Angiosarcoma of the Superior Vena Cava

R. p. ABRATT. MMED,' M. WILLIAMS, FRCSE,t M. RAFF, DCP,* N. F. DODD, MBCHB,~AND C. J. UYS, MDll

The first reported case of an angiosarcoma apparently arising from the superior vena cava is presented.
The patient, a 20-year-old white man, was treated by surgical excision and reconstruction with dacron
grafts from the left and right brachiocephalic veins to the right atrium. This was followed by a course
of mediastinal irradiation. The patient remained clinically disease-free at 24 months post-treatment.
The patient had occasional occupational exposure to polyvinyl chloride.
Cancer 52:740-743, 1983.

A NGIOSARCOMA are rare malignant tumors of vas-

cular origin. A case is presented of a patient with
an angiosarcoma which arose from the superior vena
The patient was subsequently treated by means of a course of
mediastinal irradiation with cobalt 60 using anterior and pos-
terior parallel opposed portals. A tumor dose of 5400 rad (54
cava and the treatment is discussed. The patient had Gy) was administered over 38 days in 200 rad (2 Gy) daily
occasional occupational exposure to polyvinyl chloride. fractions. A 1 cm posterior midline lead shield was used after
3000 rad (3 Gy) to protect the spinal cord.
The radiotherapy was well tolerated and the patient has
Case Report remained well with no clinical or radiologic evidence of disease
A 20-year-old white man presented with the complaint of for 24 months post-treatment.
intermittent right-sided chest pain. He had not lost weight
recently, had a good appetite and was a nonsmoker. He was Pathologic Findings
employed as an artisan in the engineering department of a
chemical company. A 7 X 5 X 4 cm mass was submitted for microscopic
The patient's clinical examination was noncontributory. A study. The tissue was ragged and dark red with areas of
chest x-ray showed a mass situated in the right anterior me- yellow and grey towards the periphery of the lesion.
diastinum (Fig. I ) . Routine investigations including bron- Histologic section revealed fibroadipose tissue which
choscopy were negative. was extensively replaced and infiltrated by a malignant
A right anterolateral thoracotomy through the bed of the vascular tumor. The architecture of the lesion was vari-
5th rib was made. A 7 cm mediastinal mass was present that able. Parts showed an angiomatous pattern consisting
was inseparable from and apparently arising from the superior
vena cava. The tumor was resected, including the superior
vena cava, from its junction with the right atrium to the junc-
tion of the left and right brachiocephalic veins. Clearance was
by a margin of approximately 1 cm inferiorly and few milli-
meters superiorly. Reconstruction was by means of separate
dacron grafts from the left and right brachiocephalic veins to
the right atrium.
The postoperative course was uneventful. A liver scan and
liver function tests were performed and found to be normal.

* Radiotherapist, Department Radiotherapy, Provincial Hospital,

Port Elizabeth, South Africa.
t Thoracic Surgeon, Port Elizabeth, South Africa.
Pathologist, Port Elizabeth. South Africa.
0 General Practitioner, Uitenhage. South Africa.
11 Professor of Pathology, University of Cape Town. Cape Town,
South Africa.
Address for reprints: R. P. Abratt. MMed, Department of Radio-
therapy, Provincial Hospital, Port Elizabeth, South Africa.
The authors thank Dr. L. Cilliers, Medical Superintendent, Provin-
cial Hospital, Port Elizabeth and Professor Sealy, Head of Department
Radiotherapy, Groote Schuur, Cape Town for permission to publish. FIG. I . X-ray chest at presentation revealing a mass situated in the
Accepted for publication May 2 I , 1982. right mediastinum.

I5/0740$ I .OO 0 American Cancer Society

0008-543>(/83/08

740
No. 4 -
ANGIOSARCOMA Abratt et ul. 74 1

FIGS. % A N D 2B. (A) Angiosarcoma

presenting as cords of atypical endo-
thelial cells ( H & E, X275). (B) An-
giosarcoma showing luminated struc-
tures containing erythrocytes that are
lined by atypical vascular endothelial
cells (H& E, X275).

of anastomosing vascular channels lined by an atypical overall morphologic features were those of angiosar-
endothelium (Fig. 2A). The endothelial lining showed coma.
crowding of cells in foci resulting in intraluminal buds. A careful evaluation of the patient’s occupational his-
The lumina were mainly empty; some, however, con- tory was then undertaken. Polyvinyl chloride was being
tained clusters of erythrocytes (Fig. 2B). The cells lining processed in the chemical plant where the patient
the lumina were round, polygonal, and fusiform. The worked, but he was not stationed in that section. He
nuclei exhibited moderate pleomorphism, occasional did, however, visit that area occasionally to adjust elec-
multinucleated forms being observed. A number of nu- trical timers. A maximum time of 20 hours was spent
clei contained prominent nucleoli. Mitoses were mild there covering the period from the commencement of
in number. Other parts were composed mainly of spin- his employment 33 months prior to his medical presen-
dle cells that were arranged in more compact bundles. tation. The vinyl chloride monomer in the polyvinyl
Their cell outlines were indistinct and vacuolation was
seen. Early clefting of the stroma was present. Hemo-
siderin deposition was noted together with evidence of
fibrosis and hyalinization. Special stains for reticulin
confirmed the presence of tumor cells within the retic-
ulin sheath (Fig. 3). No other mesenchymal elements or
epithelial cells were noted. Mucin stains were negative.
Histochemical staining of these cells for Factor VlII re-
lated antigen by the PRP method was positive.
A moderate, mainly chronic inflammatory reaction
in which plasma cells were prominent, was observed in
the vicinity of the tumor.
Electron microscopic examination of formalin-fixed
tissue showed the presence of clusters of neoplastic cells
sometimes arranged around a central lumen (Fig. 4).
These cells had large convolted nuclei with prominent
nucleoli. Organelleswere scanty, but rough endoplasmic
reticulin was observed. However, a distinctive feature
indicative of vascular origin was the presence of elon-
gated, fusiform electron dense bodies which were typical
of Weibel-Polade bodies and thus indicative that these FIG. 3. Reticulin stains reveal the presence of tumor cells within
cells were of vascular endotherlial origin (Fig. 5). The reticulin sheaths (X400).
742 CANCERAugust IS 1983 Vol. 52

have been reported. The average patient age at presen-

tation is 41 years and 1 1 cases have been reported in
patients less than 25
In view of the surgical findings of this lesion being
intimately related to the superior vena cava, the reason-
able assumption is made that this tumor was a primary
angiosarcoma of the superior vena cava. N o other tumor
mass was found at other sites. It is of interest that the
superior vena cava and the right atrium have a common
embryology. To the best of the author's knowledge, this
is the first case of angiosarcoma to be reported that was
apparently arising from the superior vena cava.
Four attempts at total surgical excision have been re-
ported in the management of cardiac angiosarcomas. In
the cases reported by Hollingswoth and Sturgil16 and
Poole-Wilson et ul..' the tumor arose from the pericar-
FIG.4. EM shows tumor cells forming luminated vascular channels.
The nuclei are large and crenated and have prominent nucleoli. Apart
dium and postoperative irradiation was administered.
from RER few cytoplasmic organelles are present (X4500). In addition, cytotoxics were administered in the first
case. The patients were reported as being well at 10 and
6 months, respectively. Hager and associates' reported
chloride product was being monitored on a monthly a case where the tumour was removed from the left
basis, as well as the vinyl chloride monomer level in the atrium with extracorporeal circulation and who has re-
working environment. These were low in all recorded mained disease-free for 3 years. Rossi d u I . successfully
~
samples and varied from 0 to 1 , 87 parts per million. excised a tumor from the right atrium using cardiac
pulmonary bypass, but the patient died 6 months later
Discussion from metastases.
Angiosarcoma can develop in a wide variety of sites Radiotherapy has a contributory role to play in the
including viscera, bones and soft tissues.' The tumor's treatment of angiosarcoma. Allaire and coworkers' have
occurrence in the heart, has been well documented. In reported a case of cardiac angiosarcoma diagnosed at
their comprehensive review of 56 cases, Grontoft and thoracotomy that was subsequently treated with radio-
Hellquist' found that the tumor almost exclusively in- therapy alone. A good local result was obtained, and the
volved the right atrium, although cases with involve- cardiac silhouette returned to near normal. The patient
ment of the pericardium, right ventricle and left atrium died 6 months postsurgery with metastases.
Bricklin and Rushton' has reported a case where the
tumor was dissected free by microsurgery from the radial
nerve. Postoperative radiation to a dose of 6000 rad (60
Gy) was given, and the patient remained tumor-free at
36 months. Lindberg et ul." have reported local recur-
rence in one of four patients treated by conservative
surgery and postoperative irradiation. Karmody and
Kim" have reported a patient treated for angiosarcoma
of the premaxilla using irradiation alone. This patient
has remained free of disease at 40 months post-treat-
ment.
The etiologic role of vinyl chloride monomer in an-
giosarcoma has been recognized since 1974." There is
no evidence that polyvinyl chloride itself is carcinogenic
or that it depolymerises to produce vinyl chloride mono-
mer. Unreacted vinyl chloride may be present i n newly
manufactured polyvinyl chloride and may be released
during storage and when the material is heated during
FIG. 5. EM shows clusters of tumor cells in which Weibel-Polade manufacturing. I'
bodies are present in cytoplasm (arrows) indicating vascular endothe-
lial origin (X7500). Inset: Cytoplasmic Weibel-Polade bodies enlarged Patients previously reported with angiosarcoma of the
( X 2 5.000). liver had been exposed to vinyl chloride monomers in
No. 4 ANGIOSARCOMA * Ahrutt et ul. 743

their working environment to doses as high as 1000 parts 3. Allaire FJ, Grimm CA, Taylor LM, Pfaff JP. Primary heman-
gioendothelioma of the heart. Rocky MI Med J 1964; 6 134-37.
per million in the 1950s, had a mean latent interval of 4. Lin TK, Stech JM, Eckert WG, Lin JJ, Farha SJ, Hagan CT.
about 20 years and a mean duration of exposure of 18 Pericardial angiosarcoma simulating pericardial effusion, by echocar-
years.I4 In contrast, the patient reported here had had diography. Chest 1978; 73981-883.
5. Rossi NP, Kioschos JM, Aschenbrener CA, Ehrenhaft JL. Pri-
an intermittent exposure totaling 20 hours over a period mary angiosarcoma of the heart. Cunccv 1976; 37:89 1-894.
of 33 months. The concentration of vinyl chloride 6. Hollingswoth JH, Sturgill BC. Treatment of primary angiosar-
monomer in the working environment was very low. coma of the heart. Am Ileurf J 1969; 78:254-258.
7. Poole-Wilson PA, Farnsworth A, Braimhridge MV, Pambakian
The authors do not consider that vinyl chloride mono- H. Angiosarcoma of pericardium: Problems in diagnosis and man-
mer was definitely an etiologic factor in this case, but agement. Br Ilmrt J 1976; 38:240-243.
it is reported for documentation purposes. 8. Hager W, Kremer K, Muller W. Angiosarcoma. Ilerzen.c-D.rfch
Med Wochenschr 1970; 95:680-684.
9. Bricklin AS, Rushton HW. Angiosarcoma of venous origin aris-
ADDENDUM ing in radial nerve. Cunccr 1977; 39:1556-1558.
10. Lindberg RD, Martin RG, Romsdahl MM, Markley HT. Con-
Patient remains clinically and radiologically clear of disease at 40 servative surgery and post operative radiotherapy in 300 adults with
months posttreatment. soft tissue sarcomas. Cuncer I98 1 ; 47:239 1-2397.
I I . Karmody CS, Kim CH. Angiosarcoma of the premaxilla. Lu-
ryngoscope 1914; 84560-564.
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