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  • ISSN: 2373-9819
    JSM Clin Case Rep 4(2): 1101.
    Submitted: 16 May 2016; Accepted: 07 June 2016; Published: 09 June 2016
    Case Report
    Perivascular Epithelioid Cell Tumor of the Kidney – Report of an Incidentaloma and its Treatment
    Daniel Paramythiotis1*, Konstantinia Kofina1, Christos Poulios2, Valentini Tzioufa-Asimakopoulou2, Vasileios Papadopoulos1 and Antonios Michalopoulos1
    1Propedeutic Surgical Department, Aristotle’s University of Thessaloniki, Greece
    2Department of Pathology, Aristotle’s University of Thessaloniki, Greece
    *Corresponding author: Daniel Paramythiotis, 1st Propedeutic Surgical Department, Aristotle’s University of Thessaloniki, A.H.E.P.A. University Hospital, Thessaloniki, L. Tzavella 2D, Pilea, 55535, Thessaloniki, Greece, Tel: 0030-6944-906619; Email: danosprx1@hotmail.com
    Abstract
    Perivascular epithelioid cell tumors (PEComas) are a relatively rare group of mesenchymal tumors that may occur in uncommon sites, including the kidney. We present the case of such a renal tumor that was found incidentally after a radiologic examination in a 53-year-old female. The patient was treated surgically and pathologic examination was diagnostic for the disease.
    Keywords: PEComas; Mesenchymal tumors; Exploratory laparotomy
    Introduction
    World Health Organization defines the Epithelioid Cell tumors (PEComas) as mesenchymal tumors composed of histologically, immunohistochemically, ultrastructurally, and genetically epithelioid distinctive cells. These cells coexpress mtuscle and melanocytic markers. This family of tumors includes angiomyolipomas, pulmonary lymphangioleiomyomatosis [1], clear cell tumors of the lung, abdominopelvic sarcomas as well as other tumors with the same characteristics, located in various sites [2]. PEComas are extremely rare tumors that can occur in any part of the human body and their natural history and prognostic features are yet undefined [3]. We report a case of an exophytic renal mass in a middle-aged woman diagnosed incidentally during imaging examinations and treated successfully through mass excision.
    Case Presentation
    The patient, a 53-year-old woman, was admitted in our surgical department after being submitted to an abdominal ultrasound examination for the evaluation of epigastric pain. This examination revealed incidentally the presence of an extrarenal mass of the right kidney, of diameter of 10 cm. The patient did not present any signs of renal dysfunction, from her medical history, or the clinical and laboratory examination, and did not complain about any other symptoms other than the aforementioned pain.
    Further investigation included an upper - lower abdomen and retroperitoneal CT scan (Figure 1), as well as an abdominal MRI (Figures 2,3). Computerized tomography – guided biopsy was performed and the pathologic examination revealed the presence of a mass containing spindle cells and showing characteristics of a PEComa.
    Surgery was considered as an optimal treatment, so an exploratory laparotomy was performed (Figure 4), and the excision of an exophytic mass of a diameter of 12cm was realized (Figure 5). Right nephrectomy wasn’t considered necessary. The patient’s postoperative condition was stable and she was discharged on the fifth postoperative day. The second pathologic examination also revealed an exophytic benign renal PEComa (Figure 6). No further treatment was considered necessary and the patient didn’t show evidence of recurrence during the followup control.
    Figure 1 CT imaging of the right pararenal mass.

    Figure 1 CT imaging of the right pararenal mass.

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    Figure 2 Abdominal MRI of the exophytic mass - Saggital section.

    Figure 2 Abdominal MRI of the exophytic mass - Saggital section.

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    Figure 3 Abdominal MRI of the exophytic mass - Coronal section.

    Figure 3 Abdominal MRI of the exophytic mass - Coronal section.

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    Figure 4 Exploratory laparotomy - Intraoperative presence of the tumor.

    Figure 4 Exploratory laparotomy - Intraoperative presence of the tumor.

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    Figure 5 Excised renal mass of a greatest diameter of 12cm.

    Figure 5 Excised renal mass of a greatest diameter of 12cm.

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    Figure 6 H&E stain X 100. Multiple spindle-shaped cells arranged in small groups or bundles. There are several vessels in myxoid stroma.

    Figure 6 H&E stain X 100. Multiple spindle-shaped cells arranged in small groups or bundles. There are several vessels in myxoid stroma.

    ×
    Discussion
    In the female genitourinary tract, PEComas have been described in the kidney, bladder, and urethra. Angiomyolipomas can be controlled through ultrasound and CT-scan for any change in their preoperative size and usually are hyper-attenuated on precontrast CT, with or without fat component [4,5].
    The expression of cathepsin K, TFE3 gene fusions and the genetic alterations of tuberous sclerosis complex have been associated with the disease [6]. The presence of heavily pigmented PEComas has also been described and must be differentiated from other pigmented tumors, such as malignant melanomas, pigmented clear cell renal cell carcinoma and composite paraganglioma.
    Although most PEComas are considered benign, some can be malignant, and classification criteria have been suggested for both renal and extrarenal lesions. Characteristics as carcinoma-like growth factors, extrarenal extension and renal vein involvement are associated with progression of the disease, so a long-term follow-up study is important [7]. In our case, oncologic evaluation didn’t consider necessary any other postoperative treatment and the patient remained healthy during follow-up control.
    The differential diagnosis is difficult and a thorough preoperative control should be performed. The pathologic examination sets the final diagnosis of this rather benign, but rare condition, in order to estimate the prognosis or the necessity of a probable additional treatment.
    References
    Cite this article: Paramythiotis D, Kofina K, Poulios C, Tzioufa-Asimakopoulou V, Papadopoulos V, et al. (2016) Perivascular Epithelioid Cell Tumor of the Kidney – Report of an Incidentaloma and its Treatment. JSM Clin Case Rep 4(2): 1101.
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