| Lymphangiosarcoma of the pubic region: a rare complication arising in congenital non-hereditary lymphedema. | |
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MedLine Citation:
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PMID: 9854166 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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A Cerri; C Gianni; M Corbellino; M Pizzuto; L Moneghini; C Crosti |
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Publication Detail:
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Type: Case Reports; Journal Article; Review |
Journal Detail:
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Title: European journal of dermatology : EJD Volume: 8 ISSN: 1167-1122 ISO Abbreviation: Eur J Dermatol Publication Date: 1998 Oct-Nov |
Date Detail:
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Created Date: 1999-01-21 Completed Date: 1999-01-21 Revised Date: 2005-11-16 |
Medline Journal Info:
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Nlm Unique ID: 9206420 Medline TA: Eur J Dermatol Country: FRANCE |
Other Details:
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Languages: eng Pagination: 511-4 Citation Subset: IM |
Affiliation:
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Clinica Dermatologica IV, Istituto di Scienze Biomediche, Ospedale San Paolo, Università degli Studi di Milano, via A. di Rudini, 8, 20142 Milano, Italy. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Combined Modality Therapy DNA, Neoplasm / analysis Female Follow-Up Studies Humans Lymphangiosarcoma / etiology*, pathology, radiotherapy, surgery Lymphedema / complications*, congenital Prognosis Pubic Symphysis Skin Neoplasms / etiology*, pathology, radiotherapy, surgery |
| Chemical | |
Reg. No./Substance:
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0/DNA, Neoplasm |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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