gms | German Medical Science

In medullary thyroid carcinoma, surgery is the only validated therapeutic option. We report the case of a 12 year old girl with a metastatic and locally advanced medullary thyroid carcinoma. At the time of presentation, the patient had severe obstructive respiratory failure due to a thyroid tumor which had grossly infiltrated a long tracheal segment, both recurrent nerves and the esophagus. Furthermore, large bilateral cervical lymph node metastases reached up to the base of the skull. In July 2004 we performed a multivisceral resection of the thyroid including a 4cm segment of the trachea, the recurrent nerves, the anterior esophageal wall and an extended radical bilateral neck dissection. Additionally, a tracheostomy and a gastrostomy had to be created. Macroscopically, the tumor could cmpletely resected. Histopathological staging revealed a medullary thyroid carcinoma UICC IV (pT4, pN1 (56/82), L1, V0, M0, R1). The girl was included in an experimental study protool of a combined high dose radio-chemotherapy. She has been followed up in 3 monthly intervals and has no evidence of residual disease or recurrence today, 18 months after surgery. With view to her permanent functional impairments we will discuss whether this ultra-radical surgical approach is justifiable.