Unique diagnostic features and successful management of a patient with disseminated lymphangiomatosis and chylothorax.
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.
Yeager
Nicholas D
ND
Sections of Hematology/Oncology, Children's Hospital, The Ohio State University, Columbus, OH 43205, USA. yeagern@pediatrics.ohio-state.edu
Hammond
Sue
S
Mahan
John
J
Davis
John Terrance
JT
Adler
Brent
B
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
D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D007155:Immunologic Factors; D016898:Interferon-alpha; D000077268:Pamidronate