Craniopharyngioma

CASE HISTORY

In June 1992 at the age of 4 years, a Hispanic boy underwent placement of a right ventriculoperitoneal (VP) shunt in Mexico followed by placement of a left VP shunt in July 1992. He underwent a pterional craniotomy on July 16, 1992, with biopsy and aspiration, and a craniopharyngioma was diagnosed. In August 1992, the VP shunts were revised. In October 1992 in the United States, the patient underwent a transcallosal craniotomy for excision of the tumor. Postoperatively, he received 5400 cGy of radiation therapy. In 1997 the tumor recurred and at age 8, he underwent a right orbitozygomatic craniotomy with near-total excision of the tumor. In June 1999 (age 10) he underwent a right pterional orbitozygomatic craniotomy with excision of the tumor followed by Gamma knife therapy. At 13 years he was brought for treatment after experiencing increasing headaches, double vision, and decreasing vision for 4 months.

His current medications are hydrocortisone (10 mg A.M., 5 MG P.M.), levothyroxine (0.125 mg, 1/day); 1-deamino-8-D-arginine vasopressin (DDAVP; 0.1 ml intranasal spray twice daily). On April 18, 2000, magnetic resonance (MR) imaging showed a cystic recurrence of the tumor (1.7 × 1.1 cm). On October 16, 2000, the lesion measured 1.9 × 1.4 cm (Fig. [1]); on March 14, 2001, it measured 2.6 × 2.1 × 1.7 cm; and on June 6, 2001, it measured 2.6 × 2.7 × 3.2 cm. The patient could not see hand motion with his right eye. Visual acuity was 20/200 in only a small nasal field in his left eye. He had complete temporal hemianopsia. He was almost completely and permanently blind. He has hypopituitarism, and the tumor threatens the hypothalamus.