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 Table of Contents  
Year : 2016  |  Volume : 7  |  Issue : 1  |  Page : 71-74  

Central myofibroma of the maxilla

Department of Oral and Maxillofacial Surgery, Punjab Government Dental College and Hospital, Amritsar, Punjab, India

Date of Web Publication22-Feb-2016

Correspondence Address:
Rajat Chowalta
Rajat Villa, H. No. 76, Shakti Nagar Jaunaji Road, Solan - 173 212, Himachal Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0976-237X.177109

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Myofibroma is a rare benign localized or generalized proliferation of myofibroblastic tissue occurring mostly in infants or children. In the oral region, most lesions occur in the mandible, lip, buccal mucosa, and tongue; however, the lesions arising in the maxilla are very rare. Myofibroma has an aggressive clinical presentation and is often treated aggressively because of an inappropriate diagnosis. A unique feature of central myofibroma of the jaws is the potential for teeth and other odontogenic structures to be involved by tumor. We report a case of myofibroma arising in the left side of the maxilla of a 12-year-old girl and describe the differential diagnosis from other spindle cell lesions of neural and smooth muscle origin. We treated the case using surgical excision under general anesthesia. Immunohistochemical staining was done for establishing the diagnosis since histopathological diagnosis with conventional staining could not distinguish myofibroma from spindle cell tumors.

Keywords: Central myofibroma, immunohistochemical staining, maxillary bone

How to cite this article:
Kaur P, Chowalta R, Lata J. Central myofibroma of the maxilla. Contemp Clin Dent 2016;7:71-4

How to cite this URL:
Kaur P, Chowalta R, Lata J. Central myofibroma of the maxilla. Contemp Clin Dent [serial online] 2016 [cited 2021 Mar 8];7:71-4. Available from:

   Introduction Top

Solitary central myofibroma [1],[2],[3] is a rare neoplasm that typically arises in soft-tissue and subcutaneous sites in the head and neck, but rarely within bone. The lesion is thought to represent a benign proliferation of the “myofibroblast.”

Foss and Ellis [4] analyzed 79 cases of myofibroma in the oral cavity and reported only four cases in maxilla. Montgomery et al.[5] analyzed nine cases of myofibroma of oral cavity and found no intraosseous maxillary tumor.

In the oral region, most lesions occur in the mandible, lip, buccal, mucosa, and tongue. However, the solitary myofibroma of maxilla is very rare.

   Case Report Top

A 12-year-old female child reported to the Department of Oral and Maxillofacial Surgery of Punjab Government Dental College and Hospital with the chief complaint of painless swelling in the left upper front region of face since 8 months.

Extraorally, the face was asymmetrical with swelling present on left anterior maxillary region measuring 6 cm mediolaterally × 5 cm superioinferiorly. The swelling extended up to the lateral wall of the nose medially and infraorbital rim superiorly causing decrease in the size of left palpebral fissure [Figure 1]. The overlying skin was normal in color and texture and not adherent to the underlying tissue swelling was firm in consistency on palpation. There was no complaint of any paresthesia.
Figure 1: Extraoral swelling extending up to the lateral wall of nose medially and infraorbital rim superiorly causing decrease in the size of left palpebral fissure

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Intraorally, the swelling extended from left upper central incisor to first molar, buccopalatally. There was displacement of canine. The first and second premolars were clinically missing in the left maxillary region. The canine was slightly mobile, and all the involved teeth were vital. Overlying mucosa was smooth but slightly inflamed with no surface ulceration. The aspiration test was negative.

Panoramic radiograph showed well-defined radiolucency extending from canine to the first molar with distal tilting of canine and the first premolar and medial tilting of the first molar. Computed tomography (CT) revealed large expansile soft-tissue attenuation mass with relation to left maxilla with area of necrosis. The mass was causing thinning and erosion of walls of maxillary antrum and medially causing partial compression of nasal airway [Figure 2]. No significant lymphadenopathy was seen. CT scan report suggested nonossifying fibroma.
Figure 2: Three-dimensional computed tomography face showing the extent of involvement of lesion

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On the basis of clinical and radiographic examination, it was provisionally diagnosed as an ossifying fibroma of maxilla. Ameloblastoma, keratocystic odontogenic tumor, odontogenic myxoma, and adenomatoid odontogenic tumor were kept as differential diagnosis. Considering the well-defined nature of lesion, excisional biopsy was planned.

On exposure, an encapsulated tumor mass was directly visualized because the alveolar and maxillary bone were destroyed. Since the canine and the first premolar were in close contact with the tumor, the whole of the tumor mass was easily shelled out from the surrounding tissues along with the two teeth and the specimen was sent for histopathological examination. The resected tumor was firm, encapsulated mass measuring 6 cm × 5 cm with well-defined borders and a whitish surface [Figure 3].
Figure 3: Firm, encapsulated mass measuring 6 cm × 5 cm with well.defined borders and a whitish surface

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The histopathological report revealed that the lesion contains sweeping fascicles of spindle-shaped cells with whorling patterns at many places. Small slit-like vascular spaces (hemangiopericytomatous pattern) were seen with areas of calcification noted. No mitotic activity was seen. Immunohistochemical analysis revealed that the tumor cells were positive for vimentin and alpha-smooth muscle actin (α-SMA), and negative for Ki67 and S-100 and focal positivity for desmin. On the basis of strong positivity for vimentin and α-SMA, the tumor was immunohistologically diagnosed as a myofibroma of the maxilla. In our case, the complete surgical excision of the lesion was performed along with involved teeth. There is no report of recurrence until date.

   Discussion Top

Myofibromas and myofibromatosis represent the mysterious group of lesions that were first described by Stout [6] as congenital multicentric fibroblastic proliferation. They were eventually characterized as phenotypically myofibroblastic tumors of infants and children by Chung and Enzinger [7] who noted the predominance of solitary tumors compared with tumors of multicentric origin.

Williams and Schrum,[8] first, classified these lesions as congenital fibrosarcomas. The patients were newborns and infants who presented with multiple nodular lesions of the skin, subcutaneous tissue, muscle, bone, and viscera. These visceral lesions adversely affect the prognosis.

Kauffman and Stout [9] subsequently subclassified congenital fibromatosis into multiple and generalized forms. However, Jones et al.[2] in accordance with Smith et al.[10] preferred the term myofibroma when describing a solitary neoplasm of this type.

The solitary myofibroma is a benign lesion found in the superficial soft-tissues and recently has been recognized as separate entity from the more aggressive deeper multiple lesions of infantile myofibromatosis. Soft-tissue lesions occur predominantly in the head and neck, including mouth, and the diagnostic criteria are described for other body sites. Intraosseous lesions are much less common but have a marked predilection for the skull and the mandible.

The etiology of myofibromas is presently unknown. Many authors have worked on this and suggested that the tumors are inherited as an autosomal dominant [11] or an autosomal recessive [12] trait. As the lesion develops from the benign proliferation of myofibroblasts, these cells are thought to play an important role in wound healing.[13] This suggests that a previous history of trauma may contribute to the development of these lesions.

Clinically, the lesions are more often seen in infants although the age range is wide. The presenting symptoms depend on site of involvement. In most of the cases, periosteal expansion may be slight or it may be a coincidental finding on the radiographic examination. Erupted and unerupted teeth may be displaced. Radiographically, the lesions appear benign and are often assumed to be odontogenic tumor or cyst,[14] because they are sharply defined radiolucencies with a variably thick sclerotic rim.

Histologically, the lesions contain two distinctive patterns seen in soft-tissue lesions. Sweeping fascicles or whorled spindle cells lie in a variably collagenous matrix. The more fibrous areas stand out as pale-staining poorly demarcated patternless zones which at low power resemble scar or chondroid. They contain larger elongate spindle- or strap-like cells often with an eosinophilic cytoplasm. The second pattern is densely cellular and comprises rounded or ovoid cells arranged with no particular architecture but containing cleft-like vascular spaces. The vascular spaces are often described as hemangiopericytomatous, but large stag's horn formations are unusual in intraosseous lesions and clefts are smaller and less numerous than in soft-tissue lesions. Occasional mitoses may be present in the cellular areas, and calcification may occur in the scar-like areas. The zoning phenomenon, which is useful in the diagnosis of soft-tissue lesions, is often not apparent in intraosseous lesions.

Due to these findings, this disease is often misdiagnosed as benign and malignant spindle cell lesions of nerve tissue or smooth muscle origin (leiomyoma).[15],[16]

Hence, immunohistochemical staining [17] is a useful tool to identify the nature of neoplastic cells and to reach an accurate diagnosis. Vimentin is the most widely expressed intermediate filament protein thought to be involved in structural processes. This protein is a general marker of cells originating in the mesenchyme. α-SMA expression is the most used marker for myofibroblast identification.

Neural lesions can be excluded because of their immunoreactivity with S-100, which are absent in myofibroma. Thus, understanding the clinical and pathological characteristics of myofibroma is very important to establish the correct diagnosis and avoid unnecessary treatment modalities.

Immunohistochemistry helps distinguish myofibroma from fibrosarcoma, the former is positive for vimentin, α-SMA and negative for desmin and S-100, whereas the latter is negative for α-SMA, also fibrosarcoma of the bones can be differentiated from myofibroma by the presence of a “herring-bone” pattern, nuclear atypia, and high mitotic counts including abnormal mitoses.

Other possible misdiagnoses include leiomyoma,[15],[16] schwannoma, fibrous histiocytoma, fibroma, hemangiopericytoma, and nodular fasciitis.[18] Nodular fasciitis gives a short preoperative duration of not more than 1–2 months in most, but not all, cases with tenderness. Extravasated red cells, chronic inflammatory cells, and multinucleated osteoclast-like giant cells are other frequently identified features. Nodular fasciitis also differs from myofibroma in its tendency to be mitotically active. Schwannoma, fibrous histiocytoma, fibroma, and hemangiopericytoma are negative for α-SMA whereas leiomyoma is positive for desmin. Such immunohistological characteristics allow these tumors to be distinguished from myofibroma. Because the tumor in our patient was positive for vimentin and α-SMA, it was diagnosed as a myofibroma.

Curettage is generally curative as the lesions shell out readily. In case of large lesions, resection with 0.5 cm is advisable and occasional recurrence is to be expected.

   Conclusion Top

Central myofibroma is a typical tumor of child-hood and adolescents in the gnathic region. Solitary myofibromas of the head and neck region, such as other sites, are biologically inert and show very little or no recurrence following excision. However, because of complex anatomy and limitations in the head and neck, especially the gnathic region, a dramatic radiographic and clinicopathologic presentation is not uncommon, making the suspicion for malignancy high in such cases. Thus, accurate diagnosis via pathological findings including immunohistochemistry is essential to avoid wrong diagnosis and the consequent unnecessary pervasive treatment therapies.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Speight PM, Dayan D, Fletcher CD. Adult and infantile myofibromatosis: A report of three cases affecting the oral cavity. J Oral Pathol Med 1991;20:380-4.  Back to cited text no. 1
Jones AC, Freedman PD, Kerpel SM. Oral myofibromas: A report of 13 cases and review of the literature. J Oral Maxillofac Surg 1994;52:870-5.  Back to cited text no. 2
Lingen MW, Mostofi RS, Solt DB. Myofibromas of the oral cavity. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;80:297-302.  Back to cited text no. 3
Foss RD, Ellis GL. Myofibromas and myofibromatosis of the oral region: A clinicopathologic analysis of 79 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;89:57-65.  Back to cited text no. 4
Montgomery E, Speight PM, Fisher C. Myofibromas presenting in the oral cavity: A series of 9 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;89:343-8.  Back to cited text no. 5
Stout AP. Juvenile fibromatoses. Cancer 1954;7:953-78.  Back to cited text no. 6
Chung EB, Enzinger FM. Infantile myofibromatosis. Cancer 1981;48:1807-18.  Back to cited text no. 7
Williams JO, Schrum D. Congenital fibrosarcoma; report of a case in a newborn infant. AMA Arch Pathol 1951;51:548-52.  Back to cited text no. 8
Kauffman SL, Stout AP. Congenital mesenchymal tumors. Cancer 1965;18:460-76.  Back to cited text no. 9
Smith KJ, Skelton HG, Barrett TL, Lupton GP, Graham JH. Cutaneous myofibroma. Mod Pathol 1989;2:603-9.  Back to cited text no. 10
Jennings TA, Duray PH, Collins FS, Sabetta J, Enzinger FM. Infantile myofibromatosis. Evidence for an autosomal-dominant disorder. Am J Surg Pathol 1984;8:529-38.  Back to cited text no. 11
Baird PA, Worth AJ. Congenital generalized fibromatosis: An autosomal recessive condition? Clin Genet 1976;9:488-94.  Back to cited text no. 12
Darby I, Skalli O, Gabbiani G. Alpha-smooth muscle actin is transiently expressed by myofibroblasts during experimental wound healing. Lab Invest 1990;63:21-9.  Back to cited text no. 13
Souza DP, Loureiro CC, Rejas RA, Sousa SO, Raitz R. Intraosseous myofibroma simulating an odontogenic lesion. J Oral Sci 2009;51:307-11.  Back to cited text no. 14
Slootweg PJ, Müller H. Localized infantile myofibromatosis. Report of a case originating in the mandible. J Maxillofac Surg 1984;12:86-9.  Back to cited text no. 15
Baden E, Doyle JL, Lederman DA. Leiomyoma of the oral cavity: A light microscopic and immunohistochemical study with review of the literature from 1884 to 1992. Eur J Cancer B Oral Oncol 1994;30B: 1-7.  Back to cited text no. 16
Hasegawa T, Hirose T, Seki K, Hizawa K, Okada J, Nakanishi H. Solitary infantile myofibromatosis of bone. An immunohistochemical and ultrastructural study. Am J Surg Pathol 1993;17:308-13.  Back to cited text no. 17
Montgomery EA, Meis JM. Nodular fasciitis. Its morphologic spectrum and immunohistochemical profile. Am J Surg Pathol 1991;15:942-8.  Back to cited text no. 18


  [Figure 1], [Figure 2], [Figure 3]

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