Virchows Archiv

, Volume 448, Issue 6, pp 843–846 | Cite as

Multifocal kaposiform haemangioendothelioma

  • Karen Deraedt
  • Vincent Vander Poorten
  • Chris Van Geet
  • Marleen Renard
  • Ivo De Wever
  • Raf Sciot
Case Report

Abstract

Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive vascular spindle cell proliferation, with resemblance to Kaposi’s sarcoma. This tumour usually occurs in skin and retroperitoneum of infants and young children and is often complicated by the Kasabach–Merritt phenomenon (KMP). A 3-year-old boy presented with a right submandibular swelling due to lymphadenopathies, a violaceous skin lesion at the left commissure of the lips and an ill-defined lesion in the right thyroid lobe. There were some signs of KMP. Histological examination revealed a typical infiltrative multilobular spindle cell proliferation with slit-like vascular spaces in these three localisations. Immunohistochemical stains showed positivity for CD34 and CD31 and many alpha-smooth muscle actin-positive spindle cells around the vascular spaces. There was no Herpes virus type 8 expression. The presented case is unique in two ways. First, thyroid involvement of KHE has never been described in the literature until now. Secondly, and most remarkably, the multifocal presentation in three anatomically distinct and separated localisations is extremely unusual.

Keywords

Kaposiform haemangioendothelioma Kasabach–Merritt phenomenon Consumptive coagulopathy Multifocal 

References

  1. 1.
    Brasanac D, Janic D, Boricic I, Jovanovic N, Dokmanovic L (2003) Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with Kasabach–Merritt syndrome. Pathol Int 53:627–631CrossRefPubMedGoogle Scholar
  2. 2.
    Chung MT, Chen CH, Chiu CH, Yang CP, Hsueh C, Jaing TH (2003) Successful nonoperative therapy for Kaposiform hemangioendothelioma involving the neck: report of 1 case. Otolaryngol Head Neck Surg 129:605–607CrossRefPubMedGoogle Scholar
  3. 3.
    Fukunaga M, Ushigome S, Ishikawa E (1996) Kaposiform haemangioendothelioma associated with Kasabach–Merritt syndrome. Histopathology 28:281–284CrossRefPubMedGoogle Scholar
  4. 4.
    Gianotti R, Gelmetti C, Alessi E (1999) Congenital cutaneous multifocal kaposiform hemangioendothelioma. Am J Dermatopathol 21:557–561CrossRefPubMedGoogle Scholar
  5. 5.
    Hardisson D, Prim MP, De Diego JI, Patron M, Escribano A, Rabanal I (2002) Kaposiform hemangioendothelioma of the external auditory canal in an adult. Head Neck 24:614–617CrossRefPubMedGoogle Scholar
  6. 6.
    Lalaji TA, Haller JO, Burgess RJ (2001) A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging. Pediatr Radiol 31:876–878CrossRefPubMedGoogle Scholar
  7. 7.
    Lyons LL, North PE, Mac-Moune Lai F, Stoler MH, Folpe AL, Weiss SW (2004) Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. Am J Surg Pathol 28:559–568PubMedGoogle Scholar
  8. 8.
    Mac-Moune Lai F, To KF, Choi PC, Leung PC, Kumta SM, Yuen PP, Lam WY, Cheung AN, Allen PW (2001) Kaposiform hemangioendothelioma: five patients with cutaneous lesion and long follow-up. Mod Path 14:1087–1092CrossRefGoogle Scholar
  9. 9.
    Martinez AE, Robinson MJ, Alexis JB (2004) Kaposiform hemangioendothelioma associated with nonimmune fetal hydrops. Arch Pathol Lab Med 128:678–681PubMedGoogle Scholar
  10. 10.
    Mentzel T, Mazzoleni G, Dei Tos AP, Fletcher CD (1997) Kaposiform hemangioendothelioma in adults. Clinicopathologic and immunohistochemical analysis of three cases. Am J Clin Pathol 108:450–455PubMedGoogle Scholar
  11. 11.
    Niedt GW, Greco MA, Wieczorek R, Blanc WA, Knowles DM (1989) Hemangioma with Kaposi’s sarcoma-like features: report of two cases. Pediatr Pathol 9:567–575PubMedCrossRefGoogle Scholar
  12. 12.
    O’Hara CD, Nascimento AG (2003) Endothelial lesions of soft tissues: a review of reactive and neoplastic entities with emphasis on low-grade malignant (“borderline”) vascular tumors. Adv Anat Pathol 10:69–87CrossRefPubMedGoogle Scholar
  13. 13.
    Perkins P, Weiss SW (1996) Spindle cell hemangioendothelioma: An analysis of 78 cases with reassessment of its pathogenesis and biologic behavior. Am J Surg Pathol 20:1196–1204CrossRefPubMedGoogle Scholar
  14. 14.
    Sarkar M, Mulliken JB, Kozakewich HP, Robertson RL, Burrows PE (1997) Thrombocytopenic coagulopathy (Kasabach–Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma. Plast Reconstr Surg 100:1377–1386PubMedCrossRefGoogle Scholar
  15. 15.
    Tello MA, Shields G, Gadre SA, Ryan M (2004) Pathology quiz case 2. Diagnosis: Kaposiform hemangioendothelioma (KHE). Arch Otolaryngol Head Neck Surg 130:991 Diag 993–994CrossRefPubMedGoogle Scholar
  16. 16.
    Tsang WY, Chan JK (1991) Kaposi-like infantile hemangioendothelioma. A distinctive vascular neoplasm of the retroperitoneum. Am J Surg Pathol 15:982–989PubMedCrossRefGoogle Scholar
  17. 17.
    Tsang WY (2002) Kaposiform haemangioendothelioma. In: Fletcher CDM, Unni KK, Mertens F (eds) World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. IARC, Lyon, pp 163–164Google Scholar
  18. 18.
    Vin-Christian K, McCalmont TH, Frieden IJ (1997) Kaposiform hemangioendothelioma. An aggressive, locally invasive vascular tumor that can mimic hemangioma of infancy. Arch Dermatol 133:1573–1578CrossRefPubMedGoogle Scholar
  19. 19.
    Wilken JJ, Meier FA, Kornstein MJ (2000) Kaposiform hemangioendothelioma of the thymus. Arch Pathol Lab Med 124:1542–1544PubMedGoogle Scholar
  20. 20.
    Zukerberg LR, Nickoloff BJ, Weiss SW (1993) Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach–Merritt syndrome and lymphangiomatosis. Am J Surg Pathol 17:321–328PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Karen Deraedt
    • 1
  • Vincent Vander Poorten
    • 2
  • Chris Van Geet
    • 3
  • Marleen Renard
    • 3
  • Ivo De Wever
    • 4
  • Raf Sciot
    • 1
  1. 1.Department of Pathology, University Hospital St. RafaëlKatholieke Universiteit LeuvenLeuvenBelgium
  2. 2.Head and Neck Surgery, University HospitalCatholic University of LeuvenLeuvenBelgium
  3. 3.Pediatrics, University HospitalCatholic University of LeuvenLeuvenBelgium
  4. 4.Oncological Surgery, University HospitalCatholic University of LeuvenLeuvenBelgium

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