Extracranial nasopharyngeal craniopharyngioma : Case report

• Published in: Neurosurgery Vol. 60; no. 4; pp. 780 - 781
• Main Authors: SHUMAN, Andrew G, HETH, Jason A, MARENTETTE, Lawrence J, BLAIVAS, Mila, MURASZKO, Karin M
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 01-04-2007; Wolters Kluwer Health, Inc
• Subjects: Biological and medical sciences; Case reports; Child; Craniopharyngioma - diagnosis; Craniopharyngioma - surgery; Humans; Magnetic resonance imaging; Male; Medical sciences; Nasopharyngeal Neoplasms - diagnosis; Nasopharyngeal Neoplasms - surgery; Neurosurgery; Pediatrics; Pituitary Neoplasms - diagnosis; Pituitary Neoplasms - surgery; Radiation therapy; Sleep apnea; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgical techniques; Treatment Outcome; Tumors; Endocrinopathy; Human; Intracranial; Sleep apnea syndrome; Nervous system diseases; Cranial base neoplasm; Respiratory disease; Nasopharynx; Extracranial; Obstructive sleep apnea; Case study; Surgery; Nasopharyngeal; Tumor; Craniopharyngioma; Child
• ISSN: 0148-396X; 1524-4040
• DOI: 10.1227/01.NEU.0000255411.69022.A1

Craniopharyngiomas (CPs) are benign tumors that almost always occur in a suprasellar location, making complete resection difficult and often necessitating radiotherapy. A case of CP presenting in an unusual location in an 8-year-old boy highlights the goals of CP treatment.
An 8-year-old boy sought treatment for symptoms of nasal obstruction and snoring. He also had a history of mild developmental delay, and his father had a thyroglossal duct cyst resected in his own youth. After tonsillectomy and adenoidectomy failed to improve the patient's symptoms, nasal endoscopy and biopsy revealed an intranasal CP. After this treatment, he experienced chronic thin brown nasal discharge. Magnetic resonance imaging further revealed tumor invading the sphenoid body and the clivus and that the tumor had no intracranial extension.
The child underwent surgical resection via a Le Fort I osteotomy approach. Complete resection was accomplished based on intraoperative frozen section pathological examination and postoperative magnetic resonance imaging results.
CP with no intracranial extension is a very rare but benign tumor. We recommend vigorous attempts to resect such tumors completely to minimize the chance of recurrence and the possible need for radiotherapy. Although radiotherapy controls CP growth quite well, it has its own risks that should be avoided if possible. Cranial base techniques may facilitate total resection.