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Licensed Unlicensed Requires Authentication Published by De Gruyter October 4, 2022

Severe consumptive hypothyroidism in hepatic hemangioendothelioma

A rare complication of a common clinical condition

  • Antonia Walther EMAIL logo , Beate Häberle , Julia Küppers , Eberhard Lurz , Irene Schmid , Heinrich Schmidt and Ilja Dubinski ORCID logo

Abstract

Objectives

Consumptive hypothyroidism may occur in hepatic hemangioendothelioma. The altered expression of deiodinases inactivates peripheral thyroid hormones. As a result, serum levels of free triiodothyronine and free thyroxine are reduced to varying degrees. There are no established recommendations for the dosage of sirolimus for this particular indication. We describe for the first time the course of treatment with low-dose sirolimus.

Case presentation

We present a 5-week-old infant with hepatic hemangioendothelioma and severe consumptive hypothyroidism. Due to hepatic infiltration he showed signs of right heart strain. Therapy of hemangioendothelioma was initiated with propranolol and, in the absence of response, methylprednisolone was added. Treatment was continued with low-dose sirolimus (due to side effects) and propranolol. Hypothyroidism was managed with levothyroxine and liothyronine.

Conclusions

Consumptive hypothyroidism due to cutaneous hemangioma and hepatic hemangioendothelioma can be managed with propranolol and low-dose sirolimus. Treatment of severe hypothyroidism may require a combinational therapy by substitution of both T3 and T4.


Corresponding author: Antonia Walther, MD, Department of Pediatrics, Dr. von Hauner Children’s Hospital, University Hospital Munich, LMU, Munich, Lindwurmstrasse 4, D-80337, Munich, Bavaria, Germany, Phone: +49 89440052811, E-mail:

Acknowledgments

The authors appreciate the clinical advice by Birgit Kammer und Julia Ley-Zaporozhan (Pediatric Radiology, Department of Clinical Radiology, Ludwig-Maximilians-University Munich).

  1. Research funding: None declared.

  2. Author contributions: All authors have accepted responsibility for the entire content of this manuscript and approved its submission.

  3. Competing interests: Authors state no conflict of interest.

  4. Informed consent: Informed consent was obtained from all individuals included in this study.

  5. Ethical approval: Not applicable.

References

1. Luongo, C, Trivisano, L, Alfano, F, Salvatore, D. Type 3 deiodinase and consumptive hypothyroidism: a common mechanism for a rare disease. Front Endocrinol 2013;4:115. https://doi.org/10.3389/fendo.2013.00115.Search in Google Scholar PubMed PubMed Central

2. Campbell, V, Beckett, R, Abid, N, Hoey, S. Resolution of consumptive hypothyroidism secondary to infantile hepatic hemangiomatosis with a combination of propranolol and levothyroxine. J Clin Res Pediatr Endocrinol 2018;10:294–8. https://doi.org/10.4274/jcrpe.4865.Search in Google Scholar PubMed PubMed Central

3. Aw, DK, Sinha, RA, Tan, HC, Loh, LM, Salvatore, D, Yen, PM. Studies of molecular mechanisms associated with increased deiodinase 3 expression in a case of consumptive hypothyroidism. J Clin Endocrinol Metab 2014;99:3965–71. https://doi.org/10.1210/jc.2013-3408.Search in Google Scholar PubMed

4. Bohinc, BN, Michelotti, G, Xie, G, Pang, H, Suzuki, A, Guy, CD, et al.. Repair-related activation of hedgehog signaling in stromal cells promotes intrahepatic hypothyroidism. Endocrinology 2014;155:4591–601. https://doi.org/10.1210/en.2014-1302.Search in Google Scholar PubMed PubMed Central

5. Pasa, MW, Scheffel, RS, Zanella, AB, Maia, AL, Dora, JM. Consumptive Hypothyroidism: case report of hepatic hemangioendotheliomas successfully treated with vincristine and systematic review of the syndrome. Eur Thyroid J 2017;6:321–7. https://doi.org/10.1159/000481253.Search in Google Scholar PubMed PubMed Central

6. Schmid, I, Klenk, AK, Sparber-Sauer, M, Maxwell, R, Häberle, BB. Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. World J Pediatr 2018;14:322–9. https://doi.org/10.1007/s12519-018-0171-5.Search in Google Scholar PubMed

7. Greenberger, S, Bischoff, J. Infantile hemangioma-mechanism(s) of drug action on a vascular tumor. Cold Spring Harb Perspect Med 2011;1:a006460. https://doi.org/10.1101/cshperspect.a006460.Search in Google Scholar PubMed PubMed Central

8. Warren, D, Diaz, L, Levy, M. Diffuse hepatic hemangiomas successfully treated using sirolimus and high-dose propranolol. Pediatr Dermatol 2017;34:e286–7. https://doi.org/10.1111/pde.13219.Search in Google Scholar PubMed

9. Joshi, K, Bolia, R, Poddar, U, Dabadgao, P. Consumptive hypothyroidism due to diffuse hepatic hemangiomas treated with propranolol therapy. Indian Pediatr 2020;57:366–8. https://doi.org/10.1007/s13312-020-1794-x.Search in Google Scholar

10. Ji, Y, Chen, S, Yang, K, Xia, C, Li, L. Kaposiform hemangioendothelioma: current knowledge and future perspectives. Orphanet J Rare Dis 2020;15:39. https://doi.org/10.1186/s13023-020-1320-1.Search in Google Scholar PubMed PubMed Central

11. Bessho, K, Etani, Y, Ichimori, H, Miyoshi, Y, Namba, N, Yoneda, A, et al.. Increased type 3 iodothyronine deiodinase activity in a regrown hepatic hemangioma with consumptive hypothyroidism. Eur J Pediatr 2010;169:215–21. https://doi.org/10.1007/s00431-009-1009-x.Search in Google Scholar PubMed

Received: 2022-07-09
Accepted: 2022-08-16
Published Online: 2022-10-04
Published in Print: 2022-12-16

© 2022 Walter de Gruyter GmbH, Berlin/Boston

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