Solitary intraosseous neurofibroma in the mandible mimicking a cystic lesion: A case report and review of literature

Table 1 Summary of reports of solitary intraosseous neurofibromas of the mandible

Ref.	Age (year)/sex	Site	Clinical features	Radiographic features	Root resorption	Size	Immunohistochemistry features	Surgical approach	Prognosis
Present case	37/F	Body	Numbness and swelling of the gum in the right lower molar area	Unilocular expansile radiolucency involving the inferior alveolar nerve canal	Yes	4.7 cm × 2.3 cm × 1.6 cm	Positive staining for S100, CD34, and H3K27me3,negative staining for SMA, NF, and EMA	Excision of the tumor and nerve	No recurrence observed nine months post-surgery
Kamalakaran et al[5]	5/M	Body-ramus	Limited mouth opening and enlarged submandibular lymph nodes	Expansile lesion with a mixture of radiolucent and radiopaque components	No	3.9 cm × 3.8 cm × 3.1 cm	Not reported	Segmental resection of the mandible	No recurrence observed one-year post-surgery
Sarkar et al[6]	2.5/M	Body-ramus	Swelling in the posterior right part of the mandible. Displacement of deciduous teeth	Well-definedexpansile solid-cystic lesion with evidence of cortical erosion	No	3.7 cm × 3.5 cm × 3.4 cm	S-100 multifocal and moderately positive, strong CD34 positivity EMA, cytokeratin, CD31, SMA, STAT-6, TLE-1, and HMB-45 negative MIB-1 index low	Preservation of the nerve while removing the mass	Not reported
Behrad et al[13]	32/F	Body	Swelling and intermittent dull pain in the left mandible	Unilocular expansile radiolucency involving the inferior alveolar nerve canal	No	3 cm × 1.5 cm × 2.4 cm	Not reported	Simple excision of the mass	Not reported
Iqbal et al[14]	13/M	Body-angle	Swelling in the posterior area of the right mandible	Ill-defined radiolucency	No	2.0 cm × 3.0 cm × 2.0 cm	S-100 positive	Simple excision of the mass	No recurrence
Inoue et al[15]	27/M	Ramus to infratemporal fossa	Progressive numbness on the lower right jaw	A nonenhanced mass in the infratemporal region, with an enlarged inferior alveolar nerve canal and foramen ovale	No	Not reported	S-100 positive	Excision of the tumor and nerve	No recurrence
Narang et al[16]	45/F	Left mandibular angle to right mental foramen	Swelling in the posterior area of the left mandible	The radiographic irregularity in the region of the inferior lacrimal canal	Yes	Not reported	S-100 positive, MIB-1 negative	Segmental resection of the mandible	Not reported
Fortier[17]	70/F	Body	Osteolytic lesion of the right posterior mandible	The clear single-eyed translucent area. Involvement of the inferior alveolar nerve canal	No	Not reported	S-100 positive	Preservation of the nerve while removing the mass	No recurrence
Gujjar et al[18]	28/F	Body	Swelling and intermittent dull pain in the left mandible	Uniform radiopacity. Involvement of the inferior alveolar nerve canal	No	3.0 cm × 4.0 cm	S-100 positive	Segmental resection of the mandible	Not reported
Saravani et al[19]	39/F	Body	Severe pain in the right posterior mandible	Relatively clear single-cyst projection shadow	No	Not reported	S-100 positive	Simple excision of the mass	No recurrence
Jangam et al[20]	62/F	Right body-left body	The lower jaw is significantly swollen. Occasionally, there is severe pain	Radiographically transparent image with clear borders, accompanied by thinning of the lower boundary	Edentulous jaw	Not reported	S-100 positive	Segmental resection of the mandible. Repair using a free fibular graft	No recurrence
Deichler et al[21]	14/M	Ramus	No clinical symptoms, discovered incidentally	Unilocular radiolucency	No	4.0 cm × 1.5 cm × 0.5 cm	Tumor cells: Vimentin positive, neurospecific enolase (NSE) positive and anti S-100 negative. Residual nerve fibres: S-100 positive; NSE positive	Simple excision of the mass	Not reported
Tao et al[22]	16/F	Ramus	Limited mouth opening, numbness in the lower left lip	Multilocular radiolucency with irregular edges	No	3.5 cm × 2.0 cm	S-100 positive	Segmental resection of the mandible. Repair using a free iliac bone graft	Not reported
Vivek et al[7]	39/F	Symphysis-parasymphysis	Spontaneous tooth loss, persistent tingling sensation in the lower lip	A relatively well-defined radiolucent area	No	Not reported	S-100 positive	Segmental resection of the mandible	No recurrence observed one-year post-surgery
Apostolidis et al8]	67/F	Body-ramus	Abnormal sensation in the lower left lip	Involvement of the inferior alveolar nerve canal	No	2.5 cm × 2 cm × 0.7 cm	Not reported	Excision of the tumor and nerve	No recurrence observed three-year post-surgery
Alatli et al[23]	37/F	Body	Abnormal sensation in the lower right lip	No abnormalities detected	No	Diameter of 1.3 centimeters	Not reported	Excision of the tumor and nerve	No recurrence observed two years post-surgery
Ueda et al[24]	37/M	Body	No clinical symptoms, discovered incidentally	Well-defined unilocular radiolucency. Involvement of the inferior alveolar nerve canal	No	Not reported	S-100 positive	Segmental resection of the mandible. Repair using a free scapular flap	No recurrence observed three years post-surgery
Papageorge et al[10]	4.5/M	Ramus-infratemporal fossa	Expansile lesion in the right mandible. Limited mouth opening, chin deviated to the left	Well-defined unilocular radiolucency. Involvement of the inferior alveolar nerve canal	No	Not reported	S-100 protein and vimentin positive	Segmental resection of the mandible. Reconstruction using autologous rib cartilage graft	No recurrence
Weaver et al[25]	22/F	Body	Swelling on the left side of the face	Well-defined unilocular radiolucency	No	5.0 cm × 3.0 cm	S-100 positive	Excision of the tumor and nerve.	No recurrence observed six months post-surgery
Polak et al[26]	60/M	Body	No clinical symptoms discovered incidentally. When palpated, a crackling sensation can be felt, similar to the sound of breaking eggshells	Unilocular radiolucency involving the mandibular canal	No	Not reported	S-100 and anti-Leu positive	Excision of the tumor and nerve	No recurrence observed six years post-surgery
Papadopoulos et al[27]	15/M	Body	Swelling and mild pain in the left mandible	Unilocular radiolucency near mental foramen	No	0.5 cm × 0.5 cm × 0.5 cm	Not reported	Excision of the tumor and nerve	No recurrence
Larsson et al[11]	25/F	Body-ramus	No clinical symptoms, discovered incidentally	Well-defined large radiolucency. Involvement of the inferior alveolar nerve canal	No	10 cm × 2.0 cm	Not reported	Excision of the tumor and nerve, with nerve end-to-end anastomosis	No recurrence observed two years post-surgery
Larsson et al[11]	46/M	Body	Swelling and slow, intermittent pain in the left mandible	Rounded, slightly radiopaque, well-circumscribed radiolucency	No	Diameter of 1 cm	Not reported	Remove all visibly abnormal tissues within the bone	No recurrence observed five months post-surgery
Ellis et al[28]	41/F	Body-ramus	Swelling in the right mandible	Poorly defined multilocular radiolucent lesion	No	Not reported	Not reported	Not reported	Not reported
Ellis et al[28]	4/F	Body	A firm lump on the right mandible	Well demarcated radiolucent lesion. Involvement of the inferior alveolar nerve canal	No	2.5 cm × 1.6 cm	Not reported	Not reported	No recurrence observed one-year post-surgery
Ellis et al[28]	8/M	Body-angle	Not reported	Well demarcated radiolucent lesion with sclerotic borders	No	6.0 cm × 4.0 cm	Not reported	Not reported	No recurrence observed one-year post-surgery
Ellis et al[28]	23/F	Body-ramus	Swelling in the posterior area of the right mandible	Radiolucent-radiopaque lesion with indistinct borders	No	Not reported	Not reported	Not reported	Recurrence observed after 3 yr. Partial mandibulectomy performed
Ellis et al[28]	4/M	Body	Swelling in the right mandible	Multilocular radiolucent lesion	No	Not reported	Not reported	Not reported	Not reported
Cundy and Matukas[29]	55/F	Body-angle	Pain and swelling in the left mandible, accompanied by discomfort while chewing	Unilocular radiolucency	No	Not reported	Not reported	Segmental resection of the mandible. Repair using a free iliac bone graft	Not reported
Cassalia and Miller[30]	16/F	Angle	No clinical symptoms, discovered incidentally	Multilocular radiolucency involving mandibular canal	No	Not reported	Not reported	Preservation of the nerve while removing the mass	No recurrence observed six months post-surgery
Sharawy and Springer[31]	22/F	Body-angle	Intermittent numbness in the left mandible, with slight swelling on the cheek side	Multilocular expansile radiolucency	No	Not reported	Not reported	Excision of the tumor and nerve.	No recurrence observed thirteen months post-surgery
Gutman et al[32]	5/F	Body	Painless swelling in the left mandible	Unilocular expansile radiolucency	No	2 cm × 4 cm	Not reported	Excision of the tumor and nerve	No recurrence observed sixteen months post-surgery
Gohel et al[12]	17/F	Body	A swollen lump on the gums of the lower right mandible	Osteolytic radiolucent lesion	No	4 cm × 1 cm	Not reported	Not reported	Not reported
Johnson et al[9]	34/F	Body	Abnormal sensation on the left side of the lower lip and tenderness in the lower left jaw during biting	Unilocular radiolucency	No	2.5 cm × 1.5 cm	Not reported	Not reported	Skin pigmentation observed six months later, followed by local recurrence two years post-surgery
CORNELL and VARGAS[33]	65/F	Body	Oral discomfort	Ill-defined unilocular lesion with unclear borders	Edentulous jaw	Not reported	Not reported	Excision of the tumor and nerve	No recurrence
BRUCE[34]	36/M	Body	A swelling on the alveolar ridge of the edentulous lower left jaw	Well-defined radiolucency involving mandibular canal	No	3 cm × 2 cm × 2 cm	Not reported	Not reported	Not reported
Blackwood and Lucas[35]	41/M	Body	Swelling in the posterior area of the left mandible	Unilocular radiolucency	Yes	2 cm × 1.5 cm ×1 cm	Not reported	Simple excision of the mass	Not reported

SMA: Smooth muscle actin; NF: Neurofilaments protein; EMA: Epithelial membrane antigen; STAT6: Signal transducer and activator of transcription 6; TLE: Temporal lobe epilepsy; HMB-45: Human melanoma black-45.

Table 2 Differential diagnosis of benign lesions in the mandible

Disease	Age (year)	Sites	Radiographic Findings
Radicular (periapical) cysts	In the 3rd to 5th decades of life	Located at the apices of dead pulp teeth, caused by inflammation of the apical tissue due to caries or trauma	Circular, unilocular low-density images at the apex of the tooth. Massive periapical cysts may cause root resorption, displacement of adjacent structures, and expansion
Dentigerous cysts	In adolescents and young adults	The upper canine and lower third molar	Clear radiolucent image around the tooth crown with cortical border causing a significant displacement. Expansion and root resorption may be present. The cortical border is usually preserved
Simple bone cysts	In the 2nd decades of life	The posterior part of the mandible	A radiolucency scalloping between the roots of the teeth
Odontogenic keratocysts	In the 3rd decades of life	The posterior part of the mandible	Unilocular/multilocular lesions with scalloped margins. May present as radiolucent around the tooth crown. May lead to cortical thinning, tooth displacement, and root resorption
Ameloblastoma	In the 3rd to 5th decades of life	The posterior part of the mandible	Well-defined unilocular/multilocular radiolucency. May appear as a pericoronal radiolucency. Extensive root resorption. Has a typical “soap bubble” appearance
Aneurysmal bone cyst	In young adults	The posterior part of the mandible	A unilocular or multilocular radiolucency with cortical expansion
Central giant cell granuloma	In adolescents and young adults	The anterior part of the mandible	Mandibular border is clearly defined. Radiolucent with granular calcifications. May cause tooth displacement, root absorption, destruction of cortical plates, and invasion into surrounding soft tissues. Early-stage lesions: Small unilocular areas (similar to odontogenic cysts). Progressive stage: Multilocularity with sparse internal septa and bone expansion
Schwannomas	Most often in the 4th and 5th decades	The posterior part of the mandible	Radiolucent, unilocular,homogeneous, and well-defined lesions with cortical expansion
Neurofibromas	In adolescents and young adults	The posterior part of the mandible	Low-density unicystic or multicystic images with clear or unclear borders. They can expand and penetrate the boundary of the dermis. Tooth root absorption and tooth displacement may also occur. Fusiform enlargement of the mandibular canal
Central hemangioma	In the 2nd decades of life	It mainly occurs in the spine. The mandible is a very rare location, with the posterior part of the mandible being slightly more common	Unilocular or multilocular lesion, with large bone marrow spaces and rough trabeculae formation. Accompanied by a typical honeycomb or soap-bubble appearance. When it occurs within the inferior alveolar canal, the canal was wider than normal
Arteriovenous malformation	In the 1st decades of life	They are uncommon lesions of the head and neck. The majority of jaw lesions occur in the mandibular ramus and body	May appear cystic due to adjacent bone resorption. May appear multilocular. When located within the inferior alveolar canal, the canal can enlarge throughout the entire course
Lymphoma	Most patients aged between 40 and 60 yr	Mandibular body	Radiolucent pattern with non-transmitted radiation particles and reactive bone attachment. May cause “moth-eaten” appearance, lamellar bone formation in periosteum, widening of mandibular canal, irregular increase in periodontal ligament width, and tooth spacing