290
Aust. N.Z. J . Surg. 1991.61. 290-294
PARANASAL SINUS CANCER
'
G.PATRICK BRIDCER, MARTYNs. MENDELSOHN, MICHAELBALDWINAND ROBERTSMEE4 Head and Neck Unit, Prince of Wales Hospital, Randwick, New South Wales This is a review of 65 patients with paranasal sinus malignancies who were treated by radical surgery. Most patients received either pre- or postoperative radiotherapy. Twenty-nine tumours arose in the maxillary antrum, seventeen in the ethmoid labyrinth and the remainder from the vestibule, nasal septum, lateral nasal wall and vault. There were 58 epithelial cancers. The 5-year survival rate for patients with adenocarcinoma was 78%, and 70% for antral squamous cell cancer and esthesioneuroblastoma. None of the melanoma patients survived free of disease for 5 years. The 5-year survival rate for the 21 patients undergoing orbital exenteration was 50%. compared with 70% when the eye was spared. Twelve free flap revascularized tissue transfer flaps were used to reconstruct large cranial and sino-orbital defects.
Key words: cancer, craniofacid, cribriform plate, ethmoid labyrinth, free nap, maxillectomy, max. Wary sinus, orbital exenteration, paranasal.
Introduction This is a surgical review of almost two decades from 1973 to 1988 during which time the philosophy regarding the management of paranasal sinus cancer has undergone many changes. This review also reflects the work of one surgeon who has been involved in the surgery of every case. Prior to the 1960s the often recommended treatment for paranasal sinus cancer was palatal fenestration, morcellation of tumour and intracavitary radiotherapy. However, the dismal survival results caused the pendulum to swing towards radical surgery supported by radiotherapy. Radical surgery implied total maxillectomy, orbital exenteration if the orbit was involved and ethmoidectomy where indicated. Even so. more advanced lesions with destruction of the pterygoid plates, involvement of the infratemporal fossa, bilaterality or infiltration of the cribriform plate were still considered inoperable. In 1%3 Ketcham ef al. described a combined cranial facial operation for paranasal sinus cancers involving the cribriform plate and overlying dura. A few years later Bridger and Shaheen showed that tumours involving but not penetrating the cribtiform plate could be extirpated by a transfrontal sinus facial approach.' In 1980 Bridger emphasized the importance of the total rhinotomy approach when an en bloc excision of the entire ethmoid labyrinth
'
'
Visiting Otolaryngologist. *Registrar in Otolaryngology. 'Plastic and Reconstructive Surgeon. 'Staff Radiotherapist. Correspondence: Dr G . Patrick Bridger. Suite I . 21 Kitchener Parade, Bankstown. NSW 2200. Australia. Accepted for publication 19 September 1990.
together with the septum and the nasal vault is indi~ a t e d The . ~ development of the free vascularized tissue transfer flap revolutionized the rehabilitation of the patient undergoing radical paranasal sinus cancer surgery. The unrestricted movements of viable skin muscle and bone made it possible to immediately repair enormous defects and eliminate almost all serious postoperative complications. Furthermore, the surgeon could now venture further with the limits of the excision and some patients with tumours involving the orbital apex, middle cranial fossa and cavernous sinus were now considered operable.
Methods From 1972 to 1988, 65 patients were treated by radical surgery for paranasal sinus malignancies. There were 46 males (71%) and 19 females (29%) with an average age of 58 years. The clinical staging and choice of treatment were always decided at a joint conference held by radiotherapists and surgeons. The extent of the primary tumour was determined by clinical and endoscopic examination supported by tomognuns or computerized axial tomography. The only patients excluded were those considered incurable, unfit or who refused operation. These patients were treated by radiotherapy or chemotherapy and are not included in this series. In formulating treatment policy it was decided that radical surgery followed as soon as possible with a cancerocidal course of external beam radiotherapy should give the best chances of success. This treatment strategy was instigated in 1973 and continues unchanged. Surgical procedures were classified as partial or total maxillectomy , unilateral and en bloc total
29 I
PARANASAL SINUS CANCER
ethmoidectomy, orbital exenteration and craniofacial excision. Partial maxillectomy usually included all the suprastructure above the hard palate and was used in ethmoid and nasal vault cancers that clearly spared the lower maxillary segment. Two surgical approaches were used for nasal and ethmoid cancers involving the anterior skull base. At fmt a transfrontal sinus facial operation was preferred when the cribrifonn plate was radiologically intact. Patients with definite anterior cranial cavity involvement were then considered inoperable. However, after 1978 a craniofacial operation with the assistance of a nemurgm was employed when the cribrifonn plate was infiltrated. In this series 16 patients underwent a transfrontal sinus clearance and 14 with more advanced disease had the combined operation. Forty-eight patients underwent postoperative radiotherapy. One patient received pre-operative radiotherapy and 7 patients were treated from other hospitals after failed radiotherapy. Nine patients were not irradiated. Included in this group were four T1 turnours (Table I) where the pathology specimen confmed very adequate surgical margins and 4 non-epithelial tumours which were considered radio-insensitive. One patient died postoperatively.
Table 1. Staging by site of 65 paranad sinus malignancies
No.
Site Ethmoid Antrum Vestibule Septum Lateral nasal wall
Nasal vault
17
TI
29
2 2
4
0
3
Stage 72 T3
1
T4
3 5
2
0 2 5 7 1 3 1 I 0
7
0
2
2
5
0
3
2
3 0
0 0
Tumours arising from the lateral nasal walls were mainly melanomas (72o/a) and were locally aggressive. Invasion of the ethmoid occurred in 86%, of the cribrifom plate in 71% and of the orbit in 43%. All the nasal vault tumours remained confined to the cribrifonn plate m a . with the exception of 2 cases, which spread to the ethmoids. PATHOLOGY
The cancers were divided into epithelial and mnepithelial histological types. There were 58 epithelial cancers (Table2), of which 26 were squamous cell and 22 of these arose in the antrum. In 2 patients the squamous cell cancer was associated with inverting papilloma. Twelve of the 14 adenocarcinomas occurred in the ethmoid. All of the esthesionemblastomas arose in the nasal vault. In the non-epithelial group there were 2 fibrosarcomas, 1 osteogenic sarcoma, a non-Hodglcin's lymphoma, 2 haemangiopericytomasand a malignant neurofibroma. Table 2. Histopathology of epithelial paranasal sinus malignancies Pathology
No.
Squamous cell carcinoma Antnun
26 22 1
septum Vestibule Adenocarcinoma Ethmoid
3 14
12
Antrum Melanoma Esthesioneumblastoma Basal cell carcinoma Undifferentiatedcarcinoma Ameloblastoma Adenoid cystic carcinoma
Total
2
6 4
3 3 1
1
58
STAGING AND SPREAD
Patients were staged according to the American Joint Committee TNM classification for maxillary cancer (1983). This was extended to include cancers in adjacent sites (Table 1). Most of the antral tumours presented at an advanced stage. Of the 29 cases, twenty-two were either T3 or T4 lesions. Orbital invasion occurred in 50%. Involvement of the cheek skin and ethmoid sinuses din 289'0, and in 31% the cancer penetrated the postemlateral maxillary wall to involve the infratemporal fossa and pterygoid bones. Seventeen tumours arose in the ethmoid labyrinth. Eleven of these involved the antrum. nine involved the cribriform plate and five penetrated into the orbit.
Results Survival curves were calculated by the actuarial method of Kaplan and Meier' and were compared using the log-rank test. The method allows for varying lengths of follow-up for all patients in the sample. For instance, in our series there were patients who were alive and followed-up for less than 5 years. The actuarial method incorporates their survival experience in the rates. Survival figures are cancer-specific survival. That is, any patient dying of intercurrent disease was classified as lost to follow-up at the date of death. All surgical procedures were radical en bloc excisions. In 50 patients (69%) the tumour invasion
BRIDGER ETAL.
292
necessitated resection of multiple sites. This was required with cancers of the ethmoid and nasal vault, and in 19 of the antral cancers. Only 15 patients (21%) underwent a local radical excision where the primary site only was taken. When survival was analysed according to pathology, patients with ethmoid adenocarcinoma fared the best. Their 5-year survival rate was 78% and only 2 of the 14 patients have died of their disease. The survival for antral squamous cell cancer and esthesioneuroblastoma were both in the vicinity of 70%. None of the melanoma patients survived free of disease for 5 years (Fig. 1). In the non-epithelial cases, both patients with fibrosarcoma and the patient with malignant neurofibroma died from their disease. When survival by site was plotted it resembled the survival pattern indicated by the tumour pathology, with the exception of nasal vault tumours (Fig. 2). This occurred because each site was predominantly of the one pathology. There were 22 antral squamous cell cancers of which four were T2 lesions, five were T3 and eleven were T4. The 5-year survival rates for these 3 stages were 100Y0, 80% and 58% respectively (Fig. 3). Orbital invasion occurred in 15 of the 29 antral cancers, 5 of 17 ethmoid tumours and 3 of 6 patients with melanoma. In only 2 patients was the globe able to be spared. The 5-year survival rate for the 21 patients undergoing orbital exenteration was significantlyworse than for those whose orbit could be saved (Fig. 4).
\
60
I
-
D
4
6
40
20
0
I
0
I
2
3 Years
Fig. 2. Survival by site for ( x ) ethmoid, (+) antrum and (0) vault turnours. P < 0.02 (0.015).
00
\
00
t
40
20 v -
0
I
2
3
6
4
Years
Fig. 3. Survival by stage (SCC of antrum)for stages (*) T2N0,(0)T3NO and (+) T4NO. 100
80
’
40
3.- 60
\
E
. V l .
20
0.
I
I
0 0
1
2
3
4
6
Years
Fig. 1. Survival by pathology for ( 0 ) ethmoid adenoca, (0)esthesioneuroblast, (*) melanoma and (0)antral SCC. P < 0.02 (0.01).
Fig. 4. Orbital exenteration (paranasal sinus primary) for (*) preservation ( n = 38) and (0)exenteration (n = 21) orbital status (P= 0. I).
PARANASAL SINUS CANCER
293
Prior to 1980, the reconstruction of the surgical defects was based on primary closure, skin grafts, local flaps and dental prostheses. After 1980, 12 free revascularized tissue transfer flaps have been used to close large defects. Two of the flaps were groin flaps and there were 10 tensor fascia lata flaps. The technical details of this repair have been described by Bridger and B a l d ~ i n . ~ In only 3 of 65 patients was the primary excision macroscopically incomplete. This occurred in 2 patients early in the series, both with extensive involvement of the anterior cranial cavity, who were operated upon using the limited transfrontal sinus approach. An additional patient with a T4 tumour had cancer left in the basisphenoid. Eleven patients died with either recurrent or persistent local disease, of which three also developed neck metastases and two developed distal disease. Five patients died with distal metastases with control at the primary site. Five patients who developed recurrent local disease are alive and free of disease a minimum of 3 years after further treatment. In two cases, excision of the pterygoid bone and surroundingmuscles was undertaken and a third patient required orbital exenteration. Another patient with a small recurrence on the lateral cavity wall was cured by a radiation mould attached to the dental prosthesis. The cryogenic probe was used for a small adenocarcinoma which recurred on the posterior surface of an orbital cavity defect. Iliscusion Carcinoma of the maxillary antrum is the most common paranasal sinus cancer and usually presents at an advanced stage. In this series, 22 of 29 cases were either T3 or T4 lesions and in only 2 patients was the cancer still confined to the antrum. Discernible bone destruction is a feature of this disease. In this series the survival rates of 80% and 58% for T3 and T4 tumours are exceptional. Hirohiko et al. reported an absolute 5-year survival rate of 49.5% for T3 and 24.4% for T4 maxillary sinus carcinomas treated by radiation plus surgery.6 Makoto er al. reported 5-year survival rates of 38% and 10% for T3 and T4 stages.' In the Toronto experience Beale and Garrett treated 55 cancers of the maxilla by radical radiation alone (RR)and 57 by radical radiation plus surgery (RR S).8 The 5-year survival rate was 40% for RR and 5 1% for RR + S. In the Prince of Wales series the primary radical ablative approach must be considered the integral factor in achieving the unusually high survival rates, although most cases received postoperative radiotherapy. All operations were en bloc excisions and cosmesis and function were not preserved if to do so jeopardized cure. This aggressive
+
approach has been made possible by the expansion of skull base surgery and the co-operation of many surgical disciplines. In most series the radiotherapy is delivered preoperatively. However, Sisson et al. found no significant difference between pre- and postoperative radio the rap^.^ Our rationale for preferring postoperative radiotherapy is as follows: (i) the cancer may have a high potential for radioresistance; (ii) radiotherapy is more successful when malignantly involved bone is excised; (iii) surgery permits accurate planning of postoperative radiotherapy; and (iv) there is better tissue healing when surgery precedes radiation. Prophylactic radiotherapy to the cervical lymph nodes was only given in those cases with involvement of the cheek tissues or palate. Cancer of the ethmoid is an uncommon disease but in this series it constituted 38% of all paranasal canceh. Ethmoid cancers spread to the orbit, infiltrate the nasal septum into the contralateral ethmoid and penetrate the cribriform plate. When there is tumour involvement of the sphenoid sinus, the optic nerve and vital structures in the cavernous sinus must be protected. It is a superiorly based cancer and in most patients the hard palate can be spared. When indicated the surgeon should remove the entire ethmoid labyrinth including the nasal passages and the overlying anterior cranial floor. Twelve of the ethmoid cancers were adenocarcinoma and, although this tumour is deemed less radiosensitive than epidermoid cancer, it is usually less infiltrative and less likely to recur or metastasize. The lymphatic drainage of the ethmoid sinus is to the lateral retropharyngeal nodes and then to the upper deep cervical chain. The incidence of nodal disease is, however, low. The results of treatment vary considerably. Although many authors report cures in the vicinity of 25-50%, Elner and Koch have reported sunival figures of approximately 75-82%." Our 5-year survival rate of 78% compares favourably. These improved figures have clearly resulted from the expansion of surgery into a craniofacial approach for all ethmoid cancers involving the base of the skull. Patients undergoing orbital exenteration had a 5year survival rate of only 50% compared with a survival rate of 70% for patients in whom the eye was preserved. Exenteration was undertaken in 21 patients who had more advanced disease. It has in the past been unit policy to recommend orbital exenteration when there is direct involvement of the orbital periostium. Recent publications indicate that, provided orbital invasion is minimal, the eye can still be spared without jeopardizing survival. Unfortunately, preservation does not guarantee satisfactory function. Patients undergoing intra-orbital excisions together with radical maxillectomy may have considerable postoperative orbital morbidity.
BRIDGER ETAL.
294
Ptosis of the eye causes visual assymetry and diplopia which can be further aggravated by interference with the extrinsic orbital muscualr functions. In most patients it is not possible to shield the retina and optic nerve from postoperative radiation and a significant number of these will develop optic atrophy, cataracts and visual loss. Hirohiko et al. reported com lications of brain necrosis and blindness in 2lO/o.'Improvements in methods for orbital support enable some patients to have extensive intra-orbital excisions and still preserve the globe, the optic nerve and its vascular supply. Although deprived of its rotatory function, the patient retains normal visual acuity. This is particularly indicated when the tumour involves the better or only eye. Melanoma involving the nasal and paranasal mucosa is a rare disease with a poor prognosis. Surgery with or without radiotherapy is recommended treatment. Trapp er al. re rted a 5-year disease-free survival rate of 259'0.~1nour series 4 patients with diffuse mucosal melanosis and multifocal disease did not survive. The other 2 patients with more localized disease have survived 2 and 3 years after treatment. Although the 5-year survival rate for esthesioneuroblastoma is approximately 509'0,series show that these patients ultimately succumb to local invasion or metastases. Our patients were treated by aggressive local surgery in combination with radiation therapy. To date only l patient has died with cerebral metastases; the other 3 remain well. Traditionally, the head and neck surgeon has relied heavily on prosthodontic colleagues for the satisfactory rehabilitation of the patient after maxillectomy. A major problem of the more radical excision is the reconstruction of the surgical defect. This has been overcome by the use of the mimvascular free tissue transfer skin and muscle flap.
Acknowledgements
Dr Peter Reay-Young and Professor Kevin Mead, radiotherapists, Drs Alex Gonski. John Matheson and Bernard Kwok, neurosurgeons, Drs Bamie
Milroy and Michael McGlynn, plastic and reconstructive surgeons, gave assistance. Dr Richard Fisher, Oncological Research Centre, gave statistical advice.
References 1. KETCHAMA. S., WILKINS R. H., VANBUREN J. M. & SMITHR. R. (1963) A combined intracranial facial approach to the paranasal sinus. Amer. J . Surg. 106, 698-703. G. P.& SHAHEEN0. H.(I%@ Radical sur2. BRIOGER gery for ethmoid cancer. J . Luryngol. Otol. 82, 81724. 3. BRIOGER G. P. (1980) Radical surgery for ethmoid cancer. Arch. Otolaryngol. Head Neck Surg. 106, 630-4. 4. KAPLANE. L. & MURP. J. (1958) Non parametric estimation from incomplete observations. J . Amer. Stat. Assoc. 53.457-81. 5 . BRILIGER G. P. & BALDWIN M. (1989) Anterior craniofacial resection for ethmoid and nasal cancer with free flap reconstruction. Arch. Otolaryngol. Head Neck Surg. 115, 308-12. 6. HROHIKIT., TADASHI K., TAKURO A. et al. (1986) The role of radiotherapy in the management of maxillary sinus carcinoma. Cancer 5 7 , 2 2 6 1 4 7. MAKW K.. KOICHI 0.. YUKIOI. et al. (1985) Pmgnostic factors influencing relapse of squamous cell carcinoma of the maxillary sinus. Cancer 55, 190-5. 8. BEALE F. A. & GARREITP. G. (1983) Cancer of the paranasal sinuses with particular reference to maxillary sinus cancer. J . Otolaryngol. 12, 337-82. 9. SISSON G. A.. BYTELL D. E.,BECKER S. P. & RUGED. (1976) Carcinoma of the paranasal sinuses and cranial-facial resection. J . Luryngol. Orol. 1.59-68. 10. EWERA. & KOCHH.(1974) Combined radiological and surgical therapy of cancer of the ethmoid. Acra Otolatyngol. 78. 270-6. 11. TRAPP T. K., Fu Y. S. & CALCATERRA T. C. (1987) Melanoma of the nasal and paranasal sinus mucosa. Arch. Otolaryngol.Head Neck Surg. 113, 1086-9.
Aust. N.Z. J . Surg. 1991.61. 290-294
PARANASAL SINUS CANCER
'
G.PATRICK BRIDCER, MARTYNs. MENDELSOHN, MICHAELBALDWINAND ROBERTSMEE4 Head and Neck Unit, Prince of Wales Hospital, Randwick, New South Wales This is a review of 65 patients with paranasal sinus malignancies who were treated by radical surgery. Most patients received either pre- or postoperative radiotherapy. Twenty-nine tumours arose in the maxillary antrum, seventeen in the ethmoid labyrinth and the remainder from the vestibule, nasal septum, lateral nasal wall and vault. There were 58 epithelial cancers. The 5-year survival rate for patients with adenocarcinoma was 78%, and 70% for antral squamous cell cancer and esthesioneuroblastoma. None of the melanoma patients survived free of disease for 5 years. The 5-year survival rate for the 21 patients undergoing orbital exenteration was 50%. compared with 70% when the eye was spared. Twelve free flap revascularized tissue transfer flaps were used to reconstruct large cranial and sino-orbital defects.
Key words: cancer, craniofacid, cribriform plate, ethmoid labyrinth, free nap, maxillectomy, max. Wary sinus, orbital exenteration, paranasal.
Introduction This is a surgical review of almost two decades from 1973 to 1988 during which time the philosophy regarding the management of paranasal sinus cancer has undergone many changes. This review also reflects the work of one surgeon who has been involved in the surgery of every case. Prior to the 1960s the often recommended treatment for paranasal sinus cancer was palatal fenestration, morcellation of tumour and intracavitary radiotherapy. However, the dismal survival results caused the pendulum to swing towards radical surgery supported by radiotherapy. Radical surgery implied total maxillectomy, orbital exenteration if the orbit was involved and ethmoidectomy where indicated. Even so. more advanced lesions with destruction of the pterygoid plates, involvement of the infratemporal fossa, bilaterality or infiltration of the cribriform plate were still considered inoperable. In 1%3 Ketcham ef al. described a combined cranial facial operation for paranasal sinus cancers involving the cribriform plate and overlying dura. A few years later Bridger and Shaheen showed that tumours involving but not penetrating the cribtiform plate could be extirpated by a transfrontal sinus facial approach.' In 1980 Bridger emphasized the importance of the total rhinotomy approach when an en bloc excision of the entire ethmoid labyrinth
'
'
Visiting Otolaryngologist. *Registrar in Otolaryngology. 'Plastic and Reconstructive Surgeon. 'Staff Radiotherapist. Correspondence: Dr G . Patrick Bridger. Suite I . 21 Kitchener Parade, Bankstown. NSW 2200. Australia. Accepted for publication 19 September 1990.
together with the septum and the nasal vault is indi~ a t e d The . ~ development of the free vascularized tissue transfer flap revolutionized the rehabilitation of the patient undergoing radical paranasal sinus cancer surgery. The unrestricted movements of viable skin muscle and bone made it possible to immediately repair enormous defects and eliminate almost all serious postoperative complications. Furthermore, the surgeon could now venture further with the limits of the excision and some patients with tumours involving the orbital apex, middle cranial fossa and cavernous sinus were now considered operable.
Methods From 1972 to 1988, 65 patients were treated by radical surgery for paranasal sinus malignancies. There were 46 males (71%) and 19 females (29%) with an average age of 58 years. The clinical staging and choice of treatment were always decided at a joint conference held by radiotherapists and surgeons. The extent of the primary tumour was determined by clinical and endoscopic examination supported by tomognuns or computerized axial tomography. The only patients excluded were those considered incurable, unfit or who refused operation. These patients were treated by radiotherapy or chemotherapy and are not included in this series. In formulating treatment policy it was decided that radical surgery followed as soon as possible with a cancerocidal course of external beam radiotherapy should give the best chances of success. This treatment strategy was instigated in 1973 and continues unchanged. Surgical procedures were classified as partial or total maxillectomy , unilateral and en bloc total
29 I
PARANASAL SINUS CANCER
ethmoidectomy, orbital exenteration and craniofacial excision. Partial maxillectomy usually included all the suprastructure above the hard palate and was used in ethmoid and nasal vault cancers that clearly spared the lower maxillary segment. Two surgical approaches were used for nasal and ethmoid cancers involving the anterior skull base. At fmt a transfrontal sinus facial operation was preferred when the cribrifonn plate was radiologically intact. Patients with definite anterior cranial cavity involvement were then considered inoperable. However, after 1978 a craniofacial operation with the assistance of a nemurgm was employed when the cribrifonn plate was infiltrated. In this series 16 patients underwent a transfrontal sinus clearance and 14 with more advanced disease had the combined operation. Forty-eight patients underwent postoperative radiotherapy. One patient received pre-operative radiotherapy and 7 patients were treated from other hospitals after failed radiotherapy. Nine patients were not irradiated. Included in this group were four T1 turnours (Table I) where the pathology specimen confmed very adequate surgical margins and 4 non-epithelial tumours which were considered radio-insensitive. One patient died postoperatively.
Table 1. Staging by site of 65 paranad sinus malignancies
No.
Site Ethmoid Antrum Vestibule Septum Lateral nasal wall
Nasal vault
17
TI
29
2 2
4
0
3
Stage 72 T3
1
T4
3 5
2
0 2 5 7 1 3 1 I 0
7
0
2
2
5
0
3
2
3 0
0 0
Tumours arising from the lateral nasal walls were mainly melanomas (72o/a) and were locally aggressive. Invasion of the ethmoid occurred in 86%, of the cribrifom plate in 71% and of the orbit in 43%. All the nasal vault tumours remained confined to the cribrifonn plate m a . with the exception of 2 cases, which spread to the ethmoids. PATHOLOGY
The cancers were divided into epithelial and mnepithelial histological types. There were 58 epithelial cancers (Table2), of which 26 were squamous cell and 22 of these arose in the antrum. In 2 patients the squamous cell cancer was associated with inverting papilloma. Twelve of the 14 adenocarcinomas occurred in the ethmoid. All of the esthesionemblastomas arose in the nasal vault. In the non-epithelial group there were 2 fibrosarcomas, 1 osteogenic sarcoma, a non-Hodglcin's lymphoma, 2 haemangiopericytomasand a malignant neurofibroma. Table 2. Histopathology of epithelial paranasal sinus malignancies Pathology
No.
Squamous cell carcinoma Antnun
26 22 1
septum Vestibule Adenocarcinoma Ethmoid
3 14
12
Antrum Melanoma Esthesioneumblastoma Basal cell carcinoma Undifferentiatedcarcinoma Ameloblastoma Adenoid cystic carcinoma
Total
2
6 4
3 3 1
1
58
STAGING AND SPREAD
Patients were staged according to the American Joint Committee TNM classification for maxillary cancer (1983). This was extended to include cancers in adjacent sites (Table 1). Most of the antral tumours presented at an advanced stage. Of the 29 cases, twenty-two were either T3 or T4 lesions. Orbital invasion occurred in 50%. Involvement of the cheek skin and ethmoid sinuses din 289'0, and in 31% the cancer penetrated the postemlateral maxillary wall to involve the infratemporal fossa and pterygoid bones. Seventeen tumours arose in the ethmoid labyrinth. Eleven of these involved the antrum. nine involved the cribriform plate and five penetrated into the orbit.
Results Survival curves were calculated by the actuarial method of Kaplan and Meier' and were compared using the log-rank test. The method allows for varying lengths of follow-up for all patients in the sample. For instance, in our series there were patients who were alive and followed-up for less than 5 years. The actuarial method incorporates their survival experience in the rates. Survival figures are cancer-specific survival. That is, any patient dying of intercurrent disease was classified as lost to follow-up at the date of death. All surgical procedures were radical en bloc excisions. In 50 patients (69%) the tumour invasion
BRIDGER ETAL.
292
necessitated resection of multiple sites. This was required with cancers of the ethmoid and nasal vault, and in 19 of the antral cancers. Only 15 patients (21%) underwent a local radical excision where the primary site only was taken. When survival was analysed according to pathology, patients with ethmoid adenocarcinoma fared the best. Their 5-year survival rate was 78% and only 2 of the 14 patients have died of their disease. The survival for antral squamous cell cancer and esthesioneuroblastoma were both in the vicinity of 70%. None of the melanoma patients survived free of disease for 5 years (Fig. 1). In the non-epithelial cases, both patients with fibrosarcoma and the patient with malignant neurofibroma died from their disease. When survival by site was plotted it resembled the survival pattern indicated by the tumour pathology, with the exception of nasal vault tumours (Fig. 2). This occurred because each site was predominantly of the one pathology. There were 22 antral squamous cell cancers of which four were T2 lesions, five were T3 and eleven were T4. The 5-year survival rates for these 3 stages were 100Y0, 80% and 58% respectively (Fig. 3). Orbital invasion occurred in 15 of the 29 antral cancers, 5 of 17 ethmoid tumours and 3 of 6 patients with melanoma. In only 2 patients was the globe able to be spared. The 5-year survival rate for the 21 patients undergoing orbital exenteration was significantlyworse than for those whose orbit could be saved (Fig. 4).
\
60
I
-
D
4
6
40
20
0
I
0
I
2
3 Years
Fig. 2. Survival by site for ( x ) ethmoid, (+) antrum and (0) vault turnours. P < 0.02 (0.015).
00
\
00
t
40
20 v -
0
I
2
3
6
4
Years
Fig. 3. Survival by stage (SCC of antrum)for stages (*) T2N0,(0)T3NO and (+) T4NO. 100
80
’
40
3.- 60
\
E
. V l .
20
0.
I
I
0 0
1
2
3
4
6
Years
Fig. 1. Survival by pathology for ( 0 ) ethmoid adenoca, (0)esthesioneuroblast, (*) melanoma and (0)antral SCC. P < 0.02 (0.01).
Fig. 4. Orbital exenteration (paranasal sinus primary) for (*) preservation ( n = 38) and (0)exenteration (n = 21) orbital status (P= 0. I).
PARANASAL SINUS CANCER
293
Prior to 1980, the reconstruction of the surgical defects was based on primary closure, skin grafts, local flaps and dental prostheses. After 1980, 12 free revascularized tissue transfer flaps have been used to close large defects. Two of the flaps were groin flaps and there were 10 tensor fascia lata flaps. The technical details of this repair have been described by Bridger and B a l d ~ i n . ~ In only 3 of 65 patients was the primary excision macroscopically incomplete. This occurred in 2 patients early in the series, both with extensive involvement of the anterior cranial cavity, who were operated upon using the limited transfrontal sinus approach. An additional patient with a T4 tumour had cancer left in the basisphenoid. Eleven patients died with either recurrent or persistent local disease, of which three also developed neck metastases and two developed distal disease. Five patients died with distal metastases with control at the primary site. Five patients who developed recurrent local disease are alive and free of disease a minimum of 3 years after further treatment. In two cases, excision of the pterygoid bone and surroundingmuscles was undertaken and a third patient required orbital exenteration. Another patient with a small recurrence on the lateral cavity wall was cured by a radiation mould attached to the dental prosthesis. The cryogenic probe was used for a small adenocarcinoma which recurred on the posterior surface of an orbital cavity defect. Iliscusion Carcinoma of the maxillary antrum is the most common paranasal sinus cancer and usually presents at an advanced stage. In this series, 22 of 29 cases were either T3 or T4 lesions and in only 2 patients was the cancer still confined to the antrum. Discernible bone destruction is a feature of this disease. In this series the survival rates of 80% and 58% for T3 and T4 tumours are exceptional. Hirohiko et al. reported an absolute 5-year survival rate of 49.5% for T3 and 24.4% for T4 maxillary sinus carcinomas treated by radiation plus surgery.6 Makoto er al. reported 5-year survival rates of 38% and 10% for T3 and T4 stages.' In the Toronto experience Beale and Garrett treated 55 cancers of the maxilla by radical radiation alone (RR)and 57 by radical radiation plus surgery (RR S).8 The 5-year survival rate was 40% for RR and 5 1% for RR + S. In the Prince of Wales series the primary radical ablative approach must be considered the integral factor in achieving the unusually high survival rates, although most cases received postoperative radiotherapy. All operations were en bloc excisions and cosmesis and function were not preserved if to do so jeopardized cure. This aggressive
+
approach has been made possible by the expansion of skull base surgery and the co-operation of many surgical disciplines. In most series the radiotherapy is delivered preoperatively. However, Sisson et al. found no significant difference between pre- and postoperative radio the rap^.^ Our rationale for preferring postoperative radiotherapy is as follows: (i) the cancer may have a high potential for radioresistance; (ii) radiotherapy is more successful when malignantly involved bone is excised; (iii) surgery permits accurate planning of postoperative radiotherapy; and (iv) there is better tissue healing when surgery precedes radiation. Prophylactic radiotherapy to the cervical lymph nodes was only given in those cases with involvement of the cheek tissues or palate. Cancer of the ethmoid is an uncommon disease but in this series it constituted 38% of all paranasal canceh. Ethmoid cancers spread to the orbit, infiltrate the nasal septum into the contralateral ethmoid and penetrate the cribriform plate. When there is tumour involvement of the sphenoid sinus, the optic nerve and vital structures in the cavernous sinus must be protected. It is a superiorly based cancer and in most patients the hard palate can be spared. When indicated the surgeon should remove the entire ethmoid labyrinth including the nasal passages and the overlying anterior cranial floor. Twelve of the ethmoid cancers were adenocarcinoma and, although this tumour is deemed less radiosensitive than epidermoid cancer, it is usually less infiltrative and less likely to recur or metastasize. The lymphatic drainage of the ethmoid sinus is to the lateral retropharyngeal nodes and then to the upper deep cervical chain. The incidence of nodal disease is, however, low. The results of treatment vary considerably. Although many authors report cures in the vicinity of 25-50%, Elner and Koch have reported sunival figures of approximately 75-82%." Our 5-year survival rate of 78% compares favourably. These improved figures have clearly resulted from the expansion of surgery into a craniofacial approach for all ethmoid cancers involving the base of the skull. Patients undergoing orbital exenteration had a 5year survival rate of only 50% compared with a survival rate of 70% for patients in whom the eye was preserved. Exenteration was undertaken in 21 patients who had more advanced disease. It has in the past been unit policy to recommend orbital exenteration when there is direct involvement of the orbital periostium. Recent publications indicate that, provided orbital invasion is minimal, the eye can still be spared without jeopardizing survival. Unfortunately, preservation does not guarantee satisfactory function. Patients undergoing intra-orbital excisions together with radical maxillectomy may have considerable postoperative orbital morbidity.
BRIDGER ETAL.
294
Ptosis of the eye causes visual assymetry and diplopia which can be further aggravated by interference with the extrinsic orbital muscualr functions. In most patients it is not possible to shield the retina and optic nerve from postoperative radiation and a significant number of these will develop optic atrophy, cataracts and visual loss. Hirohiko et al. reported com lications of brain necrosis and blindness in 2lO/o.'Improvements in methods for orbital support enable some patients to have extensive intra-orbital excisions and still preserve the globe, the optic nerve and its vascular supply. Although deprived of its rotatory function, the patient retains normal visual acuity. This is particularly indicated when the tumour involves the better or only eye. Melanoma involving the nasal and paranasal mucosa is a rare disease with a poor prognosis. Surgery with or without radiotherapy is recommended treatment. Trapp er al. re rted a 5-year disease-free survival rate of 259'0.~1nour series 4 patients with diffuse mucosal melanosis and multifocal disease did not survive. The other 2 patients with more localized disease have survived 2 and 3 years after treatment. Although the 5-year survival rate for esthesioneuroblastoma is approximately 509'0,series show that these patients ultimately succumb to local invasion or metastases. Our patients were treated by aggressive local surgery in combination with radiation therapy. To date only l patient has died with cerebral metastases; the other 3 remain well. Traditionally, the head and neck surgeon has relied heavily on prosthodontic colleagues for the satisfactory rehabilitation of the patient after maxillectomy. A major problem of the more radical excision is the reconstruction of the surgical defect. This has been overcome by the use of the mimvascular free tissue transfer skin and muscle flap.
Acknowledgements
Dr Peter Reay-Young and Professor Kevin Mead, radiotherapists, Drs Alex Gonski. John Matheson and Bernard Kwok, neurosurgeons, Drs Bamie
Milroy and Michael McGlynn, plastic and reconstructive surgeons, gave assistance. Dr Richard Fisher, Oncological Research Centre, gave statistical advice.
References 1. KETCHAMA. S., WILKINS R. H., VANBUREN J. M. & SMITHR. R. (1963) A combined intracranial facial approach to the paranasal sinus. Amer. J . Surg. 106, 698-703. G. P.& SHAHEEN0. H.(I%@ Radical sur2. BRIOGER gery for ethmoid cancer. J . Luryngol. Otol. 82, 81724. 3. BRIOGER G. P. (1980) Radical surgery for ethmoid cancer. Arch. Otolaryngol. Head Neck Surg. 106, 630-4. 4. KAPLANE. L. & MURP. J. (1958) Non parametric estimation from incomplete observations. J . Amer. Stat. Assoc. 53.457-81. 5 . BRILIGER G. P. & BALDWIN M. (1989) Anterior craniofacial resection for ethmoid and nasal cancer with free flap reconstruction. Arch. Otolaryngol. Head Neck Surg. 115, 308-12. 6. HROHIKIT., TADASHI K., TAKURO A. et al. (1986) The role of radiotherapy in the management of maxillary sinus carcinoma. Cancer 5 7 , 2 2 6 1 4 7. MAKW K.. KOICHI 0.. YUKIOI. et al. (1985) Pmgnostic factors influencing relapse of squamous cell carcinoma of the maxillary sinus. Cancer 55, 190-5. 8. BEALE F. A. & GARREITP. G. (1983) Cancer of the paranasal sinuses with particular reference to maxillary sinus cancer. J . Otolaryngol. 12, 337-82. 9. SISSON G. A.. BYTELL D. E.,BECKER S. P. & RUGED. (1976) Carcinoma of the paranasal sinuses and cranial-facial resection. J . Luryngol. Orol. 1.59-68. 10. EWERA. & KOCHH.(1974) Combined radiological and surgical therapy of cancer of the ethmoid. Acra Otolatyngol. 78. 270-6. 11. TRAPP T. K., Fu Y. S. & CALCATERRA T. C. (1987) Melanoma of the nasal and paranasal sinus mucosa. Arch. Otolaryngol.Head Neck Surg. 113, 1086-9.
