To present four rare cases of peripheral primitive neuroectodermal tumors of different sites of head and neck region. Four cases of different age (range 8-40 years) and sex (three female, one male) with rare primitive neuroectodermal tumor of sinonasal region and neck are presented. Treatment options, biological behavior and prognostic outcome are discussed herewith. Two patients succumbed to the disease within four to six months of treatment; other two patients are still under follow-up depicting the aggressive nature of the tumor. Primitive neuroectodermal tumor belongs to the class of malignant round cell tumor. Immunohistochemistry plays a pivotal role in differentiating this tumor entity. Chemoradiation was tried, but local and systemic spread occurs early and holds poor prognosis. This case series is an attempt to describe the aggressive behavior of this rare tumor with high mortality.
Synovial sarcoma is a rare tumour of head and neck. It was fi rst described by Pack and Ariel in 1950. Synovial sarcoma is said to be the commonest sarcoma of hands and feet and they are usually found adjacent to the articular surfaces. They rarely occur in extra-articular sites originating from bursa or tendon sheaths. Parapharyngeal space appears to be the site of predilection in most of the cases occurring in the head and neck region. One such case is being reported here along with its management and review of literature. Keywords Synovial sarcoma Retropharyngeal and parapharyngeal space lesion Case ReportA 17-year-old female patient presented at the ENT outdoor, Medical College and Hospitals, Kolkata, with history of gradually progressing swelling of right side of neck for last 9 months, dysphagia for 3 months and hyponasality of voice for last 2 months. There was history of weight loss during last 2-3 months.Examination of neck revealed a huge swelling of 12.5 X 7.5 cm in size occupying upper part of right anterior triangle of neck extending into the posterior triangle. The swelling was fi rm, non-tender and multinodular in feel. The skin over the swelling was free and the mobility of the swelling was restricted. The swelling was non-compressible, non-pulsatile and transillumination test was negative. No bruit was heard on auscultation. On examination of the throat, a smooth well-defi ned swelling was found, occupying right lateral pharyngeal wall pushing the tonsil medially. The swelling also extended to right side of posterior pharyngeal wall. Mucosa over the swelling was normal. The swelling was bimanually palpable. Indirect laryngoscopic examination suggested extension of the swelling to the right pyriform fossa though the lower extent could not be visualised properly. Both vocal cords were mobile. Routine blood and urine investigations were within normal limits. CT scan (Fig. 1) revealed a fairly large oblong soft tissue mass lesion in retropharyngeal plane on the right side of neck deep to the sternocleidomastoid, extending from base of skull above to the C 6 vertebral level below, having calcifi cation in walls in places with bony scalloping in underlying transverse process of C 1 .A neck exploration was performed by a modifi ed Conley's incision. The mass was found adherent to carotid sheath and prevertebral muscle, extending into opposite retropharyngeal space and above up to the base of skull. The whole mass was removed in pieces. The vagus nerve was sacrifi ced during the procedure. Neck wound was closed in layers. Postoperative period was uneventful except for hoarseness of voice.The histology of the excised mass showed a highly cellular tumour with biphasic pattern. Gland like spaces lined by epithelial cells were present amidst round to oval spindle shaped cells of small size (Fig. 2). The biphasic pattern was accentuated on reticulin stain. The histological picture suggested the diagnosis of poorly differentiated synovial sarcoma.
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