Assuntos
Linfangiossarcoma/etiologia , Linfedema/complicações , Neoplasias Cutâneas/etiologia , Idoso , Braço , Doença Crônica , Terapia Combinada , Feminino , Seguimentos , Humanos , Linfangiossarcoma/mortalidade , Linfangiossarcoma/radioterapia , Linfangiossarcoma/cirurgia , Linfedema/etiologia , Dosagem Radioterapêutica , Neoplasias Cutâneas/mortalidade , Neoplasias Cutâneas/radioterapia , Neoplasias Cutâneas/cirurgia , Síndrome , Fatores de TempoRESUMO
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.
Assuntos
Linfangiossarcoma/etiologia , Linfedema/complicações , Neoplasias Cutâneas/etiologia , Adulto , Terapia Combinada , DNA de Neoplasias/análise , Feminino , Seguimentos , Humanos , Linfangiossarcoma/patologia , Linfangiossarcoma/radioterapia , Linfangiossarcoma/cirurgia , Linfedema/congênito , Prognóstico , Sínfise Pubiana , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/radioterapia , Neoplasias Cutâneas/cirurgiaAssuntos
Adenocarcinoma/cirurgia , Braço , Neoplasias da Mama/cirurgia , Linfangiossarcoma/tratamento farmacológico , Linfedema/complicações , Segunda Neoplasia Primária/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Idoso , Antibióticos Antineoplásicos/administração & dosagem , Antineoplásicos Alquilantes/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Bleomicina/administração & dosagem , Terapia Combinada , Doxorrubicina/administração & dosagem , Feminino , Seguimentos , Humanos , Ifosfamida/administração & dosagem , Linfangiossarcoma/radioterapia , Segunda Neoplasia Primária/radioterapia , Dosagem Radioterapêutica , Neoplasias Cutâneas/radioterapia , SíndromeAssuntos
Cuidados Intraoperatórios , Neoplasias Pancreáticas/radioterapia , Neoplasias Retais/radioterapia , Sarcoma/radioterapia , Neoplasias de Tecidos Moles/radioterapia , Terapia Combinada , Feminino , Humanos , Linfangiossarcoma/radioterapia , Linfangiossarcoma/cirurgia , Pessoa de Meia-Idade , Neoplasias Pancreáticas/cirurgia , Neoplasias Retais/cirurgia , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/cirurgiaRESUMO
We have reported on four previously unpublished cases of postmastectomy lymphangiosarcoma. All of these cases had suffered from a long-lasting lymphedema of the upper extremity. Only one patient had survived over one year. After radiotherapy she lived four years from first lesion of lymphangiosarcoma. She died locally disease-free in spite of two earlier recurrences, which were also treated with radiotherapy. We believe that only in early cases can the radiotherapy give a good palliation in postmastectomy lymphangiosarcoma.