Introduction: Primary thyroid angiosarcoma (TAS) is a rare malignancy that arises from endothelial cells, and nearly all reported cases originate from the Alpine region of central Europe. Most of the patients are female, elderly, with a history of goiter. Generally, TAS arises as a painless infiltrating mass and patients present with compression symptoms due to the brisk tumor growth. Other TAS clinical presentations are varied and highly non-specific. Metastasis occur early in the disease history, and preferential sites include loco-regional lymph nodes, lungs and bone marrow.
Case report: We present a case of a 49 year old portuguese female patient presenting with a painless, rapidly growing cervical mass and complaints of hoarseness. At the physical examination we observed a large, hard mass, occupying the entirety of the left thyroid lobe. The patient underwent a fine needle aspiration of the lesion, but while suggestive of malignancy, the results were inconclusive. The cervical CT scan revealed an increased volume of the left lobe of the thyroid due to an heterogeneous mass with a long axis of 62 mm. The thoracic CT scan identified several bilateral nodules suggestive of metastatic involvement of the lung. The patient was subjected to a total thyroidectomy and central compartment neck dissection. Histologic examination confirmed the diagnosis of angiosarcoma. Immunohistochemical staining was positive for CD31, CD34 and factor VIII but negative for thyroglobulin, calcitonin and TTF-1. The patient was started on chemotherapy with paclitaxel. While undergoing the second cycle of chemotherapy a new left cervical nodular mass appeared adjacent to the internal jugular vein with no surgical indication. At this point the patient started radiation therapy treatment. Eight months after the initial diagnosis the local recurrence is stable and the pulmonary metastasis reduced in size. Currently the patient is undergoing her 22nd cycle of chemotherapy.
Discussion: As is often the case in TAS, in this case the patient presented with metastatic dissemination at the time of diagnosis. Radical surgery was an early option in order to minimize local growth symptoms. Afterwards chemotherapy associated with radiotherapy helped the patient achieve a certain degree of stability. The prognosis is poor and there is no defined management strategy for TAS. Currently the therapeutic gold-standard is radical surgery. Systemic therapy is indicated for disseminated disease control and palliative care. Otherwise, due to an extremely low incidence, therapeutic options remain very much institution-dependent.
18 - 21 May 2019
European Society of Endocrinology