J Neurosurg 49:119-120, 1978
Nasopharyngeal craniopharyngioma Case report
HESHMATOLAH MAJLESSI, M.D., AHMAD S. SHARIAT, M.D., AND ABDOLALIKATIRAI, M.D.
Departments of Neurology and Otolaryngology, Isfahan University, Isfahan, Iran w' A case is reported of craniopharyngioma with unusual extension through the base of the skull to involve the orbits, pharynx, oropharynx, and neighboring areas. KEY WORDS craniopharyngioma nasopharyngeal tumor 9
C
RANIOPHARYNGIOMASproduce striking and typical symptoms by compression of the optic pathways, pituitary, and hypothalamus. Direct invasion of brain parenchyma is exceedingly rare, ~,~ and nasopharyngeal extension of a craniopharyngioma is also uncommon?-~ This is the report of a case of craniopharyngioma with extension into the orbits, nasopharynx, and oropharynx. Case Report
This 17-year-old boy was referred to the University of Isfahan Medical Center for radiation therapy of a very large and unusual craniopharyngioma. He appeared chronically ill and cachectic, with bizarre deformity of the face. History. He had been in good health till the age of 10 years, when he developed frontal headache and blurring of vision. Examination at that time revealed bitemporal hemianopsia with anosmia. X-ray films disclosed a markedly enlarged sella turcica with destruction of the clinoid processes. At craniotomy performed at the Reza J. Neurosurg. / Volume 49 / July, 1978
9 hypopituitarism
9
Pahlavi Medical Center, Tehran, a large mass 4 • 4 cm in size was seen compressing the optic chiasm and the inferior aspect of the frontal lobes. Puncture of the mass disclosed oily fluid, which was aspirated. Because of adhesion to the neighboring structures, the tumor could not be removed, and a biopsy of the mass was obtained. The histopathological findings were consistent with craniopharyngioma. After the operation, vision improved for a period of 12 months but again worsened, with bitemporal hemianopsia. At the age of 15 years, the patient was seen again because of further visual loss and deteriorating mentation with increasing exophthalmos and hypertelorism. A year later he also developed ataxic gait, and the mass protruded through the hard palate. Examination. There was complete bilateral exophthalmos and hypertelorism. The patient had rhinolalic speech, and two ulcerated areas of the skin near the internal angle of both upper eyelids. Obvious physical signs of hypopituitarism were present. There was a large, soft, meaty mass with overlying ulcerated mucosa bulging through the hard palate and descending from the nasopharynx 119
H. Majlessi, A. S. Shariat and A. Katirai to the oropharynx (Fig. 1). X-ray films of the skull revealed extensive erosion and expansion of the sella turcica by a calcified mass, 4 X 3 cm in size. Biopsy of the orbital and nasopharyngeal mass revealed that it was a craniopharyngioma (Fig. 2). At present the patient is completely blind and demented, with severe bilateral optic atrophy and inability to walk. He has no signs of increased intracranial pressure. References
1. Grover WD, Rorke LB: Invasive craniopharyngioma. J Neuroi Neurosurg Psychiatry 31:580-582, 1968 2. Iyer CGS: Case report of an adamantinoma present at birth. J Neurosnrg 9:221-228, 1952 3. Johnson NE: Craniopharyngioma: review with a discussion of transpalatal approach. Laryngoscope 72:1731-1749, 1962 4. Podoshin L, Rolan L, Altman MM, et al: 'Pharyngeal' craniopharyngioma. J Laryngoi Otol 84:93-99, 1970 5. Prasad U, Kwi NK: Nasopharyngeal craniopharyngioma. J Laryngoi Otol 89:445-452, 1975 FIG. 1. Photograph demonstrating a mass in the mouth, exophthalmos and severe hypertelorism. Note the ulcerated areas of the upper eyelids.
Address reprint requests to: Heshmatolah Majlessi, M.D., P.O. Box 69-203, Isfahan University, Isfahan, Iran.
FIG. 2. Photomicrograph of the biopsy specimen showing the typical craniopharyngioma mosaic of sheets of squamous cell, with beginning central cyst formation. H & E, X 225. 120
J. Neurosurg. / Volume 49 / July, 1978
Nasopharyngeal craniopharyngioma Case report
HESHMATOLAH MAJLESSI, M.D., AHMAD S. SHARIAT, M.D., AND ABDOLALIKATIRAI, M.D.
Departments of Neurology and Otolaryngology, Isfahan University, Isfahan, Iran w' A case is reported of craniopharyngioma with unusual extension through the base of the skull to involve the orbits, pharynx, oropharynx, and neighboring areas. KEY WORDS craniopharyngioma nasopharyngeal tumor 9
C
RANIOPHARYNGIOMASproduce striking and typical symptoms by compression of the optic pathways, pituitary, and hypothalamus. Direct invasion of brain parenchyma is exceedingly rare, ~,~ and nasopharyngeal extension of a craniopharyngioma is also uncommon?-~ This is the report of a case of craniopharyngioma with extension into the orbits, nasopharynx, and oropharynx. Case Report
This 17-year-old boy was referred to the University of Isfahan Medical Center for radiation therapy of a very large and unusual craniopharyngioma. He appeared chronically ill and cachectic, with bizarre deformity of the face. History. He had been in good health till the age of 10 years, when he developed frontal headache and blurring of vision. Examination at that time revealed bitemporal hemianopsia with anosmia. X-ray films disclosed a markedly enlarged sella turcica with destruction of the clinoid processes. At craniotomy performed at the Reza J. Neurosurg. / Volume 49 / July, 1978
9 hypopituitarism
9
Pahlavi Medical Center, Tehran, a large mass 4 • 4 cm in size was seen compressing the optic chiasm and the inferior aspect of the frontal lobes. Puncture of the mass disclosed oily fluid, which was aspirated. Because of adhesion to the neighboring structures, the tumor could not be removed, and a biopsy of the mass was obtained. The histopathological findings were consistent with craniopharyngioma. After the operation, vision improved for a period of 12 months but again worsened, with bitemporal hemianopsia. At the age of 15 years, the patient was seen again because of further visual loss and deteriorating mentation with increasing exophthalmos and hypertelorism. A year later he also developed ataxic gait, and the mass protruded through the hard palate. Examination. There was complete bilateral exophthalmos and hypertelorism. The patient had rhinolalic speech, and two ulcerated areas of the skin near the internal angle of both upper eyelids. Obvious physical signs of hypopituitarism were present. There was a large, soft, meaty mass with overlying ulcerated mucosa bulging through the hard palate and descending from the nasopharynx 119
H. Majlessi, A. S. Shariat and A. Katirai to the oropharynx (Fig. 1). X-ray films of the skull revealed extensive erosion and expansion of the sella turcica by a calcified mass, 4 X 3 cm in size. Biopsy of the orbital and nasopharyngeal mass revealed that it was a craniopharyngioma (Fig. 2). At present the patient is completely blind and demented, with severe bilateral optic atrophy and inability to walk. He has no signs of increased intracranial pressure. References
1. Grover WD, Rorke LB: Invasive craniopharyngioma. J Neuroi Neurosurg Psychiatry 31:580-582, 1968 2. Iyer CGS: Case report of an adamantinoma present at birth. J Neurosnrg 9:221-228, 1952 3. Johnson NE: Craniopharyngioma: review with a discussion of transpalatal approach. Laryngoscope 72:1731-1749, 1962 4. Podoshin L, Rolan L, Altman MM, et al: 'Pharyngeal' craniopharyngioma. J Laryngoi Otol 84:93-99, 1970 5. Prasad U, Kwi NK: Nasopharyngeal craniopharyngioma. J Laryngoi Otol 89:445-452, 1975 FIG. 1. Photograph demonstrating a mass in the mouth, exophthalmos and severe hypertelorism. Note the ulcerated areas of the upper eyelids.
Address reprint requests to: Heshmatolah Majlessi, M.D., P.O. Box 69-203, Isfahan University, Isfahan, Iran.
FIG. 2. Photomicrograph of the biopsy specimen showing the typical craniopharyngioma mosaic of sheets of squamous cell, with beginning central cyst formation. H & E, X 225. 120
J. Neurosurg. / Volume 49 / July, 1978
