Summary: The patient was a 44-year-old woman who was transferred to our
institution with a presumed diagnosis of superior vena cava syndrome
(SVCS) secondary to thrombus formation. The patient experienced syncope,
along with headache, bilateral facial, neck, and upper-extremity
swelling, as well as shortness of breath that was progressive over
several months. The review of systems was negative for fever, night
sweats, chills, myalgias, weight loss or other constitutional symptoms.
The patient appeared cyanotic and had edema of the face and upper
extremities with engorgement of the superficial neck and chest veins.
There was no palpable lymphadenopathy or organomegaly.
Angiotropic large cell lymphoma (ALCL) of the superior vena cava (SVC)
Venograms of the upper extremities demonstrated filling defects of
the brachiocephalic and the subclavian veins bilaterally and theSVC
proximally. There was collateralization of vessels with reconstitution
of the azygous vein and opacification of the distal SVC (Figure 1). In
light of these findings and the patient’s presenting history and
physical exam, the patient underwent catheter-directed thrombolysis with
urokinase (UK) and concomitant heparin therapy. Follow-up venograms of
both upper extremities at 24 hours demonstrated no significant change in
the pattern and extent of the filling defects when compared with the
previous study (Figure 1). These findings mandated higher consideration
for a primary or metastatic intravascular malignancy.
resonance imaging (MRI) of the chest performed with and without
gadolinium revealed a mass involving the SVC and bilateral
brachiocephalic veins along with expansion of the involved vessels. The
bulk of the lesion, measuring 4.0 × 3.0 × 4.5 cm, was located within the
SVC. The lesion demonstrated intermediate signal intensity on
T1-weighted images (Figure 2) and high signal intensity on T2-weighted
images (Figure 2). Moderate enhancement of the lesion was also
demonstrated postcontrast administration (Figure2). There was no
lymphadenopathy. These findings were most consistent with a neoplastic
process involving mediastinal venous structures. The patient was
surgically explored and the identified tumor was resected.
ALCL is a rare malignancy first described by Pfleger and Tappeiner1 in 1959. It is characterized by the proliferation of large neoplastic lymphocytes2,3
within small and medium-sized blood vessels. Initially, it was believed
that malignant cells in ALCL originated fromthe endothelium of the
involved vessel. However, immuno- histological studies later delineated
their lymphoid origin.3 Most ALCLs in the literature are of B-cell type,3 although cases of T-cell origin4,5 and one case with histiocytic features6
have been described. While vasculature of any organ may be affected,
cutaneous, and central nervous system vessels are most commonly
involved.3,7 Patients usually present with a variety of
clinical features reflective of the diseased organ systems.
Lymphadenopathy and bone marrow involvement are not usually apparent.
While remission may be achieved if treatment is initiated early, the
mortality rate of this aggressive lymphoma, influenced by a usual delay
in diagnosis, remains high.8
The SVCS is typically
associated with malignancies such as lung cancer and lymphoma, which
result in extrinsic compression of the SVC. Benign causes including
intraluminal thrombosis, fibrosis, and infection also exist. Our
patient’s history of present illness was not suggestive of malignancy.
There was no lymphadenopathy, no gross pathology of other visceral
organs, and no constitutional symptoms to suggest primary cancer or
metastases. Furthermore, primary malignant disease of a large vessel,
such as the SVC, is an extraordinarily rare phenomenon. With these
considerations and the initial venogram findings in mind, priority was
given to the possibility of vascularthrombosis over malignancy.
case demonstrates the serendipitous role of thrombolytic therapy in
diagnosing nonthrombotic vascular pathology. Noteworthy is the role that
imaging, along with a basic understanding of the physiological
processes and the pharmacologic agents involved invascular thrombolysis,
played in the timely diagnosis and subsequent treatment and remission
of this rare malignancy.
ALCL is a rare neoplasm. To our knowledge, this is the first imaging
description of ALCL involving a large vessel. Although the imaging
findings are not specific, this case of ALCL serves as a reference for
consideration of other diagnoses in the differential of large-vessel
occlusive disease. While unusual in its incidence, this entity has
strong relevance to the radiologist. Patients with SVCS will benefit
from our specialty having an attentive and current awareness of the
disease pathogenesis, since the radiologist is in the unique position of
being able to identify etiology early in the course of disease,
allowing for prompt treatment that may alter prognosis.
- Pfleger L, Tappeiner J. Zur kenntnis der systemisierten
endotheliomatose der cutanen blutgefasse (reticulo-endotheliose) [On
the recognition of systematized endotheliomatosis of the cutaneous blood
vessels (reticuloenothrliosis)]. [Article in German] Hautarzt. 1959;10:359–363.
A, Lombard C, Donlon T, et al. Immunohistochemical and cytogenetic
studies indicate that malignant angioendotheliomatosis is a primary
intravascular (angiotropic) lymphoma. Cancer. 1990;66:474–479.
MR, Mills SE, Scheithauer BW, et al. Reassessment of malignant
“angioendotheliomatosis.” Evidence in favor of its reclassification as
“intravascular lymphomatosis.” Am J Surg Pathol. 1986;10:112–123.
Setoyama M, Mizoguchi S, Orikawa T, Tashiro M. A case of intravascular
malignant lymphomatosis (angiotropic large cell lymphoma) presenting
memory T cell phenotype and its expression of adhesion molecules. J Dermatol. 1992;19:263–269.
F, Roura M, Umbert I, Umert P. Malignant proliferative
angioendotheliomatosis or angiotropic lymphoma associated with a
soft-tissue lymphoma. J Am Acad Dermatol. 1992;26:101–104.
- Snowden JA, Angel CA, Winfield DA, et al. Angiotropic lymphoma: Report of a case with histiocytic features. J Clin Pathol. 1997;50:67–70.
Sheibani K, Battifora H, Winberg CD, et al. Further evidence that
“malignant angioendotheliomatosis” is an angiotropic large cell
lymphoma. N Eng J Med. 1986;314:943–948.
- Williams DB,
Lyons MK, Yanagihara T, et al. Cerebral angiotropic large cell lymphoma
(neoplastic angioendotheliosis): Therapeutic considerations. J Neurol Sci. 1991;103:16–21.