COMPILATION OF YOUNG PATIENTS WITH RARE PRESENTATION UNDERGOING RADICAL MULTIVISCERAL SURGICAL RESECTIONS AND RECONSTRUCTION

Background: Multivisceral surgical resections (MVR) are highly specialised procedures involving the surgical resection of multiple organs or tissue groups within the thorax, abdominal and pelvic cavities. Here we describe a series of patients who underwent complex major mutivisceral resections unique in their type in the world literature. Materials and Methods: Case 1: Patient underwent Subtotal Sternectomy with en-bloc 3rd-6th partial rib resection with sternal reconstruction by Sandwich technique (2 layers of Prolene mesh with PMMA cement interposition) & Pectoralis Myocutaneous flap. Postoperative histopathology was suggestive of Epithelioid Malignant Peripheral Nerve Sheath tumor arising in a Schwannoma. Case 2: Patient underwent enbloc radical resection of the retroperitoneal tumor with right nephrectomy, subtotal cholecystectomy, partial right hemidiaphragm resection with vascular dissection following 5 cycles neoadjuvant chemotherapy. Postoperative histopathology showed features suggestive of Extra skeletal Ewing’s sarcoma, Grade III. Case 3: Patient underwent left radical nephrectomy with left colectomy with para –aortic lymph node dissection with pancreatic and extensive retroperitoneal dissection with colocolic anastomosis and omentectomy. Postoperative histopathology was suggestive of Fumarate hydratase deficient renal cell carcinoma infiltrating the perinephric fat, Gerota’s fascia and left adrenal with high grade. Results: Case 1: Postoperatively patient received adjuvant chemotherapy and radiation therapy. Patient is disease free for the past 2 years and is on regular followup. Case 2: Patient received adjuvant chemotherapy and radiation therapy at native place. Patient developed  unsalvageable local recurrence 8 months later and succumbed to the disease. Case 3: Patient has completed adjuvant chemotherapy. Patient is disease free and on regular followup. Conclusion: Multivisceral surgical resections (MVR) as part of extensive major surgical oncology resection procedures is feasible with least peri-operative morbidity for rare tumors in complex locations with meticulous technical expertise. The radicality and completeness of surgical resection  determines the short-term and long-term oncological outcomes.