ABSTRACT
OBJECTIVES: Primitive neuroectodermal tumors (PNET) are rare highly aggressive neoplasms. The diagnosis is made by histopathology with the support of immunohistochemistry (IHC) and cytogenetics. The aggressive multimodality treatment is recommended for the management of these tumors. The purpose of our study is to review our experiences in the diagnoses and treatment of PNET of the kidneys. MATERIALS AND METHODS: We retrospectively reviewed the data of all the patients, who were treated for the PNET of the kidneys at our institute between April and March 2011 and compared with the available literature. RESULTS: A total of eight patients were treated for PNET of the kidney. Out of the eight patients, four were males and four females. Nearly 50% of our patients had inferior vena caval thrombus at the time of presentation. The diagnosis was made on histopathology supported by IHC. Out of the eight patients, one patient had intraoperative death due to massive pulmonary thromboembolism and another died on the 7th post‑operative day due to disseminated intravascular coagulation and multiorgan failure. Rest six patients were treated with post‑operative chemotherapy or a combination of chemotherapy and radiotherapy. For these six patients, overall median survival was 45 months with a 3 year disease‑free survival of 66% and 5 year survival of 44%. CONCLUSIONS: PNET of the kidneys are rare peripheral neuroectodermal tumors with an aggressive clinical course. These tumors carry a very poor prognosis. An aggressive treatment approach using a combination of surgery, chemotherapy and radiotherapy is recommended for a reasonable survival in these tumors.
ABSTRACT
El Cáncer Renal se presenta habitualmente como un hallazgo de las imágenes abdominales y cuando se manifiesta clínicamente lo hace a través de los efectos que produce una gran masa abdominal o las metástasis a distancia. En este trabajo se presenta un caso de cáncer renal cuyas manifestaciones clínicas fueron secundarias a la obstrucción de las venas supra hepáticas, es decir un síndrome de Budd-Chiari florido por la presencia de un trombo tumoral en la cava retro hepática. El diagnóstico oportuno y una evaluación adecuada con imágenes permitieron la ejecución de un plan de tratamiento que incluyó un abordaje combinado abdominal y torácico que permitió un tratamiento no solo sintomático sino que potencialmente curativo de un cáncer renal con esta rara presentación.
Renal Cancer usually occurs as a finding of abdominal imaging and when it clinically manifests itself it does so through a large abdominal mass or distant metastases. In this paper we present a case of renal cancer whose clinical manifestations were secondary to obstruction of hepatic veins, a Budd-Chiari syndrome because of the presence of a tumor thrombus in the retro hepatic vena cava. Early diagnosis and appropriate imaging evaluation allowed the implementation of a treatment plan that included a combined abdominal and thoracic approach allowing not only a symptomatic treatment but potentially curative treatment of renal cancer with this rare presentation.