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Case Report
02 (
02
); 082-084
doi:
10.4103/2454-6798.197376

Leiomyosarcoma of tongue: A case report and review of literature

Department of Radiotherapy, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Address for correspondence: Dr. Mranalini Verma, Department of Radiotherapy, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow - 226 014, Uttar Pradesh, India. shilpisinghal2003@gmail.com

Licence
Disclaimer:
This article was originally published by Thieme Medical and Scientific Publishers Private Ltd. and was migrated to Scientific Scholar after the change of Publisher.

Abstract

Leiomyosarcoma (LMS) of the tongue is an extremely rare mesenchymal tumor. Till now, we came across about 24 cases of tongue LMS reported in the literature. Here, we are presenting the case of a 50-year-old female with 4 months history of ulcerative growth on the tongue along with difficulty in swallowing and tongue movement who was diagnosed with LMS of the tongue on histopathology. He was managed with surgical excision followed by radiotherapy without any recurrence or metastasis after 6 months of follow-up.

Keywords

Carcinoma
leiomyosarcoma
tongue

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.

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
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

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 India11,13,14 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.

Table 1 Previously reported cases of primary leiomyosarcoma of tongue

Year

Location

Age (years)/sex

Treatment

188415

Tongue

33/male

-

19051

Tongue

44/male

-

193817

Tongue

50/male

-

193817

Base of tongue

29/female

-

194418

Base of tongue

32/male

-

196219

Tip of tongue

1/male

Excision

196520

Tip of tongue

43/male

-

196921

Dorsum of tongue

11/female

Excision

197022

Tip of tongue

54/male

Excision

198623

Base of tongue

2.5/male

Excision and chemotherapy

199324

Margin of tongue

48/female

Excision

19949

Base of tongue

70/male

Radiation therapy

199425

Tip of tongue

60/male

Excision

19958

Lateral border of tongue

80/female

Patient refused treatment

199613

Base of tongue

22/male

Excision

199816

Lateral border of tongue

57/male

Excision and radiotherapy

200026

Tongue

15/female

Excision and chemotherapy

200027

Lateral border of tongue

67/male

Excision

199929

Margin of tongue

42/male

-

200328

Lateral border of tongue

62/female

Excision

200529

Tip of tongue

67/male

Excision

200530

Lateral border of tongue

32/male

Excision and chemotherapy

200631

Lateral border of tongue

57/female

Excision

200632

Tip of tongue

54/female

Excision

200633

Lateral border of tongue

52/female

Excision

200710

Tongue

79/female

Excision

200734

Tongue

97/female

Excision

200834

Lateral border of tongue

46/female

Excision

201035

Lateral border of tongue

55/male

Excision

201414

Lateral border of tongue

35/male

Excision

20121

Base of tongue

77/male

Excision

20127

Tongue

54/female

Chemotherapy

201411

Base of tongue

38/female

Excision and radiotherapy

2015

Tongue (our case)

50/female

Excision

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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