A. Calderoni et al., AUTOIMMUNE PROCESSES AS PARANEOPLASTIC MA NIFESTATIONS IN FAMILIAL BREAST-CANCER, Deutsche Medizinische Wochenschrift, 119(36), 1994, pp. 1194-1198
In two patients, mother and daughter, L with breast cancer a paraneopl
astic syndrome of probably autoimmune pathogenesis developed in the co
urse of the neoplastic disease. In the mother the cancer occurred when
she was aged 51, first in the left and then, 4 years later, in the ri
ght breast. A local recurrence 18 years later caused a pericardial eff
usion and further tumour recurrences were associated with symptoms lik
e those in Sjogren's syndrome, as well as bullous pemphigoid and polyn
europathy. Antinuclear antibodies, extractable nuclear antigen antibod
ies and rheuma factors were demonstrated in serum. Both symptoms and a
ntibody titres regressed under treatment with tamoxifen and various cy
tostatic drugs. The patient died aged 74 years. - The daughter underwe
nt a lumpectomy for breast cancer when aged 39 years, followed by mast
ectomy for local recurrence after 2 years. A further 2 years later she
was found to be anaemic with a positive Coombs test (1 : 1024) and an
tibodies against IgG and complement. Metastases developed later. In he
r case, too, clinical and serological findings clearly regressed under
treatment with tamoxifen and chemotherapy. She died, aged 45 years, f
rom the consequences of further metastases.