Consumptive Hypothyroidism: Case Report of Hepatic Hemangioendotheliomas Successfully Treated with Vincristine and Systematic Review of the Syndrome

• Published in: European thyroid journal 2017-11, Vol.6 (6), p.321-327
• Main Authors: Weber Pasa, Marina, Selbach Scheffel, Rafael, Borsatto Zanella, André, Maia, Ana Luiza, Dora, José Miguel
• Format: Article
• Language: English
• Subjects: Clinical Thyroidology / Original Paper
• ISSN: 2235-0640; 2235-0802
• DOI: 10.1159/000481253

Objectives: To provide a comprehensive description of consumptive hypothyroidism syndrome (CHS), a severe form of hypothyroidism that occurs due to the high expression levels of thyroid hormone inactivation enzyme type 3 deiodinase (D3) in tumors. Study Design: Case report and systematic review. Results: A 7-month-old girl with a diagnosis of massive hepatic hemangioendotheliomas was treated with high doses of thyroid hormones and tumor-directed chemotherapy with vincristine. The tumor displayed excellent response, and euthyroid status was regained. A systematic review on the databases PubMed/Medline and Embase was performed, using the term “Consumptive AND “Hypothyroidism.” From the 33 selected references, we extracted 42 case reports of CHS: 36 children and 6 adults. The laboratory profile at diagnosis displayed high TSH and low T 4 and T 3 serum levels. The serum reverse T 3 and D3 activity levels were high in all patients tested. In children, 97% had vascular tumors, whereas in adults 33% were vascular tumors, 33% fibrous tumors, and 33% gastrointestinal stromal tumors. The conservative treatment was predominant in children, while in adults all cases were treated with surgery. Death occurred in 16% of children and 33% of adults. Conclusions: CHS is a rare form of hypothyroidism that occurs in children and adults, usually linked to hepatic vascular tumors. The condition is associated with high lethality. Prompt diagnosis, followed by high-dose thyroid hormone replacement and tumor-directed therapy are the keys to optimize outcomes.