Successful resection of a PNET tumor of the kidney with vena caval and cardiac invasion
Hong-Ru Chen BA, Sarah Fryberger MD, Jeffrey D. Lee MD, Linda L. Wong MD
This case report was published in the August, 2006 edition of Surgical Rounds. The patient is a 17 year old female who presented with progressive increase in abdominal girth and fatigue over a three month period. She also reported dyspnea and heart
palpitations on minimal exertion.
Her physical examination was significant for pleural effusions, a fluid wave with abdominal distention, and massive bilateral lower extremity edema. Her workup included a computed tomography (CT) which showed a large retroperitoneal mass involving the right kidney and extending into the renal vein, inferior vena cava, right atrium, and hepatic veins.
An open biopsy of the mass showed a primitive neuroectodermal tumor (PNET) with flourescent in situ hybridization (FISH) revealing the EWSR gene translocation consistent with Ewing sarcoma.
The patient received three cycles of vincristine, doxorubicin, and cyclophosphamide, which was stopped because of a pulmonary infection with Aspergillus. After clearing her fungal infection, she underwent exploratory laparotomy and sternotomy to attempt curative resection. The patient underwent circulatory arrest and the lower vena cava, renal vein, and right atrium were opened with removal of the tumor. The patient cardiopulmonary arrest was then reversed and the right kidney and tumor was removed.
Her postoperative course was significant for pancreatitis and coagulopathy. She was discharged home on postoperative day 16.
Editors discussion: This is an aggressive resection for an aggressive tumor. PNET’s are most commonly found in the soft tissues of the head, neck, trunk, and extremities, PNET’s of the kidney are exceeding rare, with a few dozen case reports constituting the literature. Use of special stains and markers such as CD99, neuron-specific enolase (NSE), and monoclonal antibodies can be used in making the diagnosis. In most of the previously reported cases, the patients did not receive neoadjuvant therapy but did receive postoperative adjuvant therapy. In 28 case reports where outcomes were reported, 9 patients had complete cures with surgery and chemotherapy.
Copyright 2006 Insidesurgery.com