Leimyosarcoma of the buccal mucosa and review of literature

Deepak Kumar J Nagpal; Prashant R Prabhu; Amisha Shah; Sangeeta Palaskar

doi:10.4103/0973-029X.110732

An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists



CASE REPORT

Year : 2013 \| Volume : 17 \| Issue : 1 \| Page : 149

Leimyosarcoma of the buccal mucosa and review of literature

Deepak Kumar J Nagpal¹, Prashant R Prabhu¹, Amisha Shah², Sangeeta Palaskar¹
¹ Department of Oral and Maxillofacial Pathology, Sinhgad Dental College and Hospital, Maharashtra, India
² Department of Oral and Maxillofacial Pathology, M. A. Rangoonwala College of Dental Sciences and Research Centre, Pune, Maharashtra, India

Date of Web Publication

18-Apr-2013

Correspondence Address:
Deepak Kumar J Nagpal
Department of Oral and Maxillofacial Pathology, Sinhgad Dental College and Hospital, Pune 411 041, Maharashtra
India

Source of Support: None, Conflict of Interest: None

Check

DOI: 10.4103/0973-029X.110732

Abstract

Leiomyosarcoma (LMS) is an uncommon malignant spindle cell tumor of the head and neck region. The occurrence is particularly rare in the buccal mucosa of the oral cavity. It is a rapidly growing tumor with aggressive behavior and poor prognosis. Method: This article presents a rare case of primary leimyosarcoma of the buccal mucosa in a 35 year old female and retrospective analysis of primary oral LMS published in the English literature since past 20 years is done. Diagnosis was confirmed by immunohistochemistry profile showing positivity for vimentin, smooth muscle actin (SMA), high proliferative index displayed by Ki-67, focal positivity for pan-CK and negativity for S-100. Conclusion: Based on the presence of malignant spindle cells showing positivity for vimentin and SMA, a diagnosis of leiomyosarcoma was made.

Keywords: Ki-67, leiomyosarcoma, smooth muscle actin, spindle cell

How to cite this article:
Nagpal DJ, Prabhu PR, Shah A, Palaskar S. Leimyosarcoma of the buccal mucosa and review of literature. J Oral Maxillofac Pathol 2013;17:149

How to cite this URL:
Nagpal DJ, Prabhu PR, Shah A, Palaskar S. Leimyosarcoma of the buccal mucosa and review of literature. J Oral Maxillofac Pathol [serial online] 2013 [cited 2020 Jul 10];17:149. Available from: http://www.jomfp.in/text.asp?2013/17/1/149/110732

Introduction

Tumors of the smooth muscle are rare in the head and neck region especially the buccal mucosa due to the scarcity of this tissue in this region and thus are frequently seen in the gastrointestinal and female genital tract because of the preponderance of smooth muscle at these sites. ^[1],[2] In the head and neck region, these tumors are believed to arise from the tunica media of the blood vessels. Clinically, they are very aggressive, and the prognosis is poor. ^[3] In the oral cavity most of the cases are seen in the mandible, maxilla, tongue, cheek, hard and soft palate, floor of the mouth and lip. ^[4],[5] They are less frequently seen on the buccal mucosa. We report a rare case of leiomyosarcoma of the buccal mucosa.

Case report

A 35-year-old female presented with difficulty in opening the mouth since past 1 month. On clinical examination an ulcerative lesion on the left buccal mucosa adjacent to the maxillary left third molar was observed. The lesion was non-tender and firm in consistency, measuring about 1 × 1 cm in dimension, with inflamed surrounding margins. Lymph nodes were not palpable in the cervical region. An axial computed tomography scan showed an iso-dense mass in the right buccal mucosal region causing the swelling [Figure 1]. Excisional biopsy was done under local anesthesia, and the formalin fixed specimen was processed for histopathological examination. Microscopic examination revealed spindle shaped cells proliferating in various patterns [Figure 2]. The cells showed cellular and nuclear pleomorphism, increased mitotic figures, and prominent nucleoli. Single and multinucleated giant cells containing dark eosinophilic cytoplasm in a myxomatous connective tissue were also observed. Inflammatory cells and hyalinized blood vessels with spindle cells proliferation around them were evident. Immunohistochemical staining showed strong positivity for vimentin [Figure 3], smooth muscle actin (SMA) [Figure 4], and high proliferative index displayed by Ki-67 [Figure 5]. Desmin, S-100, and pancytokeratin were negative. These immunohistochemical findings satisfy the criteria for leiomyosarcoma. Due to loss of follow-up further treatment of the patient could not be completed.

Figure 1: An axial computed tomography scan showing an iso-dense mass in the right buccal mucosal region causing facial swelling

Click here to view

Figure 2: Photomicrograph showing interlacing fascicles and bundles of spindle cells (H and E, ×100)

Click here to view

Figure 3: Photomicrograph showing immunopositivity for vimentin (original magnification ×400)

Click here to view

Figure 4: Photomicrograph showing immunopositivity for smooth muscle actin (original magnification ×400)

Click here to view

Figure 5: Photomicrograph displaying proliferative activity by Ki-67 (x200)

Click here to view

Discussion

The literature review of primary leiomyosarcoma of the oral cavity in past 20 years retrieved is listed in [Table 1]. Cases of nasopharynx, and metastatic tumors from other organs were excluded. The most frequent site was mandible accounting for 20 cases, followed by maxilla 15 cases, 10 cases in tongue and 9 cases in buccal mucosa, 2 each in lip, floor of the mouth, hard palate, soft palate and maxillary sinus. Out of 63 patients, 33 patients were male (56%) and 30 were female (44%) [Figure 6]. Ages ranged from 6 years to 90 years. Prognostically, 28 cases were alive with no disease during the follow-up period which ranged from 6 months to 120 months. Twenty-one patients died of this disease during the follow-up period which ranged from 1 month to 37 months. Six patients were alive with disease. Information was not available for nine patients who were lost during the follow-up and remaining three cases died of other causes.

Figure 6: Graph showing sex distribution of primary oral leiomyosarcomas

Click here to view

Table 1: Data of primary oral LMS from the English literature

Click here to view

Soft tissue sarcomas are relatively rare neoplasms that may arise in any anatomic region. Occurrence in the head and neck accounts for less than 1% of all malignant tumors in this site. ^[5] Only 3-10% of leiomyosarcoma (LMS) cases arise in the head and neck [Figure 7]. Smooth muscles is sparse in the head and neck region and are mainly found in the walls of blood vessels, erector pili musculature of the skin, circumvallate papillae and myoepithelial cells of the salivary glands. LMS may also be derived from the undifferentiated pluripotential cells. ^[15],[19] Clinically most often LMS presents as a nodular painless, well circumscribed mass, adherent firmly to the surrounding tissues which sometimes may be ulcerated as was in our presented case. There is no age and sex predilection. ^[20] Histologically LMS typically displays spindle cells with abundant cytoplasm and centrally placed blunt ended cigar shaped nuclei of varying sizes. Multinucleated giant cells are common. Microscopic criteria for well differentiated LMS include: (i) A pattern of interlacing bundles of smooth muscle cells, (ii) a high mitotic rate, (iii) pleomorphism, and (iv) bizarre cell forms. ^[21] Immunopositivity for vimentin, SMA, and muscle specific actin MSA has been demonstrated in LMS. Some have also shown immunopositivity for desmin, but this feature is not consistent. The tumor should be immunonegative for S-100 and cytokeratins ^[22],[23] These histopathological features along with immunohistochemical profiles aids in differentiating LMS from other similar spindle cell malignancies like malignant fibrous histiocytoma, fibrosarcoma, etc., [Table 2].

Figure 7: Graph showing sites of occurrence of primary oral leiomyosarcomas

Click here to view

Table 2: Differential diagnosis and immunohistochemical profile of similar spindle cell tumors[22,23]

Click here to view

Oral LMS tend to metastasize to the cervical nodes and lungs, and therefore when LMS is identified, it is necessary to determine whether the lesion is primary or secondary. The likelihood of distant metastasis is related to histologic grade and tumor size; the risk is highest for large, high grade lesions. ^[24] Because of its similarity to other sarcomas composed of spindle cells like fibrosarcoma, neurogenic sarcoma and malignant fibrous histiocytoma, use of special stains and immunohistochemistry becomes of utmost importance for early diagnosis and prompt management of the cases. ^[14] Early wide surgical excision with radical neck dissection for lymph node metastasis remains the mainstay of treatment. ^[25] Palliative radiotherapy and chemotherapy have been tried with variable results. Overall the prognosis of LMS is poor and hence early diagnosis is the key to the management. ^[26]

Conclusion

Oral LMS are rare lesions of the head and neck with aggressive behavior and poor prognosis in most of the cases. Appropriate clinical and histopathological evaluation supplemented with immunohistochemistry is a must. Early and accurate diagnosis followed by radical treatment is of utmost importance for improving the prognosis of these tumors.

References

1.	Yadav R, Bharathan S. Leiomyosarcoma of the buccal mucosa: A case report with immunohistochemistry findings. J Oral Sci 2008;50:215-8.
2.	Mesquita RA, Migliari DA, de Sousa SO, Alves MR. Leiomyosarcoma of the buccal mucosa: A case report. J Oral Maxillofac Surg 1998;56:504-7.
3.	Gustafson P, Willén H, Baldetorp B, Fernö M, Akerman M, Rydholm A. Soft tissue leiomyosarcoma. A population-based epidemiologic and prognostic study of 48 patients, including cellular DNA content. Cancer 1992;70:114-9.
4.	Bishwajit M, Fatema S, Sanjay J, Nidhi S, Sonal S, Ankur B. Leiomyosarcoma of mandible: A case report and review of literature. Int. Journal of Contemporary Dentistry 2010;1:58-63.
5.	Schenberg ME, Slootweg PJ, Koole R. Leiomyosarcomas of the oral cavity. Report of four cases and review of the literature. J Craniomaxillofac Surg 1993;21:342-7.
6.	Vilos GA, Rapidis AD, Lagogiannis GD, Apostolidis C. Leiomyosarcomas of the oral tissues: Clinicopathologic analysis of 50 cases. J Oral Maxillofac Surg 2005;63:1461-77.
7.	Izumi 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.
8.	Krishnan V, Miyaji CM, Mainous EG. Leiomyosarcoma of the mandible: A case report. J Oral Maxillofac Surg 1991;49:652-5.
9.	Sumida T, Hamakawa H, Otsuka K, Tanioka H. Leiomyosarcoma of the maxillary sinus with cervical lymph node metastasis. J Oral Maxillofac Surg 2001;59:568-71.
10.	Yang SW, Chen TM, Tsai CY, Lin CY. A peculiar site of leiomyosarcoma: The tongue tip - Report of a case. Int J Oral Maxillofac Surg 2006;35:469-71.
11.	Mitsudo K, Tohnai I, Fujimoto Y, Sawaki Y, Sugimura T, Nishiguchi H, et al. Leiomyosarcoma of the maxilla: effective chemotherapy with docetaxel (DOC) and cisplatin (CDDP) using superselective intra-arterial infusion via superficial temporal artery. Oral Oncol Extra 2006;42:258-62.
12.	Centeno CR, Nadini F, Adam R, Godoy H, Reichart PA. Primary leiomyosarcoma of the mandible. Oral Oncology Extra 2006;42:40-5.
13.	Carter LC, Aguirre A, Boyd B, DeLacure MD. Primary leiomyosarcoma of the mandible in a 7-year-old girl: Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87:477-84.
14.	Lo Muzio L, Favia G, Mignogna MD, Piattelli A, Maiorano E. Primary intraoral leiomyosarcoma of the tongue: An immunohistochemical study and review of the literature. Oral Oncol 2000;36:519-24.
15.	Amarapala H, Tilakratne WM. Leimyosarcoma of oral cavity: report of seven cases and review of literature. Oral Oncology Extra 2006;42:14-7.
16.	Ethunandan M, Stokes C, Higgins B, Spedding A, Way C, Brennan P. Primary oral leiomyosarcoma: A clinico-pathologic study and analysis of prognostic factors. Int J Oral Maxillofac Surg 2007;36:409-16.
17.	Pinheiro Jde J, Alves Sde M Jr, Okuda E, Jorge WA, Jaeger RG, de Araújo NS. Primary leiomyosarcoma of the mandible. A case report. Med Oral Patol Oral Cir Bucal 2007;12:E56-59.
18.	Yan B, Li Y, Pan J, Xia H, Li LJ. Primary oral leiomyosarcoma: A retrospective clinical analysis of 20 cases. Oral Dis 2010;16:198-203.
19.	Piattelli A, Artese L. Leiomyosarcoma of the tongue: A case report. J Oral Maxillofac Surg 1995;53:698-701.
20.	Fasanmade AW, Newman L. Primary leimyosarcoma of oral cavity: a case report and review of literature. Can J Plast Surg 2002;10:113-6.
21.	Martis C. Leiomyosarcoma of the maxilla: Report of two cases. J Oral Surg 1978;36:62-5.
22.	Neville BW, Damm DD, Allen CM, Bouquot JE. Soft Tissue Tumors. Oral and Maxillofacial Pathology. 5 ^th ed. New Delhi: Saunders; 2004. p. 486.
23.	Enzinger FM, Weiss SW. Leiomyosarcoma. Soft Tissue Tumors. 2 ^nd ed. New Delhi: B.I. Publications; 1988. p. 402-21.
24.	Kang BC, Jang DD, Lee SK. Oral leiomyosarcoma in a woodchuck (Marmota monax). J Vet Med Sci 2005;67:353-5.
25.	Pellitteri PK, Ferlito A, Bradley PJ, Shaha AR, Rinaldo A. Management of sarcomas of the head and neck in adults. Oral Oncol 2003;39:2-12.
26.	Willers H, Hug EB, Spiro IJ, Efird JT, Rosenberg AE, Wang CC. Adult soft tissue sarcomas of the head and neck treated by radiation and surgery or radiation alone: Patterns of failure and prognostic factors. Int J Radiat Oncol Biol Phys 1995;33:585-93.