Abstract
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.
Highlights
Perivascular Epithelioid Cell tumors represent rare mesenchymal tumors; the first PEComa was described by Apitz in 1943; since only case reports and few small series are reported in the literature [1]
Whenever arising into retroperitoneal space, PEComa often exhibits a slow but steady growth; in this site PEComa must be distinguished from clear cell sarcoma, smooth muscle neoplasm, gastrointestinal stromal tumor (GIST)
Adrenal gland could not be identified with certainty. These findings suggested the presence of a voluminous renal angiomyolipoma or, as alternatives, an adrenal mass or a retroperitoneal liposarcoma
Summary
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. 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. 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. Conclusion: Retroperitoneal PEComa often presents as a bulky mass with renal and adrenal involvement.
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