DUMB-BELL SHAPEDGIST OF THE OESOPHAGUS
MA Gore, Sandhya P Iyer, Ajay A Gujar, Varun Dixit, Sandeep R Baliga
Department of Surgery, LTMGH and LTMMC, Sion, Mumbai 400 022.
A 20 year old unmarried female presented with retrosternal pain since one year. On further investigations, a mass was seen arising along the thoracic oesophagus extending to the lower end of the stomach encircling the whole oesophagus. An exploratory laparotomy and thoracotomy was done and oesophagectomy was done with cervical oesophagogastrostomy. Post op recovery of patient was uneventful.
CASE REPORTA 20 yr. old female presented with chief complaint of retrosternal pain since one year. The pain was constant, dull aching and localized in nature. The pain was not associated with vomiting or dysphagia. However since 15 days, the patient had developed dysphagia to solid food. The patient had no history of haematemesis, melaena, breathlessness or haemoptysis. There was no lump in the abdomen or neck swelling. The respiratory and cardiovascular examination was also normal. The X-ray chest revealed mediastinal widening. A CT scan of the chest showed the mass to arise from the entire length of the oesophagus engulfing the entire circumference. No significant compromise of the oesophageal lumen was seen. There was no evidence of enlarged lymph nodes or any other mass lesions. A CT scan guided biopsy disclosed the tumour to be a benign spindle cell tumour. Oesophagogastroscopy showed extrinsic compression of the oesophageal lumen but no mucosal abnormality. The scope could be negotiated into the stomach. After anaesthetic workup, the patient was taken up for surgery.
Exploratory laparotomy was done first, which showed the involvement of lower oesophagus without lymphadenopathy. Frozen section of the tissue from mass confirmed the benign nature of the tumour. Thoracotomy revealed that the mass was in fact dumb-bell shaped with intervening normal oesophagus of 3 cm. As the entire length of esophagus was involved and the tumor was arising from the whole wall of the esophagus, the patient was subjected to total esophagectomy with a cervical esophagogastrostomy.
The patient had an uneventful recovery. The ICD tube was removed on the 5th postoperative day. An oral conray swallow was done on the 7th postoperative day, which did not show any evidence of leak.The histopathology report revealed a benign gastrointestinal stromal tumour (GIST). Immunohistochemistry of the tumour was done and it was reported as a low-grade gastrointestinal stromal tumour with desmin staining. On follow up of patient at 6 months, patient was found to be asymptomatic.
Stromal tumours of the gastrointestinal tract represent relatively rare lesions that are thought to arise from connective tissue elements located along the entire length of the gut.  These neoplasms are rare and account for 1% of all GI tumours.  They have been classified as GI smooth muscle tumours or stromal tumours. GISTs occur predominantly in persons over 40 years of age with an equal sex incidence. GISTs occur throughout the GIT, but are most common in the stomach (60-70%) and small intestine (30%). They are rare in oesophagus, colon and rectum.  Histologically, they may show a spindle cell or epithelioid pattern. They may present with myogen or neurogen differentiation, mixed differentiation or with morphological features, which cannot be further specified. These tumours arise from the lower 1/3rd of the oesophagus. GISTs include most tumours previously designated as leiomyoma, cellular leiomyoma, leiomyoblastoma and leiomyosarcoma.  Evaluation of malignant tumours show intra-abdominal spread or liver metastases. Immunohistochemically they are positive for vimentin, desmin, musle specific actin (MSA), alpha smooth muscle actin (SMA), chicken gizzard actin, cytokeratin, glial fibrillary acidic protein, synaptophysin, chromagramin.  Surgical resection is the mainstay of treatment. 
Fig 1 : Photograph of the tumour cut vertically showing the smooth and noninvolved mucosa
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