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The authors report a series of 30 adults with medulloblastoma treated after surgery between 1975 and 1990, Half the patients presented a classical medulloblastoma and the other half presented desmoplastic medulloblastoma. Brainstem infiltration was noted in 3 patients, cerebellar peduncle infiltration in 6 cases and 4 patients had cerebrospinal fluid (CSF) involvement. Tumor resection was achieved in all patients except 1, and in 5 surgery was incomplete. All patients but 2 received postoperative cerebral radiotherapy. Six patients did not receive chemotherapy before radiotherapy, 7 patients received CCNU-Vincristine concomitantly with and after irradiation, 1 patient was given a CHOP regimen, and the remaining 16 patients received the 8 drugs in a 1-day (8/1) regimen before irradiation. The median follow-up time was 104 months. The 5- and 10-year overall survival rates were 58.5% and 41%, respectively. Two toxic deaths occurred under the 8/1 regimen, and no better survival was observed for this group of patients in comparison with those receiving other regimens. Persistance of malignant cells in the CSF, brainstem involvement, cerebellar peduncle infiltration, non-radical surgery and a postoperative performance status (PS) of more than 2 were significantly correlated with an adverse outcome for overall survival in adult patients with medulloblastoma.

Between 06.86 and 11.89, 88 medulloblastoma or primitive neuroectodermic tumour (PNET) localised in the posterior fossa have been included in the M7 multicentric protocol, 82 received the totality of the radiotherapy treatment and were evaluable for this study. Twenty-two of these 82 patients relapsed: their radiotherapy treatment is analysed in the present study. In 10 cases out of the 22 relapses treatment failure was probably due to a radiotherapeutic imperfection. This study confirms the necessity of a strict radiotherapy control, particulary in multicentric study.

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.

Male breast cancer represents about only 1% of all breast cancers. We have analysed a retrospective, multicentric series of 404 patients, initially non-metastatic, with mean age of 63 years. The 5 and 10-year overall survival rates were 65 and 36% respectively. Sixty-eight patients developed secondary cancer. From ten patients who already presented with cancer (2.5%) 3 cases corresponded to prostatic cancer treated by estrogen. Four had synchronous cancer (1%). Three and eight patients respectively had a synchronous and metachronous contralateral breast cancer (2.7% of bilateral cancer). Forty-three other patients (10.6%) developed metachronous cancer. The main tumor types were: prostate (9), lung (6), colon and rectum (6), esophagus (4). Four patients developed various hematologic malignancies and 14 patients, various types of solid tumors. From these 43 patients, 27 died; 19 as a result of secondary cancer. This represents 9% of all deaths among the 404 patients. While the bilateral cancer rate is similar to women, the second cancer rate appears to be higher in men. From hematological malignancies, chemotherapy and radiotherapy do not seem to contribute to this high incidence of second cancer.

Ductal carcinoma in situ of the breast is very rare in men, representing 0-7% of all male breast cancers. We analysed 15 cases from a retrospective multicentric series of 404 patients (3.7%). It occurs earlier than infiltrating carcinoma (mean age: 55 years), sometimes before 40 years of age. The main symptoms are bloody nipple discharge or retro areolar mass. Modified radical mastectomy constitutes the basic treatment. Lower axillary dissection can eventually be indicated in comedocarcinoma or in tumors larger than 25 mm. The main histologic subgroup is papillary carcinoma, pure or intracystic. As is the case in women, local recurrence, invasive or not, rarely occurs. Theoretically, the cure rate approaches 100%. However, as in all cases of breast cancer in men, an important number of deaths due to secondary cancer or intercurrent disease have been noted. Until now, no clear etiologic factors have been found.

A 26-year-old woman delivered a normal child 5 years after bone marrow transplantation for severe aplastic anemia. The conditioning regimen comprised high dose cyclophosphamide and thoraco-abdominal irradiation (6 Gy). This and two previous cases demonstrate that normal pregnancy can follow total body or thoracoabdominal irradiation.

Despite great histological polymorphism, clinical features of supratentorial brain tumors are mainly limited to neurological deficit, and focal epilepsy and/or symptoms secondary to raised intracranial pressure. The treatment relies on surgery and radiotherapy; chemotherapy has not yet demonstrated its efficacy. Failures are explained by the limitation of neurosurgery, and by the toxicity of the radiotherapy that precludes the use of high dosages. Some good vital results are hampered by neuropsychic sequellae.

Over a 20-year period (1969-1983), 570 infiltrating primary breast carcinomas were conservatively treated in Lyons. Two different protocols were used: a first group of 162 patients with T1 T2 less than 3 cm NO lesions was treated, between 1963 and 1973, by lumpectomy and external beam irradiation with 60cobalt. The 5 and 10 year overall survival rates are 86% and 66% respectively. Local and regional failure rates are 10% and 2.5%. Cosmetic result was good or excellent in 70% of patients with only 5% poor results; a second group of 408 patients with T1 T2 less than 3 cm lesions was treated between 1973 and 1983 by tumorectomy and axillary dissection, followed by cobalt irradiation and 192iridium boost. The 5-year overall survival rate was 90% and, at 5 years, the probability of failure is 5% in the breast and 2% in the axilla. Cosmetic results are comparable in the 2 groups. Comparison of these 2 groups indicates an improvement in local control with Iridium boost. No obvious change in axillary recurrence rate and overall survival rate was observed. Boost modalities are discussed, with regard to cosmetic results. A prospective randomized study was initiated in 1986, in order to assess the value of the boost in the conservative treatment of breast cancer.

From August 1991 to November 1994, we observed brain metastasis in 15 pts with colorectal cancer (CRC):4 Males/11 Females, median age 59 y (43–69), CRC staging 7B, 4C, 4D. Treatment of the primitive tremor consisted with surgery (8 pts) plus chemotherapy (CT) (4 pts) or radiation therapy (RT) (3 pts). 12 pts developed non CNS metastases mostly, hepatic and/or pulmonary (9 pts). 3 pts had isolated CNS metastases. Median time to first metastasis was 18 months (0–66) and median time to CNS metastases was 27 months (1–129). CNS treatment was RT (36 Gy) in all pts, 2 had a previous surgical excision. 10 pts died of CNS metastases, 3 pts of other metastases ± local recurrence. Median survival time was 3 months (0–17). 2 pts are alive with disease at 2 and 17 months. Brain metastases, classically unusual In CRC become more frequent, potentially because of the wider use of systematic CT. Despite RT, their prognosis remains very poor.

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.

Summary Aggressive fibromatosis (also known as desmoid tumors) arise from musculo-aponeurotic tissues and are histologically benign. They are however locally aggressive. Local relapse occurs in 20 to 100% of treated patients and is specially frequent following incomplete resection. We report 9 cases of children treated at Leon Berard Center between 1979–1991 by surgery and post operative treatment. Patients and methods Between 1979 and 1991, 9 children (7 male, 2 female) with aggressive fibromatosis were treated at Leon Berard Center. Ages ranged from 24 to 204 months. Five were seen at first relapse and 4 were treated in first intent. All specimens were reviewed by the Department of Pathology, (histological confirmation of aggressive fibromatosis was confirmed in all cases). Involved sites of disease include: limbs (4), supraclavicular (2), face (2) and sacrum (1). Initial surgery consisted of wide excision in 7 patients with a macroscopic residual disease, and a microscopic residual disease in 2 cases. Despite partial surgery in 5/9 patients who were treated at other institutions, no further post-operative treatment was proposed and these patients were referred at Centre Leon Berard with local recurrences following first surgical resection. All of them underwent a record surgery at time of relapse. Four patients were referred for initial diagnosis of desmoid tumors and had an incomplete surgery. Four children were treated with postoperative radiation, 2 with radiation and chemotherapy, 1 child with radiation therapy, chemotherapy and endocrine therapy and 1 with endocrine therapy alone. Results: Local control Six patients relapsed within 4 to 12 months from surgery. Three were controlled. Among the controlled patients, 1 received radiation therapy alone (45 Gy), the second received chemora-diation (Ifosfamide, Vincristin, Adriamycin + 45 Gy); the third received Tamoxifen (20 mg/m 2 ). Second surgery was performed on the 6 patients who failed locally: macroscopic complete resection was performed but with positive margin in 3 cases. Gross residual disease was present in two cases, the last one achieved microscopically complete resection. No additional treatment was administered to the child who underwent a complete resection; he is alive at 10 years follow up. Adjuvant treatment was delivered to 5 patients: radiation therapy alone (2 pts); chemoradia-tion and TAM (2); chemotherapy alone (1). Survival All but one patients are still alive with a 93 months median follow up. Seven of 9 children are free of recurrence at last review. Nevertheless, one is still alive but with active disease. The last one died of disease 28 months after the initial surgery by locally uncontrolled tumor. Conclusion Primary treatment is surgical resection and should be as complete as possible. This is difficult to achieve. No patient in our study was completely resected. Most of children relapsed after the initial surgery and patients may died as a consequence of locally uncontrolled tumor. Theses findings confirm that fibromatosis are aggressive tumor which warrant aggressive therapy. Local control may be achieved with adjuvant ″aggressive″ treatment such as radiotherapy.

In part I of this article, we reviewed the general aspects of childhood brain tumors. In part II, we present the most common neoplasms and their treatment. The quality of the surgical resection appears to be of first importance in the prognosis. Progress in radiotherapy and chemotherapy have contributed to the improvement of long-term results. However, they are responsible of late deleterious effects. Further progress in therapy must be assessed with in view a critical evaluation of the quality of life.

Massive chemotherapy with in vitro purged autologous bone marrow transplantation has benefited from better understanding of massive chemotherapy for solid tumors, and better search for bone marrow involvement; it may now be used for treatment of poor prognosis neuroblastomas. Authors report preliminary results of 5 cases (4 IV and 1 p III b stage of TNM classification) completely treated in one institution (Centre Léon-Bérard, Lyon, France). Three patients are in complete remission, and 2 have relapsed (median follow up: 18 months). Authors comment on these results.

The most frequent solid tumors in the pediatric age group are central nervous system tumors. Despite dramatic improvements in neurosurgery, radiotherapy and chemotherapy, the overall prognosis remains poor, and is often associated with late deleterious effects, including neuropsychologic disorders, endocrine deficiencies and physical abnormalities. In part I, we describe the common presenting symptoms and signs in children with brain tumors. We expose the significant advances in histology, immunohistochemistry, cytogenetic, radiology and therapeutic approaches during the past ten years.

One hundred and ninety six patients underwent brain irradiation for metastases between 1973 and 1981. Complete follow-up was possible in 180 cases. CT appeared to be the best diagnostic tool. Whatever the type of the tumor, irradiation improved symptoms and signs in 65.3 p. 100 of cases and neurological status in 37 p. 100 of cases. The median survival was 4.2 months, the mean survival 6.6 months, the percent survival at 1 year 11 p. 100. It is necessary to irradiate the whole brain with doses of 40-45 Gy in 4.5 weeks. Shorter periods of irradiation allows a short inhospital stay but implies anti-oedematous treatment. Chemotherapy did not improve the results. Excision surgery had limited indications: isolated metastases, accessible site in zones with no functional importance, good performance status, local control obtained for primary tumor, no extra-cerebral metastases. Surgery must be followed by whole brain irradiation. Derivation is necessary when increased intracranial pressure and treatment resistance are present. Irradiation indications are large. The results were palliative but valuable: irradiation was always indicated except when disorders of consciousness were present. Tolerance was usually good.

About 10% of patients developed severe sequelae following radiotherapy, which had been associated with surgical treatment, even though they were, or appeared to be cured. Complications affected the skin and soft tissues (causing skin retraction, loss of substance, and sometimes trismus) and bone (necrosis required surgical treatment, and when there was associated cutaneous dystrophy, resulted in exposure of the bone and severe functional and esthetic problems). A critical study of the radiotherapeutic techniques used suggests that the dose given should be reduced, the fields modified, and Cobalt used in preference to electrons in most cases.

Prophylaxis with high-titered anti CMV globulins was studied in 2 pilot trials using 2 different schedules. Results were compared with a historical group without prophylaxis.

The treatment of a certain number of children with very severe dentomaxillary sequelae after radiotherapy for facial and intracranial lesions raises many problems. These include the type of reconstructive therapy, the protection of the maxillodental structures, the value of local and alimentary hygiene, and the quantity and quality of dental treatment needed. Further difficulties are encountered because of dental malformations (absence of roots), increased rate of eruption of the teeth in the arch (contrary to the accepted opinions), and objectives should be modest when attempting maxillofacial orthopedic procedures.

Between 1976 and 1981, 160 patients with non oat-cell carcinoma of the lung underwent a post-operative irradiation. Most of these patients presented with advanced disease, 103 of them with lymphatic involvement. Adjuvant chemotherapy was performed in 48 cases and did not improve the survival rate. Crude survival is 26% at 5 years. No severe complication was related with irradiation. The rate of local relapse was reduced by the irradiation mainly if the dose was above 45 Gy. With doses between 46 and 60 Gy the 5 year survival rate was 33% (11/34) and the rate of local failure was 18%. It is possible that such an irradiation increases the survival rate. Post-operative irradiation seemed advisable after surgery when there is a risk of local residual disease.