Journal of Oral Research and Review

CASE REPORT
Year
: 2018  |  Volume : 10  |  Issue : 1  |  Page : 15--19

Leiomyosarcoma of the maxilla


Zeynep Bayramoglu1, Zeynep Gümrükçü2,  
1 Center for Oral and Dental Health, Erzurum, Turkey
2 Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Karadeniz Technical University, Trabzon, Turkey

Correspondence Address:
Zeynep Gümrükçü
Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Karadeniz Technical University, Trabzon
Turkey

Abstract

Leiomyosarcomas (LMSs) are relatively uncommon mesenchymal neoplasms of smooth muscle differentiation. Only 1% of the lesions occur in the head and neck region. Surgical excision with radiotherapy and chemotherapy is the most commonly executed procedure for recurrent and metastatic tumors. We report a rare LMS case of the maxilla, defined in a 19-year-old male patient. Following the diagnosis of a malignant neoplasm on frozen section, wide resection, with a 1 cm clear margin, was performed. The lesion was then sent for histopathological examination. Spindle cell malignancy was found in histopathological sections of the lesion, histologically consistent with LMS. A radiographical evaluation, magnetic resonance imaging, computerized tomography, and positron emission tomography were performed to detect metastasis. Signs of local recurrence or distant metastasis were not noted at the 12-month follow-up following the definitive surgical procedure.



How to cite this article:
Bayramoglu Z, Gümrükçü Z. Leiomyosarcoma of the maxilla.J Oral Res Rev 2018;10:15-19


How to cite this URL:
Bayramoglu Z, Gümrükçü Z. Leiomyosarcoma of the maxilla. J Oral Res Rev [serial online] 2018 [cited 2019 Jul 18 ];10:15-19
Available from: http://www.jorr.org/text.asp?2018/10/1/15/224541


Full Text

 Introduction



Leiomyosarcoma (LMS) is an uncommon, malign, mesenchymal neoplasm that originates from the smooth muscles, constituting 7% of the soft tissue tumors.[1] It accounts for 1% of the head and neck tumors.[2] LMS is usually seen in the retroperitoneal area, uterine, and gastrointestinal regions owing to the high amount of smooth muscle in these regions.[3] Its location at the level of the oral cavity is considered extremely rare.[1] Clinically, LMS occurs as a painless mass with a well-defined structure. Immunohistochemical confirmation is required for a definitive diagnosis.[1] Complete resection is the recommended treatment option, although not always feasible in the head and neck region.[2] LMS has an aggressive nature and low survival rate. The prognosis for LMS is generally poor owing to its high metastasis and recurrence rate.[1]

We report on a case of maxillary leiomyosarcoma, arising in a 19-year-old male patient and involving the upper alveolar mucosa. The clinical history, radiology, pathology, and 12-month follow-up are presented along with a brief discussion of the literature.

 Case Report



A 19-year-old male patient was referred to the Center for Oral and Dental Health, Erzurum, Turkey with a chief complaint of mobility in the teeth and inadequate chewing function [Figure 1]. In clinical examination, a well-demarcated, firm nodule extending from the left second premolar to the maxillary tuberosity without ulceration, was detected. The lesion was slightly tender to palpation and not fixed to the adjacent tissue. The patient did not report any pain except minimal sensitivity during palpation. Mobility was observed in the molar teeth. No regional lymphadenopathy was detected.{Figure 1}

Radiographically, a small amount of bone resorption, which is not large enough to consider a malignancy in the molar region, was observed [Figure 2]. Because of the radiographical and clinical appearance of the lesion, a preliminary diagnosis of giant cell tumor was considered. Excisional biopsy under local anesthesia was planned. The lesion was excised and the molar teeth were extracted [Figure 3]. A frozen biopsy was performed with the excised material during operation. Malignancy was revealed from frozen section evaluation therefore wide resection of the surgical area with 1 cm safety margins was performed during operation. The area was closed primarily with 3/0 silk suture [Figure 4]. The specimen sent was for histopathological examination. To examine the presence of metastasis the patient was referred for ultrasonography (US), positron emission tomography (PET) scanning, and magnetic resonance imaging (MRI) postoperatively.{Figure 2}{Figure 3}{Figure 4}

Histopathological findings

The spindle cells were distributed in a whirlpool pattern on hematoxylin and eosin (H & E) staining (×10) [Figure 5]. Typical spindle cells with mitotic figures containing cytoplasmic vacuoles and atypical mitotic figures and necrotic foci that prove aggressive behavior of the lesion have been observed together (×40) [Figure 6]. A positive result was shown for the alpha-smooth muscle actin and spindle cells on smooth muscle actin antibody staining that proves the presence of LMS (×100) [Figure 7].{Figure 5}{Figure 6}{Figure 7}

Ultrasonography findings

A node measuring 23 mm × 10 mm was detected in the right adjacent cervical area. Another echogenic ovoid node measuring, 14 mm × 7 mm, was observed in the left adjacent cervical area. The nodes were found to be reactive. No other mass was identified in the surrounding tissue. For further evaluation, MRI was suggested.

Magnetic resonance imaging findings

There was no pathological involvement observed in MRI evaluation results. An ovoid and non-reactive lymph node, measuring 27 mm × 11 mm in size, was detected [Figure 8].{Figure 8}

F-18 fluorodeoxyglucose positron emission tomography findings

F-18 fluorodeoxyglucose (FDG) uptake was detected to be within normal limits in the cerebral, cerebellar, left maxillary molar, and upper left jugular areas, as well as in the liver and lung parenchyma on FDG PET/computerized tomography (CT). A few lymph nodes were reported in the left jugular area, with no FDG uptake. A slight increase in FDG uptake was detected in the skeletal system. A lesion, measuring 29 mm × 14 mm was, observed medial to the proximal tibia. This finding was not pathologic and follow-up was recommended for the left jugular and tibia region.

Based on the PET/CT findings, radiologist recommendations and since metastatic foci were not detected, additional surgery was not performed. No other specific findings of metastasis were observed on US, CT, MRI, and PET/CT evaluation. The postoperative course was uneventful. Any evidence of tumor recurrence was not detected at the 12-month follow-up [Figure 9] and [Figure 10].{Figure 9}{Figure 10}

 Discussion



Leiomyosarcoma is a rare neoplasm in the oral cavity. Nagpal et al.[4] reported different number of cases of LMS in several regions such as mandible (20), maxilla (15), tongue (10), buccal mucosa (9), floor of the mouth (2) hard palate (2), lip (2), soft palate (2), and maxillary sinus (2).

Although the exact etiology of the lesion is uncertain, several cases relating to trauma, chemicals, estrogenic stimulation, ionizing radiation, and the Epstein-Barr virus have also been reported in the literature.[5] However, our case has failed to show an etiologic factor that is considered to trigger the occurrence of the LMS.

LMS is slightly more common in men and may occur at any age.[1] Early age LMS has also been reported in a few English literature.[6],[7] Therefore, our case is a rare and valuable case in terms of the age of our patient.

A definitive diagnosis of LMS depends on the light microscopic and immunohistological findings. The typical view of the LMS contains spindle cells as seen in our case. The differential diagnosis of spindle cells should include rhabdomyosarcoma, spindle cell carcinoma, fibrosarcoma, myofibrosarcoma, malignant schwannoma, fibroblastic osteosarcoma, solitary fibrous tumor, angiosarcoma, malignant fibrous histiocytoma, and melanoma.[8]

Excision, enucleation, curettage, radical surgery, radiation therapy, and chemotherapy have been all suggested as treatment options.[8] Surgical wide-field resection is the most preferred treatment protocol for LMS.[9] In the present case, wide-field resection was preferred as a treatment choice. Based on the results of clear margins obtained from the histopathologic examination and the lack of metastasis following the US, MRI, CT, and PET/CT analyses, we opted not to resect further.

LMS dissemination frequently extends to the lungs. Lymph nodal involvement tends to occur later. LMS has higher metastatic potential than fibrosarcoma and liposarcoma.[3] Dissection of the regional lymph nodes is indicated if there is evidence of nodal involvement, not observed in the present case.[5]

The prognosis of intraoral LMS is poor due to high metastatic and recurrence rates. Distant metastasis (pulmonary or hepatic) is seen at a ratio of 20%. The long-term follow-up of the patient is essential in LMS cases because of the unpredictable course of the tumor.[8],[10]

 Conclusion



An unfavorable LMS prognosis depends on the metastatic nature of the lesion and difficulties with the resection of the maxillary lesions. The overall number of reported cases with LMS in the maxilla region is small. Therefore, reporting future cases and carrying out long-term follow-up is necessary to describe the biological behavior of this tumor.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Suárez-Alén F, Otero-Rey E, Peñamaría-Mallón M, García-García A, Blanco-Carrión A. Oral leiomyosarcoma: The importance of early diagnosis. Gerodontology 2015;32:314-7.
2Kazemian A, Kamian SH, Hoseini MS, Azizi MR. Leiomyosarcoma of the tongue: Report of a case. Iran J Radiat Res 2005;3:143-7.
3Kim SM, Myoung H, Choung PH, Kim MJ, Lee SK, Lee JH, et al. Metastatic leiomyosarcoma in the oral cavity: Case report with protein expression profiles. J Craniomaxillofac Surg 2009;37:454-60.
4Nagpal DK, Prabhu PR, Shah A, Palaskar S. Leimyosarcoma of the buccal mucosa and review of literature. J Oral Maxillofac Pathol 2013;17:149.
5Crossman T, Ward P, Herold J. Leiomyosarcoma of the tongue: A case report. Br J Oral Maxillofac Surg 2008;46:e69-70.
6Yannopoulos K, Stout AP. Smooth muscle tumors in children. Cancer 1962;15:958-71.
7Botting AJ, Soule EH, Brown AL Jr. Smooth muscle tumors in children. Cancer 1965;18:711-20.
8Rodini CO, Pontes FS, Pontes HA, Santos PS, Magalhaes MG, Pinto DS. Oral leiomyosarcomas: Report of two cases with immunohistochemical profile. Oral Med Oral Pathol Oral Radiol Endod 2007;104:50-5.
9Izumi K, Maeda T, Cheng J, Saku T. Primary leiomyosarcoma of the maxilla with regional lymph node metastasis. Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;80:310-9.
10Lo Muzio L, Favia G, Farronato G, Piattelli A, Maiorano E. Primary gingival leiomyosarcoma. A clinicopathological study of 1 case with prolonged survival. J Clin Periodontol 2002;29:182-7.