LYMPHATIC RESEARCH AND BIOLOGY

Volume 9, Number 1, 2011 Case Report

ª Mary Ann Liebert, Inc.
DOI: 10.1089/lrb.2010.0025

18
F-FDG PET/CT in a Rare Case of Stewart–Treves
Syndrome: Future Implications and Diagnostic Considerations

Mads Radmer Jensen, M.D.,1 Lars Friberg, M.D.,1

Tonny Karlsmark, M.D., D.M.Sci.,2 and Jens Bülow, M.D., D.M.Sci.1

Abstract

Background: The aim of this article is to illustrate the possible applications of 18F-fluorodeoxyglucose positron
emission tomography/computer tomography (18F-FDG PET/CT) in chronic extremity lymphedema and its
complications.
Methods and Results: 18F-FDG PET/CT findings in a rare case of Stewart–Treves Syndrome (STS), angiosarcoma
secondary to chronic extremity lymphedema, are presented. Lymphedema of the extremities is a debilitating
disease characterized by chronic swelling due to interstitial edema caused by insufficient lymphatic drainage
capacity. Progression with skin thickening, subcutaneous fibrosis, and increased adipose tissue volume is com-
mon. Chronic inflammation has been suggested as a key pathophysiologic component. STS is a rare complication
with a very poor prognosis; however, early diagnosis and radical treatment is associated with increased survival.
Thus, accurate pretreatment staging is paramount. 18F-FDG PET/CT is highly sensitive in detecting increased
glucose metabolism as seen in many types of cancer and inflammation. The role of 18F-FDG PET/CT in the
management of lymphedema and its complications has to our knowledge yet to be described. This case documents
high 18F-FDG uptake in STS, but is at the same time an example of the low specificity of this imaging modality.
Conclusions: We suggest that 18F-FDG PET/CT has the potential to become an important tool in the staging and
treatment planning of Stewart–Treves syndrome. Furthermore, 18F-FDG-accumulation may be a sensitive tool in
detecting low grade inflammation in the skin and subcutis, which has been suggested to cause tissue remodeling in
lymphedema progression. However, further studies are needed to elucidate this theory.

Introduction The uptake of 18F-FDG is a measure of tissue carbohydrate

L ymphedema of the lower extremities is a progressive
debilitating disease characterized by chronic swelling
due to interstitial edema caused by insufficient lymphatic
drainage capacity.1,2,3 Lower extremity lymphedema is of-
ten undiagnosed or misdiagnosed, and treatment therefore
absent, delayed, or inadequate.4–7 Progression is common
and is mainly caused by abundant remodeling of the skin
and subcutaneous tissue with dermal thickening, subcuta-
neous fibrosis, and increased adipose tissue deposition.8–11
The underlying pathophysiologic process is still incom-
pletely understood, but evidence from animal lymphedema
models show that inflammation probably plays a key
role.12,13
A rare but often fatal complication of lymphedema is
Stewart–Treves syndrome (STS), which is defined as the de-
velopment of a highly malignant angiosarcoma secondary to
chronic lymphedema.14
metabolism and is visualized by PET/CT scanning. 18F-FDG
PET/CT has been demonstrated to be a very sensitive tool in
detecting inflammation and many neoplasms.15,16 The role of
18
F-FDG PET/CT in the management of lymphedema and its
complications has to our knowledge yet to be elucidated.
Future possible applications are illustrated by the following
case report.
Case Report
A 44-year-old woman with known lymphoscintigraphy-
verified lymphedema (Fig. 1) presented with newly devel-
oped macular skin changes in the affected lower left extremity
(Fig. 2). The patient had acquired secondary lymphedema 6
years earlier following repeated surgery for an intrapelvic
carcinoma of unknown origin involving resection of the
sigmoid colon and multiple retroperitoneal lymph nodes.
An initial histological skin biopsy showed no signs of

Departments of 1Clinical Physiology and Nuclear Medicine and 2Dermatology and Venerology, Bispebjerg Hospital, University Hospital
of Copenhagen, Denmark.

61
62 JENSEN ET AL.

FIG. 2. Clinical photograph at admission showing macular

skin lesions in the affected lower left extremity.

FIG. 1. 99mTc-nanocolloid lymphoscintigraphy showing no

visualization of subcutaneous lymphatic collectors or lymph
nodes in the affected lower left extremity on either the early
(15 min post injection, left) or the late scan (120 min post in-
jection, right). In the lower right extremity, there are signs of
tracer leakage from subcutaneous lymphatic collectors at
knee level.

malignancy. The symptoms were interpreted as cellulitis,

but repeated treatments with antibiotics had no effect. In the
months prior to admission, the lymphedema progressed rap-
idly, resulting in a total weight gain of approximately 35 kilo-
grams. The skin changes progressed and ulceration and clinical
signs of cellulitis developed. The patient was admitted for
optimized compression treatment and intravenous antibiotics.
Among a host of different diagnostic examinations to de-
termine the cause of the sudden edema progression, a 18F-
FDG PET/CT scan was performed to rule out recurrence of
the intrapelvic neoplasm (Fig. 3). The scan showed massive
edema of the subcutaneous tissue of the lower left extremity
and surprisingly a pronounced 18F-FDG accumulation pri-
marily in the skin and subcutaneous tissue, with sharp de-
marcation at the ankle and groin level. There was no evidence
of intrapelvic tumor recurrence. In spite of large treatment
efforts to treat the cellulitis and reduce the edema (compres-
sion, elevation, loop-diuretics, broad spectrum antibiotics), FIG. 3. 18F-FDG PET/CT showing massive subcutaneous
the patient’s condition continued to deteriorate over the edema (left) and increased 18F-FDG uptake in the skin and
following weeks with treatment refractory edema and pro- subcutaneous tissue with unusual sharp demarcations (right)
gressive ulceration (Fig. 4). A second 18F-FDG PET/CT scan in the affected lower left extremity.
PET/CT IN STEWART–TREVES SYNDROME
FIG. 4. Clinical photograph approximately 8 weeks after
admission showing extensive progression of skin lesions.
was performed 6 weeks later to make sure a neoplasm was not
overlooked due to the massive FDG accumulation in the af-
fected leg on the first scan (Fig. 5). It showed a marked de-
crease in overall 18F-FDG-uptake, however, multiple focal
lesions in the skin and subcutaneous tissue remained. A his-
tologic skin biopsy was repeated due to the lack of treatment
effect and the diagnosis of angiosarcoma was made. The
general condition of the patient was too poor to offer surgery
or chemotherapy and she died shortly thereafter due to the
massive tumor burden, infection, and kidney failure.
Discussion
The case report described above represents an example of
the possible applications of 18F-FDG PET/CT in chronic ex-
tremity lymphedema and its complications. Lymphedema is a
chronic progressive disease but the speed of progression is very
individual. It is, however, generally accepted that conservative
treatment with manual lymphatic drainage massage, com-
pression therapy, and meticulous skin care results in a better
outcome.17 The pathophysiologic mechanisms governing skin
and subcutaneous tissue remodeling in extremity lymphedema
have yet to be fully elucidated. If chronic inflammation plays a
central role, then the degree of inflammation may be associated
with the progression speed. 18F-FDG PET/CT scanning has
been shown to be a very sensitive modality in the diagnosis of
focal inflammatory processes.15 We suggest that 18F-FDG PET/
63
FIG. 5. The second 18F-FDG PET/CT 6 weeks later show-
ing a decrease in edema volume and soft tissue 18F-FDG
accumulation compared to the first scan, but multifocal le-
sions with increased 18F-FDG uptake in the skin and sub-
cutaneous tissue in the lower left extremity remain.
CT can become an important tool in identifying the patients at
risk of tissue remodelling progression.
STS is a rare and often fatal complication to chronic lym-
phedema with an estimated incidence in breast cancer-related
lymphedema (BCRL) of 0.07% –0.45%.18 The average debut of
STS in BCRL is 10 years after mastectomy and the prognosis is
poor with a median survival of 19 months. In lower extremity
lymphedema, STS is even rarer but seems to have a later debut
(average 19 years) and perhaps a slightly better prognosis
(median survival 34 months).19
Local extensive spread via satellite lesions and metastasis
to the lungs are common.18 Early diagnosis and radical sur-
gical treatment is associated with a better prognosis.19 Thus
pretreatment staging is paramount in choosing the correct
treatment modality. 18F-FDG PET/CT has the potential to
become a first line choice in initial staging and treatment
follow-up because STS has been demonstrated to have a high
18
F-FDG uptake.20–22
This case furthermore represents one of the possible pitfalls
in applying 18F-FDG PET/CT on lymphedema patients. In the
first scan, the STS changes were masked by the inflammation
caused by the present cellulitis. The STS changes first became
evident after the cellulitis had been sufficiently treated. Since
18
F-FDG accumulate in all cells with a high glucose metabolism
such as some forms of cancer cells and activated immune cells,
the modality has a low specificity under circumstances where
infection and malignancies are present concomitantly.15
Author Disclosure Statement
No competing financial interests exist.
64 JENSEN ET AL.

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