Granular cell tumors: a report of six cases

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1 Aoym et l. World Journl of Surgicl Oncology 2012, 10:204 WORLD JOURNAL OF SURGICAL ONCOLOGY CASE REPORT Grnulr cell tumors: report of six cses Kei Aoym *, Tkko Kmio, Azus Hirno, Akiyoshi Seshimo nd Shingo Kmeok Open Access Astrct Grnulr cell tumor is soft tissue neoplsm tht origintes in the nervous system nd rises t virtully ny ody site, ut is minly found in the skin, orl cvity or digestive trct. Most re enign nd reportedly mlignnt cses re rre, occurring in only 1% or 2% of cses. We report on our findings in six ptients who developed grnulr cell tumor in the mmmry glnd, esophgus, sucutneous tissue nd muscle. Of six ptients, two hd grnulr cell tumor locted in the rest, two in the sumucos of the esophgus, nd the other two under the skin of the left xillry cvity nd in the right ltissimus dorsi muscle, respectively. One of the two ptients with tumor in the sumucos of the esophgus lso hd esophgel cncer. Ptients gerngedfrom41to70yers(verge,59.1yers). Two ptients with tumor in the sumucos of the esophgus were men, nd the others were women. All of them were given dignosis of grnulr cell tumor y tissue iopsy nd exmintion of excised specimens, ut no evidence of mlignncy ws found. No recurrence hs een noted in the ptients who underwent surgicl tumor removl. Keywords: Grnulr cell tumor Bckground Grnulr cell tumor is soft tissue neoplsm tht origintes in the nervous system nd rises t virtully ny ody site, ut is minly found in the skin, orl cvity or digestive trct [1-3]. Most of them re enign nd reportedly mlignnt cses re rre, occurring in only 1% or 2% of cses [4-7]. We report on our findings in six ptients who developed grnulr cell tumor in the mmmry glnd, esophgus, sucutneous tissue nd muscle. Cse presenttion Ptient 1 A 63-yer-old womn. After n nomly ws detected y mmmogrphy (MMG) in medicl check-up t hospitl, she ws referred to our hospitl for further exmintion. No mjor diseses were recorded in her pst history. By plption, tumor with good motility nd smooth surfce wsfeltontheouterqudrntoftherightrest.mmg reveled tumor shdow with spicule in the outer right prt, nd ultrsonogrphy (US) showed n irregulr shped tumorintherightcregion(figure1).needletissueiopsy did not revel mlignnt cells, ut oth MMG nd US yielded findings indicting the presence of rest cncer (invsive ductl crcinom); therefore, lumpectomy ws * Correspondence: k-oym@surg2.twmu.c.jp Deprtment of Surgery II, Tokyo Women s Medicl University, 8-1, Kwd-cho, Shinjuku-ku, Tokyo , Jpn performed. Histopthologic exmintion confirmed the dignosis of grnulr cell tumor (Figure 2). Ptient 2 A 41-yer-old womn. After n nomly ws detected y MMG in medicl check-up t hospitl, she ws referred to our hospitl for further exmintion. No mjor diseses were recorded in her pst history. By plption, n indurtion ws felt on the inner upper qudrnt of the left rest. US reveled n irregulr shped tumor in the left A region (Figure 1). Becuse the imges suggested the presence of rest cncer, needle tissue iopsy ws performed. Histopthologic exmintion reveled grnulr cell tumor. Ptient 3 A 70-yer-old mn cme to our hospitl to hve surgery for esophgel cncer nd underwent detiled exmintion. There were no significnt diseses or symptoms in his medicl history. Upper gstrointestinl endoscopy reveled sumucosl tumor (SMT) t position elow the esophgel cncer lesion (Figure 3). Histopthologic exmintion of the resected specimen fter esophgel cncer surgery yielded dignosis of grnulr cell tumor (Figure 2) Aoym et l.; licensee BioMed Centrl Ltd. This is n Open Access rticle distriuted under the terms of the Cretive Commons Attriution License ( which permits unrestricted use, distriution, nd reproduction in ny medium, provided the originl work is properly cited.

2 Aoym et l. World Journl of Surgicl Oncology 2012, 10:204 Pge 2 of 6 Cse 1 Cse 2 Figure 1 () Cse 1. Ultrsonogrphy reveled tumor 8 9 mm in size with irregulr orders nd rupture of the nterior order of the mmmry glnd. Mmmogrphy reveled tumor with irregulr orders nd spicule. () Cse 2. Ultrsonogrphy reveled tumor 7 8 mm in size with irregulr orders nd rupture of the nterior order of the mmmry glnd. Ptient 4 A 67-yer-old mn. Upper gstrointestinl endoscopy showed SMT in the esophgus t position out 30 cm from the incisor tooth (Figure 3), nd tissue iopsy ws performed. Histopthologic exmintion yielded dignosis of grnulr cell tumor. Ptient 5 A 60-yer-old womn felt tumor-like lump in the nterior xillry line outside of the right rest, nd visited our hospitl. MMG nd US did not disclose ovious evidence of tumor in the rest, ut reveled n ovl-shped, low-echoic tumor in the ltissimus dorsi muscle (Figure 4). Histopthologic exmintion fter lumpectomy yielded dignosis of grnulr cell tumor (Figure 2c). Ptient 6 A 54-yer-old womn felt tumor-like lump in the left xillry cvity, nd visited our hospitl. US reveled n ovl-shped, low-echoic tumor under the skin in the left xillry region (Figure 4). Lumpectomy ws performed nd grnulr cell tumor ws dignosed y postopertive histopthologic exmintion (Figure 2d). Of six ptients, two hd grnulr cell tumor locted in the rest, two in the sumucos of the esophgus, one under the skin of the left xill nd one within the right ltissimus dorsi muscle. One of the two ptients hving tumor in the sumucos of the esophgus lso hd esophgel cncer. The ptients ge rnged from 41 to 70 yers (verge, 59.1 yers). Two ptients hving tumor in the sumucos of the esophgus were men, nd the others were women. All of them were given dignosis of grnulr cell tumor y needle tissue iopsy nd iopsy of excised specimens, ut no evidence of mlignncy ws found (Figure 2-d). No recurrence hs een noted in ptients who underwent surgicl tumor removl (Tle 1). A grnulr cell tumor tht hs occurred in the rest is often dignosed to e invsive ductl crcinom y imging dignostic techniques, nd ptients in such cse showed spicule on MMG nd n irregulr shped tumor on US. A grnulr cell tumor tht hs developed in the sumucos of the esophgus usully ppers s non-pedunculted SMT with smooth surfce nd ple yellow color, nd oth of our two cses presented the sme chrcteristics. The incidence of grnulr cell tumor in the skin is high, ut it is very rre for it to develop in the skeletl muscle. One of our ptients ws found with grnulr cell tumor in the skeletl muscle (ltissimus dorsi muscle) (Tle 1). Discussion Grnulr cell tumor ws first reported y Arikossoff [1] y the nme of grnulr cell myolstom. One of its chrcteristics is tht eosinophilic grnules re contined

3 Aoym et l. World Journl of Surgicl Oncology 2012, 10:204 Pge 3 of 6 Cse 1 Cse 3 Cse 5 Cse 6 c Figure 2 () Cse 1. Histopthologic exmintion showed tht the tumor cells hd numerous grnules, nd immunostining reveled S-100- positive (H&E stining, 100). () Cse 3. Histopthologic exmintion showed sumucosl tumor consisting of cells contining eosinophilic grnules, nd immunostining reveled S-100-positive. The tumor ws not metstsis of esophgel cncer, nd hd no signs of mlignncy (H&E stining, 1). (c) Cse 5. Histopthologic exmintion reveled tumor in the strited musculr tissue. The tumor cells possessed numerous eosinophilic grnules, nd the tumor itself consisted of dense prolifertion of lrge tumor cells (H&E stining, 200). d: Cse 6. Histopthologic exmintion reveled eosinophilic grnules inside the cell odies. The cell nucleus ws ovl, nd the nucleoplsmic rtio ws low (H&E stining, 400). d in the cytoplsm of cells. Since the positive rtes for S-100 protein nd neuron specific enolse re high, currently it is thought tht this tumor origintes from Schwnn cells. They ccount for n incidence of 0.5% mong soft tissue tumors, nd it hs een reported tht they primrily occur in or eneth the skin, or in the sumucos of the tongue, throt, chest wll, or ronchus. The tumor is commonly found in those ged 10 to 50 yers old, nd more often in women thn in men [3,8-11]. Grnulr cell tumor of the rest is lrgely disese ffecting women, s it is with other rest mlignncies, ut hs lso een descried in men, ccounting for 6.6% of grnulr cell tumor of the rest [12]. Aout 2% of ll grnulr cell tumor cses re mlignnt [4-7]. A tumor 3 cm or less in size cn e regrded s enign. However, if the tumor grows rpidly nd forms n ulcer, mlignncy should e suspected. From histopthologic perspective, Fnurg Smith nd collegues [5] proposed the following six criteri to determine whether tumor is mlignnt or not: (1) the presence of necrosis, (2) the emergence of spindle cells, (3) vcuolr nucleus with n enlrged nucler ody, (4) increse in nucler division (2 mitoses/10hpf), (5) increse in the nucleoplsmic rtio, nd (6) polymorphism. If none of these dignostic criteri re met, the tumor is considered to e enign. If one or two criteri re met, the tumor is considered to e typicl, nd if three or more criteri re met, the tumor is considered to e mlignnt. Sonoe nd collegues [6] divided mlignnt grnulr cell tumors into two groups: those tht re mlignnt oth histologiclly nd cliniclly, nd those tht re histologiclly enign ut cliniclly mlignnt. The most common metstsis sites of mlignnt grnulr cell tumor re the lymph nodes, followed y the lungs.

4 Aoym et l. World Journl of Surgicl Oncology 2012, 10:204 Pge 4 of 6 Cse 3 Cse 4 Figure 3 () Cse 3. Upper gstrointestinl endoscopy suggested esophgel cncer, nd reveled sumucosl tumor of yellowish white t position elow the cncer. Totl thorcic esophgectomy ws performed nd the sumucosl tumor ws lso removed s comined resection. The mcroscopic dimeter of the sumucosl tumor ws out 9 mm. () Cse 4. Upper gstrointestinl endoscopy performed for screening purpose reveled trpezoidl protruded lesion of yellowish-white t position 30 cm from the incisor tooth. Insufficient tumor resection often results in locl recurrence, nd hs tendency to spred oth lymphogenously nd hemtogenously. Reportedly, chemotherpy nd rdiotherpy tretments cnnot e expected to e effective, with surgicl resection eing the primry option [4-7]. Resection with dequte mrgins is necessry ecuse the tumor hs no cpsule nd is prolifertion invsive. All tumors in our six cses were smll in dimeter, nd enign histologiclly (Tle 1). In mny cses, grnulr cell tumor tht hs occurred in the rest is dignosed to e invsive ductl crcinom y imging, nd our ptients with such condition lso showed spicultion on MMG imges [13-18]. The MMG nd US ppernces of grnulr cell tumor pose dignostic dilemm ecuse of its similrity to rest mlignncy. A grnulr cell tumor tht hs developed in the sumucos of the esophgus is non-pedunculted SMT with smooth surfce nd ple yellow color, nd oth of our two cses presented the sme chrcteristics [19-22]. Grnulr cell is uncommon, minly occurring on the skin, tongue nd orl cvity s single nodule. The disese in 30% to 45% of cses ffects the skin, followed y the re of the hed nd neck, where the most frequent loction is introrl in the tongue nd the soft nd hrd plte [11,19-24]. Other loctions ffected re the rest, the gstrointestinl trct, the respirtory trct, the thyroid glnd, the urinry ldder, the centrl nervous system, nd femle genitli. Loction in the skeletl muscle region, s in our cse, is rre. One of our ptients ws found with grnulr cell tumor in the skeletl muscle [25]. In the gstrointestinl trct, the tumor often occurs in the esophgus nd is rrely found in the lrge intestine [19-22]. Grnulr cell tumor tht hs developed in the gstrointestinl trct must e differentited from other sumucosl tumors such s stetom, smooth muscle tumor, neurogenic tumor nd gstrointestinl stroml tumor. On endoscopic exmintion, the tumor is very hrd, smooth-surfced sumucosl tumor which ppers s yellow or yellowish-white hemisphericl protrusion with thin mucous memrne, nd is sometimes clled "molr tooth" or "sweet corn." It is rre tht ulcerous lesions or recesses re oserved on the mucosl surfce [19-22]. For differentil dignosis of grnulr cell tumor in the sucutneous tissue nd muscle, cndidtes include: mlignnt firous histiocytom, lveolr soft prt srcom, desmoid, grnulomtous, nd nodulr fsciitis [7,23-25]. Grnulr cell tumor of the rest rises from intrloulr rest strom nd occurs within the distriution of the cutneous rnches of the suprclviculr

5 Aoym et l. World Journl of Surgicl Oncology 2012, 10:204 Pge 5 of 6 Cse 5 Cse 6 Figure 4 () Cse 5. Ultrsonogrphy reveled n irregulrly ordered, low echoic tumor tht protruded in convex shpe from the intrinsic muscle lyer of the ltissimus dorsi muscle. The size ws mm. () Cse 6. Ultrsonogrphy reveled smoothly ordered tumor with posterior echo ttenution, hving heterogeneous content nd mm in size. Tle 1 Clinicopthologic findings in grnulr cell tumor cses Cse Age Sex Site Dimeter Dignosis 1 63 Femle Mmmry glnd 9 mm Benign 2 41 Femle Mmmry glnd 8 mm Benign 3 70 Mle Esophgus 9 mm Benign 4 67 Mle Esophgus 6 mm Benign 5 60 Femle Skeletl muscle (ltissimus 14 mm Benign dorsi muscle) 6 54 Femle Axillry cvity (sucutneous tissue) 18 mm Benign nerve [17,18]. When occurring in the rest, the tumor is often found in the upper inner qudrnt unlike rest cncer tht is found in the upper outer qudrnt [17,18]. Since the tumor ffects the innervtion of the skin, contrctions or shrinkge of the skin sometimes occur. Trditionl imging techniques, including MMG nd US scnning, re widely employed when investigting the presence mss. However, the issue with these modlities in cses of grnulr cell tumor is tht the rdiologicl findings re often indistinguishle from rest cncer. On MMG, the tumor is seen s sustntil roundshped lesion with distinct edges of the hyperplsi invding into the surrounding tissues, nd irregulrity, specultion, isodensity sometimes ssocited with hypodense rims, nd heterogenicity re commonly oserved (Figure 1). Mss clcifiction ws not oserved in our cses. On the other hnd, on US, the edges of the hyperplsi re not distinct nd ttenution of posterior echo often occurs. Common fetures include solid, heterogeneous, poorly defined msses with high depth/width rtio (Figure 1). They re generlly hypo-echoic nd disply posterior shdowing with corse internl echo nd high oundry echo. For these resons, it is necessry to perform differentil dignosis etween rest cncer nd invsive ductl crcinom sed on the imges [13-18]. The rnge of US findings re s rod s those of MMG, they re often suggestive of mlignncy nd most importntly there re no recognized fetures specific for grnulr cell tumor of the rest. Conclusion Grnulr cell tumor is neoplsm tht develops in the soft tissues, minly in the skin, orl cvity nd gstrointestinl trct, ut the tumor is reltively rre. Grnulr

6 Aoym et l. World Journl of Surgicl Oncology 2012, 10:204 Pge 6 of 6 cell tumor in ll of our cses ws enign, nd no recurrence fter resection hs een found. Consent In ccordnce with the regultions of the Humn Investigtion Committee of the Tokyo Women s Medicl University, written informed consent ws otined from the ptient for puliction of this report nd ny ccompnying imges. Arevitions MMG: mmmogrphy; SMT: sumucosl tumor; US: ultrsonogrphy. Competing interest The uthors declre no conflicts of interest with respect to uthorship nd/ or puliction of this rticle. Authors contriutions KA designed the study, reserched the literture, nd drfted the mnuscript. TK, AH, AS, nd SK prticipted in the study design nd coordintion, nd helped to collect dt. All uthors hve red nd pproved the mnuscript. Funding No extrmurl funding ws used for this study. Received: 24 Ferury 2012 Accepted: 10 Septemer 2012 Pulished: 29 Septemer 2012 References 1. Arikossoff A: Uer Myome, usgehend von der quergestreiften wilkuerlichen muskultur. Virchows Arch Pthol Ant 1926, 260: Weiss SW, Goldlum JR: Enzinger nd Weiss s Soft Tissue Tumor (fifth ed.). St Louis: Mosy; 2008: Lck EE, Worshm GF, Cllihn MD, Crwford BE, Klppench S, Rowden G, Chun B: Grnulr cell tumor: clinicopthologic study of 110 ptients. J Surg Oncol 1980, 13: Khnsur T, Blducci L, Tvssoli M: Grnulr cell tumor. Clinicl spectrum of the enign nd mlignnt entity. Cncer 1987, 60: Fnurg-Smith JC, Meis-Kindlom JM, Fnte R, Kindlom LG: Mlignnt grnulr cell tumor of soft tissue: dignostic criteri nd clinicopthologic correltion. Am J Surg Pthol 1998, 22: Sonoe H, Iwt J, Furiht M, Moriki T, Ohtsuki Y: Mlignnt grnulr cell tumor: report of cse nd review nd the literture. Pthol Res Prct 1998, 194: Ktok A, Koike M, Kurnuki K, Wkym K, Ymd S, Aksk Y: A cse of mlignnt grnulr cell tumor risen in the chest wll (in Jpnese with English strct). J Jpn Surg Assoc 2005, 66: Kne S, Wtne I, Lotuco L: Multiple grnulr-cell tumors of the scending colon: microscopic study. Dis Colon Rectum 1978, 21: Vnce SF III, Hudson RP Jr: Grnulr cell myolstom. Clinicopthologic study of forty-two ptients. Am J Clin Pthol 1969, 52: Ordóñez NG: Grnulr cell tumor: review nd updte. Adv Ant Pthol 1999, 6: Becelli R, Perugini M, Gsprini G, Cssoni A, Fin F: Arikossoff s tumor. J Crniofc Surg 2001, 12: Luwers K, Bestmn TJ, Bergmns G, Molderez C: Grnulr cell tumor of the mle rest. Act Chir Belg 2008, 108: Ohnishi H, Nishihr K, Tme K, Mitsuym S, Ae R, Toyoshim S, Ae E: Grnulr cell tumors of the rest: report of two cses. Jpn J Surg 1996, 26: Aktsu T, Koyshi H, Uemtsu S, Tmgw E, Shinozki H, Kse K, Koyshi K, Otsuk S, Muki M, Kitjim M: Grnulr cell tumor of the rest preopertively dignosed y fine- needle spirtion cytology: report of cse. Jpn J Surg 2004, 34: Aoym K, Kmio T, Ohchi T, Kmeok S: A cse of grnulr cell rest tumor (in Jpnese with English strct). J Jpn Surg Assoc 2005, 66: Tkei J, Nkmur S, Suzuki K: Grnulr cell tumor of the rest dignosed y core needle iopsy: cse report (in Jpnese with English strct). Jpn J Brest Cncer Clin 2005, 20: Ptel A, Lefemine V, Yousuf SM, Aou-Smr W: Grnulr cell tumor of pectorl muscle mimicking rest cncer. Cses J 2008, 6: Brown AC, Audisio RA, Regitnig P: Grnulr cell tumour of the rest. Surg Oncol 2011, 20: Nrr SL, Tomzzi C, Dtt V, Ismil MK: Grnulr cell tumor of the esophgus: report of five cses nd review of the literture. Am J Med Sci 2008, 335: Mekw H, Mekw T, Yuki K, Sto K, Tmzki Y, Kudo K, Wd R, Mtsumoto M: Multiple esophgogstric grnulr cell tumors. J Gstroenterol 2003, 38: Kwmur A, Nishikw S, Tnid N, Tmur K, Ohno T, Ohm I, Kno M, Shimoym T, Sno T: Grnulr cell tumor of the scending colon: cse report. Gstoroenterologi Jponic 1987, 22: Nkchi A, Miyzot H, Oshiro T, Shimoji H, Shirishi M, Muto Y: Grnulr cell tumor of the rectum: cse report nd review of the literture. J Gstroenterol 2000, 35: Hzn C, Fnqmn W: Multiple cutneous grnulr- cell tumors. Dermtol Online J 2007, 27: Gross VL, Lynfield V: Multiple cutneous grnulr cell tumors: cse report nd review of the literture. Cutis 2002, 69: Ntsume S, Hsegw H, Skmoto E, Komtsu S, Hiromtsu T, Tt T: A cse of typicl grnulr cell tumor in rectus dominis muscle (in Jpnese with English strct). J Jpn Surg Assoc 2006, 67: doi: / Cite this rticle s: Aoym et l.: Grnulr cell tumors: report of six cses. World Journl of Surgicl Oncology :204. Sumit your next mnuscript to BioMed Centrl nd tke full dvntge of: Convenient online sumission Thorough peer review No spce constrints or color figure chrges Immedite puliction on cceptnce Inclusion in PuMed, CAS, Scopus nd Google Scholr Reserch which is freely ville for redistriution Sumit your mnuscript t