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Spindle Cell Hemangioendothelioma: Natural History Over 43 Years

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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.
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Case Report
Spindle Cell Hemangioendothelioma:
Natural History Over 43 Years
Meghan Flood, BSc
1
, Daniel Mckee, MD
1
, and Donald Lalonde, MD
1
Abstract
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.
Keywords
spindle cell hemangioendothelioma, spindle cell hemangioma, long term follow-up, posterior chest wall, vascular tumour
Introduction
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.
Case Report
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
strong possibility.
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
management.
1,2
1
Dalhousie Medical School, Dalhousie University, Saint John, New Brunswick,
Canada
Corresponding Author:
Dr Donald Lalonde, Dalhousie Medical School, Dalhousie University, Suite
C204 600 Main Street, Saint John, New Brunswick, Canada E2K 1J5.
Email: dlalonde@drlalonde.ca
Plastic Surgery Case Studies
1-3
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.
Discussion
Spindle cell hemangioendotheliomas were first described in
the literature by Weiss and Enzinger in 1986 in a series of
26 cases.
3
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
shunts.
1
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.
1
Eltorky et al theorized that
spindle cell hemangioendotheliomas were reactive processes
that remained dormant until local disturbances of blood flow
developed.
2
Imayama et al used ultrasound analyses to support
the hypothesis of a benign mass rather than malignant.
4
Perkins
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.
5
The term “spindle cell hemangiomas” is now preferred by
some purists as the mass is felt not to have malignant potential.
6
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-
defined nodules.
3,6
Masses present primarily in distal extremi-
ties.
3,6,7
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.
1,2,4
Recurrence after excision is very common: higher than 50%to
60%in 1 study.
7
The masses may present at any age, with a
slight male predominance found in 1 study.
4
Conclusion
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.
Funding
The author(s) received no financial support for the research, author-
ship, and/or publication of this article.
References
1. Fletcher CD, Beham A, Schmid C. Spindle cell haemangioen-
dothelioma: a clinicopathological and immunohistochemical study
indicative of a non-neoplastic lesion. Histopathology. 1991;18(4):
291-301.
2. Eltorky M, McC Chesney T, Sebes J, Hall JC. Spindle cell heman-
gioendothelioma. Report of three cases and review of the literature.
J Dermatol Surg Oncol. 1994;20(3):196-202.
3. Weiss SW, Enzinger FM. Spindle cell hemangioendothelioma.
A low-grade angiosarcoma resembling a cavernous heman-
gioma and Kaposi’s sarcoma. Am J Surg Pathol. 1986;10(8):
521-530.
4. Imayama S, Murakamai Y, Hashimoto H, Hori Y. Spindle cell
hemangioendothelioma exhibits the ultrastructural features of
reactive vascular proliferation rather than of angiosarcoma. Am J
Clin Pathol. 1992;97(2):279-287.
5. Perkins P, Weiss SW. Spindle cell hemangioendothelioma. An
analysis of 78 cases with reassessment of its pathogenesis and
biologic behaviour. Am J Surg Pathol. 1996;20(10):1196-1204.
6. Requena L, Kutzner H. Hemangioendothelioma. Semin Diagn
Pathol. 2013;30(1):29-44.
7. Gray SS, Eltorky MA, Riascos RF, Montilla RD. Spindle cell
hemangioma reoccurrence in the hand: case report. Hand (N Y).
2012;7(2):194-199. doi:10.1007/s11552-012-9397-1.
Flood et al 3
ResearchGate has not been able to resolve any citations for this publication.
Article
Seventy-eight cases of spindle cell hemangioendothelioma (SCH) were studied to reevaluate its pathogenesis and determine its long-term behavior. Most of the original findings were confirmed by this study. The tumor occurred at all ages (8-78 years; median, 32 years; mean, 34 years). Males and females were equally affected. The tumor developed as a superficially located mass of the distal extremities (upper, 32 cases; lower, 30 cases). Four patients (5%) also had Maffucci's syndrome. The lesions were circumscribed red-brown masses occasionally containing phleboliths that consisted of cavernous blood spaces alternating with cellular areas consisting of collapsed vascular spaces separated by spindled fibroblastic cells. Often the endothelium lining the collapsed blood spaces appeared epithelioid with cytoplasmic vacuolization. The spindled fibroblastic cells lacked significant atypia and had at most a low level of mitotic activity. As a significant departure from what was originally reported, more than half of these cases (58%) were partially or completely intravascular. The vein containing the tumor often had an irregularly attenuated wall with small intimal herniations and intimal papillae traversing the lumen. Similar intimal changes in adjacent vessels suggest that SCH grows as a multifocal or contiguous process within vessels. Follow-up information was obtained in 40 cases, ranging from 1 month to 40 years (mean, 5.4 years). Despite conservative excisions in most patients (simple excision, 83%; wide local excision, 13%; amputation, 2%), prognosis was excellent. Fifty-eight percent experienced recurrences, but no patient developed metastasis and no patient died of the direct effects of the tumor, although one patient with Maffucci's syndrome developed a concurrent angiosarcoma. We conclude that SCH is a primary benign vascular neoplasm or malformation similar to angiomatosis in which alterations in blood flow might explain some of the secondary features. Areas of diminished blood flow result in vascular collapse with formation of the “cellular” zones, and areas of vascular engorgement with stasis promote thrombosis and organization. Local “recurrences” probably represent contiguous spread along or multifocal involvement of a vessel. Because there is no evidence that this lesion has metastatic potential, we suggest that the lesion be designated spindle cell hemangioma for solitary lesions and spindle cell hemangiomatosis for multifocal lesions.
Article
background. Spindle cell hemangioendothelioma lesions are uncommon, affect a wide age range in both sexes, and show a predilection for skin and subcutaneous tissue of the extremities. objective. To present three cases of spindle cell hemangioendothelioma and review the literature. methods. Three cases of spindle cell hemangioendothelioma are presented. results. Two of our cases first presented very early in life and progressed by local recurrences over many years. None of our cases showed evidence of metastases, but the lesions exhibited local aggressive and invasive behavior. Histologically, the lesions consist of alternating areas of dilated, thin walled cavernous vascular spaces and solid areas composed predominantly of spindle cells and clusters of epithelioid endothelial cells with intracytoplasmic vacuoles. That spindle cell hemangioendothelioma is a non-neoplastic lesion and not a neoplasm of borderline malignancy is suggested by the following observations from our cases: the repeated presence of organized intravascular thrombi in all cases, the early clinical presentation in two cases, and the presence of some degree of vascular malformation at the periphery of lesions. conclusion. Both clinicians and pathologists should be aware of the existence of this lesion in order to diagnose and treat an affected patient correctly.
Article
Spindle cell hemangioma (SCH) was first described by Weiss and Enzinger in 1986 [44]. They presented the initial 26 cases and at the time concluded that the vascular lesion was a vascular tumor of low-grade malignancy, and therefore, they termed the lesion a spindle cell hemangioendothelioma. Since then, various investigations have been conducted, including immunohistochemical studies, revealing they are benign lesions [4, 13, 14]. These lesions affect both sexes equally and have a predilection for the extremities. There are about 186 cases of SCH reported in the English literature, and 40 of the cases involved the hand [1–11, 13–26, 28–30, 32–47]. SCH can present as a solitary lesion (spindle cell hemangioma) or multifocal lesions (spindle cell hemangiomatosis) [33]; both types have been found in the hand. Lesions tend to develop during childhood or early adulthood. They most often have an indolent coarse [9, 13], but can become painful and disfiguring [8, 17]. Most lesions occur in the dermis and subcutaneous tissues, but have been found in the viscera, spinal cord, cervix, and oral cavity [26, 36, 40, 41, 43]. Spindle cell hemangiomas have features of both cavernous hemangiomas and Kaposi's sarcoma. Microscopically, this vascular lesion consists of vascular proliferations lined by a thin layer of endothelium within solid areas of mostly spindle cells [6]. There is minimal or no mitosis. The percentage of cavernous areas versus solid areas varies greatly [10, 14]. Thrombi or phleboliths can be found within these cavernous spaces [14, 40] and can be evident in radiographs [8]. The standard of care is wide local excision, but caution must be taken when there is multifocal disease not to cause further disability. Additionally, the reoccurrence rate is greater than 50% [33]. We present a case of a female patient who had a local reoccurrence of SCH in her hand 14 years after the original lesion was excised.
Article
Hemangioendothelioma is the term used to name those vascular neoplasms that show a borderline biological behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. Although originally spindle cell hemangioendothelioma was proposed as a specific clinicopathologic variant of hemangioendothelioma, currently, it is considered as an entirely benign lesion, and thus, the name spindle cell hemangioma seems to be the most accurate for this lesion. Authentic hemangioendotheliomas involving the skin and soft tissues include papillary intralymphatic angioendothelioma (also known as Dabska tumor), retiform hemangioendothelioma, kaposiform hemangioendothelioma, epithelioid hemangioendothelioma, pseudomyogenic hemangioendothelioma (also known as epithelioid sarcoma-like hemangioendothelioma), and composite hemangioendothelioma. Each of these neoplasms exhibit characteristic histopathologic features. The most characteristic finding of papillary intralymphatic hemangioendothelioma consists of papillary tufts, with a central hyaline core lined by hobnail-like endothelial cells protruding into the lumina. Retiform hemangioendothelioma is an infiltrative neoplasm composed of elongated arborizing vessels, arranged in an anastomosing pattern that resembles that of the rete testis, and lined by a single layer of hobnail-like endothelial cells that protrude within the narrow lumina. Kaposiform hemangioendothelioma is composed of several solid poorly circumscribed nodules, and each nodule is composed of a mixture of small capillaries and solid lobules of endothelial cells arranged in a glomeruloid pattern. A frequent finding consists of the presence of areas of lymphangiomatosis adjacent to the solid nodules. Epithelioid hemangioendothelioma is composed of cords, strands, and solid aggregates of round, oval, and polygonal cells, with abundant pale eosinophilic cytoplasm, vesicular nuclei, and inconspicuous nucleoli, embedded in a fibromyxoid or sclerotic stroma. Many neoplastic cells exhibit prominent cytoplasmic vacuolization as an expression of primitive vascular differentiation. Pseudomyogenic hemangioendothelioma is a poorly circumscribed, fascicular lesion with infiltrative borders composed of round or oval neoplastic cells, with vesicular nuclei and inconspicuous nucleoli, and ample homogeneous eosinophilic cytoplasm, giving them a rhabdomyoblastic appearance. Finally, composite hemangioendothelioma is the term used to name locally aggressive vascular neoplasms of low-grade malignancy showing varying combinations of benign, low-grade malignant, and high-grade malignant vascular components. From the immunohistochemical point of view, proliferating cells of all hemangioendotheliomas express a lymphatic endothelial cell immunophenotype. Most hemangioendotheliomas are low-grade vascular neoplasms, with a tendency to recur locally and a low metastatic potential, mostly to regional lymph nodes. Epithelioid hemangioendothelioma, especially large lesions and those located in deep soft tissues, seems to have a more aggressive biological behavior.
Article
A patient with spindle cell hemangioendotheliomas was followed from 1964 to the present time, allowing the authors the opportunity to examine the lesions in the early, mature, and old phases. Organizing thrombi of different stages associated with slit-like vascular proliferation were always observed, whereas cavernous vascular spaces predominated as the lesions became older. Each spindle cell hemangioendothelioma initially developed relatively rapidly and was sometimes painful but then persisted as a silent nodule for decades. Transmission and scanning electron microscopic studies revealed that endothelial cells tended to digitate into the slit-like proliferating channels, became attached to other cells by means of tight junctions, and thus obstructed the channels at sites where thrombi developed repeatedly. The vascular spaces, ranging in nature from slit-like to cavernous, were outlined further by a relatively sparse mantle of ramified or dendritic interstitial cells that corresponded to spindle cells. Most of the cells appeared simply to be fibroblasts, but they developed the features of pericytes when they were close to the endothelial lining of well-developed vascular lumens. Large vascular spaces and phleboliths were surrounded by smooth muscle cells. Approximately 20% of the interstitial cells were dendritic macrophages characterized by phagocytic activity, presence of many lysosomes, and Factor XIIIa expression. The long and characteristic clinical course, the histologic evidence that thrombosis and its organization was continually occurring within the lesions, and the ultrastructural finding that spindle cell hemangioendotheliomas were composed of different microvascular segments from capillaries to veins, suggest that spindle cell hemangioendotheliomas may develop from a cycle of recanalization after thrombosis that occurs repeatedly because of the unique endothelial growth that was noted. This is in contrast with the previous conception that they were low-grade angiosarcomas.
Article
Spindle cell haemangioendothelioma is an uncommon vascular lesion, of which 35 cases have been previously reported. A further 20 cases are reported herein. These lesions affected a wide age range in both sexes, and showed a predilection for the extremities. Twelve patients had multiple lesions and, in these cases, local progression over many years but no true recurrence was typical. None of the 20 patients developed metastases. but one later developed an angiosarcoma. Individual cases were associated with congenital lymphoedema, Klippel-Trenaunay syndrome and early-onset varicose veins. Histologically, in addition to the admixture of cavernous spaces and solid spindle cell/epithelioid cell areas, the presence both of irregularly distributed and perivascular smooth muscle cells and of malformed variably-sized vessels at the periphery of almost every lesion was noted in each case. Reticulin staining, immunohistochemistry and electronmicroscopy revealed the presence of primitive vessel formation and partial endothelial differentiation in the solid spindle cell areas. Combining these data with those from previously published series, it is suggested that spindle cell haemangioendothelioma is a non-neoplastic lesion (rather than a borderline malignancy as it is currently regarded) and that its development correlates with histological and/or clinical evidence of a malformed vasculature at the affected site.
Article
Twenty-six cases of a newly recognized form of vascular tumor are presented. The tumor may occur at any age, has a male predominance, and develops preferentially in the dermis and subcutaneous tissues of the distal extremities. Histologically it combines the features of both a cavernous hemangioma and Kaposi's sarcoma. It is composed of thin-walled cavernous vessels which may be dilated, partially collapsed, or filled with organizing thrombi and phleboliths. These areas are intimately associated with spindled areas reminiscent of Kaposi's sarcoma. The spindled areas differ from Kaposi's sarcoma by the presence of occasional epithelioid endothelial cells, which sometimes display vacuolization. Follow-up information in 14 cases indicates that nine patients experienced "recurrences." One patient, who also received radiotherapy, developed regional lymph node metastasis 40 years after diagnosis and following 19 recurrences. No patient, however, has died of his disease, despite relatively limited surgical excision. The term "spindle cell hemangioendothelioma" is suggested for this vascular tumor of low-grade malignancy.
Article
Spindle cell hemangioendothelioma lesions are uncommon, affect a wide age range in both sexes, and show a predilection for skin and subcutaneous tissue of the extremities. To present three cases of spindle cell hemangioendothelioma and review the literature. Three cases of spindle cell hemangioendothelioma are presented. Two of our cases first presented very early in life and progressed by local recurrences over many years. None of our cases showed evidence of metastases, but the lesions exhibited local aggressive and invasive behavior. Histologically, the lesions consist of alternating areas of dilated, thin walled cavernous vascular spaces and solid areas composed predominantly of spindle cells and clusters of epithelioid endothelial cells with intracytoplasmic vacuoles. That spindle cell hemangioendothelioma is a non-neoplastic lesion and not a neoplasm of borderline malignancy is suggested by the following observations from our cases: the repeated presence of organized intravascular thrombi in all cases, the early clinical presentation in two cases, and the presence of some degree of vascular malformation at the periphery of lesions. Both clinicians and pathologists should be aware of the existence of this lesion in order to diagnose and treat an affected patient correctly.
Article
Seventy-eight cases of spindle cell hemangioendothelioma (SCH) were studied to reevaluate its pathogenesis and determine its long-term behavior. Most of the original findings were confirmed by this study. The tumor occurred at all ages (8-78 years; median, 32 years; mean, 34 years). Males and females were equally affected. The tumor developed as a superficially located mass of the distal extremities (upper, 32 cases; lower, 30 cases). Four patients (5%) also had Maffucci's syndrome. The lesions were circumscribed red-brown masses occasionally containing phleboliths that consisted of cavernous blood spaces alternating with cellular areas consisting of collapsed vascular spaces separated by spindled fibroblastic cells. Often the endothelium lining the collapsed blood spaces appeared epithelioid with cytoplasmic vacuolization. The spindled fibroblastic cells lacked significant atypia and had at most a low level of mitotic activity. As a significant departure from what was originally reported, more than half of these cases (58%) were partially or completely intravascular. The vein containing the tumor often had an irregularly attenuated wall with small intimal herniations and intimal papillae traversing the lumen. Similar intimal changes in adjacent vessels suggest that SCH grows as a multifocal or contiguous process within vessels. Follow-up information was obtained in 40 cases, ranging from 1 month to 40 years (mean, 5.4 years). Despite conservative excisions in most patients (simple excision, 83%; wide local excision, 13%; amputation, 2%), prognosis was excellent. Fifty-eight percent experienced recurrences, but no patient developed metastasis and no patient died of the direct effects of the tumor, although one patient with Maffucci's syndrome developed a concurrent angiosarcoma. We conclude that SCH is a primary benign vascular neoplasm or malformation similar to angiomatosis in which alterations in blood flow might explain some of the secondary features. Areas of diminished blood flow result in vascular collapse with formation of the "cellular" zones, and areas of vascular engorgement with stasis promote thrombosis and organization. Local "recurrences" probably represent contiguous spread along or multifocal involvement of a vessel. Because there is no evidence that this lesion has metastatic potential, we suggest that the lesion be designated spindle cell hemangioma for solitary lesions and spindle cell hemangiomatosis for multifocal lesions.