title: Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly.
abstract: OBJECTIVE
  To describe the clinical and imaging characteristics of a new lymphatic disorder with a unique histological pattern and poor prognosis.
  
  
  METHODS
  An observational, retrospective study identified and characterized 20 patients with distinct lymphatic histopathology referred to the Vascular Anomalies Center at Boston Children's Hospital between 1995 and 2011.
  
  
  RESULTS
  The median age at onset was 6.5 years (range, birth to 44 years). Clinical and radiologic findings suggested a generalized process. The most common presentations were respiratory symptoms (50%), hemostatic abnormalities (50%), and an enlarging, palpable mass (35%). All patients had mediastinal involvement; 19 patients developed pericardial (70%) and/or pleural effusions (85%). Extrathoracic disease manifested in bone and spleen and less frequently in abdominal viscera, peritoneum, integument, and extremities. Despite aggressive procedural and medical therapies, the 5-year survival was 51% and the overall survival was 34%. Mean interval between diagnosis and death was 2.75 years (range, 1-6.5 years).
  
  
  CONCLUSIONS
  We describe a clinicopathologically distinct lymphatic anomaly. We propose the term kaposiform lymphangiomatosis (KLA) because of characteristic clusters or sheets of spindled lymphatic endothelial cells accompanying malformed lymphatic channels. The intrathoracic component is most commonly implicated in morbidity and mortality; however, extrathoracic disease is frequent, indicating that KLA is not restricted to pulmonary lymphatics. The mortality rate of KLA is high despite aggressive multimodal therapy.
authors.affiliation: Dana Farber/Boston Children's Cancer and Blood Disorders Center, Boston Children's Hospital, Boston, MA; Harvard Medical School, Boston, MA. Electronic address: Stacy.Croteau@childrens.harvard.edu.
authors.forename: Stacy E
authors.initials: SE
authors.lastname: Croteau
authors.affiliation: Department of Pathology, Boston Children's Hospital, Boston, MA; Harvard Medical School, Boston, MA.
authors.forename: Harry P W
authors.initials: HP
authors.lastname: Kozakewich
authors.affiliation: Department of Pathology, Boston Children's Hospital, Boston, MA; Harvard Medical School, Boston, MA.
authors.forename: Antonio R
authors.initials: AR
authors.lastname: Perez-Atayde
authors.affiliation: Department of Surgery, Boston Children's Hospital, Boston, MA; Harvard Medical School, Boston, MA.
authors.forename: Steven J
authors.initials: SJ
authors.lastname: Fishman
authors.affiliation: Division of Interventional Radiology, Boston Children's Hospital, Boston, MA; Harvard Medical School, Boston, MA.
authors.forename: Ahmad I
authors.initials: AI
authors.lastname: Alomari
authors.affiliation: Division of Interventional Radiology, Boston Children's Hospital, Boston, MA; Harvard Medical School, Boston, MA.
authors.forename: Gulraiz
authors.initials: G
authors.lastname: Chaudry
authors.affiliation: Department of Plastic and Oral Surgery, Boston Children's Hospital, Boston, MA; Harvard Medical School, Boston, MA.
authors.forename: John B
authors.initials: JB
authors.lastname: Mulliken
authors.affiliation: Dana Farber/Boston Children's Cancer and Blood Disorders Center, Boston Children's Hospital, Boston, MA; Harvard Medical School, Boston, MA.
authors.forename: Cameron C
authors.initials: CC
authors.lastname: Trenor
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D042783:Endothelial Cells / Q000473:pathology; D005260:Female; D006390:Hemangioendothelioma / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D059885:Kasabach-Merritt Syndrome / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D008198:Lymph Nodes / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D012514:Sarcoma, Kaposi / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D015996:Survival Rate / Q000639:trends; D014481:United States / Q000453:epidemiology; D055815:Young Adult
keywords: GLA; Generalized lymphatic anomaly; KHE; KLA; Kaposiform hemangioendothelioma; Kaposiform lymphangiomatosis; MRI; Magnetic resonance imaging