Primary extramedullary plasmacytoma of the sigmoid colon with perforation: a case report

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1 Kitmur et l. Surgicl Cse Reports (2018) 4:28 CASE REPORT Open Access Primry extrmedullry plsmcytom of the sigmoid colon with perfortion: cse report Fumims Kitmur 1*, Koichi Doi 1, Hiroyuki Ishiodori 1, Tetsufumi Ohchi 1 nd Hideo B 2 Astrct Bckground: Extrmedullry plsmcytoms ccount for 4% of ll plsm cell tumors nd occur minly in the upper respirtory trct; gstrointestinl system involvement is rre. Extrmedullry plsmcytom of the colon with perfortion hs not een reported. Cse presenttion: A 77-yer-old womn with 1-yer history of lower dominl pin nd nuse ws dmitted to our hospitl. An dominl computed tomogrphy scn reveled sigmoid tumor with perfortion. The ptient underwent emergency surgery. Pthologicl exmintion led to dignosis of plsmcytom of the colon. The ptient did not undergo postopertive djuvnt chemotherpy. She hs hd no recurrence in 14 months of regulr follow-up. Conclusions: We hve herein descried rre cse of extrmedullry plsmcytom of the gstrointestinl trct with perfortion involving the sigmoid colon. Keywords: Extrmedullry plsmcytom, Colon neoplsms, Perfortion Bckground A plsm cell tumor is n immunoprolifertive monoclonl disese of the B cell line tht origintes from mlignnt trnsformed plsm cells. Plsmcytom includes solitry plsmcytom of one nd solitry extrmedullry plsmcytom. Solitry extrmedullry plsmcytom hs een rrely reported, nd its nturl history nd dignosis re uncler. Most such plsmcytoms occur in the nsophrynx or upper respirtory trct; only 10% of reported cses hve involved the gstrointestinl trct. The stomch nd smll intestine re the most commonly involved sites in the gstrointestinl trct [1 3]. Primry isolted extrmedullry plsmcytom of the colon is extremely rre. No previous reports hve descried plsmcytom of the colon with perfortion. We herein report rre cse of primry isolted extrmedullry plsmcytom of the colon with perfortion nd descrie the ptient s postopertive clinicl course. * Correspondence: fumimskitmur@yhoo.co.jp 1 Deprtment of Gstroenterologicl Surgery, Noeok Hospitl, Shinkoji, Noeok, Miyzki , Jpn Full list of uthor informtion is ville t the end of the rticle Cse presenttion A 77-yer-old womn with 1-yer history of lower dominl pin nd nuse ws dmitted to our hospitl. Blood exmintion showed evidence of n inflmmtory response (Tle 1), nd dominl computed tomogrphy reveled sigmoid tumor with perfortion (Fig. 1). We suspected sigmoid cncer with perfortion, nd the ptient underwent emergency surgery. Open lprotomy reveled n extensive mss involving the sigmoid colon with surrounding contmintion (Fig. 2). The dominl mss ws removed en loc, including resection of the sigmoid colon. The domen ws flushed to remove contmintion. An rtificil nus ws mde. Histopthologic exmintion showed tht the ovl mss ws composed of diffuse prolifertion of plsm cells (Fig. 3). At the concvity of the site of the perfortion showed the tumor cell infiltrted into the suseros nd necrosis of tissue. But we were unle to identify the site of the perfortion pthologiclly. The surgicl mrgins were free from tumor cells. Immunohistochemicl exmintion reveled positivity for CD79 (Fig. 3), immunogloulin G, nd lmd light chin (Fig. 4, ). Other mrkers (CD10, CD20, nd kpp light 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 Kitmur et l. Surgicl Cse Reports (2018) 4:28 Pge 2 of 5 Tle 1 Blood exmintion Blood count Biochemicl prmeters Cogultion prmeters WBC 8090/μL TP 3.5 g/dl PT (s) 14.3 RBC /μl Al 1.59 g/dl PT (%) 63.3 H 11.5 g/dl T-il 1.19 mg/dl PT-INR 1.21 Plt /μl AST 28 IU/L APTT (s) 45.4 ALT 18 IU/L ATIII 49% ALP 127 IU/L FDP 40.0 μg/ml LDH 219 IU/L D-dimers ng/ml γ-gtp 14 IU/L Tumor mrkers BUN 15.6 mg/dl CEA 1.6 ng/ml Cr 0.4 mg/dl CA U/mL N 134 meq/l Cl 3.6 meq/l CRP mg/dl WBC white lood cells, RBC red lood cells, H hemogloin, Plt pltelets, CEA crcinoemryonic ntigen, CA19-9 cncer ntigen 19-9, TP totl protein, Al lumin, T-il totl iliruin, AST sprtte trnsminse, ALT lnine trnsminse, ALP lkline phosphtse, LDH lctte dehydrogense, γ-gtp gmm glutmyl trnsferse, BUN lood ure nitrogen, Cr cretinine, N sodium, Cl chloride, CRP C-rective protein, PT prothromin time, PT-INR prothromin time interntionl normlized rtio, APTT ctivted prtil thromoplstin time, ATIII ntithromin III, FDP firin degrdtion products chin) were negtive (Fig. 4c). Pthologicl exmintion led to dignosis of plsmcytom of the colon. The ptient underwent one mrrow iopsy nd one imging to exclude ssocited multiple myelom. Her peripherl lood smer, serum protein electrophoresis, nd urine immunoelectrophoresis for Bence-Jones protein were norml. Postopertively, the ptient ws dischrged without ny complictions. She did not undergo postopertive djuvnt chemotherpy nd hs hd no recurrence in 14 months of regulr follow-up. Discussion Extrmedullry plsmcytom ccounts for only 3 to 5% of ll plsm cell diseses. These tumors my e solitry or my precede, ccompny, or follow the onset of multiple myelom. Solitry extrmedullry plsmcytom hs rrely een reported, nd its nturl history nd dignosis re uncler. Dignosis of solitry extrmedullry plsmcytom requires the exclusion of ssocited multiple myelom, which is determined y the sence of Bence-Jones protein in the urine, norml serum Fig. 1 Plin dominl computed tomogrphy. Huge tumor is present t the sigmoid colon, nd free ir (rrows) is seen round the tumor Fig. 2 Mcroscopic exmintion. A type 1 tumor is present in the sigmoid colon. The sigmoid colon is surrounded y contmintion. Arrows indicte concvity suspected the site of perfortion of the tumor

3 Kitmur et l. Surgicl Cse Reports (2018) 4:28 Pge 3 of 5 c Fig. 4 Immunohistochemicl exmintion., In situ hyridiztion shows tht most of the tumor expresses immunogloulin G nd lmd light chin mrna. IgG, immunogloulin G; λ-lc, lmd light chin. c In contrst, there is no expression of kpp light chin mrna. κ-lc, kpp light chin c Fig. 3 Microscopic exmintion., Histopthologic exmintion of the resected tumor shows diffuse prolifertion of typicl plsm cells (hemtoxylin nd eosin). c Immunohistochemicl exmintion shows CD79 stining electrophoresis, nd norml one mrrow iopsy [4]. Our present cse met these criteri. Alexiou et l. [5] reported tht extrmedullry plsmcytom most often occurs in the nsophrynx or upper respirtory trct (82.2%). Only 17.8% of cses involve the gstrointestinl trct. The stomch nd smll intestine re the most commonly involved sites in the gstrointestinl trct. Primry isolted extrmedullry plsmcytom of the colon is extremely rre, occurring in only % of cses [5]. Therefore, its clinicl fetures nd prognosis re not well known. The clinicl presenttion of extrmedullry plsmcytom of the colon is vrile nd my include dominl pin, intestinl leeding, nd dirrhe. Griel nd Svu [6] reported rre cse in which n extrmedullry plsmcytom ws found with ileocecl junction perfortion secondry to colonoscopic injury. This is the only previous report to descrie extrmedullry plsmcytom with gstrointestinl perfortion (Tle 2). In the present cse, we were unle to determine the cuse of the perfortion y pthologic exmintion. We consider tht the tumor ws necrosed nd perforted; otherwise, s the tumor grew, the intestinl internl pressure incresed, resulting in perfortion of the sigmoid colon. Postopertive chemotherpy hs no effect on the course of extrmedullry plsmcytom. Our ptient did not undergo postopertive djuvnt chemotherpy, nd

4 Kitmur et l. Surgicl Cse Reports (2018) 4:28 Pge 4 of 5 Tle 2 Well-documented cses of plsmcytom of the colon Author/yer Sex Age (yers) Loction Clinicl fetures Therpy Vsiliu nd Pop/1928 F 47 Sigmoid Anorexi, epigstric pin,? glndulr enlrgement Brown nd Lier/1939 M 57 Colon, rectum Rectl discomfort? Hmpton nd Gndy/1957 F 43 Rectum Rectl pin nd leeding Rectosigmoid resection Miller/1970 M 35 Cecum Anemi Right hemicolectomy Willim/1970 M 84 Cecum Anemi Right hemicolectomy Neilson/1972 F 82 Sigmoid Pin Resection Wing/1975 F 82 Ascending colon Pin Right hemicolectomy Shw/1976 F 47 Cecum Dirrhe Resection Stples/1977 M 61 Sigmoid Incidentl opertive finding Resection Dniel/1977 M 21 Descending colon Pin, nuse, vomiting Left hemicolectomy Allion/1977 M 61 Sigmoid None Sigmoid colectomy Adekunle/1978 M 35 Cecum Pin Right hemicolectomy Terrence/1982 F 20 Trnsverse colon Pin, rectl leeding Trnsverse colon resection Sidni/1985 M 52 Sigmoid Pin, Resection rectl leeding Rechrd/1987 M 77 Cecum Weight loss, nemi, pin, fecl occult lood Right hemicolectomy Sverio Ligto/1996 M 45 Heptic flexure of the colon Anemi Extended right hemicolectomy Hollnd/1997 M 62 Sigmoid colon Pin Sigmoid colectomy Lttuneddu/2004 M 86 Sigmoid colon Pin, rectl leeding, stheni Segmentl resection of the left colon Gupt/2007 M 42 Diffuse colon Dirrhe Sutotl colectomy Jones/2008 M 65 Sigmoid colon Dysuri, dominl pin Sigmoid colon resection Jone/2008 M 57 Sigmoid colon Ftigue, melen Hrtmnn resection Doki/2008 M 64 Ascending colon Pin Right hemicolectomy, lymph node dissection, excision of Gerot s fsci, prtil resection of the posterior portion of the liver Colldo Pcheco/2009 M 74 Right colon Dirrhe, pin, rectl? leeding Kodni/2011 M 42 Sigmoid Fecl occult lood Endoscopic sumucosl resection Nkgw/2011 F 84 Cecum nd rectum Medicl exmintion Endoscopic sumucosl resection Lee/2013 M 45 Trnsverse colon Pin Extended left hemicolectomy Zihni/2013 M 54 Descending colon Pin nd wekness Left hemicolectomy nd smll intestinl resection Hn/2014 M 49 Trnsverse colon Pin Left hemicolectomy Emmnuel/2014 M 62 Cecum Perfortion during Right hemicolectomy dignostic colonoscopy Prnel/2015 F 72 Right colon Ftigue, light-hededness, dyspne, drk stool F femle, M mle Right hemicolectomy Distl ilel resection

5 Kitmur et l. Surgicl Cse Reports (2018) 4:28 Pge 5 of 5 she hs hd no relpse to dte. However, creful followup is required. Becuse primry isolted extrmedullry plsmcytom in the colon is very rre, the clinicl course, tretment guidelines, nd prognosis remin uncler. Further study of the clinicl fetures of primry isolted extrmedullry plsmcytom of the colon is necessry to ensure tht dequte tretment is dministered. Conclusions We hve descried rre cse of extrmedullry plsmcytom of the gstrointestinl trct with perfortion of the sigmoid colon. In this cse, the prognosis ws good ecuse of pproprite tretment involving erly surgery. Acknowledgements The uthors would like to thnk Angel Moren, DVM, ELS, from Ednz Group ( for editing drft of this mnuscript. Funding This study did not receive ny specific grnt from funding gencies in the pulic, commercil, or not-for-profit sectors. Authors contriutions FK wrote the finl mnuscript nd performed the literture serch. KD supervised the writing of the mnuscript. HI nd TO performed the surgery. All uthors red nd pproved the finl mnuscript. Consent for puliction Written informed consent ws otined from the ptient for the puliction of this report nd ny ccompnying imges. Competing interests The uthors declre tht they hve no competing interests. Pulisher s note Springer Nture remins neutrl with regrd to jurisdictionl clims in pulished mps nd institutionl ffilitions. Author detils 1 Deprtment of Gstroenterologicl Surgery, Noeok Hospitl, Shinkoji, Noeok, Miyzki , Jpn. 2 Deprtment of Gstroenterologicl Surgery, Grdute School of Medicl Science, Kummoto University, Kummoto, Jpn. Received: 1 August 2017 Accepted: 22 Mrch 2018 References 1. Lieross RH, H CS, Cox JD, Weer D, Delslle K, Alexnin R. Clinicl course of solitry extrmedullry plsm. Rdiother Oncol. 1999;52: Hmpton JM, Gndy JR. Plsmcytom of the gstro-intestinl trct. Ann Surg. 1957;145: Asselh F, Crow J, Slvin G, Sowter G, Sheldon C, Asselh H. Solitry plsm of the intestine. Histopthology. 1982;6: Meritt JW Jr. Plsmcytom of gstrointestinl trct. Ann Surg. 1955;142: Alexiou C, Ku RJ, Dietzfelinger H, Kremer M, Spiess JC, Schrtzenstller B, et l. Extrmedullry plsmcytom: tumor occurrence nd therpeutic concepts. Cncer. 1999;85: Griel EM, Svu M. Discovery of rre ileocecl plsmcytom. J Surg Cse Rep 2014(3):rju016. doi:

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