Heterogeneity and Phenotypic Diversity of Multiple Sistemic Lymphangiomatosis
Received Date: Nov 27, 2019 / Accepted Date: Dec 13, 2019 / Published Date: Dec 20, 2019
The lymphatic abnormalities should be considered as entities with local and systemic involvement, of continuous spectrum, being in the majority of cases a diagnosis of exclusion, making the differential diagnosis between entities difficult.
Gorham Stout syndrome should be suspected in a patient with rapid and progressive osteolysis findings with cortical bone loss and presence of bilateral pleural effusion of chylous features. Considering the use of radiological studies and biopsy of the lesion as diagnostic methods. Prioritizing chylothorax treatment to be present.
Generalized lymphatic abnormality or lymphangiomatosis is suspected in similar symptoms, without progressive bone involvement.
Management is complicated, as there are no clinical trials. It is suggested to start with conservative management without the cynical situation of the patient allows it, emphasizing nutrition and the priority treatment of chylothorax if present, resorting to alternatives such as radiotherapy or surgery but does not achieve adequate control.
Keywords: Lymphangiomatosis; Heterogeneity; Sporadic malformations; PI3K/AKT/mTOR pathway; RAS pathway
Citation: Ordoñez KP, Suarez SR, Morillo JSG (2019) Heterogeneity and Phenotypic Diversity of Multiple Sistemic Lymphangiomatosis. Diagn Pathol Open 4:157.
Copyright: © 2019 Ordoñez KP, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Select your language of interest to view the total content in your interested language
Share This Article
- Total views: 129
- [From(publication date): 0-0 - Apr 02, 2020]
- Breakdown by view type
- HTML page views: 110
- PDF downloads: 19