Non-alpine primary thyroid angiosarcoma

SUMMARY Thyroid angiosarcoma is an extremely rare malignancy, which occurs more frequently in the alpine region, likely associated with iodine deficiency and endemic goiter. This is an aggressive neoplasm that usually harbors a poor prognosis. We report the case of a 49-year-old Portuguese female pa...

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Main Authors: Nádia Mourinho Bala, Pedro Simões, José Maria Aragüés, Ricardo Veiga, Sílvia Guerra, Cristina Valadas
Format: Article
Language:English
Published: Brazilian Society of Endocrinology and Metabolism 2022-06-01
Series:Archives of Endocrinology and Metabolism
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S2359-39972022005006201&tlng=en
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author Nádia Mourinho Bala
Pedro Simões
José Maria Aragüés
Ricardo Veiga
Sílvia Guerra
Cristina Valadas
author_facet Nádia Mourinho Bala
Pedro Simões
José Maria Aragüés
Ricardo Veiga
Sílvia Guerra
Cristina Valadas
author_sort Nádia Mourinho Bala
collection DOAJ
description SUMMARY Thyroid angiosarcoma is an extremely rare malignancy, which occurs more frequently in the alpine region, likely associated with iodine deficiency and endemic goiter. This is an aggressive neoplasm that usually harbors a poor prognosis. We report the case of a 49-year-old Portuguese female patient presenting with a large nodule in the anterior neck region, with rapid growth and associated dysphonia. The neck ultrasound showed a hypoechogenic and heterogeneous thyroid nodule, with a larger axis of 44 mm. The fine needle aspiration cytology was not conclusive, and a biopsy of the lesion was performed. The result was suggestive of a mesenchymal tumor constituted by spindle cells and vascular clefts, showing positivity for endothelial markers and negativity for thyroglobulin, calcitonin and TTF1. The chest CT scan performed before surgery showed multiple pulmonary nodules suggestive of secondary lesions. The patient was submitted to total thyroidectomy and lymph node dissection in order to relieve compressive symptoms. A diagnosis of thyroid angiosarcoma was made after histologic examination of the surgical specimen. Despite undergoing multiple lines of palliative chemotherapy, the pulmonary lesions increased in size and number. The patient died due to respiratory failure 29 months after the diagnosis. Thyroid angiosarcoma is a rare malignancy, generally with poor prognosis. In our case, the patient presented with pulmonary metastases at diagnosis, which is a negative prognostic factor. Due to its rarity, data regarding management and treatment of this disease are scarce.
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spelling doaj.art-46e4074711954e0c962c3f4725293f212022-12-22T03:27:22ZengBrazilian Society of Endocrinology and MetabolismArchives of Endocrinology and Metabolism2359-42922022-06-0110.20945/2359-3997000000460Non-alpine primary thyroid angiosarcomaNádia Mourinho Balahttps://orcid.org/0000-0002-5834-4848Pedro Simõeshttps://orcid.org/0000-0002-4734-7621José Maria Aragüéshttps://orcid.org/0000-0003-0711-277XRicardo Veigahttps://orcid.org/0000-0001-6188-4709Sílvia Guerrahttps://orcid.org/0000-0002-1969-2487Cristina Valadashttps://orcid.org/0000-0002-2368-4447SUMMARY Thyroid angiosarcoma is an extremely rare malignancy, which occurs more frequently in the alpine region, likely associated with iodine deficiency and endemic goiter. This is an aggressive neoplasm that usually harbors a poor prognosis. We report the case of a 49-year-old Portuguese female patient presenting with a large nodule in the anterior neck region, with rapid growth and associated dysphonia. The neck ultrasound showed a hypoechogenic and heterogeneous thyroid nodule, with a larger axis of 44 mm. The fine needle aspiration cytology was not conclusive, and a biopsy of the lesion was performed. The result was suggestive of a mesenchymal tumor constituted by spindle cells and vascular clefts, showing positivity for endothelial markers and negativity for thyroglobulin, calcitonin and TTF1. The chest CT scan performed before surgery showed multiple pulmonary nodules suggestive of secondary lesions. The patient was submitted to total thyroidectomy and lymph node dissection in order to relieve compressive symptoms. A diagnosis of thyroid angiosarcoma was made after histologic examination of the surgical specimen. Despite undergoing multiple lines of palliative chemotherapy, the pulmonary lesions increased in size and number. The patient died due to respiratory failure 29 months after the diagnosis. Thyroid angiosarcoma is a rare malignancy, generally with poor prognosis. In our case, the patient presented with pulmonary metastases at diagnosis, which is a negative prognostic factor. Due to its rarity, data regarding management and treatment of this disease are scarce.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S2359-39972022005006201&tlng=en
spellingShingle Nádia Mourinho Bala
Pedro Simões
José Maria Aragüés
Ricardo Veiga
Sílvia Guerra
Cristina Valadas
Non-alpine primary thyroid angiosarcoma
Archives of Endocrinology and Metabolism
title Non-alpine primary thyroid angiosarcoma
title_full Non-alpine primary thyroid angiosarcoma
title_fullStr Non-alpine primary thyroid angiosarcoma
title_full_unstemmed Non-alpine primary thyroid angiosarcoma
title_short Non-alpine primary thyroid angiosarcoma
title_sort non alpine primary thyroid angiosarcoma
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S2359-39972022005006201&tlng=en
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