Perivascular epithelioid tumours (PEComas) of the gynaecological tract

Conlon, Niamh; Soslow, Robert A.; Murali, Rajmohan

doi:10.1136/jclinpath-2015-202945

Cited by 78 publications

(86 citation statements)

References 89 publications

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“…The PEC cell has abundant clear to eosinophilic granular cytoplasm (Figures 9a and b) and variably stains for melanocytic markers (HMB45, MelanA, MiTF, cathepsinK, and TFE3) (Figures 9c and d) with variable expression of smooth muscle markers. [87][88][89][90][91][92] A strong association with lymphangiomyomatosis and tuberous sclerosis exists. 88,90,91 PEComa may share with epithelioid leiomyosarcoma the following features: (1) Among smooth muscle markers, desmin followed by actin and h-caldesmon are most commonly expressed especially in the spindle areas of the tumor.…”

Section: Tumor Cell Necrosismentioning

confidence: 99%

“…[87][88][89][90][91][92] A strong association with lymphangiomyomatosis and tuberous sclerosis exists. 88,90,91 PEComa may share with epithelioid leiomyosarcoma the following features: (1) Among smooth muscle markers, desmin followed by actin and h-caldesmon are most commonly expressed especially in the spindle areas of the tumor. Remember that smooth muscle tumors may focally express HMB45, thus, when applying immunohistochemistry, the diagnosis of PEComa is made when two melanocytic markers are positive (HMB45, cathepsinK, MelanA) with +/ − positivity for muscle markers (desmin, smooth muscle actin, h-caldesmon).…”

Section: Tumor Cell Necrosismentioning

confidence: 99%

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Practical issues in uterine pathology from banal to bewildering: the remarkable spectrum of smooth muscle neoplasia

Oliva

2016

Modern Pathology

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Among mesenchymal tumors of the uterus, smooth muscle neoplasms are most common. The wide morphologic spectrum, especially within the category of leiomyomas, is responsible for diagnostic problems more frequently with leiomyosarcoma (including mitotically active, apoplectic, and leiomyoma with bizarre nuclei) but also with endometrial stromal tumors. In the former scenario, clinical information, gross appearance as well as strict utilization of morphologic criteria including cytologic atypia, mitotic activity, and tumor cell necrosis are clues in establishing the correct diagnosis. It is important to keep in mind that mitotic rate thresholds vary for the different subtypes of leiomyosarcoma. Of note, p16 should be used with caution in supporting a diagnosis of leiomyosarcoma as it is often positive in leiomyomas with bizarre nuclei and leiomyomas with apoplectic change (in the latter most frequently and more intense near areas of necrosis). MED12 mutations have also a very limited role in this differential diagnosis. Endometrial stromal tumors are by far, less common than smooth muscle tumors, but can be confused with leiomyosarcomas if they are associated with an undifferentiated uterine sarcoma and the low-grade component is overlooked or they have a myxoid/fibroblastic morphology. The differential diagnosis may be confounded if the latter is associated with a high-grade endometrial stromal sarcoma. It is important to highlight that CD10 is not a reliable marker in these differentials and should be used as a part of a panel of antibodies that also includes desmin and h-caldesmon. Two other recently categorized tumors in the uterus that merit special mention are PEComa and inflammatory myofibroblastic tumor as they enter in the differential diagnosis of smooth muscle tumors. PEComa may be part of the tuberous sclerosis syndrome and may show either a predominantly epithelioid or spindle morphology or combination thereof. Rarely, it may contain melanin pigment. There is variable positivity for HMB-45, MelanA, MiTF, and CathepsinK, and some tumors have been shown to express TFE-3 especially when associated with "clear cell" morphology. Patients with adverse outcome have tumors with ≥ 2 of the following features: ≥ 5 cm, infiltration, high-grade cytologic features, mitotic rate ≥ 1/50 high-power fields, necrosis, or lymphovascular invasion. Inflammatory myofibroblastic tumor is important to recognize as it often mimics myxoid smooth muscle tumors, either benign or malignant. The presence of an associated lymphoplasmacytic infiltrate should alert to that possibility and ALK studies (immunostain or FISH) are helpful in establishing this diagnosis. These tumors can behave in a malignant manner if large, associated with abundant myxoid change, brisk mitotic rate or show tumor cell necrosis.

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Section: Tumor Cell Necrosismentioning

confidence: 99%

Section: Tumor Cell Necrosismentioning

confidence: 99%

Practical issues in uterine pathology from banal to bewildering: the remarkable spectrum of smooth muscle neoplasia

Oliva

2016

Modern Pathology

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“…20 While 5% of all uterine PEComas occur in the context of tuberous sclerosis, most tumors seem to be sporadic. 7 For published CAPUs in particular, there is only one case affecting a patient with tuberous sclerosis, to the best of our knowledge.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…To date, less than 80 cases of uterine PEComa have been reported in the literature. 7 Although the majority of these cases show a good overall prognosis and can be definitively cured by a complete surgical excision, locoregional recurrences, distant metastases, and disease-related deaths do rarely occur. In fact, one of the challenges faced by pathologists is how to predict the biological behavior of PEComas, because no single histopathologic feature is in and of itself a marker of an aggressive biology.…”

mentioning

confidence: 99%

Predicting the Behavior of Perivascular Epithelioid Cell Tumors of the Uterine Corpus

Acosta

Adley

2017

Archives of Pathology &Amp; Laboratory Medicine

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Perivascular epithelioid cell tumors (PEComas) are rare neoplasms that share phenotypic features with angiomyolipomas, clear cell sugar tumors, and lymphangioleiomyomatosis. They presumably represent the neoplastic counterpart of a yet-unidentified perivascular epithelioid cell that expresses smooth muscle and melanocytic immunomarkers. The uterus is the second most common site of origin for perivascular epithelioid cell tumors, after the retroperitoneum. Although most uterine perivascular epithelioid cell tumors are clinically benign and can be cured by a complete surgical excision, there is a subset characterized by both local and distant dissemination. Unfortunately, no single histopathologic or immunohistochemical parameter can accurately predict the clinical behavior of these tumors, which is why the 2012 World Health Organization classification of tumors of the female reproductive organs suggests the use of several criteria to predict the risk of aggressive clinical behavior. Here we review those perivascular epithelioid cell tumors of the uterine corpus with aggressive clinical behavior reported in the literature, and we discuss their most relevant clinical and histopathologic features.

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“…All PEComas are composed by epithelioid cells which expresses melanocytic and myogenic markers, such as HMB-45, desmin and actin [6,7]. It has been reported in literature both uterine and ovarian PEComas [8][9][10], however the most common location in the female genital tract is uterus [11]. Here, we report a case of a primary uterine PEComa, with a very aggressive clinical behavior.…”

Section: Introductionmentioning

confidence: 95%

Case 1 − A case of gynecological PEComa

Fontanella

Carcangiu

Lorusso

et al. 2017

CBN

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Perivascular epithelioid cell tumors (PEComas) are a heterogeneous group of rare mesenchymal neoplasms composed of epithelioid cells which express melanocytic and myogenic markers, such as HMB-45, desmin and actin. In March 2013, we visited a postmenopausal 51-year-old woman with a suspected diagnosis of uterine PEComa diagnosed by a dilation and curettage of the lining of the uterus. After the histological revision of the formalin-fixed paraffin-embedded (FFPE) material by our expert pathologist, we confirmed the diagnosis and referred the patient for complete primary surgery. On November 2013, the patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy with no macroscopically detectable residual cancer. However, in January 2014, due to voiding dysfunction and inconstant vaginal bleeding, the patient underwent magnetic resonance imaging (MRI) which documented multiple irregular lesions in the pelvis suspected as recurrent PEComa. Considering the early relapse of PEComa after optimal primary surgery, we suggested a systemic treatment with the combination of gemcitabine and docetaxel. For logistic reasons, the patients started the chemotherapy in her district hospital. After two cycles of chemotherapy the patient died due to treatment-related complications.

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Perivascular epithelioid tumours (PEComas) of the gynaecological tract

Cited by 78 publications

References 89 publications

Practical issues in uterine pathology from banal to bewildering: the remarkable spectrum of smooth muscle neoplasia

Practical issues in uterine pathology from banal to bewildering: the remarkable spectrum of smooth muscle neoplasia

Predicting the Behavior of Perivascular Epithelioid Cell Tumors of the Uterine Corpus

Case 1 − A case of gynecological PEComa

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