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Orbit. 2019 Feb;38(1):79-80. doi: 10.1080/01676830.2018.1436569. Epub 2018 Feb 6.

Sirolimus-induced regression of a large orbital lymphangioma.

Author information

1
a Eye Center, Medical Center and Medical Faculty , University of Freiburg , Freiburg , Germany.
2
b Center for Orbital Diseases, Medical Center and Medical Faculty , University of Freiburg , Freiburg , Germany.
3
c Department of Neuroradiology, Medical Center and Medical Faculty , University of Freiburg , Freiburg , Germany.
4
d Department of Pediatric Hematology and Oncology, Medical Center and Medical Faculty , University of Freiburg , Freiburg , Germany.
5
e Center of Vascular Anomalies, Medical Center , University Freiburg , Freiburg , Germany.

Abstract

Microcystic lymphatic malformations are difficult to treat surgically, especially when located in the orbital apex. Recently, pharmacologic inhibition of the mTOR pathway by sirolimus was reported as a safe and efficacious treatment option for lymphatic malformations (also known as lymphangiomas). We report the case of a young male patient in which a unilateral, retrobulbar lymphatic malformation regressed to a large extent under treatment with 1 mg sirolimus given orally twice a day over a period of six months.

KEYWORDS:

Lymphangioma; lymphatic malformation; mTOR; orbit; sirolimus

PMID:
29405800
DOI:
10.1080/01676830.2018.1436569
[Indexed for MEDLINE]

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