Perirenal perivascular epithelioid cell tumor (PEComa) coexisting with other malignancies: a case report

Danilewicz, Marian; Strzelczyk, Janusz; Wągrowska-Danilewicz, Małgorzata

doi:10.5114/pjp.2017.67623

Cited by 5 publications

(5 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Usually, PEC surrounds the blood vessels, arranging radially around the lumen, forming bunch- and web-shaped structures, while in a minority of cases, it has a focal association with the blood vessel walls. In all cases, PEC replaces the normal smooth muscle and collagen in the muscular wall of the vessel[17,38,41]. A sclerosing variant of PEComa with predominant sclerotic and hyalinized stroma has also been reported[17,25,27,28,40].…”

Section: Discussionmentioning

confidence: 99%

Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature

Touloumis¹,

Giannakou²,

Sioros³

et al. 2019

WJCC

View full text Add to dashboard Cite

BACKGROUNDThe perivascular epithelioid cell tumour (PEComa) family of tumours mainly includes renal and hepatic angiomyolipomas, pulmonary lymphangioleiomyomatosis and clear cell “sugar” tumour of the lung. Several uncommon tumours with similar morphological and immunophenotypical characteristics arising at a variety of sites (abdominal cavity, digestive tract, retroperitoneum, skin, soft tissue and bones) are also included in the PEComa family and are referred to as PEComas not otherwise specified.CASE SUMMARYWe present a 37-year-old female patient who underwent resection of an 8.5 cm × 8 cm × 4 cm retroperitoneal tumour, which eventually was diagnosed as PEComa of uncertain biological behaviour. Three years after the operation, the patient remains without any evidence of recurrence. A search was performed in the Medline and EMBASE databases for articles published between 1996 and 2018, and we identified 31 articles related to retroperitoneal and perinephric PEComas. We focused on sex, age, maximum dimension, histological and immunohistochemical characteristics of the tumour, follow-up and long-term outcome. Thirty-four retroperitoneal (including the present one) and ten perinephric PEComas were identified, carrying a malignant potential rate of 44% and 60%, respectively. Nearly half of the potentially malignant PEComas presented with or developed metastases during the course of the disease.CONCLUSIONRetroperitoneal PEComas are not as indolent as they are supposed to be. Radical surgical resection constitutes the treatment of choice for localized disease, while mammalian target of the rapamycin (mTOR) inhibitors constitute the most promising therapy for disseminated disease. The role of mTOR inhibitors as adjuvant or neoadjuvant therapies needs to be evaluated in the future.

show abstract

Section: Discussionmentioning

confidence: 99%

Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature

Touloumis¹,

Giannakou²,

Sioros³

et al. 2019

WJCC

View full text Add to dashboard Cite

show abstract

“…Immunohistochemistry plays a crucial role in avoiding misdiagnosis of PEComa: they stain for HMB-45, Melan-A, SMA, vimentin; they may be positive for desmin, CD-31, CD-34. They are negative for CgA (chromogranin A), Syn (synaptophysin), CK (creatine kinase), CD-117, CD-10, AFP (alpha fetoprotein) and EMA (epithelial membrane antigen) [18,19]. Contrast ultrasonography, contrastenhanced computed tomography and magnetic resonance imaging have been proposed for the imaging assessment of PEComa.…”

Section: Discussionmentioning

confidence: 99%

Large Retroperitoneal Perivascular Epithelioid Cell Neoplasm (PEComa): A Case Report and a Brief Review

Lucandri¹,

Lirici²,

Fassari³

et al. 2021

SCR

View full text Add to dashboard Cite

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.

show abstract

“…Perivascular epithelioid cell tumors (PEComas) are mesenchymal neoplasms composed of histologically and immunohistochemically distinctive perivascular epithelioid cells as described by the World Health Organization (WHO) [1]. It is a family of tumors that occurs most commonly in females and can be found in many locations.…”

Section: Introductionmentioning

confidence: 99%

“…Only 160 cases of AML PEComa have been described [2]. Only four cases of PEComas with perirenal manifestation have been reported [1].…”

Section: Introductionmentioning

confidence: 99%