Giant carotid chemodectoma treated with a combination of surgery and CyberKnife radiotherapy: a case report and review of the literature.

BACKGROUND: Paragangliomas are rare vascular neuroendocrine tumors that develop in the extra-adrenal paraganglion tissue. They occur most commonly at the carotid bifurcation, where they are known as carotid body tumors. Most paragangliomas are benign, locally aggressive, infiltrative tumors. Approximately 10% of patients with paragangliomas develop distant metastases, 10% present with multiple or bilateral tumors (mostly carotid body tumors), and 10% have a family history of paragangliomas. The malignant transformation of carotid body tumors has been reported in 6% of cases. CASE PRESENTATION: We present the case of a 64 year-old Caucasian woman with a gigantic glomic tumor mass in the neck. Twenty years before the consultation, the patient had undergone an unsuccessful attempt to remove the mass. Over the last 3 years, the patient had felt enlargement of the mass at an increased rate, almost doubling the prior size. Angio magnetic resonance imaging showed a 9 cm paratracheal mass on the left cervical side that laterally displaced the sternocleidomastoid muscle and 2 c m of the trachea. Due to the change in the tumor behavior, the maxillofacial team at Ruber International Hospital decided to remove the tumor surgically after embolization. During the surgery the tumor was gently dissected from the carotid an removed from the carotid bifurcation uneventfully. Two small nodes adhering tightly to the internal carotid adventitia and the posterior torn hole were left in place to avoid any potentially life-threatening complications. The final biopsy confirmed the initial diagnosis of carotid body paraganglioma and showed a Ki-67 expression of 19%. Due to the aggressive growth behavior and high Ki-67 expression of the tumor, the patient was referred to the CyberKnife Unit of Ruber International Hospital for treatment of the remaining nodes. CONCLUSIONS: The management of cervical paragangliomas is difficult and remains a challenge. Although the likelihood of tumor control is high with surgical or radiotherapeutic treatments, we currently lack consensus regarding the best treatment option. Nevertheless, in selected complex cases, such as the case we present, the combination of surgery and radiosurgery may allow complete local tumor control with minimal morbidity.

External dacryocystorhinostomy and transnasal canthopexy: new details of combined surgery.

PURPOSE: To describe the authors' modified combined surgical technique for external dacryocystorhinostomy and transnasal canthopexy. METHODS: A retrospective medical record review was performed including patients with late posttraumatic telecanthus and nasolacrimal duct obstruction treated by their combined dacryocystorhinostomy-transnasal canthopexy technique. In this technique, the bony window is extended superiorly and posteriorly further than in standard dacryocystorhinostomy, to allow pulling the canthus though the window, but at the same time not disturbing the suture of the dacryocystorhinostomy anastomosis. The lacrimal sac opening is performed under the canthal tendon, and only an anterior anastomosis is performed. Silicone intubation was performed only in cases with evidence of canalicular disease, marked sac inflammation or atrophic sac. The wires are fixed to the contralateral orbit, passed through the 2 middle holes of a 4-hole straight 1,7 mm microplate. The microplate is placed on the contralateral side to avoid in-fracture of the contralateral orbital bones from the pressure exerted by the transnasal wires. RESULTS: Combined external dacryocystorhinostomy-transnasal canthopexy surgery was performed on 13 eyelids of 11 consecutive patients for correction of medial telecanthus and nasolacrimal duct obstruction. Proper canthal position and lacrimal pathway patency were achieved in all cases after a mean follow up of 14.6 months. CONCLUSIONS: Combined dacryocystorhinostomy-transnasal canthopexy surgery with superior and posterior enlargement of the bony window avoided crossing of the wires and flaps and achieved a high success rate in the reconstruction of the lacrimal drainage pathway. This technique proved to be effective in the treatment of posttraumatic telecanthus with nasolacrimal duct obstruction.