Extract
Lymphangiomatosis is a rarely encountered syndrome from the category of
diffuse, benign
neoplasms1. The
lesions are believed to result from abnormal development and proliferation of
the lymphatic
system1-4.
The most common areas of involvement are the mediastinum, lung, heart, pleura,
pericardium, and, less commonly, bone, including the vertebrae and
ribs1-11.
Skeletal involvement usually consists of multiple rounded, cystic lesions that
most commonly occur in the femur and
pelvis1,6,11-13.
The lesions can be classified into three categories: (1) lymphangioma simplex
lesions, which are composed of capillary sized, thin-walled lymphatic channels
usually affecting the
skin1,6,7;
(2) cystic lymphangiomas or hygromas, which are fluid-filled lesions ranging
from a few millimeters to many centimeters in size and most commonly occur in
the axilla or
neck1,14-16;
and (3) lymphangioma cavernosum, which consists of dilated lymphatic channels
often with fibrous adventitial coats and which usually affects visceral organs
and bone17. All
three types can be present simultaneously in patients with
lymphangiomatosis1,4.
The family of the patient was informed that data concerning the case would be
submitted for publication.