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Solitary neurofibroma of the maxilla
453
BROWNE
patient demonstrated an ischemic catastrophe. Although these vascular accidents arose without histologic confirmation of giant cell arteritis, the two consultant physicians and the consultant dermatologist in charge of these patients considered the diagnosis to be that of giant cell arteritis. References 1. Henderson AH: Tongue pain with giant cell arteritis. Br. Med J 4:337, 1967 2. Horler AR, Foster JB: Progress in Clinical Medicine, 7th ed.
London, Churchill-Livingstone, 1978, pp 426-430 3. Missen GAK: Gangrene of the tongue. Br Med J 1:1393. 1961
glossitis 4. Howard S, Cremin MD: Acute parenchymatous with gangrene of the tongue. Lancet 2:410, 1959 5. Kinmont PDC, McCallum DI: Skin manifestations of giant cell arteritis. Br J Dermatol 76:229, 1964 6. Davis AE, Davis TP: Gangrene of the tongue caused by temporal arteritis. Med J Aust 2:459, 1966 7. Meadows SP: Temporal or giant cell arteritis. Proc R Sot Med 59:329, 1966 8. Allen P: Giant cell arteritis presenting with necrosis of the tongue. Br J Oral Surg 18:162, 1980 9. Hicks K, Lee FI: Necrosis of the tongue secondary to cranial arteritis. Br J Oral Surg 18:166, 1980 10. Dufetelle JF, Tapie B: Necrose linguale au cours d’une maladie de Horton. Rev Stomatol Chir Maxillofac 8: 1013, 1976 11. Wilkinson IMS: Giant cell arteritis. Br J Hosp Med 15: 154. 1976
Solitary Neurofibroma
of the Maxilla
GORDON L. BRADY, DMD,* DAVID L. SCHAFFNER, DDS, MS,t EDWIN D. JOY, JR, DDS,$ RICHARD L. MORRIS, MD,5 AND KENNA S. GIVEN, MD” Although the solitary neurofibroma is not an uncommon lesion, its occurrence within bone is a rather rare phenomenon. In his review of intraosseous nerve sheath tumors, Gordon’ found only 12 acceptable cases of central neurotibroma, all of which were located within the jaws. Ellis and Abrams, * in reviewing the literature on benign nerve sheath neoplasms of the jaws, found a distinct predilection for occurrence in the posterior mandible; only three cases were reported in the maxilla. This case deals with a large solitary neurofibroma of the left posterior maxilla diagnosed and treated at the Medical College of Georgia. Report of a Case In September 1979, a 20-year-old black woman presented to the oral surgery clinic with a chief complaint of swelling in her left maxilla, which produced a marked facial asymmetry (Fig. I) and pain on mastication. She believed that the problem was due to an abscessed tooth and stated that the swelling had been present for approximately one year. It had slowly increased in size since that time. Oral examination revealed a large, bulbous, exophytic mass extending from the maxillary tuberosity anteriorly to the premolar area (see Figure 3). The cortical plates were expanded from just left of the midpalatal raphe lat-
erally to the mucobuccal fold. The second premolar and the first molar were extremely mobile, and the first molar was extruded. The mandibular teeth and alveolar ridge occluded with the tumor mass. The lesion was firm and covered by an intact mucosa, which appeared hyperemic in some areas. The left naris was occluded, and the left malar area of the face was enlarged. No paresthesia or ocular findings were noticed. Otherwise, the results of the patient’s medical history and physical examination were within normal limits. Facial bone and panoramic radiographs (Fig. 2), as well as antero posterior and lateral tomograms, were obtained to determine the extent of the lesion. These films showed the mass to be an extensive, spherical, well-circumscribed, uniformly radiolucent lesion without signs of ossification. It occupied the entire left maxillary antrum and extended superiorly to the orbital floor and medially to the sphenoid and ethmoid sinuses. The first and third molars were totally engulfed by the tumor with no evidence of bony support. The second molar was missing. A biopsy was obtained from the palatal aspect of the mass. Grossly, the tissue had a uniform grayish white color and a firm, fleshy texture. The lesional tissue was sharply demarcated from the overlying mucosa. Histologic sections (Figs. 4 and 5) showed the lesional tissue
* Formerly Chief Resident in Oral and Maxillofacial Surgery at the Medical College of Georgia; now in private practice in Decatur, Georgia. t Assistant Professor of Oral Pathology and Oral Biology. $ Professor and Chief of Oral Surgery. B Assistant Professor of Plastic Surgery. I’Associate Professor and Chief of Plastic Surgery. Received from the Medical College of Georgia, Augusta, Georgia, 30901. Address correspondence and reprint requests to Dr. SchatTner. 0278.2391/82/0700/0453 $00.80 @ American Association of Oral and Maxillofacial Surgeons
MAXILLARY
NEUROFIBROMA
FIGURE 3. Above, lejl, Immorally, a bulbous tumor mass with an intact mucosa extending from the tuberosity to the premolar area. FIGURE 4. Above, right, Low-power photomicrograph shows a myxoid lesion separated from the overlying lamina propria by a distinct line of demarcation. (Hematoxylin and eosin, original magnification x 50.) FIGURE 5. Right, Photomicrograph showing spindle-shaped tumor cells with long cytoplasmic processes and delicate serpentine collagen fibers supported by a myxomatous stroma. (Hematoxylin and eosin, original magnification x 125.)
to be characterized by a myxomatous network of collagen and reticulin fibers, which occasionally coalesced to form fiber fascicles. The fiber-producing cells were generally spindle-shaped, with the nucleus polarized toward one end of the cell and a long, delicate cytoplasmic process extending from the opposite pole. The nuclei of these cells were isomorphic, ovoid, and heterochromatic, with indistinct nucleoli. The neoplastic cells were interpreted as Schwann cells. Several prominent trabeculae of metaplastic bone were rimmed by a layer of plump osteoblasts. The tumor tissue was separated from the overlying mucosa by a distinct line of demarcation. The final diagnosis was myxoid neurofibroma. In October 1979, the patient was admitted to the Eugene Talmadge Memorial Hospital by the department of oral surgery in cooperation with the department of plastic surgery, for surgical resection of the tumor. At this time, physical examination failed to reveal additional lesions that might have represented neurofibromas, and no cafe-au-h& pigmentation of the skin was identified. A left radical maxillectomy via a Ferguson type of incision was performed, providing a surgical specimen measuring 8 x 7 x 5 cm. The size of the tumor necessitated sacrifice of the orbital floor, infraorbital rim, and ethmoid sinus, and transection of the nasolacrimal duct. Reconstruction of the surgical defect was begun by the insertion of Teflon sheeting to replace the orbital floor. The nasolacrimal duct was sutured to the lateral nasal mucosa, and a Silastic catheter was inserted to maintain patency. A portion of the left fiih rib was obtained and grafted from the nasal side of the defect to the zygoma to reestablish the contour of the inferior orbital rim. At this point, a latissimus dorsi myocutaneous flap measuring 8 x 22 cm, extending from the axilla to the midline of the
back, was elevated transversely from just below the tip of the scapula. The thoracodorsal vascular bundle was preserved to maintain viability of the flap. The flap was threaded through the pectoral fascia and platysma into the oral cavity and was rotated 90 degrees so that the cutaneous surface faced intraorally (Fig. 6). A strip of the flap was de-epithelialized transversely, dividing it into two cutaneous areas. The flap was sutured from the buccal borders of the defect across to the palatal mucosa. The part with intact epithelium was positioned as the lateral nasal wall, the muscular portion being placed adjacent to the reconstructed orbital rim and floor. The facial flap was then repositioned and sutured. The postoperative course was uneventful. At one year the operative site was healing well, the facial contour was
FIGURE 6. Intraoperative view showing the latissimus dorsi flap being passed from beneath the platysma into the oral cavity.
455
BRADY ET AL
satisfactory (Fig. 7), and there was no evidence of recurrent disease. Discussion The true incidence of the neurofibroma vs the schwannoma has been difficult to determine from the literature because of a general lack of agreement among pathologists and clinicians as to the terminology for these tumors. The common terms for the benign nerve sheath neoplasms-neurofibroma, neurilemmoma, schwannoma, and neurinoma-have been used haphazardly and often interchangeably. Contributing to this confusion is a lack of understanding of the histogenesis of the cellular components both of the peripheral nerve and of these tumors. Harkin and Reed,3 for example, believe on the basis of ultrastructural and tissue culture studies that the Schwann cell and the perineural cell are identical and should both be labelled as Schwann cells that may give rise to either of the nerve sheath neoplasms. The ultrastructural studies of Lazarus and Trombetta,J on the other hand, have delineated a distinct difference between Schwann cells and perineural fibroblasts, suggesting that they represent are two distinct cell types, which may or may not be related developmentally. They have proposed use of the term “perineurinoma” in addition to the schwannoma and neurotibroma, depending on the cell types observed ultrastructurally. Regardless of the histogenesis and ultrastructural morphology, the histologic criteria for the diagnosis of neurofibroma and schwannoma are now well defined, and the two entities should not be confused. The schwannoma is an encapsulated tumor exhibiting Antoni A tissue, with its associated acellular Verocay bodies and palisaded nuclei, and/or Antoni type B tissue. The neurofibroma, on the other hand, is usually a well-delineated but nonencapsulated tumor with spindle-shaped cells supported by a collagenous or myxomatous background. Soft tissue solitary neurofibromas are relatively common lesions. Of 303 benign nerve sheath tumors reviewed by Das Gupta et aL5 45% occurred in the head and neck region. Most of these involved the neck, and only about 9% were located intraorally. Within the oral cavity, the tongue is the site of predilection, followed by the buccal mucosa as per the series of cases reported by Chenick and Eversole.’ The primary symptom of the soft tissue tumors is a painless swelling. Intraosseous nerve sheath neoplasms are extremely unusual, as is illustrated by the survey of Fawcett and Dahlin,’ who identified only seven of these tumors in a series of 3987 primary bone neoplasms. This low incidence is difficult to explain
FIGURE 7. One year postoperatively the patient shows satisfactory facial contour.
when one considers that bone is richly endowed with both peripheral sensory nerves and nerve fibers of the autonomic nervous system.’ The jaws contain large nerve trunks, such as the inferior alveolar nerve, which pass through a relatively long expanse of bone, as well as numerous smaller axons, which accompany the vascular supply of the bone within the nutrient canals. These anatomic features have been proposed to explain the great predilection of these tumors for occurrence in the jaws.‘.*,’ Gordon’ has reviewed the literature of primary nerve sheath tumors of bone, reclassifying them as to the appropriate histologic designation where necessary, in order to provide a truer representation of the relative incidence of these tumors. Fifty-one schwannomas were identified, 17 of which occurred in the mandible and five in the maxilla, confirming the distinct predilection for the jaws. Other tumors were identified in the sacrum, ulna, femur, spine, and a variety of other bones. Of the twelve cases of neurofibroma that were reviewed from the literature, all cases occurred centrally within the jaws, ten within the mandible and two in the maxilla. More recently, Ellis and Abrams2 have reviewed the intraosseous benign nerve sheath tumors of the jaws. Their 28 cases from the literature and seven new cases (five neurofibromas and two schwannomas) showed a 2: 1 predilection for females and a usual occurrence in a young age group, the average age of patients being 29 years. Most patients presented with a chief complaint of swelling or expansion of the jaws. Pain and paresthesia were also common symptoms. Radiographically, the schwannomas tended to present as well-circumscribed, unilocular radiolucencies. The neurofibromas showed more radiographic variation, presenting as either well or poorly circumscribed, unilocular or multilocular radiolucencies. The clinical behavior of all benign nerve sheath tumors is similar in that all usually grow slowly, behave in an indolent manner, and rarely recur following removal. The one exception may be that
456
MAXILLARY
neurofibromas and schwannomas appear to have a somewhat different potential for malignant transformation. Although malignant change in a benign schwannoma has been reported, it is rare.8 Neurofibromas, on the other hand, undergo malignant transformation with greater frequency, especially when associated with neurofibromatosis, in which the incidence of malignant transformation has been reported to be as high as 13%.s The treatment for both the neurofibroma and the schwannoma should reflect the benign clinical behavior of these tumors. Local excision with preservation of the associated nerve trunk, if possible, is the treatment of choice.’ Summary A case of a large central neurofibroma of the left posterior maxilla is presented. This lesion was treated by a left radical maxillectomy because of the extensive nature of the tumor. The surgical defect was reconstructed with a latissimus dorsi myocuta-
NEUROFIBROMA
neous flap. The incidence and clinical features of intraosseous benign nerve sheath tumors are discussed. References 1. Gordon EJ: Solitary intraosseous neurilemmoma of the tibia. Clin Orthop 117:271, 1976 2. ElIis GL, Abrams AM: Intraosseous benign neural sheath neoplasms of the jaws. Oral Surg 44:7311 1977 3. Harkin JC. Reed RJ: Tumors of the nerinheral nervous svstern. Washington DC, Armed ForceszInstitute of Pathology, 1969, p29 4. Lazarus SS, Trombetta LD: Ultrastructural identification of a benign perineurial cell tumor. Cancer 41:1823, 1978 5. Das Gupta TK et al: Benign solitary schwannomas (neurilemomas). Cancer 24:355, 1%9 6. Cherrick HM, Eversole LR: Benign neural sheath neoplasm of the oral cavity. Oral Surg 32:900, 1971 7. Fawcett KJ, Dahlin DC: Neurilemmoma of bone. Am J Clin Path01 47:759, 1967 8. Carstons HB, Schrodt, RG: Malignant transformation of a benign encapsulated neurilemoma. Am J Clin Path01 51:144, 1969 9. Hosoi K: Multiple neurofibromatosis (von Reckhnghausen’s disease) with special reference to malignant transformation. Arch Surg 22:258, 1931
The appearance of the code at the bottom of the first page of an article in this journal indicates the copyright owner’s consent that copies of the article may be made for personal or internal use, or for personal or internal use of specific clients. This consent is given on the condition, however, that the copier pay the stated per-copy fee through the Copyright Clearance Center, Inc., 21 Congress Street, Salem, Massachusetts 01970, for copying beyond that permitted by Sections 107 or 109 of the U.S. Copyright Law. This consent does not extend to other kinds of copying, such as copying for general distribution, for advertising or promotional purposes, for creating new collective works, or for resale.
BROWNE
patient demonstrated an ischemic catastrophe. Although these vascular accidents arose without histologic confirmation of giant cell arteritis, the two consultant physicians and the consultant dermatologist in charge of these patients considered the diagnosis to be that of giant cell arteritis. References 1. Henderson AH: Tongue pain with giant cell arteritis. Br. Med J 4:337, 1967 2. Horler AR, Foster JB: Progress in Clinical Medicine, 7th ed.
London, Churchill-Livingstone, 1978, pp 426-430 3. Missen GAK: Gangrene of the tongue. Br Med J 1:1393. 1961
glossitis 4. Howard S, Cremin MD: Acute parenchymatous with gangrene of the tongue. Lancet 2:410, 1959 5. Kinmont PDC, McCallum DI: Skin manifestations of giant cell arteritis. Br J Dermatol 76:229, 1964 6. Davis AE, Davis TP: Gangrene of the tongue caused by temporal arteritis. Med J Aust 2:459, 1966 7. Meadows SP: Temporal or giant cell arteritis. Proc R Sot Med 59:329, 1966 8. Allen P: Giant cell arteritis presenting with necrosis of the tongue. Br J Oral Surg 18:162, 1980 9. Hicks K, Lee FI: Necrosis of the tongue secondary to cranial arteritis. Br J Oral Surg 18:166, 1980 10. Dufetelle JF, Tapie B: Necrose linguale au cours d’une maladie de Horton. Rev Stomatol Chir Maxillofac 8: 1013, 1976 11. Wilkinson IMS: Giant cell arteritis. Br J Hosp Med 15: 154. 1976
Solitary Neurofibroma
of the Maxilla
GORDON L. BRADY, DMD,* DAVID L. SCHAFFNER, DDS, MS,t EDWIN D. JOY, JR, DDS,$ RICHARD L. MORRIS, MD,5 AND KENNA S. GIVEN, MD” Although the solitary neurofibroma is not an uncommon lesion, its occurrence within bone is a rather rare phenomenon. In his review of intraosseous nerve sheath tumors, Gordon’ found only 12 acceptable cases of central neurotibroma, all of which were located within the jaws. Ellis and Abrams, * in reviewing the literature on benign nerve sheath neoplasms of the jaws, found a distinct predilection for occurrence in the posterior mandible; only three cases were reported in the maxilla. This case deals with a large solitary neurofibroma of the left posterior maxilla diagnosed and treated at the Medical College of Georgia. Report of a Case In September 1979, a 20-year-old black woman presented to the oral surgery clinic with a chief complaint of swelling in her left maxilla, which produced a marked facial asymmetry (Fig. I) and pain on mastication. She believed that the problem was due to an abscessed tooth and stated that the swelling had been present for approximately one year. It had slowly increased in size since that time. Oral examination revealed a large, bulbous, exophytic mass extending from the maxillary tuberosity anteriorly to the premolar area (see Figure 3). The cortical plates were expanded from just left of the midpalatal raphe lat-
erally to the mucobuccal fold. The second premolar and the first molar were extremely mobile, and the first molar was extruded. The mandibular teeth and alveolar ridge occluded with the tumor mass. The lesion was firm and covered by an intact mucosa, which appeared hyperemic in some areas. The left naris was occluded, and the left malar area of the face was enlarged. No paresthesia or ocular findings were noticed. Otherwise, the results of the patient’s medical history and physical examination were within normal limits. Facial bone and panoramic radiographs (Fig. 2), as well as antero posterior and lateral tomograms, were obtained to determine the extent of the lesion. These films showed the mass to be an extensive, spherical, well-circumscribed, uniformly radiolucent lesion without signs of ossification. It occupied the entire left maxillary antrum and extended superiorly to the orbital floor and medially to the sphenoid and ethmoid sinuses. The first and third molars were totally engulfed by the tumor with no evidence of bony support. The second molar was missing. A biopsy was obtained from the palatal aspect of the mass. Grossly, the tissue had a uniform grayish white color and a firm, fleshy texture. The lesional tissue was sharply demarcated from the overlying mucosa. Histologic sections (Figs. 4 and 5) showed the lesional tissue
* Formerly Chief Resident in Oral and Maxillofacial Surgery at the Medical College of Georgia; now in private practice in Decatur, Georgia. t Assistant Professor of Oral Pathology and Oral Biology. $ Professor and Chief of Oral Surgery. B Assistant Professor of Plastic Surgery. I’Associate Professor and Chief of Plastic Surgery. Received from the Medical College of Georgia, Augusta, Georgia, 30901. Address correspondence and reprint requests to Dr. SchatTner. 0278.2391/82/0700/0453 $00.80 @ American Association of Oral and Maxillofacial Surgeons
MAXILLARY
NEUROFIBROMA
FIGURE 3. Above, lejl, Immorally, a bulbous tumor mass with an intact mucosa extending from the tuberosity to the premolar area. FIGURE 4. Above, right, Low-power photomicrograph shows a myxoid lesion separated from the overlying lamina propria by a distinct line of demarcation. (Hematoxylin and eosin, original magnification x 50.) FIGURE 5. Right, Photomicrograph showing spindle-shaped tumor cells with long cytoplasmic processes and delicate serpentine collagen fibers supported by a myxomatous stroma. (Hematoxylin and eosin, original magnification x 125.)
to be characterized by a myxomatous network of collagen and reticulin fibers, which occasionally coalesced to form fiber fascicles. The fiber-producing cells were generally spindle-shaped, with the nucleus polarized toward one end of the cell and a long, delicate cytoplasmic process extending from the opposite pole. The nuclei of these cells were isomorphic, ovoid, and heterochromatic, with indistinct nucleoli. The neoplastic cells were interpreted as Schwann cells. Several prominent trabeculae of metaplastic bone were rimmed by a layer of plump osteoblasts. The tumor tissue was separated from the overlying mucosa by a distinct line of demarcation. The final diagnosis was myxoid neurofibroma. In October 1979, the patient was admitted to the Eugene Talmadge Memorial Hospital by the department of oral surgery in cooperation with the department of plastic surgery, for surgical resection of the tumor. At this time, physical examination failed to reveal additional lesions that might have represented neurofibromas, and no cafe-au-h& pigmentation of the skin was identified. A left radical maxillectomy via a Ferguson type of incision was performed, providing a surgical specimen measuring 8 x 7 x 5 cm. The size of the tumor necessitated sacrifice of the orbital floor, infraorbital rim, and ethmoid sinus, and transection of the nasolacrimal duct. Reconstruction of the surgical defect was begun by the insertion of Teflon sheeting to replace the orbital floor. The nasolacrimal duct was sutured to the lateral nasal mucosa, and a Silastic catheter was inserted to maintain patency. A portion of the left fiih rib was obtained and grafted from the nasal side of the defect to the zygoma to reestablish the contour of the inferior orbital rim. At this point, a latissimus dorsi myocutaneous flap measuring 8 x 22 cm, extending from the axilla to the midline of the
back, was elevated transversely from just below the tip of the scapula. The thoracodorsal vascular bundle was preserved to maintain viability of the flap. The flap was threaded through the pectoral fascia and platysma into the oral cavity and was rotated 90 degrees so that the cutaneous surface faced intraorally (Fig. 6). A strip of the flap was de-epithelialized transversely, dividing it into two cutaneous areas. The flap was sutured from the buccal borders of the defect across to the palatal mucosa. The part with intact epithelium was positioned as the lateral nasal wall, the muscular portion being placed adjacent to the reconstructed orbital rim and floor. The facial flap was then repositioned and sutured. The postoperative course was uneventful. At one year the operative site was healing well, the facial contour was
FIGURE 6. Intraoperative view showing the latissimus dorsi flap being passed from beneath the platysma into the oral cavity.
455
BRADY ET AL
satisfactory (Fig. 7), and there was no evidence of recurrent disease. Discussion The true incidence of the neurofibroma vs the schwannoma has been difficult to determine from the literature because of a general lack of agreement among pathologists and clinicians as to the terminology for these tumors. The common terms for the benign nerve sheath neoplasms-neurofibroma, neurilemmoma, schwannoma, and neurinoma-have been used haphazardly and often interchangeably. Contributing to this confusion is a lack of understanding of the histogenesis of the cellular components both of the peripheral nerve and of these tumors. Harkin and Reed,3 for example, believe on the basis of ultrastructural and tissue culture studies that the Schwann cell and the perineural cell are identical and should both be labelled as Schwann cells that may give rise to either of the nerve sheath neoplasms. The ultrastructural studies of Lazarus and Trombetta,J on the other hand, have delineated a distinct difference between Schwann cells and perineural fibroblasts, suggesting that they represent are two distinct cell types, which may or may not be related developmentally. They have proposed use of the term “perineurinoma” in addition to the schwannoma and neurotibroma, depending on the cell types observed ultrastructurally. Regardless of the histogenesis and ultrastructural morphology, the histologic criteria for the diagnosis of neurofibroma and schwannoma are now well defined, and the two entities should not be confused. The schwannoma is an encapsulated tumor exhibiting Antoni A tissue, with its associated acellular Verocay bodies and palisaded nuclei, and/or Antoni type B tissue. The neurofibroma, on the other hand, is usually a well-delineated but nonencapsulated tumor with spindle-shaped cells supported by a collagenous or myxomatous background. Soft tissue solitary neurofibromas are relatively common lesions. Of 303 benign nerve sheath tumors reviewed by Das Gupta et aL5 45% occurred in the head and neck region. Most of these involved the neck, and only about 9% were located intraorally. Within the oral cavity, the tongue is the site of predilection, followed by the buccal mucosa as per the series of cases reported by Chenick and Eversole.’ The primary symptom of the soft tissue tumors is a painless swelling. Intraosseous nerve sheath neoplasms are extremely unusual, as is illustrated by the survey of Fawcett and Dahlin,’ who identified only seven of these tumors in a series of 3987 primary bone neoplasms. This low incidence is difficult to explain
FIGURE 7. One year postoperatively the patient shows satisfactory facial contour.
when one considers that bone is richly endowed with both peripheral sensory nerves and nerve fibers of the autonomic nervous system.’ The jaws contain large nerve trunks, such as the inferior alveolar nerve, which pass through a relatively long expanse of bone, as well as numerous smaller axons, which accompany the vascular supply of the bone within the nutrient canals. These anatomic features have been proposed to explain the great predilection of these tumors for occurrence in the jaws.‘.*,’ Gordon’ has reviewed the literature of primary nerve sheath tumors of bone, reclassifying them as to the appropriate histologic designation where necessary, in order to provide a truer representation of the relative incidence of these tumors. Fifty-one schwannomas were identified, 17 of which occurred in the mandible and five in the maxilla, confirming the distinct predilection for the jaws. Other tumors were identified in the sacrum, ulna, femur, spine, and a variety of other bones. Of the twelve cases of neurofibroma that were reviewed from the literature, all cases occurred centrally within the jaws, ten within the mandible and two in the maxilla. More recently, Ellis and Abrams2 have reviewed the intraosseous benign nerve sheath tumors of the jaws. Their 28 cases from the literature and seven new cases (five neurofibromas and two schwannomas) showed a 2: 1 predilection for females and a usual occurrence in a young age group, the average age of patients being 29 years. Most patients presented with a chief complaint of swelling or expansion of the jaws. Pain and paresthesia were also common symptoms. Radiographically, the schwannomas tended to present as well-circumscribed, unilocular radiolucencies. The neurofibromas showed more radiographic variation, presenting as either well or poorly circumscribed, unilocular or multilocular radiolucencies. The clinical behavior of all benign nerve sheath tumors is similar in that all usually grow slowly, behave in an indolent manner, and rarely recur following removal. The one exception may be that
456
MAXILLARY
neurofibromas and schwannomas appear to have a somewhat different potential for malignant transformation. Although malignant change in a benign schwannoma has been reported, it is rare.8 Neurofibromas, on the other hand, undergo malignant transformation with greater frequency, especially when associated with neurofibromatosis, in which the incidence of malignant transformation has been reported to be as high as 13%.s The treatment for both the neurofibroma and the schwannoma should reflect the benign clinical behavior of these tumors. Local excision with preservation of the associated nerve trunk, if possible, is the treatment of choice.’ Summary A case of a large central neurofibroma of the left posterior maxilla is presented. This lesion was treated by a left radical maxillectomy because of the extensive nature of the tumor. The surgical defect was reconstructed with a latissimus dorsi myocuta-
NEUROFIBROMA
neous flap. The incidence and clinical features of intraosseous benign nerve sheath tumors are discussed. References 1. Gordon EJ: Solitary intraosseous neurilemmoma of the tibia. Clin Orthop 117:271, 1976 2. ElIis GL, Abrams AM: Intraosseous benign neural sheath neoplasms of the jaws. Oral Surg 44:7311 1977 3. Harkin JC. Reed RJ: Tumors of the nerinheral nervous svstern. Washington DC, Armed ForceszInstitute of Pathology, 1969, p29 4. Lazarus SS, Trombetta LD: Ultrastructural identification of a benign perineurial cell tumor. Cancer 41:1823, 1978 5. Das Gupta TK et al: Benign solitary schwannomas (neurilemomas). Cancer 24:355, 1%9 6. Cherrick HM, Eversole LR: Benign neural sheath neoplasm of the oral cavity. Oral Surg 32:900, 1971 7. Fawcett KJ, Dahlin DC: Neurilemmoma of bone. Am J Clin Path01 47:759, 1967 8. Carstons HB, Schrodt, RG: Malignant transformation of a benign encapsulated neurilemoma. Am J Clin Path01 51:144, 1969 9. Hosoi K: Multiple neurofibromatosis (von Reckhnghausen’s disease) with special reference to malignant transformation. Arch Surg 22:258, 1931
The appearance of the code at the bottom of the first page of an article in this journal indicates the copyright owner’s consent that copies of the article may be made for personal or internal use, or for personal or internal use of specific clients. This consent is given on the condition, however, that the copier pay the stated per-copy fee through the Copyright Clearance Center, Inc., 21 Congress Street, Salem, Massachusetts 01970, for copying beyond that permitted by Sections 107 or 109 of the U.S. Copyright Law. This consent does not extend to other kinds of copying, such as copying for general distribution, for advertising or promotional purposes, for creating new collective works, or for resale.