Malignant extramucosal esophageal tumors are rare. We publish a case of an extramucosal esophageal tumor which turned out to be a yolk sac tumor. This type of tumor is extremely rare. We present the initial and also late management of this case (from the diagnostic to the management of the late complications). The patient was admitted for dysphagia. Contrast thoraco-abdominal CT scan revealed an important retrocardiac mass; echoendoscopy with biopsy confirmed one malignant extra-mucosal esophageal tumor. We performed an esophagectomy through triple approaches (McKeown) â?" right thoracotomy, laparotomy, and left cervicotomy for anastomosis. The pathological report raised the suspicion of a mediastinal tumor with germ cells or a poorly differentiated carcinoma. The immunohistochemistry tests confirmed a yolk sac tumor. The patient was referred to the oncologist and was treated with etoposide and cisplatin chemotherapy. Subsequently, the patient developed respiratory failure secondary to giant hiatal hernia which was surgically treated via laparoscopy.
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