3 research outputs found

    Do Primitive Neuroectodermal Tumors of the Kidney Have a Predilection for Inferior Vena Cava Involvement? A Case Series and Review of the Literature

    Get PDF
    The primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare neoplasm, the diagnosis of which mainly depends upon histopathology, immunohistochemistry (IHC), and cytogenetics. A handful of cases reported in the literature mention about aggressive features of this neoplasm. The purpose of our study was to review our experience in not only the diagnosis and management of the patients with renal PNET but also to highlight its propensity to involve inferior vena cava (IVC) and also present a rare occurrence of Ewing’s sarcoma (ES)/PNET of the renal pelvis. The clinical, operative, and histopathology records of four patients of renal PNET treated between January 2017 and December 2019 were reviewed and data analyzed concerning the available literature. Out of the four patients treated, two had level III and IV IVC thrombus, and one had dense desmoplastic adhesions with the IVC wall. One of the cases had a rare presentation of ES/PNET of the renal pelvis. All patients were managed surgically, while only one patient received adjuvant chemotherapy and following up with remission for the last 2 years and 4 months. On IHC, cluster of differentiation-99 (CD-99) was positive in all patients, and three were positive for Friend leukemia integration-1. PNET of the kidney is primarily an immunohistopathological diagnosis. This neoplasm has an increased propensity for the local invasion of surrounding structures. A multimodality approach with surgery, chemotherapy, and radiotherapy could offer better outcomes, although the prognosis of these tumors remains poor

    Management Outcomes of Large Renal Angiomyolipoma Presenting with Wunderlich Syndrome—Experience from a Tertiary Center

    Get PDF
    Renal angiomyolipoma is an uncommon, benign-mixed mesenchymal tumor consisting of thick-walled blood vessels, smooth muscles, and mature adipose tissues. Twenty percent of these tumors are associated with tuberous sclerosis. Wunderlich syndrome (WS), an acute nontraumatic spontaneous perirenal hemorrhage, can be a presentation of large angiomyolipoma. This study evaluated the presentation, management, and complications of renal angiomyolipoma with WS in eight patients who presented to the emergency department between January 2019 and December 2021. The presenting symptoms included flank pain, palpable mass, hematuria, and bleeding in the perinephric space on computerized tomography. Demographic data, symptoms at presentation, comorbidities, hemodynamic parameters, the association with tuberous sclerosis, transfusion requirements, need for angioembolization, surgical management, Clavien–Dindo complication, duration of hospital stay, and 30-day readmission rates were evaluated. The mean age of presentation was 38 years. Of the eight patients, five (62.5%) were females and 3(37.5%) were males. Two (25%) patients had tuberous sclerosis with angiomyolipoma, and three (37.5%) patients presented with hypotension. The mean packed cell transfusion was three units, and the mean tumor size was 7.85 cm (3.5–25 cm). Three of them (37.5%) required emergency angioembolization to prevent exsanguination. Embolization was unsuccessful in one patient (33%) who underwent emergency open partial nephrectomy, and one (33%) patient developed post-embolization syndrome. A total of six patients underwent elective surgery—four underwent partial nephrectomy (laparoscopic - 1, robotic - 1, open - 2) and two underwent open nephrectomy. Three patients encountered Clavien–Dindo complications (Grade 1, n = 2 and IIIA, n = 2). WS is a rare, life-threatening complication in patients with large angiomyolipoma. Judicious optimization, angioembolization, and prompt surgical intervention will help deliver better outcomes

    Trifecta Outcomes in Open, Laparoscopy or Robotic Partial Nephrectomy: Does the Surgical Approach Matter?

    Get PDF
    This retrospective study evaluated perioperative outcomes of open partial nephrectomy (OPN), laparoscopic partial nephrectomy (LPN), and robot-assisted partial nephrectomy (RAPN) and identified predictive factors of Trifecta achievement for renal tumors that underwent partial nephrectomy (PN) in a single institutional cohort. The study involved patients who underwent PN from January 2011 to July 2018. Trifecta was defined as absence of perioperative complications, no positive surgical margins, and ischemia time <30 min. Fifty-five PN procedures were reviewed: 28 OPN, 14 LPN, and 13 RAPN. OPN, LPN and RAPN had similar median tumor size (5.75, 5.25, and 5 cm), nephrometry score (7, 6, and 6), and preoperative creatinine (1.09, 1.1, and 1.1 mg/dl, respectively). Blood loss was higher for OPN (550 ml) than for LPN (400 ml) and RAPN (300 ml), P = 0.042. Drain was removed after 6 days in OPN which was higher than LPN and RAPN (4.5 and 4 days, respectively), P = 0.008. OPN, LPN, and RAPN had similar median operative time (190, 180, and 180 min, respectively), P = 0.438. Median postoperative stay for OPN, LPN, and RAPN was 5, 6.5, and 10 days, respectively. Trifecta outcomes of 73.1%, 64.3%, and 61.53% were achieved in OPN, LPN, and RAPN, respectively, P = 0.730. It was concluded that Trifecta outcomes had no significant difference among OPN, LPN, and RAPN. LPN can produce as good results as RAPN. Keeping in mind the cost-effectiveness, LPN holds an important position in developing countries where expenditure by patient is a major factor
    corecore