Leiomyoma of the maxilla: Case report with review of literature

Siddana Gouda Siddana; Manjunath Muniraju; Deepak Thipasandra Ashwathappa; Sangeetha

doi:10.4103/0972-1363.145021

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CASE REPORT

Year : 2014 | Volume : 26 | Issue : 3 | Page : 331-334

Leiomyoma of the maxilla: Case report with review of literature

Siddana Gouda Siddana¹, Manjunath Muniraju², Deepak Thipasandra Ashwathappa², Sangeetha³
¹ Department of Oral Medicine and Radiology, College of Dental Science, Bhavnagar, Gujarat, India
² Department of Oral Medicine and Radiology, Vokkaligara Sangha Dental College and Hospital, Bangalore, Karnataka, India
³ Department of Oral Pathology, KLE Institute of Dental Sciences, Bangalore, Karnataka, India

Date of Submission	09-Jul-2014
Date of Acceptance	04-Nov-2014
Date of Web Publication	19-Nov-2014

Correspondence Address:
Siddana Gouda Siddana
Department of Oral Medicine and Radiology, College of Dental Science, Amargadh, Bhavnagar, Gujarat
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/0972-1363.145021

Abstract

Leiomyoma is a benign smooth muscle tumor that may appear in any location of the body and is most commonly seen in the uterus and gastrointestinal tract. The occurrence of leiomyoma is rare in the orofacial region due to the scarcity of smooth muscles in this region. Overall incidence in the orofacial region is 0.065%. It is most commonly seen in the age group of 40-50 years without much gender difference. Clinically, it appears as a well-defined asymptomatic swelling. Histologically, angioleiomyoma is the most common type of leiomyoma in the orofacial region. Management is done by surgical excision with safe margins. Recurrence of the tumor is rare. We hereby present a case of leiomyoma at a rare site, its treatment, and a review of literature.

Keywords: Benign, smooth muscle, tumor

How to cite this article:
Siddana SG, Muniraju M, Ashwathappa DT, Sangeetha. Leiomyoma of the maxilla: Case report with review of literature . J Indian Acad Oral Med Radiol 2014;26:331-4

How to cite this URL:
Siddana SG, Muniraju M, Ashwathappa DT, Sangeetha. Leiomyoma of the maxilla: Case report with review of literature . J Indian Acad Oral Med Radiol [serial online] 2014 [cited 2022 May 22];26:331-4. Available from: https://www.jiaomr.in/text.asp?2014/26/3/331/145021

Introduction

Leiomyoma is a benign mesenchymal tumor of the smooth muscle origin and is most commonly seen in uterus and gastrointestinal tract. The occurrence of leiomyoma in the orofacial region is less reported, with an incidence of 0.065%. This low rate of occurrence is due to the scanty amount of smooth muscles seen in this region. ^[1],[2]

Leiomyoma in the orofacial region may arise from either the smooth muscle found around the blood vessels, the embryonic remnants of mesenchymal cells like circumvallate papilla of the tongue, or the excretory ducts of salivary glands. ^[2] Leiomyoma is commonly seen in the 4 ^th -5 ^th decades with variable sex predilection. ^[3] In some cases, it may be associated with pain or mild tenderness with secondary symptoms like bleeding, difficulty in swallowing, mobility of teeth, etc. ^[4] Clinically, leiomyoma must be differentiated from salivary gland tumors, vascular malformation and its malignant form, leiomyosarcoma. ^[5] The tumor shows plenty of spindle cells with cigar-shaped nuclei on special staining. ^[6] Recurrence of the tumor is rare. ^[7]

Case Report

A 38-year-old male patient reported to the Department of Oral Medicine and Radiology with the complaint of swelling in the left side of the face. On eliciting the history, it was found that the swelling was present since the past few years. There was no history of trauma. Initially, the swelling was small in size and slowly increased to the present size, and was not associated with pain. His medical and family history was non-contributory. On inspection, the swelling appeared diffuse because of the soft tissue contour (but on palpation, it was well defined). On extraoral examination, the swelling was present over the left side of the midface, measuring about 3 × 3 cm and extending superoinferiorly from the ala-tragus line to the level of corner of the mouth. It extended anteroposteriorly around 2 cm posterior to the left ala of nose to a region 3 cm anterior to the tragus. Skin over the swelling appeared normal [Figure 1]a. On palpation, the swelling was firm and tender with distinct borders and without any rise in temperature.

Figure 1: (a) Extraoral photograph showing swelling in the left check region. (b) Intraoral photograph showing well-defi ned swelling in the upper left buccal vestibule

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Intraorally, a well-defined swelling was seen in the left buccal vestibule, measuring about 1 × 2 cm with no changes in the overlying mucosa. On palpation, it was firm and tender. There were no signs of teeth displacement or missing teeth [Figure 1]b. Based on the history and clinical examination, a provisional diagnosis of fibroma was made. Lipoma, neuroma, peripheral giant cell granuloma, rhabdomyoma, leiomyoma, dermoid cyst, and minor salivary gland tumor were considered as the differential diagnoses.

Radiographic evaluation revealed no associated changes. On fine needle aspiration cytology, abundant clusters of spindle and ovoid cells with some cells having cigar-shaped nuclei with blunted ends were seen [Figure 2]. The tumor was excised under general anesthesia. The excised specimen was red and oval in shape, measuring about 2 × 2 cm [Figure 3]. Histopathological examination of the excised specimen revealed well-encapsulated mass with hypercellularity. The spindle-shaped cells were arranged in long fascicles with numerous blood vessels having thickened walls. The cells had eosinophilic cytoplasm with blunt and cigar-shaped nuclei [Figure 4]. Special staining with Masson trichrome stain was carried out to confirm the origin of the spindle cells [Figure 5]. A final diagnosis of benign spindle cell tumor, angioleiomyoma, was made.

Figure 2: Aspiration cytology showing spindle-shaped cells

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Figure 3: Excised tumor mass

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Figure 4: Photomicrograph showing numerous spindle-shaped cells with large vascular spaces

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Figure 5: Positive Masson trichrome staining for smooth muscle cells

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Discussion

In 1854, Virchow first described a case of leiomyoma. ^[8] Leiomyoma is a benign smooth muscle tumor that may appear in any location of the body. It most commonly occurs in the uterus (95%), gastrointestinal tract, and skin (4-5%). It has been reported rarely occurring in the orofacial region, with the incidence being about 0.065%. ^[1],[2] Based on the anatomic site and the origin of the smooth muscle, leiomyomas are classified into three types: Piloleiomyoma, angioleiomyoma, and genital leiomyoma. ^[9] The World Health Organization classified leiomyoma histologically into three types: Leiomyoma (solid), angioleiomyoma (vascular leiomyoma), and leiomyoblastoma (epithelial leiomyoma). ^[10] Various authors have expressed different views on the pathophysiology of leiomyoma. According to Duhig and Ayer, ^[11] the vascular leiomyoma represents only a stage within a continuous maturation process of smooth muscle fibers. The maturation sequence is as follows: Hemangioma, angioma, vascular leiomyoma, leiomyoma, and solid leiomyoma. According to Damm and Neville, ^[12] solid leiomyoma is histologically different from angioleiomyoma. Hence, they opined that the two entities should be regarded separately.

The leiomyomas are infrequent in the orofacial region. The case reported here features a rare site of occurrence of an angioleiomyoma, viz., the orofacial region. In 1884, Blanc reported the first case of oral leiomyoma. ^[13] These leiomyomas arise either from the smooth muscles of the tunica media of arteries in the orofacial region or from the embryonic remnants such as lingual duct or circumvallate papilla. ^[2] Even the congenital form of leiomyoma has been reported. ^[14] The most frequent locations are lip (48.6%), hard and soft palate (21.1%), tongue (9.2%), and cheek (9.2%). ^[15] Angioleiomyoma (60-69%) is the most common type compared to solid leiomyoma (30%) and leiomyoblastoma (1.3%) in the orofacial region. ^[14]

Clinically, they look like a smooth-surfaced submucosal nodule, and the color of the lesion varies depending upon their vascularization and depth. Fifty-five percent of the cases appear red, blue, or purple in color. ^[16] Usually, the oral leiomyomas are asymptomatic; but in some cases, they may cause toothache, loose tooth, difficulty in deglutition, shortness of breath, referred pain in temporomandibular joint (TMJ), etc., depending upon their size and location. ^[4] Pain in angioleiomyomas may be due to the nerve fibers in the stroma or wall of the tumors, or the contraction of blood vessels with local ischemia. ^[17] Our case presented with an extraoral diffuse swelling with mild tenderness and no change in the color over the skin or in the upper buccal vestibule, suggesting the deep-seated nature of the lesion.

Based on the patient's history and clinical examination, we considered fibroma, lipoma, neuroma, peripheral giant cell granuloma, rhabdomyoma, leiomyoma, dermoid cyst, and minor salivary gland tumor as the possible diagnoses. Benign mesenchymal tumors such as fibroma, myofibroma, neurofibroma, schwannoma, lipoma, and benign lesions of salivary gland origin such as a deep-seated mucocele and pleomorphic adenoma should also be considered in the differential diagnosis of intraoral leiomyoma. Similarly, vascular lesions such as hemangioma, pyogenic granuloma, lymphangioma, and malignant lesions such as leiomyosarcoma are some of the oral tumors which can present as leiomyoma. Therefore, these should be excluded before making the final diagnosis. ^[18]

No radiographic changes were seen in the orthopantomograph and paranasal sinus view of the skull. Fine needle aspiration cytology showed abundant cohesive clusters of spindle-shaped cells, some of them having cigar-shaped nuclei with blunted ends. Considering the radiographic and cytologic findings, the lesion was excised under general anesthesia. Macroscopically, the lesion was reddish pink and round, measuring about 1.5 × 1.5 cm in dimension. The maximum size reported in literature is 3 cm. ^[3] Histopathological examination revealed well-encapsulated mass with hypercellularity. The cells were spindle shaped and arranged in long fascicles. The cells had eosinophilic cytoplasm with blunt and cigar-shaped nuclei with minimal mitotic figures. Increase in the mitotic activity in the tumor should be dealt with seriously, as it may be the malignant form of the tumor, leiomyosarcoma. Masson trichrome stain proves specifically useful in these cases, as it stains the muscle fibers and collagen differently, thereby helping in identification of muscle fibers. ^[7],[19]

A differential diagnosis of fibrous histiocytoma, neurilemmoma, or leiomyosarcoma should also be considered, which can be ruled out by histopathology and special stains. ^[20] Masson trichrome and Van Gieson's stains are specific for muscle cells and collagen fibers, whereas Mallory's phosphotungstic acid (PTAH) and positive immunohistochemical marker desmin may aid to differentiate leiomyoma from other spindle cell tumors such as myofibroblastoma. ^[21] Lack of histiocytes and multinucleated giant cells differentiates it from fibrous histiocytoma, while cytokeratin negativity differentiates it from a synovial tumor. ^[18] S-100 negativity differentiates leiomyoma from neurilemmoma.

Leiomyoma is managed by surgical excision with safe margins. Recurrences have been reported rarely and are attributed to the incomplete excision of the tumor. ^[7]

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