Radiological features of perivascular epithelioid cell tumours (PEComas) in a paediatric patient – Case report

Abstract

Introduction. Perivascular epithelioid cell tumour (PEComa) is a rare family of mesenchymal tumours composed of epithelioid cells. Due to its very rare occurrence, little information is available on the imaging characteristics of this type of lesion. Case Report. A 16-year-old patient was admitted for diagnosis of an incidentally found lesion of unclear origin on MRI of the lumbar spine. Targeted MRI revealed a pathological solid-cystic mass within the pelis, in the left rectovesical pouch, infiltrating the bladder wall. A biopsy of the tumour was performed. On histopathological examination, a PEComa tumour was diagnosed. Control examinations were performed. The tumour was treated with embolization, surgical resection and Sirolimus therapy. Conclusions. The tumour showed the characteristic features of PEC-oma on imaging studies reported in the literature. Radiological diagnosis is not fully specific, therefore histopathological examination is necessary for a definitive diagnosis.

A Group Contribution Analogy as Selection Criteria for Nutrients Fermentation Media in the Production of Tacrolimus, Sirolimus and Ascomycin

“…Sirolimus (Fig. 2), empirical formula C 51 H 79 NO 13, also known as rapamycin with molar mass 914.17 g/mol and sold under the brand name Rapamune is a macrolide compound that is used to coat coronary stents, prevent organ transplant rejection, treat a rare lung disease called lymphangioleiomyomatosis [9], and treat perivascular epithelioid cell tumor (PEComa) [10]. It has immunosuppressant functions in humans and is especially useful in preventing the rejection of kidney transplants [11].Sirolimus was initially developed as an antifungal agent [12].…”