A Young Man with an Abnormal Chest X-ray

Abstract

Gastrointestinal stromal tumors(GIST) are an uncommon neoplasms of the GI tract. We present a rare case of esophageal GIST in a previously healthy young male. A 30 year old male presented to the ED after waking up ith cough, eigastric pain, nausea and vomiting. A chest x- ray showed upper mediastinal widening. Contrast enhanced CT scan incshowed a posterior mediastinal mass. Transbronchial needle aspiration and biopsy of a subcarinal mass was performed which revealed necrotic tissuecand was not diagnostic. Repeat biopsy with right video assisted thoracic surgery was performed. Immunohistochemistry was positive for CD117. A repeat chest x-ray 3 months later revealed resolution of previously described mass. He remained well until 10 months after initial presentation when he presented with chest pain, back pain and dry cough. A chest CT revealed a recurrent 5 cm subcarinal density. Right thoracotomy and resection of the mass was performed. Pathology results showed GIST with high mitotic rate. Patient was started on Imatinib. Endoscopic ultrasound two months later showed a residual mass. Fine needle aspiration and biopsy was positive for GIST. He underwent esophagectomy. Histologic evaluation revealed high grade epithelioid GIST. Patient has been asymptomatic without recurrence since.Figure 1Figure 2Figure 3GISTs affect mainly older individuals. It occurs mainly in stomach and express KIT or cell surface marker cd117 in 85-90% of cases, which is a tyrosine kinase receptor. Initial patient evaluation is usually with CT scan. Radical surgery is the treatment of choice in primary resectable GISTs. Almost all are associated with a risk of recurrence with rate of 40-50%. Imatinib Mesylate, inhibitor of KIT Tyrosine Kinase is used as an adjuvant therapy to decrease the risk of post operative recurrence. High risk tumors have a risk of 66% recurrence, making it obvious that surgery alone is not sufficient to provide long term survival. It is also used as neoadjuvant in cases that complete surgical resection is impossible and also for reducing tumor size. Since patients with high risk GIST relapse within 2 years, the follow up should be adopted to tumor criteria. CTS in the technique of choice for follow up. Our patient is particularly unique because of the age of presentation and location of tumor. Immunohistochemistry for CD117 has a high value of specificity for GIST. Treatment is complete resection. Recurrence is mainly dependent on mitotic activity. Imatinib is a useful adjuvant.