J. Neurosurg. / Volume 32 / June, 1970

Giant Cell Tumors of the Sphenoid Bone*

JAMES D. GEISSINGER, M.D., EDIR B. SIQUEIRA, M.D., AND EMANUEL R. ROSS, M.D. Division o/Neurological Surgery, Department of Surgery, and Departments of Neurology, Psychiatry, and Pathology, Chicago Wesley Memorial Hospital, and Northwestern University Medical School, Chicago, Illinois

LTHOUGH giant cell tumors have been reacted promptly to light, however. The vi- reported in different areas of the sual acuity were 20/13 bilaterally corrected. skull and paranasal sinuses, their oc- The fundi and visual fields were normal. The currence in the sphenoid bone is rare. Since remaining cranial nerves, tests of strength, it may closely simulate other lesions about reflexes, sensation, and cerebellar function the dorsum sellae and parasellar region, the were all normal. The plantar responses were recognition of such lesions should be of spe- flexor bilaterally. cial interest to the neurological surgeon. Hospital Course. A few hours after hospi- talization, the paralysis of the left lateral rec- Case Report tus muscle progressed and became complete. A 20-year-old housewife in the 34th week On the day preceding her admission this pa- of pregnancy entered the hospital on Febru- tient had been examined not only by an oph- ary 19, 1969, with complaints of double vi- thalmologist but also by a neurologist. Nei- sion and headache. She had been asympto- ther physician was able to detect any weak- matic until early February, 1969, when she ness of the ocular muscles. Initially this ra- began to have mild frontal headaches. Two pid deterioration in the function of the left days later she became aware of double vision sixth cranial nerve was felt most likely to be which was at first intermittent, but later be- secondary to a rapidly expanding lesion. came persistent. Several days thereafter she Examination of the blood revealed a he- developed a drooping left eyelid. During the mogiobin of 11.1 gm%. The routine labora- week prior to admission the pains became tory work was otherwise within normal lim- localized about the left eye and along the left its. The fasting blood sugar was 90 mg%, side of the nose; she also complained of loss and the blood urea nitrogen 9 mg%. Radio- of appetite, restless sleep, and generalized logical examination of the chest was normal. weakness. Skull films revealed that although the ante- The remaining review of neurological, en- rior portion of the floor of the sella was nor- docrine, and general medical systems was mal, the posterior portion showed marked normal. bony destruction. Only the upper portion of Examination. The patient was found to be the dorsum sellae was visible (Fig. 1 left). alert, cooperative, and in no acute distress. These changes were better defined on lateral The general physical examination was unre- laminograms of the sella (Fig. 1 right). A markable except for the abdomen. The uter- left carotid arteriogram was interpreted as ine fundus was palpable 3 cm above the um- normal. A retrograde right brachial cerebral bilicus. A few weak, intermittent uterine arteriogram showed questionable straight- contractions were discerned. ening and backward displacement of the ter- Neurological examination revealed mod- minal portion of the basilar artery. A techne- erate weakness of the left lateral rectus mus- tium-99 brain scan was considered normal. cle. There was also a partial paresis of the The clinical impression rested between a left third cranial nerve characterized by par- clivus chordoma and a sarcoma originating tial ptosis and pupillary inequality, the left within the sphenoid sinus. In view of her be- pupil being larger than the right. Both pupils ing close to term, careful consideration was Received for publication July 22, 1969. given as to the advisability of active therapy * This study was aided by Training Grant NB- prior to the delivery. The clinical history 5408 from the National Institute of Neurological suggested a rapidly progressing lesion in Diseases and Stroke, U.S.P.H.S. view of the abrupt onset of the 6th cranial 665 666 J.D. Geissinger, E. B. Siqueira and E. R. Ross

FIG. 1. Le/t: Plain lateral skull film showing marked destruction of the dorsum sellae and posterior portion of the floor of the sellae turcica. Right: Lateral laminogram of the skull further clarifying the bony destruction of the dorsum sellae. nerve paralysis. It was felt that the risk of vesicular, and usually contained a single letting her strain during delivery plus effects small nucleolus. The cytoplasm varied in of the analgesics required to allay her pain amount and was finely granular. Amongst were greater than the prompt diagnosis and these giant cells, spindle as well as ovoid- institution of therapy. The obstetrical service shaped stromal cells were present. Many of concurred with our opinion, and a biopsy of the stromal cell nuclei were moderately vesi- the lesion was planned. cular, and resembled the nuclei noted within Operation. On February 26, 1969, an at- the giant cells. Other nuclei were noted tempt at biopsy through a transseptal sphe- which were spindle-shaped and hyperchro- noidotomy was made under local anesthesia, matic. Normal mitotic figures were rare. but satisfactory entry into the sphenoid sinus Masson trichrome stain revealed only iso- was not possible. lated traces of collagen fibers. Increased vas- On March 3, under general anesthesia and cularity was not present. The impression was with the use of the image intensifier, a trans- that of a grade I benign giant cell tumor of labial, transsphenoidal approach to the dot- bone. sum sellae was attained. There was no gross Postoperative Course. The patient's post- tumor visible within the sphenoid sinus. operative course was uncomplicated. Irradi- However, fragments of soft tissue were cu- ation therapy was begun on March 7, 1969, retted from the region of the clivus and dor- and a projected plan of 4500 fads to the tu- sum sellae for histological examination (Fig. mor through two lateral ports over 5 weeks 2). The patient tolerated the procedure well. was instituted. On March 8, 1969, she went The biopsy specimen consisted of small ir- on to an uncomplicated delivery of a normal regular tan-gray to dark red-brown frag- 2268 grn baby boy. By the time of discharge ments of tissue. Microscopic examination from the hospital on March 14, 1969, she (Fig. 3) revealed the presence of many mul- had an almost complete paralysis of the right tinucleated cells generally containing num- sixth cranial nerve, but was otherwise doing erous nuclei. The nuclei were frequently cen- well. trally located, round to ovoid, moderately Subsequently she completed her course of