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Non-Epithelial Malignant Neoplasms of the Larynx
Auris'Nasus'Larynx (Tokyo) 21,
178~180
(1994)
Non-Epithelial Malignant Neoplasms of the Larynx Waldemar NAROZNY, M.D., Wojciech OZDZn\~SKI, M.D., and Boguslaw MIKASZEWSKI Department of Otorhinolaryngology, Gdansk School of Medicine, Gdansk, Poland
From 1948 to 1992, 14 patients with non-epithelial malignant neoplasm of the larynx were seen in the ENT Department of the Gdansk School of Medicine. During this period, 2,249 patients with malignant laryngeal neoplasms were treated. The choice of therapy (laryngectomy, total or partial, excision by thyrotomy, irradiation or chemotherapy) depended on the size and location of the tumor and its histopathological nature. Significant for these tumors were: unchanged mucosa covering the tumor, rare metastases to the cervical lymph nodes, difficulties in estimating a histopathological diagnosis and determination, in certain types their unifocal nature. Non-epithelial malignant neoplasms of the larynx are rare; their total frequency among all neoplasms of the larynx probably does not exceed 2%.1-3 The low incidence of these tumors, their histopathological variety, and the relatively small number of reports on bigger clinical data do not promote establishment of similar therapy methods for these tumors. The purpose of the paper was to analyze the material of 14 non-epithelial malignant laryngeal neoplasms. All the cases were analyzed and consideration given to age and sex of the patients, the primary location of tumor, histopathological diagnosis, treatment, and its result. The authors try to establish, based on the obtained data, the clinical features common for this kind of laryngeal neoplasms. MATERIALS AND METHODS
Fourteen patients with histologically proved non-epithelial malignant neoplasms of the larynx were seen at the ENT Department, Gdansk School of Medicine during the 45-year period from 1948 to 1992. During this period 2,249 patients with malignant laryneal neoplasms were examined. Table 1 shows this material. For these 14 patients, the median age was 51 years, the youngest being 31 years, and the oldest 82 years of age. There were 9 men and 5 women. Histologically, there were four cases of chondrosarcoma, three cases of rhabdomyosarcoma, two cases of fibrosarcoma, two cases of plasmocytoma, and one case each of neurofibroma sarcomatosum, lymphoblastic lymphoma, and extraske1etal chordoid sarcoma. All the tumors were covered by intact, smooth mucosa. Cervical lymph nodes were palpable in only one patient. All four chondrosarcomas were located in the subglottic region of the larynx and arose from the posterior part of the cricoid cartilage. Surgical treatments were performed: two patients underwent total laryngectomy, and the other two tumors were excised by thyrotomy. The follow-up periods ranged from 5 to 17 years. None of the patients died of the disease. In two cases with rhabdomyosarcoma the lesion was located in the supraglottis, in the third case the tumor was in supraglottis, glottis and sub glottis. In all the cases, total laryngectomy Received 13 December 1993; accepted 16 May 1994. Correspondence should be addressed to: Waldemar Narozny, Department of Otorhinolaryngology, Gdansk School of Medicine, ul D~binki 7, bud 16, 80-211 Gdansk, Poland. 178 Auris'Nasus'Larynx (Tokyo) Vol. 21 (1994)
Table 1.
Non-epithelial malignant neoplasms of the larynx.
Partient, age, sex
Site of tumor
Histopathological examinations
Ij31jM
SG
Chondrosarcoma
Total laryngectomy
5yr; NE
2j48jF
SG
Chondrosarcoma
Thyrotomy and excision
7yr; NE
3j57jF
G+SG
Chondrosarcoma
Total laryngectomy
17yr; NE
4j63 jM
SG
Chondrosarcoma
Thyrotomy and excision
5yr; NE
5j38jF
SpG
Rhabdomyosarcoma
Total laryngectomy + neck dissection, irradiation, and chemotherapy
Died after 12 months; DOD
6j54jM
SpG
Rhabdomyosarcoma
Total laryngectomy
Died after 2 months; DOD
1. Total laryngectomy +
Died after 2 months; DOD
7j57jM SG+G+SpG Rhabdomyosarcoma
Treatment
irradiation 2. Recurrence: chemotherapy
Follow-up
8j57jM
SpG
Fibrosarcoma
Total laryngectomy and excision tumor of the pharynx and esophagus
13 months; NE
9j73 jM
G+SpG
Fibrosarcoma
Total laryngectomy
Lost to follow-up
lOj82jM
SG+G
Neurofibroma sarcomatosum
I. Thyrotomy and excision 2. Recurrence: total laryngectomy
4 months; NE
IIj51jM
SpG
Solitary extramedullary Irradiation plasmocytoma
Died after 4 months; DOD
12 j60jM
SpG
Solitary extramedullary Partial laryngectomy plasmocytoma and excision tumor of the pharynx
5yr; NE
13 j82jF
SpG
Lymphoblastic lymphoma
Irradiation
5yr; NE
14j55jF
SpG
Extraskeletal chordoid sarcoma
Partial laryngectomy and neck dissection + irradiation
I yr; NE
The symbol explanation: SG, sub glottis; G, glottis; SpG, supraglottis; NE, no evidence of disease; DOD, died of disease.
was performed; in patient number 5 it was associated with unilateral neck dissection. The surgery was followed by radiotherapy in patient number 7 and combined radiotherapy and chemotherapy in patient number 5. Both the patients were treated by irradiation with 6OCO at a dose of 4,500cGy. All the patients with rhabdomyosarcoma died by the end of first year after treatment, by generalized spread of neoplasm. Two cases of fibrosarcoma were located in the supraglottis. In both patients total laryngectomy was performed, in patient number 8 it was associated with tumor excision of the pharynx and esophagus. Evaluation of therapy is difficult because the patient was lost to follow-up. Solitary extramedullary plasmocytomas in two cases were located in the supraglottis. Patient number 12 was treated by partial laryngectomy and tumor excision of the pharynx, with Auris 'Nasus 'Larynx (Tokyo) Vol. 21 (1994)
179
a good result (after 5 years there was no evidence of disease). Patient number 11 was irradiated (with 6OCO at a dose of 5,280cGy) and he died after 4 months by generalized spread of the neoplasm. The lymphoblastic lymphoma was located in the supraglottis. The result of treatment (irradiation with 6OCO at a dose of 5,280cGy) was successful. The neurofibroma sarcomatosum and extraskeletal chordoid sarcoma were treated surgically. In the first case patient was lost to follow-up, in the second case no evidence of disease has been observed for 1 year after treatment. RESULTS AND DISCUSSION
In our material, there is a predominance of sarcomas which derive from cartilaginous tissue and cross-striated muscles. Of the 14 non-epithelial malignant laryngeal tumors, there were only 2 fibrosarcomas, which corresponds with most of the literature. 1-6 Smooth, unchanged mucosa covered all the tumors which were seen in our patients; similar observations were made by other authors. 1.2 It was also difficult to obtain the proper biopsy specimen for microscopic examination. It is significant for this group of tumors that the pathologist had some difficulties in establishing a reliable diagnosis and that the clinician had to determine the unifocal nature of the tumor (in certain types).1-3,S In our material, excluding cartilaginous tumors, all the neoplasms were located in the supraglottis. Chondrosarcomas were located in the subglottis and derived from the posterior part of the cricoid cartilage. Appropriate treatment methods for malignant non-epithelial laryngeal neoplasms have not been definitively determined yet, and according to review of the literature, tumors of this same histological structure are treated in various ways in different institutes. 1-3 It is difficult to evaluate our results because of the relatively small number of patients. Remarkable is the successful surgical treatment of chondrosarcomas and failure in treatment of rhabdomyosarcomas (in spite of combination of several modalities). CONCLUSION
1) Non-epithelial malignant neoplasms of the larynx occur rarely (in our material 0.62% of all malignant tumors); 2) these tumors: a) are usually covered by intact mucosa; b) rarely metastasize to the cervical lymph nodes; c) are difficult to differentiate in histopathological examination; d) must be determined as unifocal neoplasms (in certain types). REFERENCES
Batsakis JG, Fox JE: Supporting tissue neoplasms of the larynx. Surg Gynecol Obstet 131 :989-997, 1970. 2. Burkhardt A, Meyer-Breiting E: Unusual malignant tumors. Meyer-Breiting E, Burkhardt A (eds): Tumours of the Larynx, pp 143-167, Springer-Verlag, Berlin Heidelberg, New York, London, Paris, Tokyo, 1988. 3. Gorenstein A, Neel B, Weiland L, et al: Sarcomas of the larynx. Arch Otolaryngol 106:8-12, 1980. 4. Ferlito A, Nicolai P, Barion U: Critical comments on laryngeal fibrosarcoma. Acta Otorhinolaryn1.
gol Belg 37:918-925, 1983.
5.
Michaels L: Non-epidermoid epithelial neoplasms. Michaels L (ed): Pathology of the Larynx, PP 277-340, Springer-Verlag, Berlin, Heidelberg, New York, Tokyo, 1984. 6. Shaw H: Tumours of the larynx. Ballantyne J, Groves J (eds): Scott-Brown's Diseases of the Ear, Nose and Throat. The Pharynx and the Larynx, 4th ed, pp 421-508, Butterworths, London, Boston, Sydney, Wellington, Durban, Toronto, 1979.
180 Auris·Nasus·Larynx (Tokyo) Vol. 21 (1994)
178~180
(1994)
Non-Epithelial Malignant Neoplasms of the Larynx Waldemar NAROZNY, M.D., Wojciech OZDZn\~SKI, M.D., and Boguslaw MIKASZEWSKI Department of Otorhinolaryngology, Gdansk School of Medicine, Gdansk, Poland
From 1948 to 1992, 14 patients with non-epithelial malignant neoplasm of the larynx were seen in the ENT Department of the Gdansk School of Medicine. During this period, 2,249 patients with malignant laryngeal neoplasms were treated. The choice of therapy (laryngectomy, total or partial, excision by thyrotomy, irradiation or chemotherapy) depended on the size and location of the tumor and its histopathological nature. Significant for these tumors were: unchanged mucosa covering the tumor, rare metastases to the cervical lymph nodes, difficulties in estimating a histopathological diagnosis and determination, in certain types their unifocal nature. Non-epithelial malignant neoplasms of the larynx are rare; their total frequency among all neoplasms of the larynx probably does not exceed 2%.1-3 The low incidence of these tumors, their histopathological variety, and the relatively small number of reports on bigger clinical data do not promote establishment of similar therapy methods for these tumors. The purpose of the paper was to analyze the material of 14 non-epithelial malignant laryngeal neoplasms. All the cases were analyzed and consideration given to age and sex of the patients, the primary location of tumor, histopathological diagnosis, treatment, and its result. The authors try to establish, based on the obtained data, the clinical features common for this kind of laryngeal neoplasms. MATERIALS AND METHODS
Fourteen patients with histologically proved non-epithelial malignant neoplasms of the larynx were seen at the ENT Department, Gdansk School of Medicine during the 45-year period from 1948 to 1992. During this period 2,249 patients with malignant laryneal neoplasms were examined. Table 1 shows this material. For these 14 patients, the median age was 51 years, the youngest being 31 years, and the oldest 82 years of age. There were 9 men and 5 women. Histologically, there were four cases of chondrosarcoma, three cases of rhabdomyosarcoma, two cases of fibrosarcoma, two cases of plasmocytoma, and one case each of neurofibroma sarcomatosum, lymphoblastic lymphoma, and extraske1etal chordoid sarcoma. All the tumors were covered by intact, smooth mucosa. Cervical lymph nodes were palpable in only one patient. All four chondrosarcomas were located in the subglottic region of the larynx and arose from the posterior part of the cricoid cartilage. Surgical treatments were performed: two patients underwent total laryngectomy, and the other two tumors were excised by thyrotomy. The follow-up periods ranged from 5 to 17 years. None of the patients died of the disease. In two cases with rhabdomyosarcoma the lesion was located in the supraglottis, in the third case the tumor was in supraglottis, glottis and sub glottis. In all the cases, total laryngectomy Received 13 December 1993; accepted 16 May 1994. Correspondence should be addressed to: Waldemar Narozny, Department of Otorhinolaryngology, Gdansk School of Medicine, ul D~binki 7, bud 16, 80-211 Gdansk, Poland. 178 Auris'Nasus'Larynx (Tokyo) Vol. 21 (1994)
Table 1.
Non-epithelial malignant neoplasms of the larynx.
Partient, age, sex
Site of tumor
Histopathological examinations
Ij31jM
SG
Chondrosarcoma
Total laryngectomy
5yr; NE
2j48jF
SG
Chondrosarcoma
Thyrotomy and excision
7yr; NE
3j57jF
G+SG
Chondrosarcoma
Total laryngectomy
17yr; NE
4j63 jM
SG
Chondrosarcoma
Thyrotomy and excision
5yr; NE
5j38jF
SpG
Rhabdomyosarcoma
Total laryngectomy + neck dissection, irradiation, and chemotherapy
Died after 12 months; DOD
6j54jM
SpG
Rhabdomyosarcoma
Total laryngectomy
Died after 2 months; DOD
1. Total laryngectomy +
Died after 2 months; DOD
7j57jM SG+G+SpG Rhabdomyosarcoma
Treatment
irradiation 2. Recurrence: chemotherapy
Follow-up
8j57jM
SpG
Fibrosarcoma
Total laryngectomy and excision tumor of the pharynx and esophagus
13 months; NE
9j73 jM
G+SpG
Fibrosarcoma
Total laryngectomy
Lost to follow-up
lOj82jM
SG+G
Neurofibroma sarcomatosum
I. Thyrotomy and excision 2. Recurrence: total laryngectomy
4 months; NE
IIj51jM
SpG
Solitary extramedullary Irradiation plasmocytoma
Died after 4 months; DOD
12 j60jM
SpG
Solitary extramedullary Partial laryngectomy plasmocytoma and excision tumor of the pharynx
5yr; NE
13 j82jF
SpG
Lymphoblastic lymphoma
Irradiation
5yr; NE
14j55jF
SpG
Extraskeletal chordoid sarcoma
Partial laryngectomy and neck dissection + irradiation
I yr; NE
The symbol explanation: SG, sub glottis; G, glottis; SpG, supraglottis; NE, no evidence of disease; DOD, died of disease.
was performed; in patient number 5 it was associated with unilateral neck dissection. The surgery was followed by radiotherapy in patient number 7 and combined radiotherapy and chemotherapy in patient number 5. Both the patients were treated by irradiation with 6OCO at a dose of 4,500cGy. All the patients with rhabdomyosarcoma died by the end of first year after treatment, by generalized spread of neoplasm. Two cases of fibrosarcoma were located in the supraglottis. In both patients total laryngectomy was performed, in patient number 8 it was associated with tumor excision of the pharynx and esophagus. Evaluation of therapy is difficult because the patient was lost to follow-up. Solitary extramedullary plasmocytomas in two cases were located in the supraglottis. Patient number 12 was treated by partial laryngectomy and tumor excision of the pharynx, with Auris 'Nasus 'Larynx (Tokyo) Vol. 21 (1994)
179
a good result (after 5 years there was no evidence of disease). Patient number 11 was irradiated (with 6OCO at a dose of 5,280cGy) and he died after 4 months by generalized spread of the neoplasm. The lymphoblastic lymphoma was located in the supraglottis. The result of treatment (irradiation with 6OCO at a dose of 5,280cGy) was successful. The neurofibroma sarcomatosum and extraskeletal chordoid sarcoma were treated surgically. In the first case patient was lost to follow-up, in the second case no evidence of disease has been observed for 1 year after treatment. RESULTS AND DISCUSSION
In our material, there is a predominance of sarcomas which derive from cartilaginous tissue and cross-striated muscles. Of the 14 non-epithelial malignant laryngeal tumors, there were only 2 fibrosarcomas, which corresponds with most of the literature. 1-6 Smooth, unchanged mucosa covered all the tumors which were seen in our patients; similar observations were made by other authors. 1.2 It was also difficult to obtain the proper biopsy specimen for microscopic examination. It is significant for this group of tumors that the pathologist had some difficulties in establishing a reliable diagnosis and that the clinician had to determine the unifocal nature of the tumor (in certain types).1-3,S In our material, excluding cartilaginous tumors, all the neoplasms were located in the supraglottis. Chondrosarcomas were located in the subglottis and derived from the posterior part of the cricoid cartilage. Appropriate treatment methods for malignant non-epithelial laryngeal neoplasms have not been definitively determined yet, and according to review of the literature, tumors of this same histological structure are treated in various ways in different institutes. 1-3 It is difficult to evaluate our results because of the relatively small number of patients. Remarkable is the successful surgical treatment of chondrosarcomas and failure in treatment of rhabdomyosarcomas (in spite of combination of several modalities). CONCLUSION
1) Non-epithelial malignant neoplasms of the larynx occur rarely (in our material 0.62% of all malignant tumors); 2) these tumors: a) are usually covered by intact mucosa; b) rarely metastasize to the cervical lymph nodes; c) are difficult to differentiate in histopathological examination; d) must be determined as unifocal neoplasms (in certain types). REFERENCES
Batsakis JG, Fox JE: Supporting tissue neoplasms of the larynx. Surg Gynecol Obstet 131 :989-997, 1970. 2. Burkhardt A, Meyer-Breiting E: Unusual malignant tumors. Meyer-Breiting E, Burkhardt A (eds): Tumours of the Larynx, pp 143-167, Springer-Verlag, Berlin Heidelberg, New York, London, Paris, Tokyo, 1988. 3. Gorenstein A, Neel B, Weiland L, et al: Sarcomas of the larynx. Arch Otolaryngol 106:8-12, 1980. 4. Ferlito A, Nicolai P, Barion U: Critical comments on laryngeal fibrosarcoma. Acta Otorhinolaryn1.
gol Belg 37:918-925, 1983.
5.
Michaels L: Non-epidermoid epithelial neoplasms. Michaels L (ed): Pathology of the Larynx, PP 277-340, Springer-Verlag, Berlin, Heidelberg, New York, Tokyo, 1984. 6. Shaw H: Tumours of the larynx. Ballantyne J, Groves J (eds): Scott-Brown's Diseases of the Ear, Nose and Throat. The Pharynx and the Larynx, 4th ed, pp 421-508, Butterworths, London, Boston, Sydney, Wellington, Durban, Toronto, 1979.
180 Auris·Nasus·Larynx (Tokyo) Vol. 21 (1994)