Occurrence of Rhabdomyosarcoma After Surgery Combined with oral Sirolimus for Mixed Vascular Malformation of the Tongue.

Abstract

Vascular malformation is the general term of a kind of lesions originated from lymphatic vessels and vascular tissues, which contains a variety of components called mixed vascular malformation. Rhabdomyosarcoma (RMS) is a kind of soft tissue sarcoma, originating from striated muscle cells or mesenchymal cells. RMS and vascular malformation mostly occur in children, and common in the head and neck, but their simultaneous occurrence is rare. A 9-year-old boy who was hospitalized for a second attack of combined vascular malformation: hemolymphangioma. The child experienced severe upper airway obstruction and tongue bleeding. Postoperative pathology demonstrated hemolymphangioma combined with RMS. Subsequently, he was transferred to the oncology department for chemotherapy and lately died of RMS with lung metastasis. The secondary RMS may be related to the usage of sirolimus. Because of its uncertain border, vascular malformation in the oral and maxillofacial region is difficult to completely remove by surgical resection, and local recurrence could be often observed. Due to its rapid progress and continuous bleeding, the possibility of malignant tumor should be considered and multidisciplinary comprehensive treatment should be actively taken. Besides, family history of related malignant tumors and immune function should be investigated in detail before choosing the application of oral sirolimus.