Sonography of Lymphangioleiomyoma in Lymphangioleiomyomatosis: Demonstration of Diurnal Variation in Lesion Size
Nilo A. Avila1,
Andrew J. Dwyer1,
Diane V. Murphy-Johnson1,
Pamela Brooks2 and
Joel Moss2
1 Diagnostic Radiology Department, Warren Grant Magnuson Clinical Center,
National Institutes of Health, 10 Center Dr., MSC 1182, Bldg. 10, Rm. 1C-658,
Bethesda, MD 20892-1182.
2 Pulmonary-Critical Care Medicine Branch, National Heart, Lung and Blood
Institute, National Institutes of Health, Bethesda, MD 20892-1590.
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Fig. 1. —51-year-old woman with mild lung involvement with
lymphangioleiomyomatosis who complained of pelvic discomfort and urinary
frequency. Transabdominal longitudinal sonogram of pelvis shows large simple
cyst (arrow) adjacent to uterus (U).
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Fig. 2. —33-year-old woman with severe lung involvement with
lymphangioleiomyomatosis who complained of increased abdominal girth that
worsened during day. Transabdominal longitudinal sonogram of right pelvis
shows thick-walled complex mass (arrows) with central anechoic
space.
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Fig. 3. —42-year-old woman with mild lung involvement with
lymphangioleiomyomatosis diagnosed after biopsy of pelvic mass to exclude
ovarian cancer. Abdominal longitudinal sonogram shows large retroperitoneal
mass (arrows) isoechoic to liver, containing serpiginous central
anechoic spaces that had no flow on Doppler interrogation and were thought to
represent dilated lymphatic channels.
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Fig. 4A. —25-year-old woman having severe lung involvement with
lymphangioleiomyomatosis, diagnosed after biopsy of pelvic mass to exclude
ovarian cancer. Patient complained of chronic back pain and urinary frequency
that worsened during day. Transverse sonogram obtained at 8:30 am at level of
right kidney shows complex mass (arrows) that measures 3.3 x
4.8 x 5.5 cm in anteroposterior, transverse, and longitudinal
diameters.
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Fig. 4B. —25-year-old woman having severe lung involvement with
lymphangioleiomyomatosis, diagnosed after biopsy of pelvic mass to exclude
ovarian cancer. Patient complained of chronic back pain and urinary frequency
that worsened during day. Follow-up sonogram obtained at 2:45 pm shows
interval increase in size of cystic portion of mass (arrows) that
then measured 4.8 x 5.0 x 7.0 cm in anteroposterior, transverse,
and longitudinal diameters.
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Fig. 4C. —25-year-old woman having severe lung involvement with
lymphangioleiomyomatosis, diagnosed after biopsy of pelvic mass to exclude
ovarian cancer. Patient complained of chronic back pain and urinary frequency
that worsened during day. Transabdominal longitudinal sonogram of left adnexa
obtained at 8:15 am shows complex left pelvic mass (arrows) that
measures 2.5 x 2.6 x 3.1 cm in anteroposterior, transverse, and
longitudinal diameters.
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Fig. 4D. —25-year-old woman having severe lung involvement with
lymphangioleiomyomatosis, diagnosed after biopsy of pelvic mass to exclude
ovarian cancer. Patient complained of chronic back pain and urinary frequency
that worsened during day. Follow-up transabdominal sonogram obtained at 3:00
pm shows interval increase in size of left adnexal mass (arrows) that
then measured 3.7 x 3.8 x 3.8 cm in anteroposterior, transverse,
and longitudinal diameters.
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Fig. 5. —Scatterplot of percentage change in volume as function of
morning volume indexes of lymphangioleiomyomas in 21 patients. Morning volume
indexes (anteroposterior x transverse x longitudinal measurements)
ranged from 1 to 521 cm3 (median, 38 cm3). Percentage
change in volume ranged from 10% to 484% (median, 38%). Greater percentage
changes in volume were more common in smaller masses.
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Fig. 6A. —43-year-old woman having moderate involvement of lungs with
lymphangioleiomyomatosis. Endovaginal longitudinal sonogram of right adnexa
obtained at 10:00 am shows solid mass (arrows) isoechoic to uterus
and situated between right iliac vessels and right ovary.
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Fig. 6B. —43-year-old woman having moderate involvement of lungs with
lymphangioleiomyomatosis. Follow-up sonogram obtained at 2:50 pm shows
increase in size of mass (calipers), which now contains anechoic spaces in
addition to solid component. ov = ovary, v = pelvic vessel.
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Fig. 7A. —Proposal models for effects of lymphangioleiomyomatosis (LAM)
on lymphatics. Models of effect of LAM on lymphatics show normal lymphatic
vessel with unidirectional valve and normal direction of lymph flow
(arrows) (a), proliferation of abnormal smooth-muscle cells (LAM
cells) on walls of lymphatic that causes mural thickening and luminal
narrowing (b), and obstruction of lymph flow by LAM cells that results in
dilatation of lymphatic proximal to obstruction, creating lymphangioleiomyoma
(c).
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Fig. 7B. —Proposal models for effects of lymphangioleiomyomatosis (LAM)
on lymphatics. Models show diurnal variation in size of lymphangioleiomyomas
between morning and afternoon. Normal lymphatic flow (arrows) is
shown in morning (a). By late afternoon, lymphangioleiomyoma has increased in
size (b). Phenomenon results from combination of factors: increased lymph flow
during day caused by increased lymph production after meals and increased
return of chyle from extremities after normal daily exercise.
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Copyright © 2005 by the American Roentgen Ray Society.
