J Cutan Pathol 2007: 34: 797–800doi: 10.1111/j.1600-0560.2006.00708.xBlackwell Munksgaard. Printed in Singapore

Copyright # Blackwell Munksgaard 2006

Journal of

Cutaneous Pathology

Spindle cell hemangioma: report ofa case presenting in the oral cavity

Background: Spindle cell hemangioma, formerly termed spindlecell hemangioendothelioma, is an uncommon benign vascular tumor.Presentation in the oral cavity is rare with only two previously reportedcases.Methods: We report a case of spindle cell hemangioma thatpresented as an asymptomatic mass of the buccal mucosa in a44-year-old man.Results: The tumor was a well-circumscribed vascular proliferationof spindled to epithelioid endothelial cells. Tumor cells withintracytoplasmic vascular lumens were present in several areas. Larger,ectatic, thin-walled vessels lined by a single layer of flattenedendothelial cells were evident at the periphery and focally in morecellular areas. The tumor was positive for CD31 and CD34.Conclusions: Spindle cell hemangioma rarely presents in the oralcavity and needs to be considered in the differential diagnosis of oralcavity vascular tumors to avoid misdiagnosis as a more aggressivevascular tumor.

Sheehan M, Roumpf SO, Summerlin D-J, Billings SD. Spindle cellhemangioma: report of a case presenting in the oral cavity.J Cutan Pathol 2007; 34: 797–800. # Blackwell Munksgaard 2006.

Michael Sheehan1, Sheila O.Roumpf1, Don-John Summerlin1

and Steven D. Billings1,2

1Department of Pathology and LaboratoryMedicine and2Department of Dermatology, IndianaUniversity School of Medicine, Indianapolis,IN, USA

Steven D. Billings, MD, Departments of Pathologyand Laboratory Medicine and Dermatology, IndianaUniversity School of Medicine, 350 West 11thStreet, Room 4018, Indianapolis, IN 46202-4108,USATel: (317) 491-6489Fax: (317) 491-6424e-mail: mpsheeha@iupui.edu

Accepted for publication November 3, 2006

Spindle cell hemangioma is a benign vascular tumorthat typically occurs in acral locations.1–4 Pre-sentation in the oral cavity is quite rare and apotential source for misdiagnosis of this lesion as amore aggressive vascular tumor such as epithelioidhemangioendothelioma, Kaposi sarcoma or angio-sarcoma.5,6

Case report

A 44-year-old man presented with an asymptomatic,firm, 1.0-cm submucosal mass of the buccal mucosa.The patient had no known significant healthproblems and specifically had no history of humanimmunodeficiency virus infection. At the time ofexcision, the clinical differential diagnoses includedbenign mesenchymal tumors and salivary glandtumors. No other mucosal lesions were present.The excisional specimen consisted of a 1.2 3

0.8 3 0.8-cm ellipsoid segment of mucosa. Histo-logically, the tumor was relatively well circumscribed

(Fig. 1) but incompletely excised. The neoplasmdisplayed a lobular growth pattern (Fig. 2). Through-out the tumor there were ectatic, thin-walled vesselslined by a monolayer of flat endothelial cellsreminiscent of cavernous hemangiomas intermixedwith more cellular areas, composed of bland spindledand epithelioid cells (Fig. 3). Focal vascular throm-bosis was present within the tumor (Fig. 4). Endo-thelial cells with intracytoplasmic vascular lumens,so-called �blister cells’ were prominent in severalfields (Fig. 5). Immunohistochemical stains for vas-cular markers CD31 and CD34 were positive.

A diagnosis of spindle cell hemangioma wasrendered. The patient remained disease free 13months after the initial excision.

Discussion

Spindle cell hemangioma was first described in 1986by Weiss and Enzinger as spindle cell hemangioen-dothelioma, reflecting the original belief that this

797

neoplasm was of intermediate malignancy.4 As moreexperience was gained with this entity, its benignbehavior was recognized with subsequent reclassifi-cation as spindle cell hemangioma.1,2,7,8 There ishistologic and ultrastructural evidence that thistumor is probably a reactive process rather thana true neoplasm.1,7,8

Spindle cell hemangioma is a superficial neoplasmthat most commonly presents on the distal extremityof middle-aged adults as multiple or solitary sub-cutaneous nodules.5,6 Approximately one-half ofcases are intravascular and the presence of multiplelesions within the same region is attributed tointravascular extension of the lesion. Intravasculargrowth also contributes to the high rate of localrecurrence.

Involvement of the oral cavity by spindle cellhemangioma is rare, with only two previouslyreported cases.5,6 One case presented as a sub-

mucosal lesion of the buccal-mandibular fold ina 12-year-old girl,6 while the other presented on thepalate of a 55-year-old man.5 Like our case, boththese presented as small (, 2 cm), painless masses.The clinical differential diagnoses in these cases, likeour case, included benign salivary gland tumors vs.other mesenchymal tumors. Vascular lesions werenot suspected.Histologically, spindle cell hemangioma shows

variable cellularity imparting a lobular architectureon low power. Large, ectatic, vascular spaces linedby flattened endothelial cells are frequently presentand may have thrombi. Between the dilated vascularspaces there is a proliferation of spindled cellscomposed of endothelial cells, pericytes and fibro-blasts. Interspersed within the spindled areas areepithelioid endothelial cells that frequently haveintracytoplasmic vascular lumens. Spindle cellhemangioma generally lacks significant nuclearatypia and rarely shows mitotic activity.

Fig. 2. The tumor was composed of a spindled cells arranged in

a vaguely lobular growth pattern.

Fig. 1. Scanning magnification showed a circumscribed prolifera-

tion of spindled cells in the subepithelial stroma.

Fig. 3. Interspersed throughout the tumor between were ectatic,

vascular spaces lined by bland endothelial cells. Surrounding the

ectatic, vascular spaces there was a proliferation of spindled cells

with slit-like vascular channels.

Fig. 4. Focal intravascular fibrin thrombi were present.

Sheehan et al.

798

The differential diagnosis centers on other tumorsof vascular origin. The presence of epithelioidendothelial cells with intracytoplasmic lumens raisesthe possibility of an epithelioid hemangioendothe-lioma, a vascular neoplasm of intermediate biologicbehavior that may also rarely present in the oralcavity.9–12 However, spindle cell hemangioma lacksthe infiltrative growth pattern and characteristicmyxohyaline matrix seen in typical epithelioidhemangioendothelioma.Because of the prominent spindled areas, spindle

cell hemangioma is easily mistaken for Kaposisarcoma. In the original description, Enzinger andWeiss considered spindle cell hemangioma as a dis-tinctive low-grade angiosarcoma resembling a cav-ernous hemangioma and Kaposi sarcoma. Kaposisarcoma of the oral cavity is well documented in thesetting of acquired immune deficiency syndromeand may also occur in non-immunocompromisedpatients.13,14 In contrast to spindle cell hemangi-oma, Kaposi sarcoma has a more infiltrative growthwith some nuclear atypia and a higher mitotic rate.Kaposi sarcoma lacks the epithelioid endothelialcells of spindle cell hemangioma. In selected cases,immunohistochemical stains for the human herpeslatent nuclear antigen can be helpful. Nuclearimmunoreactivity for this marker is consistentlypresent in Kaposi sarcoma and not spindle cellhemangioma.15

Angiosarcoma can present in the oral cavity andmay occasionally have a predominantly spindledmorphology.16 Angiosarcoma, however, has aninfiltrative growth pattern and shows significantnuclear atypia, a high mitotic rate and atypicalmitotic figures, features not seen in spindle cellhemangioma.

Given the dearth of previously reported cases ofspindle cell hemangioma involving the oral cavity, itis difficult to draw conclusions regarding the riskfactors or biologic behavior of spindle cell heman-gioma. Most cases of cutaneous spindle cellhemangioma are sporadic in nature, but a smallpercentage is associated with conditions withvascular abnormalities such as Mafucci’s syndrome,lymphedema, Klippel-Trenaunay syndrome andearly-onset varicose veins.1,17 No such associationhas been reported in spindle cell hemangiomaarising in the oral cavity. Cutaneous spindle cellhemangioma is known for a high rate of localrecurrence. This has not been observed in our case orpreviously reported cases of oral spindle cellhemangioma, but there probably remains a risk oflocal recurrence. We recommend that patients shouldbe apprised of a potential risk of local recurrence andhave clinical follow up. Follow up could beaccomplished as a part of routine dental care.

In summary, we present a case of spindle cellhemangioma arising in the oral cavity of a 44-year-old otherwise healthy man. Spindle cell hemangi-oma should be considered in the differentialdiagnosis of vascular tumors in the oral cavity inorder not to be misinterpreted as a more aggressivevascular tumor.

References

1. Fletcher CD, Beham A, Schmid C. Spindle cell haemangio-

endothelioma: a clinicopathological and immunohistochemical

study indicative of a non-neoplastic lesion. Histopathology

1991; 18: 291.

2. Perkins P, Weiss SW. Spindle cell hemangioendothelioma. An

analysis of 78 cases with reassessment of its pathogenesis and

biologic behavior. Am J Surg Pathol 1996; 20: 1196.

3. Scott GA, Rosai J. Spindle cell hemangioendothelioma. Report

of seven additional cases of a recently described vascular

neoplasm. Am J Dermatopathol 1988; 10: 281.

4. Weiss SW, Enzinger FM. Spindle cell hemangioendothelioma.

A low-grade angiosarcoma resembling a cavernous hemangi-

oma and Kaposi’s sarcoma. Am J Surg Pathol 1986; 10: 521.

5. Ide F, Obara K, Enatsu K, Mishima K, Saito I. Rare vascular

proliferations of the oral mucosa. Oral Surg Oral Med Oral

Pathol Oral Radiol Endod 2004; 97: 75.

6. Tosios K, Koutlas IG, Kapranos N, Papanicolaou SI. Spindle-

cell hemangioendothelioma of the oral cavity. A case report.

J Oral Pathol Med 1995; 24: 379.

7. Fletcher CD. The non-neoplastic nature of spindle cell

hemangioendothelioma. Am J Clin Pathol 1992; 98: 545.

8. 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: 279.

9. Tong GX, Hamele-Bena D, Borczuk A, Monaco S, Khosh MM,

Greenebaum E. Fine needle aspiration biopsy of epithelioid

hemangioendothelioma of the oral cavity: report of one case and

review of literature. Diagn Cytopathol 2006; 34: 218.

Fig. 5. High power examination showed bland spindled cells

admixed with groups of endothelial cells with intracytoplasmic

vascular lumens.

Spindle cell hemangioma

799

10. Chi AC, Weathers DR, Folpe AL, Dunlap DT, Rasenberger K,

Neville BW. Epithelioid hemangioendothelioma of the oral

cavity: report of two cases and review of the literature. Oral

Surg Oral Med Oral Pathol Oral Radiol Endod 2005;

100: 717.

11. Orsini G, Fioroni M, Rubini C, Piattelli A. Epithelioid

hemangioendothelioma of the oral cavity: report of case.

J Oral Maxillofac Surg 2001; 59: 334.

12. Flaitz CM, McDaniel RK, Mackay B, Kennady MC, Luna

MA, Hicks MJ. Primary intraoral epithelioid hemangioendo-

thelioma presenting in childhood: review of the literature and

case report. Ultrastruct Pathol 1995; 19: 275.

13. Ficarra G, Eversole LE. HIV-related tumors of the oral cavity.

Crit Rev Oral Biol Med 1994; 5: 159.

14. Jindal JR, Campbell BH, Ward TO, Almagro US. Kaposi’s

sarcoma of the oral cavity in a non-AIDS patient: case report

and review of the literature. Head Neck 1995; 17: 64.

15. Patel RM, Goldblum JR, Hsi ED. Immunohistochemical

detection of human herpes virus-8 latent nuclear antigen-1 is

useful in the diagnosis of Kaposi sarcoma. Mod Pathol 2004;

17: 456.

16. Fanburg-Smith JC, Furlong MA, Childers EL. Oral and

salivary gland angiosarcoma: a clinicopathologic study of 29

cases. Mod Pathol 2003; 16: 263.

17. Fanburg JC, Meis-Kindblom JM, Rosenberg AE. Multiple

enchondromas associated with spindle-cell hemangioendothe-

liomas. An overlooked variant of Maffucci’s syndrome. Am J

Surg Pathol 1995; 19: 1029.

Sheehan et al.

800