Introduction.Mixed epithelial and stromal tumour of the kidney (MEST) is a rare and distinctive neoplasm accounting for 0.2% of all renal cancers. Most of these tumours behave in a benign fashion but 13 cases with malignant transformation have already been reported. We present the first case of an extremely aggressive MEST with rapid recurrence after radical treatment, demonstrating objective response to chemotherapy.Case presentation.A 31-year-old female presented to the hospital complaining of gross hematuria. Computed tomography (CT) revealed an intraparenchymal mass in the left kidney forming a tumour thrombus in the inferior vena cava (IVC). Metastatic disease was ruled out and, under the clinical diagnosis of renal cell carcinoma, left radical nephrectomy with IVC thrombectomy was performed. The histopathological examination confirmed malignant MEST of the kidney. At the follow-up 12 months after surgery, a recurrent tumour in the left paravertebral area and a tumour thrombus in the IVC were detected. A second surgery was recommended and the mass from the paravertebral area was removed, so resection of the IVC with prosthetic replacement was performed. The histopathologic examination confirmed a recurrent malignant MEST. At the follow-up three months after the second surgery disease progression was diagnosed, so chemotherapy with ifosfamide and doxorubicin was initiated. The CT scan performed 14 months after the chemotherapy confirmed a stable process of the disease with no signs of progression.Conclusions.A literature review and our case report confirm the existence of extremely aggressive malignant MEST that shows response to chemotherapy. However, more reports are needed to improve our understanding about the biology of the MEST to develop any recommendations on personalized therapy.
Po operacijos pagal Nesbito komplikacijos išsivystė 5 (10,2 proc.) pacientams, be komplikacijų operacija praėjo 44 (89,8 proc.) pacientams. Grupėje, kurioje komplikacijų nenustatyta, vidutinė operacijos trukmė -85,14 ± 20,59 min., o grupėje, kuroje išsivystė pooperacinės komplikacijos, -90 ± 43,78 min., kai p = 0,693. Grupėje, kurioje išsivystė komplikacijos, vidutinis akytkūniuose išpjautų langų skaičius -3,75 ± 2,06, o grupėje, kurioje komplikacijų nenustatyta, -4 ± 2, kai p = 0,817. Pacientams, kuriems atlikta simetrinė varpos plastika, vidutinė operacijos trukmė buvo 92,50 ± 20,6 min., o grupėje, kurioje asimetrinė -70,42 ± 22,2 min., kai p = 0,004. Vidutinė hospitalizacijos trukmė -5,2 ± 4,91 dienos. Išvados. Įgimto varpos kreivumo chirurginis gydymas pagal Nesbito yra efektyvus metodas siekiant ištiesinti varpą. Įgimtas varpos kreivumas dažniausiai operuojamas jauniems, lytiškai aktyviems pacientams, dėl to nuo operacijos sėkmės priklauso psichologinė ir seksualinė paciento gyvenimo kokybė. aBstract Key words: congenital penile curvature, penile plastic surgery, surgical treatment. Objective. The main purpose of this study is evaluate surgical treatment results of congenital penile curvature at Vilnius university hospital Santariskiu klinikos Urology center. Patients and methods. From 1999 to 2014, Vilnius University Hospital Urology Center, 50 patients were surgical treated for congenital penile curvature. 49 of them it was made by Nesbit and only for one was performed penile lengthening and straightening surgical treatment. Before surgery by used sol. saline which cause an erection was evaluated fault location, size and depth of the penis curvature. Results. From 1999 to 2014, Vilnius University Hospital Urology Center was performed 49 operations by Nesbit, for clinical diagnosis of congenital penile curvature. The average age of 22.7 ± 29.4 years (the youngest -17 years old, the oldest -40 years old patient). With ventral deviation were 20 (40.0 %) patients, lateral -15 (30.0 %) patients, dorsal -7 (14.0 %), and for 8 (16.0 %) patients were diagnosed mixed deviation. The highest measured the tilt angle of the penis before the surgery -was 90°, the minimum -15°. After surgery by Nesbit, complications developed in 5 (10.2%) patients, without compli-
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