|Ahead of print publication
Central angioleiomyoma of the mandible: A rare entity
Rizwan Hamid1, Altaf Chalkoo1, Saima Tariq1, Sheikh Bilal2, Suhail Wani3
1 Department of Oral Medicine and Radiology, Government Dental College and Hospital, Srinagar, Jammu and Kashmir, India
2 Department of Pathology, GMC, Srinagar, Jammu and Kashmir, India
3 Department of Pathology, SKIMS, Srinagar, Jammu and Kashmir, India
Department of Oral Medicine and Radiology, Government Dental College and Hospital Srinagar, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
The leiomyoma is a benign smooth-muscle neoplasm commonly found in the female genital tract, gastrointestinal tract, or skin. Leiomyomas of the oral cavity are unusual. Oral leiomyomas are uncommon due to the paucity of the smooth muscle in the mouth (except in blood vessels) and thus the involvement of jaw bones is extremely rare. Leiomyomas have been classified as solid angiomyoma, angioleiomyoma (vascular leiomyoma), and epithelioid variants. Angioleiomyomas are benign mesenchymal tumors derived from smooth muscle, which rarely occur in the oral cavity. Malignant transformation probably does not occur but careful histopathologic examination is still necessary to differentiate these benign lesions from their malignant counterparts due to different prognosis. Although uncommon in the maxilla and mandible, they should be included in the differential diagnosis of radiolucent lesions of jaw bones. An extensive search of literature was carried out on the Medline-PubMed and Google Scholar database using the keywords such as leiomyoma, angioleiomyoma, jaw bones, maxilla, mandible, intra-osseous to thoroughly search and collect all the reported cases of intraosseous leiomyoma (but our search was not limited to these terms only). To the best of our knowledge, only 23 cases of intraosseous leiomyomas have been reported so far in the jaw bones, among which only 8 belonged to angioleiomyomas. Herein, we report the 9th case of intraosseous angioleiomyoma, one of the variants of leiomyoma and overall 24th intraosseous leiomyoma in a 6-year-old female child, together with conventional histopathologic and immunohistochemical findings.
Keywords: Angioleiomyoma, benign, intraoral, leiomyoma, smooth muscle, solid variant
| > Introduction|| |
Leiomyoma, a benign tumor of smooth-muscle origin, is most commonly located in the female genital tract (95% of cases) followed by the gastrointestinal tract and subcutaneous tissues. It rarely occurs in the oral cavity. In an extensive study of 7748 smooth-muscle tumors, Farman reported only five cases located to the oral cavity. The first oral presentation was reported by Blanc in 1884. According to the World Health Organization, leiomyomas are divided into two categories: angioleiomyoma and leiomyoma of deep soft tissue. Leiomyomas are benign smooth-muscle neoplasms that are classified under three histologic subtypes: solid leiomyoma, angioleiomyoma (vascular leiomyoma), and epithelioid leiomyoma (bizarre leiomyoma or leiomyoblastoma). Angioleiomyomas are solitary forms of leiomyomas that usually occur in the subcutis and are the most common variant of leiomyomas affecting the oral cavity. From a clinical point of view, angioleiomyomas are usually superficial, well-circumscribed, and asymptomatic small lesions with slow growth, covered by normal mucosa, and sometimes bluish or purple in color., The most common oral sites for angioleiomyomas are the lips (27.46%), followed by the tongue (18.30%), cheeks and palate (15.49%), gingiva (8.45%), and mandible (5.63%). Intraosseous leiomyomas of the jaws, thought to originate from vascular smooth muscles or pericytes, are extremely rare. The greatest incidence is in the 40–59 year age group., We report a rare intraosseous angioleiomyoma in a 6-year-old female child, together with conventional histopathologic and immunohistochemical findings.
| > Case Report|| |
A 6-year-old female patient was referred to the Department of Oral Medicine and Radiology with the chief complaint of swelling in the lower left posterior region of jaw. As per her records, 3 months back, the patient developed pain and swelling on the lower left posterior region of jaw. It was associated with mobility of lower left second deciduous molar, for which a pediatric dentist was consulted. Her pediatric dentist took intraoral periapical radiographs in the same region and found radiolucency associated with lower left second deciduous molar. It was decided to extract the tooth under local anesthesia, which was done 2 months back. Shortly after extraction, her mother's impression was that the lesion grew fairly rapidly to its present size [Figure 1]a and [Figure 1]b. Her medical history was noncontributory and her family had no significant medical problems. Extraoral examination revealed facial asymmetry on the left side [Figure 2]. The regional lymph nodes were normal. Intraoral examination revealed a rubbery mass of 2.5 cm × 3.5 cm in dimensions in left mandibular premolar area on the alveolar crest, with a small ulcer on its surface [Figure 1]a and [Figure 1]b. The growth appears to emanate from the extraction socket [Figure 1]a and [Figure 1]b. The lesion had the same color as that of the surrounding normal tissue, with a lobulated surface, fibrous consistency, firm on palpation, and nontender. On palpation, expansion and perforation of cortices was present. An intraoral periapical radiograph [Figure 3] revealed a radiolucent area between the lower left first deciduous molar and the first permanent molar, suggestive of osseous erosion consistent with cupping of the underlying bone. Radiographic examination on the orthopantomographic film [Figure 4] disclosed an “ill-defined” radiolucency in relation to lower left deciduous molars (between the 74 and the 36), with missing 75 and root resorption of the distal root of 74. The clinical differential diagnosis included a “central giant-cell granuloma (CGCG)” and a benign mesenchymal neoplasm. Ultrasonography with intraoral probe of the lesion showed a hypoechoic lesion with high vascularity on color Doppler [Figure 5]. An incisional biopsy was performed under local anesthesia, and a tissue specimen, approximately 0.4 cm × 0.3 cm × 0.2 cm in dimension was retrieved and sent for histopathologic examination.
|Figure 1: (a and b) Rubbery mass with a lobulated surface, fibrous in consistency, nontender, and firm on palpation|
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|Figure 3: An intraoral periapical radiograph reveals a radiolucent area between the lower left first deciduous molar and the permanent first molar|
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|Figure 4: An ill-defined radiolucency in relation to lower left deciduous molars (between the 74 and the 36) with missing 75 and root resorption of the distal root of 74|
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|Figure 5: Ultrasonography of the lesion shows a hypoechoic lesion with high vascularity on color Doppler|
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Microscopic examination showed the lesion was composed of large vascular channels of varying caliber, surrounded by thick walls of irregularly arranged, spindle-shaped cells, and the tumor cells were large, with indistinct cell margins, scant, and faintly eosinophilic cytoplasm as well as elongated nuclei with rounded edges [Figure 6]. Histopathologically, a diagnosis of spindle cell neoplasm was made. As all the spindle cell neoplasms show similar histopathologic features, immunohistochemistry (IHC) was suggested. Positive immunostaining for smooth-muscle actin [Figure 7] and desmin were observed in the tumor cells, whereas CD34 was positive in the endothelium [Figure 8]. S-100 staining was negative in the tumor cells [Figure 9]. Based on the IHC report, a confirmatory diagnosis of vascular leiomyoma was established, and total excision of the lesion was planned. The surgery was performed by maxillofacial surgery department under general anesthesia and no complications were observed during the consecutive postoperative sessions in the past few months. The patient has been followed for 6 months with no recurrence.
|Figure 6: Microscopic examination shows the lesion was composed of large vascular channels of varying caliber, surrounded by thick walls of irregularly arranged, spindle-shaped cells (original magnification ×400)|
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|Figure 7: Positive immunostaining for smooth-muscle actin (original magnification ×100)|
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| > Discussion|| |
Leiomyoma is defined by the World Health Organization as a “circumscribed, benign, often cutaneous tumor composed of intersecting bundles of mature smooth muscle cells.” Leiomyoma of the mandible is rare and angioleiomyoma of the mandible is even rarer. An extensive search of computerized database was carried out on the Medline-PubMed and Google Scholar using the MeSH terms: leiomyoma, angioleiomyoma, jaw bones, maxilla, mandible, intra-osseous, but our search was not limited to these terms only. The bibliographies of articles identified by means of Medline-PubMed and Google Scholar were also searched to thoroughly search and collect all the reported cases of intraosseous leiomyoma. To the best of our knowledge, only 23 cases of intraosseous leiomyomas have been published thus far in the jawbones in the world literature and among them only 8 cases were of angioleiomyomas [Table 1]. The rarity of intraosseous leiomyoma could be explained by the paucity of smooth-muscle cells in the bone where the origin is likely from tunica media of blood vessels or some heterotopic embryonic tissue. Whereas in the oral cavity, other sources such as circumvallate papillae and ductus lingualis for the tongue and aberrant arrectores pilorum muscles for the cheek have also been additionally suggested.
|Table 1: Summary of total cases of angioleiomyoma in jaw bones found in the literature|
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It has been suggested that vascular leiomyomas are a stage in a continuous process of smooth -muscle proliferation, and the sequence of progression is presented as hemangioma, angioma, vascular leiomyoma, leiomyoma, and solid leiomyoma. In the present case, clinical presentation, radiographic impression, and surgical findings suggested an intraosseous origin and could be from the smooth muscle of vascular walls or heterotopic embryonic tissues. It can be seen at any age. Age of the 23 patients ranged from 8 months to 75 years, with a mean age of 37.83 years and a median age of 27 which is in accordance with the review of Liang et al. Out of the 23 patients, males were affected more than females in the ratio of 2:1, which is the same for central angioleiomyoma (M: F is 2:1). The male preponderance is concurrent with the literature. Clinical presentation varies from being asymptomatic to painful swelling with or without cortical destruction. Nonspecific pain may be the main manifestation of intraosseous leiomyoma. Most of the lesions of leiomyoma were found in posterior mandible followed by anterior mandible. Only two cases have been reported in the posterior region of upper jaws. All the cases of intraosseous angioleiomyoma so far reported in the world literature,, [16,,,, including the present case were found exclusively in the lower jaws. Radiographically, intraosseous leiomyoma presents as radiolucency, which may be unilocular or multilocular with either ill-defined or well-defined sclerotic borders. Most of the cases were of unilocular variety and the rim of sclerosis suggests a slow growth rate consistent with benign tumors. Depending on the nature and location of the lesion, it may cause cortical expansion and/or destruction. Root resorption and displacement may also be present. Because of its rarity, intraosseous leiomyoma is usually not considered in a radiographic differential diagnosis. More likely diagnostic considerations for this radiographic presentation include central giant cell lesion, ameloblastoma, myxoma, traumatic bone cyst, hemangioma, neurofibroma, and sarcoma. In our case, the initial diagnosis was a CGCG.
The World Health Organization classified leiomyomas into three histological groups: (a) vascular (angioleiomyoma), (b) solid, and (c) epithelioid (leiomyoblastomas). Histologically, the tumor is composed of spindle cells arranged in a whorled and interlaced fascicular pattern. The cells show elongated nuclei with fusiform or blunt ends and perinuclear vacuolization can sometimes be noted. Mature smooth-muscle cells have the distinctive characteristics of small and uniform nucleus and broad eosinophilic cytoplasm., In the present case, the microscopic findings were in accordance with the abovementioned features of angioleiomyoma.
Angiomyomas have tortuous mass of thick-walled blood vessels between intertwining bundles of smooth muscle. The epithelioid leiomyoma has rounded or a polygonal cell with a clear zone around the nucleus and the cytoplasm is acidophilic. Duhig and Ayer stated that vascular leiomyoma is a step in the transformation from hemangioma to solid leiomyoma through a process of continuous proliferation of smooth muscles. Svane in reviewing 114 cases of leiomyoma found that 74% were vascular leiomyoma, 25% were solid, and only 1% were leiomyoblastomas. Baden et al. reviewed 142 cases of oral leiomyoma and found 67% to be vascular, 31.7% to be solid leiomyoma, and only 1.3% to be leiomyoblastoma. In our review, most of the patients reported to have solid variety of leiomyoma and nine patients had angiomyoma, including the present case. It would seem that intraosseous leiomyomas histologically are not predominantly vascular, as are the soft-tissue oral leiomyomas. Microscopic diagnosis may be difficult since the spindle cell proliferation has many similarities with mucocele, lipoma, neurofibroma, schwannoma, fibroma, fibrosarcoma, myofibroblastoma, other myofibroblastic proliferations, and hemangiopericytoma. Masson's trichrome stains smooth-muscle fibers red and collagen fibers green or blue and may be used to differentiate leiomyoma from neurofibroma or neurilemmoma as Damm and Neville recommend Mallory's phosphotungstic acid hematoxylin as the most suitable stain for demonstrating myofibrils. IHC is necessary for arriving at the final diagnosis, as the more common solitary myofibroma of the jaws may have a similar microscopic appearance and is actin-positive, but is also desmin-negative. In addition, the solitary myofibroma occurs almost exclusively in the mandible of patients under 18 years of age. Differentiation of leiomyoma from leiomyosarcoma is not always easy because there are no clear-cut diagnostic criteria for malignancy in a leiomyoma. In previous reports, a tendency for rapid growth of oral leiomyomas which may indicate a malignant predisposition was noticed and doubling time, maximum tumor diameter, mitotic count, Ki-67 labeling index [representing growth fraction], and p-53 reactivity [indicating malignancy] were utilized to establish the exact nature of the lesion.
For oral leiomyomas, immunohistochemical staining with vimentin, desmin, a smooth-muscle actin were positive, whereas glial fibrillary acidic protein, cytokeratin, endothelin, S-100, neuron-specific enolase, and myoglobin CD56 were not present. In our case, immunohistochemical findings revealed positivity for vimentin, smooth-muscle actin, and negativity for S-100 protein. These features helped us to exclude fibroblastic and neural tumors while indicating myogenic differentiation of the tumor. Angioleiomyomas are benign tumors that cause minor problems. Local surgical excision with an adequate margin of unaffected tissue is the suitable therapy., The postoperative prognosis is generally good and this tumor rarely recurs. Only a few cases of recurrence are reported that too in vascular lesion and they are probably related to the incomplete resection of the lesion.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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