Solitary neurofibroma of the mandible: Case report and review of the literature

65

POLAK ET AL

J Oral Maxillofac

Surg

47:65-98.1969

Solitary Neurofibroma

of the Mandible:

Case Report and Review of the Literature MICHEL POLAK, MD,* GEORGES POLAK, DDS,t CLAUDE BROCHERIOU, MD,+ AND JACQUES VIGNEUL, MD$ Tumors of the peripheral nervous system, neurofibromas and schwannomas, often occur in the head and neck region. Das Gupta et al found that among 303 cases of benign nerve sheath tumors, 44.8% occurred in this region and ~9% occurred intraorally.’ We report a case of a neurofibroma of the mandible. This location is unusual; none of the cases of Das Gupta et al, nor of the 19 neurofibromas and 18 schwannomas reported by Cherrick and Eversole,’ occurred in the oral cavity. Report of a Case A 60-year-old man was seen by his dentist for his annual visit. A panoramic radiograph showed a unilocular radiolucent area in the of right mandible, underlying the first molar. The area was well circumscribed, with a homogenous background, and was centered over the mandibular canal. The canal appeared to be enlarged (Fig 1). The tooth overlying the lesion showed no evi* Laboratoire de neuropathologie, Hopital de la Salpetriere, Paris. t Private practice, Montgeron, France. $ Professeur d’anatomo-pathologie, Laboratoire d’ anatomie pathologique, Hopital Saint-Louis, Paris. § Professeur au College de medecine, Chirurgien Maxillofacial, Paris. Address correspondence and reprint requests to Dr M. Polak: 7 rue Claude Bernard, 75005 Paris, France. 0 1989 American geons

Association

of Oral and Maxillofacial

FIGURE 2. sion.

Tomography

showing the well-circumscribed

le-

dence of pulpal involvement or thickening of the periodontal ligament. The lamina dura at the apex was intact. The lesion was indolent and the patient did not complain of dysesthesia or pain. There was no expansion of the vestibule, anesthesia in the distribution of the mental nerve, or swelling in the area; but an eggshell type of crackling was felt on palpation. The presumed diagnosis was either nerve tumor or hemangioma. Tomography did

Sur-

0278-2391/89/4701-0013$3.00/O

FIGURE 1. Panoramic radiograph showing the radiolucent area and the enlargement of the mental canal.

FIGURE 3. Photomicrograph of a section from the tumor showing bundles of spindle-shaped cells within a delicate stroma. (Hematoxylin-eosin. Original magnification, X4Cltl)

66

SOLITARY NEUROFIBROMA

Table 1.

OF THE MANDIBLE

Case Reports of Solitary Neurofibroma of the Mandible

Age Site

tyr)

Sex

Blackwood and LucasI

41

M

Bruce”

36

M

2

M

Cassalia and Miller”

16

F

Cornell and Vargas’*

65

F

Cundy and Matukas” Ellis et al2

55

F

4

M

Ellis et al”

23

F

Ellis et al”

8

M

Ellis et a12’

41

F

Freedus and Doyle2’

21

M

Friedman”

12

F

Giamminola et alz3

21

M

Giamminola et al23

4

F

Goldman24

33

M

Gnepp and Keyes2’

39

F

Gnepp and Keyes25

24

F

5

F

Johnson et al”

34

F

Larsson et al**

46

M

Left mandible, second molar area

Larsson et al’*

25

F

Right mandible

Reference

Caldwell et aIn’

Gutman et a126

Left mandible, second premolar to first molar

Left mandible, molar region Right mandible, distal to third molar Left mandible, molar region Left mandible, molar region Right mandible, molar region and angle Right mandible, second molar to coronoid process Left mandible, body and angle Right mandible, premolar region to coronoid process Right mandible, body and ramus Right mandible, first premolar to third molar Angle of the mandible to the first molar Angle of the mandible Mandible, first premolar into ramus Left mandible, under first molar and tirst and second premolars Left mandible

Left mandible, premolar region Left mandible, molar region

(Continued

Radiographic Appearance

Symptoms

Follow-up*

Radiolucency

Buccal expansion, clicking in ear

None

Mandibular canal not involved, osteolytic defect Poorly defined radiolucency Multilocular radiolucency

Pain

None

Lingual swelling

Recurred, 2 mo; NR, 2 yr NR, 6 mo

Poorly defined radiolucency

Discomfort, crepitation of buccal plate Pain and swelling

None

Multilocular radiolucency

Swelling

None

Poorly defined multilocular radiolucency Well-demarcated radiolucency Poorly defined multilocular radiolucency Multilocular radiolucency Well-defined radiolucency

Swelling

Recurred, 3 yr; NR, 2 yr

None

NR. 3 mo

Swelling

None

Swelling

None

Swelling

None

Radiolucency

Pain, cortical expansion

None

Radiolucency, root resorption of primary molar Radiolucency

Pain, cortical expansion

None

Recurrent pain

None

Radiolucent area

None

NR. 18 mo

Moderately well-defined unilocular radiolucency Unilocular radiolucency Radiolucency

Asymptomatic

NR, 29 mo.

Lingual swelling

NR, 16 mo.

Initially none, recurrent lesion: pain paresthesia Swelling

Recurred, 6 mo

Swelling

NR, 2 yr

Radiolucency

Rounded, slightly radioopaque, well-circumscribed area Upper part of the ramus to the first molar area on following

page)

None

None

NR, 5 mo

67

POLAK ET AL

Table 1. Case Reoorts of Solitary Neurofibroma of the Mandible ICont’dI Age (yr)

Sex

23

F

et al”

15

M

Prescott and White”

5

M

Schro@’

46

F

Sharawy and SpringeP3

22

F

Singer et al34

30

F

Thoma3’

39

F

Villa et al36

17

F

Reference Morgan and Morgan” Papadopoulos

Site Right mandible, anterior Left mandible, anterior Right mandible, first molar Body and ramus of mandible Left mandible, third molar to angle Left mandible, posterior Premolar and molar area Premolar and molar area

Radiographic Appearance

Follow-up*

Symptoms

Radiolucency

Slight pain

Recurred, 9 yr

Radiolucency below the left mental foramen Circumscribed radiolucency Large radiolucency

Slight pain on palpation

None

Swelling

NR, 4 yr

Pain and cortical expansion Paresthesia, tenderness, buccal expansion Paresthesia and pain

None

Cortical expansion

None

Cortical expansion

None

Multilocular radiolucency Radiolucency Mandibular canal expanded Osteolytic changes in area

NR, 13 mo

None

* NR, no recurrence.

not contribute to the diagnosis (Fig 2). Selective angiography of the external carotid artery excluded a diagnosis of hemangioma. The tumor was easily enucleated under general anesthesia. Because the nerve anterior to the tumor seemed to be involved, it was removed. Microscopic examination of the tumor showed bundles of ovoid or spindle-shaped cells intermingled with a delicate fibrillar stroma (Fig 3). The nerve was in contact with the tumor. Numerous mast cells were scattered throughout the stroma. Immunohistochemical studies showed positive staining of the tumor cells for anti-S100 protein antibodies and anti-Leu 7 antibodies.3 A diagnosis of neurotibroma was made. Six years after surgery, the patient is free of recurrence and no signs of von Recklinghausen disease have appeared. Discussion The difference between schwannoma and neurofibroma has prognostic value. Malignant transformation of both solitary4 and multiple schwannomas is extremely rare. The potential malignancy of solitary neurotibroma is probably also negligible, but in 5% to 16% of the patients with neurofibromatosis malignant changes occur in one or more neurofibromas .5-7 Schwannoma is a benign encapsulated neoplasm that arises within a nerve and is composed primarily of cells in a poorly collagenized stroma.’ Current data suggest that the tumor cell is the schwann ce11.9F’0The tumor consists of variable amounts of two types of tissue, Antoni A and Antoni B.*,” The treatment for schwannoma is surgical excision. Recurrence is rare, perhaps because the tumor is well encapsulated. It is usually a solitary lesion, commonly located on the acoustic nerve, a dorsal root

in the spinal canal, or on peripheral nerve trunk.12 When multiple, the tumors may be associated with neurofibromatosis. l3 The solitary neurofibroma is a benign, slowly growing neoplasm, relatively circumscribed but nonencapsulated, originating within a nerve and composed of schwann cells, perineural cells, and mature collagen. Ultrastructural features suggest that the original cell may be the perineural tibroblast.9*‘0 Microscopically, the tumor consists of scattered fusiform cells with hyperchromatic nuclei. The cells are set in a myxomatous matrix of broad collagen fibers. Mast cells, lymphocytes, and small nerve fascicles can be seen in the tumor. The absence of encapsulation makes complete surgical removal of a solitary neurofibroma difEcult. Incomplete removal probably accounts for some cases of recurrence. In our case, the excision was not limited to the core of the tumor but also involved its extensions; this may account for the nonrecurrence. Therefore, it is important not only to remove the main portion of the tumor but also to excise the possible extensions along the nerve. The distinction between solitary neurofibromas and the neurofibromas of neurofibromatosis is mainly clinical; the microscopic features are the same. However, some authors have stated that the neurofibroma of neurofibromatosis is usually less sharply limited from the surrounding normal tissue. l1 A particular typ e known as plexiform neurofibroma seems to occur only in patients with neurofibromatosis.8~‘o Neurofibromas often occur in the cervicofacial region. Among 66 neurofibroma in the facial region,

SOLITARY NEUROFIBROMA

the following distribution was found in the literature: tongue, 12; palate, 12; mandibular ridge/ vestibule, 15; maxillary ridge/vestibule, 9; buccal mucosa, 10; lip, 4; mandibular intrabony, 2; gingiva, 1; and mouth floor, 1.2y9The mandibular intrabony location is rare. In the literature, we found only 29 cases of solitary neurotibroma of the mandible (Table 1). ‘4-36When neurofibroma occurs in the mandible, there appears to be a female predominance. The mean age of patients is 24 years. Tumors most often occur in the posterior mandible, swelling is the most common clinical symptom of intramandibular neurofibroma, and mandibular enlargement and pain are observed less frequently. Radiologically, the tumor appears as a nonspecific, unilocular or multilocular, poorly defined or welldemarcated, radiolucency . In one case, a radiopaque area was described.28 An expansion of the mandibular canal may be seen. Among 13 cases of solitary neurofibroma of the mandible that were followed up, four recurrences were reported (a recurrence rate of about 30%, higher than for schwannoma), suggesting that regular examinations for recurrence should be conducted. Signs of neurofibromatosis are found less frequently in cases of neurofibroma of the mandible than in neurofibroma in other locations; we noted only three cases of mandibular intrabony neurotibroma associated with neurotibromatosis reported in the literature.37-39 Summary A case is presented of a neurofibroma of the mandible without recurrence 6 years after surgery. Search for neurofibromatosis was negative. Eradication of the tumor was explained by the extent of the operation that was performed.

9.

10.

11. 12.

13. 14. 15. 16. 17. 18. 19. 20.

21. 22. 23. 24. 25. 26. 27. 28. 29. 30.

References 1. Das Gupta TK, Brasfield RD, Strong EW, et al: Benign solitary schwannomas (neurilemomas). Cancer 24:355, 1969 2. Cherrick HM, Eversole LR: Benign neural sheath neoplasm of the oral cavity: Report of thirty-seven cases. Oral Surg 32900, 1971 3. Perentes E, Rubinstein LJ: Recent applications of immunoperoxidase histochemistry in human neuro-oncology. Arch Path01 Lab Med 111:7%, 1987 4. Carstens PHB, Schrodt GR: Malignant transformation of a benign encapsulated neurilemoma. Am J Clin Path01 51:144, 1969 5. Preston FW, Walsh WS, Clarke TH: Cutaneous neurotibromatosis (van Recklinghausen disease): Clinical manifestations and incidence of sarcoma in 61 male patients. Arch Surg 64:813, 1952 6. Holt FJ, Wright EM: Radiologic features of neurotibromatosis. Radiology 51647, 1948 7. Saxen E: Tumors of sheats of peripheral nerves-Studies on their structure, histogenesis and symtomatology. Acta Path01 Microbial Stand 79: 1, 148 (suppl) 8. Harkin JC, Reed RJ: Tumors of the Peripheral Nervous Sys-

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32. 33. 34.

35. 36.

37.

38. 39.

OF THE MANDIBLE

tern. Washington, DC, Armed Forces Institute of Pathology. 1968 Chen SY, Miller AS: Neurotibroma and schwannoma of the oral cavity: A clinical and ultrastructural study. Oral Surg 47:522, 1979 Chomette G, Auriol M. Tranbaloc P, Bertrand JCh: Schwannomes et neurolibromes de la region cervico-faciale. Arch Anat Cytol Path01 32:69, 1984 Wright BA, Jackson D: Neural tumors of the oral cavity. Oral Path01 49:509, 1980 Leroux-Robert J: Tumeurs nerveuses O.R.L. et cervicofaciales. Actualites de carcinologie cervico-faciale, Masson ed. Paris Abel1 MR. Hart WR, Olsen JR: Tumors of the peripheral nervous system. Hum Pathol 1:503, 1970 Blackwood JH, Lucas RB: Neurotibroma of the mandible. Proc R Sot Med 44:864. 1951 Bruce KW: Solitary neurotibroma (neurillemmoma. schwannoma) of the oral cavity. Oral Surg 7: 1150, 1954 Caldwell JB, Hughes KW, Cox RS: Neurofibroma of the mandible: Report of case. J Oral Surg 19:166, 1961 Cassalia PT. Miller AS: Solitary central neurotibroma of the mandible. Br J Oral Surg 8:270, 1971 Cornell CF. Vargas HA: Intraosseous neurotibroma of the mandible. Oral Surg 8:34, 1955 Cundy RL. Matukas VJ: Solitary intraosseous neurotibroma of the mandible. Arch Otolaryngol 96:81. 1972 Ellis GL. Abrams AM, Melrose RJ: Intraosseous benign neural sheath neoplasms of the jaws: Report of seven new cases and review of the literature. Oral Surg 44:731, 1977 Freedus MS, Doyle PK: Multiple neurofibromatosis with oral manifestations. J Oral Surg 33:360. 1975 Friedman MM: Neurotibromatosis of bone. Am J Roentgenol 51:623. 1944 Giamminola E. Fabri N, Merlini C: Neurofibroma of the mandible. Riv ha1 Stomatol 20:318, I%5 Goldman HM: Case reports from the army medical museum. Am J Orthod 30:289. 1944 Gnepp DR. Keyes GG: Central neurotibroma of the mandible. J Oral Surg 39:125, 1981 Gutman D, Griffel B. Munk J. et al: Solitary neurotibroma of the mandible. Oral Surg 17:1. 1964 Johnson HS, Wannamaker GT, Humes JJ, et al. Central neurofibroma: Report of a case. Oral Surg 12:379, 1959 Larsson A, Praetorious F. Hjorting-Hansen E: Intraosseous neurofibroma of the jaws. Int J Oral Surg 7:494, 1978 Morgan GA, Morgan PR: Neurilemmoma-neurofibroma. Oral Surg 25:182, I%8 Papadopoulos H. Zachariades N, Angelopoulos AP: Neurofibroma of the mandible. Int J Oral Surg 10:293, 1981 Prescott GH, White RE: Solitary central neurotibroma of the mandible: Report of case and review of the literature. J Oral Surg 28:305, 1970 Schroff J: Solitary neurofibroma of the oral cavity. JADA 32:199, 1945 Sharawy A. Springer J: Central neurofibroma occurring in the mandible. Oral Surg 25:817, I%8 Singer CF. Gienger GL, Kullbom TL: Solitary intraosseous neurofibroma involving the mandibular canal: Report of case. J Oral Surg 31:127, 1973 Thoma K: Oral Pathology (ed 4). St Louis, Mosby. 1954 Villa V, Laico JE, Banez L: Central neurofibroma of the mandible associated with an occasional spontaneous hemorrhage. Oral Surg 15:836, 1%2 Lorson EL, Delong PE, Osbon DB, et al: Neurotibromatosis with central neurofibroma of the mandible. J Oral Surg 35:733. 1977 Fawcett KJ, Dahlin DC: Neurolemmoma of bone. Am J Clin Path01 47~759, 1%7 Uhlmann E. Grossman A: Von Recklinghausen’s neurofibromatosis with bone manifestations. Ann Intern Med 14:225. 1940