title: Pulmonary lymphangiomatosis: a report of two cases.
abstract: Lymphangiomatosis, a rare diseases of controversial origin, occurs in individuals of any age, regardless of gender, but is predominantly seen in younger individuals. It often presents with thoracic involvement, although, the bones, spleen and liver can also be affected. Histologically, the pulmonary involvement includes proliferation, complex anastomoses and secondary dilatation of the lymphatic vessels. Clinically, the presentation is variable. Although radiographic findings can be suggestive of the disease, the final diagnosis is made histologically. We report two cases of lymphangiomatosis, both in females: one was oligosymptomatic and is being treated for the disease; the other had a more progressive form, was diagnosed quite late and ultimately died of the disease.
authors.affiliation: Universidade Federal do Paraná, Curitiba, PR, Brasil. funpul@hc.ufpr.br <funpul@hc.ufpr.br>
authors.forename: Adma Silva
authors.initials: AS
authors.lastname: de Lima
authors.forename: Mariane Gonçalves
authors.initials: MG
authors.lastname: Martynychen
authors.forename: Roseni Teresinha
authors.initials: RT
authors.lastname: Florêncio
authors.forename: Lêda Maria
authors.initials: LM
authors.lastname: Rabello
authors.forename: João Adriano
authors.initials: JA
authors.lastname: de Barros
authors.forename: Dante Luis
authors.initials: DL
authors.lastname: Escuissato
mesh_terms: D000328:Adult; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D001706:Biopsy; D003952:Diagnostic Imaging; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016898:Interferon-alpha / Q000627:therapeutic use; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D008208:Lymphatic System / Q000473:pathology; D010996:Pleural Effusion / Q000000981:diagnostic imaging; D011859:Radiography
chemical_list: D020533:Angiogenesis Inhibitors; D016898:Interferon-alpha