title: Unique diagnostic features and successful management of a patient with disseminated lymphangiomatosis and chylothorax.
abstract: Disseminated lymphangiomatosis is a rare vascular tumor characterized by a proliferation of abnormal lymphatic channels that often involves multiple organ systems. One particularly morbid manifestation of this disorder is the presence of bony lytic lesions with associated chylothorax. Because of its unusual nature, this condition is often a diagnostic and therapeutic challenge. In this report, we present the diagnostic features, including a unique radiologic finding, and successful management of a 7-year-old girl with this condition using a combination of aggressive surgery and medical treatment with interferon and pamidronate.
authors.affiliation: Sections of Hematology/Oncology, Children's Hospital, The Ohio State University, Columbus, OH 43205, USA. yeagern@pediatrics.ohio-state.edu
authors.forename: Nicholas D
authors.initials: ND
authors.lastname: Yeager
authors.forename: Sue
authors.initials: S
authors.lastname: Hammond
authors.forename: John
authors.initials: J
authors.lastname: Mahan
authors.forename: John Terrance
authors.initials: JT
authors.lastname: Davis
authors.forename: Brent
authors.initials: B
authors.lastname: Adler
mesh_terms: D050071:Bone Density Conservation Agents / Q000008:administration & dosage; D002648:Child; D002916:Chylothorax / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy*; D003937:Diagnosis, Differential; D004164:Diphosphonates / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage; D016898:Interferon-alpha / Q000008:administration & dosage; D018192:Lymphangioleiomyomatosis / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy*; D000077268:Pamidronate; D010997:Pleural Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy*; D014057:Tomography, X-Ray Computed
chemical_list: D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D007155:Immunologic Factors; D016898:Interferon-alpha; D000077268:Pamidronate