Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Spindle Cell Hemangioma

  • Thomas BrennEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_5468-1
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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. Multiple tumors often affect the same anatomic location.

Microscopy

The tumors are located in the dermis and superficial subcutis and present as relatively circumscribed cellular nodules (Fig. 1). They are composed of thin-walled irregular and dilated vascular structures which may appear congested and contain fibrin thrombi (Fig. 2). Solid areas of bland spindle cells are arranged in short fascicles and are closely associated with narrow vascular spaces (Fig. 3). An additional feature is the presence of clusters of vacuolated epithelioid endothelial cells (Fig. 4). Cytologic atypia is not seen and mitotic activity is rare. The tumors frequently show a partial or less commonly entirely intravascular growth. Abnormally formed vessels are commonly found in the periphery of the tumor.
Fig. 1

Spindle cell hemangioma. Well-circumscribed nodular and cellular tumor

Fig. 2

Spindle cell hemangioma. The tumor shows thin-walled dilated and often congested vascular channels

Fig. 3

Spindle cell hemangioma. Bland appearing uniform spindle cells are arranged in short fascicles adjacent to compressed vascular channels

Fig. 4

Spindle cell hemangioma. In areas, clusters of epithelioid endothelial cells with cytoplasmic vacuolization are found

Immunophenotype

The endothelial cells express endothelial cell markers including podoplanin, but they are negative for WT1. The surrounding spindle cells lack the endothelial phenotype but are positive for SMA.

Molecular Features

Mutations in the IDH1 and IDH2 genes are common (Kurek et al. 2013; Pansuriya et al. 2011).

Differential Diagnosis

The spindle cells in nodular Kaposi sarcoma express CD34 and HHV-8. Spindle cell angiosarcoma shows a more infiltrative growth pattern and pronounced cytologic atypia. The tumor cells show an endothelial phenotype.

References and Further Reading

  1. 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.CrossRefGoogle Scholar
  2. 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.CrossRefGoogle Scholar
  3. 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.CrossRefGoogle Scholar
  4. 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.CrossRefGoogle Scholar

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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Pathology and Laboratory Medicine and The Arnie Charbonneau Cancer InstituteCumming School of Medicine, University of CalgaryCalgaryCanada