ARTICLE
Spindle cell haemangioendothelioma (SCH) is a rare vascular neoplasm
first described as a distinct entity by Weiss and Henzinger in 1986 [1].
These authors classified this vascular tumor as a low grade variant of
angiosarcoma, combining features of Kaposi's sarcoma and cavernous haemangioma.
Since then, three other major series [2-4] and occasional case reports
[5-8] have been published. In 1991, Fletcher et al. [3] reported
20 additional cases and suggested that SCH is a non-neoplastic lesion
associated with malformed vasculature at the affected site.
Here we report a new case of SCH and review the main clinical and histopathological
features of this rare lesion. Differential diagnoses with respect to other
vascular tumors are discussed.
Case report
A 48-year-old man was referred to us because of reddish-purple, angiomatoid
nodules and plaques in the dorsal region and on the sole of the right
foot, especially on and between the second, third and fourth toes (Fig.
1). The lesions had an 8-year history and had recently increased
in size, with some local discomfort.
Routine blood chemistry, including the helper/suppressor ratio, was
normal. HIV test was negative.
Histological examination of a biopsy specimen
(Fig. 2 and
3) showed vascular lacunae, dilated to different degrees,
and cordons of solid cells consisting mainly of a component resembling
spindle cells. A lymphoplasma cell infiltrate was evident. The vascular
lacunae were sometimes associated with elongated papillary structures,
consisting of an axis of fibroconnective tissue, usually with few cells,
covered by endothelial type elements, often swollen and protruding into
the vessels. Sometimes the cell cordons consisted of cells of histiocytoid
appearance. Some cells were intensely vacuolized but no cell atypia or
mitotic figures were observed. Immunohistochemical studies with monoclonal
antibody CD34, specific for mature endothelial cells, showed labelling
of the cells lining the vascular spaces and coating the papillary formations
(Fig. 4). Slight focal
positivity for factor VIII-related antigen and Ulex europaeus lectin
I was found.
Discussion
SCH is a rare, vascular neoplasm, characterized by the slow but progressive
growth of single or multiple, painless, reddish blue nodules in the dermis
and subcutis of the distal extremities. They may occur at any age, in
either sex [2, 3].
The lesions increase in size or number over a period of several months
to many years. The patients with multiple nodules tend to develop new
lesions in the same anatomical area over many years. These new lesions
are not true recurrences, because they arise in previously unaffected
skin. To date, only one case has been reported as progressing to metastases
and this was after various recurrences, local irradiation and histological
transformation to conventional angiosarcoma. This exceptional case may
also be considered a radiation-induced sarcoma [1].
SCH usually arises in association with a local
abnormality of blood flow in the affected site, whether congenital or
acquired. Some authors have therefore suggested [3] that SCH is a non-neoplastic,
benign, reactive, vascular lesion rather than a true, low grade endothelial
neoplasm or a borderline malignancy, as it is currently regarded.
Histologically [2-4], all lesions are localized either in the dermis
or superficial subcutis and are rarely ulcerated. There are not generally
any significant epidermal changes. Each lesion consists predominantly
of a mixture of thin-walled, variably dilated vascular spaces and areas
of solid spindle cells. The dilated spaces often contain recent or organized,
sometimes calcified thrombi and are often associated with elongated, papillary
structures, composed of an acellular fibrous core covered by rather plump,
monomorphic endothelial cells. The solid areas are composed principally
of cells with poorly defined, eosinophilic cytoplasm which have either
spindle-shaped nuclei, or plump, rounded vesicular nuclei. These cells
are usually uniform in appearance, lack nuclear hyperchromasia and generally
show no mitotic activity. Moreover, numerous slit-like spaces, often filled
with red blood cells, are evident in these solid spindle-celled areas.
Other peculiar histological findings of SCH include perivascular bundles
of smooth muscle cells and large, malformed thick or thin walled vessels
reminiscent of an arterio-venous malformation.
The vascular nature of SCH is demonstrated immunohistochemically by
a positive reaction for factor VIII-related antigen, CD34, CD3 1 and Ulex
europaeus lectin I and, ultrastructurally, by the presence of Weibel-Palade
bodies in the cytoplasm of the spindle and epithelioid cells.
Histological differential diagnoses [2-4] must be made with respect
to cavernous haemangioma, arteriovenous haemangioma and Kaposi's sarcoma.
The first two conditions do not have a prominent spindle cell proliferation.
Kaposi's sarcoma does not have cavernous vascular spaces, with multiple
thrombi, epithelioid endothelial cells and an absence of cellular atypia.
Other conditions that usually need to be distinguished from SCH are histiocytoid
haemangioma, bacillary angiomatosis, angiosarcoma and intravascular, papillary,
endothelial hyperplasia (Masson's tumour). The similarity between SCH
and the latter entity can be striking; however, the unusual location of
SCH in an extravascular site, its frequent multiplicity and the presence
of histiocytoid endothelial cells should help distinguish between the
two.
Regarding treatment, complete surgical excision is indicated for localized,
solitary lesions and close follow-up is also advisable, since local recurrences
may occur after several months or years. The benefits of a complementary
treatment with chemotherapy or radiation therapy are not well established.
Moreover, these treatments could be unnecessary or even dangerous.
REFERENCES
1. Weiss SW, Enzinger FM. Spinde cell haemangioendothelioma. A low grade
angiosarcoma resembling a cavernous hemangioma and Kaposi's sarcoma. Am
J Surg Pathol 1986; 10: 521-30.
2. Scott GA, Rosai J. Spindle cell haemangioendothelioma. Report of
seven additional cases of a recently described vascular neoplasm. Am
J Dermatopathol 1988; 10: 282-8.
3. Fletcher CDM, Beham A, Schmid C. Spindle cell haemangioendothelioma:
a clinicopathological and immunohistochemical study indicative of a non-neoplastic
lesion. Histopathology 1991; 18: 291-301.
4. Ding J, Hashimoto H, Imayama S, Tsuneyoshi M, Enjoji M. Spindle cell
haemangioendothelioma: probably a benign vascular lesion not a low grade
angiosarcoma. A clinicopathological, ultrastructural and immunohistochemical
study. Virchows Archiv A Pathol Anat 1992; 420: 77-85.
5. Lessard M, Barnhill L. Spindle cell haemangioendothelioma of the
skin. J Am Acad Dermatol 1988; 18: 393-5.
6. Bruscagin C, Betti R, Perotta E, Palvarini M. Emangioendotelioma
a cellule fusate. G Ital Dermatol Venereol 1994; 129: 113-8.
7. Lawson JP, Scott G. Spindle cell haemangioendothelioma and enchondromatosis
(a form of Maffucci's syndrome) in a patient with acute myelocytic leukemia.
Case report 602. Skeletal Radiol 1990; 19: 158-62.
8. Steinbach LS, Ominsky SH, Shpall S, Perkocha LA. MR imaging of spindle
cell haemangioendothelioma. J Comput Assist Tomogr 1991; 15: 155-7.
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