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 ?
Exacerbation of familial renal lymphangiomatosis during pregnancy.
Known specific causes include congenital mediastinal lymphangiectasia (cystic hygroma) (5), gastric carcinoma (6, 7), lymphangiomatosis of the chest/ Gorham syndrome (8, 9), primary mediastinal neoplasms such as lymphoma and germ cell tumor (6, 10), mediastinal hamartoma (8), infection with tuberculosis (11), deep vein thrombosis with superior vena cava syndrome (12), pancreatitis (13), allergic alveolitis (14), and Behcet's disease (15).
Lymphangiomatosis in the lung or renal angiomyolipoma Definite diagnosis: two major or one major + two minor criteria Suspicious diagnosis: one major + one minor criteria Possible diagnosis: one major or two minor criteria Table 2.
Little Alfie is thought to be one of only three people in the UK to suffer from the rare condition lymphangiomatosis, which has left him with a growth so big surgeons are unable to remove it.
Diffuse pulmonary lymphangiomatosis shows diffuse proliferation of lymphatic vascular spaces and smooth muscles, mimicking LAM.
The diagnosis could be suspected on plain radiograph but MRI is the better diagnostic imaging modality as it can rule out other differential diagnoses such as neurofibromatosis, haemangiomatosis, lymphangiomatosis, Proteus syndrome and fibrolipomatous hamartoma.
At post-mortem, a diagnosis of lymphangiomatosis was made.
1 Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis) Table 2.
Miscellaneous Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis) * Guidelines for Classification of Congenital Systemic-to-Pulmonary Shunts 1.
Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome.
The woman, who was diagnosed with lymphangiomatosis in 1998, was transferred to the hospital from a Kochi hospital in September after hormone treatment failed to take effect and her condition worsened.
This finding confirmed the diagnosis of splenic hemangiomatosis as opposed to lymphangiomatosis or peliosis.