Extensive malignant schwannoma of the mandibular nerve
M a s a n o b u Ohnishi 1, Yoshihiro T a n a k a 1, Tutomu Tutui 2, S a d a h i k o Bann 3 Department of 1Dentistry and Maxillo-Faeial Surgery, 2Department of Pediatrics, 3Department of Neurosurgery, Kobe City General Hospital, Kobe, Japan
Case report M. OhnishL Y. Tanaka, 77. Tutug S. Bann: Extensive malignant schwannoma o f the mandibular nerve. Int. J. Oral Maxillofac. Surg. 1992; 21:280 281. Abstract. An extensive malignant schwannoma of the mandibular nerve in an 8year-old boy is presented. The clinical presentation, light microscopic findings, and radiographic features are described. The patient was successfully treated with surgery in combination with chemotherapy. He is still well, without evidence of metastasis or local recurrence, 5 years postoperatively.
Malignant schwannomas or neurofibrosarcomas originate from Schwann cells or nerve sheath cells and are extremely rare in the maxillofacial region. A malignant schwannoma usually presents as a painless, expanding mass. Symptoms of weakness, atrophy, or paresthesia may occur in the areas involved. A b o u t one-half of malignant schwannomas develop in people suffering from von Recklinghausen's disease. As with other sarcomas, a high percentage of these patients experience local recurrences and also distant metastases. Results of treatment by radiation and chemotherapy have been disappointing. The case of an 8-year-old Japanese boy is described in which the tumor arose from the mandibular nerve. Case report An 8-year-old Japanese boy presented to the neurosurgery department, complaining of ptosis with limited upward movement of his upper left eyelid (Fig. 1). He had been well, without complaints, until a few days before, when ptosis became visible. The past history was noncontributory and the family history revealed no neurologic diseases. His left pupil was slightly dilated and the visual acuity field was narrow. Neurologic examination revealed functional impairment of the third and fifth cranial nerves and dysesthesia of the left lower lip. No enlarged lymph nodes were palpable in the cervical region. A careful intraoral and extraoral examination detected no masses and no bony expansion of the mandible. Physical examination failed to reveal any
signs or symptoms compatible with neurofibromatosis. The radiographs showed an expanded mandibular canal and expanded foramen ovale (Fig. 2). Computed tomography showed that an extensive mass occupied the pterygomaxillary space in continuity with the expanded mandibular canal and the expanded foramen ovale. The intracranial tumor was removed by neurosurgeons acting under the tentative diagnosis of benign trigeminal neurinoma. The histology revealed the tumor to be highly cellular, comprising spindle-shaped cells with nuclear hyperchromatism and frequent mitoses (Fig. 3). Immunoperoxidase staining for the neural crest marker S-100 protein was positive, whereas NSE was negative. The tumor was diagnosed as malignant schwannoma. The patient was treated with one course of T-9 combination chemotherapy ~~,after which the extracranial residual tumor was removed in a delayed salvage operation 2 months later. Postoperatively, he was treated with T-9 combination chemotherapy for a total of four cycles. He has been followed up for a period of 5 years and so far has been free of symptoms and has shown no evidence of metastasis.
Key words: malignant schwannoma; mandibular nerve; adjuvant chemotherapy. Accepted for publication 15 June 1992
but involvement of the mandibular nerve is extremely rare ~,3,5,9,x°J2,~3. Only three cases, including that presented here, have been described in which the t u m o r arose in a child under 10 years old3.5,6. Radiologically the t u m o r does not differ m u c h from a central benign tumor, although widening of the inferior
Discussion Malignant schwannomas are tumors of Schwann cells and may arise anywhere in the body. There appears to be no sex predilection, and most patients suffer from neurofibromatosis (von Recklinghausen's disease) and are aged 30-50 years 2'4J4. GRATZ et al. 9 mentioned that only 26 cases of malignant schwannoma of the oral cavity have been reported,
Fig. 1. Front view of 8-year-old Japanese boy with sudden ptosis and limited upward movement of upper left eyelid. Neurologic examination revealed functional impairment of third and fifth cranial nerves, and dysesthesia of left lower lip.
Malignant sehwannoma of mandibular nerve
281
university hospitals. Laryngoscope 1991: 101: 1044-9. 2. BOJSEN-MOLLERM, MYHRE-JENSENO. A
consecutive series of 30 malignant schwannomas. Acta Pathol Microbiol Immunol Scand 1984: 92: 147-55. 3. COLMENERO C, RIVERS T, PATRON M,
SIERRAI, GAMALLOC. Maxillofacial malignant peripheral nerve sheath tumours. J Craniomaxillofac Surg 1991: 19:40 6. 4. DAS GUPUTA TK. Tumors of the peripheral nerves. Clin Neurosurg 1977: 25: 574-90. 5. DAVID D J, SPECULAND B, VERNONROBERTSB, SACHRP. Malignant schwannoma of the inferior dental nerve. Br J Plast Surg 1978: 31:323 33. 6. DEWANSK, BIHANIVK, MEHTAPA. Ma-
Fig. 2. Panoramic radiograph showing expanded mandibular canal.
alveolar canal is an important radiographic feature. DAVID et al. 5 classified the radiologic findings of malignant schwannoma o f the inferior alveolar nerve into two varieties. There may be either a cystic lesion of the mandible or a more obvious cylindrical enlargement of the mandibular canal. Both radiographic features were shown in the case presented (Fig. 2). Malignant schwannomas are highly aggressive tumors. They may ap-
pear as a circumscribed mass arising from a nerve. The t u m o r often seems to be encapsulated, but this usually is pseudoencapsulation secondary to compression of surrounding structures. They tend to recur locally within a relatively short time. The 5-year survival rate of these tumors is reported to vary from 37.6% TM to 65.7% 7 in general, but the prognosis of those arising from the inferior alveolar nerve is very poor. Only two patients, including the patient presented, survived beyond 5 years 1'3'5'6'1°'12. To date, chemotherapy seems to have a limited role in the management of this tumor; however, occasional responses have been reported with Adriamycincontaining drug combinations 8. SOROILCO et al. 14 reported that the results of adjuvant chemotherapy after surgery appeared to be encouraging, and they felt that adjuvant chemotherapy should be considered in patients with malignant schwannomas, especially in cases of von Recklinghausen's disease. A wide surgical resection, however, is still the preferred m e t h o d of treatment, augmented by combination chemotherapy.
Acknowledgment. The authors are grateful to Dr SHIRANEfor valuable guidance in histologic examination.
Fig. 3. Microphotograph (HE x 40) of surgical specimen showing tumor mass to be highly cellular, comprising spindle-shaped cells with nuclear hyperchromatism and frequent mitoses (insert HE x 200).
References 1. BAILET JW, ABEMAYORE, ANDREWSJC, ROWLAND JP, Fu YS, DAWSONDE. Malignant nerve sheath tumors of head and
neck: a combined experience from two
lignant schwannoma of the inferior alveolarnerve: report of a case. J Oral Med
1983: 38: 90-3. 7. GrIosu BC, GHosrr L, Huvos AG, FORTHORJG. Malignant schwannoma. A clinicopathological study. Cancer 1973: 31: 184-90. 8. GOLDMANRL, JONES SE, HEUS~NKVELD RS. Combination chemotherapy of metastatic malignant schwannoma with vincristine, Adriamycin cyclophosphamide, and imidazole carboxamide; a case report. Cancer 1977: 39:1955 8. 9. GRATZ KW, MAK~K M, SAILERHF. Malignant meranotic schwannoma of the oral cavity. Int J Oral Maxillofac Surg 1991: 20: 236-8. 10. KamuoDe CS. Malignant schwannoma of the trigeminal nerve. Otolaryngol Head Neck Surg 1979: 87: 594-8. 11. ROSENG, CAPARROSB, NIRENBERGA, et al. Ewing's sarcoma: ten-year experience with adjuvant chemotherapy. Cancer 1981: 47: 2204-13. 12. ROBERTSON I, COOK MG, WILSON DF, HENDERSONDW. Malignant schwannoma of cranial nerves. Pathology 1983: 15: 421-9. 13. SIRASUNAK, FUKUDAY, KITAMURAR, et al. Malignant schwannoma of the mandible. Int J Oral Maxillofac Surg 1986: 15: 77~6. 14. SORDILLOPP, HELSONL, HAJDUSI, et al. Malignant schwannoma - clinical characteristics, survival, and response to therapy. Cancer 1981: 47: 25034. Address: Dr Masanobu Ohn&hi Department of Dentistry and Maxillo-Facial Surgery Kobe City General Hospital 6 6 Minatojima-Nakamachi Chuo-ku, Kobe, 650 Japan
M a s a n o b u Ohnishi 1, Yoshihiro T a n a k a 1, Tutomu Tutui 2, S a d a h i k o Bann 3 Department of 1Dentistry and Maxillo-Faeial Surgery, 2Department of Pediatrics, 3Department of Neurosurgery, Kobe City General Hospital, Kobe, Japan
Case report M. OhnishL Y. Tanaka, 77. Tutug S. Bann: Extensive malignant schwannoma o f the mandibular nerve. Int. J. Oral Maxillofac. Surg. 1992; 21:280 281. Abstract. An extensive malignant schwannoma of the mandibular nerve in an 8year-old boy is presented. The clinical presentation, light microscopic findings, and radiographic features are described. The patient was successfully treated with surgery in combination with chemotherapy. He is still well, without evidence of metastasis or local recurrence, 5 years postoperatively.
Malignant schwannomas or neurofibrosarcomas originate from Schwann cells or nerve sheath cells and are extremely rare in the maxillofacial region. A malignant schwannoma usually presents as a painless, expanding mass. Symptoms of weakness, atrophy, or paresthesia may occur in the areas involved. A b o u t one-half of malignant schwannomas develop in people suffering from von Recklinghausen's disease. As with other sarcomas, a high percentage of these patients experience local recurrences and also distant metastases. Results of treatment by radiation and chemotherapy have been disappointing. The case of an 8-year-old Japanese boy is described in which the tumor arose from the mandibular nerve. Case report An 8-year-old Japanese boy presented to the neurosurgery department, complaining of ptosis with limited upward movement of his upper left eyelid (Fig. 1). He had been well, without complaints, until a few days before, when ptosis became visible. The past history was noncontributory and the family history revealed no neurologic diseases. His left pupil was slightly dilated and the visual acuity field was narrow. Neurologic examination revealed functional impairment of the third and fifth cranial nerves and dysesthesia of the left lower lip. No enlarged lymph nodes were palpable in the cervical region. A careful intraoral and extraoral examination detected no masses and no bony expansion of the mandible. Physical examination failed to reveal any
signs or symptoms compatible with neurofibromatosis. The radiographs showed an expanded mandibular canal and expanded foramen ovale (Fig. 2). Computed tomography showed that an extensive mass occupied the pterygomaxillary space in continuity with the expanded mandibular canal and the expanded foramen ovale. The intracranial tumor was removed by neurosurgeons acting under the tentative diagnosis of benign trigeminal neurinoma. The histology revealed the tumor to be highly cellular, comprising spindle-shaped cells with nuclear hyperchromatism and frequent mitoses (Fig. 3). Immunoperoxidase staining for the neural crest marker S-100 protein was positive, whereas NSE was negative. The tumor was diagnosed as malignant schwannoma. The patient was treated with one course of T-9 combination chemotherapy ~~,after which the extracranial residual tumor was removed in a delayed salvage operation 2 months later. Postoperatively, he was treated with T-9 combination chemotherapy for a total of four cycles. He has been followed up for a period of 5 years and so far has been free of symptoms and has shown no evidence of metastasis.
Key words: malignant schwannoma; mandibular nerve; adjuvant chemotherapy. Accepted for publication 15 June 1992
but involvement of the mandibular nerve is extremely rare ~,3,5,9,x°J2,~3. Only three cases, including that presented here, have been described in which the t u m o r arose in a child under 10 years old3.5,6. Radiologically the t u m o r does not differ m u c h from a central benign tumor, although widening of the inferior
Discussion Malignant schwannomas are tumors of Schwann cells and may arise anywhere in the body. There appears to be no sex predilection, and most patients suffer from neurofibromatosis (von Recklinghausen's disease) and are aged 30-50 years 2'4J4. GRATZ et al. 9 mentioned that only 26 cases of malignant schwannoma of the oral cavity have been reported,
Fig. 1. Front view of 8-year-old Japanese boy with sudden ptosis and limited upward movement of upper left eyelid. Neurologic examination revealed functional impairment of third and fifth cranial nerves, and dysesthesia of left lower lip.
Malignant sehwannoma of mandibular nerve
281
university hospitals. Laryngoscope 1991: 101: 1044-9. 2. BOJSEN-MOLLERM, MYHRE-JENSENO. A
consecutive series of 30 malignant schwannomas. Acta Pathol Microbiol Immunol Scand 1984: 92: 147-55. 3. COLMENERO C, RIVERS T, PATRON M,
SIERRAI, GAMALLOC. Maxillofacial malignant peripheral nerve sheath tumours. J Craniomaxillofac Surg 1991: 19:40 6. 4. DAS GUPUTA TK. Tumors of the peripheral nerves. Clin Neurosurg 1977: 25: 574-90. 5. DAVID D J, SPECULAND B, VERNONROBERTSB, SACHRP. Malignant schwannoma of the inferior dental nerve. Br J Plast Surg 1978: 31:323 33. 6. DEWANSK, BIHANIVK, MEHTAPA. Ma-
Fig. 2. Panoramic radiograph showing expanded mandibular canal.
alveolar canal is an important radiographic feature. DAVID et al. 5 classified the radiologic findings of malignant schwannoma o f the inferior alveolar nerve into two varieties. There may be either a cystic lesion of the mandible or a more obvious cylindrical enlargement of the mandibular canal. Both radiographic features were shown in the case presented (Fig. 2). Malignant schwannomas are highly aggressive tumors. They may ap-
pear as a circumscribed mass arising from a nerve. The t u m o r often seems to be encapsulated, but this usually is pseudoencapsulation secondary to compression of surrounding structures. They tend to recur locally within a relatively short time. The 5-year survival rate of these tumors is reported to vary from 37.6% TM to 65.7% 7 in general, but the prognosis of those arising from the inferior alveolar nerve is very poor. Only two patients, including the patient presented, survived beyond 5 years 1'3'5'6'1°'12. To date, chemotherapy seems to have a limited role in the management of this tumor; however, occasional responses have been reported with Adriamycincontaining drug combinations 8. SOROILCO et al. 14 reported that the results of adjuvant chemotherapy after surgery appeared to be encouraging, and they felt that adjuvant chemotherapy should be considered in patients with malignant schwannomas, especially in cases of von Recklinghausen's disease. A wide surgical resection, however, is still the preferred m e t h o d of treatment, augmented by combination chemotherapy.
Acknowledgment. The authors are grateful to Dr SHIRANEfor valuable guidance in histologic examination.
Fig. 3. Microphotograph (HE x 40) of surgical specimen showing tumor mass to be highly cellular, comprising spindle-shaped cells with nuclear hyperchromatism and frequent mitoses (insert HE x 200).
References 1. BAILET JW, ABEMAYORE, ANDREWSJC, ROWLAND JP, Fu YS, DAWSONDE. Malignant nerve sheath tumors of head and
neck: a combined experience from two
lignant schwannoma of the inferior alveolarnerve: report of a case. J Oral Med
1983: 38: 90-3. 7. GrIosu BC, GHosrr L, Huvos AG, FORTHORJG. Malignant schwannoma. A clinicopathological study. Cancer 1973: 31: 184-90. 8. GOLDMANRL, JONES SE, HEUS~NKVELD RS. Combination chemotherapy of metastatic malignant schwannoma with vincristine, Adriamycin cyclophosphamide, and imidazole carboxamide; a case report. Cancer 1977: 39:1955 8. 9. GRATZ KW, MAK~K M, SAILERHF. Malignant meranotic schwannoma of the oral cavity. Int J Oral Maxillofac Surg 1991: 20: 236-8. 10. KamuoDe CS. Malignant schwannoma of the trigeminal nerve. Otolaryngol Head Neck Surg 1979: 87: 594-8. 11. ROSENG, CAPARROSB, NIRENBERGA, et al. Ewing's sarcoma: ten-year experience with adjuvant chemotherapy. Cancer 1981: 47: 2204-13. 12. ROBERTSON I, COOK MG, WILSON DF, HENDERSONDW. Malignant schwannoma of cranial nerves. Pathology 1983: 15: 421-9. 13. SIRASUNAK, FUKUDAY, KITAMURAR, et al. Malignant schwannoma of the mandible. Int J Oral Maxillofac Surg 1986: 15: 77~6. 14. SORDILLOPP, HELSONL, HAJDUSI, et al. Malignant schwannoma - clinical characteristics, survival, and response to therapy. Cancer 1981: 47: 25034. Address: Dr Masanobu Ohn&hi Department of Dentistry and Maxillo-Facial Surgery Kobe City General Hospital 6 6 Minatojima-Nakamachi Chuo-ku, Kobe, 650 Japan
