Frontal Sinus Cancer Manifested as a Frontal Mucocele John M. Robinson, FRCS
During the period 1972 to 1974, 12 mucopyoceles were seen. Subsequently, three were shown to have an underlying neoplasm. Evaluation of the roentgenograms demonstrated minor dif\s=b\
frontal
ferences between those with and those without a malignant basis. In previous reports of frontal sinus cancer, the descriptions of the roentgenograms suggest that it is possible that these features were also present. The difficulty in deciding the sinus of origin in such cancers has often been discussed, but the possibility that neoplasms of the orbital lobe of the lacrimal gland may occur in the frontal sinus is usually not considered. Anatomically, the intimate proximity of this lobe to the frontal sinus suggests that a neoplasm could develop in this manner. I believe that this was true in two of the patients reported herein.
(Arch Otolaryngol 101:718-721, 1975)
moid carcinoma of the frontal sinus, Brownson and Ogura6 noted that in 12 patients, a preoperative diagnosis of mucocele or pyocele was made.
were thought to only one patient was cancer suspected radiologically. They concluded that the pathologist was usually the first to make the cor¬ rect diagnosis.
Clinically, only
two
have cancer, and in
I
report three additional
of cancer of the frontal sinus. In all three, a provisional diagnosis of fron¬ tal sinus mucocele was made, the true diagnosis being established histologi¬ cally. Examination of the roentgen¬ ograms of each revealed certain com¬ mon features which may be helpful in making the correct diagnosis preopercases
atively.
REPORT OF CASES
Cancer
of the frontal sinus is an uncommon condition,13 but in the reported cases, many have been
diagnosed clinically4-7 or radiologically5'8 as a frontal sinus mucocele. In a review of .33 cases of epider-
Accepted for publication May 26, 1975. From the Institute of Laryngology and Otology, London University, London. Dr Robinson is now practicing in Cairns, Queensland, Australia. Reprint requests to Mac Donnell's Building, 26 Abbott St, Cairns, Queensland 4870, Australia (Dr Robinson).
72-year-old man was first in November 1972. His left eye had been prominent for 37 years, but had not caused visual disturbance. However, in the preceding three months, he was aware of discomfort above the left eye, which was then becoming increasingly prominent. Examination showed severe proptosis with displacement of the globe inferolaterally. There was considerable chemosis, and a deficiency could be palpated in the supraorbital ridge, lateral to the supraorbital notch (Fig 1). Sinus roentgenograms showed clouding of the left frontal sinus, with considerable Case l.-A
seen
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hyperostosis and destruction of the frontal bone. The appearances were thought to be due to a pyocele with periostitis, though the possibility of a neoplastic lesion could not be excluded (Fig 2). The sinus was explored through an inci¬ sion below the eyebrow. The bone of the or¬ bital roof was believed to be irregular. On entering the sinus, mucopus was found, and although the mucosa was thickened, no gross tumor
was
visible.
Histologie examination disclosed mu¬ coepidermoid carcinoma in the sinus mu¬ cosa and periorbital fat. The tumor was invading trabecular new bone from the or¬ bital roof.
Radiotherapy
with cobalt 60 as the to a maximum of 6,448 rads was de¬ livered to the affected sinus. Six weeks source
later, repeat sinus roentgenograms
were
unchanged. Tomography demonstrated considerable hyperostosis of the frontal bone and greater wing of the sphenoid bone (Fig 3). The sinus was re-explored, and a frontoethmoidectomy was performed. Al¬ though no tumor was visible, results of his¬ tologie examination confirmed the pres¬ of residual tumor. The patient's recovery was uneventful. Chemosis settled rapidly through the prop¬ tosis remained unchanged. Despite the continued presence of the tumor, he re¬ mained well and symptom free, except for a persistence of proptosis. Fourteen months later, because of recurrence of con¬ siderable chemosis and increasing prop¬ tosis, it was decided to exenterate the orbit ence
(Fig 4).
Fig
1.—Patient with
displacement of globe and chemosis.
Fig 3.—Posteroanterior tomographic cut considerable hyperostosis of frontal bone.
(patient 1) showing
Fig 2—Posteroanterior projection (patient 1). Excessive bone destruction and hyperostosis in left frontal sinus are particularly evident. Orbital roof is virtually destroyed and small osteoma can be seen. There is no sinus expansion.
Fig 4.—Recurrence of chemosis. Note absence of consequence of radiotherapy.
This procedure was performed extraperiosteally, and a gross tumor was found to be visible in the roof of the sinus. The eye¬ lids were "delashed" and sewn together. Subsequently, he enjoyed a further symp¬ tom-free period of three months, until he developed hemiparesis on the right side. Angiograms and brain and EMI scans demonstrated a space-occupying lesion in the left frontal lobe, extending by a nar¬ row track from the left orbit. A remarkable improvement was achieved by frontal craniotomy with the release of necrotic brain and pus, but histologie ex¬ amination of the brain showed invasion by mucoepidermoid carcinoma. This improvement was maintained for three months, after which he deteriorated rapidly and died in January 1975. Unfortu¬ nately, postmortem examination was not
possible.
Case 2.—A 43-year-old woman was first in August 1971. She complained of or¬ bital discomfort on the right side and epiphora of eight months' duration. She seen
also noticed a swelling above the right eye and double vision when tired. Examination disclosed a tender hard mass above the inner canthus of the right eye.
Sinus roentgenograms showed partial clouding of both frontal sinuses, with bony hyperostosis that was most severe in the region of the intersinus septum (Fig 5). There was minimal expansion of the sinus on that side. Tomography revealed erosion of the anterior wall of the frontal sinus with considerable hyperostosis (Fig 6). The appearances were believed to be consistent with a pyocele and osteitis. The sinus was explored by an incision below the eyebrow, and a tumor was en¬ countered invading the floor of the frontal sinus. Mucopus was contained within the sinus. Results of histologie examination showed well-differentiated squamous cell carcinoma invading both periorbital fat and sinus mucosa. Radiotherapy with Cobalt 60 as the source to a maximum of 6,520 rads was de-
Downloaded From: http://archotol.jamanetwork.com/ by a Oakland University User on 06/01/2015
eyebrow,
a
livered over a period of 53 days. The pa¬ tient remained well, except for persisting diplopia. Twenty-one months later, the sinus was re-explored, but no macroscopic or microscopic evidence of tumor could be found. At her most recent review, 40 months after onset, there was no evidence of recurrence. Case 3.—A 49-year-old man was first seen in October 1974. At that time, he com¬ plained of swelling around the left eye of six weeks' duration. Examination showed proptosis and dis¬ placement of the globe inferolaterally. A mucocele was felt beneath the middle of the supraorbital ridge. Sinus roentgenograms showed severe hyperostosis, thickening of mucosa (espe¬ cially in the region of the intersinus sep¬ tum), and some destruction of the frontal bone. There was only a moderate degree of sinus expansion. The appearances were be¬ lieved to be due to a mucocele (Fig 7). The sinus was explored through an inci¬ sion just below the eyebrow. On entering
Fig 5.—Posteroanterior projection (patient 2). There Is severe hyperostosis and soft tissue mass in region of interslnus septum. Although sinus is partially clouded, there Is only minimal expan¬ sion.
Fig 6 (right).—Lateral tomogram of patient 2 demonstrating ero¬ sion of anterior wall of frontal sinus associated with considerable hyperostosis. the sinus, thick mucus was encountered. The bone of the posterior wall was found to be deficient, and a small amount of white tumor was found in the region of the intersinus septum. A frontoethmoidectomy was performed, and all visible tumor was re¬ moved. Histologie examination revealed clear cell neoplasia. There was severe vascularity and formation of glandular acini. The clear cells gave a strongly positive reaction to sudan III stain, but surprisingly,8 were negative to the PAS stain. Electron mi¬ croscopy revealed large amounts of introcytoplasmic lipids and numerous glyco¬ gen granules. Many microvilli were seen between the cells. It was thought that the tumor was probably a metastatic deposit from a renal carcinoma, even though the fat is usually in smaller droplets in this condition.9 Intravenous pyelograms and renal arteriograms demonstrated a space-occupy¬ ing lesion in the left kidney. A total body scan failed to show any additional metas¬ tatic deposits apart from that in the frontal sinus. A nephrectomy was per¬ formed one month after the sinus was explored, and results of histologie exami¬ nation confirmed a moderately well-differ¬ entiated renal tubular adenocarcinoma of the clear cell type.
COMMENT
The typical radiologie appearances of frontal sinus mucoceles are well known,10 and may be regarded as the result of an expanding cystic lesion that ultimately produces bone ero-
sion. With this model in mind, it can be expected that radiologie observa¬ tion of bone erosion will, to some ex¬ tent, depend on the stage of patho¬ genesis when the patient is initially examined. This may explain why the reported incidence of bone erosion in frontal mucoceles is so variable, being present in 8 of 56 patients reported by Skolnik et al,11 compared to 11 of 12 by Wolfowitz and Solomon.12 Per¬ haps it is important that these latter figures are from cases occurring in South African Negroes. Sclerosis is observed less frequently and usually interpreted as due to reactive osteitis or osteomyelitis. Hence, in the same series 11 of 5611 and two of twelve12 had sclerosis. When reading previous reports of frontal sinus cancer, it is striking that radiologie evidence of bone de¬ struction is usually discussed.1·1316 It has been reported that this finding is highly suggestive of cancer when con¬ sidering the paranasal sinuses as a whole.1 In an excellent review of 33 cases of epidermoid cancer of the frontal sinus, Brownson and Ogura6 noted radiologie evidence of bone de¬ struction in 48%, and remarked that it was usually greater than expected. In most cases, tomography had not been employed, and it can be expected that this investigation would have re¬ vealed a far greater incidence of bone
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Fig 7.—Posteroanterior projection (patient 3). Again, there is severe hyperostosis and mucosal thickening, especially at intersinus septum, with only minimal sinus ex¬
pansion.
erosion. However, in reporting four cases of frontal sinus cancer, Frew8 noted bone destruction in all four, and stated that there was nothing to distinguish the true nature of the le¬ sion radiologically. Indeed, the radiologic similarities between frontal sinus cancer and mucoceles have pre¬ viously been stressed.4 Severe sclerosis in frontal sinus cancer was observed by both Connor16 and Brunner,17 and the latter author has correctly drawn attention to other causes of this, namely, fibrous dysplasia, Paget disease, and chronic
osteomyelitis.
In view of these
reports,
a more
critical evaluation of the three cases described was undertaken. It was ob¬ served that in all cases, while the changes present were consistent with a frontal mucocele, the bone destruc¬ tion and hyperostosis were out of pro¬ portion to sinus expansion. Since it has been reported that bone erosion occurs late in the course of a muco¬ cele, this is unexpected. It is possible that this was also true in other re¬ ported instances of frontal cancer in which a provisional diagnosis of fron¬ tal osteomyelitis was made.5·6·1418 These findings are particularly strik¬ ing in patient 1, but also evident in patients 2 and 3. This observation does not deny the ample evidence that infection in a mucocele can cause rapid bone destruction,19 but none of these three patients had clinical evi¬ dence of infection. This observation clearly is not conclusive, but aware¬ ness of it may be sufficient to enable a preoperative diagnosis, especially if there are atypical features as in patients 1 and 2. During the three-year period in which these patients were observed, nine other frontal mucoceles or pyoceles were seen. This figure reflects the specialized nature of the hospital, but it does serve to emphasize the ne¬ cessity for the clinician to be alert for unusual causes of mucocele. The question of sinus of origin in cancer in this region has been de¬ bated in the past, and obviously, when there is a large tumor, it may be impossible to resolve.6 However, a review of the 21 ethmoidal carci¬ nomas that were seen at this hospital
in the same three-year period re¬ vealed only one patient in whom the frontal sinus was involved; in that in¬ stance, the tumor was extensive and involved both frontal sinuses. In both patient 1 and patient 2, the tumor ap¬ peared to originate from the floor of the sinus, and a positive biopsy speci¬ men was also obtained from periorbital fat. Clearly, it is possible that the tumor could have originated in the orbit and spread into the frontal sinus. The lack of gross tumor in the sinus at operation would support this view, though this observation has been made before in frontal sinus cancer.9·18
The intimate anatomic relationship of the orbital lobe of the lacrimal gland to the floor of the frontal sinus is not usually considered. This gland is surprisingly large, and fills the superotemporal quadrant of the orbit.20 The orbital lobe lies above the levator palpebrae superioris with its upper surface closely connected to the fron¬ tal periosteum.21 A tumor in this site would not be expected to produce the
usual radiologie findings of lacrimal gland cancer, namely, expansion 0Í the lacrimal fossa, bone destruction, and sclerosis, but would erodo directly into the frontal sinus. Subsequently, the opportunity to view the lacrimal gland in situ at three orbital exenterations has confirmed this anatomic
of particular interest since there had been a latent period of seven years with minimal proptosis prior to first
observation. Six months following
bitotomy,
pursued
a
or-
the tumor recurred and more
aggressive
course,
characteristic of recurrent tumors in
The parallel with patient 1 is obvious. Clearly, it is both possible and likely that the tumors of patients 1 and 2 arose in the orbital lobe of the lacrimal gland. If these this
area.
case
tumors of the lacrimal gland, or¬ bital exenteration would be the cor¬ rect initial surgical approach, with clearance of the sinus as necessary. A radical surgical approach to fron¬ tal sinus cancer has been advocated by both Kapur and Fairman18 and Brownson and Ogura,6 yet no one cen¬ ter has sufficient experience to state unequivocally that this would produce improved survival. It may be argued that the die is cast at presentation, and a major exenteration would only compound the patient's suffering. Certainly, the surgeon who is treat¬ ing these patients should approach the problem with palliation foremost in his mind, since radical exentera¬ tion in this area is impractical.22 The prolonged, apparently tumor free course of patient 2 illustrates this point, since ablative surgery with its resultant deformity would not have been in the patient's best interests. were
relationship. In 29
cases of lacrimal gland' cancer Mayo Clinic, Henderson26 re¬ ported one mucoepidermoid carcino¬
at the ma
The
and
one
squamous cell carcinoma.
mucoepidermoid carcinoma
was
Glynn Lloyd, FFR, originally discovered the radiologie changes. Leslie Michaels, MD, per¬ formed the histologie examination for case 3. John Wright, MD, and Donald Harrison, MD, al¬ lowed publication of details of patients under their
care.
References 1. Lewis JS, Castro EB: Cancer of the nasal cavity and paranasal sinuses. J Laryngol 86:255\x=req-\
262, 1972.
2. Frazell EL, Lewis JS: Cancer of the nasal and accessory sinuses: A report of the management of 416 patients. Cancer 16:1293\x=req-\ 1301, 1963. 3. Lederman M: Cancer of the upper jaw and nasal chambers. Proc R Soc Med 62:65-72, 1969. 4. Tamari MJ, O'Neil JJ: Abstract of mucoceles. Ill Med J 97:96-97, 1950. 5. Mitchell JF: Carcinoma of the frontal sinus. J Laryngol Otol 74:423-430, 1960. 6. Brownson RJ, Ogura JH: Primary carcinoma of the frontal sinus. Laryngoscope 81:71-89, 1971. 7. Brownson RJ: Adenocarcinoma of the prostate presenting as a frontal sinus mucocele. Laryngoscope 79:458-469, 1969. 8. Frew IJ: Frontal sinus carcinoma. J Laryngol Otol 83:383-396, 1969.
cavity
9. Connor DH, Taylor HB, Helwig EB: Cutaneous metastasis of renal cell carcinoma. Arch Pathol 76:339-346, 1963. 10. Zizmor J, Noyek AM: Cysts, benign tumors and malignant tumors of the paranasal sinuses. Otolaryngol Clin North Am 6:490-492, 1973. 11. Skolnik EM, Loewy A, Smoler J, et al: Swellings of the forehead (mucoceles). Trans Am Acad Ophthalmol Otolaryngol 69:387-395,
1965. 12. Wolfowitz BL, Solomon A: Mucoceles of the frontal and ethmoid sinus. J Laryngol Otol
86:79-82, 1972. 13. Sinha AK, Prasad GP: Carcinoma of frontal sinus. J Laryngol Otol 84:943-946, 1970. 14. Kantawala SA: Primary carcinoma of the frontal sinus: A case report. J Postgrad Med 18:99-100, 1972. 15. Bordley JE, Bosley WR: Mucoceles of the frontal sinus: Causes and treatment. Ann Otol Rhinol Laryngol 82:696-702, 1973.
Downloaded From: http://archotol.jamanetwork.com/ by a Oakland University User on 06/01/2015
16. Connor CE: Metastatic hypernephroma of right frontal, ethmoid and maxillary sinuses. Arch Otolaryngol 28:994-998, 1938. 17. Brunner H: Primary tumors of the frontal bone. Arch Otolaryngol 57:158-172, 1953. 18. Kapur YP, Fairman HD: A case of carcinoma of the frontal sinus. J Laryngol Otol
72:995-1000, 1958.
19. Pastore PN: Radiologic changes in the formation of frontal sinus mucopyocele. Laryngoscope 82:2186-1292, 1972.
20. Henderson JW: Intrinsic neoplasms of the lacrimal gland, in Orbital Tumors. Philadelphia, WB Saunders Co, 1973, pp 24, 439-442. 21. Gray H: Visual apparatus, in Warwick R, Williams PH: Grays Anatomy, ed 35. Norwich, England, Longman, Green & Co, Ltd, 1973, p 1132. 22. Harrison DF: The management of malignant tumors of the nasal sinuses. Otolaryngol Clin North Am 4:159-177, 1971.
During the period 1972 to 1974, 12 mucopyoceles were seen. Subsequently, three were shown to have an underlying neoplasm. Evaluation of the roentgenograms demonstrated minor dif\s=b\
frontal
ferences between those with and those without a malignant basis. In previous reports of frontal sinus cancer, the descriptions of the roentgenograms suggest that it is possible that these features were also present. The difficulty in deciding the sinus of origin in such cancers has often been discussed, but the possibility that neoplasms of the orbital lobe of the lacrimal gland may occur in the frontal sinus is usually not considered. Anatomically, the intimate proximity of this lobe to the frontal sinus suggests that a neoplasm could develop in this manner. I believe that this was true in two of the patients reported herein.
(Arch Otolaryngol 101:718-721, 1975)
moid carcinoma of the frontal sinus, Brownson and Ogura6 noted that in 12 patients, a preoperative diagnosis of mucocele or pyocele was made.
were thought to only one patient was cancer suspected radiologically. They concluded that the pathologist was usually the first to make the cor¬ rect diagnosis.
Clinically, only
two
have cancer, and in
I
report three additional
of cancer of the frontal sinus. In all three, a provisional diagnosis of fron¬ tal sinus mucocele was made, the true diagnosis being established histologi¬ cally. Examination of the roentgen¬ ograms of each revealed certain com¬ mon features which may be helpful in making the correct diagnosis preopercases
atively.
REPORT OF CASES
Cancer
of the frontal sinus is an uncommon condition,13 but in the reported cases, many have been
diagnosed clinically4-7 or radiologically5'8 as a frontal sinus mucocele. In a review of .33 cases of epider-
Accepted for publication May 26, 1975. From the Institute of Laryngology and Otology, London University, London. Dr Robinson is now practicing in Cairns, Queensland, Australia. Reprint requests to Mac Donnell's Building, 26 Abbott St, Cairns, Queensland 4870, Australia (Dr Robinson).
72-year-old man was first in November 1972. His left eye had been prominent for 37 years, but had not caused visual disturbance. However, in the preceding three months, he was aware of discomfort above the left eye, which was then becoming increasingly prominent. Examination showed severe proptosis with displacement of the globe inferolaterally. There was considerable chemosis, and a deficiency could be palpated in the supraorbital ridge, lateral to the supraorbital notch (Fig 1). Sinus roentgenograms showed clouding of the left frontal sinus, with considerable Case l.-A
seen
Downloaded From: http://archotol.jamanetwork.com/ by a Oakland University User on 06/01/2015
hyperostosis and destruction of the frontal bone. The appearances were thought to be due to a pyocele with periostitis, though the possibility of a neoplastic lesion could not be excluded (Fig 2). The sinus was explored through an inci¬ sion below the eyebrow. The bone of the or¬ bital roof was believed to be irregular. On entering the sinus, mucopus was found, and although the mucosa was thickened, no gross tumor
was
visible.
Histologie examination disclosed mu¬ coepidermoid carcinoma in the sinus mu¬ cosa and periorbital fat. The tumor was invading trabecular new bone from the or¬ bital roof.
Radiotherapy
with cobalt 60 as the to a maximum of 6,448 rads was de¬ livered to the affected sinus. Six weeks source
later, repeat sinus roentgenograms
were
unchanged. Tomography demonstrated considerable hyperostosis of the frontal bone and greater wing of the sphenoid bone (Fig 3). The sinus was re-explored, and a frontoethmoidectomy was performed. Al¬ though no tumor was visible, results of his¬ tologie examination confirmed the pres¬ of residual tumor. The patient's recovery was uneventful. Chemosis settled rapidly through the prop¬ tosis remained unchanged. Despite the continued presence of the tumor, he re¬ mained well and symptom free, except for a persistence of proptosis. Fourteen months later, because of recurrence of con¬ siderable chemosis and increasing prop¬ tosis, it was decided to exenterate the orbit ence
(Fig 4).
Fig
1.—Patient with
displacement of globe and chemosis.
Fig 3.—Posteroanterior tomographic cut considerable hyperostosis of frontal bone.
(patient 1) showing
Fig 2—Posteroanterior projection (patient 1). Excessive bone destruction and hyperostosis in left frontal sinus are particularly evident. Orbital roof is virtually destroyed and small osteoma can be seen. There is no sinus expansion.
Fig 4.—Recurrence of chemosis. Note absence of consequence of radiotherapy.
This procedure was performed extraperiosteally, and a gross tumor was found to be visible in the roof of the sinus. The eye¬ lids were "delashed" and sewn together. Subsequently, he enjoyed a further symp¬ tom-free period of three months, until he developed hemiparesis on the right side. Angiograms and brain and EMI scans demonstrated a space-occupying lesion in the left frontal lobe, extending by a nar¬ row track from the left orbit. A remarkable improvement was achieved by frontal craniotomy with the release of necrotic brain and pus, but histologie ex¬ amination of the brain showed invasion by mucoepidermoid carcinoma. This improvement was maintained for three months, after which he deteriorated rapidly and died in January 1975. Unfortu¬ nately, postmortem examination was not
possible.
Case 2.—A 43-year-old woman was first in August 1971. She complained of or¬ bital discomfort on the right side and epiphora of eight months' duration. She seen
also noticed a swelling above the right eye and double vision when tired. Examination disclosed a tender hard mass above the inner canthus of the right eye.
Sinus roentgenograms showed partial clouding of both frontal sinuses, with bony hyperostosis that was most severe in the region of the intersinus septum (Fig 5). There was minimal expansion of the sinus on that side. Tomography revealed erosion of the anterior wall of the frontal sinus with considerable hyperostosis (Fig 6). The appearances were believed to be consistent with a pyocele and osteitis. The sinus was explored by an incision below the eyebrow, and a tumor was en¬ countered invading the floor of the frontal sinus. Mucopus was contained within the sinus. Results of histologie examination showed well-differentiated squamous cell carcinoma invading both periorbital fat and sinus mucosa. Radiotherapy with Cobalt 60 as the source to a maximum of 6,520 rads was de-
Downloaded From: http://archotol.jamanetwork.com/ by a Oakland University User on 06/01/2015
eyebrow,
a
livered over a period of 53 days. The pa¬ tient remained well, except for persisting diplopia. Twenty-one months later, the sinus was re-explored, but no macroscopic or microscopic evidence of tumor could be found. At her most recent review, 40 months after onset, there was no evidence of recurrence. Case 3.—A 49-year-old man was first seen in October 1974. At that time, he com¬ plained of swelling around the left eye of six weeks' duration. Examination showed proptosis and dis¬ placement of the globe inferolaterally. A mucocele was felt beneath the middle of the supraorbital ridge. Sinus roentgenograms showed severe hyperostosis, thickening of mucosa (espe¬ cially in the region of the intersinus sep¬ tum), and some destruction of the frontal bone. There was only a moderate degree of sinus expansion. The appearances were be¬ lieved to be due to a mucocele (Fig 7). The sinus was explored through an inci¬ sion just below the eyebrow. On entering
Fig 5.—Posteroanterior projection (patient 2). There Is severe hyperostosis and soft tissue mass in region of interslnus septum. Although sinus is partially clouded, there Is only minimal expan¬ sion.
Fig 6 (right).—Lateral tomogram of patient 2 demonstrating ero¬ sion of anterior wall of frontal sinus associated with considerable hyperostosis. the sinus, thick mucus was encountered. The bone of the posterior wall was found to be deficient, and a small amount of white tumor was found in the region of the intersinus septum. A frontoethmoidectomy was performed, and all visible tumor was re¬ moved. Histologie examination revealed clear cell neoplasia. There was severe vascularity and formation of glandular acini. The clear cells gave a strongly positive reaction to sudan III stain, but surprisingly,8 were negative to the PAS stain. Electron mi¬ croscopy revealed large amounts of introcytoplasmic lipids and numerous glyco¬ gen granules. Many microvilli were seen between the cells. It was thought that the tumor was probably a metastatic deposit from a renal carcinoma, even though the fat is usually in smaller droplets in this condition.9 Intravenous pyelograms and renal arteriograms demonstrated a space-occupy¬ ing lesion in the left kidney. A total body scan failed to show any additional metas¬ tatic deposits apart from that in the frontal sinus. A nephrectomy was per¬ formed one month after the sinus was explored, and results of histologie exami¬ nation confirmed a moderately well-differ¬ entiated renal tubular adenocarcinoma of the clear cell type.
COMMENT
The typical radiologie appearances of frontal sinus mucoceles are well known,10 and may be regarded as the result of an expanding cystic lesion that ultimately produces bone ero-
sion. With this model in mind, it can be expected that radiologie observa¬ tion of bone erosion will, to some ex¬ tent, depend on the stage of patho¬ genesis when the patient is initially examined. This may explain why the reported incidence of bone erosion in frontal mucoceles is so variable, being present in 8 of 56 patients reported by Skolnik et al,11 compared to 11 of 12 by Wolfowitz and Solomon.12 Per¬ haps it is important that these latter figures are from cases occurring in South African Negroes. Sclerosis is observed less frequently and usually interpreted as due to reactive osteitis or osteomyelitis. Hence, in the same series 11 of 5611 and two of twelve12 had sclerosis. When reading previous reports of frontal sinus cancer, it is striking that radiologie evidence of bone de¬ struction is usually discussed.1·1316 It has been reported that this finding is highly suggestive of cancer when con¬ sidering the paranasal sinuses as a whole.1 In an excellent review of 33 cases of epidermoid cancer of the frontal sinus, Brownson and Ogura6 noted radiologie evidence of bone de¬ struction in 48%, and remarked that it was usually greater than expected. In most cases, tomography had not been employed, and it can be expected that this investigation would have re¬ vealed a far greater incidence of bone
Downloaded From: http://archotol.jamanetwork.com/ by a Oakland University User on 06/01/2015
Fig 7.—Posteroanterior projection (patient 3). Again, there is severe hyperostosis and mucosal thickening, especially at intersinus septum, with only minimal sinus ex¬
pansion.
erosion. However, in reporting four cases of frontal sinus cancer, Frew8 noted bone destruction in all four, and stated that there was nothing to distinguish the true nature of the le¬ sion radiologically. Indeed, the radiologic similarities between frontal sinus cancer and mucoceles have pre¬ viously been stressed.4 Severe sclerosis in frontal sinus cancer was observed by both Connor16 and Brunner,17 and the latter author has correctly drawn attention to other causes of this, namely, fibrous dysplasia, Paget disease, and chronic
osteomyelitis.
In view of these
reports,
a more
critical evaluation of the three cases described was undertaken. It was ob¬ served that in all cases, while the changes present were consistent with a frontal mucocele, the bone destruc¬ tion and hyperostosis were out of pro¬ portion to sinus expansion. Since it has been reported that bone erosion occurs late in the course of a muco¬ cele, this is unexpected. It is possible that this was also true in other re¬ ported instances of frontal cancer in which a provisional diagnosis of fron¬ tal osteomyelitis was made.5·6·1418 These findings are particularly strik¬ ing in patient 1, but also evident in patients 2 and 3. This observation does not deny the ample evidence that infection in a mucocele can cause rapid bone destruction,19 but none of these three patients had clinical evi¬ dence of infection. This observation clearly is not conclusive, but aware¬ ness of it may be sufficient to enable a preoperative diagnosis, especially if there are atypical features as in patients 1 and 2. During the three-year period in which these patients were observed, nine other frontal mucoceles or pyoceles were seen. This figure reflects the specialized nature of the hospital, but it does serve to emphasize the ne¬ cessity for the clinician to be alert for unusual causes of mucocele. The question of sinus of origin in cancer in this region has been de¬ bated in the past, and obviously, when there is a large tumor, it may be impossible to resolve.6 However, a review of the 21 ethmoidal carci¬ nomas that were seen at this hospital
in the same three-year period re¬ vealed only one patient in whom the frontal sinus was involved; in that in¬ stance, the tumor was extensive and involved both frontal sinuses. In both patient 1 and patient 2, the tumor ap¬ peared to originate from the floor of the sinus, and a positive biopsy speci¬ men was also obtained from periorbital fat. Clearly, it is possible that the tumor could have originated in the orbit and spread into the frontal sinus. The lack of gross tumor in the sinus at operation would support this view, though this observation has been made before in frontal sinus cancer.9·18
The intimate anatomic relationship of the orbital lobe of the lacrimal gland to the floor of the frontal sinus is not usually considered. This gland is surprisingly large, and fills the superotemporal quadrant of the orbit.20 The orbital lobe lies above the levator palpebrae superioris with its upper surface closely connected to the fron¬ tal periosteum.21 A tumor in this site would not be expected to produce the
usual radiologie findings of lacrimal gland cancer, namely, expansion 0Í the lacrimal fossa, bone destruction, and sclerosis, but would erodo directly into the frontal sinus. Subsequently, the opportunity to view the lacrimal gland in situ at three orbital exenterations has confirmed this anatomic
of particular interest since there had been a latent period of seven years with minimal proptosis prior to first
observation. Six months following
bitotomy,
pursued
a
or-
the tumor recurred and more
aggressive
course,
characteristic of recurrent tumors in
The parallel with patient 1 is obvious. Clearly, it is both possible and likely that the tumors of patients 1 and 2 arose in the orbital lobe of the lacrimal gland. If these this
area.
case
tumors of the lacrimal gland, or¬ bital exenteration would be the cor¬ rect initial surgical approach, with clearance of the sinus as necessary. A radical surgical approach to fron¬ tal sinus cancer has been advocated by both Kapur and Fairman18 and Brownson and Ogura,6 yet no one cen¬ ter has sufficient experience to state unequivocally that this would produce improved survival. It may be argued that the die is cast at presentation, and a major exenteration would only compound the patient's suffering. Certainly, the surgeon who is treat¬ ing these patients should approach the problem with palliation foremost in his mind, since radical exentera¬ tion in this area is impractical.22 The prolonged, apparently tumor free course of patient 2 illustrates this point, since ablative surgery with its resultant deformity would not have been in the patient's best interests. were
relationship. In 29
cases of lacrimal gland' cancer Mayo Clinic, Henderson26 re¬ ported one mucoepidermoid carcino¬
at the ma
The
and
one
squamous cell carcinoma.
mucoepidermoid carcinoma
was
Glynn Lloyd, FFR, originally discovered the radiologie changes. Leslie Michaels, MD, per¬ formed the histologie examination for case 3. John Wright, MD, and Donald Harrison, MD, al¬ lowed publication of details of patients under their
care.
References 1. Lewis JS, Castro EB: Cancer of the nasal cavity and paranasal sinuses. J Laryngol 86:255\x=req-\
262, 1972.
2. Frazell EL, Lewis JS: Cancer of the nasal and accessory sinuses: A report of the management of 416 patients. Cancer 16:1293\x=req-\ 1301, 1963. 3. Lederman M: Cancer of the upper jaw and nasal chambers. Proc R Soc Med 62:65-72, 1969. 4. Tamari MJ, O'Neil JJ: Abstract of mucoceles. Ill Med J 97:96-97, 1950. 5. Mitchell JF: Carcinoma of the frontal sinus. J Laryngol Otol 74:423-430, 1960. 6. Brownson RJ, Ogura JH: Primary carcinoma of the frontal sinus. Laryngoscope 81:71-89, 1971. 7. Brownson RJ: Adenocarcinoma of the prostate presenting as a frontal sinus mucocele. Laryngoscope 79:458-469, 1969. 8. Frew IJ: Frontal sinus carcinoma. J Laryngol Otol 83:383-396, 1969.
cavity
9. Connor DH, Taylor HB, Helwig EB: Cutaneous metastasis of renal cell carcinoma. Arch Pathol 76:339-346, 1963. 10. Zizmor J, Noyek AM: Cysts, benign tumors and malignant tumors of the paranasal sinuses. Otolaryngol Clin North Am 6:490-492, 1973. 11. Skolnik EM, Loewy A, Smoler J, et al: Swellings of the forehead (mucoceles). Trans Am Acad Ophthalmol Otolaryngol 69:387-395,
1965. 12. Wolfowitz BL, Solomon A: Mucoceles of the frontal and ethmoid sinus. J Laryngol Otol
86:79-82, 1972. 13. Sinha AK, Prasad GP: Carcinoma of frontal sinus. J Laryngol Otol 84:943-946, 1970. 14. Kantawala SA: Primary carcinoma of the frontal sinus: A case report. J Postgrad Med 18:99-100, 1972. 15. Bordley JE, Bosley WR: Mucoceles of the frontal sinus: Causes and treatment. Ann Otol Rhinol Laryngol 82:696-702, 1973.
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16. Connor CE: Metastatic hypernephroma of right frontal, ethmoid and maxillary sinuses. Arch Otolaryngol 28:994-998, 1938. 17. Brunner H: Primary tumors of the frontal bone. Arch Otolaryngol 57:158-172, 1953. 18. Kapur YP, Fairman HD: A case of carcinoma of the frontal sinus. J Laryngol Otol
72:995-1000, 1958.
19. Pastore PN: Radiologic changes in the formation of frontal sinus mucopyocele. Laryngoscope 82:2186-1292, 1972.
20. Henderson JW: Intrinsic neoplasms of the lacrimal gland, in Orbital Tumors. Philadelphia, WB Saunders Co, 1973, pp 24, 439-442. 21. Gray H: Visual apparatus, in Warwick R, Williams PH: Grays Anatomy, ed 35. Norwich, England, Longman, Green & Co, Ltd, 1973, p 1132. 22. Harrison DF: The management of malignant tumors of the nasal sinuses. Otolaryngol Clin North Am 4:159-177, 1971.
