594
MALIGNANT SCHWANNOMA OF THE TRIGEMINAL NERVE COLLIN S. KARMODY, MD BOSTON, MASSACHUSETIS
Malignant schwannomas are rare in the head and neck, even though benign schwannomas of this area are common. A case of a malignant schwannoma of the fifth cranial nerve with symptoms of otalgia and serous otitis media is described. About 20 such cases have been reported in the literature.
SCHWANNOMAS are tumors that originate from the Schwann cells of nerve sheaths and are common on the cranial nerves. Most benign schwannomas are isolated tumors on peripheral nerves, and the most common sites are in the head, the neck, and extremities.' Malignant schwannomas are mainly found in association with von Recklinghausen's disease, and although solitary benign schwannomas are comparatively common, solitary malignant schwannomas are rare. A case report of a solitary malignant schwannoma of the fifth cranial nerve is presented, and the relevant literature is reviewed. Histologically, a typical schwan noma is composed of a dense pattern of spindle cells with elongated, dark-staining nuclei. The cells are tightly packed together and may have a whorling pattern. Two histologic types are recognized. Antoni Type A is characterized by a parallel (palisading)
Submitted for publication Sept 1, 1978. From Tufts University School of Medicine, Boston. Presented at the 1978 Annual Meeting of the American Academy of Otolaryngology, Las Vegas, Sept lOU
Reprint requests to Tufts-New England Medical Center, 171 Harrison Ave, Boston, MA 02111 lOr karmody).
alignment of nuclei surrounding a central zone of cytoplasm called a Verocay body. Anton; Type B may have no discernible cell pattern. The two types are frequently found together in the same tumor. Typically, there are no neurites in a schwannoma. A malignant schwan noma is usually a firm tumor with a histologic pattern of a dense population of plump spindle cells with varying amounts of mitosis. There are no specific patterns that differentiate histologically a malignant schwannoma from other spindle-cell tumors. Stout et al2 believed that an important diagnostic feature for identifying a malignant schwan noma was the gross evidence of its neural origin.
CASE REPORT
A 70-year-old man had an eight-month history of episodes of numbness, soreness, pain around the right ear radiating to the right lower jaw, and right-sided hearing loss. He had been healthy for most of his life and had had no major illness. On examination there was hypesthesia to touch and pin prick of the lower right two thirds of the face, paresis of the right soft palate, and a right serous otitis media. The temporalis, masseter, and pterygoid muscles were normal. The other cranial nerves functioned normally and symmetrically. Routine analyses of the blood and urine were normal. Radiologic studies of the skull, paranasal sinuses, temporomandibular joints, and mastoids revealed only a possible widening of the right foramen ovale and the right foramen rotundum. This was further supported by tomograms, but it was believed to be an anatomic variant. Biopsy specimens of the right superficial temporal artery and of the nasopharynx were normal. A tentative diagnosis of vascular pain was made, and treatment with 40 mg of prednisone per day and pain medication gave only mild relief. Six months later there was additional weakness of the right masseter
Oto/aryngol Head Neck Surg 87:594-598 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 4, 2016
MALIGNANT SCHWANNOMAS and right temporal is muscles and a right peripheral facial paresis. Skull roentgenograms now revealed a further widening of the right foramen ova Ie (Fig 1). A lumbar puncture, a brain scan, and angiograms were all normal. It was believed that the symptoms were caused by a mass lesion in the right infratemporal fossa, and a biopsy was planned.
595
A transantral approach was used. A sublabial incision was made in the gingival-buccal sulcus, and the anterior wall of the maxillary sinus was opened extensively. Immediately on entering the sinus, the right infraorbital nerve was identified and found to be thickened to about three times normal size. A biopsy specimen of the nerve was taken. The mucosa of the posterior wall of the maxillary sinus was incised
FiK 1.-Tomowam of floor of middle cranial fossa. Note widen inK and loss of cortical rim of riKhl foramen ovalo.
Oto/aryngol Head Neck Sutg 87:594-598 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 4, 2016
596
COLLIN S. KARMODY
and elevated as an inferiorly based flap . The bony posterior wall was removed piecemeal, and the periosteum, incised. The internal maxillary artery was identified and ligated. The fat was teased away until the base of the pterygoid plate was identified . The region of the foramen ovale was approached by working posteriorly and laterally . Biopsy specimens were also taken from this area . All biopsy specimens showed a highly cellular tumor. There were numerous, tightly packed, spindle-shaped cells with dark , highl y chromatic nuclei . There was a moderate amount of mitosis (Fig 2). Reticul in stains were not helpful, and a Bodian stain demonstrated a few neurites per high-power field . It was difficult to distinguish the cellular components from fibrocytes. The specimen was reviewed by six pathologists from different centers, and the diagnosis of sarcoma was made by four consultants. Two consultants believed that it was an undifferentiated carcinoma. As the tumor was obviously malignant, and as it appeared to be confined to the nerve trunks, a diagnosis of malignant schwannoma (neurogenic sarcoma) was accepted . The infrate m p o ral fossa and the base of the skull were treated with radiation therapy to
6,000 rads in six weeks, with a substantial amelioration of symptoms that lasted for one month . Six months later a complete paralysis of the right face had developed . There was now a mass involving the right alveolus with roentgenographic evidence of gross expansion of the mandibular canal (Fig 3). Histologically, this mass was identical to the first specimen. Radiation therapy to the lower jaw to a total of 3,000 rads did not control the local pain . Five months later there were nodular metastases on the skin of the right lower face, and there was fixation of the right tongue. Bleomycin and bone scans showed increased uptake in the region of the right cheek and right mandible. A course of bleomycin failed to control the lesions, and the patient died from a mass ive pulmonary embolus three years after the onset of his symptoms. At autopsy, the tumor was found to involve the gasserian ganglion and all divisions of the trigeminal nerve, particularly the second and third divisions. There was substantial residual tumor in the right mandible, and a small subependymal astrocytoma of the right occipital lobe was also found .
.
..,
, , ·..." ,··..."L
~.. .
~.
.
Fig 2.-Biopsy specimen of right infraorbital nerve. Numerous spindle cells are present with no discernible pattern . A few questionable mitotic ligures are seen.
Otolaryngol Head Neck Surg 87:594-598 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 4, 2016
MALIGNANT SCHWANNOMAS
597
Fig 3.-Right mandibular canal with gross expansion. There is marked erosion of bone anteriorly.
DISCUSSION DasGupta et ai' reviewed 303 patients with benign solitary neurilemomas. Of these, 44.8% (136) occurred in the head and neck. DasGupta and Brasfield] also reviewed 232 patients with solitary malignant schwannomas. Only 8% (18) of these were located in the head and neck region. This difference in the locations of benign and malignant schwannomas suggests that there is little or no relationship between the two tumors; that is, that malignant schwannomas do not arise from benign neoplasms. D' Agostino et al,· using strict criteria for a diagnosis of malignancy, studied 24 patients with malignant neoplasms of nerves. None of these involved the cranial nerves. Their findings supported those of Stout- that
gross evidence of neural origin was an important diagnostic feature. Schisano and Olivecrona! studied 442 cases of intracranial neuromas, and of these, only 2.9% (15) involved the gasserian ganglion and trigeminal root. Eversoles collected 13 cases of malignant schwan noma involving the jaws, found in the literature. Four of these involved erosion of bone. Six lesions were located centrally in the jaws, and one involved the mandibular canal. Katz et al 7 reported 15 extracranial solitary neurogenous neoplasms of the face and neck. Only one of these originated in the trigeminal nerve, specifically from the infraorbital nerve. Grinberg and levy8 described a case of a malignant schwannoma of the supraorbital nerve, one of the terminal branches of the trigeminal nerve. Glassauer and Tendons stated that
Otolaryngol Head Neck Surg 87:594-598 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 4, 2016
598
COLLIN S. KARMODY
only 95 cases of trigeminal neuroma had ever been reported. They did not specify whether there were malignant changes in any of the cases. Rand10 described a middle-aged man with a fibroendothelioma of the left gasserian ganglion. Rand further referred to a publication by Goodhart (1886) describing a fibrosarcoma of the left gasserian ganglion.
radical local excision is sometimes unfeasible, and the prognosis for a patient with a malignant schwannoma of the cranial nerve is poor.
A review of the literature suggests that it is almost impossible to accurately collect the reported cases of malignant schwannoma involving the trigeminal nerve because of the variations in nomenclature and the difficulty of histologic diagnosis. At least 18 to 20 malignant schwannomas of the trigeminal nerve and its terminal branches have been reported in the literature, and this figure is probably low. These tumors are seldom aggressively malignant, and they spread locally along the nerve trunks and, later, through the blood stream. True nodal involvement was not reported, even in the larger series),·
2. Stout AP: Tumors of the Peripheral Nervous System. Atlas of Tumor Pathology, Armed
The treatment of malignant schwannoma is radical surgical removal, and those patients who have survived five years or more without recurrence have had limb amputations or radical local excision. In the series reported by D' Agostino et al,· only four of 24 patients survived more than five years, and all of these had radical amputations. It is not surprising that our patient survived only three years after diagnosis. When a tumor originates from a cranial nerve, even
REFERENCES
1. DasGupta TK, Brasfield RD, Strong EW, et al: Benign solitary schwannomas (neurilemomas). Cancer 24:355-366, 1969.
Forces Institute of Pathology, 1949, Section 2, pt 6. 3. DasGupta TK, Brasfield RD: Solitary malignant schwannoma. Ann Surg 171:419-428,1970. 4. D'Agostino AN, Soule EH, Miller RH: Primary malignant neoplasms of nerves (malignant neurilemomas) in patients without manifestations of multiple neurofibromatosis (Von Recklinghausen's Disease). Cancer 16:10031014, 1963. 5. Schisano G, Olivecrona H: Neurinomas of the gasserian ganglion and trigeminal root. ) Neurosurg 17:306-322, 1960. 6. Eversole lR: Central benign and malignant neural neoplasms of the jaws: A review. ) Oral Surg 27:716-721, 1969. 7. Katz AD, Passy V, Kaplan l: Neurogenous . neoplasms of major nerves of the face and neck. Arch Surg 103:51-56, 1971. 8. Grinberg MA, levy NS: Malignant neurilemoma of the supraorbital nerve. Am ) Ophthalmol 78:489-492, 1974. 9. Glassauer FG, Tandon PN: Trigeminal neuromas in adolescents. ) Neurol Neurosurg Psychiatry 32:562-568, 1969. 10. Rand CW: Tumor of the left gasserian ganglion. Surg Gynecol Obstet 40:49-54, 1925.
Oto/aryngol Head Neck Surg 87:594-598 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 4, 2016
MALIGNANT SCHWANNOMA OF THE TRIGEMINAL NERVE COLLIN S. KARMODY, MD BOSTON, MASSACHUSETIS
Malignant schwannomas are rare in the head and neck, even though benign schwannomas of this area are common. A case of a malignant schwannoma of the fifth cranial nerve with symptoms of otalgia and serous otitis media is described. About 20 such cases have been reported in the literature.
SCHWANNOMAS are tumors that originate from the Schwann cells of nerve sheaths and are common on the cranial nerves. Most benign schwannomas are isolated tumors on peripheral nerves, and the most common sites are in the head, the neck, and extremities.' Malignant schwannomas are mainly found in association with von Recklinghausen's disease, and although solitary benign schwannomas are comparatively common, solitary malignant schwannomas are rare. A case report of a solitary malignant schwannoma of the fifth cranial nerve is presented, and the relevant literature is reviewed. Histologically, a typical schwan noma is composed of a dense pattern of spindle cells with elongated, dark-staining nuclei. The cells are tightly packed together and may have a whorling pattern. Two histologic types are recognized. Antoni Type A is characterized by a parallel (palisading)
Submitted for publication Sept 1, 1978. From Tufts University School of Medicine, Boston. Presented at the 1978 Annual Meeting of the American Academy of Otolaryngology, Las Vegas, Sept lOU
Reprint requests to Tufts-New England Medical Center, 171 Harrison Ave, Boston, MA 02111 lOr karmody).
alignment of nuclei surrounding a central zone of cytoplasm called a Verocay body. Anton; Type B may have no discernible cell pattern. The two types are frequently found together in the same tumor. Typically, there are no neurites in a schwannoma. A malignant schwan noma is usually a firm tumor with a histologic pattern of a dense population of plump spindle cells with varying amounts of mitosis. There are no specific patterns that differentiate histologically a malignant schwannoma from other spindle-cell tumors. Stout et al2 believed that an important diagnostic feature for identifying a malignant schwan noma was the gross evidence of its neural origin.
CASE REPORT
A 70-year-old man had an eight-month history of episodes of numbness, soreness, pain around the right ear radiating to the right lower jaw, and right-sided hearing loss. He had been healthy for most of his life and had had no major illness. On examination there was hypesthesia to touch and pin prick of the lower right two thirds of the face, paresis of the right soft palate, and a right serous otitis media. The temporalis, masseter, and pterygoid muscles were normal. The other cranial nerves functioned normally and symmetrically. Routine analyses of the blood and urine were normal. Radiologic studies of the skull, paranasal sinuses, temporomandibular joints, and mastoids revealed only a possible widening of the right foramen ovale and the right foramen rotundum. This was further supported by tomograms, but it was believed to be an anatomic variant. Biopsy specimens of the right superficial temporal artery and of the nasopharynx were normal. A tentative diagnosis of vascular pain was made, and treatment with 40 mg of prednisone per day and pain medication gave only mild relief. Six months later there was additional weakness of the right masseter
Oto/aryngol Head Neck Surg 87:594-598 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 4, 2016
MALIGNANT SCHWANNOMAS and right temporal is muscles and a right peripheral facial paresis. Skull roentgenograms now revealed a further widening of the right foramen ova Ie (Fig 1). A lumbar puncture, a brain scan, and angiograms were all normal. It was believed that the symptoms were caused by a mass lesion in the right infratemporal fossa, and a biopsy was planned.
595
A transantral approach was used. A sublabial incision was made in the gingival-buccal sulcus, and the anterior wall of the maxillary sinus was opened extensively. Immediately on entering the sinus, the right infraorbital nerve was identified and found to be thickened to about three times normal size. A biopsy specimen of the nerve was taken. The mucosa of the posterior wall of the maxillary sinus was incised
FiK 1.-Tomowam of floor of middle cranial fossa. Note widen inK and loss of cortical rim of riKhl foramen ovalo.
Oto/aryngol Head Neck Sutg 87:594-598 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 4, 2016
596
COLLIN S. KARMODY
and elevated as an inferiorly based flap . The bony posterior wall was removed piecemeal, and the periosteum, incised. The internal maxillary artery was identified and ligated. The fat was teased away until the base of the pterygoid plate was identified . The region of the foramen ovale was approached by working posteriorly and laterally . Biopsy specimens were also taken from this area . All biopsy specimens showed a highly cellular tumor. There were numerous, tightly packed, spindle-shaped cells with dark , highl y chromatic nuclei . There was a moderate amount of mitosis (Fig 2). Reticul in stains were not helpful, and a Bodian stain demonstrated a few neurites per high-power field . It was difficult to distinguish the cellular components from fibrocytes. The specimen was reviewed by six pathologists from different centers, and the diagnosis of sarcoma was made by four consultants. Two consultants believed that it was an undifferentiated carcinoma. As the tumor was obviously malignant, and as it appeared to be confined to the nerve trunks, a diagnosis of malignant schwannoma (neurogenic sarcoma) was accepted . The infrate m p o ral fossa and the base of the skull were treated with radiation therapy to
6,000 rads in six weeks, with a substantial amelioration of symptoms that lasted for one month . Six months later a complete paralysis of the right face had developed . There was now a mass involving the right alveolus with roentgenographic evidence of gross expansion of the mandibular canal (Fig 3). Histologically, this mass was identical to the first specimen. Radiation therapy to the lower jaw to a total of 3,000 rads did not control the local pain . Five months later there were nodular metastases on the skin of the right lower face, and there was fixation of the right tongue. Bleomycin and bone scans showed increased uptake in the region of the right cheek and right mandible. A course of bleomycin failed to control the lesions, and the patient died from a mass ive pulmonary embolus three years after the onset of his symptoms. At autopsy, the tumor was found to involve the gasserian ganglion and all divisions of the trigeminal nerve, particularly the second and third divisions. There was substantial residual tumor in the right mandible, and a small subependymal astrocytoma of the right occipital lobe was also found .
.
..,
, , ·..." ,··..."L
~.. .
~.
.
Fig 2.-Biopsy specimen of right infraorbital nerve. Numerous spindle cells are present with no discernible pattern . A few questionable mitotic ligures are seen.
Otolaryngol Head Neck Surg 87:594-598 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 4, 2016
MALIGNANT SCHWANNOMAS
597
Fig 3.-Right mandibular canal with gross expansion. There is marked erosion of bone anteriorly.
DISCUSSION DasGupta et ai' reviewed 303 patients with benign solitary neurilemomas. Of these, 44.8% (136) occurred in the head and neck. DasGupta and Brasfield] also reviewed 232 patients with solitary malignant schwannomas. Only 8% (18) of these were located in the head and neck region. This difference in the locations of benign and malignant schwannomas suggests that there is little or no relationship between the two tumors; that is, that malignant schwannomas do not arise from benign neoplasms. D' Agostino et al,· using strict criteria for a diagnosis of malignancy, studied 24 patients with malignant neoplasms of nerves. None of these involved the cranial nerves. Their findings supported those of Stout- that
gross evidence of neural origin was an important diagnostic feature. Schisano and Olivecrona! studied 442 cases of intracranial neuromas, and of these, only 2.9% (15) involved the gasserian ganglion and trigeminal root. Eversoles collected 13 cases of malignant schwan noma involving the jaws, found in the literature. Four of these involved erosion of bone. Six lesions were located centrally in the jaws, and one involved the mandibular canal. Katz et al 7 reported 15 extracranial solitary neurogenous neoplasms of the face and neck. Only one of these originated in the trigeminal nerve, specifically from the infraorbital nerve. Grinberg and levy8 described a case of a malignant schwannoma of the supraorbital nerve, one of the terminal branches of the trigeminal nerve. Glassauer and Tendons stated that
Otolaryngol Head Neck Surg 87:594-598 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 4, 2016
598
COLLIN S. KARMODY
only 95 cases of trigeminal neuroma had ever been reported. They did not specify whether there were malignant changes in any of the cases. Rand10 described a middle-aged man with a fibroendothelioma of the left gasserian ganglion. Rand further referred to a publication by Goodhart (1886) describing a fibrosarcoma of the left gasserian ganglion.
radical local excision is sometimes unfeasible, and the prognosis for a patient with a malignant schwannoma of the cranial nerve is poor.
A review of the literature suggests that it is almost impossible to accurately collect the reported cases of malignant schwannoma involving the trigeminal nerve because of the variations in nomenclature and the difficulty of histologic diagnosis. At least 18 to 20 malignant schwannomas of the trigeminal nerve and its terminal branches have been reported in the literature, and this figure is probably low. These tumors are seldom aggressively malignant, and they spread locally along the nerve trunks and, later, through the blood stream. True nodal involvement was not reported, even in the larger series),·
2. Stout AP: Tumors of the Peripheral Nervous System. Atlas of Tumor Pathology, Armed
The treatment of malignant schwannoma is radical surgical removal, and those patients who have survived five years or more without recurrence have had limb amputations or radical local excision. In the series reported by D' Agostino et al,· only four of 24 patients survived more than five years, and all of these had radical amputations. It is not surprising that our patient survived only three years after diagnosis. When a tumor originates from a cranial nerve, even
REFERENCES
1. DasGupta TK, Brasfield RD, Strong EW, et al: Benign solitary schwannomas (neurilemomas). Cancer 24:355-366, 1969.
Forces Institute of Pathology, 1949, Section 2, pt 6. 3. DasGupta TK, Brasfield RD: Solitary malignant schwannoma. Ann Surg 171:419-428,1970. 4. D'Agostino AN, Soule EH, Miller RH: Primary malignant neoplasms of nerves (malignant neurilemomas) in patients without manifestations of multiple neurofibromatosis (Von Recklinghausen's Disease). Cancer 16:10031014, 1963. 5. Schisano G, Olivecrona H: Neurinomas of the gasserian ganglion and trigeminal root. ) Neurosurg 17:306-322, 1960. 6. Eversole lR: Central benign and malignant neural neoplasms of the jaws: A review. ) Oral Surg 27:716-721, 1969. 7. Katz AD, Passy V, Kaplan l: Neurogenous . neoplasms of major nerves of the face and neck. Arch Surg 103:51-56, 1971. 8. Grinberg MA, levy NS: Malignant neurilemoma of the supraorbital nerve. Am ) Ophthalmol 78:489-492, 1974. 9. Glassauer FG, Tandon PN: Trigeminal neuromas in adolescents. ) Neurol Neurosurg Psychiatry 32:562-568, 1969. 10. Rand CW: Tumor of the left gasserian ganglion. Surg Gynecol Obstet 40:49-54, 1925.
Oto/aryngol Head Neck Surg 87:594-598 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 4, 2016
![[Malignant schwannoma of the trigeminal nerve].](/assets/img/hold.png)
