European Annals of Otrhinolrynglog, Head and Neck dseases 131 (2014) 83-85 Elsevier Masson France EM|consulte ‘wonvem-consulte.com/en ‘Available online at ScienceDirect ELSEVIER ‘wun sciencedirect.com MASSON Original article Head and neck sarcoma: Analysis of 29 cases J. Barosa*, J. Ribeiro®, L. Afonso”, J, Fernandes‘, E. Monteiro 2x0 department. Coltrane hospitals, Pace Mae Pinta 3000 Cobra, Portus "Patol depareent, Opa nce stat Raa Dr Anti Bernardin de ea {200-072 Porta Prt “ENT deparanent porta oncology institute, Rue Ando Bemardin de Aimed 3200-072 Pot, Potigal ARTICLE INFO ABSTRACT Feyworts Survival Objectives: The purpose of this study was to analyze the outcome of patients suffering from headland eck sarcomas and to identity indicators of outcome. ‘Matera and methods: The medica record oF43 patients treated between 2000 and 2010 were analyzed Al patients were suffering from primary head and neck sarcoma. The Final study sample included 25 patients, Ress: Mean survival was 56 months. Overall 2-and 5-year survivorship was 69% and 31% respectively. Parameters positively influencing survival were: male gender: non-smoker: alcohol consumption: age “T8yeats: tumor size <5em; location (nasl pyramid, jaw and maxillary sinus) Rhabdomyesarcoma and synovial sarcoma were the histological forms positively influencing prognosis. Age was the only Darameter significantly influencing survival (P<0.05). Conclusions: The present overall 5-year survivorship was similar tothe lower limit the avalable itera ‘ure data Age waste only proven indicator of outcome. inorder to have more reliable dtaitisessental to set up broader databases. (© 2014 Published by Elsevier Masson SAS. 1. Introduction grade, tumor size and depth, as well as the presence of remote or regional metastases [4 Sarcoma ofthe head and neck is very rare, representing only 1% ofall primary tumors arising within the head and neck region {1 and accounting for 410% of all sarcomas [2 Sarcomas are divided into two types: Soft tissue sarcomas and bonejearilage sarcomas [3]. Most (~80%) are of soft tissue origin, with only 20% of bony or cartilaginous origin ||. ‘They originate from mesenchymal cells and are a diverse group that arses from many different tissues, including bone, cartilage, ‘muscle, fat, blood vessels and nerves (2) Sarcomas of the head and neck are malignant tumors with a wide spectrum of histological subtypes and sites of origin, but are grouped together because of similarities in prognostic actors, clin- eal presentation, derivation from the embryonic mesoderm and overall outcome [4 ‘Although more than $0 histological subtypes have been Identi- fed, the current staging criteria used to determine treatment are universal for almost all subtypes and depend on the histological ™ Coresponding author. el: 35123940040, Ema dares: pbtesatgmalcom ( Barsa}catosibirogmsilcom (Rive rtonmogmaitcom (L- Aonso osoneteranestsmai.com (-Femmandes),cuieotmontroogiaiicom (e, Monee), pong 0.1016janor20121.007 1879-72966 204 Published by Escier Masson SAS. ‘Management ofthese neoplasms presents a great challenge [5] ‘The general concepts of sarcoma management are not univer- sally applied in head and neck, The delicate anatomy of the head {and neck limits the ability to obtain wide surgical margins. This may bbe the reason why there isa higher local recurrence rate and worse disease-specific survival in head and neck sarcomas compared t0 other sites [5] ‘The optimal treatment is complete resection (6 Due to the rarity of head and neck sarcomas in adults and the small number of treatment centers, there is not enough clinical evidence-based data in the literature to provide sufficient patient ‘numbers to identify prognostic factors or associated influences on ‘overall survival (4) ‘Asa result, information about sarcomas i scattered throughout the literature ‘There isan urgent need for reliable data [2) ‘The aim of this study i to analyze the clinical findings, manage ‘mentand survival of patients suffering from head and neck sarcoma ‘and (ofind indicators of outcome. 2, Materials and methods A retrospective study was conducted from 2000 to 2010, based ‘on analysis of 43 patients’ medical records.o |. Baran a uropeon Annas of Otorhinolaryngol, ead and Neck eases 131 (2014) 83-86 valet Sotitase Ce fae Got Sng Alsen) ee Taegan ee a a aes : Pa tea a emer St a ee ie : g 6 1 Seatmaar rors ; e a Seas eaeeee eee : P22 og ee oe eeetetae i Ea see esp att eget enecrere : eos ee eee eee Et : eee seen eee See taieg el eee eee genset Sys F Byer aa epee ere : os ; amu ot ot 2 SAase Souoees er : Seen eee eee : eee ie oe teeter Pee gee eee ae! Hl au ie" eee i 5 Hu 2 5 8 yes eee = 2 eb eR Se eee a eae : ee eerste See ese ceceeeteeer eae rye ee peice ees Rone See Gee : 73 ohh {Ray sou Races Pete : i nn eae i ee aey SRS etemeans Pee : Bont 28S inet : ete : a pe a a Oe Rea pce ane Histologically proven, sarcoma of the head and neck was the ables ential pow Fee una) as Measuring pe Insufclent information abou any of he anlyced paraneters “Hing : was the only exon enero AEE Wwehad axeisto 29 cases witncomperenformation Table), Sint q a Epierilogialparametersandpatentsurvvaiererecordea, SS i e Age alcohol consumption siaking. gender amor foaion, Roms : os treamentradioterapy, chemotherapy or surgery metastases, (ana 3 ar furor recurenceandsologcal subtypes were theparametes Mm" : a mayen ene - Ghoncrosarcoma 2 B All. statistical analyses were carried out using the 1BM- ‘qawuheaes : = ess version 180 software package (Iteration Business Sidina ts iictona I % Machines~Statistical Package for the Social Sciences - Armonk, New York, USA). Survival curves were calculated using the Kaplan-Meier method. ‘The area under the curve was calculated with 95% confidence interval 3. Results ‘The study sample included 20 males and nine females, aged between 5 and 90 years (Tables 2-5). Mean age was 45.9 +25 years. The most frequent symptoms at presentation were: neck mass (62%), epistaxis (14%) and dysphonia(14%). ‘Osteosarcoma was the most common tumor, arising in 34%0f, patients (10 cases). able Mansur dtal, Overall survival (0S Overalls * 058) fone 3 3 ‘The remaining histologically confirmed sarcomas were chab- Gomyosarcoma (5 cases, 17%], angiosarcoma, leiomyosarcoma, ‘malignant peripheral nerve sheath tumor (3 cases each, 10%),chon= Grosarcoma (2 cases, 72), and Kapos's sarcoma, synovial sarcoma and malignant fibrous histiocytoma (1 case each, 3%). “The most common locations were the maxillary sinus and upper aerodigestive tract, with 11 patients each (39%), and the face with six cases (21%), ‘Most tumors were smaller than 5 em (20 cases, 69%) ean Surv details (MS). Mean survival pe location re Mandible 4 wes engi cosa 3 2 Tongue 2 a5 Nasopharax 1 2| Bara ea Europa Amal f Otorhinolaryngol, Head and Nek dieses 11 (2014) 89-86 85 nates gical margins z, = ; at 5. Siew " fae a F 8 wl a " ns eae ae nh ae cleo Without 15 423 (vera eurvival time (Months) 77 Survival function Surgery was the most frequent treatment (24 cases, 83%), fol- lowed by radiotherapy (18 patients, 62%) and chemotherapy (14 cases, 48%). Fi: Kaplan-Meier Sea oneal survival euve (60 month}. ‘Surgical margins were negative in nine patients, close in four, postive in five and unspecified in 11 cases, ‘Mean survival was 564+ 64.3 months, ‘Two- and'- year overall survivorship (OS) was 69% and 31% respectively (Fg. 1), ‘The parameters shovring a trend toward positive impact on oe} survival were: male gender; non-smoker; positive alcohol con- sumption; age > 18 years; umor size 18 ‘rabtes ? ‘Mean sural tals (MS). Mean survival cording octher parameters oe ee we ale lw ES -EEEEEP- 449 -SPEFECPECECI TSP PEEPS PECECERS TE ‘vera uri Tne (Monte) ‘Smoking Flg.2 Kaplan-Meier age curve (0005). ‘with 4 43 ; Table? ‘cel : ie ‘Mean survival eae Sal signiance of te sated parameters Withour a 525 wae SSCS ender ee vam Mae 20 sig Cene be8 ae ecu ost <8 : see Tamer sine bi a Metwases bie Sem ° a Histology rie) Metastases i 5 m2 4, Discussion ecurreees Sarcomas of the head and neck region are aheterogeneous group ‘with 18 ns ‘of malignancies that display a wide spectrum of clinical behavior Without i 3S, iL86 |. ara ec a uropean Annas of tarhinolayngpay, Head and Neck eases 131 (2014) 83-85 Most recent publications reported series of about 50 patients ‘or fewer, and are often not comparable. To date, the two largest Published series included no more than 100 patients (2. Comparison of survival outcomes between studies is challeng- ing for several reasons ‘inclusion and exclusion criteria according to sarcoma subtype lffer from one study to another: ‘=a long study periad is required to report meaningfully on a rare disease such as head and neck sarcoma + considerable changes in sarcoma clasification as wells inimag- ing and primary and adjuvant treatment protocols may have ‘occurred since the study period; ‘+ advances in immunohistochemistry together with changes in ‘nomenclature seem to have made accurate diagnosis of head and rneck sarcoma more reliable, but new studies incorporating these ‘developments are lacking (4), The exclusion of 14 patients in the present study wasa selection bias implicit in the study design (incomplete information was the only exclusion criterion), ‘The male/female ratio found inthe literature was 1/1.1 [3]. The present series in contrast, had a clear predominance of males. Mean age was 45.9 years, which is consistent withthe literature 1378) ‘The most frequent clinical manifestations were similar to those reported in the literature: neck mass (62%). epistaxis (14%) and dysphonia (14%) [3]. ‘Osteosarcoma is the most common sarcoma in the head and neck region among adults, and rhabdomyosarcoma is the most ‘common in children |). ‘These findings are confirmed by the present study. In the literature, head and neck sarcoma is associated with 5- year overall survivorship ranging from 32% to 87% [3.911], The present study found 5-year survivorship of 31% whichis close to the lower limit ofthe literature. In some reviews, margin status, tumor grade and tumor size have been reported as prognostic factors, while in other studies no prognostic factors could be identified (4) In the present study, age was the only statistically significant variable (P<005), and therefore the only survival predictor. ‘Optimal treatment of most patients with sarcomas of the head and neck depends on a multiisciplinary approach with surgery, ‘chemotherapy and radiotherapy (|. Itisimportantthatthese patients be treated ina specialized unit ‘with a multidiseiplinary framework [2 The management of head and neck sarcomas is primarily sur- ‘ica, followed by adjuvant radiotherapy for high-grade sarcomas, large tumors and close or positive margins (4) In the present study, surgery was the most frequent treatment, {sin other head and neck cancers, primary combination treat~ ments of radiotherapy and chemotherapy have obtained increasing interest Inthe present study, patients who were treated with radiothe- rapy and chemotherapy had longer survival, Inthe literature, approximately 1010 30% of patientsexperience remote metastases [12], “The present study found remote metastases in 17% of patients. Moreover, some outcomes of the study are very difficult to explain, such as: * lower average survival in patients with negative surgical margins; «longer survival rates in patients with tumor recurrence: * longer survival rates in patients with alcohol consumption ‘These results may be due to the fact that the study had 4 smallfheterogeneous sample, and to the different degrees of agaressiveness of sarcomas. 5. Conclusion All findings were limited by the fact that the sample was small and heterogeneous. ‘This analysis showed a 5-year survival of 318, which is close to the lower limit the available data in the literature (32-87%) 4], ‘Age was the only proven indicator of outcome. ‘New studies with larger samples are needed, in order to have reliable data ‘Therefore, to identity relevant information, itis essential to set Lup prospective multicenter databases. 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