lymphangiomatosis


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lymphangiomatosis

A rare lesion that usually affects women of child-bearing years, which is characterised by well-demarcated osteolytic lesions variably accompanied by sclerosis.

Clinical findings
Slowly progressive dyspnoea, chylous effusion, recurring pneumothorax, haemoptysis, massive haemorrhage.

DiffDx
Fibrous dysplasia.
References in periodicals archive ?
Propranolol for intractable diffuse lymphangiomatosis.
Bone lymphangiomatosis: Treatment with percutaneosu cementoplasty.
Miscellaneous Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis) (a) Modifications to the previous classification are represented in bold.
In children, these tumors may be part of a systemic lymphangiomatosis syndrome, which involves the mediastinum, lungs, liver, kidney, GI and GU tracts.
This discrepancy is most likely due to the inhibition of osteoblastic differentiation by the aggressive lymphangiomatosis which also explains the lack of a sclerotic rim around the cysts in this patient.
Colby et al., "Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome," American Journal of Respiratory and Critical Care Medicine, vol.
Langer, "Assessment of the lymphatic system in patients with diffuse lymphangiomatosis by magnetic resonance imaging," European Journal of Radiology, vol.
It is known by different synonyms such as renal lymphangiomatosis, peripelvic lymphangiectasia, hygromarenale, polycystic disease of renal sinuses8.
Splenic lymphangiomas may be single or multiple (lymphangiomatosis).
Diagnostic Criteria for Tuberous Sclerosis (2012) Major Criteria Minor Criteria Cortical tubers Non-renal hamartoma Subependymal nodules Confetti-like skin lesions Hypomelanocytic macule Multiple renal cysts (at least 3 or more and larger than 5 mm) Shagreen patch Hypopigmented patch in the retina Angiofibroma in the face (more than three) Ungal and periungal fibromas Retinal hamartomas Rhabdomyoma in the heart Lymphangiomatosis in the lung Angiomyolipoma in the kidney (more than two) Definite diagnosis: 2 major or 1 major + >2 minor criteria Possible diagnosis: 1 major or >2 minor criteria Table 2.
CT and MR imaging in cystic abdominal lymphangiomatosis. J Comput Assist Tomogr 1989;13:534-6.