Perivascular Epithelioid Cell Tumor of the Buttock Region

Rehman, Rafey; Aoun, Mariam; Levitin, R.; Quinn, Thomas J.; Kabolizadeh, Peyman

doi:10.7759/cureus.15252

Cited by 3 publications

(4 citation statements)

References 10 publications

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“…Meanwhile, the muscle markers SMA, MSA and desmin were positive only in 44%, 25%, and 22% cases, respectively. Muscle markers were less abundant than melanocytic markers and tended to be less abundant than those in PEComas of other primary sites (Table 4) (Charli-Joseph et al 2014;Conlon et al 2015;Chen et al 2016;Zhang et al 2017Zhang et al , 2021Bao et al 2019;Zhong et al 2020;Rehman et al 2021). In general, epithelioid PEComas tend to show higher expression of melanocytic markers than that of myogenic markers, and spindle cell PEComas show an opposite expression profile (Conlon et al 2015).…”

Section: Discussionmentioning

confidence: 97%

“…Common sites of PEComa occurrence are the uterus, kidney, liver, lung, abdominopelvic soft tissues, gastrointestinal organs, retroperitoneum, and skin (Folpe et al 2005;Ligel et al 2008;Hornick and Fletcher 2008;Doyle et al 2013). PEComas of the bone and soft tissues are very rare, and the literature is restricted to case reports and small case series (Mahera et al 1997;Kuroda et al 2000;Diment and Colecchia 2003;Fukunaga 2004;Harris et al 2004;Folpe et al 2005;Mai and Belanger 2006;Pikoulis et al 2007;Osei et al 2007;Blechet et al 2007;Boussouga et al 2008;Argani et al 2010;Yamashita and Fletcher 2010;Varshney et al 2011;Alnajar et al 2018;Harvey et al 2019;Zhong et al 2020;Rehman et al 2021). Consequently, the clinical course, clinicopathological characteristics, and treatment strategies for PEComas of the bone and soft tissues are largely unknown.…”

Section: Introductionmentioning

confidence: 99%

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Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissue: a Japanese Musculoskeletal Oncology Group (JMOG) multi-institutional study

Izubuchi

Hamada

Tanzawa

et al. 2023

J Cancer Res Clin Oncol

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Purpose Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissues are rare mesenchymal neoplasms, some of which are malignant. However, their clinical and pathological characteristics remain unclear. This study was performed to investigate the clinical and pathological characteristics of PEComas in bone and soft tissues by leveraging information from the Japanese Musculoskeletal Oncology Group. Methods Nine patients, including four male and five female patients with a median age of 50 years, were retrospectively reviewed. PEComas of the visceral organs, including the uterus and retroperitoneum, were excluded. Results Eight tumors arose in the soft tissue and one in the bone, with a mean size of 8.8 cm. Four patients showed local recurrence or distant metastasis. The 1-year survival rate was 78%. Pathologically, eight tumors were classified as malignant and one as having uncertain malignancy potential. Half of the tumors showed high MIB-1 index values of > 30%. Immunohistochemically, the melanocyte marker HMB45 was expressed in 89% of the cases, and muscle-specific markers were expressed only in 30–50% of the cases. Transcription factor binding to IGHM enhancer 3 (TFE3) expression was positive in 100% of the patients. Tumors with high expression of TFE3 were classified as PEComas with malignant potential according to Folpe’s classification. Conclusions Bone and soft tissue PEComas may have a higher malignancy potential than other visceral PEComas and are more likely to develop as TFE3-rearranged PEComas.

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Section: Discussionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissue: a Japanese Musculoskeletal Oncology Group (JMOG) multi-institutional study

Izubuchi

Hamada

Tanzawa

et al. 2023

J Cancer Res Clin Oncol

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“…Since metastasis may occur up to 10 years after surgical resection, regular follow-up is necessary. [5–7]…”

Section: Discussionmentioning

confidence: 99%

“…Since metastasis may occur up to 10 years after surgical resection, regular follow-up is necessary. [5][6][7] As PEComa is a rare disease, there is no established standardized treatment, but the main treatment is currently radical resection. Radical resection is recommended for metastatic tumors, but a poor prognosis is predicted.…”

Section: Discussionmentioning

confidence: 99%

Treatment of malignant perivascular epithelioid cell tumor (PEComa) on the knee with an anterolateral thigh free flap: A case report

et al. 2023

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Rationale: The World Health Organization defines a perivascular epithelioid cell tumor (PEComa) as a mesenchymal neoplasia composed of perivascular epithelioid cells with characteristic morphological and immunohistochemical features. Although PEComas have the potential to behave in a malignant fashion, malignant PEComas are extremely rare. Patient concerns: An 83-year-old man visited our clinic presented with palpable, painless, and movable mass in the right knee area. Diagnoses: Malignant PEComa was diagnosed by incisional biopsy. No metastases was confirmed by radiologic imaging including PET/CT, magnetic resonance imaging, high resolution computed tomography. Interventions: We performed wide excision of the mass and used an anterolateral thigh free flap to reconstruct the defect on the right knee. Outcomes: The permanent histopathology showed malignant PEComa was totally resected. The flap which was performed to cover the defect was survived and the patient discharge without any complications. Lessons: PEComa can metastasize to various anatomical regions. Although there is no established standardized treatment, radical resection is still considered the cornerstone of treatment. Rapid and appropriate defect coverage is important to improve the patient’s prognosis.

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Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissue: A Japanese Musculoskeletal Oncology Group (JMOG) multi-institutional study

Izubuchi

Hamada

Tanzawa

et al. 2023

Preprint

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Purpose: Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissues are rare mesenchymal neoplasms, some of which are malignant. However, their clinical and pathological characteristics remain unclear. This study was performed to investigate the clinical and pathological characteristics of PEComas in bone and soft tissues by leveraging information from the Japanese Musculoskeletal Oncology Group. Methods: Ten patients, including four male and six female patients with a median age of 49 years, were retrospectively reviewed. PEComas of the visceral organs, including the uterus and retroperitoneum, were excluded. Results: Nine tumors arose in the soft tissue and one in the bone, with a mean size of 9.3 cm. Four patients showed local recurrence or distant metastasis. The 1-year survival rate was 77%. Pathologically, eight tumors were classified as malignant and two as having uncertain malignancy potential. Half of the tumors showed high MIB-1 index values of >30%. Immunohistochemically, the melanocyte marker HMB45 was expressed in 90% cases, and muscle-specific markers were expressed only in 30–50% cases. Transcription factor binding to IGHM enhancer 3 (TFE3) expression was positive in 89% of the patients. Tumors with high expression of TFE3 were classified as PEComas with malignant potential according to Folpe’s classification. Conclusions: Bone and soft tissue PEComas may have a higher malignancy potential than other visceral PEComas and are more likely to develop as TFE3-rearranged PEComas.

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Perivascular Epithelioid Cell Tumor of the Buttock Region

Cited by 3 publications

References 10 publications

Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissue: a Japanese Musculoskeletal Oncology Group (JMOG) multi-institutional study

Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissue: a Japanese Musculoskeletal Oncology Group (JMOG) multi-institutional study

Treatment of malignant perivascular epithelioid cell tumor (PEComa) on the knee with an anterolateral thigh free flap: A case report

Perivascular epithelioid cell tumors (PEComas) of the bone and soft tissue: A Japanese Musculoskeletal Oncology Group (JMOG) multi-institutional study

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