PARANASAL SINUS CARCINOMA CAUSING ORBITAL MUCOCELE R. K.
GUERRY, M.D.,
AND J. LAWTON S M I T H ,
M.D.
Miami, Florida
Mucoceles account for 3 to 4% of orbital tiirrjnrs.1 This diagnosis, when proven in cases of unilateral proptosis, traditionally provokes a sigh of relief from all con cerned. Longevity is relatively unthreatened, and, with proper surgical attention, the prog nosis is excellent. Recent experience with three patients, however, has changed our attitude toward this usually benign tumor. In each of these instances a typical frontal sinus mucocele extending into the orbit was the presenting picture of an otherwise asymp tomatic squamous cell carcinoma. CASE REPORTS
Case 1—A 68-year-old woman was first seen in May 1971 complaining of vertical diplopia. She was in good health until 1969 when asymptomatic proptosis of the left eye was noted on a routine ophthalrnologic examination. Skull x-ray films and orbital tomograms were negative. A radioactive iodine uptake test was normal and showed border line suppression after liothyronine sodium (Cytomel). Her presumed diagnosis was thyroid ophthalmopathy. Later in 1969 she developed intermittent vertical diplopia. One year later it was almost constant and she consulted another ophthalmologist who noted 6 mm of proptosis and some congestion of her conjunctival vessels on the left side. She was ad vised to use an occluder over one eye. On May 17, 1971, corrected visual acuity was 20/15 in both eyes. There was mild blepharoptosis and 6 mm of proptosis on the left (Fig. 1, top). The left eye was somewhat downwardly displaced, and fullness was noted in the upper nasal aspect of the orbit. The ocular motility was full but for marked restriction of movement of the left eye up and in (Fig. 1, bottom). The pupils, visual fields, and slit-lamp examination were normal. Dilated indirect ophthalmoscopic examination revealed a few striae in the extreme upper nasal quadrant of the left fundus. A review of photographs taken in 1954 showed no exophthalmos. However, proptosis of the left eye was definitely present in 1964, and further photographs documented progression over the next three years. Skull x-ray films showed clouding of From the Bascom Palmer Eye Institute, De partment of Opbthalmology, University of Miami School of Medicine, Miami, Florida. Reprint requests to J. Lawton Smith, M.D., P.O. Box 520875, Biscayne Annex, Miami, FL 33152.
the frontal sinus and destruction of the superior medial rim of the left orbit. Orbital venography showed an anteromedially placed left orbital mass. In June 1971, the left orbit was explored, and a typical frontal sinus mucocele was found, in cised, and drained. Postoperatively the proptosis decreased to 1 mm, and the vertical diplopia dis appeared. Within one year, however, she had a recurrence of 4 mm of proptosis, and she was referred to an otolaryngologist who resected the mucocele in May 1972. Biopsy material for histologic examination was taken from the defect in the floor of the sinus and from the wall of the mucocele. This was reported as orbital mucocele. Although the proptosis disappeared, she com plained of persistent pain behind the left eye. This led to a partial ethmoidectomy and removal of one turbinate in May 1973; an antrostomy in February 1974; and an osteosinusectomy and antrostomy in June 1974. These procedures did not relieve her pain. On Sept. 23, 1974, she presented for neuroophthalmologic reevaluation because of the per sistent pain behind the left eye. There was 2 mm of proptosis on the left and hypesthesia over the first and second trigeminal divisions on the left side. Skull x-ray films and orbital tomograms showed a recurrence of the mucocele, this time more posteriorly in the left orbit. Because of the protracted painful course in this patient, the pathol ogy was reviewed. Sections from the bone around the defect in the floor of the frontal sinus taken at the second operative procedure showed definite invasive squamous cell carcinoma (Fig. 2). Neurosurgical consultation was obtained. A left carotid angiogram showed no evidence of intracranial extension. Left frontal craniotomy was per formed, and the left orbit was explored extra-
Fig. 1 (Guerry and Smith). Case 1. Top, For ward gaze; bottom, up and right gaze. 943
944
AMERICAN JOURNAL OF OPHTHALMOLOGY
NOVEMBER, 1975
Fig. 2 (Guerry and Smith). Case 1. Histologic section (hematoxylin and eosin, X440). durally, revealing extensive tumor infiltration throughout the left frontal and ethmoid sinuses and the diploic space of the orbital roof. We at tempted to excise as much of the tumor as possible, and she was then treated with radiotherapy. Case 2—A 72-year-old man, first seen in January 1971, complained of swelling of the right brow and blepharoptosis of the upper eyelid. He gave a long history of sinus headaches and described intermit tent soreness and swelling of the right brow for three to four years. Two weeks before examination he first noted blepharoptosis and consulted an otolaryngologist, who washed out his right antrum.
Fig. 3 (Guerry and Smith). Case 2. Top, For ward gaze; bottom, with eyes closed, note fullness over right brow.
He had mild adult onset diabetes. Otherwise his past history was unremarkable. On Jan. 18, 1971, corrected visual acuity was 20/15 in both eyes. There was moderate blepharop tosis on the right (Fig. 3, top) with fullness over the right brow (Fig. 3, bottom). On palpation, a doughy area of resistance was felt under the superior lateral aspect of the orbital rim. Pupils, motility, slit-lamp examination, and dilated in direct ophthalmoscopy were unremarkable. Subtle hypesthesia in the first trigeminal division on the right was noted. Skull and sinus x-ray films taken two months before were normal. These examinations were re peated, and again no abnormalities were seen. On Jan. 29, the right orbit was explored, and a typical frontal sinus mucocele was encountered. This was incised and drained, and tissue was sent for routine histologic examination. Examination of the biopsy material revealed well-differentiated squamous cell carcinoma infiltrating the wall of the mucocele. Over the next two months he received 7,000 rads to the right orbit and supraorbital area. Initially he did well, but ten months later he returned with a recurrence of swelling over the right brow- He complained of pain and numbness in the first tri geminal division on the right side. On examination his visual acuity, fields, motility, and fundi were normal. Sinus x-ray films and tomograms of the right orbit showed a soft tissue mass in the inferolateral aspect of the right frontal sinus with exten sive involvement of the sphenoid bone. On Dec. 8, he underwent radical surgical excision of his recur rent tumor that involved the orbit, frontal sinus, and the dura mater of the anterior fossa on the right side. The postoperative course was compli cated, and he died Sept. 3, 1972. Autopsy was not performed. Case 3—A 64-year-old man was first seen in 1964. On routine examination he had proptosis of
VOL. 80, NO. 5
ORBITAL MUCOCELE
945
the left eye. He gave a history of chronic sinusitis and alcoholism. Sinus x-ray films showed evidence of chronic sinusitis and a typical left frontal sinus mucocele extending into the left orbit (Fig. 4). Review of x-ray films taken one year previously showed the mucocele present at that time. The patient was not seen again until January 1966. In the interim there had been definite progression of the proptosis and repeat x-ray films showed enlargement of the mucocele. The left frontal sinus was explored surgically, and a large cystic tumor extended through a defect in the floor into the medial aspect of the left orbit. The nasofrontal duct was obliterated by tumor. Path ologic examination showed this to be squamous cell carcinoma. Postoperatively the patient did poorly, and he died of bilateral bronchopneumonia on the 24th day. An autopsy revealed extensive tumor involvement of the left frontal and ethmoid sinuses. DISCUSSION
In his 1921 Hunterian lecture, Howarth 2 denned mucocele as follows: "By the term mucocele of a sinus is meant the accumu lation and retention within it of a mucous secretion owing to obstruction of its outlet Fig. 4 (Guerry and Smith). Case 3. Arrows with thinning and possible distention of one show leading edge of mucocele. or more of the walls of the sinus." In the adult the sinus of origin is virtually always the frontal or ethmoid, the former occurring cases will serve to reemphasize this rela five times more frequently than the latter.3 tionship. Often, the mucocele involves both sinuses, In all three cases carcinoma of the paraand its true Origin cannot be determined. nasal sinuses was not suspected because the Mucoceles arising from the sphenoid and clinical and rarlinlrigir'prpsprirarirm w a s typi maxillary sinuses are extremely rare. cal qf a rn'irncplp—Thoro was- unilateral Howarth 2 emphasized the role of obstruc blepharoptOSJS, proptosis WJth infprntpmtion of the sinus ostium in the pathogenesis poral displacement of the globe, good visual of mucocele. Bordley and Bosley,4 in a re acuity, diplopia (Case 1), and a history of cent review of 56 cases of frontal sinus chronic sinusitis. In Cases l and 6 where mucocele, stated that obstruction of the proptosis was present for at least seven nasofrontal duct was found at surgery in and two years, respectively, the time course all 56 patients. While chronic inflammation, was misleading. However, squamous cell infection, anatomic variation, trauma, sur carcinoma in this area has a long and often gery, and tumor have been described as re asymptomatic course. It is possible, of sponsible for obstruction of the ostium, we course, that carcinoma was not responsible are interested in the tumor. Lederef's 8 1953 for the mucocele at all: rather, it occurred text is representative of the older literature concomitant with the mucocele, both being in that it includes neoplasm in this list. related to chronic inflammation. Two pa Henderson's 6 excellent recent text on orbital tients (Cases 2 and 3) may have had cystic tumors makes no mention of this point. The tumors rather than mucoceles. The point is, relationship between tumor and m.icocek however, that tumor should always be has been virtually forgotten. These three suspeclcd in this condition. Mere incision
946
AMERICAN JOURNAL OF OPHTHALMOLOGY
and drainage without histologic verification are never sufficient in its management. The cause for obstruction of the sinus ostium should be determined. Reese7 found mucocele a common cause of unilateral proptosis when clinical and radiologic criteria were used to make the diagnosis. However, in his series of 877 consecutive cases of orbital neoplasms and simulating lesions studied histologically, there was not a single case of mucocele. He attributed this to the fact that "tissue from this lesion is never sent to the laboratory."7 These cases demonstrate the need for care ful pathologic examination. Bone as' well as soft tissue should be included in the specimen submitted. In the first case the one tumor-containing frag ment had to undergo decalcification and was thus overlooked in the original pathologic report. While the ultimate outcome in this patient (Case 1) may not have been influenced by this error, the proper initial diagnosis could have spared her four subsequent operations. The x-ray films in Cases 1 and 3 were textbook representations of frontal sinus mucocele. In Case 2, the x-ray films were completely normal, even on review. There are several lessons to be learned here. In this condition, tomography is essential, for the changes of a mucocele are often so subtle that the diagnosis cannot be made on x-ray films. More important, the pres
NOVEMBER, 1975
ence of concomitant carcinoma cannot be ruled out on either tomography or x-ray films. One must first suspect its presence, obtain sufficient tissue at the time of surgery, and insist on careful histopathologic exami nation in all mucoceles. SUMMARY
Three patients presented with typical orbital mucoceles. However, subsequent his topathologic study revealed paranasal sinus concomitant squamous cell carcinoma in all three cases. Careful histologic investigation is necessary before considering orbital mucocele a benign process. REFERENCES
1. Henderson, J. W.: Orbital Tumors. Philadel phia, W. B. Saunders Co., 1973, p. 107. 2. Howarth, W. G.: Hunterian Lecture. Muco cele and pyocele of the nasal accessory sinuses. Lancet 2:744, 1921. 3. Bonninghaus, G.: Die Operationen an den Nebenhohlen der Nase. In Katz-Blumenfeld's Handbuch der speziellen Chirurgie des Ohres und der oberen Luftwege, vol. 3. Leipzig, Kaditsch, 1923, p. 89. 4. Bordley, J. E., and Bosley, W. R.: Mucoceles of the frontal sinus. Causes and treatment. Ann. Otolaryngol. 82:699, 1973. 5. Lederer, F. L.: Diseases of the Ear, Nose, and Throat. Philadelphia, F. A. Davis Co., 1953, p. 575. 6. Henderson, J. W.: Orbital Tumors. Philadel phia, W. B. Saunders Co., 1973, pp. 105-113. 7. Reese, A. B.: Tumors of the Eye. New York, Harper and Row, 1963, pp. 533-536.
GUERRY, M.D.,
AND J. LAWTON S M I T H ,
M.D.
Miami, Florida
Mucoceles account for 3 to 4% of orbital tiirrjnrs.1 This diagnosis, when proven in cases of unilateral proptosis, traditionally provokes a sigh of relief from all con cerned. Longevity is relatively unthreatened, and, with proper surgical attention, the prog nosis is excellent. Recent experience with three patients, however, has changed our attitude toward this usually benign tumor. In each of these instances a typical frontal sinus mucocele extending into the orbit was the presenting picture of an otherwise asymp tomatic squamous cell carcinoma. CASE REPORTS
Case 1—A 68-year-old woman was first seen in May 1971 complaining of vertical diplopia. She was in good health until 1969 when asymptomatic proptosis of the left eye was noted on a routine ophthalrnologic examination. Skull x-ray films and orbital tomograms were negative. A radioactive iodine uptake test was normal and showed border line suppression after liothyronine sodium (Cytomel). Her presumed diagnosis was thyroid ophthalmopathy. Later in 1969 she developed intermittent vertical diplopia. One year later it was almost constant and she consulted another ophthalmologist who noted 6 mm of proptosis and some congestion of her conjunctival vessels on the left side. She was ad vised to use an occluder over one eye. On May 17, 1971, corrected visual acuity was 20/15 in both eyes. There was mild blepharoptosis and 6 mm of proptosis on the left (Fig. 1, top). The left eye was somewhat downwardly displaced, and fullness was noted in the upper nasal aspect of the orbit. The ocular motility was full but for marked restriction of movement of the left eye up and in (Fig. 1, bottom). The pupils, visual fields, and slit-lamp examination were normal. Dilated indirect ophthalmoscopic examination revealed a few striae in the extreme upper nasal quadrant of the left fundus. A review of photographs taken in 1954 showed no exophthalmos. However, proptosis of the left eye was definitely present in 1964, and further photographs documented progression over the next three years. Skull x-ray films showed clouding of From the Bascom Palmer Eye Institute, De partment of Opbthalmology, University of Miami School of Medicine, Miami, Florida. Reprint requests to J. Lawton Smith, M.D., P.O. Box 520875, Biscayne Annex, Miami, FL 33152.
the frontal sinus and destruction of the superior medial rim of the left orbit. Orbital venography showed an anteromedially placed left orbital mass. In June 1971, the left orbit was explored, and a typical frontal sinus mucocele was found, in cised, and drained. Postoperatively the proptosis decreased to 1 mm, and the vertical diplopia dis appeared. Within one year, however, she had a recurrence of 4 mm of proptosis, and she was referred to an otolaryngologist who resected the mucocele in May 1972. Biopsy material for histologic examination was taken from the defect in the floor of the sinus and from the wall of the mucocele. This was reported as orbital mucocele. Although the proptosis disappeared, she com plained of persistent pain behind the left eye. This led to a partial ethmoidectomy and removal of one turbinate in May 1973; an antrostomy in February 1974; and an osteosinusectomy and antrostomy in June 1974. These procedures did not relieve her pain. On Sept. 23, 1974, she presented for neuroophthalmologic reevaluation because of the per sistent pain behind the left eye. There was 2 mm of proptosis on the left and hypesthesia over the first and second trigeminal divisions on the left side. Skull x-ray films and orbital tomograms showed a recurrence of the mucocele, this time more posteriorly in the left orbit. Because of the protracted painful course in this patient, the pathol ogy was reviewed. Sections from the bone around the defect in the floor of the frontal sinus taken at the second operative procedure showed definite invasive squamous cell carcinoma (Fig. 2). Neurosurgical consultation was obtained. A left carotid angiogram showed no evidence of intracranial extension. Left frontal craniotomy was per formed, and the left orbit was explored extra-
Fig. 1 (Guerry and Smith). Case 1. Top, For ward gaze; bottom, up and right gaze. 943
944
AMERICAN JOURNAL OF OPHTHALMOLOGY
NOVEMBER, 1975
Fig. 2 (Guerry and Smith). Case 1. Histologic section (hematoxylin and eosin, X440). durally, revealing extensive tumor infiltration throughout the left frontal and ethmoid sinuses and the diploic space of the orbital roof. We at tempted to excise as much of the tumor as possible, and she was then treated with radiotherapy. Case 2—A 72-year-old man, first seen in January 1971, complained of swelling of the right brow and blepharoptosis of the upper eyelid. He gave a long history of sinus headaches and described intermit tent soreness and swelling of the right brow for three to four years. Two weeks before examination he first noted blepharoptosis and consulted an otolaryngologist, who washed out his right antrum.
Fig. 3 (Guerry and Smith). Case 2. Top, For ward gaze; bottom, with eyes closed, note fullness over right brow.
He had mild adult onset diabetes. Otherwise his past history was unremarkable. On Jan. 18, 1971, corrected visual acuity was 20/15 in both eyes. There was moderate blepharop tosis on the right (Fig. 3, top) with fullness over the right brow (Fig. 3, bottom). On palpation, a doughy area of resistance was felt under the superior lateral aspect of the orbital rim. Pupils, motility, slit-lamp examination, and dilated in direct ophthalmoscopy were unremarkable. Subtle hypesthesia in the first trigeminal division on the right was noted. Skull and sinus x-ray films taken two months before were normal. These examinations were re peated, and again no abnormalities were seen. On Jan. 29, the right orbit was explored, and a typical frontal sinus mucocele was encountered. This was incised and drained, and tissue was sent for routine histologic examination. Examination of the biopsy material revealed well-differentiated squamous cell carcinoma infiltrating the wall of the mucocele. Over the next two months he received 7,000 rads to the right orbit and supraorbital area. Initially he did well, but ten months later he returned with a recurrence of swelling over the right brow- He complained of pain and numbness in the first tri geminal division on the right side. On examination his visual acuity, fields, motility, and fundi were normal. Sinus x-ray films and tomograms of the right orbit showed a soft tissue mass in the inferolateral aspect of the right frontal sinus with exten sive involvement of the sphenoid bone. On Dec. 8, he underwent radical surgical excision of his recur rent tumor that involved the orbit, frontal sinus, and the dura mater of the anterior fossa on the right side. The postoperative course was compli cated, and he died Sept. 3, 1972. Autopsy was not performed. Case 3—A 64-year-old man was first seen in 1964. On routine examination he had proptosis of
VOL. 80, NO. 5
ORBITAL MUCOCELE
945
the left eye. He gave a history of chronic sinusitis and alcoholism. Sinus x-ray films showed evidence of chronic sinusitis and a typical left frontal sinus mucocele extending into the left orbit (Fig. 4). Review of x-ray films taken one year previously showed the mucocele present at that time. The patient was not seen again until January 1966. In the interim there had been definite progression of the proptosis and repeat x-ray films showed enlargement of the mucocele. The left frontal sinus was explored surgically, and a large cystic tumor extended through a defect in the floor into the medial aspect of the left orbit. The nasofrontal duct was obliterated by tumor. Path ologic examination showed this to be squamous cell carcinoma. Postoperatively the patient did poorly, and he died of bilateral bronchopneumonia on the 24th day. An autopsy revealed extensive tumor involvement of the left frontal and ethmoid sinuses. DISCUSSION
In his 1921 Hunterian lecture, Howarth 2 denned mucocele as follows: "By the term mucocele of a sinus is meant the accumu lation and retention within it of a mucous secretion owing to obstruction of its outlet Fig. 4 (Guerry and Smith). Case 3. Arrows with thinning and possible distention of one show leading edge of mucocele. or more of the walls of the sinus." In the adult the sinus of origin is virtually always the frontal or ethmoid, the former occurring cases will serve to reemphasize this rela five times more frequently than the latter.3 tionship. Often, the mucocele involves both sinuses, In all three cases carcinoma of the paraand its true Origin cannot be determined. nasal sinuses was not suspected because the Mucoceles arising from the sphenoid and clinical and rarlinlrigir'prpsprirarirm w a s typi maxillary sinuses are extremely rare. cal qf a rn'irncplp—Thoro was- unilateral Howarth 2 emphasized the role of obstruc blepharoptOSJS, proptosis WJth infprntpmtion of the sinus ostium in the pathogenesis poral displacement of the globe, good visual of mucocele. Bordley and Bosley,4 in a re acuity, diplopia (Case 1), and a history of cent review of 56 cases of frontal sinus chronic sinusitis. In Cases l and 6 where mucocele, stated that obstruction of the proptosis was present for at least seven nasofrontal duct was found at surgery in and two years, respectively, the time course all 56 patients. While chronic inflammation, was misleading. However, squamous cell infection, anatomic variation, trauma, sur carcinoma in this area has a long and often gery, and tumor have been described as re asymptomatic course. It is possible, of sponsible for obstruction of the ostium, we course, that carcinoma was not responsible are interested in the tumor. Lederef's 8 1953 for the mucocele at all: rather, it occurred text is representative of the older literature concomitant with the mucocele, both being in that it includes neoplasm in this list. related to chronic inflammation. Two pa Henderson's 6 excellent recent text on orbital tients (Cases 2 and 3) may have had cystic tumors makes no mention of this point. The tumors rather than mucoceles. The point is, relationship between tumor and m.icocek however, that tumor should always be has been virtually forgotten. These three suspeclcd in this condition. Mere incision
946
AMERICAN JOURNAL OF OPHTHALMOLOGY
and drainage without histologic verification are never sufficient in its management. The cause for obstruction of the sinus ostium should be determined. Reese7 found mucocele a common cause of unilateral proptosis when clinical and radiologic criteria were used to make the diagnosis. However, in his series of 877 consecutive cases of orbital neoplasms and simulating lesions studied histologically, there was not a single case of mucocele. He attributed this to the fact that "tissue from this lesion is never sent to the laboratory."7 These cases demonstrate the need for care ful pathologic examination. Bone as' well as soft tissue should be included in the specimen submitted. In the first case the one tumor-containing frag ment had to undergo decalcification and was thus overlooked in the original pathologic report. While the ultimate outcome in this patient (Case 1) may not have been influenced by this error, the proper initial diagnosis could have spared her four subsequent operations. The x-ray films in Cases 1 and 3 were textbook representations of frontal sinus mucocele. In Case 2, the x-ray films were completely normal, even on review. There are several lessons to be learned here. In this condition, tomography is essential, for the changes of a mucocele are often so subtle that the diagnosis cannot be made on x-ray films. More important, the pres
NOVEMBER, 1975
ence of concomitant carcinoma cannot be ruled out on either tomography or x-ray films. One must first suspect its presence, obtain sufficient tissue at the time of surgery, and insist on careful histopathologic exami nation in all mucoceles. SUMMARY
Three patients presented with typical orbital mucoceles. However, subsequent his topathologic study revealed paranasal sinus concomitant squamous cell carcinoma in all three cases. Careful histologic investigation is necessary before considering orbital mucocele a benign process. REFERENCES
1. Henderson, J. W.: Orbital Tumors. Philadel phia, W. B. Saunders Co., 1973, p. 107. 2. Howarth, W. G.: Hunterian Lecture. Muco cele and pyocele of the nasal accessory sinuses. Lancet 2:744, 1921. 3. Bonninghaus, G.: Die Operationen an den Nebenhohlen der Nase. In Katz-Blumenfeld's Handbuch der speziellen Chirurgie des Ohres und der oberen Luftwege, vol. 3. Leipzig, Kaditsch, 1923, p. 89. 4. Bordley, J. E., and Bosley, W. R.: Mucoceles of the frontal sinus. Causes and treatment. Ann. Otolaryngol. 82:699, 1973. 5. Lederer, F. L.: Diseases of the Ear, Nose, and Throat. Philadelphia, F. A. Davis Co., 1953, p. 575. 6. Henderson, J. W.: Orbital Tumors. Philadel phia, W. B. Saunders Co., 1973, pp. 105-113. 7. Reese, A. B.: Tumors of the Eye. New York, Harper and Row, 1963, pp. 533-536.
