Cellular Myxoma of the Vocal Cord: A Case Report and Review of the Literature

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1 Cse Report doi: /tjpth Cellulr Myxom of the Vocl Cord: A Cse Report nd Review of the Literture J. Fernndo VAL-ERNAL 1, Mrí MARTINO 2, M. Yolnd LONGARELA 3 1 Pthology Unit, Medicl nd Surgicl Sciences Deprtment, University of Cntbri nd IDIVAL, Sntnder, Spin 2 Antomicl Pthology Service, Mrqués de Vldecill University Hospitl, Medicl Fculty, University of Cntbri nd IDIVAL, Sntnder, Spin 3 Er, Nose, nd Throt Service, Mrqués de Vldecill University Hospitl nd IDIVAL, Sntnder, Spin ASTRACT Myxoms re rre in the vocl cords. A 69-yer-old mn ws dmitted with one-yer history of progressive dysphoni. Lryngoscopy reveled polypoid mss on the right vocl cord. The dignosis ws cellulr myxom. A review of the literture including the present cse reveled eleven reported cses of myxom. Ten cses were clssic myxom. To the best of our knowledge, cellulr myxom hs not been previously reported in the vocl cord. Hypercellulrity does not ffect the behvior of cellulr myxom. However, its recognition is importnt to prevent confusion with the group of low-grde myxoid srcoms. Cellulr myxom should be considered in the differentil dignosis of ny vocl cord mss. Key Words: Lrynx, Vocl cord, Myxom, Cellulr myxom, Myxoid srcom INTRODUCTION Clssic myxom is benign mesenchyml pucicellulr tumor chrcterized by blnd spindle nd stellte shped cells embedded in hypovsculr, bundnt loose myxoid strom. The cellulr vrint of this tumor shows hypercellulrity, more numerous collgen fibers, nd incresed vsculrity (1,2). Myxoms of the lrynx re very uncommon. The reported sites of involvement re the vocl cords, the ryepiglottic fold, nd the epiglottis. They re more common in the vocl cords. As fr s we re wre, only ten cses of vocl cord myxoms hve been previously reported. We describe herein cse of the cellulr vrint of myxom in the right vocl cord nd review the literture. To the best of our knowledge, cellulr myxom (CM) in vocl cord hs not been previously reported. CASE REPORT A 69-yer-old mn ws dmitted to the hospitl with one-yer history of progressive dysphoni. There ws no ssocited pin, stridor, hemoptysis or weight loss. He ws currently consuming 40 g of lcohol dy nd hd quit smoking 20 yers go. The ptient ws dignosed with polyp of the right vocl cord 20 yers go, but he refused the surgicl tretment. Medicl history ws lso significnt for tril fibrilltion with multiple embolisms in the vertebrobsilr rtery, brchiocephlic trunk, nd mesenteric rtery nd chronic heptic disese with thrombosis of the right portl vein. Syndromic ssocitions were not present. Flexible lryngoscopy reveled lrge polypoid lesion on the right cord with preserved mobility. The ptient underwent phonosurgery under generl nesthesi. The specimen consisted of glistening white, geltinous, polypoid mss mesuring 0.9 x 0.6 x 0.3 cm. Histopthologicl exmintion reveled n excrescent tissue frgment consisting of squmous mucos tht ws prtilly trophic nd mesenchyml neoplsm (Figure 1A). The tumor showed spindled nd stellte cells suspended in bckground of loose myxoid mtrix. Cell density ws vrible throughout the tumor with hypercellulr (Figure 1) nd hypocellulr res (Figure 1C). Hypercellulr res occupied bout 90% of the tumor. In these res, there were more numerous blood vessels nd collgen fibers (Figure 1D). In ddition, occsionl thick-wlled vessels with smooth muscle in their wlls were present (Figure 2A). Tumor cells were uniform nd blnd in ppernce (Figure 2). They showed smll hyperchromtic nuclei with scnt tpering eosinophilic cytoplsm (Figure 2C). Scttered muciphges were lso observed. Fluid-filled microcystic spces were seen occsionlly. Cellulr pleomorphism, multinucleted gint cells, mitoses, or necrosis were not present. The myxoid mtrix stined positive with Alcin blue t ph 2.5 (Figure 2D). Immunohistochemicl study Received : Accepted : Correspondence: J. Fernndo VAL-ERNAL Pthology Unit, Medicl nd Surgicl Sciences Deprtment, University of Cntbri, SANTANDER, SPAIN E-mil: pvbj@humv.es Phone: , ext:

2 VAL-ERNAL JF et l: Vocl Cord Myxom reveled diffuse positivity for vimentin (Figure 3A) nd focl positivity for CD34 (Figure 3) in the constituent cells. These cells were not rective for S100 protein, neurofilment protein, epithelil membrne ntigen, cludin-1, GLUT1, smooth muscle ctin nd MUC4. Ki-67 lbeled only few nuclei of the squmous epithelium. The deep surgicl border ws very close to the tumor boundry. The ptient ws dischrged in hours. One month lter his voice ws much improved. No signs of recurrence were observed. C 2 DISCUSSION Pure myxoms re very infrequent in the vocl cords. A review of the literture reveled only ten previously reported cses (Tble I) (3-12). Men ptient ge is 58.2 yers (SD 13.5; rnge yers). The tumor is more frequent in mles (mle: femle, 4.5:1). The min complint vries from horseness or dysphoni to dyspne (Tble I). The neoplsm cn present with life-thretening dyspne requiring trcheotomy (5,11). One cse showing sleep pne ws cured fter removl of the tumor (6). The verge D Figure 1: A) Vocl cord lesion showing squmous mucos. Submucos is occupied by cellulr myxomtous neoplsm (H&E; x100). ) Are with vrible cell density (H&E; x200). C) Hypocellulr nd hypovsculr re (H&E; x200). D) Cellulr re contining incresed collgen fibers nd vessels (H&E; x200).

3 VAL-ERNAL JF et l: Vocl Cord Myxom mximum dimeter of the tumor ws 1.03 cm (SD 0.62; rnge cm). The mjority of the lesions re locted on the right vocl cord (2:1). Ten cses were clssic myxom. The present cse ws CM. As fr s we re wre, CM hs not been reported in the vocl cord. Excision of vocl cord myxom is considered curtive. In one cse, removl of the neoplsm ws incomplete. Recurrence is possible in theory but it hs never been reported (Tble I). Cells of myxom originte from modified fibroblstic cells tht lck the bility to polymerize collgen. As n lterntive, they produce n excessive mount of glycosminoglycns giving them geltinous ppernce on gross exmintion. C The process suggests n underlying loclized error in tissue metbolism (13). CM is chrcterized by hypercellulr res tht occupy from 10 to 90% of the tumor. These foci hve incresed number of cells, more prominent vsculrity, incresed collgen content nd less extrcellulr myxoid mtrix thn clssic myxom. The hypercellulr regions re not ssocited with cytologic typi, multinucleted gint cells, mitotic ctivity, or necrosis. Vessels re cpillry-sized but occsionl thickwlled vessels with smooth muscle in their wlls cn be present. CMs usully show sprse pucicellulr res of clssic myxom with scnt cpillry-sized vessels (1,2). D Figure 2: A) Prominent vessels, some of which re thick-wlled contining smooth muscle (H&E; x200). ) Uniform nd cytologiclly blnd spindle cells in modertely hypercellulr region. Tumor cells re seprted by mucoid mtrix nd generlly do not touch one nother (H&E; x400). C) High mgnifiction ppernce of hypercellulr re. Nuclei re uniform nd pyknotic with tpered cytoplsms. Cells lck nucler typi (H&E; x400). D) Cells re suspended in bundnt mucoid mteril tht stins positively with Alcin blue t ph 2.5. A thick-wlled vessel cn be seen in the center of the imge (Alcin blue; x200). 3

4 VAL-ERNAL JF et l: Vocl Cord Myxom All the cses of CM reported out of the lrynx hve behved in benign fshion with only smll risk of locl nondestructive recurrence if not excised completely (1,2). Thus, in generl, simple complete locl excision is the dequte tretment. The min differentil dignosis includes myxoid neurofibrom, low-grde myxofibrosrcom, low-grde fibromyxoid srcom, nd myxoid liposrcom. Myxoid neurofibrom shows spindled elongted cells with tpering, wvy or bent nuclei nd ple indistinct cytoplsms embedded in bundnt myxoid bckground. Intrlesionl neurl fibers re demonstrted with neurofilment protein. esides, considerble number of cells re positive for S100 protein (14,15). CM, unlike low-grde myxofibrosrcom, does not show ny cytonucler typi nd does not hve the clssicl curviliner vsculr rchitecture, often with perivsculr increse of cellulrity (16). Low-grde fibromyxoid srcom is diffusely more cellulr nd is chrcterized by lternting myxoid nd collgenous zones contining blnd spindle cells with whorled growth pttern. It my show res of hylinizing spindle cells with gint rosettes. MUC4 immunostining hs been found to be highly sensitive nd specific for the dignosis (17). Myxoid liposrcom hs smll, blnd spindle-shped or more rounded cells, lipoblsts nd typiclly delicte plexiform or brnching chicken-wire cpillry vsculture (18). Figure 3: A) Hypercellulr region showing diffuse rectivity for vimentin (IHC; x200). ) Hypocellulr region showing focl positivity for CD34 (IHC; x200). Tble I: Vocl cord myxoms reported in literture Cse/Reference 1/[3] 2/[4] 3/[5] 4/[6] 5/[7] 6/[8] 7/[9] 8/[10] 9/[11] 10/[12] 11/Present report 4 Age (yers) / Sex 64/M 57/M 62/M 42/M 46/M 74/F 48/F 36/M 65/M 77/M 69/M Min complint Site Left Dyspne Dyspne, sleep pne oth Left Dyspne / Left Size (cm) Not reported Not reported Type Removl Recurrence Clssic Incomplete Not reported Not reported Cellulr Complete

5 VAL-ERNAL JF et l: Vocl Cord Myxom In conclusion, CM of the vocl cord is benign mesenchyml tumor tht shows foci of incresed cellulrity nd vsculrity, with presence of thick-wlled vessels, nd incresed collgen content. The recognition of this tumor is importnt to void misdignosis of ny type of lowgrde myxoid srcom. Although very rre, CM should be considered in the differentil dignosis of ny vocl cord mss to llow for dequte tretment. Surgery is considered curtive. REFERENCES 1. Nielsen GP, O Connell JX, Rosenberg AE. Intrmusculr myxom. A clinicopthologic study of 51 cses with emphsis on hypercellulr nd hypervsculr vrints. Am J Surg Pthol. 1998;22: vn Roggen JFG, McMenmin ME, Fletcher CDM. Cellulr myxom of soft tissue: A clinicopthologicl study of 38 cses confirming indolent clinicl behviour. Histopthology. 2001;39: Hdley J, Grdiner Q, Dilkes M, oyle M. Myxom of the lrynx: A cse report nd review of the literture. J Lryngol Otol. 1994;108: Tsunod K, Nosk K, Housui M, Murno E, Ishikw M, Immur Y. A rre cse of lryngel myxom. J Lryngol Otol. 1997;111: Kim KM, Kim SC, Jeong HJ, Kie JH. Myxom: Life-thretening benign nonepithelil tumor of the lrynx. Yonsey Med J. 1997;38: Orliguet O, Pépin JL, Vele D, Kelkel E, Pinel L, Lévy P. Hunter s syndrome nd ssocited sleep pne cured by CPAP nd surgery. Eur Respir J. 1999;13: Idrees MT, Hessler R, Terris D, Mixson C, Wng Y. Unusul polypoid lryngel myxom. Mt Sini J Med. 2005;72: Nkmur A, Iguchi H, Kusuki M, Ymne H, Mtsud M, Osko S. Lryngel myxom. Act Otolryngol. 2008;128: Ali S, McDougll G, Wllce W. Myxom-rre lryngel presenttion. Internet J Otorhinolryngol. 2008;11: Song YS, Jng S-H, Min KW, N W, Jng SM, Jun YJ, Pik SS. Myxom of the lrynx presenting s nodule. Koren J Pthol. 2008;42: Singh, Uddesh SK. Lryngel myxom: Emergency mngement. Ntl J Med Res. 2014;4: Ritchie A, Youngermn J, Fntsi JE, Khn L, Cocker RS. Lryngel myxom: A cse report nd review of the literture. Hed Neck Pthol. 2014;8: Weiss SW, Goldblum JR. Enzinger nd Weiss s soft tissue tumors. 4 th ed. St. Louis, Missouri: Mosby Inc; Liu J, Wong CF, Lim F, Knglingm J. Glottic neurofibrom in n elderly ptient: A cse report. J Voice. 2013;27: Mevio E, Glioto P, Scelsi M, Re P. Neurofibrom of vocl cord: Cse report. Act Otorhinolryngol elg. 1990;44: Nishimur G, Sno D, Hnshi M, Ymnk S, Tnigki Y, Tguchi T, Horiuchi C, Mtsud H, Mikmi Y, Tsukud M. Myxofibrosrcom of the hypophrynx. Auris Nsus Lrynx. 2006;33: Cown ML, Thompson LD, Leon ME, ishop JA. Low-Grde fibromyxoid srcom of the hed nd neck: A clinicopthologic series nd review of the literture. Hed Neck Pthol. 2016;10: Kubiczkow J, Gerwtowsk W. Myxoid liposrcom of the lrynx. Otolryngol Pol. 1997;51 Suppl 25: