Primary Leiomyosarcoma of the Thyroid Gland

Section of Endocrine Surgery, Department of Surgery, Yale University School of Medicine, New Haven, Connecticut 06520-8062, USA.
Thyroid (Impact Factor: 4.49). 05/2008; 18(4):425-8. DOI: 10.1089/thy.2007.0276
Source: PubMed


Primary leiomyosarcomas of the thyroid gland are rare. We present the case of a 65-year-old woman with a rapidly enlarging neck mass for 2 months. The preoperative differential diagnosis included medullary thyroid cancer, anaplastic thyroid cancer, and primary versus metastatic sarcoma. The patient underwent total thyroidectomy, bilateral central neck dissections, and cervical thymectomy; she is currently being treated with ifosfamide and adriamycin. We review the literature on leiomyosarcoma of the thyroid, including the differential diagnoses, pathology, and alternative treatment strategies, including surgery and adjuvant therapy.

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    • "Rapid locoregional infiltration and diffuse brain or lung metastases are responsible for the high mortality rate. Total or near-total thyroidectomy, for the majority of thyroid pathologies, associated with therapeutic modified radical neck dissection should be considered for intrathyroidal disease (17–21). Chemotherapy has not shown any therapeutic efficacy. "
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    ABSTRACT: Primary smooth muscle tumors of the thyroid gland are extremely rare neoplasms. Due to their rarity, clinical case studies concerning management are lacking. According to a literature review, only 19 cases of primary thyroid leiomyosarcomas (TLs) have been reported. In the majority of patients, the prognosis is poor since adjuvant radiochemotherapy is ineffective on local recurrence and on longterm survival. In this study, we report the case of a 77yearold male affected by a rapidly enlarging mass of the anterior neck, associated with bilateral lung metastases, and increasing dysphagia and dyspnea during the previous 6 months. A Tir4 neoplasm fine needle cytological diagnosis of the right thyroid lobe was reached and the patient underwent total thyroidectomy (TT). Definitive histological examination identified a TL. The patient succumbed 40 days later due to respiratory distress. A literature review was performed and TL differential diagnoses, management, including alternative treatment strategies, and adjuvant therapy were analyzed. TL is an aggressive rare mesenchymal malignant tumor. Although an improved multimodal approach is often necessary, TT and neck dissection represent the treatment of choice and are often the only possible therapy. Adjuvant radiochemotherapy appears to be ineffective and a high mortality rate is observed. TL remains a fatal tumor, and innovative and more effective therapeutic strategies to improve management and outcomes are required.
    Full-text · Dataset · Nov 2013
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    ABSTRACT: Malignant mesenchymoma of the thyroid is extremely rare. We report such a tumor involving the bilateral lobes of the thyroid which showed simultaneous chondrosarcomatous, osteosarcomatous, fibrosarcomatous and rhabdomyosarcomatous differentiation. The patient was a 52-year-old woman admitted with a history of facial swelling, neck thickness and swallowing discomfort of one month's duration. Sonographic examination indicated a thyroid mass involving the bilateral lobes. Macroscopically, the tumors of both lobes were well demarcated, solid, greyish-white, and multinodular on the cut surface. Some nodules were translucent in appearance and hard in texture. Microscopically, the tumor was composed of small primitive mesenchymal cells with osteoid formation resembling the small cell variant of osteosarcoma interspersed with multiple cartilaginous nodules that indicated chondrosarcomatous differentiation. Some tumor cells showed prominent rhabdomyoblastic differentiation with eosinophilic cytoplasm and eccentric nuclei. Fibrosarcomatous areas were also observed. Immunohistochemically, the small primitive mesenchymal cells were positive for vimentin and CD99 and negative for CD56, Syn, CgA, CK, TG, TTF-1, calcitonin, and S-100. The tumor cells in the rhabdomyosarcomatous area were MyoD1 and muscle-specific actin positive. Molecular analysis for BRAFand RAS gene alterations showed no point mutation. The tumor recurred four months after surgery and tumor thrombi were suspected in the bilateral internal carotid arteries on ultrasonography. Primary malignant mesenchymoma of the thyroid is a high-grade malignant tumor with a poor prognosis. Its differerential diagnosis includes anaplastic carcinoma and other rare sarcomas with chondroid, osteoid, and other mesenchymal metaplasia.
    Preview · Article · Mar 2010 · Tumori
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    Full-text · Article · Mar 2010 · Arquivos Brasileiros de Endocrinologia & Metabologia
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