Carcinomas of the nasal septum By THOMAS A. WEIMERT, JOHN G. BATSAKIS (Ann Arbor, Michigan) and DALE H. RICE (LOS Angeles) of the nasal cavity and paranasal sinuses make up less than 1 per cent of all cancer and approximately 3 per cent of malignant neoplasms of the upper respiratory and digestive tracts. Nearly 90 per cent of the identifiable primary malignancies of the nasal cavity are squamous cell carcinomas. While they may arise from a number of sites, the mucosa of the nasal septum is a relatively unusual locus. This has been verified by Deutsch (1966) who was able to collect but 27 cases after a review of the world literature. The relative rarity of septal carcinomas precludes any single institution's ability to garner information or experience in the management of these neoplasms. In this report, we present 14 new cases collected from two teaching institutions (University of Michigan and UCLA) and extend the literature review to 1977.
MALIGNANCIES
Report of cases and analysis of data
The Table presents clinical and pathological data on 14 patients with histologically verified primary squamous cell carcinomas of the nasal septum. The patients were treated by the Departments of Otolaryngology of two University Medical Centers (The University of Michigan and UCLA) and their affiliated hospitals between 1960 and 1977. Ten of the patients were males. The age of the patients, at the time of diagnosis, ranged from 73 to 23 years. The time that signs or symptoms (mass, crusting, intermittent bleeding) preceded diagnosis varied from one month to six years. Ten of the carcinomas arose on the anterior septum and three of these involved the mucocutaneous junction. The posterior septum was the site of two carcinomas. The size of two of the neoplasms at the time of diagnosis precluded identification of a specific site of origin. Thirteen of the 14 carcinomas were histologically graded as well differentiated squamous cell carcinoma. Only one carcinoma was a nonkeratinizing carcinoma. Surgical excision was the primary modality of therapy in 12 patients. In two of these subjects, a total rhinectomy was performed. Two patients received radiotherapy after biopsy alone and two of the patients with, surgical excision received post-operative irradiation. One patient with two recurrences received external radiation as the primary form of treatment. 209
64 M 58 M 55 F 23 F 73 M 64 M 41 M 61 F 53 M
67 M
55 M
53 F
72 M 48 M
10
11
12
13 14
Afic/Scx
1 2 3 4 5 6 7 8 9
Case Histopatholoey Initial treatment
Ant. septum 1-0 cm Two-thirds of septum involved
Posterior septum 3 0 cm
Entire septum involved
Well differentiated Well differentiated
Well differentiated
Well differentiated
0
US yrs.
2
9 yrs. Died 3 yrs. Metastases to bone and brain Recurrences treated by (1) total rhinectomy and (2) maxillectomy
First recurrence treated with ext. radiation Second recurrence treated by total rhinectomy Died 1 \ years. Metastases to bilateral neck nodes, bone and lungs 2 mths.
5 yrs. 5 yrs. 5 yrs. 11 yrs. lyr8 mths. 9 yrs. 10 yrs. 2J yrs.
Follow-up
0 1 0 0 0 0 0 0 0
Recurrences
0 Local excision and irradiation 0 Partial rhinectomy Biopsy and irradiation (pt. 2 refused surgical procedure)
Total rhinectomy
Local excision Muco-cut.junction 1 • 5 cmWell differentiated Poorly differentiated Local excision Ant. septum 1 • 0 cm Post, septum 2-0 cm Well differentiated Local excision Ant. septum 1-0 cm Well differentiated Local excision Muco-cut. junction 1- 0 cm Well differentiated Local excision Muco-cut.junction 1-5cm Well differentiated Total rhinectomy Ant. septum 3 0 cm Well differentiated Local excision Ant. septum 1 • 5 cm Well differentiated Local excision Post, septum Well differentiated Local excision and irradiation Well differentiated Irradiation Ant. septum 10 cm
Site/Size
TABLE
m
n
2
X
w
>
>
p
pa H •
m
>
CARCINOMAS OF THE NASAL SEPTUM
211
Twelve patients are living without evidence of neoplasm. Two of these patients sustained recurrences during their follow-up period. Two patients are dead of their disease and both manifested disseminated metastases before death. Discussion
Squamous cell carcinoma of the nasal septum was first reported over a century ago (Gibb, 1902). With the inclusion of the 14 cases presented in this report, there are 97 cases of the entity recorded in the literature (Badib et at., 1969; Bomer and Arnold, 1967; DesPrez and Kiehn, 1967; Deutsch, 1966; Goepfert et al, 1974; Leffall and White, 1967; Lyons, 1969; MacComb and Martin, 1967; Wang, 1976; Whitcomb, 1969; Yarrington et ah, 1969). Analysis of this number of cases allows an extended perception of the disease over that presented by Deutsch (1966) when he reviewed 27 cases collected from the world literature. There is clearly a male predominance in the disorder. In the 50 cases in which the sex of the patient is identified, 38 patients were males. The majority of patients have been in their fifth to seventh decades of life at the time of diagnosis. The neoplasm is unusual before age 40. The youngest patient recorded is the 23-year-old woman of the present series (Case 4). Symptoms referable to the lesion are usually present several months before biopsy is taken. In the present scries, the duration between onset of symptoms and biopsy ranged from one month to six years. Patients often postpone seeking medical attention because early in their course their symptoms are minimal and often not unlike those of disorders they have experienced in the past, e.g. nasal polyps, rhinitis, allergy, etc. The signs most identifiable with septal carcinoma are a persistent crusting and intermittent bleeding. Ten of our 14 patients presented with one or the other or both. Not infrequently, a patient will feel a tender 'sore' or wart-like growth on the anterior septum. Pain and nasal obstruction are late manifestations. The neoplasm arises on the anterior portion of the septum in the greatest number of cases. Usually the anterior carcinomas involve the mucocutaneous junction. In this regard, it is of interest that only three of the ten patients in our group of patients with an anterior septal lesion had junctional extension. The posterior septum has been the identifiable site in six patients (two in the present series). Two growth patterns account for the majority of septal carcinomas: papillary and sessile. In these growth patterns, the neoplasm is usually a well differentiated and keratinizing squamous cell carcinoma. Nonkeratinizing or minimally keratinizing squamous cell carcinomas that are poorly differentiated are unusual to rare (Parker, 1958). The degree of cellular differentiation does not seem to correlate with the age of the patient, location and size of the neoplasm, predilection for local metastases, or prognosis. Guidance for management cannot be provided by a histological grading of the carcinoma.
212
T. A. WEIMERT, J. G. BATSAKIS AND D. H. RICE
Review of the literature indicates that approximately one-third of patients were initially treated by surgical excision alone, one-third with radiation therapy (external supervoltage or radium implants) and onethird by a combination of irradiation and surgical excision. The results of radiotherapy versus surgical excision in publications cannot be considered as criteria for a comparison of their relative effectiveness. Surgical excision has been employed for early lesions while irradiation or combination therapy has most often been reserved for more advanced disease. Capps and Williams (1950), utilizing external radiation, reported a less than 50 per cent cure rate. Far more favourable results have been reported by Parker (1958) and Wang (1976). Whitcomb (1969) and others have enjoyed therapeutic success with small (less than 2-5 cm) lesions with the use of radium implants. Combination therapy with surgical excision following radiotherapy has been advocated by Lyons (1969) for all primary septal carcinomas. Most therapists reserve this regimen for advanced lesions. Following the precepts of MacComb and Martin (1942) and reiterated by Devine et al. (1957) squamous cell carcinomas confined to the nasal septum have most often been managed by surgical excision. In this respect, Sooy (1950) has stressed the importance of a sufficiently radical initial resection. Small carcinomas at or near the mucocutaneous junction are especially dangerous. The margins of clearance are difficult to identify because of submucosal infiltration and spread along fascial planes. Local excision should minimally consist of a one centimeter cuff of uninvolved mucosa and the underlying mucoperichondrium and cartilage, with preservation of the contra-lateral mucosa. Early cancer of the anterior nasal septum is treated equally well by external radiation therapy, radium needle implants, or surgical excision. The choice of treatment often depends upon the expected cosmetic result. The advantage of a surgical excision is that there is no subsequent nasal dryness and late chondritis. The prognosis of a patient with carcinoma of the nasal septum correlates inversely with the size of the tumour at diagnosis and the presence of metastases. Regional metastases may present early or relatively late in the course of the disease. They occur in approximately 10 per cent of reported cases. Spread is generally first to the perivascular lymph node associated with the facial artery at the notch of the mandible or to lymph nodes overlying the parotid gland. Jugulo-digastric and submandibular lymph nodes may also be involved. Because of the midline position of the lesion, metastases may occur in either side of the neck. For this reason and because of the low incidence of metastases, a prophylactic neck dissection should not be performed, but be reserved for those patients with clinical evidence of lymph node involvement. Regardless of the mode of treatment or combination of techniques,
CARCINOMAS OF THE NASAL SEPTUM
213
metastatic disease almost excludes a successful outcome. Including two of our patients (Cases 11 and 14), only two of ten reported patients with metastases were free of neoplasm at the time of publication. Summary
Fourteen new cases of primary squamous cell carcinoma of the nasal septum arc presented. Their inclusion brings the total reported in the literature to 97. Early, confined neoplasms are amenable to cure by surgical excision, external irradiation or radium implants. Prognosis does not correlate with histological grade of the neoplasm but is inversely related to the size of the carcinoma at the time of diagnosis and the presence of metastases. REFERENCES BADIB, A. Q., KUROHARA, S. S., WEBSTER, J. H. and SHEDD, D. P. (1969) American Journal of
Roentgenology, 106, 824. BOMER, D. L., and ARNOLD, G. E. (1967) Ada Otolaryngologica (Supplement), Stockholm, 289. CAPPS, F. C. W., and WILLIAMS, I. G. (1950) Proceedings Royal Society of Medicine, 43, 665. DES PREZ, J. D., and KIEHN, C. L. (1967) American Journal of Surgery, 114, 587. DEUTSCH, H. J. (1966) Annals of Otology, Rhinology and Laryngology, 75, 1049. DEVINE, K. D., SCANLO.N, P. W., and FIGI, F. A. (1957) Journal of the American Medical Association, 163, 617. GIBB, J. S. (1902) New York State Journal of Medicine, 2, 24. GoEPFtRT, H., GUILLAMONDEQUI, O. M., JESSE, R. H., and LINDBERG, R. D. (1974) Archives of
Otolaryngology, 100, 8. LEFFALL, L. D., Jr., and WHITE, J. E. (1966) American Journal of Surgery, 112, 43fi. LYONS, G. D. (1969) Archives of Otolaryngology, 89, 47. MACCOMB, W. S., and MARTTN, H, E. (1942) American Journal of Roentgenology, 47, 11. MACCOMB, W. S., and MARTIN, H. E. (1967) Cancer of the Head and Neck. Williams and Wilkins, Baltimore, pp. 334-356. PARKER, R. G. (1958) American Journal of Roentgenology, 80, 766. SOOY, F. A. (1950) Laryngoscope, 60, 964. WANG, C. C. (1976) Cancer, 38, 100. WHITCOMB, W. P. (1969) American Journal of Roentgenology, 105, 550. YARRINGTON, C. T., JAQUISS, G. W., and SPRINKLE, P. M. (1969) Transactions American
Academy Ophthalmology and Otolaryngology, 73, 1178.
MALIGNANCIES
Report of cases and analysis of data
The Table presents clinical and pathological data on 14 patients with histologically verified primary squamous cell carcinomas of the nasal septum. The patients were treated by the Departments of Otolaryngology of two University Medical Centers (The University of Michigan and UCLA) and their affiliated hospitals between 1960 and 1977. Ten of the patients were males. The age of the patients, at the time of diagnosis, ranged from 73 to 23 years. The time that signs or symptoms (mass, crusting, intermittent bleeding) preceded diagnosis varied from one month to six years. Ten of the carcinomas arose on the anterior septum and three of these involved the mucocutaneous junction. The posterior septum was the site of two carcinomas. The size of two of the neoplasms at the time of diagnosis precluded identification of a specific site of origin. Thirteen of the 14 carcinomas were histologically graded as well differentiated squamous cell carcinoma. Only one carcinoma was a nonkeratinizing carcinoma. Surgical excision was the primary modality of therapy in 12 patients. In two of these subjects, a total rhinectomy was performed. Two patients received radiotherapy after biopsy alone and two of the patients with, surgical excision received post-operative irradiation. One patient with two recurrences received external radiation as the primary form of treatment. 209
64 M 58 M 55 F 23 F 73 M 64 M 41 M 61 F 53 M
67 M
55 M
53 F
72 M 48 M
10
11
12
13 14
Afic/Scx
1 2 3 4 5 6 7 8 9
Case Histopatholoey Initial treatment
Ant. septum 1-0 cm Two-thirds of septum involved
Posterior septum 3 0 cm
Entire septum involved
Well differentiated Well differentiated
Well differentiated
Well differentiated
0
US yrs.
2
9 yrs. Died 3 yrs. Metastases to bone and brain Recurrences treated by (1) total rhinectomy and (2) maxillectomy
First recurrence treated with ext. radiation Second recurrence treated by total rhinectomy Died 1 \ years. Metastases to bilateral neck nodes, bone and lungs 2 mths.
5 yrs. 5 yrs. 5 yrs. 11 yrs. lyr8 mths. 9 yrs. 10 yrs. 2J yrs.
Follow-up
0 1 0 0 0 0 0 0 0
Recurrences
0 Local excision and irradiation 0 Partial rhinectomy Biopsy and irradiation (pt. 2 refused surgical procedure)
Total rhinectomy
Local excision Muco-cut.junction 1 • 5 cmWell differentiated Poorly differentiated Local excision Ant. septum 1 • 0 cm Post, septum 2-0 cm Well differentiated Local excision Ant. septum 1-0 cm Well differentiated Local excision Muco-cut. junction 1- 0 cm Well differentiated Local excision Muco-cut.junction 1-5cm Well differentiated Total rhinectomy Ant. septum 3 0 cm Well differentiated Local excision Ant. septum 1 • 5 cm Well differentiated Local excision Post, septum Well differentiated Local excision and irradiation Well differentiated Irradiation Ant. septum 10 cm
Site/Size
TABLE
m
n
2
X
w
>
>
p
pa H •
m
>
CARCINOMAS OF THE NASAL SEPTUM
211
Twelve patients are living without evidence of neoplasm. Two of these patients sustained recurrences during their follow-up period. Two patients are dead of their disease and both manifested disseminated metastases before death. Discussion
Squamous cell carcinoma of the nasal septum was first reported over a century ago (Gibb, 1902). With the inclusion of the 14 cases presented in this report, there are 97 cases of the entity recorded in the literature (Badib et at., 1969; Bomer and Arnold, 1967; DesPrez and Kiehn, 1967; Deutsch, 1966; Goepfert et al, 1974; Leffall and White, 1967; Lyons, 1969; MacComb and Martin, 1967; Wang, 1976; Whitcomb, 1969; Yarrington et ah, 1969). Analysis of this number of cases allows an extended perception of the disease over that presented by Deutsch (1966) when he reviewed 27 cases collected from the world literature. There is clearly a male predominance in the disorder. In the 50 cases in which the sex of the patient is identified, 38 patients were males. The majority of patients have been in their fifth to seventh decades of life at the time of diagnosis. The neoplasm is unusual before age 40. The youngest patient recorded is the 23-year-old woman of the present series (Case 4). Symptoms referable to the lesion are usually present several months before biopsy is taken. In the present scries, the duration between onset of symptoms and biopsy ranged from one month to six years. Patients often postpone seeking medical attention because early in their course their symptoms are minimal and often not unlike those of disorders they have experienced in the past, e.g. nasal polyps, rhinitis, allergy, etc. The signs most identifiable with septal carcinoma are a persistent crusting and intermittent bleeding. Ten of our 14 patients presented with one or the other or both. Not infrequently, a patient will feel a tender 'sore' or wart-like growth on the anterior septum. Pain and nasal obstruction are late manifestations. The neoplasm arises on the anterior portion of the septum in the greatest number of cases. Usually the anterior carcinomas involve the mucocutaneous junction. In this regard, it is of interest that only three of the ten patients in our group of patients with an anterior septal lesion had junctional extension. The posterior septum has been the identifiable site in six patients (two in the present series). Two growth patterns account for the majority of septal carcinomas: papillary and sessile. In these growth patterns, the neoplasm is usually a well differentiated and keratinizing squamous cell carcinoma. Nonkeratinizing or minimally keratinizing squamous cell carcinomas that are poorly differentiated are unusual to rare (Parker, 1958). The degree of cellular differentiation does not seem to correlate with the age of the patient, location and size of the neoplasm, predilection for local metastases, or prognosis. Guidance for management cannot be provided by a histological grading of the carcinoma.
212
T. A. WEIMERT, J. G. BATSAKIS AND D. H. RICE
Review of the literature indicates that approximately one-third of patients were initially treated by surgical excision alone, one-third with radiation therapy (external supervoltage or radium implants) and onethird by a combination of irradiation and surgical excision. The results of radiotherapy versus surgical excision in publications cannot be considered as criteria for a comparison of their relative effectiveness. Surgical excision has been employed for early lesions while irradiation or combination therapy has most often been reserved for more advanced disease. Capps and Williams (1950), utilizing external radiation, reported a less than 50 per cent cure rate. Far more favourable results have been reported by Parker (1958) and Wang (1976). Whitcomb (1969) and others have enjoyed therapeutic success with small (less than 2-5 cm) lesions with the use of radium implants. Combination therapy with surgical excision following radiotherapy has been advocated by Lyons (1969) for all primary septal carcinomas. Most therapists reserve this regimen for advanced lesions. Following the precepts of MacComb and Martin (1942) and reiterated by Devine et al. (1957) squamous cell carcinomas confined to the nasal septum have most often been managed by surgical excision. In this respect, Sooy (1950) has stressed the importance of a sufficiently radical initial resection. Small carcinomas at or near the mucocutaneous junction are especially dangerous. The margins of clearance are difficult to identify because of submucosal infiltration and spread along fascial planes. Local excision should minimally consist of a one centimeter cuff of uninvolved mucosa and the underlying mucoperichondrium and cartilage, with preservation of the contra-lateral mucosa. Early cancer of the anterior nasal septum is treated equally well by external radiation therapy, radium needle implants, or surgical excision. The choice of treatment often depends upon the expected cosmetic result. The advantage of a surgical excision is that there is no subsequent nasal dryness and late chondritis. The prognosis of a patient with carcinoma of the nasal septum correlates inversely with the size of the tumour at diagnosis and the presence of metastases. Regional metastases may present early or relatively late in the course of the disease. They occur in approximately 10 per cent of reported cases. Spread is generally first to the perivascular lymph node associated with the facial artery at the notch of the mandible or to lymph nodes overlying the parotid gland. Jugulo-digastric and submandibular lymph nodes may also be involved. Because of the midline position of the lesion, metastases may occur in either side of the neck. For this reason and because of the low incidence of metastases, a prophylactic neck dissection should not be performed, but be reserved for those patients with clinical evidence of lymph node involvement. Regardless of the mode of treatment or combination of techniques,
CARCINOMAS OF THE NASAL SEPTUM
213
metastatic disease almost excludes a successful outcome. Including two of our patients (Cases 11 and 14), only two of ten reported patients with metastases were free of neoplasm at the time of publication. Summary
Fourteen new cases of primary squamous cell carcinoma of the nasal septum arc presented. Their inclusion brings the total reported in the literature to 97. Early, confined neoplasms are amenable to cure by surgical excision, external irradiation or radium implants. Prognosis does not correlate with histological grade of the neoplasm but is inversely related to the size of the carcinoma at the time of diagnosis and the presence of metastases. REFERENCES BADIB, A. Q., KUROHARA, S. S., WEBSTER, J. H. and SHEDD, D. P. (1969) American Journal of
Roentgenology, 106, 824. BOMER, D. L., and ARNOLD, G. E. (1967) Ada Otolaryngologica (Supplement), Stockholm, 289. CAPPS, F. C. W., and WILLIAMS, I. G. (1950) Proceedings Royal Society of Medicine, 43, 665. DES PREZ, J. D., and KIEHN, C. L. (1967) American Journal of Surgery, 114, 587. DEUTSCH, H. J. (1966) Annals of Otology, Rhinology and Laryngology, 75, 1049. DEVINE, K. D., SCANLO.N, P. W., and FIGI, F. A. (1957) Journal of the American Medical Association, 163, 617. GIBB, J. S. (1902) New York State Journal of Medicine, 2, 24. GoEPFtRT, H., GUILLAMONDEQUI, O. M., JESSE, R. H., and LINDBERG, R. D. (1974) Archives of
Otolaryngology, 100, 8. LEFFALL, L. D., Jr., and WHITE, J. E. (1966) American Journal of Surgery, 112, 43fi. LYONS, G. D. (1969) Archives of Otolaryngology, 89, 47. MACCOMB, W. S., and MARTTN, H, E. (1942) American Journal of Roentgenology, 47, 11. MACCOMB, W. S., and MARTIN, H. E. (1967) Cancer of the Head and Neck. Williams and Wilkins, Baltimore, pp. 334-356. PARKER, R. G. (1958) American Journal of Roentgenology, 80, 766. SOOY, F. A. (1950) Laryngoscope, 60, 964. WANG, C. C. (1976) Cancer, 38, 100. WHITCOMB, W. P. (1969) American Journal of Roentgenology, 105, 550. YARRINGTON, C. T., JAQUISS, G. W., and SPRINKLE, P. M. (1969) Transactions American
Academy Ophthalmology and Otolaryngology, 73, 1178.
