Spindle cell hemangioma D18.0

White and Enzinger 1986

Benign (probably reactive) vascular neoplasm, usually at the base of a vascular malformation.

Occurs in all age groups (1st-8th decade of life); preferred in adolescence and early adulthood; no gender preference.

Most often on the lower leg. Malformation may also affect only internal organs (paranasal sinuses, lungs).

Beginning with solitary, firm, usually not clearly distinguishable, symptomless, red to blue-red, deeply cutaneous or subcutaneously located nodule. Loco-regional appositional growth.

Known associations are Klippel-Trénaunay syndrome, Maffucci syndrome, congenital lymphedema and epitheloid hemangiodendothelioma (rare).

Circumscribed, solitary or multiple mostly intravascular tumor convolutes.

Thin-walled, cavernous cavities with organized thrombi and floating endothelial papillae (signs of papillary endothelial hyperplasia). Cell-rich areas with spindle-cell, also vacuolated, epithelioid elements with intraplasmatic vacuoles as well as features of spindle-shaped pericytes. Formation of slit-like cavities. Nuclear atypia are absent.

Immunohistology: CD31 pos. epithelioid cells and smooth muscle actin pos. spindle-shaped pericytes.

1. Chan JKC et al (1997) Vascular tumors with a prominent spindle cell component. Curr Diagn Pathol 4: 76-90
2. Chavva S et al (2015) Rare case of spindle cell haemangioma. J Clin Diagn Res 9: 19-21.
3. Coras B et al (2003) Spindle cell hemangioma. Dermatol Surg 29: 875-878.
4. Duqing X et al. (2019) Multiple spindle cell hemangiomas in both lungs: a rare case report and review of the literature. J Cardiothorac Surg 14:86.
5. Oukessou Y et al (2021) Spindle cell hemangioma in the infratemporal fossa: A unique case report. Int J Surg Case Rep 78:38-41.
6. Keel SB et al (1999) Hemorrhagic epithelioid and spindle cell hemangioma: a newly recognized, unique vascular tumor of bone. Cancer 85: 1966-1672
7. Kramer D et al (2016) Multifocal spindle cell hemangioma: report of two cases. Indian J Dermatol Venereol Leprol 82: 93-95.
8. Setoyama M et al (2000) Spindle cell hemangioendothelioma successful treatment with recombinant interleukin-2. Br J Dermatol 142: 1238-1239.
9. Perkins P, Weiss SW (1996) Spindle cell hemangioendothelioma. An analysis of 78 cases with reassessemtn of its pathogenensis and biological behaviour. Am J Surg Pathol 20: 1196-1204
10. Tomasini C et al. Spindle cell hemangioma. Dermatology 199: 274-276
11. Weiss SW, Enzinger FM (1986) Spindle cell hemangioendothelioma. A low-grade angiosarcoma resembling a cavernous hemangioma and Kaposi's sarcoma. Am J Surg Pathol 10: 521-530