Lymphangiosarcoma of the pubic region: A rare complication arising in congenital non-hereditary lymphedema

Amilcare Cerri, Claudia Gianni, Mario Corbellino, Massimo Pizzuto, Laura Moneghini, Carlo Crosti

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Lymphangiosarcoma is a rare, aggressive, vascular neoplasm arising in chronic congenital or acquired lymphedema. Although it is most frequently associated with post-mastectomy lymphedema (Stewart-Treves's syndrome), lymphangiosarcoma can exceptionally arise in congenital hereditary lymphedema (Milroy's syndrome and Meige's syndrome) and non-hereditary lymphedema (congenital, praecox or forme tarde lymphedemas). We report a case of lymphangiosarcoma of the pubic region, supported by immunohistochemical studies, in a 42-year-old woman affected by congenital, non-hereditary lymphedema of the left genital region and homolateral lower limb. In addition, molecular analysis demonstrated the absence of Kaposi's sarcoma- associated Herpes virus (KSHV) DNA sequences in tumour lesions. To our knowledge, this is the first case of lymphangiosarcoma associated with congenital non-hereditary lymphedema confined to the pubic region. The literature concerning the cases of lymphangiosarcoma arising in congenital hereditary and non-hereditary lymphedema is reviewed. Moreover, we emphasized the importance of regular clinical controls in all patients affected by chronic lymphedema. In fact, although the prognosis of this neoplasm is very poor, a prompt diagnosis and a rapid, ablative surgery associated with radiation therapy can increase the possibility of survival of these patients.

Original languageEnglish
Pages (from-to)511-514
Number of pages4
JournalEuropean Journal of Dermatology
Issue number7
Publication statusPublished - 1998


  • Congenital lymphedema
  • KSHV
  • Lymphangiosarcoma
  • Meige's disease
  • Milroy's disease
  • Non-hereditary lymphedema
  • Vascular tumours

ASJC Scopus subject areas

  • Dermatology


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