Ann Olol 84: 1975

GIANT CELL TUMOR OF THE SPHENOID BONE

I. M. GUPTA, M.D. O. P. GUPTA, M.S.

H. C. SAMANT, M.S.

P. L. BHATIA, M.S.

A. K. AGARWAL, M.D.

G. C. PANT, M.D. VARANASI, INDIA SUMMARY - The clinical features of the giant cell tumor of the sphenoid bone have been discussed and a case report has been added to the fourteen cases reported in the literature. Such cases may first report to an ophthalmologist, an otolaryngologist, a neurologist, or an internist. They should consider this condition in a patient who complains of headache, ocular symptoms such as diplopia, and diminution of vision progressing to complete blindness. The presence of multiple cranial nerve palsies involving II, III, IV, V, and VI nerves in various combinations and the sellar erosion in the lateral x-ray of the skull are quite suggestive of this tumor which should be confirmed by biopsy. The telecobalt therapy appears to give the best results.

Giant cell tumor chiefly occurs in the long bones, infrequently in the small bones, jaws and vertebrae, and only rarely involves the cranial bones. Only 14 cases of this tumor in the sphenoid bone have been reported in the literature.' The present paper not only adds another case to this small list, but also is meant to review the salient features of this condition which are likely to be missed by the clinicians because of its rarity. CASE

REPORT

A 20-year-old male was referred from the Department of Ophthalmology on January 3, 1973, with history of severe headache in the forehead. blurring of vision in the left eye, and diplopia of about one and one-half months' duration. A week later, his left eye became totally blind and vision in the right eye also gradually diminished. Within two weeks, he was blind in both eyes and began to feel heaviness and loss of sensation to touch on the left side of his face. His headache persisted but there

was no vomiting, fever, or cough. He had developed bilateral nasal obstruction 15 days prior to his reporting and had two light attacks of epistaxis from the left nostril. Examination of cranial nerves revealed paralysis of left occulomotor, left trochlear, left trigeminal, and both abducent nerves. His left eye ball was bulging forwards and medially, and both the eyes showed convergent squint (Fig. 1 A). The pupils were dilated, and there was no perception of light, the fundus showing primary optic atrophy in both eyes. There was complete bilateral nasal obstruction owing to a smooth and fleshy nasopharyngeal mass which was pushing down the soft palate (Fig. 1 B) and restricting its movement. The mass was firm in consistency, bled on touch, and was attached to the roof of nasopharynx. There was no cervical lymphadenopathy. The skull x-rays showed a soft tissue mass in the nasopharynx with destruction in the region of the sphenoidal

From the Departments of Pathology, Otolaryngology, Radiology and Radiotherapy, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India.

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Fig. 1. (A) Front view showing convergent squint, vacant look, and mouth breathing. (B) Mouth opened to show depression of the soft palate.

sinus. The pituitary fossa and the dorsum sellae were not discernible (Fig. 2 A). The posterior part of the nasal septum was also found to be destroyed (Fig. 2 B). .The left carotid angiogram was not remarkable. Biopsy provoked excessive bleeding. The histopathology revealed giant cell tumor with characteristic stromal plump cells interspersed with numerous giant cells of the osteoclast type. The unusual feature was the presence of abundant

osteoid, but the tumor lacked the stromal sarcomatous reaction of osteosarcoma (Fig. 3 A and B). The patient was given 4,500 rad by telecobalt in four weeks' time. The size of the tumor, as well as the proptosis of the left eye ball, decreased. The patient's general condition improved and his headache disappeared. However, within three weeks following radiotherapy his condition worsened once

Fig. 2. (A) Lateral x-ray of the skull showing destruction in the region of sphenoidal sinus and soft tissue mass in nasopharynx while the pituitary fossa and dorsum sellae are not discernible. (B) X-ray of the skull-basal view showing obliteration of the anterior part of the nasopharynx with destruction of the posterior part of nasal septum.

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Fig. 3. (A) Microphotograph showing tumor giant cells with numerous nuclei widely scattered within abundant cytoplasm. (B) Striking association of tumor osteoid with osteoclastomatous giant cells (approx. X345, H & E stain).

again. The nasopharyngeal mass increased in size, the proptosis became bilateral, and a vascular lobulated mass could be seen in the right nasal cavity above the middle meatus. Surgical excision of the tumor mass was attempted by transpalatal approach. The tumor was found to be in6ltrating the muscles of the soft palate and eroding the sphenoid bone. It was very friable and bled profusely. The portions of the tumor infiltrating the soft palate and the anterior part of the sphenoid bone could not be excised completely. The patient's postoperative period was uneventful, and he was discharged on April 12, 1973. He expired one and one-half months later without reporting for a follow-up. DISCUSSION

The rarity of sphenoid bone involvement by giant cell tumor makes it worthwhile to review the clinical features of all the 14 cases published so far in the background of the present case in order to delineate some common features which could be attributable to this disease entity.1.12 The age and sex distribution of the giant cell tumor of the sphenoid corresponds to its occurrence in other bones with maximum incidence in the second ( 33.3%) and third (40%) decades of life with a preponderance affecting females (66.6%).13

Headache (86.7%) may be hemicranial, bifrontal, or generalized; it was frontal and very severe in our patient. Among ocular symptoms, diplopia (53.3%) with unilateral blurring and diminution of vision (46.6%) appear early, progressing to complete blindness and then proptosis. In later stages, the opposite eye may also be affected by the same sequence of events, i.e., diminution of vision" amblyopia, and proptosis.P-? Multiple cranial nerve palsies involving II, III, IV, V, and VI nerves in various combinations occur. The most common involvements are those of the occulomotor and the abducent nerves (40%) and trochlear nerve (33.3%), while the trigemenial nerve palsy, mainly of tbe maxillary division with infraorbital hypoaesthesia, occurred in our patient and two other cases only.3.4 These cranial nerve palsies are mainly unilateral. In the later stages of the disease, contralateral palsy of the optic and the abducent nerves may also occur.s-" Additional clinical features of this condition may be nausea and vomiting, amenorrhea, retro-orbital pain, and olfactory hallucinations.t-s-" The appearance of a friable and vascular mass in the nasopharynx and nose causing nasal obstruction is an uncommon feature and occurs probably in the later stages of the disease. Such a mass was present only in our patient and a case reported

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by Peimer." The duration of the symp- to be considered are clivus chordoma toms at the time of first consultation and glomus jugulare. Another imporbears a direct relationship to the rate tant disease to be differentiated is the of growth of the tumor. The growth Brown tumor of hyperparathyroidism was very rapid in seven cases (46.7%); which may present as an isolated giant therefore, the patients presented within cell tumor of the middle cranial Iossa.!" three months of the onset of symptoms. This entity, however, has abnormal calcium and serum phosphorous values. Radiologically, the sellar erosion is The carcinoma of the sphenoid sinus the most consistent feature discernible also produces deep constant headache, (84.6%). Other roentgenological fea- radiological signs of sphenoid sinus detures may be destruction of posterior struction, and extraocular paralysis, clinoids, basisphenoid, clivus and rare- usually without cervical node metasfection of the floor of the pituitary fossa, tases.!" The Brown tumor can be differopaque soft tissue shadow within the entiated from giant cell tumor of the sphenoidal sinus, and a mass in the sphenoid bone only by biopsy. nasopharynx.t-s-lv-'! Special x-ray techHistopathologically, the giant cell nics such as vertebral and carotid anziography and pneumoencephalography tumors must be differentiated from the reparative granulomas which occur in a do not seem to contribute much. comparatively younger age group. The Preoperative biopsy appears to have true giant cell tumors have a larger been an exception rather than a rule in number of giant cells, greater cellularity these cases because of the inaccessible of the stroma, and larger and more acnature of the tumor. In the majority tive-looking stromal cells. The connecof the instances, the histopathological tive tissue cells in the reparative grandiagnosis was obtained only after sur- ulomas show stromal cells and are more gery. In one case each, the biopsy flbroblastic.!" material was obtained by translabial These cases have been treated diftranssphenoidal and transseptal transsphenoidal routes.t-P In our patient, ferently by different workers. Appartransnasal biopsy was possible due to ently until more cases of this disease entity are reported, it will be difficult a big tumor mass in the nasopharynx. to decide the best line of treatment. Potter and McClennan 14 have men- Total excision is almost an impossibility tioned in the differential diagnosis of at this site, more so because a very the giant cell tumor of sphenoid, con- small excisable tumor is difficult to ditions such as orbital neurofibromatosis and plasmocytoma, nasopharyngeal detect. Partial excision alone or even fibroma and carcinoma, craniopharyn- With postoperative irradiation does not gioma, primary sarcoma of the bone, appear to give good results. The telesecondary metastatic lesions, large cobalt therapy offers the best progpituitary adenoma, meningioma, and nosis. In our case, partial excision was carotid angioma. Among the rare lesions done only after irradiation had failed. INSTITUTE OF MEDICAL SCIENCES, BANARAS HINDU UNIVERSITY, VARANASI, INDIA,

REFERENCES 1. Emley WE: Giant cell tumor of the sphenoid bone - A case report and review of the literature. Arch Otolaryngol 94:369-374, 1971 2. Geschickter CF, Copeland MM: Tumors

at Bone, ed. 3. Philadelphia, JB Lippencott Co, 1949, p. 288-323 3. Echols DH: Giant cell tumor of the sphenoid bone - Report of a case. J Neuro-

surg 2:16-20, 1945 4. McNerney JC: Giant cell tumor of bones of the skull. J Neurosurg 6: 169-174, 1949 5. Hondousa AB: Osteoclastoma in relationship to the nose. J Larnygotol 65:549559, 1951 6. Peimer R: Benign giant cell tumor of

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TUMOR OF SPHENOID BONE

the skull and nasal sinuses. Arch Otolaryngol 60:186-193, 1954 7. Ramamurthi B, Visvanathan GS, Pillai KM: Osteoclastoma of the skull. J Neurosurg 12:287-290, 1955 8. Michael LA: Giant cell tumor of the sinuses. Laryngoscope 69:320-328, 1959 9. Kanaka TS, Balasubramaniam V: Giant cell tumor of the skull. Neurol India 14:5760, 1966

10. Eller JL, Decker JT, Brittis AL: Roentgen therapy for a giant cell tumor of the sphenoid bone - A case report. Radiol Clin North Am 37:36-44, 1968 11. Pitkethly DT, Kempe LG: Giant cell tumors of the sphenoid - Report of two cases, J Neurosurg 30:301-304, 1969

12. Geissinger JD, Siqueira EB, Roes ER: Giant cell tumors of the sphenoid bone. J

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Neurosurg 32:665-670, 1970 13. Hutter RVP, Worcester IN Jr, Francis KC, et al. Benign and malignant giant cell tumors of bone: A clinicopathological analysis of the natural history of the disease. Cancer 15:653-690, 1962 14. Potter GD, McClennan BL: Malignant giant cell tumor of the sphenoid bone and its differential diagnosis. Cancer 25: 167170, 1970 15. Raskin P, Rowe SN, Field JB: Hyperparathyroidism presenting as a giant cell tumor of the middle cranial fossa. Ann Intern Med 69:301-304, 1968 16. Van Wart CA, Dedo HH, McCoy EG: Carconoma of sphenoid sinus. Ann Otol Rhinol Laryngol 82:318-322, 1973 17. Dehlin DC, Cupps RE, Johnson EW Jr: Giant cell tumor - A study of 195 cases. Cancer 25:1061-1070, 1970

OTOLARYNGOLOGIC ASSEMBLY The annual Otolaryngologic Assembly of 1975 will be held November 8-14, 1975, in the Eye and Ear Infirmary of the University of Illinois Hospital. The Department of Otolaryngology of the Abraham Lincoln School of Medicine, University of Illinois at the Medical Center offers a condensed basic and clinical program for practicing otolaryngologists under the direction of Emanuel M. Skolnik, M.D., with Burton J. Soboroff, M.D. as co-chairman. Inquiries may be sent to: Otolaryngology, P.O. Box 6998, Chicago, Illinois 60680.

RADIOLOGY CONFERENCE A Conference on Radiology in Otolaryngology and Ophthalmology will be held on November 28 and 29, 1975, under the guidance of Galdino E. Valvassori, M.D. For further information write to: G. E. Valvassori, M.D., Radiology Department, Abraham Lincoln School of Medicine, P.O. Box 6998, Chicago, Illinois 60680.

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Giant cell tumor of the spenoid bone.

The clinical features of the giant cell tumor of the sphenoid bone have been discussed and a case report has been added to the fourteen cases reported...
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