Myofibroblastický nádor jícnu

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Title in English Myofibroblastic tumour of the oesophagus
Authors

VACULOVÁ Jitka KUNOVSKÝ Lumír DOLINA Jiří PLÁNKA Ladislav ŠENKYŘÍK Jan ŠTĚRBA Jaroslav PAVLOVSKÝ Zdeněk EID Michal KALA Zdeněk JABANDŽIEV Petr

Year of publication 2021
Type Conference abstract
MU Faculty or unit

Faculty of Medicine

Citation
Description Introduction: Inflammatory myofibroblastic tumour of the oesophagus is a rare neoplasia that can occur in any anatomical localization, most commonly in the lungs, while oesophageal involvement is very rare. Although this tumor can occur at any age, it is most common in children and young adults. It is a tumor with intermediate risk of malignant potential and metastasizes rarely. Surgical resection is the most common choice of therapy. We present here a case report of a 13-year-old boy with myofibroblastic tumor of the esophagus who underwent surgical resection and has now been in remission for eight years. Methods. Thus, all available literature regarding the diagnosis and treatment of this rare disease was searched, both for resectable and inoperable stages. Case description and results: a 13-year-old boy was seen for a history of dysphagia of two months duration. He had no other symptoms and no weight loss was observed. A radiographic passage through the oesophagus was performed and revealed stenosis, and a CT scan revealed a 25 × 20 × 15 mm infiltration arising from the oesophageal wall. On completion of gastrofibroscopic examination, esophageal stenosis was confirmed at 20cm from the incisors, impassable for endoscope. Biopsy taken during endoscopy was unremarkable. Therefore, the patient subsequently underwent thoracotomy with collection of a perioperative biopsy, where myofibroblastic tumor of the esophagus was confirmed according to definitive histology. Radical surgical resection of the tumor was then successfully performed. The patient has been regularly dispensed with, including endoscopic follow-up, and is currently in complete remission of the disease. Conclusion. Surgical resection is the preferred method of treatment. The risk of tumor recurrence with complete surgical resection is very low. However, patients with this diagnosis should be dispensed with. For locally advanced inoperable stages, targeted molecular therapy is effective. Chemotherapy is still a treatment option for advanced cancer.
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