Synonyms
The tumors were initially reported as “spindle cell hemangioendothelioma.”
Definition
Benign vascular proliferation composed of ectatic vascular channels, vacuolated endothelial cells, and spindled pericytes. The tumors may represent a vascular malformation.
Clinical Features
Incidence
The tumors are rare, but no data on incidence exist. A subset of tumors is seen in patients with Mafucci or Klippel-Trenaunay syndrome.
Age
A wide age range is affected with a peak in young adulthood (Fletcher et al. 1991; Perkins and Weiss 1996).
Sex
There is no gender predilection.
Site
There is a predilection for the distal extremities (Fletcher et al. 1991; Perkins and Weiss 1996).
Treatment
Simple excision is adequate
Outcome
Spindle cell hemangioas are benign with rare local recurrence. Further lesions may develop at the same anatomic site.
Macroscopy
Spindle cell hemangioma presents as solitary or multiple slowly enlarging, erythematous to bluish nodules measuring up to few centimeters....
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References and Further Reading
Fletcher, C. D., Beham, A., & Schmid, C. (1991). Spindle cell haemangioendothelioma: A clinicopathological and immunohistochemical study indicative of a non-neoplastic lesion. Histopathology, 18(4), 291–301.
Kurek, K. C., Pansuriya, T. C., van Ruler, M. A., van den Akker, B., Luks, V. L., Verbeke, S. L., Kozakewich, H. P., Sciot, R., Lev, D., Lazar, A. J., Fletcher, C. D., & Bovée, J. V. (2013). R132C IDH1 mutations are found in spindle cell hemangiomas and not in other vascular tumors or malformations. The American Journal of Pathology, 182(5), 1494–1500.
Pansuriya, T. C., van Eijk, R., d’Adamo, P., van Ruler, M. A., Kuijjer, M. L., Oosting, J., Cleton-Jansen, A. M., van Oosterwijk, J. G., Verbeke, S. L., Meijer, D., van Wezel, T., Nord, K. H., Sangiorgi, L., Toker, B., Liegl-Atzwanger, B., San-Julian, M., Sciot, R., Limaye, N., Kindblom, L. G., Daugaard, S., Godfraind, C., Boon, L. M., Vikkula, M., Kurek, K. C., Szuhai, K., French, P. J., & Bovée, J. V. (2011). Somatic mosaic IDH1 and IDH2 mutations are associated with enchondroma and spindle cell hemangioma in Ollier disease and Maffucci syndrome. Nature Genetics, 43(12), 1256–1261.
Perkins, P., & Weiss, S. W. (1996). Spindle cell hemangioendothelioma. An analysis of 78 cases with reassessment of its pathogenesis and biologic behavior. The American Journal of Surgical Pathology, 20(10), 1196–1204.
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Brenn, T. (2020). Spindle Cell Hemangioma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5468-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5468-1
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