Sphincter-saving resection by cluneal arched skin incision for a gastrointestinal stromal tumor (GIST) of the lower rectum: a case report

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1 Tzw et l. Surgicl Cse Reports (2017) 3:8 DOI /s CASE REPORT Sphincter-sving resection y clunel rched skin incision for gstrointestinl stroml tumor (GIST) of the lower rectum: cse report Hirofumi Tzw 1*, Yuzo Hirt 1, Yoshio Kug 2, Toshihiro Nishid 3 nd Hideto Skimoto 1,4 Open Access Astrct Bckground: Plnning the surgicl strtegy for gstrointestinl stroml tumor (GIST) t the posterior wll of the lower rectum is difficult, s the procedures for the lower rectum re hmpered y poor visuliztion nd my cuse nl dysfunction or discomfort. We report novel procedure to resect sumucosl tumor of the rectum. Cse presenttion: A 75-yer-old womn presented with metrorrhgi. Endovginl ultrsonogrphy showed low echoic tumor. Computed tomogrphy showed n enhnced tumor, mesuring cm, ehind the rectum. Mgnetic resonnce imging reveled sumucosl tumor of the rectum, mesuring 5.3 cm t its gretest dimension. Colonoscopy showed tht the distl tumor mrgin ws 1 cm ove the dentte line. Core needle iopsy of the tumor reveled the rectl GIST. After receiving neodjuvnt imtini tretment, the tumor size decresed to 3.5 cm. During the opertion, we pproched the rectum nd resected the posterior rectl wll, including the cm tumor with sfety mrgin, mking n rched incision t the uttocks to form skin flp with the ptient in jckknife position. The histopthologicl dignosis ws GIST of the rectum. Her norectl sphincter function ws well preserved. No recurrence ws seen during the 2-yer follow-up. Conclusions: This novel pproch improves the opertive field visiility in resecting tumor with sfety mrgin nd preserves ptient s norectl sphincter function. Keywords: Rectl GIST, Clunel rched skin incision, Sphincter-sving opertion Bckground Gstrointestinl stroml tumors (GISTs) occur most often in the stomch (60%) nd the smll intestine (35%) [1]. GISTs of the colon nd rectum constitute <5% of ll cses nd occur more often in the rectum [2]. Surgery with histologiclly negtive mrgins is the recommended primry tretment for nonmetsttic GISTs [3]. Neodjuvnt therpy of imtini, selective tyrosine kinse inhiitor, for rectl GISTs, with the im of preserving the nus, is still chllenging therpy tht typiclly tkes severl months [4]. Plnning the surgicl strtegy for GIST t the posterior wll of the lower rectum is difficult, s the * Correspondence: thiroes@gmil.com 1 Deprtment of Surgery, Chugoku Rosi Hospitl, 1-5-1, Tgy, Hiro, Kure City, Hiroshim , Jpn Full list of uthor informtion is ville t the end of the rticle procedures for the lower rectum re hmpered y poor visuliztion nd my cuse nl dysfunction or discomfort. We descrie our experience with ptient who underwent novel pproch, which improves the opertive field visiility in resecting tumor with sfety mrgin nd preserves ptient s norectl sphincter function. Cse presenttion A 75-yer-old womn with metrorrhgi visited gynecology clinic. Trnsvginl ultrsound showed retroperitonel tumor. She ws referred to our hospitl for detiled exmintion. The results of the lortory exmintions were s follows: white lood cell count, 5660/mm 3 (norml rnge ); red lood cell count, /mm 3 (norml rnge ); hemogloin level, 12.5 g/dl (norml rnge ); pltelet count, The Author(s) Open Access This rticle is distriuted under the terms of the Cretive Commons Attriution 4.0 Interntionl License ( which permits unrestricted use, distriution, nd reproduction in ny medium, provided you give pproprite credit to the originl uthor(s) nd the source, provide link to the Cretive Commons license, nd indicte if chnges were mde.

2 Tzw et l. Surgicl Cse Reports (2017) 3:8 Pge 2 of /mm 3 (norml rnge ); serum lood ure nitrogen level, 18.6 mg/dl (norml rnge 8 20); serum cretinine level, 0.46 mg/dl (norml rnge ); nd tumor mrker levels were within the norml rnges (crcinoemryonic ntigen, 3.9 ng/ml nd CA, U/mL). The lortory dt were within norml limits, insted of demonstrting them. She hd no history of serious illnesses, opertions, or hospitliztions. Computed tomogrphy (CT) showed n enhnced tumor, mesuring cm, ehind the rectum (R) (Fig. 1 nd c). Pelvic mgnetic resonnce imging (MRI) detected well-defined tumor with low T1 nd high T2 intensities (Fig. 2, ). Colonoscopic exmintion reveled tht sumucosl tumor t the posterior rectl wll, nd tht the distl tumor mrgin ws 1 cm ove the dentte line (Fig. 2c). Core needle iopsy of the tumor reveled undles of spindle cells with positive immunohistochemicl stining for c-kit nd CD34, ut negtive for S100 proteins. The pthologicl findings led to the dignosis of rectl GIST. Neodjuvnt therpy ws initited with imtini 400 mg orlly dily to reduce the resection rnge. The therpy ws scheduled for 6 months nd would e followed y surgicl removl of the remining tumor. CT t 1 month fter strting chemotherpy showed tht the tumor size hd decresed to 4.5 cm. Unfortuntely, 4 months fter the initition of neodjuvnt therpy, the ptient developed generlized erythemtous ppules with severe itching. CT exmintion nd colonoscopy reveled tht the tumor size hd decresed to cm (Figs. 1, d, nd 2d). The ptient underwent prtil sphinctersving rectl resection with cretion of n ileostomy. The ptient ws plced in prone jckknife position on the operting tle, with the legs slightly ducted nd the uttocks strpped prt using dhesive tpes from the uttocks to the tle (Fig. 3). First, the skin flp ws mde y clunel rched skin incision etween the sucutneous ft nd lvtory muscle. We then pproched the posterior rectl wll through the side of the gluteus mximus muscle nd lvtory muscle of the nus. The Wldeyer s fsci ws incised to expose the tumor nd the red rectl wll. The tumor, lifted vi digitl rectl exmintion, ws extrcted with sfety mrgin (Fig. 3, d, nd e). The rectl wll ws closed using the Gmee s method (sorle suture), with suturing of the muscle lyer (3 0 silk) (Fig. 3c). A 19-Fr. soft silstic tue drin ws plced ner the suture line, nd port site closure ws completed. A temporry ileostomy ws creted vi lproscopic surgery. Histopthologicl exmintion reveled tht the tumor ws locted in the musculris of the rectum, with negtive mrgin (Fig. 4), nd hd widespred centrl necrosis vi effective response from imtini (Fig. 4). Microscopic exmintion showed the tumor ws consisted of undle-like prolifertions of spindle-shped cells (Fig. 4c). The immunohistologicl findings showed tht the tumor cells stined negtively with S100 nd SMA nd positively with c-kit nd CD34; the tumor ws dignosed to e KIT-positive GIST (Fig. 4d). The c d Fig. 1 Coronl CT imges showed n enhnced tumor mesuring cm ehind the rectum. After the tretment of imtini, coronl CT imges reveled tht the tumor size decresed to 3.5 cm. c Axil CT imges showed n enhnced tumor t the right posterior side of the rectum. d After tretment of imtini, xil CT imges reveled tht the tumor size decresed to 3.5 cm

3 Tzw et l. Surgicl Cse Reports (2017) 3:8 Pge 3 of 6 c d Fig. 2 T1-weighted MRI showed low intensity tumor. T2-weighted MRI showed high intensity tumor. c Colonoscopic exmintion reveled sumucosl tumor t the posterior rectl wll, nd tht the distl tumor mrgin ws 1 cm ove the dentte line. d Colonoscopic exmintion reveled tht the tumor mrkedly decresed fter the tretment of imtini postopertive course ws uneventful, nd the ileostomy ws closed 2 months lter. Postopertive nl function ws preserved, nd the opertive scr ws fine nd inconspicuous (Fig. 5 nd ). Neither locl recurrence nor distnt metstsis ws noted during the 2-yer follow-up without djuvnt therpy. Discussion GISTs re rre ut re, nevertheless, the most common mesenchyml neoplsms of the gstrointestinl trct. Mzur nd Clrk first introduced the term GIST in 1983 tht constitute round 1% of ll primry gstrointestinl cncers [5]. Specific muttions in the so-clled KIT c rectum d e gluteus mximus muscle lvtor muscle skin flp Fig. 3 The ptient ws plced in the jckknife position, with the uttocks prted y dhesive strpping. This is the imge of the tumor resection, showing n opening of the rectum (rrow). c The rectl wll ws closed using the Gmee s method with suturing of the muscle lyer. d The tumor, lifted vi digitl rectl exmintion, ws extrcted with sfety mrgin. e This is the schem of Fig. 3

4 Tzw et l. Surgicl Cse Reports (2017) 3:8 Pge 4 of 6 Fig. 4, The resected tumor ws cm in size. The ruery-hrd tumor with widespred centrl necrosis ws completely cpsulized. c Microscopic exmintion (hemtoxylin-eosin stining, originl mgnifiction: 400) reveled formtion of spindle-shped cells. d Immunohistochemicl stining of the tumor cells for c-kit reveled strong positive findings oncogene re the most common cuse of the development of this tumor [6]. They cn e found in the stomch (51%), jejunum nd ileum (36%), nd colon nd rectum (5 7%), ut cn lso occur extr-gstrointestinlly in the mesentery or omentum in rre cses [7, 8]. Size nd mitotic ctivity contriute to the risk estimtion for mlignnt ehvior of GIST, ccording to the Ntionl Cncer Institute (NCI) consensus clssifiction [3, 4, 9]. Surgery remins the therpy of choice for ptients with primry GISTs, with no evidence of metstsis nd should e the initil therpy if the tumor is techniclly resectle nd ssocited with n cceptle moridity risk [3]. The gol of the opertion is complete gross resection with negtive microscopic mrgin (R0 resection) without leeding nd rupture of the pseudocpsule [10]. Neodjuvnt imtini tretment is lso n option to fcilitte functionpreserving surgeries for tumors in the gstro-esophgel junction nd rectum [11, 12]. Imtini Mesylte is n orlly dministered competitive inhiitor of the tyrosine kinse ssocited with the KIT protein (stem cell fctor receptor), ABL protein, nd pltelet derived growth fctor receptors. Mny studies hve shown the effectiveness of imtini in the tretment of GISTs since its first report in 2001 [13]. As for rectl GISTs, severl reports demonstrted tht neodjuvnt imtini tretment improved R0 resection rtes nd decresed the risk of postopertive moridity [11, 14]. In the Ntionl Comprehensive Cncer Network guidelines, it is recommended tht neodjuvnt Fig. 5 The opertive scr ws fine nd inconspicuous (rrow). Colonoscopy ws performed efore closing the ileostomy. The exmintion showed fine scr (rrow) with no stenosis

5 Tzw et l. Surgicl Cse Reports (2017) 3:8 Pge 5 of 6 imtini tretment should e considered if dominoperinel resection is necessry to chieve negtive resection mrgin, or if the surgeon feels tht multiviscerl resection my e required [15]. However, in the restricted spce of the pelvis, complete resection of lrge rectl GIST is difficult nd often necessittes dominoperinel resection or intersphincteric resection (ISR), with or without djcent orgn resection. Miettinen et l. reported the tretment of 144 cses of norectl GIST [2]. In this study, the smller tumors ( 2 cm) were typiclly treted y enucletion only, excluding one cse. Tumors tht were 2 5 cm were lso usully treted with locl excision. Lrge tumors (>5 cm) were commonly removed y dominoperinel or nterior resection with impirment of the sphincter function (15 primry cses nd 2 cses for the tretment of recurrence). There re some reports in the literture descriing trnsnl, trnscoccygel, nd trnsvginl pproches for the locl excision of GISTs locted in the lower rectum with the im of decresing the moridity rte. Trnsnl excision is the most minimlly invsive pproch; however, there is limit to the distnce from the dentte line. Koscinski et l. reported tht trnsnl excision is pproprite for lesions locted t n verge distnce of 3 cm from the dentte line [16]. Furthermore, whether this procedure is possile is often dominted y the physique of the ptient. Bledy indicted tht trnscoccygel excision ws especilly useful for lesions t the posterior rectl wll nd pproprite for lesions locted t n verge distnce of 5 cm from the dentte line [17]. However, trnscoccygel excision provides poor field of view ecuse of its high moridity rte, such s postopertive fistul occurring in 21% of ptients [18]. The choice of locl resection tht preserves the nl function must result in negtive mrgin different from the conventionl extend opertion. In our cse, the 5.3-cm sized tumor situted t the posterior wll of the lower rectum nd the distl tumor mrgin ws 1 cm ove the dentte line. Neodjuvnt imtini therpy ws scheduled for 6 months s the references which reported, in rndomized clinicl studies, the cumultive incidence of response lmost reched plteu fter tretment for 6 8 months, nd disese progression occurred in some ptients even in this period [19, 20]. After the dministrtion of imtini, the tumor size decresed to 3.5 cm. The possile opertions for selection were dominoperinel resection, ISR, or locl resection. However, we voided dominoperinel resection nd ISR to preserve her nl function. The tumor ws too lrge to select the trnsnl pproch for locl resection. The typicl opertion using the posterior pproch for rectl tumors is the method reported y Krske [21]. This method hs stright-line incision ecuse the surgicl field view is poorer thn tht of ours. On the other hnd, the surgicl field view is cler with nl dysfunction y cutting the sphincter in Mson s method [22]. Finlly, we chose the sphincter-sving opertion using clunel rched skin incision, which hs lredy een reported in Jpnese literture [23]. In this cse, we performed the opertion on the sis of this literture [23]. It is esy to pproch the puorectl muscle nd externl nl sphincter y mking skin flp with n rch-shped incision t the uttocks. After splitting oth sides of the posterior rectum, the tumors lifted from the inside of the rectum were resected with the sfety mrgin. The dvntges of this method include preserving the nl function nd providing cler view during the opertion. In ddition, surgicl site infection is prevented s the skin incision in our method is fr from the nus. Conclusions We descrie our experience with the ptient who underwent novel pproch tht improves the opertive field visiility in resecting tumor with sfety mrgin nd preserves ptient s norectl sphincter function. Arevitions CT: Computed tomogrphy; GISTs: Gstrointestinl stroml tumors; ISR: Intersphincteric resection; MRI: Mgnetic resonnce imging; NCI: Ntionl Cncer Institute Authors contriutions HT, YH, nd HS wrote the mnuscript. YK dignosed this cse nd performed the chemotherpy. HT nd YH performed the opertion. TN dignosed the disese pthologiclly. All uthors conceived the study, prticipted in its design nd coordintion, nd helped drft the mnuscript. All uthors red nd pproved the finl mnuscript. Competing interests The uthors declre tht they hve no competing interests. Consent for puliction Written informed consent ws otined from the ptient for the puliction of this cse report nd ny ccompnying imges. A copy of the written informed consent is ville for review y the Editor-in-Chief of this journl. Author detils 1 Deprtment of Surgery, Chugoku Rosi Hospitl, 1-5-1, Tgy, Hiro, Kure City, Hiroshim , Jpn. 2 Deprtment of Internl Medicine, Chugoku Rosi Hospitl, 1-5-1, Tgy, Hiro, Kure City, Hiroshim , Jpn. 3 Deprtment of Dignostic Pthology, Chugoku Rosi Hospitl, 1-5-1, Tgy, Hiro, Kure City, Hiroshim , Jpn. 4 Deprtment of Gstroenterologicl nd Trnsplnt Surgery, Applied Life Sciences, Institute of Biomedicl nd Helth Sciences, Hiroshim University, 1-2-3, Ksumi, Minmi-ku, Hiroshim , Jpn. Received: 14 Novemer 2016 Accepted: 27 Decemer 2016 References 1. Miettinen M, Lsot J. Gstrointestinl stroml tumors: review on morphology, moleculr pthology, prognosis, nd differentil dignosis. Arch Pthol L Med. 2006;130(10): Miettinen M, Furlong M, Srlomo-Rikl M, Burke A, Soin LH, Lsot J. Gstrointestinl stroml tumors, intrmurl leiomyoms, nd leiomyosrcoms in the rectum nd nus: clinicopthologic, immunohistochemicl, nd moleculr genetic study of 144 cses. Am J Surg Pthol. 2001;25(9): Demetri GD, Mehren MV, Antonescu CR, DeMtteo RP, Gnjoo KN, Mki RG, et l. NCCN Tsk force report: updte on the mngement of ptients with gstrointestinl stroml tumors. J Ntl Compr Cnc Netw. 2010;8 suppl 2:S1 41. quiz S42-44.

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