Journal of Neuro-Ophthalmology, March 1990, Volume 10, Issue 1

Papillary carcinoma of the sphenoid sinus associated with sphenoid sinus abscess presenting as cavernous sinus syndrome. A case report.

Primary carcinoma of the sphenoid sinus is a rare tumor that may present with dramatic neuro-ophthalmological-symptoms and signs of which spheno-cavernous syndrome is the best known clinical entity. The most frequently encountered histological types of the sphenoid carcinomas are squamous cell carcinoma and papillary carcinoma, in decreasing order of frequency. In this article, a papillary carcinoma of the sphenoid sinus associated with sphenoid sinus abscess is presented. We are not aware of previously reported papillary carcinoma of the sphenoid sinus associated with sphenoid sinus abscess presenting as a cavernous sinus syndrome.

Journal of ClIII/ cal Neuro- ophthalmology 10( 1): 18- 20, 1990. Papillary Carcinoma of the Sphenoid Sinus Associated with Sphenoid Sinus Abscess Presenting as Cavernous Sinus Syndrome A Case Report Ahmet <; olak, M. D., Kemal Benli, M. D., Teoman D6nmez, M. D., and Behsan Onol, M. D. © 1990 Raven Press, Ltd., New York Primary carcinoma of the sphenoid sinus is a rare tumor that may present with dramatic neuro- ophthalmologi­cal- symptoms and signs of which spheno- cavernous syndrome is the best known clinical entity. The most frequently encountered histological types of the sphe­noid carcinomas are squamous cell carcinoma and pap­illary carcinoma, in decreasing order of frequency. In this article, a papillary carcinoma of the sphenoid sinus associated with sphenoid sinus abscess is presented. We are not aware of previously reported papillary carcinoma of the sphenoid sinus associated with sphenoid sinus abscess presenting as a cavernous sinus syndrome. Key Words: Papillary carcinoma- Spheno- cavernous syndrome- Sphenoid sinus abscess. From the Departments of Neurosurgery and Pathology, Hacettepe University School of Medicine, Ankara, Turkey. Address correspondence and reprint requests to Ahmet ( olak, M. D, Tip Fakulte'l Cadde'l 36/ 5, Abidinpa~ a, Ankara, To · .: 18 Carcinoma of the sphenoid sinus, first described in 1891, is very rare, constituting only 0.3% of all sinus cancers ( 1- 4). When it occurs, it is apt to produce neuro- ophthalmological symptoms and signs that are nonspecific until the sinus wall is penetrated, Specific neurological symptoms and signs of the sphenoid sinus carcinoma are charac­terized most commonly by the spheno- cavemous syndrome and less frequently by isolated sixth cra­nial nerve palsy and visual loss on the ipsilateral side ( 1,4- 6). CASE REPORT A 31- year- old male patient was admitted to the Department of Neurosurgery with a 2- month his­tory of diplopia and ptosis, He had also suffered from headaches and diminished visual acuity of the right eye, On neurological examination, right and left pu­pils were 4 and 3 mm, respectively. The light reflex in the right eye was decreased. Visual acuity was 20/ 200 in the right eye and 20/ 20 in the left eye. He had complete ptosis and total ophthalmoplegia on the right side. There was anesthesia in the distri­bution of the first and second divisions of the right trigeminal nerve. Exophthalmos was found to be 18 mm ( base 110) in the right eye. Nasopharyngeal examination was negative, and plain radiograms of the skull revealed a soft tissue density filling the sphenoid sinus and eroding the sella turcica. The other physical signs and laboratory findings were normal. Angiography showed significant displace­ment of the neighboring vessels. Computerized to­mography revealed a massive tumor involvement PAPILLARY CARCINOMA OF THE SPHENOID SINUS 19 of the sphenoid sinus and posterior part of the ethmoid sinus with erosion of the right sphenoid wall and clinoid to spread into the right cavernous sinus. It also showed a hypodense area in the sphenoid sinus resembling an abscess ( Fig. 1A and B). Transnasal sphenoidotomy and biopsy were performed and 8 cc of gray- yellow abscess material was drained. Gram stain of this material showed huge amounts of polymorphonuclear leu­kocytes ( PNL). Cultures were negative. Histo­pathological examination of the specimen revealed a papillary carcinoma ( Fig. 2). Antibiotic therapy was administered and irradi­ation of 5,000 rad was performed. Chemotherapy was given. He was alive 14 months after surgery. DISCUSSION Malignancies of the paranasal sinuses comprise between 0.2 and 2% of all human cancers ( 2- FIG. 1. ( A and B) Axial enhance~ compute. d. tomo­grams, showing a soft tissue denSity mass ~ nslng the sphenoid sinus and extending int~ th~ region of the cavernous sinus, posterior ethmoid SinUS, and sella turcica with bony destruction. FIG. 2. ( A and B) Histopathological appearances of the transnasal biopsy, showing the papillary carci­noma with inflammatory cells. 4,5,7,8). Frontal and sphenoid sinus carcinomas are quite rare, accounting for < 1% of all paranasal sinus malignancies ( 2,3,5,7,8). The review of the reported cases showed that only four of 127 cases of paranasal sinus malignancies originated from the sphenoid sinus ( 1,7- 9). Thus, carcinoma of the sphenoid sinus appears to constitute only 0.3% of all the paranasal sinus tumors ( 1,2,4). The cause of the low incidence of this tumor is unknown. It may be related to the paucity of the glandular element in its mucosa and to its location ( 9). The most fre­quent histological type is squamous cell carci­noma, followed by transitional cell carcinoma ( 5). Harbison et a1. reported that only one of the 42 cases with sphenoid sinus carcinoma was papillary carcinoma ( 5). All patients with sphenoid sinus carcinoma have neuro- ophthalmological symptoms and signs at the same time in their course. Spheno- cavernous syndrome is the most prevalent, consisting of in­volvement of the 3rd, 4th, 6th, and the 1st and 2nd divisions of the 5th cranial nerves ( 5,6). In addi­tion, optic nerve palsy may be found frequently. This syndrome would suggest extension to the or­bital apex and/ or the cavernous sinus. In our case, diagnosis was reached by direct J Clin Neuro- ophthalmol, Vol. 10, No. 1, 1990 20 A. C; OLAK ET AL. open biopsy, performed by the transnasal ap­proach. It was difficult to diagnose this case be­cause of the additional infection. Sphenoid sinus abscess is a rare condition. It may be the result of the superinfection of the sinus in our case. We are not aware of previously reported papil­lary carcinoma of sphenoid sinus associated with sphenoid sinus abscess presenting as a spheno­cavernous syndrome. Therefore, it must be noted that some sinus infections may be located on sphe­noid sinus carcinomas. REFERENCES 1. Hara HJ. Malignant tumors of the paranasal sinuses. West J Surg Obstetrics GynecoI1955; 63: 348- 54. J '~ IJ, 2. Jackson RT, Fitz- Hugh GS, Constable we. Malignant neo­plasms of the nasal cavities and paranasal sinuses. A ret­rospective study. Laryngoscope 1982; 87: 488- 95. 3. Kenady DE. Cancer of the paranasal sinuses. Surg Clin North Am 1986; 66: 119- 31. 4. Parsons JT, Mendelhall MW, Mancuso AA, Cassisi IN, Mil­lion RR. Malignant tumors of the nasal cavity and ethmoid and sphenoid sinuses. Int J Radiat Oncol Bioi Phys 1988; 14: 11- 22. 5. Harbison JW, Lessell S, Selhorst JB. Neuro- ophthalmology of sphenoid sinus carcinoma. Brain 1984; 107: 855- 70. 6. Neil RM. Spheno--- cavernous syndrome. In: Walsh and Hoyt's Clinical Neuro-- ophthalmology, 4th ed., Vol. 2. Balti­morelLondon: Williams and Wilkins, 1985: 672- 3. 7. Adams G, Duvall AJ, Smith D, Pollak K. Malignant tumors of the paranasal sinuses. Minn Med 1974; 57: 562- 8. 8. Lewis JS, Castro EB. Cancer of the nasal cavity and para­nasal sinuses. JLaryngol Otol 1972; 86: 255- 62. 9. Robin PE, Shortridge RTJ. Lateralization of the tumors of the nasal cavity and paranasal sinuses and its relationship to aetiology. Lancet 1979; 15: 695- 6.