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Spindle cell hemangiomaD18.0

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 05.08.2021

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Synonym(s)

Spindle cell hemangioendothelioma; spindle cell hemangioma; Spindle cell hemangioma; Spindle cell hemangioma:

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HistoryThis section has been translated automatically.

White and Enzinger 1986

DefinitionThis section has been translated automatically.

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

ManifestationThis section has been translated automatically.

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

LocalizationThis section has been translated automatically.

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

Clinical featuresThis section has been translated automatically.

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).

HistologyThis section has been translated automatically.

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.

Differential diagnosisThis section has been translated automatically.

Kaposi's Sarcoma

TherapyThis section has been translated automatically.

Excision can be difficult due to the diffuse growth, but it seems to be the most promising method of treatment. The risk of recurrence is high (> 50%).

LiteratureThis section has been translated automatically.

  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

Authors

Last updated on: 05.08.2021

Author: Prof. Dr. med. Peter Altmeyer