Prenatal diagnosis of pentalogy of Cantrell in the third trimester.

We report about the prenatal diagnosis of pentalogy of Cantrell in the third trimester. In this case sonographic evaluation revealed mild form of ectopia cordis, severe omphalocele, small meningomyelocele, mild hydrocephalus, severe polyhydramnios and allantoic cyst of the umbilical cord. The pregnancy was terminated by a caesarean section at 35 weeks of pregnancy because of severe polyhydramnios. The neonate died shortly after delivery and the obduction confirmed the diagnosis of pentalogy of Cantrell. We discuss the reason of the late prenatal diagnosis in this case, the importance of early prenatal diagnosis and the options of pregnancy management.

Gemcitabine-oxaliplatin combination for ovarian cancer resistant to taxane-platinum treatment: a phase II study from the GINECO group.

Advanced ovarian carcinoma in early progression (<6 months) (AOCEP) is considered resistant to most cytotoxic drugs. Gemcitabine (GE) and oxaliplatin (OXA) have shown single-agent activity in relapsed ovarian cancer. Their combination was tested in patients with AOCEP in phase II study. Fifty patients pre-treated with platinum-taxane received q3w administration of OXA (100 mg m(-2), d1) and GE (1000 mg m(-2), d1, d8, 100-min infusion). Patient characteristics were a : median age 64 years (range 46-79),and 1 (84%) or 2 (16%) earlier lines of treatment. Haematological toxicity included grade 3-4 neutropaenia (33%), anaemia (8%), and thrombocytopaenia (19%). Febrile neutropaenia occurred in 3%. Non-haematological toxicity included grade 2-3 nausea or vomiting (34%), grade 3 fatigue (25%),and grade 2 alopecia (24%). Eighteen (37%) patients experienced response. Median progression-free (PF) and overall survivals (OS) were 4.6 and 11.4 months, respectively. The OXA-GE combination has high activity and acceptable toxicity in AOCEP patients. A comparison of the doublet OXA-GE with single-agent treatment is warranted.

[Retrospective analysis of 108 ductal carcinomas in situ of the breast treated by radiosurgery association]. / Analyse rétrospective de 108 carcinomes canalaires in situ mammaires traités par association radiochirurgicale.

PURPOSE: To evaluate survival and prognostic factors of 108 patients with clinically or mammographically detected ductal carcinoma in situ (DCIS), treated from 1980 to 1996 by complete local excision followed by external irradiation. PATIENTS AND METHODS: The median age was 51 (range 37-80). All the patients underwent surgery consisting of a wide resection of the mammary gland harbouring the tumour. The surgical specimens were sent to the pathologists to get information on histology and margin clearance; all the slides were reviewed by one of us to assess the tumoral diameter. External beam therapy was delivered within 8 weeks after surgery. The prescribed irradiation dose was 50 Gy in 25 fractions to be given in 5 weeks. The median duration of follow-up was 93 months (range 40-173). RESULTS: There were nine patients with local recurrence (8.3%); three patients had local recurrence of DCIS and six patients developed invasive breast cancer. The treatment of local recurrence consisted of mastectomy with or without axillary dissection (eight cases) and quadrantectomy (one case). The 5-year and 10-year ipsilateral recurrence-free rate was respectively 92 and 89%. The 10-year cause specific survival was 100%. In univariate analysis, size>or=10 mm, age<45 years old and margin status were significant P=0,02, P=0,03, P=0,005; margin status was significant in multivariate analysis (P<0,02). CONCLUSION: These results are in keeping with those of the literature. They could be improved by the mass screening campaign, which is going on since January 1990 among women aged 50-74 years.

[Cardiac metastasis from a Merkel cell skin carcinoma. A case report]. / Métastase cardiaque d'un carcinome cutané à cellules de Merkel. A propos d'un cas.

The authors report the case of a cardiac metastasis of a Market cell skin tumour in a 72 year old woman, presenting with chest pain and infero-lateral myocardial ischaemia simulating an acute coronary syndrome. The diagnosis, suspected on echocardiography, was confirmed by thoracic CT scan. Markel cell carcinoma is a rare skin tumour classified among the malignant neuroendocrine tumours. It has a high metastatic potential, especially to the gastrointestinal tract and the lung. On the other hand, cardiac metastases are quite exceptionally rare.

[Adjuvant radiotherapy after radical prostatectomy. Apropos of a series of 73 patients in Lyons (France)]. / La radiothérapie adjuvante après prostatectomie radicale. A propos d'une série lyonnaise de 73 patients.

PURPOSE: Descriptive analysis of adjuvant radiation therapy after radical prostatectomy. MATERIALS AND METHODS: From 1986 to 1993, 73 patients (median age, 64.5 years; Gleason score > or = 7 : 36 pts; T1:22; T2:40; T3:11) were included into the study. On the operative specimen, the cancer grades were pT2:5 (involvement of the apex), pT3:67, pT4:1, pN1-2:8. Radiation therapy was performed after a mean resting period of 112 days. The target volume was the prostatic area. The technique used was a four-field box with an 18 MV-X photon beam. The dose was 50 Gy/20 fractions/5 weeks. No hormonal treatment was administered, except for 5 patients for a short duration. RESULTS: The median follow up was 46 months. One anastomotic local failure was salvaged by trans-urethral resection, three distant metastatic failures. Out of 72 patients with a PSA < 3 ng/mL at the end of radiotherapy, 13 showed an isolated elevation. The 5-year overall survival rate was 93%. The event-free survival was 72% after 5 years. Pathological differentiation and Gleason score were significantly correlated with the survival. There was no complication related to radiotherapy. CONCLUSION: Elective adjuvant radiation therapy for pT3 prostate adenocarcinoma after radical prostatectomy provides a good local control with minimal morbidity.

Curative endocavitary irradiation of small rectal cancers and preoperative radiotherapy in T2 T3 (T4) rectal cancer. A brief overview of the Lyon experience.

The aim of this study was the analysis of 414 patients treated by endocavitary irradiation for small T1 (T2) infiltrating adenocarcinomas between 1951-93 and of 337 patients treated by preoperative radiotherapy for T2 T3 (T4) rectal cancer, between 1978-92. Endocavitary irradiation was delivered with Papillon's technique using the PHILLIPS RT-50 machine. Preoperative external beam radiotherapy was given to the posterior pelvis only with an accelerated schedule of 39 Gy in 13 fractions over 18 days. Endocavitary irradiation with the use of intra-rectal ultrasound for patient selection resulted in a local control rate of 91% with no complication even in the medically inoperable patients. Preoperative external beam radiotherapy followed by radical resection resulted in a 90% pelvic control rate. Sphincter-sparing surgery was possible in 60% of patients with low or middle rectal lesions.

[Radiotherapy in cancer of the rectum]. / La radiothérapie dans le cancer du rectum.

The response of adenocarcinoma of rectum to radiotherapy is dependent on the use of irradiation techniques that ensure a sufficient dose be applied to the tumor without exceeding tolerance limits of pelvic tissues. This has been clearly demonstrated by results of contact radiotherapy over the last 30 years or so. Doses of about 100 Gy provide local control in 90% of cases and a 5 year survival rate of 80% in patients with highly selected tumors. For several years now, contact radiotherapy has been associated with external irradiation and iridium therapy with resulting sterilization of T2 and even T3 or N1 tumors in inoperable cases. Endorectal ultrasound imaging is essential for selecting those patients requiring radiotherapy alone and for evaluating the results, but in more than 90% of patients, surgery remains basic treatment for cancer of rectum. The incidence of local recurrence, very difficult to treat, is non negligible, however, but combining radiotherapy with surgery has been shown to reduce the rate of by 50%. Opinions differ on the relative efficacy of pre- or post-operative radiotherapy, but tolerance to this treatment is good in both cases if the technique used limits radiation to the posterior pelvis. Results of a Swedish randomized trial demonstrated greater efficacy for pre-operative radiotherapy for controlling local spread. Meta analysis showed that irradiation produces a definite gain in local control with a resulting gain in survival of about 5 to 10%. In the USA, chemotherapy based on 5 FU combined with post-operative radiotherapy was equally favorable in terms of survival. Pre-operative radiotherapy may also increase the chance of conservation of the sphincter. Although numerous points concerning chronology and mode of treatment remain open to discussion, a combination of radiotherapy and surgery now appears as standard treatment for rectal cancer.