Spindle Cell Hemangioendothelioma:
Natural History Over 43 Years
Meghan Flood, BSc
, Daniel Mckee, MD
, and Donald Lalonde, MD
In this case study, a 33-year-old male presented initially to our clinic in 1989 with a soft tissue mass on his left posterior chest wall
that had previously been debulked 3 times beginning in 1973. Pathology from these previous excisions confirmed a spindle cell
hemangioendothelioma. Following the previous excisions, the mass recurred and slowly increased in size but did not cause any
serious functional effects. At the patient’s first clinical visit with our plastic surgery team in 1989, we offered radical surgical
resection of the mass, which was the standard of practice at the time. He declined radical resection. We have followed the patient
clinically for over 26 years. Since presentation, the mass has not metastasized or caused any local functional problems.
spindle cell hemangioendothelioma, spindle cell hemangioma, long term follow-up, posterior chest wall, vascular tumour
Spindle cell hemangioendotheliomas, also known as spindle
cell hemangiomas, were first described in 1986 as low-grade
angiosarcomas. These tumors present as non-tender, slow-
growing subcutaneous nodules that commonly recur following
excision. More recently, these tumours have been labelled as
benign vascular tumours. We present the natural history of
1 case over 43 years.
In 1989, a 33-year-old otherwise healthy patient presented to
our clinic with a mass on his left posterior upper back present
since childhood. He had previously gone through 3 surgical
debulking of the mass between 1973 and 1989 before his refer-
ral to plastic surgery. All previous attempts at debulking the
mass led to recurrence. Previous pathology reports indicated
that the tumour was a spindle cell hemangioendothelioma. At
the time of presentation to our clinic, the tumour was non-
tender and did not limit him functionally. The patient did not
have any signs of malignancy.
On examination, the mass measured 10 cm 15 cm. It was
diffuse, firm, non-tender, multilobulated, involved muscle and
was fixed to the chest wall. A computed tomography (CT) scan
revealed a large multinodular lesion infiltrating a large part of
the skin and subcutaneous fat, the latissimus muscle, soft tissue
between the ribs, and the erector spinae muscle. Several
nodules were felt to be recurrences of the original growth.
There was no evidence of distant metastases.
In 1989, spindle cell hemangioendotheliomas were thought
to be a form of soft tissue sarcoma, with a low risk of metas-
tases. Radical resection was suggested for these tumours.
With the patient’s history of multiple recurrences, our plastic
surgery team in conjunction with thoracic surgery felt that a
wide radical excision would be the best course of action. The
patient declined surgery, given recurrence of the tumour was a
In 1995, CT and magnetic resonance imaging (MRI)
showed a modest progression of tumour growth over 6 years.
The nodules abutted the paraspinal muscles and extended lat-
erally through the intercostal muscles at the level of the spleen.
More recent literature suggested no malignant potential for
hemangioendotheliomas supporting continued non-surgical
Dalhousie Medical School, Dalhousie University, Saint John, New Brunswick,
Dr Donald Lalonde, Dalhousie Medical School, Dalhousie University, Suite
C204 600 Main Street, Saint John, New Brunswick, Canada E2K 1J5.
Plastic Surgery Case Studies
ª2017 The Author(s)
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In 2015, a repeat CT and MRI confirmed significant change
from previous imaging and no evidence of metastases or local
invasion suggestive of invasive cancer (Figure 1). The mass
had doubled over 20 years (Figures 2 and 3). The mass contin-
ued to have no functional implications for the patient.
Spindle cell hemangioendotheliomas were first described in
the literature by Weiss and Enzinger in 1986 in a series of
The authors categorized this lesion as a low-grade
angiosarcoma, a vascular neoplasm of borderline malignancy.
These rare painless masses typically presented in the subcuta-
neous tissue and increased slowly over several years. Recur-
rence following excision is common.
In 1991, Fletcher et al published 20 case series and sug-
gested that spindle cell hemangioendotheliomas were more
likely to be benign lesions with surrounding arteriovenous
Each tumour contained dilated vascular spaces with
recent or organized thrombi. Fletcher et al proposed that spin-
dle cell hemangioendotheliomas may be a reactive vascular
lesion associated with a local abnormality of blood flow that
may be congenital or acquired.
Eltorky et al theorized that
spindle cell hemangioendotheliomas were reactive processes
that remained dormant until local disturbances of blood flow
Imayama et al used ultrasound analyses to support
the hypothesis of a benign mass rather than malignant.
and Weiss presented a large case series of 78 spindle cell
hemangioendotheliomas and showed that they do not metasta-
size; however, they recurred commonly following excision.
The term “spindle cell hemangiomas” is now preferred by
some purists as the mass is felt not to have malignant potential.
Despite the changing position on pathology and prognosis, the
presentation of spindle cell hemangioendotheliomas has
remained consistent. Typically, a patient will present with a
solitary mass or multiple non-tender, subcutaneous, well-
Masses present primarily in distal extremi-
Typically, nodules slowly increase in size or number
over the course of years. Pathologically, these nodules are
characterized by large, dilated endothelial-lined vascular
spaces with thin-walled vessels partially filled with organized
thrombi and fascicles of relatively uniform spindle cells.
Recurrence after excision is very common: higher than 50%to
60%in 1 study.
The masses may present at any age, with a
slight male predominance found in 1 study.
We present a long-term case report supporting the conservative
management of spindle cell hemangioendotheliomas. Spindle
cell hemangiomas behave as benign lesions.
Figure 1. Posterior–anterior view of the chest using contrast-
enhanced computerized tomography. There are multiple soft tissue
nodules ranging in size from 1 to 2.5 cm and extending over an area of
12 cm. These include heterogeneous mixed lytic and sclerotic lesions
involving the ribs.
Figure 2. Posterior view of a congenital spindle cell hemangioen-
dothelioma measuring 30 cm 20 cm involving the left posterior
chest wall. Note the additional 2 cm midline vascular nodule.
Figure 3. Posterolateral view of a congenital spindle cell heman-
gioendothelioma involving the left posterior chest wall.
2Plastic Surgery Case Studies
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to
the research, authorship, and/or publication of this article.
The author(s) received no financial support for the research, author-
ship, and/or publication of this article.
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