Malignant Epithelioid Angiomyolipoma of the Kidney (Malignant Perivascular Epithelioid Cell Neoplasm)

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منابع مشابه

“Malignant” Perivascular Epithelioid Cell Neoplasm: Risk Stratification and Treatment Strategies

Purpose. Perivascular epithelioid cell tumors (PEComas) are a rare collection of tumors characterized by a myomelanocytic phenotype, and PEComas occurring in "nonclassic" anatomic distributions are known as perivascular epithelioid cell tumor not otherwise specified (PEComa-NOS). This review aims to compile and analyze cases of PEComa-NOS in an effort to better define their natural history. Des...

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Wünderlich syndrome from a malignant epithelioid angiomyolipoma.

Received August 2008 Accepted December 2008 INTRODUCTION Angiomyolipoma of the kidney has classically been considered as a tumor of the connective tissue composed of fat, vascular tissue, and smooth muscle. In most cases, it is a tumor with benign behavior that may appear sporadically or associated with tuberous sclerosis syndrome. Macroscopically, these tumors are greyish yellow in color, and ...

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Malignant perivascular epithelioid cell tumor of the uterus

Perivascular epithelioid cell tumors (PEComas) are a rare collection of tumors arising in a wide array of anatomic locations and characterized by a myomelanocytic phenotype. PEComas which occur in non-classic anatomic distributions are known as perivascular epithelioid cell tumor-not otherwise specified (PEComa-NOS), and one of the most common primary sites for PEComa-NOS is the uterus. The ris...

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Malignant perivascular epithelioid cell tumor of the retroperitoneum.

Perivascular epithelioid cell tumors (PEComas) are a rare type of mesenchymal neoplasms characterized by a proliferation of perivascular cells with an epithelioid phenotype and expression of myo-melanocytic markers. The majority of PEComas seem to be benign and usually their prognosis is good. Malignant cases are extremely rare, exhibiting a malignant course with local recurrences and distant m...

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Malignant Perivascular Epithelioid Cell Tumor of the Esophagus

Malignant perivascular epithelioid cell tumor (PEComa) is a rare tumor composed of hybrid tumor cells characterized by immunoreactivity for both melanocytic and smooth muscle markers. This paper describes the uncommon esophageal location of an 8 cm PEComa in a 75-year-old Caucasian man who was presented with ingravescent dysphagia. Although PEComas arising within the gastrointestinal tract are ...

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ژورنال

عنوان ژورنال: Mayo Clinic Proceedings

سال: 2020

ISSN: 0025-6196

DOI: 10.1016/j.mayocp.2019.11.015