Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Leiomyosarcoma of the Head and Neck Soft tissue sarcomas of the head and neck are rare tumors. It is critical that the otolaryngologist be familiar with these unusual lesions because early diagnosis and aggressive initial management remain the mainstay of therapy. The small number of reported smooth muscle tumors in the head and neck reflects the paucity of smooth muscle found in this region. Smooth muscle is derived from primitive mesenchyme and is found mainly in the walls of blood vessels, erector pili musculature of the skin, circumvallate papilla, primitive mesenchyme and myoepithelial cells of salivary glands. Smooth muscle tumors in this region presumable originate from these sources. The benign counterpart of leiomyosarcoma is leiomyoma. Differentiation is based on the presence of mitotic figures. The world literature contains eighty cases of leiomyoma arising in head and neck structures, most commonly the tongue. Primary treatment is local excision. Leiomyosarcoma is the malignant smooth muscle tumor. The incidence of malignancy of head and neck soft tissue tumors (30%) is dramatically higher than that reported for the female GU tract (0.5%). Leiomyosarcomas account for only 4% of head and neck sarcomas. Since 1950, only 38 cases of leiomyosarcoma of the head and neck structures have been reported. Twenty-seven patients were male, and eleven were female. Ages ranged from 2 months to 88 years, the average age being 45 years. A bimodal tendency exists with 40% of cases recorded in patients one to twenty-nine years old and 40% occurring in patients 51 to 67 years of age. Six cases occurred in children. Zieler is credited with the first report of leiomyosarcoma. He and subsequently Hayn discussed the same case of leiomyosarcoma of the cheek. Milian, Diss and Levy reported two cases of leiomyosarcoma arising from old lupus vulgaris scars on the cheek. Geever is credited with the first report in the English literature, a 34-year-old man with leiomyosarcoma of the retromolar region. The sites of occurrence reported for the 38 cases of head and neck leiomyosarcoma are: scalp/soft tissues (9); hypopharynx (3); nose (3); tongue (3); trachea (3); floor of mouth (3); gingiva (2); neck veins (2); and soft palate (2). Single case reports have also identified the parapharyngeal space, lips, trachea, thyroid (primary or metastatic), external auditory canal, maxillary sinus and hard palate as potential sites of occurrence of leiomyosarcoma. Microscopically, the cells are spindle-shaped with blunt-ended nuclei growing in interlacing cords. Nuclear palisading and myofibrils are usually recognizable. Anaplastic features of large, bizarre, pyknotic nuclei and mitotic features are seen in varying degrees. The number of mitotic figures per high power field is an important feature of malignancy. Tumors which exhibit one mitotic figure per five high power fields are considered malignant. Difficult cases require the entire specimen to be evaluated since the malignant pattern may only be present in isolated parts of the tumor. Immunohistochemical identification of desmin, vimentin, actin and myoglobin are helpful in diagnosis. Electron microscopy will demonstrate intracytoplasmic microfilament bundles with numerous cytoplasmic dense bodies, micropinocytotic vesicles and a partly discontinuous basal lamina. Presenting signs and symptoms of leiomyosarcomas of the head and neck structures are generally nonspecific because the primary lesions occur in diverse locations and are usually referable to the location in which they arise. Epistaxis, dysphagia, hoarseness, fever, stridor, and cough have been reported depending upon the site of the lesion. Pain and tenderness are rare in leiomyosarcomas whereas they are more prominent features of leiomyomas. Most commonly encountered is a slowly enlarging, discrete, firm, nonulcerated painless mass. The lesion is submucosal or subcutaneous, reflecting its mesenchymal origin. Grossly, the lesions are smooth, firm and discretely circumscribed. Larger lesions may show focal areas of hemorrhage and necrosis. Based on a large review of leiomyosarcomas of the superficial soft tissues, Stout and Hill concluded that lesions of soft tissue origin 2.5 cm or larger were more likely malignant. Thirty-four of the 38 reported cases had local disease. Only three patients had concomitant palpable neck nodes, suggesting a low incidence of early lymphatic metastasis. One patient had metastatic disease at the time of presentation. The initial treatment in 80% of the 31 patients was wide local excision. Radical neck dissection was performed in the three patients with palpable disease. Surgical intervention combined with radiation therapy, chemotherapy or both was used in three patients. Nineteen patients survived without evidence of disease for four months to 12 years, an average of 48 months. All 19 patients who survived underwent initial wide local excision. Eight patients experienced local recurrence. Late regional lymph node metastasis developed in 10% of cases. Distant metastases developed in seven patients (20%). The usual route of metastatic spread for leiomyosarcoma is theorized to be the bloodstream to the lungs. Five had pulmonary metastases, one patient developed subcutaneous infiltration and another had metastatic disease to the lumbar spine. Eight patients developed local recurrence. One patient received radiation therapy alone, and five underwent further surgical resection. Tumor recurred in three patients, 3, 4, and 6 years after therapy, suggesting that patients deserve long-term follow-up. Recurrent disease was contained by surgical treatment in one-half of the patients where follow-up was available. Patients who were not amenable to complete containment of their disease by surgical resection did not survive. Chemotherapy and radiotherapy alone without surgery did not contain recurrent tumor. Successful treatment of oral leiomyosarcomas may be obtained only with wide local excision with radical neck dissection in the presence of palpable neck disease. A 1 cm margin of resection is considered minimal. Case Presentation A 64-year-old retired engineer was referred to the medical service at the VAMC in September 1992 for evaluation and treatment of a left pancoast tumor. Past history was significant for cigarette smoking and alcohol consumption. A tongue lesion was discovered and the patient was seen in consultation by the Otolaryngology service. Physical examination revealed a 3 cm pale red, circumscribed lesion of the lateral, right side of the oral tongue with a margin of surrounding induration. Incisional biopsy of the tongue lesion revealed invasive, poorly differentiated squamous carcinoma, and fine needle aspiration of the lung mass was consistent with metastatic squamous cell carcinoma. Radiation was elected as the primary treatment modality after consideration of the advanced state of his disease. A six-week course of radiation therapy was delivered to the primary and pulmonic sites, ending in October (30Gy intraoral cone/20Gy external beam). The patient had significant response with apparent tumor regression. However, submucosal induration persisted and a biopsy was interpreted as squamous atypia with radiation change. The decision was made to follow the patient closely radiographically and clinically. Over the ensuing months, several biopsies of the tongue were performed and interpreted as nondiagnostic, squamous atypia, radiation change, pseudosarcomatous proliferation, atypical proliferation and ulceration. On May 7, 1993 an incisional biopsy was performed which was interpreted as leiomyosarcoma. The recommendation of the tumor board was surgical excision. 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