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311
Lymphangioscintigraphy in AIDSAssociated Kaposi Sarcoma
Marlys
H. Witte1 Milan
George
Fiala2
C. McNeiIl3
Charles L. Witte1 Walter H. Williams3 John Szabo4
Kaposi sarcoma, a common arise from the vasculature
nature and extent used an improved
opportunistic
and possibly
neoplasm from
of lymphatic involvement technique of whole-body
nodeficiency-virus-seropositive
sarcoma had bilateral human serum albumin
men (40-51
lymphatic
complicating
in AIDS-associated
lymphangioscintigraphy. years
AIDS,
endothelium.
is thought
To evaluate
to the
Kaposi sarcoma, we Six human-immu-
old) with AIDS and extensive
Kaposi
handMBq). intradermal injection of 0.05 ml of scanning “Tc-labeled with (500 and/or MCi, 18.5 After sequential whole-body a digital gamma camera, the findings were interpreted by comparing them with findings from similar studies in 30 other patients without AIDS or Kaposi sarcoma (26 with primary or secondary lymphedema and four with normal extremities). Unlike in normal limbs, where lymphangioscintigraphy displayed early lymphatic truncal and regional nodal filling with radionuclide, in patients with Kaposi sarcoma, lymphangioscintigraphy disclosed a variety of abnormal patterns with some features distinct and others resembling lymphatic dysplasia as seen in primary and secondary Iymphedema These included focal accumulation of tracer within lymphatic channels in the distribution of cutaneous Kaposi lesions; delayed tracer transport with absent, faint, or intense regional lymph nodal uptake; and retarded or impeded lymphatic drainage with tracer intensification in the region of Kaposi sarcoma plaques. The impaired lymphatic drainage and nodal dysfunction seen on scintigrams in patients with AIDS-associated Kaposi sarcoma suggest a close connection between the lymphatic system and this disorder. foot
AJR 155:311-315, August 1990
Received vision March
February 7, 1990; 1 5, 1990.
accepted
after
Presented in part at the annual meeting American Federation for Clinical Research, 1989, Washington, DC.
re-
of the May
This work was supported in part by the Arizona Disease Control Research Commission, Contracts 8277-000000-1-1-AT-6625 and -ZB-7492.
Once regarded into international
Dating back to Kaposi’s original description, the lymphatic vasculature has long been postulated as an important site of origin and involvement [1 -3]. Lymphedema, which commonly accompanies classical and African Kaposi disease [4], also has been recognized increasingly in epidemic Kaposi sarcoma associated with AIDS and involves multiple swollen, woody, and weeping sites (such as the legs, arms, genitalia,
Department of Surgery, University of Arizona College of Medicine, 1501 N. Campbell Ave., Tucson, AZ 85724. Address reprint requests to M. H. Witte.
as a curiosity in the Western world, Kaposi sarcoma catapulted prominence in 1 981 as the heraldic lesion of the epidemic of AIDS.
palate,
and face)
[3, 5].
1
2
Department
Center,
39000
of Medicine, Bob
Hope
Eisenhower
Dr., Rancho
Medical Mirage,
CA
92270. Department of Radiology, University College of Medicine, 1501 N. Campbell son, AZ 85724. 3
of Arizona Ave., Tuc-
4 Department of Nuclear Medicine, Eisenhower Medical Center, 39000 Bob Hope Dr., Rancho Mirage, CA 92270.
0361-803x/90/1552-031
C American Roentgen
1 Ray Society
Lymphatic trunks and regional nodes are now readily accessible to improved visualization and functional examination by a refinement of the simple, noninvasive method of radionuclide lymphangioscintigraphy [6]. In order to shed further light on the pathogenesis ofthis opportunistic neoplasm, the peripheral lymphatic system was examined by lymphangioscintigraphy in six patients with AIDS accompanied by cutaneous Kaposi sarcoma of the extremities. The findings were compared with results of earlier studies in patients with normal limbs and in those with either primary Subjects
or secondary and
lymphedema.
Methods
The patient population mosexual men (ages,
included
40-51
six
human-immunodeficiency-virus
years; mean, 46.7 years) with extensive
(HIV)-seropositive
histologically
ho-
proved
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312
WITTE
ET AL.
AJR:155,
August
1990
Fig. 1.-Characteristic lymphangioscintigrams (anterior projection). A, Normal adult subject (legs). B, 17-year-old man with secondary right leg lymphedema from irradiation and excision of a retropentoneal neuroblastoma as an infant. C, 76-year-old woman, 9 years after left modified radical mastectomy and regional irradiation. D, 42-year-old woman, 8 years after right modified radical mastectomy. Note normal lymphatic channels and regional nodes on uninvolved side (BD) with initially cleariy defined lymphatic trunks followed by progressive dispersion of radionuclide throughout lymphedematous limb. Compare with Fig. 2. (Portions of A and B are reproduced with permission from McNeilI et aI. [6].)
mucocutaneous
Kaposi
accompanied
sarcoma
by worsening
lymphadenopathy.
(duration,
peripheral
On the basis
of constitutional
symptoms,
B or the
classification
IV NYU
4 months
edema
of the extent
all patients
of disease
years)
2
regional
and
presence
to either the lIlA-
conformed
of Kaposi
to
or prominent
sarcoma
[7].
Three
patients
had visceral involvement with Kaposi sarcoma, and five had low Thelper lymphocyte (CD4) counts (mean, 122/tI) with advanced disease later complicated by Pneumocystis carinii pneumonia or infection with cytomegalovirus or Candida despite azidothymidine drug administration. Four of them died within 1 -6 months. One patient, who had taken the drug IMREG-1 (lmreg, Inc., New Orleans, LA) for 1 year, has remained stable (CD4 count, 685/tI). Light Kaposi
microscopic sarcoma
copy
examination in each
showed
not only
typical
cells but also proliferating lymphatic For
or blood isotopic
(1 8.5
MBq)
dorsum
highly
gamma
bound
injected
foot camera
scintigrams
injection
site
with
migration light
next
A
B
in primary lymphedema. in a 39-year-old man with a long history of lymphedema show intense dispersion of radionuclide in the right leg without lymphatic truncal bands, consistent with “hypoplasia,” which was confirmed by conventional lymphography (top). B, Scintigrams of a 16-year-old woman with left leg lymphedema show no transport of radionuclide in left leg (“aplasia”). Compare with Fig. 1. (Modified with permission from McNeill et al [6].) External markers are at level of xiphoid (X) pubis (P), and knees (K). Fig. 2.-Lymphangioscintigrams A, Scintigrams (bottom)
of the was
imaged
of radionuclide
exercise,
serial
1 hr and 3-6
Either
channels
micros-
with
spindle
obstructing
the
Discussion). 0.05
ml containing human
intradermally
into
500
serum
a web
a Toshiba
a low-energy,
capable of serial “sweeps” body
(see
confirmed
electron
cells effectively
‘Tc-Iabebed
or hand.
lesions
patients,
vascular
endothelial lumen
skin
in two
irregular
vascular
was of the
digital
and
lymphangioscintigraphy, of
(99mTcHSA)
of excised
patient,
space
on
or a General
high-resolution
Ci
albumin the
Electric collimator
of the torso was used to obtain whole-
peripheral for was
whole-body
and
1 0-1
central
5 mm
detected. images
lymphatic
or until
significant
After the patient were
obtained
system.
The
cephalad
performed during
the
hr later. Findings
in AIDS-associated Kaposi sarcoma were compared with those in 30 other patients without AIDS or Kaposi sarcoma but with primary or secondary arm or leg lymphedema, usually unilateral, and four control subjects without edema, most of whom were described previously [6]. Results Except for a transient “burning sensation” mal injection, isotopic lymphangioscintigraphy discomfort or adverse sequelae. Well-defined
during intraderwas without sequential im-
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AJR:155,
August
LYMPHANGIOSCINTIGRAPHY
1990
OF
KAPOSI
313
SARCOMA
..-
Fig. 3.-A, 43-year-old man with AIDS-associated Kaposi sarcoma. B-D, Lymphangioscintigrams of legs of same patient (bottom) show prompt lymphatic transport of radionuclide to large regional nodes (clinically palpable) with numerous focal accumulations of radionuclide intensity over time in calf and mid thigh matching distilbution of cutaneous Kaposi lesions shown in A. Lymphangioscintigrams of arms (top) show a similar but less dramatic pattern. Midline rounded densities are markers on sternal notch and xiphoid (top) and pubis and knees (bottorn).
.
.
‘
.
.
C
‘St
I
1
.
e
I
D
ages of peripheral and central lymphatic anatomy (Figs. 1 and 2) reflecting interstitial transport of protein were displayed and, as shown previously [6], corresponded structurally and functionally to conventional contrast lymphograms by direct lympathic cannulation. Normally (Fig. 1 A), peripheral and central lymph trunks and draining regional lymph nodes were visualized on scintiscans within 1 5 mm, contrasting sharply with minimal background counts in nonlymphatic tissues. In secondary or obstructive Iymphedema(Figs. 1 B-i D), discrete linear “bands” representing lymphatic trunks typically were accompanied by delayed transit of the radionuclide, absent or faintly visualized regional lymph nodes, and subsequent dispersion of the radionuclide outlining the tissue contours of the limb. In primary lymphedema, transport from the injection site was delayed (Fig. 2A) or absent (Fig. 2B), and lymphatic trunks were either poorly defined with prompt dispersion of 99Tc-HSA at the injection site (hypoplasia) or images of lymphatic trunks and lymph nodes were absent and transport of 99mTc-HSA was negligible (aplasia). Although patterns in AIDS-associated Kaposi sarcoma vared even in different limbs of the same patient (Figs. 3-5), specific scintigraphic abnormalities were noted. These included delayed or absent absorption of radionuclide from the injection site into the lymphatics (four of six patients); pre-
Fig. 4.-A, 50-year-old man with AIDS-associated facial, genital, and peripheral lymphedema with
Kaposi ichthyotic,
sarcoma. Note ligneous
skin
changes and Kaposi lesions.
$ A
B
B, Lymphangioscintigrams show absence of radionuclide transport (lymphatic obliteration?) in legs (bottom), whereas in right arm (top), uptake of radionuclide into lymphatic trunks Is slow and faint with radionuclide dispersion outlining forearm Kaposi lesions. No axillary nodes are seen on either side. Rounded densities in midline denote markers at sternal notch, xiphoid, pubis, and knees (top to bottom, respectively).
When this patient (CD4)
count
was studied,
was