JSCR 2013; 1 (3 pages) doi:10.1093/jscr/rjs049 Case Report Post radiation myofibrosarcoma of hypopharynx T.S. Korampalli*, Bipin Mathew and N.D. Stafford CastleHillHospital,Cottingham, Hull, UK *Correspondence address.Castle Hill Hospital,DaisyResearch Building, Cottingham HU16 5JQ, UK. E-mail: [email protected] Received1December2012;accepted30December2012 Malignant tumours of myofibroblasts are rare lesions. Postradiation sarcoma is a rare poten- tiallatesequelofionizingradiation.Wepresentthefirstcaseofmyofibrosarcomaofthehypo- pharynx in a 67-year-old man, occurring 16years afterradiotherapy forlaryngeal carcinoma. INTRODUCTION a polypoidal tumour measuring 50(cid:2)32(cid:2)21mm in dimen- sions. Microscopy showed spindle cells arranged in short Most malignant tumours in the hypopharynx arecarcinomas. fascicles and sheets. Individual cells showed short spindle There are some reports of hypopharyngeal sarcomas such as and elongated hyper-chromatic nuclei with mild-to-moderate angiosarcoma, liposarcoma and synovial sarcoma [1]. amountof pale to eosinophilic cytoplasm (Fig.2). Therewas Ionizing radiation is an important modality of treatment for no evidence of squamous or glandular differentiation. There head and neck cancer. However, radiation-induced sarcoma was no dysplasia within the adjacent squamous epithelium. of the head and neck is a rare long-term complication of On immunohistochemistry, diffuse staining for smooth radiation therapy. muscle actin (SMA) in a tram track pattern was noted. In We report the first case of myofibrosarcoma of the hypo- addition, a minor population of tumour cells stained for pharynx occurring 16 years after radiotherapy to a well- desmin (Fig. 3). Therewas no staining for AE1 AE3, pancy- differentiated squamous cell carcinoma of larynx. Our tokeratin, H-caldesmon, myogenin, CK 5/6, CK14, p63, objective isto highlight this rare clinicopathological entity to 34BE12, CD34, CK7, CD56, EMA, S100, melan A, c-KIT head and neck surgeons andhistopathologists. and CD99. The final diagnosis of a myofibrosarcoma FNCLCC grade 2 was made. The surgical margins werefree of tumour. CASE REPORT A 67-year-old man presented to our ENT department with DISCUSSION historysuggestive of globus feelingin the throat. The patient had received a full course of radiotherapy to the larynx for Sarcomas are malignancies derived from the mesoderm. well-differentiated T1N0M0,squamous cell carcinoma of the They account for ,1% of head and neck malignancies and larynx. There was no past history of hereditary conditions ,10% of all soft tissue sarcomas [2]. predisposing to sarcomas, including Recklinghausen’s Myofibrosarcoma was first reported in 1992 [3]. They are disease, hereditary forms of retinoblastoma and Wilms malignant tumours of myofibroblasts, with predilection for tumour. Direct laryngoscopic examination revealed a postcri- the head and neck region followed byextremities and trunk. coid tumour extending to the oesophagus. Biopsy was They occur across a wide age range (9–75 years, mean age reported as a grade 2 spindle cell sarcoma. Computed tomo- 40 years)withslight male predominance. graphic scans demonstrated a post-cricoid lesion extending Radiotherapy plays a key role in the treatment of headand into the cervical oesophagus (Fig. 1). No cervical lymph- neck malignancy. Cumulative incidence of sarcoma after ra- adenopathyor distant metastases were noted. diation therapy ranges from 0.03 to 0.3%, [4]. A significant The patient underwent a total laryngopharyngectomy proportion (16–36%) of these was malignant fibrous with flap repair. Macroscopically, the hypopharynx showed histiocytoma. PublishedbyOxfordUniversityPressandJSCRPublishingLtd.Allrightsreserved.#TheAuthor2013. ThisisanOpenAccessarticledistributedunderthetermsoftheCreativeCommonsAttributionLicense(http://creativecommons.org/ licenses/by-nc/3.0/),whichpermitsnon-commercialuse,distribution,andreproductioninanymedium,providedtheoriginalworkis properlycited.Forcommercialre-use,[email protected]. Page 2of 3 T.S.Korampalli and R.K. Bhatnagar Cahan et al. developed the first set of criteria for a et al. revised these to include other soft tissue sarcomas. sarcoma to be appropriately identified as radiation induced. Cahan et al. and Laskin et al. [5] modified these criteria: However, this applied only to bone sarcomas. Later, Murray (i) a prior historyof radiation; (ii) development of a sarcoma in the field of radiation; (iii) a latency period of at least 2 years between radiation and appearance of the sarcoma and (iv) evidence that the sarcoma is histologically different from the irradiated primary cancer. All the above criteria were satisfied in ourcase. According to recent studies, the range of radiation doses associatedwiththedevelopmentofpostradiationsofttissue sarcomasis8–100Gy,withameandosefrom40to50Gy[6]. Figure1: CTscanshowingthepostcricoidcarcinomaextendingdown toupperoesophagus. Figure2: Scanningmagnification. Figure3: (cid:2)5and(cid:2)10magnificationshowsacellulartumourwithshorttoelongatedcellswithscantinterveningstroma. Postradiationmyofibrosarcoma Page 3of 3 The latency interval has ranged from 2 to 65 years, but likely to increase due to efficacyof current cancer therapies the mean latency is 15 years [6]. Sheppard and Libshitz andprolongedsurvival.Ourcasereportsuggeststhatpatients foundthelatencyintervaltobeinverselyrelatedtotheradi- treated withthe radiotherapy for head and neck malignancies ationdose[7]. should be followed up carefullyas early diagnosis and treat- Post radiation sarcomas are aggressive, with a high inci- ment atan earlystage is crucial to sarcomas. dence of local recurrence and distant metastasis [8]. A sur- vival rate of 29% after 5 years was reported by Wiklund et al. [9]. Surgical resection is the preferred treatment when REFERENCES possible, on the basis of the site and sizeof tumour. 1. Nouri H, Hassani R, Aderdour L, Raji A. The well-differentiated Histopathologically myofibrosarcomas are characterized liposarcomaofthehypopharynx.EurAnnOtorhinolaryngolHeadNeck byacellular proliferation of spindle-shaped cells arranged in Dis2011;128:143–5. intersecting fascicles and sheets within scant collagenousto 2. 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