[Persistent Müllerian ducts syndrome]. / Syndrome de persistance des canaux de Müller.

Persistent Müllerian ducts syndrome is a rare form of internal male pseudohermaphroditism, characterized by the presence of the uterus, fallopian tubes and upper vagina in an otherwise normally virilised male with a 46 XY karyotype. It is the result of a deficiency in anti-Müllerian hormone or abnormality of this hormone receptor. Often, the diagnosis is made incidentally during surgical exploration for cryptorchidism or herniorrhaphy, and exceptionally during cancer of ectopic testis. We discuss a rare case of this syndrome revealed by a bilateral intra-abdominal gonadal tumor.

Paget disease of the male nipple.

Breast cancer occurring in the mammary gland of men is infrequent. It accounts for 0.8% of all breast cancers, which is less than one per cent of all newly diagnosed male cancers and 0.2% of male cancer deaths. However, Paget disease of the male nipple is extremely rare. We report a single case of Paget disease with infiltrative ductal carcinoma of the breast in a 61-year-old man.

[Giant epithelioid hemangioma of the hand]. / Hémangiome épithélioïde géant de la main.

Epithelioid vascular tumors are a heterogeneous group characterized by epithelioid-appearing endothelial cells. Included are benign vascular tumors (epithelioid haemangioma), borderline tumors (epithelioid haemangioendothelioma), and malignant tumors (epithelioid angiosarcoma). We describe a case of epithelioid haemangioma.

Human pharmacokinetic study of heated intraperitoneal oxaliplatin in increasingly hypotonic solutions after complete resection of peritoneal carcinomatosis.

PURPOSE: We studied the pharmacokinetics of heated intraoperative intraperitoneal (i.p.) oxaliplatin (LOHP) solution and its safety profile in increasingly hypotonic solutions. This is the first clinical study of i.p. chemohyperthermia with hypotonic solutions. METHODS: Patients with peritoneal carcinomatosis (PC) underwent complete cytoreductive surgery followed by intraoperative i.p. chemohyperthermia (IPCH) with successive dextrose solutions of 300, 200, 150 and 100 mosm/l. LOHP (460 mg/m(2)) was administered in 2 liters of solution/m(2) at an i.p. temperature of 42-44 degrees C for 30 min. IPCH was performed using an open procedure (skin pulled upwards) with a continuous closed circuit. Patients received intravenous leucovorin (20 mg/m(2)) and 5-fluorouracil (400 mg/m(2)) just before IPCH to maximize the effect of LOHP. i.p. plasma and tissue samples were analyzed by means of atomic absorption spectrophotometry. Sixteen consecutive patients with PC of either gastrointestinal or peritoneal origin were treated. The safety of the procedure was studied. RESULTS: Pharmacokinetics: The mean duration of the entire procedure was 7.7 +/- 2.6 h. Half the LOHP dose was absorbed within 30 min at all dose levels. Absorption was not higher with hypotonic solutions than with isotonic solutions. The area under the curve of LOHP in plasma did not increase with decreasing osmolarity of the i.p. solutions. Intratumoral LOHP penetration was high; it was similar to that at the peritoneal surface, and about 18 times higher than that in nonbathed tissues. LOHP penetration was not significantly increased by using hypotonic solutions. SAFETY: There was a very high incidence of unexplained postoperative peritoneal bleeding (50%) and unusually severe thrombocytopenia in the 150 and 100 mosm/l groups. CONCLUSION: Contrary to experimental studies, this clinical study showed no increase in tumoral or systemic penetration of LOHP with i.p. hypotonic solutions (200, 150 or 100 mosm/l) during IPCH. A high incidence of i.p. hemorrhage and thrombocytopenia was observed.

Heated intra-operative intraperitoneal oxaliplatin after complete resection of peritoneal carcinomatosis: pharmacokinetics and tissue distribution.

PURPOSE: This article reports the pharmacokinetics (PK) of heated intra-operative intraperitoneal oxaliplatin and its tolerance profile. Oxaliplatin has demonstrated significant activity in advanced colorectal cancer, and this is the first publication concerning its intraperitoneal administration. METHODS: Twenty consecutive patients with peritoneal carcinomatosis (PC) of either gastrointestinal or uniquely peritoneal origin underwent complete cytoreductive surgery followed by intra-operative intraperitoneal chemo-hyperthermia (IPCH) with increasing doses of oxaliplatin. We performed IPCH using an open procedure (skin pulled upwards), at an intraperitoneal temperature of 42-44 degrees C, with 2 l/m2 of 5% dextrose instillate in a closed circuit. The flow-rate was 2 l/min for 30 min. Patients received intravenous leucovorin (20 mg/m2) and 5-fluorouracil (400 mg/m2) just before the IPCH to maximize the effect of oxaliplatin. We treated at least three patients at each of the six intraperitoneal oxaliplatin dose levels (from 260 to 460 mg/m2) before progressing to the next. We analysed intraperitoneal, plasma and tissue samples with atomic absorption spectrophotometry. RESULTS: The mean duration of the entire procedure was 8.4 +/- 2.7 h. Half the oxaliplatin dose was absorbed in 30 min at all dose levels. Area under the curve (AUC) and maximal plasma concentration (Cmax) increased with dose. At the highest dose level (460 mg/m2), peritoneal oxaliplatin concentration was 25-fold that in plasma. AUCs following intraperitoneal administration were consistently inferior to historical control AUCs after intravenous oxaliplatin (130 mg/m2). Intratumoral oxaliplatin penetration was high, similar to absorption at the peritoneal surface and 17.8-fold higher than that in non-bathed tissues. Increasing instillate volume to 2.5 l/m2 instead of 2 l/m2 dramatically decreased oxaliplatin concentration and absorption. There were no deaths, nor severe haematological, renal or neurological toxicity, but we observed two fistulas and three deep abscesses. CONCLUSIONS: Heated intraperitoneal chemotherapy gives high peritoneal and tumour oxaliplatin concentrations with limited systemic absorption. We recommend an oxaliplatin dose of 460 mg/m2 in 2 l/m2 of 5% dextrose for intraperitoneal chemo-hyperthermia, at a temperature of 42-44 degrees C over 30 min. We may be able to improve these results by increasing the intraperitoneal perfusion duration or by modifying the instillate composition.

Curative treatment of peritoneal carcinomatosis arising from colorectal cancer by complete resection and intraperitoneal chemotherapy.

BACKGROUND: Peritoneal carcinomatosis (PC) is fatal, despite standard systemic chemotherapy. A new approach that combines maximal surgery with maximal regional chemotherapy has potential to cure selected patients who have colorectal PC. The authors have reported the oncologic results of this combined treatment. METHODS: The authors performed a retrospective study of 64 patients who had PC arising from colorectal adenocarcinomas, 19 (29.6%) of whom also had other metastases. These patients were treated by complete resection of all detectable tumors and by a 5-day course of early intraperitoneal chemotherapy (EPIC) with mitomycin C, then by 5-fluorouracil (n = 37), or by intraoperative intraperitoneal chemohyperthermia (IPCH) with mitomycin C, alone or combined with cisplatin (n = 27), in 2 separate trials. In the trial of IPCH, aimed at selecting the most reliable procedure in terms of spatial diffusion and thermal homogeneity, the 27 patients were treated with 7 different procedures. The extent of PC was assessed precisely by using a peritoneal index. The median follow-up period for the entire patient population was 51.7 months. RESULTS: The postoperative mortality and morbidity rates were 9.3% and 54.6%, respectively. Most severe complications occurred in patients who required extensive cytoreductive surgery. Global and disease-free survival rates were respectively 60.1% and 54.7% at 2 years and were 27.4% and 18.4% at 5 years. Results were significantly better (P = 0.04) when patients were metastasis-free (apart from PC) and when the peritoneal index was lower than 16 (P = 0.005). IPCH seemed to be more effective than EPIC for treatment of PC. CONCLUSION: This treatment plan, which combined maximal surgery with maximal regional chemotherapy, cured approximately 25% of patients. This strategy was mainly applicable to patients with limited intraperitoneal cancer volume and no extraperitoneal involvement. IPCH proved to be more effective than EPIC but more difficult to use correctly. Future results should improve through routine use of the optimal hyperthermia procedure, with improvements in the composition of instillate, better patient selection, and the reduction in the rate of complications that occurs with physician experience.

Usefulness of intraoperative radiofrequency thermoablation of liver tumours associated or not with hepatectomy.

INTRODUCTION: A probe emitting radiofrequency (RF) waves is able to destroy tumour tissue by thermal ablation. The purpose of this study was to undertake a prospective estimation of the benefit of RF thermoablation of liver tumours during hepatic and extrahepatic resections aimed at obtaining an R0 status in patients in whom disease is notoriously considered unresectable. METHOD: Twenty-one patients underwent surgery between January 1997 and September 1999. In 17 cases, RF was associated with a hepatectomy and in nine of these cases with resection of extrahepatic lesions. In two cases, extensive resection of extrahepatic lesions was associated with RF to treat liver metastases, and in two cases RF was ultimately performed alone. The mean number of liver metastases was 6.2+/-4.3 (range 1-15) per patient. A total of 32 lesions were treated with RF. The mean size of the 33 RF-thermoablated tumours was 13.6+/-9.7 mm (range 5-52 mm), and in all but one case, a Pringle manoeuvre was performed during the RF procedure. RESULTS: A probable R0-resection was obtained in 18 cases. No operative deaths or any RF-related complications occurred. If we exclude the case in which it was clearly impossible to destroy liver metastases intraoperatively, only one local recurrence occurred (3%) among the 32 thermoablated lesions after a mean follow-up of 17.3 months. The 2-year overall and disease-free survival rates for this initially unresectable population were 94.7% and 22%, respectively. CONCLUSION: Intraoperative use of RF to destroy unresectable liver tumours increases the rate of curative resections. Future progress in RF technology and adequate vascular clamping during RF should increase this rate.

[Bone metastasis revealing a primary prostate cancer]. / Métastase osseuse révélatrice d'un cancer prostatique simulant une tumeur primitive.

Spiculated reactive periosteal bone is usually found in cases of primary malignant bone tumors; rarely in secondary bone lesions. The authors report a case of bone tumor of the clavicle in a 62 year-old patient with "sunburst " periosteal reaction on radiographs. The metastatic nature of this lesion from an unknown prostate carcinoma was confirmed by immunohistochemical studies of the clavicle biopsy. Tumorectomy was performed and the patient's status is stable with a follow-up of 10 months.

[Squamous cell carcinoma in an augmentation of the ilial bladder for tuberculosis]. / Carcinome malpighien sur iléocystoplastie d'agrandissement pour vessie tuberculeuse.

The development of cancer on the ileal graft after augmentation ileocystoplasty benign bladder disease is a little known complication. The authors report a case of squamous cell carcinoma in the ileal bladder occurring 31 years after augmentation ileocystoplasty for tuberculous bladder, in a 60-year-old patient. The ileal bladder was resected and a new augmentation ileocystoplasty was performed. The postoperative course was uneventful. Histological examination of the operative specimen showed infiltration of all of the intestinal wall. The patient died one year after, in a context of peritoneal carcinomatosis.

[Primary leiomyosarcoma of the kidney. A case report]. / Léiomyosarcome primitif du rein. A propos d'un cas.

Primary sarcomas of the kidney are exceptional, representing 1 to 3% of all renal tumours in adults. They have a poor prognosis. The authors report a case of primary leiomyosarcoma of the kidney in a 44-year old patient, presenting in the form of low back pain and haematuria. Treatment consisted of radical nephrectomy. Two months later, the patient presented with hepatic metastases. The patient is currently receiving chemotherapy.

[Reno-sigmoid fistula in a tuberculous pelvic ectopic kidney]. / Fistule réno-sigmoïdienne sur un rein ectopique pelvien tuberculeux.

Renocolic fistulas are rare lesions, generally involving the ascending and descending colon. The authors report a case of reno-sigmoid fistula occurring in a tuberculous pelvic ectopic kidney. Nephrectomy with segmental sigmoid resection was completed by antituberculous treatment for 9 months. The patient presents a good general condition with normal renal function and normal intestinal transit with a follow-up of 19 months.