Severe consumptive hypothyroidism in hepatic hemangioendothelioma

J Pediatr Endocrinol Metab. 2022 Oct 4;35(12):1560-1564. doi: 10.1515/jpem-2022-0347. Print 2022 Dec 16.

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.

Keywords: consumptive hypothyroidism; hemangioendothelioma; sirolimus.

Publication types

  • Case Reports

MeSH terms

  • Hemangioendothelioma* / complications
  • Hemangioendothelioma* / drug therapy
  • Humans
  • Hypothyroidism* / complications
  • Hypothyroidism* / drug therapy
  • Infant
  • Liver Neoplasms* / complications
  • Male
  • Propranolol / therapeutic use
  • Sirolimus / therapeutic use
  • Thyroid Hormones / therapeutic use
  • Thyroxine
  • Triiodothyronine

Substances

  • Propranolol
  • Thyroxine
  • Triiodothyronine
  • Thyroid Hormones
  • Sirolimus