Giant rare esophageal malignancy in adolescent boy: a case report

Abstract

Synovial sarcoma is rare soft tissue tumour affecting mostly extremities and less commonly head and neck, lungs, heart and digestive system in adult age group. Only about 20 such cases arising from oesophagus have been reported in literature but none have been reported in adolescent age group with large size in Indian population. So here we present a case report of synovial sarcoma of oesophagus seen in a 14-year-old boy and successful management. Our patient was a 14-year boy who came to our outpatient department (OPD) department with complaints of dysphagia to and retrosternal pain from past 2 months. A contrast enhanced computed tomography (CT) scan showed vertically oriented long and large hypodense lesion arising from right anterolateral wall of pharyngoesophageal junction to upper two-thirds of esophagus from C6 to D8 causing >50% narrowing of trachea. Upper gastrointestinal (GI) endoscopy was done which showed linear submucosal lesion from 17 to 28 cm from incisors. After anaesthetic clearance patient was planned and taken up for minimally invasive esophagectomy. Intraoperatively bulky submucosal growth extending from just cricopharynx to lower thoracic esophagus till inferior pulmonary ligament. Histopathology report came out as synovial sarcoma spindle cell type with Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade 2. After tumor board discussion, adjuvant therapy with doxorubicin and ifosfamide was given for five courses. After Six months follow up, the boy is free from tumor recurrence. Synovial sarcoma in esophagus is very rare with nearly 20 reported cases, of which most were polypoid and located in upper and mid esophagus and biphasic histology. The treatment for patients with localized disease is surgery with an adequate wide margin combined with adjuvant chemotherapy and/or radiation is regarded as the preferable treatment. Esophagectomy mostly with minimally invasive approach is preferred surgical option. Robotic minimally invasive surgery is also a safe and feasible option. Clinical follow-up has been limited, and synovial sarcoma is widely known to recur or metastasize after long disease-free intervals.