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Resume But de l'etude L'amelioration spectaculaire de la survie des patients traites pour une maladie de Hodgkin (MH) a permis l'analyse du devenir des patients survivant a long terme. Ces derniers ont eu un certain nombre de complications, dont des neoplasies secondaires constituant le probleme le plus important. Plusieurs travaux recents font etat d'une augmentation de l'incidence des cancers du sein (CS) chez les jeunes femmes et les enfants traites pour une MH. Materiel et methodes Dans une etude retrospective realisee dans 11 centres de lutte contre le cancer, nous avons recense 63 femmes traitees pour MH et ayant ulterieurement developpe un cancer du sein. Nous avons analyse les resultats obtenus chez 13 patientes (21 %) qui ont eu un cancer du sein bilateral soit synchrone (cinq patientes), soit metachrone (huit patientes). L'âge moyen lors du traitement de la MH etait de 19 ans. Sept patientes avaient ete traitees par irradiation exclusive (comprenant une irradiation sus-diaphragmatiquedetype ≪ mantelet ≫ et six par association chimiotherapie-radiotherapie. Resultats Le delai moyen d'apparition du premier cancer du sein a ete de 16 ans. Selon la classification TNM, on notait: neuf tumeurs de stade T0, quatre tumeurs de stade T1, cinq tumeurs de stade T2, une tumeur de stade T3, deux tumeurs de stade T4 et cinq tumeurs de stade Tx; il y avait 17 carcinomes infiltrants, deux fibrosarcomes et sept carcinomes canalaires in situ. Un envahissement ganglionnaire a ete retrouve dans dix des 15 curages realises pour des cancers infiltrants. Globalement, 17 tumeurs ont ete traitees par chirurgie radicale et neuf par association radiochirurgicale conservatrice. Avec un suivi moyen de 70 mois (extremes: 15–125), trois patientes ont eu une recidive locale et/ou regionale et quatre des metastases. Huit patientes etaient en vie sans signe de maladie et une est decedee d'affection intercurrente. Conclusion Dans la litterature, les cancers du sein representent de 6,3 a 9 % des neoplasies secondaires apres traitement pour MH, mais le risque est beaucoup plus eleve chez les jeunes filles traitees avant 20 ans, et plus particulierement avant 15 ans. Les facteurs favorisants sont: l'irradiation, la jeunesse lors du traitement, la chimiotherapie par agents alkylants, et peut-etre des facteurs genetiques. Ces femmes doivent beneficier d'une surveillance senologique tres reguliere et prolongee, cela au minimum des la fin de la 10e annee apres la fin du traitement de la MH. Un traitement conservateur pour ces cancers du sein secondaires est parfois possible.

Between 1979 and 1990, 149 patients with non-metastatic thymomas were treated in ten French cancer centers. Patients were staged according to the 'GETT' classification, derived from that of Masaoka. There were 13 stage I patients, 46 stage II, 58 stage III and 32 stage IVA. Gross total resections were performed in 63 cases, subtotal resections in 31 cases and in 55 cases a biopsy alone was performed. All patients received radiotherapy and 74 were given post-operative chemotherapy. Median follow-up was 7.7 years. Local control was achieved in 117 cases (78.5%) and was influenced by the stage of the disease (p < 0.01) and the extent of surgery (p < 0.01). Twenty-six patients developed metastatis after a median period of 9 months. Five- and ten-year disease-free survival rates were 59.5% (51-67%) and 49.5% (39-60%), respectively, and were influenced by the stage of the disease (p < 0.01), the extent of surgery (p < 0.001) and a mediastinal compression on presentation (p = 5 x 10(-6)). Four factors could predict a worse overall survival in the multivariate analysis: mediastinal compression on presentation (p < 0.001), absence of chemotherapy (p < 0.001), biopsy alone (p = 0.003), and young age (p = 0.013). A worse DFS was predicted by mediastinal compression on presentation (p < 0.001), absence of chemotherapy (p < 0.001), young age (p = 0.006), and stages III-IVA (p = 0.04). Future therapeutic strategies are discussed and the literature is reviewed.

In a retrospective multicentric analysis, 63 women treated between 1941 to 1988 for Hodgkin's disease (HD) subsequently developed 76 breast cancers (BC). The median age at diagnosis of HD was 26 years (range 7-67), and 22 women (35%) were 20 years old or less. Exclusive radiotherapy (RT) was used in 36 women (57%) and combined modalities with chemotherapy (CT) in 25 (39%). Breast cancer occurred after a median interval of 16 years (range 2-40) and the median age at diagnosis of the first BC was 42 years (range 25-73). TNM classification (UICC, 1978) showed 10 T0 (non-palpable lesions) (13%), 20 T1 (26%), 22 T2 (29%), 8 T3 (11%), 7 T4 (9%) and 9 Tx (12%), giving altogether a total of 76 tumours, including, respectively, 5 and 8 bilateral synchronous and metachronous lesions. Among the 68 tumours initially discovered, 53 ductal infiltrating, one lobular infiltrating and two medullary carcinomas were found. Moreover, two fibrosarcomas and 10 ductal carcinoma in situ (DCIS) were also found. Among 50 axillary dissections for invasive carcinomas, histological involvement was found in 31 cases (62%). 45 tumours were treated by mastectomy, without (n = 35) or with (n = 10) RT. 27 tumours had lumpectomy, without (n = 7) or with RT (n = 20). 2 others received RT only, and one only CT. 7 patients (11%) developed isolated local recurrence. 20 patients (32%) developed metastases and all died; 38 are in complete remission, whereas 5 died of intercurrent disease. The 5-year disease-specific survival rate by the Kaplan-Meier method was 61%. The 5-year disease-specific survival rate for pN0, pN1-3 and pNor = 3 groups were 91%, 66% and 0%, respectively (P0.0001) and 100%, 88%, 64% and 23% for the T0, T1, T2 and T3T4 groups, respectively. These secondary BCs seem to be of two types: a large number of aggressive tumours with a very unfavourable prognosis (especially in the case of pN3 and/or T3T4); and many tumours with a 'slow development' such as DCIS and microinvasive lesions, especially in patients treated exclusively by RT. Moreover, a very unusual rate of bilateral tumours (21%) was observed. These secondary BC could be 'in field', in 'border of field' or 'out of field'. However, a complete analysis of doses delivered by supradiaphragmatic irradiation was often very difficult, due to large variations in several parameters. We conclude that young women and girls treated for HD should be carefully monitored by clinical examination, mammography and ultrasonography.

From 1960 to 1986, 397 cases of non-metastatic male breast cancer (MBC) treated in 14 French regional cancer centres were reviewed. The median age was 64 years (range 25-93). TNM classification (UICC, 1978) showed seven T0, 79 T1, 162 T2, 31 T3, 74 T4 and 44 unclassified tumours (Tx). Clinical positive lymph nodes were found in 31% of the patients. 24 patients received radiotherapy only, and 373 underwent surgery, 247 of these with postoperative irradiation. Adjuvant chemotherapy and hormonal therapy were used in 71 and 68 patients, respectively. There were 382 infiltrating carcinomas and 15 pure ductal carcinoma in situ. Lymph node involvement was found in 56% of infiltrating carcinoma. The oestrogen (ER) and progesterone (PgR) receptors were positive in 79% and 77%, respectively, of examined cases. Isolated local and regional recurrence were observed in 8.8% and 4.5% of cases, respectively and 40% of patients developed metastases. The crude survival rates by Kaplan-Meier method were 65% and 38% at 5 and 10 years, respectively, and the disease-specific survival rates (without death due to intercurrent disease or second cancer) was 74% at 5 years and 51% at 10 years. The disease-specific survival rate for pN- and pN+ groups were 77% and 39% at 10 years. The prognostic factors were clinical size (T) and histological axillary status (pN-/pN+). The relative risk of death for pN- was 1.0, 2.0 and 3.2 in the T0-T1, T2 and T3-T4 groups, respectively. For pN+, these relative risks increased 1.9, 3.9 and 6.0 in the same groups. The optimal treatment include modified radical mastectomy and irradiation for cases with risk factors of local relapse (nodal invasion, large tumour with cutaneous or muscular involvement). Locoregional failure had unfavourable prognosis. First-line adjuvant treatment seems to be tamoxifen, due to the very high rate of positive hormonal receptors and the old age of the patients, which contraindicate chemotherapy in many cases. The prognosis of patients with breast cancer is the same in male and female patients when disease-specific survival rate, tumour size and axillary involvement are compared.

Background: Few germline BRCA2 rearrangements have been described compared with the large number of germline rearrangements reported in the BRCA1 gene. However, some BRCA2 rearrangements have been reported in families that included at least one case of male breast cancer. Objective: To estimate the contribution of large genomic rearrangements to the spectrum of BRCA2 defects. Methods: Quantitative multiplex PCR of short fluorescent fragments (QMPSF) was used to screen the BRCA2 gene for germline rearrangements in highly selected families. QMPSF was previously used to detect heterozygous deletions/duplications in many genes including BRCA1 and BRCA2 . Results: We selected a subgroup of 194 high risk families with four or more breast cancers with an average age at diagnosis of ⩽50 years, who were recruited through 14 genetic counselling centres in France and one centre in Switzerland. BRCA2 mutations were detected in 18.6% (36 index cases) and BRCA1 mutations in 12.4% (24 index cases) of these families. Of the 134 BRCA1/2 negative index cases in this subgroup, 120 were screened for large rearrangements of BRCA2 using QMPSF. Novel and distinct BRCA2 deletions were detected in three families and their boundaries were determined. We found that genomic rearrangements represent 7.7% (95% confidence interval 0% to 16%) of the BRCA2 mutation spectrum. Conclusion: The molecular diagnosis of breast cancer predisposition should include screening for BRCA2 rearrangements, at least in families with a high probability of BRCA2 defects.

Material From 1960 to 1990, 581 cases of male breast cancer (MBC) were reviewed in 19 Cancer Institutes in France. 23 (4%) were pure DCIS. The median age was 56.5 years (ext. 26–77). Five patients had less than 40 years (22%). Gynecomastia was found in 10 out of 23 patients (43%). Three had family history of B. C. According to TNM classification, we found 6 impalpable lesions (T0) discovered by serosanguineous nipple discharge, 7 T1, 6 T2, and 4 Tx. Treatment The surgery consisted of 3 lumpectomies, 16 modified, 2 subcutaneous and 2 radical mastectomies. 16 patients had axillary dissections and 6 irradiation on the chest wall. Histology All cases were pure DCIS: in 14 the subtype was clearly identified: papillary (4), papillary intracystic (3), mixed papillary and cribriform (3), comedocarcinoma (2), cribriform (1), apocrine (1). Three patients had local recurrences: two occurred in the patients initially treated by lumpectomy alone: the first was again a DCIS, but the second was an infiltrating carcinoma; this patient died by metastases. The last relapse occurred on the chest wall in a patient treated by mastectomy. One patient developed a contralateral DCIS. Two patients developed a lung and kidney cancer respectively. In the literature the rate of DCIS in man varies from 0 to 16%. The serosanguineous nipple discharge seems a frequent symptom, especially in young men. The main histologic subtype is papillary (pure or intracystic). Mastectomy is the treatment of choice.

Introduction The second cancers represents the most important problem among the survivors of HD. A possible link with chemotherapy (CT) and radiotherapy (RT) is suggested but genetic and immunologic factors may also be involved. Material In seven Cancer Centers, we found 35 women, previously treated for HD, who developed 37 BC. The median age at diagnosis of HD was 25 years, with 12 less than 20 years. HD stage was: 1 = 3, II = 21, III = 5, IV = 4, NS = 2. 33 women received supradiaphragmatic RT with doses varying from 35 to 45 Gy. 16 women received CT (mainly MOPP). The median interval between the diagnosis of HD and BC was 16 years. According to TNM, we found: 2 T0, 10 Tl, 12 T2, 4 T3, 6 T4 and 3 Tx. 32 were ductal infiltrating carcinoma, 2 medullary, 2 in situ and one fibrosarcoma. Axillary involvement was present in 51% of cases. Mastectomy was performed in 23 cases, a radiosurgical conservative treatment in 12 and exclusive radiotherapy in 2. Fourteen women underwent chemotherapy. Results 7 women had local relapse of BC and 15 had metastases (40%). Three had contralateral metachronous BC. 17 women are in complete remission for both diseases; 15 died of BC. Three women died of intercurrent disease. Conclusion The women treated for HD, especially before 20 years, seem to have an increased risk of subsequent BC. According to other reports, we confirm that these BC are frequently aggressive, with rapid evolution and high risk of bilaterality. Consequently, a regular mammographic follow-up is necessary to detect these lesions earlier, to allow a better prognosis and a possible conservative treatment.