Large Retroperitoneal Perivascular Epithelioid Cell Neoplasm (PEComa): A Case Report and a Brief Review
| Autor: | Giorgio Lucandri, Marco Maria Lirici, Alessia Fassari, Vito Pende, Domenico Campagna, Marco Giordano, Emanuele Santoro |
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| Rok vydání: | 2021 |
| Předmět: |
perivascular epithelioid cell
Abdominal pain medicine.medical_specialty Angiomyolipoma soft tissue tumor business.industry Urinary system medicine.medical_treatment medicine.disease Perivascular Epithelioid Cell Nephrectomy angiomyolipoma PEComa soft tissue tumor perivascular epithelioid cell angiomyolipoma clear cell sugar tumor lymphangiomyomatosis lymphangiomyomatosis medicine.anatomical_structure Renal capsule Medicine Abdomen PEComa Radiology clear cell sugar tumor medicine.symptom business Immunostaining |
| Zdroj: | Surgical Case Reports. :1-6 |
| ISSN: | 2613-5965 |
| DOI: | 10.31487/j.scr.2021.07.10 |
| Popis: | Objective: To describe a case of retroperitoneal perivascular epithelioid cell tumor (PEComa) and to discuss the main features of this rare pathology. Introduction: PEComas represent a rare cluster of neoplasms with uncertain origin; their precursor cells are spindle-shaped and characterized by a myomelanocytic phenotype, so only immunohistochemical staining makes a definitive diagnosis possible. To date, less than three hundred cases are reported in Literature and retroperitoneal site accounts for 7-8% of overall locations. Case Report: Middle-aged female has visited for abdominal pain and urinary complaints; physical findings and imaging demonstrated a huge inhomogeneous mass occupying right abdomen and arising from renal capsule. After multidisciplinary evaluation, patient has been addressed to open surgery and an en-bloc resection of the mass, with right nephrectomy and adrenalectomy. Immunohistochemical staining made a diagnosis of PEComa possible. After an uneventful postoperative stay, the patient entered a follow up protocol, without signs of local recurrence and distant metastases. Conclusion: Retroperitoneal PEComa often presents as a bulky mass with renal and adrenal involvement. Surgical resection should be aimed to obtain a complete removal with negative margins; this makes compartment surgery and en-bloc resection mandatory. Immunostaining is the key methods for a correct diagnosis. |
| Databáze: | OpenAIRE |
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