Cystic angiomatosis, a heterogeneous condition: four new cases and a literature review

Najm, A. , Soltner-Neel, E., Le Goff, B., Guillot, P., Maugars, Y. and Berthelot, J.-M. (2016) Cystic angiomatosis, a heterogeneous condition: four new cases and a literature review. Medicine, 95(43), e5213. (doi: 10.1097/MD.0000000000005213) (PMID:27787381) (PMCID:PMC5089110)

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Abstract

Background: Cystic angiomatosis (CA) is a rare disorder causing bony cysts. It displays some similarity to Gorham–Stout disease (GSD), but has a much better local prognosis, despite the larger number of cysts. These 2 conditions also differ in terms of their location, visceral involvement, and response to treatment. Methods: We report 4 cases of CA, including 1 sclerosing form, which we compare with cases from a literature review performed with PRISMA methodology. Results: We reviewed 38 articles describing 44 other patients. Mean age at diagnosis for the 48 patients (our 4 patients + the 44 from the review) was 22.5 years, and 28 of the patients were men. The femur was involved in 81% (n = 39), the pelvis in 73% (n = 35), the humerus in 52% (n = 25), the skull in 48% (n = 23), and the vertebrae in 44% (n = 21). Visceral lymphangiomatosis (either clinical, or detected on autopsy) was also reported in 35% (n = 18) of the patients. The spleen was the most frequently involved organ (n = 12), followed by the lungs and pleura (n = 8). Liver cysts and/or chylothorax were rarely reported (5 cases), but were invariably fatal. Radiation therapy on bone or soft tissue masses was ineffective, as was interferon alpha, in the 2 patients in which this drug was tested. The efficacy of bisphosphonate was at best equivocal. Conclusion: The progression of CA is unpredictable and treatments effective against GSD, such as bisphosphonates and radiotherapy, have proved ineffective for this condition. New treatments are thus urgently required.

Item Type:Articles
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Najm, Dr Aurelie
Authors: Najm, A., Soltner-Neel, E., Le Goff, B., Guillot, P., Maugars, Y., and Berthelot, J.-M.
College/School:College of Medical Veterinary and Life Sciences > School of Infection & Immunity
Journal Name:Medicine
Publisher:Lippincott, Williams and Wilkins
ISSN:0025-7974
ISSN (Online):1536-5964
Copyright Holders:Copyright © 2016 The Authors
First Published:First published in Medicine 95(43): e5213
Publisher Policy:Reproduced under a Creative Commons License

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