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Desmoplastic small round cell tumor (DSRCT) is a rare mesenchymal tumor usually affecting young patients. Local dissemination is common, and liver is the most common site for extraperitoneal metastases. Multimodal management has been shown to be the most effective treatment. Some authors consider liver metastases especially bi-lobar disease as a contraindication for surgical resection. We present a case of a DSRCT with bi-lobar metastases in an adult patient who underwent multi-modal management along with hepatectomy. A 51-year-old man was found to have a large intraperitoneal mass with bi-lobar liver metastases during work up for new onset reflux and abdominal pain. Biopsy confirmed it as DSRCT. The patient was treated with multi-modal therapy including cytoreductive surgery (CRS), hyperthermic intraperitoneal chemotherapy (HIPEC), two-stage hepatectomy (TSHP) combined with ( 90 Y) Yittrium-90 radioembolization of the right hepatic lobe and systemic chemotherapy. The patient had a right-sided pleural empyema one month after the surgery for which he required right-sided video-assisted thoracoscopy and decortication. He remains disease free at 2 years follow-up. DSRCT with bi-lobar liver metastases are best managed with multimodal therapy. TSHP seems to be a feasible and safe option in selected cases, with a potentially good outcome.

journal of gastrointestinal oncology

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy combined with two-stage hepatectomy for multiple and bilobar desmoplastic small round cell tumor liver metastases