Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report

Abstract

Peripheral primitive neuroectodermal tumor (PNET) of the kidney is a rare, aggressive tumor known for its recurrence and metastatic potential. Despite the frequency of venous extension to the renal veins and inferior vena cava, pulmonary tumor embolism at the initial presentation is not common. We report a case of 22-year-old female with PNET of the kidney who presented with tumor embolism in the inferior vena cava (IVC) and bilateral pulmonary artery. The patient underwent surgical resection and histopathological analysis confirmed the presence of tumor within the IVC and pulmonary arteries. The patient received adjuvant chemotherapy and is currently doing well on follow-up. Core tip: Traditionally, an aggressive renal mass with pulmonary tumor embolism is an entity well described in renal cell carcinomas. However, renal primitive neuroectodermal tumor (PNET), a rare and aggressive tumor, can have a similar presentation. The severity of the clinical manifestations of pulmonary tumor embolism is highly variable and less predictable. Bland thrombus often mimics tumor emboli in imaging and histopathology is often confirmatory. Tumoral extension into the pulmonary artery is not necessarily associated with pulmonary metastasis. Surgical removal of the thrombus along with the primary tumor followed by adjuvant chemotherapy may prolong the survival in these patients.Chinnaa S, Das CJ, Sharma S, Singh P, Seth A, Purkait S, Mathur SR. Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report. World

Primary renal primitive neuroectodermal tumour causing Budd–Chiari syndrome: a rare case report

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