Perivascular epithelioid cell tumor
Angiomyolipoma – kidney, histopathology
PEComas are mesenchymal tumors made up of histologically and immunohistochemically distinct perivascular epithelioid cells (PECs). Angiomyolipomas (AMLs), lymphangiomyomatosis, and lung clear-cell tumors are all related mesenchymal neoplasms. Similar lesions may appear in various visceral and soft tissue organs. The PEC (which has no known normal tissue counterpart) is the cell of origin, and the uterus is the most common place of involvement. The majority of PEComas are benign and do not recur following radical surgery to remove them locally. A well-described malignant or metastatic mutation in some cases has a uniformly fatal outcome.
Abnormal mTOR signaling has been identified in molecular pathophysiology studies, which supports the use of mTOR inhibitors (mTORIs) as a treatment choice. In metastatic renal-cell carcinoma, current recommendations suggest tyrosine-kinase inhibitors (TKIs) after cytoreductive nephrectomy. A case of metastatic renal PEComa was treated with an adjuvant TKI (sunitinb) and mTORIs in this study. Sunitinib was chosen as the first-line therapy because it inhibits multiple growth factors, including VEGFR1, VEGFR2, and VEGFR3, as well as PDGFRs, KIT, and FLT3R, and we had previous experience with it at our institution.
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Sarcomas: enfoque del patólogo
PEComa (Perivascular Epithelioid Cell Tumors) is an extremely rare mesenchymal neoplasm, especially the malignant form that originates in the lungs. Just eight cases of malignant or malignant potential pulmonary PEComa have been reported so far. Firm diagnostic criteria for malignant pulmonary PEComa must be identified as soon as possible. Presentation of an event In a 54-year-old man, we report a difficult case of malignant pulmonary PEComa combined with primary adenocarcinoma. There was high Melan-A and weak transcription factor E3 (TFE3) protein expression in the PEComa-like tumor, but no TFE3 gene rearrangement. A poorly differentiated primary lung adenocarcinoma was described as the carcinoma-like nodule. Conclusions and discussion Our case study explored the problem of diagnosing benign or malignant criteria for this unusual tumor, since it was the first case of malignant pulmonary PEComa synchronous with a primary adenocarcinoma.
affiliations of the writers
Contributions of Writers
The study was planned by YCH and JKZ. The experiments were carried out by JKZ and LZ, and the pathologic and imaging data were interpreted by HHT. The molecular analysis was completed by RYZ and HHT. The technical assistance was guaranteed by WJD and KKY. JZ contributed to the revision of the article structure as well as the discussion of the diagnosis by offering conceptual advice. The manuscript was written by JKZ and LZ. YCH was in charge of the whole project. The final manuscript was read and accepted by all writers and contributors. Author-in-Residence In answer to:
Perivascular epithelioid cell tumour (medical condition
The case of a 46-year-old woman with primary malignant perivascular epithelioid cell neoplasm (PEComa) of the femur was examined in this study. Following trauma, the patient presented with a 5-month history of right distal thigh pain. A mixed lytic and sclerotic lesion with subtle areas of cortical destruction and soft tissue extension was discovered on radiographs of the right distal femur, suggesting an aggressive tumor. A core biopsy showed an epithelioid tumor with granular cell characteristics, but it was difficult to make a definitive diagnosis. The patient had a resection of the distal femur and reconstruction with a distal femoral megaprosthesis and hinged knee replacement due to the violent features on radiologic examination. The postresection pathology revealed epithelioid, granular cell, and spindled cell morphologies, as well as biphasic immunoreactivity for melanocytic and smooth muscle markers, leading to a final diagnosis of primary bone PEComa. This was a malignant PEComa because of the large tumor size (>5 cm), rapid mitotic rate, infiltrative growth pattern, high nuclear grade and cellularity, and the presence of necrosis. The case was examined in detail, including radiographic (radiographs, magnetic resonance imaging, and positron emission tomography scans) and histologic findings, as well as a review of the literature.
Mesenchymal kidney tumors – dr. matoso (hopkins
Assoc. Prof. Koray Hekimoglu, Department of Radiology, Baskent University School of Medicine, Fevzi Cakmak Road, 10. Street Nu: 45 Bahcelievler, Ankara 06490, Turkey. [email protected] [email protected] [email protected] kor
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PEComa, or primary perivascular epithelioid cell tumor of the liver, is a very rare mesenchymal tumor. The most common anatomic location of these tumors is the gastrointestinal tract with perivascular distribution. So far, only a few cases of hepatic PEComa have been reported. Malignant PEComas are aggressive and have a poor prognosis, so early detection is critical. We present a case of a 79-year-old woman with a liver mass that was oddly placed. PEComa was diagnosed histopathologically after a partial curative hepatectomy. During the 6-month follow-up, there was no proof of recurrence.