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Challenging Conventional Limits of Inoperability in Abdominal Desmoids and Sarcomas

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Challenging Conventional Limits of Inoperability in Abdominal Desmoids and Sarcomas Challenging Conventional Limits of Inoperability in Abdominal Desmoids and Sarcomas Kishore Iyer, MBBS, FRCS (Eng), FACS Associate Professor of Surgery and Pediatrics Intestinal Transplant Program Mount Sinai Medical Center New York [email protected] Disclosures • Consultant to NPS/Shire Pharmaceuticals • Scientific Advisory Board member to Merck Pharmaceuticals • NOT a surgical oncologist Soft tissue tumors at the mesenteric root • Desmoids or sarcomas • Often “non-resectable” by virtue of location • massive small bowel resection • Test of operability? • relationship to Superior Mesenteric Artery Latchford et al, Br J Surg 2006 Aorta SMA Primary/sec/tertiary branches Small bowel Aorta SMA Primary/sec/tertiary branches Small bowel The problem Underlying benign, indolent nature of the tumor Latchford et al, Br J Surg 2006 FAP and Desmoids : CGA-ICC Stage Definition Pts Years from Rapid Died of (n) colectomy growth desmoid to desmoid I • Asymptomatic 21 7.5 0 0 • <10 cm • not growing II • Mild symptoms 36 5.8 3 (8%) 0 • <10 cm • not growing • Moderate symptoms or 26 2.4 3 (12%) 4 (15%) bowel or ureteral III obstruction • 10-20 cm • slow growth IV • Severe symptoms 18 1.4 6 (33%) 8 (44%) • >20 cm • rapid growth Church J, et al. Dis Colon Rectum 51:897, 2008 Mortality of Intra-abdominal desmoids in FAP Quintini et al, Annals of Surgery. 255(3):511-516, March 2012. SB Transplant for desmoids • 14 allo-tx in 11 patients Patient Graft • All in the setting of FAP • 8 survivors 1 year 69.2% 62.5% – 1 on supplemental PN – 7 patients off PN 5 year 69.2% 50% • 3 ex-vivo resection and auto-tx – 2 survivors – 1 requires supplementary PN Moon JI et al, JSO. 2005;92: 284-291 Omaha experience, n = 21 in 23 y SB > 60 cm SB < 60 cm Wheeler et al, Cancers 2012 Aim • Planned surgical approach to “non- resectable’ tumors at the mesenteric root – Early vascular control and segmental dissection – Estimation of bowel loss – Prepare for ex-vivo resection and auto- transplant if feasible – Consider intestinal allo-transplant if severe sbs – All cases reviewed by multi-disciplinary team Results, n = 16 • 14 desmoids – 9 in setting of FAP – 7 Church Stage IV and 1 Church Stage III • 2 sarcomas • 12 patients alive • 6 after intestinal allo-transplant • 1 after ex-vivo resection and intestinal auto-transplant • 10 survivors on a normal diet, 1 on tpn (most recent resection) 1 aborted resection; died N = 16 2 medical rx 10 resected 3 ex-vivo resection + auto-tx 1 remains tumor-free; 3 > 220 cm of residual sb; normal diet 2 tumor-free, full diet 1 with 150 cm of 1 died at local ED 4 weeks post-auto tx; residual sb; R0; “cardiac”; no autopsy 6 with ≤ 60 cm of sb 1 bowel ischemia on day 7 6 sb tx 1 Died at home of ‘swine’ H1N1 Isolated SBtx (4 with 5 with normal graft function; colon) full diet; 2 recurrences Alive; tumor-free; normal diet “Ultra-extreme” short gut, n = 3 • 1 patient with ischemic bowel following auto- transplant with complete mid-gut loss • Radical tumor excision accomplished in 2 patients with complete excision of mid-gut – Stapled off gastric remnant (1 with gastro-colostomy) – Tube drainage of gastric remnant – Biliary tube for temporary biliary drainage – Stapled off left colon/rectal stump • All 3 patients alive with good graft function following SB transplant •Massive, multi-focal desmoid (FAP) •Gastric outlet obstruction/sepsis/entero-enteric fistulae •Failed chemo over 24 months • Sulindac/tamoxifen/doxorubicin/navelbine •Complete tumor clearance, with extensive bowel resection • Bilateral ureterolysis and bladder repair • Iliac reconstruction (contra-lateral artery and vein) • Bilateral salpingo-oophorectomy • 2 years on tpn •Isolated Intestine Transplant with colon, Oct 2012 – normal diet • Recurrent sarcoma; prior central pancreatectomy • SMA and SMV effaced • Ex-vivo resection of tumor with pancreatico-duodenectomy • Intestinal auto-transplantation (11/2008) • Tumor-free > 7 years; normal diet Surgical planning • Good cross- sectional imaging • Selected small bowel series/contrast enema • Prep for ex-vivo resection, flushing and auto-tx • Ureteric stents • Preparation for ureteric excision & reconstruction • Vascular dissection & reconstruction – Extra-anatomic bypass? • Nerve repair! The problem of recurrence • Intra-abdominal tumors • LRFS of 76% at 5 years • LRFS of 57% for tumors > 10 cm • 47% of R2 resections – intra-abominal • 8 of 15 patients with FAP had R2 resections Aimee et al, Ann Surg 2013. 258; 347 - 353 The primary tumor - Complete tumor excision with excision of midgut - Staged isolated intestinal transplant with colon The recurrence Biology still trumps! Recurrences • Of 16 patients: – 1 patient R2 resection (FAP) – 1 patient with local recurrence after massive, radical resection – 2 abdominal wall recurrences after transplant – 1 massive resected; stable para-spinal recurrences – 1 stable on tamoxifen Conclusions • Aggressive surgical approach to tumors at the root of the mesentery, may allow complete resection of “non-resectable” tumors • Patient selection is key to improving outcomes • Often benign course of underlying desmoid tumor • Weigh considerable risks and benefits carefully • Limit to rapidly growing, large, symptomatic tumors • May allow avoidance of intestinal transplantation in some cases • Rapid access to intestinal transplantation is critical .
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