Kaposiform Lymphangiomatosis: Pathologic Aspects in 43 Patients.
Am J Surg Pathol
; 46(7): 963-976, 2022 07 01.
Article
em En
| MEDLINE
| ID: mdl-35385405
ABSTRACT
Kaposiform lymphangiomatosis is an uncommon generalized lymphatic anomaly with distinctive clinical, radiologic, histopathologic, and molecular findings. Herein, we document the pathology in 43 patients evaluated by the Boston Children's Hospital Vascular Anomalies Center from 1999 to 2020. The most frequent presentations were respiratory difficulty, hemostatic abnormalities, and a soft tissue mass. Imaging commonly revealed involvement of some combination of mediastinal, pulmonary, pleural, and pericardial compartments and most often included spleen and skeleton. Histopathology was characterized by dilated, redundant, and abnormally configured lymphatic channels typically accompanied by dispersed clusters of variably canalized, and often hemosiderotic, spindled lymphatic endothelial cells that were immunopositive for D2-40, PROX1, and CD31. An activating lesional NRAS variant was documented in 9 of 10 patients. The clinical course was typically aggressive, marked by hemorrhage, thrombocytopenia, diminished fibrinogen levels, and a mortality rate of 21%.
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Base de dados:
MEDLINE
Assunto principal:
Células Endoteliais
/
Pulmão
Idioma:
En
Ano de publicação:
2022
Tipo de documento:
Article