Leiomyosarcoma of tongue: A case report and review of literature

Introduction

Leiomyosarcoma (LMS) is malignant neoplasm originating from smooth muscle account for 3–7% of soft tissue sarcomas which occurs frequently in uterine myometrium, gastrointestinal tract, retroperitoneum, skin, and subcutaneous tissue,¹ but rare in the oral cavity because of paucity of smooth muscle in that site, but when present they are usually localized on the tongue, lips, and palate.² It may arise as primary, radiation-associated, or metastatic tumor.³ Primary LMS of the tongue is an exceedingly rare. Here, we describe a case of LMS of the tongue in a 50-year-old female.

Case Report

We present the case of a 50-year-old female with an LMS of the tongue. She had reported to Oncosurgery Department with the complaint of ulcerative growth on the tongue along with difficulty in closing mouth and tongue movement for the last 4 months. She was tobacco-chewer for last 20 years with no other addiction history. She denies any exposure to chemicals, drugs, and sharp tooth. Her family history was also not significant. Her vital parameters were within normal range that she was conscious, alert, with blood pressure of 120/70 mm Hg, respiratory rate of 20/min, pulse rate of 110/min, and oxygen saturation of 92% on room air and her systemic evaluation was unremarkable. On local examination, she had a large ulcerative growth (size ∼ 5 cm × 5 cm) arising from the right anterolateral aspect of anterior tongue. Computed tomography scan imaging showed heterogeneously enhancing mass of size 4.5 cm × 3.5 cm × 5.4 cm on right anterolateral aspect of anterior tongue along with loss of fat interface between the mass and underlying intrinsic muscles of tongue Figure 1. Biopsy of mass lesion revealed a malignant epithelial neoplasm and immunohistochemistry (positive for alpha smooth muscle actin and desmin, and negative for cytokeratin and S-100) are suggestive of LMS. Then, she was hospitalized and underwent wide excision right side glossectomy with Type II modified radical neck dissection. The definitive histopathological diagnosis performed on surgical specimen revealed LMS of tongue with absence of neoplastic infiltration of surgical resection margin and all 14 lymph nodes; results of immunohistochemical study were positive for alpha-smooth muscle actin and desmin, negative for cytokeratin and S-100 protein further re-enforces the same diagnosis.

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Figure 1

Compute tomography scan showing heterogeneously enhancing mass of size 4.5 cm × 3.5 cm × 5.4 cm on right anterolateral aspect of anterior tongue along with loss of fat interface between mass and underlying intrinsic muscles of tongue

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In view of tumor depth of 3 cm and tumor size more than 5 cm, patient was for postoperative radiotherapy with a dose of 60 Gy/30 fractions/6 weeks with X-6MV on a linear accelerator without concomitant chemotherapy.

Discussion

LMS is an uncommon malignant mesenchymal neoplasm originating from smooth muscle. It occurs frequently in the gastrointestinal tract and female genital tract.¹ Due to paucity of smooth muscle in head and neck region, only 3–7% of LMS cases occur in head and neck region.¹ However, when present in head and neck region, they are usually localized on the tongue, lips, and palate.² The cause of LMS is still uncertain, although association with trauma, estrogen therapy, ionizing radiation, and Epstein–Barr virus has been documented in the literature.⁴

Clinically, LMS often presents as a rapidly growing, painless, discrete mass firmly adherent to surrounding soft tissue.⁴ LMS become large by the time diagnosis is confirmed because of very few associated symptoms.⁴ Due to nonspecific clinical presentation, diagnosis of LMS is based primarily on pathologic criteria. Immunohistochemistry or electron microscopy must be carried out to achieve more specific differential diagnosis.^5,6 The histological criteria include the presence of pleomorphism, bizarre cell forms, pattern of interlacing bundles of smooth muscle cells, and high mitotic rate.⁷ Immunohistochemical study of LMS was consistently positive for alpha smooth muscle actin, vimentin, desmin, and negative for S-100 protein and cytokeratins.^8,9

The only effective treatment is complete resection with sufficient tumor-free borders and postoperative radiotherapy when necessary.¹⁰ Radical neck dissection is needed in cases with lymphadenopathy. However, there is insufficient evidence to support the efficacy of radiotherapy and chemotherapy. Till now, only one case was reported who was treated with radiotherapy alone because of surgically inoperable condition.⁹ In this case, no local recurrence or distant metastasis was reported after 1.5 years of follow-up. In another two cases, radiotherapy at a dosage of 65 Gy/28 fractions/3 months was used after surgical excision.^9,11 Chemotherapy was recommended in cases with inoperable and metastatic disease.¹² A combination of ifosfamide and doxorubicin was used in one case.⁷ However, further research is needed to document the efficacy of adjuvant therapy in LMS of tongue. Prognosis of tongue LMS is good if clear surgical margin is achieved after excision.

Till now, we only know of 34 cases reported (including our patient’s case) of primary LMS of the tongue with four cases from India^11,13,14 including one of the authors Table 1. Review of 34 cases revealed that there were 18 males and 16 females with an age range from 1 year to 97 years with no predilection for any specific age group. The sites of tumor lesions in the tongue include the tip, the lateral border, and the base. In 93% (24/26) patients, excision is the main treatment.

This case report highlights that although LMS of the tongue is a rare mesenchymal tumor, we should be familiar with this unusual lesions because early diagnosis and aggressive management are the mainstay of therapy.