [6]. On the other hand, similarly as in the last cited study diffuse positive staining with smooth muscle actin reflected the myoid differentiation.…”

Section: Discussionsupporting

confidence: 57%

“…To date, the extremely rare perivascular epithelioid cell tumor has been described in various anatomic locations: in lungs [1], pancreas [2], uterus [6,7], bone [8], retroperitoneum [9], ovary [10], liver [11], cutis [12], transverse colon [4] and in intraorbital location [3]. As was mentioned above, close perirenal location concerns only three cases of PEComas [5,13,14].…”

Section: Discussionmentioning

confidence: 99%

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Perirenal perivascular epithelioid cell tumor (PEComa) coexisting with other malignancies: a case report

Danilewicz¹,

Strzelczyk²,

Wągrowska-Danilewicz³

2017

pjp

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Perivascular epithelioid cell tumor (PEComa) is a very rare lesion and is described by the World Health Organization (WHO) as a mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. In this report we describe PEComa with perirenal manifestation, which is exceedingly rare and to our best knowledge up to now worldwide only three cases have been described. Despite the reports that most PEComas are benign, this tumor met criteria for malignancy and coexisted with mucinous gallbladder cancer and nonresectable pancreatic head tumor. We concluded that despite the rarity of perirenal PEComas, in cases with an unusual epithelioid histological pattern the diagnosis of PEComa should also be taken into consideration on the basis of the immunohistochemical study.

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Section: Discussioncontrasting

confidence: 99%

Section: Discussionsupporting

confidence: 57%

Section: Discussionmentioning

confidence: 99%

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Perirenal perivascular epithelioid cell tumor (PEComa) coexisting with other malignancies: a case report

Danilewicz¹,

Strzelczyk²,

Wągrowska-Danilewicz³

2017

pjp

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“…2 These neoplasms have a broad spectrum of morphology and behavior and may involve the kidney, lung, liver, pancreas, common bile duct, jejunum, rectum, falciform ligament of the liver, heart, urinary bladder, prostate, breast, bone, soft tissues [3][4][5][6][7][8][9][10] and female genital tract organs, 11,8 among which the uterus is the most frequently involved. 3,4,[12][13][14][15][16][17][18][19] In this site, most of these tumors are benign or of uncertain malignant potential, 4,13,16,19 although rare malignant cases have been reported. 3,12,14,15,17,18 We report the clinicopathologic features of two perivascular epithelioid cell tumors ('PEComa') of the uterus with aggressive features, including one malignant and one with uncertain malignant potential.…”

mentioning

confidence: 99%

“…3,4,[12][13][14][15][16][17][18][19] In this site, most of these tumors are benign or of uncertain malignant potential, 4,13,16,19 although rare malignant cases have been reported. 3,12,14,15,17,18 We report the clinicopathologic features of two perivascular epithelioid cell tumors ('PEComa') of the uterus with aggressive features, including one malignant and one with uncertain malignant potential.…”

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confidence: 99%

Perivascular epithelioid cell (PEC) tumors of the uterus: a clinicopathologic study of two cases with aggressive features

et al. 2005

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We report the clinicopathologic, immunohistochemical and ultrastructural features of two unusual tumors of the uterus composed of spindle and epithelioid cells strongly positive for HMB45. The two patients of 56 and 48 years of age had, respectively, hemoperitoneum and abnormal uterine bleeding. Morphologically, both tumors showed atypia and extensive necrosis. The neoplastic cells express immunohistochemically both melanogenesis (HMB45) and smooth muscle markers (actin). Ultrastructural analysis showed the presence of intracytoplasmic membrane-bound granules. We viewed these neoplasms as perivascular epithelioid cell (PEC) tumors with aggressive features. Follow-up has shown the death of one patient whereas the other is alive without disease 36 months after the surgery. The two patients were evaluated for signs of tuberous sclerosis complex, and findings were negative.

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Perivascular epithelioid cell tumors (PEComa) of the gynecologic tract

Bennett

Oliva

2020

Genes Chromosomes & Cancer

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PEComas of the female genital tract are rare mesenchymal neoplasms that are most common in the uterus, but also may occur in other gynecologic locations. As they morphologically and immunohistochemically resemble smooth muscle tumors, distinction between the two entities is often challenging, and may be aided by molecular analysis. Thus far, two distinct molecular groups‐classic PEComas with TSC mutations and TFE3‐translocation associated PEComas with TFE3 fusions have been described. Recognition of the first group is imperative as these patients may benefit from targeted therapy with mTOR inhibitors, if malignant. This review will focus on recognition of the morphologic and immunophenotypic features of PEComas, as well as the role of molecular testing in their diagnosis and treatment, analysis of the different algorithms to predict behavior, and differential diagnosis.

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Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): an immunohistochemical study and review of the literature

Cited by 55 publications

References 10 publications

Perirenal perivascular epithelioid cell tumor (PEComa) coexisting with other malignancies: a case report

Perirenal perivascular epithelioid cell tumor (PEComa) coexisting with other malignancies: a case report

Perivascular epithelioid cell (PEC) tumors of the uterus: a clinicopathologic study of two cases with aggressive features

Perivascular epithelioid cell tumors (PEComa) of the gynecologic tract

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