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Objective: To assess the efficacy of 5% imiquimod cream on undifferentiated vulvar intraepithelial neoplasia (VIN), a disease caused by high-risk human papillomavirus., Design: Prospective, uncontrolled study., Setting: University hospital vulvar clinic. Patients Twelve consecutive patients treated with 5% imiquimod cream for undifferentiated VIN between March 1, 1999, and May 31, 2001., Intervention: Self-application of 5% imiquimod cream, initially 3 times a week, then adjusted according to tolerance, for up to 7 months according to clinical response., Main Outcome Measures: Therapeutic response, clinically assessed by successive photographs and histologically confirmed for complete responders, was scored as complete, partial (> or =50% decrease in lesion size), or failure. Tolerance was evaluated at each visit., Results: A total of 3, 4, and 5 patients achieved complete response, partial response (> or =75% reduction in lesion size for all such cases), and failure, respectively. Mean duration of treatment was 3.6 months (37.3 applications), 5.0 months (50.7 applications), and 3.4 months (25.2 applications) for complete responders, partial responders, and failures, respectively. Follow-up after treatment was 5 to 18, 14 to 32, and 2 to 28 months, respectively, with 1 partial responder lost to long-term follow-up. No patient developed invasive carcinoma. All but 2 patients experienced vulvar discomfort, resulting in treatment withdrawal for 3. Two patients had flulike symptoms., Conclusions: Imiquimod cream could be a therapeutic option for undifferentiated VIN. Although poorly tolerated, this self-applied treatment could spare patients, either totally or partially, the classic painful and sometimes mutilating treatments of VIN. Controlled, randomized studies are needed to evaluate its efficacy and tolerance.

Introduction: Laparoscopic surgery of the colon is becoming more and more popular. However, regarding sigmoid resection, controversy remains concerning the extent of mobilisation, particularly regarding the splenic flexure. We developed a technique for anterior resection that meets all surgical standards: the anterior approach., Materials and Methods: From October 1999 to March 2001, 50 patients with benign diseases of the colon underwent laparoscopically assisted sigmoid resection. A completely anterior approach for mobilisation of the left hemicolon was used in all cases. Positioning the patients in Trendelenburg position on the extreme right side enabled primary ligation of the inferior mesenteric vein and artery as well as complete mobilisation of the splenic flexure from the middle. A transanal circular stapling device was used to reanastomose the colon 10-12 cm from the anus., Results: There were conversion and complication rates of 10% each, and three patients needed to be reoperated. The median operating time was 180 min. Patients could be dismissed on the 14th postoperative day., Conclusion: To establish an operative standard, this technique has so far been used only for benign colon diseases. According to our experience, we think that it meets all oncological standards. Use of this technique in the treatment of malignant diseases seems therefore justified.

Introduction: Dermatofibrosarcoma protuberans is a tumor of intermediate malignancy characterized by its aggressive local growth due to pseudopodium-like outgrowths and marked propensity to recur after surgical excision. To achieve complete cure with conventional surgery, surgical margins up to 5 cm are required, leading to wide scars. Mohs' micrographic surgery is used for the removal of certain malignant tumors, both ensuring complete excision by examination of all margins as well as minimizing normal tissue loss. However, differentiating minimal residual tumor from normal skin can be difficult on the frozen sections used in Mohs' micrographic surgery. Our aim was to develop a procedure of Mohs' micrographic surgery in conjunction with rush formalin-fixed, paraffin-embedded tangential sections., Patients and Methods: Ten consecutive cases have been prospectively treated since 1998. Under local anesthesia, the tumor was first excised including lateral margin of 1 cm and a deep margin including the underlying muscle aponevrosis. A 2 to 3 mm thick horizontal section of the surgical bed was then removed, rush formalin-fixed, paraffin-embedded, tangentially sectioned, hematin-eosin stained, and eventually stained with an anti-CD34 monoclonal antibody. While waiting for pathology results, the surgical bed was not definitively closed. If excision was incomplete, an oriented complementary excision was performed., Results: Excision was complete after the first stage in 7 patients and incomplete only deeply in 3. Lateral surgical margins were reduced to 1.3 cm in all patients, facilitating wound closure: direct suture (5 patients), controlled wound healing (3 patients) or flap coverage (2 patients). No recurrence has been observed after a mean follow-up of 26 months., Discussion: The use of Mohs' micrographic surgery in conjunction with rush formalin-fixed, paraffin-embedded tangential sections reduce surgical margins in dermatofibrosarcoma protuberans. This procedure would be interesting in difficult sites, such as the genitalia, the breast, or the periarticular regions. Other cases, and longer follow-up are however necessary to validate this promising technique.

Optimal interpretation of the results of intraoperative parathyroid hormone (IOPTH) monitoring during neck exploration for primary hyperparathyroidism (pHPT) is still controversial. The reliability of the "50% rule" in multiglandular disease (MGD) is often disputed, mostly because of competing pathophysiologic paradigms. The aim of this study was to ascertain and corroborate the ability of IOPTH monitoring to detect MGD in a practice, combining conventional and alternative parathyroidectomy techniques. This is a retrospective single institution analysis of 69 consecutive patients undergoing cervical exploration for pHPT by various approaches. The IOPTH measurements were performed after induction of anesthesia but prior to skin incision and 10 minutes after excision of the first visualized enlarged parathyroid gland. In this series, 55 patients (80%) had single adenomas, and 14 patients (20%) had MGD. In 8 of the 14 patients with MGD, IOPTH levels were obtained sequentially after removal of every enlarged gland. Of these 8 patients, 6 (75%) had a false-positive decrease (decrease below 50% of baseline value in presence of another enlarged gland) failing to predict the presence of a second enlarged gland. In 2 cases IOPTH monitoring provided a true-negative result, correctly predicting MGD. If MGD is defined by gross morphologic criteria, IOPTH monitoring fails to predict the presence of MGD reliably. However, if MGD is defined by functional criteria, the course of these patients does not seem significantly affected. The importance of these findings must be further investigated, especially with regard to the outcome of minimally invasive parathyroid procedures.

Objectives: To systematically review the literature for studies reporting on the role of Mohs' micrographic (MMS) surgery in the treatment of skin tumors. To show how it is performed in France., Design: We reviewed with a quality grid all studies indexed in MEDLINE before 2003/01/01 and published in English or French. Data were extracted by two independent reviewers., Main Outcome Measures: Quality of clinical studies, recurrence rates, number of patients lost to follow-up., Results: No randomized study was found among the 493 references found. Studies of lower quality, on procedures similar to MMS, or previous systematic reviews were therefore selected. In tumors such as basal (BCC) or spinous (SCC) cell carcinoma, microcystic adnexal carcinoma, dermatofibrosarcoma protuberans, and Merkel cell carcinoma, MMS commonly induced lower recurrence rates than figures reported for conventional treatments and/or reduced surgical margins. Studies on melanoma were of low quality., Conclusions: Although no evidence-based guidelines could be developed, MMS should be used mainly for larger, morphea, micronodular or infiltrative-type, or recurrent BCCs located in danger zones, but also (sometimes with a slightly modified procedure) in microcystic adnexal carcinomas, dermatofibrosarcoma protuberans, Merkel cell carcinoma, and in aggressive forms of SCC. Randomized, controlled studies should be performed.

Background: Familial mediterranean fever belongs to the periodic fever syndromes. During the attacks, fever is associated with abdominal pain, arthralgia, or both. Cutaneous involvement occurs in 7 to 46 p. 100 of cases and mainly consists in erysipelas-like erythema. We report on three patients treated for familial Mediterranean fever who developed unusual cutaneous lesions., Observations: All the patients had long past history of familial mediterranean fever without cutaneous involvement except, for the third patient who had pseudo-erysipela. The first patient had diffuse Sweet's syndrome-like lesions, the second developed long lasting panniculitis of the thigh and the third had a persistent and lichenified erysipela-like plaque. In two patients, skin histology revealed an inflammatory infiltrate with neutrophils. In all cases, an increase in the colchicine dose led to the rapid resolution of the lesions., Discussion: In our 3 case reports, the lesions were particular because of their atypical clinical appearance, their long duration, and they differed from the usual pseudo-erysipela aspect. Histopathologically, the lesions were similar to pseudo-erysipela, which has led some authors to hypothesize that cutaneous lesions of familial mediterranean fever belong to neutrophilic dermatoses. This hypothesis is supported by the response to the increase in colchicine doses.

Introduction: Cutaneous Bowen's disease is an intra-epidermal squamous cell carcinoma., Patients and Method: Ten cases of cutaneous Bowen's disease diagnosed among 8 patients were treated by scanned high energy carbon dioxide laser between November 1996, and March 1998. A biopsy was performed in all patients before treatment., Results: The post-treatment follow-up extended from 1 to 4 years with an average follow-up of 2 years and 11 months. Only one patient, whose lesion was located on the auricle, presented a recurrence after one year. The remaining patients did not present any recurrence during their last control: six patients were followed for two years or more and one patient for one year. We demonstrate a histological and clinical correlation between the number of carbon dioxide laser passes before a clinical endpoint and the thickness of the epidermal carcinoma treated., Discussion: This new treatment has comparable efficacy to other treatments. It can be applied to extensive lesions without sequelae except for the risk of residual hypopigmentation.

Germline specific point mutations in the gene encoding fibroblast growth factor receptor 3 (FGFR3) are associated with autosomal dominant human skeletal dysplasia and craniosynostosis syndromes. Mutations identical to the germinal activating mutations found in severe skeletal dysplasias have been identified in certain types of cancer: at low frequency in multiple myeloma and cervix carcinoma and at high frequency in bladder carcinoma. We analysed, by SSCP and sequencing, the prevalence of FGFR3 mutations in 116 primary tumours of various types (upper aerodigestive tract, oesophagus, stomach, lung and skin). The regions analysed encompassed all FGFR3 point mutations previously described in severe skeletal dysplasia and cancers. No mutations were detected in the tumour types examined, suggesting that FGFR3 mutations are restricted to a few tumour types, the evidence to date suggesting that they are very specific to bladder carcinomas.

As communicating link between the deep and the superficial venous system the perforating veins are of special importance. With the introduction of the subfascial videoendoscopic dissection of the perforating veins the interest in anatomic and topographic aspects of the surgical treatment of insufficient perforators has increased. Different pathophysiological conditions can cause insufficient perforating veins. We can distinguish between a primary form and a secondary form caused by an advanced varicosis of the main superficial veins. A third entity consists in an insufficiency created by a postthrombotic alterations of the deep venous system. Primary insufficient perforators should be treated surgically. In the case of a secondary insufficiency the indication is dependent on the extent of the pathological findings. If there is an obstruction of the deep venous system which causes the insufficiency of a perforator the vein should not be divided. The videoendoscopic subfascial dissection of the insufficient perforating veins has several advantages compared with direct percutaneous exposure. The technique is easy to learn, however it requires profound knowledge about the compartmental spaces and the topographic anatomy of the perforating veins in the lower leg.

Objective: To assess the efficacy and safety of all-trans-retinoic acid (ATRA), a retinoid with antitumour activity that inhibits in vitro the growth of Kaposi's sarcoma cells, in patients with low-risk AIDS-associated Kaposi's sarcoma., Design: Non-randomized phase II study, using a group sequential procedure to determine whether the response rate to ATRA was above 10%., Setting: Nine referral French centres., Patients: Twenty HIV-seropositive men with CD4 cells > or = 200 x 10(6)/l, low-risk Kaposi's sarcoma [T0I0S0 according to the classification of AIDS Clinical Trials Group (ACTG)] not previously treated with systemic anti-Kaposi's sarcoma agents, and with at least four measurable lesions were included., Interventions: ATRA was given orally 45 mg/m2 daily for 12 weeks., Main Outcome Measure: Tumour response evaluated according to ACTG criteria., Results: Nineteen patients were evaluated for response: partial response, stabilization and progression were found in eight (42%), seven (37%), and four (21%) patients, respectively. Gradual flattening and lightening of lesions was observed in responders after at least 2 months of ATRA. All patients with partial response at week 12 pursued ATRA for another 15+/-7 weeks. Further improvement was observed in six patients. Median duration of response was 332 days. Cheilitis, transient headaches and skin dryness were the main toxicities noted. No significant changes in HIV viral burden or serum interleukin-6 pathways were observed., Conclusions: ATRA is well tolerated and effective enough in Kaposi's sarcoma patients to warrant further evaluation.

70% of the esophageal diverticula are pharyngoesophageal diverticula. They are caused by a malfunction of the superior esophageal sphincter. Surgical treatment consisted of a myotomy of the superior esophageal sphincter and the resection of the diverticulum through a lateral cervical incision. With the described new endoscopic transoral technique the septum between the diverticulum and the esophagus is divided by a stapling device and both lumina are combined resulting in a esophago-diverticulostomy. At the same time a myotomy of the transverse part of cricopharyngeal muscle and the proximal esophageal wall is performed. Due to the design of the stapling device usually a small pouch remains at the bottom of the diverticulum. A modification of the stapler instrument allowed us to shorten the length of this pouch. The time of the endoscopic procedure is markedly reduced compared to the conventional surgical intervention. There is no necessity for special postoperative wound care and the hospital stay is considerably shortened. Furthermore serious complications like fistulas and lesions of the recurrent nerve, encountered with the open surgical intervention, are avoided. This new technique combines the advantages of a minimal invasive approach and the development of new instruments with the confided principles of the conventional operation in treating the pathogenetical mechanisms of Zenker's diverticulum. To assess the value of the technique long term follow up studies as well as comparative trials will be needed.

Radioiodine and surgery are the therapeutic tools of choice for the definitive treatment of patients suffering from hyperthyroidism. The decision on the therapy to choose for an individual patient will depend on several different factors: etiology of the hyperthyroidism, size of the goiter, age and general medical considerations, availability of radioiodine therapy or of an experienced endocrine surgeon, and the patient's own preferences. It is important for the general practitioner attending and advising a patient to have a basic knowledge of both therapeutic modalities. An overview of indications, results and risks of the radioiodine and surgical therapy is presented.

Since minimally invasive surgery makes high demands on the skill, concentration and endurance of the surgeon, requisite training in this operating technique is of overriding importance. Basic training on pelvitrainers serves to enable the participant to become acquainted with the instruments and the video technique and to gain experience in handling the camera. A realistic simulation of intraoperative situations is not possible. With pulsatile organ perfusion (POP) nearly all abdominal, thoracic, vascular, urological and gynecological surgical procedures, as well as the encountered complications, can be imitated, widely replacing the need for animal experiments. Complex operations which are bound to anatomical structures, have to be practised on anaesthetized animals, as before.

Between 1988 and 1992 we treated in our hospital 79 patients with a complicated diverticular disease. We analysed and compared two groups (group A 42 cases January 1988 to February 1991/retrospectively, group B 37 cases March 1991 to December 1992/prospectively). We studied our operative procedure in respect to the postoperative morbidity and lethality, the number of performed and persistent stomata and the mean length of stay. The complications of diverticular disease in both groups were similar. Since March 1991 we performed primary resection with primary anastomosis in 89% of the patients, three-stage resection or primary resection with an anastomosis and a proximal colostomy was no more done. The Hartmann procedure was rarely chosen (only in 4 of 37 patients). In spite of forcing the primary resection with a primary anastomosis in group B, the complication rate (local and general) decreased from 35.7% to 29.7%, the lethality rate from 9.5% to 2.7%. The number of performed stomata showed a great difference with 22 in group A against 4 in group B. The mean length of stay was 31 versus 22 days. Therefore we consider primary resection with an anastomosis for the best therapy in complicated diverticular disease.

Since the introduction of laparoscopic cholecystectomy as method of choice for the treatment of gallstone disease, many authors have proposed a preoperative intravenous cholangiogram as an alternative to routine intraoperative cholangiography. To assess the value of intravenous cholangiography we performed a prospective randomized study with 100 patients undergoing cholecystectomy for gallstone disease. Patients with predefined criteria indicating possible choledocholithiasis were excluded. In none of the 50 performed i.v.-cholangiograms was evidence of a stone in the common bile duct or an anatomic variation of the biliary tract found. There was no significant difference in regard to intra- and postoperative complications between the group with preoperative cholangiography and the group without intravenous cholangiogram. We conclude that there is no significant advantage in performing routine preoperative intravenous cholangiography, and recommend the selective use of intraoperative cholangiography.

The percentages for recurrence or persistence of hyperparathyroidism after operation for pHPT vary between 3 and 7% according to the pertinent literature. These groups of authors have a broad experience based on a large number of patients treated. At our institute we have operated for pHPT an average of 5 patients per year. Given our limited experience we reexamined 100 consecutive patients which were operated for pHPT between 1968 and 1988 in order to determine our own rate of recurrence. The rate for persistent or recurrent hyperparathyroidism was at 8% slightly higher than in the literature reviewed. When reexamining the patients we took the history concerning the preoperative symptomatology and its postoperative course. Furthermore we compared a radioimmunoassay measuring the midmolecule region of the parathyroid hormone with a immunochemiluminometric assay determining the intact and biologically active hormone regarding sensitivity and specificity. The results are presented and compared with the literature reviewed.

The "in situ" bypass technique was adopted by our group in 1986. A total of 53 "in situ" bypasses were carried out until June 1989. The worst symptoms were incapacitating claudication, rest pain or ischemic necrosis, each in about one third of the patients. The site of the distal anastomosis was the popliteal artery below the knee in 40 instances, the peroneal artery in 7 and other infrapopliteal vessels in 6. Valve incisions were performed uneventfully with reusable instruments featuring a sharp-edged blade and an indicator for rotation control of the blade ("Insitutom RC"). Vein branches were located mostly by angiography, but residual arteriovenous fistula remained a vexing problem. In the beginning they were ligated under local anaesthesia. Later on they were dealt with successfully using transluminal embolization. Patients were last examined in July 1990 when the follow-up time ranged from 12 to 51 months. 4 patients had died and 5 were lost to follow-up. Patency rates were evaluated according to the life-table method. They were gratifying for femoro-popliteal bypasses with 87 percent after one year and 83 percent after two and three years. Femoro-tibial-peroneal bypasses performed below average, but their number was too small to draw conclusions.

The "in situ" bypass technique was adopted by our group in 1986. A total of 53 "in situ" bypasses were carried out until June 1989. The worst symptoms were incapacitating claudication, rest pain or ischemic necrosis, each in about one third of the patients. The site of the distal anastomosis was the popliteal artery below the knee in 40 instances, the peroneal artery in 7 and other infrapopliteal vessels in 6. Valve incisions were performed uneventfully with re-usable instruments featuring a sharp-edged blade and an indicator for rotation control of the blade ("Insitutom RG"). Vein branches were located mostly by angiography, but residual arterio-venous fistula remained a vexing problem. In the beginning they were ligated under local anaesthesia. Later on they were dealt with successfully using transluminal embolization. Patients were last examined in July 1990 when the follow-up time ranged from 12 to 51 months. 5 patients had died and 5 were lost to follow-up. Patency rates were evaluated according to the life-table method. They were gratifying for femoro-popliteal bypasses with 87% after one year and 83% after two and three years. Femoro-tibial-peroneal bypasses performed below average, but their number was too small to draw conclusions.

Five patients with penile melanosis are described. The lesions consisted of macular hyperpigmentation of the penile shaft and/or glans. Histologically only hyperpigmentation of the basal cell layer was observed with no melanocytic hyperplasia. Patients had follow-up for 3 to 13 years; enlargement of some lesions was observed, but the condition remained benign. The main differential diagnostic problem was with acral lentiginous melanoma. Penile melanosis shares clinical and microscopic features with Laugier-Hunziker syndrome of the buccal mucosa and vulvovaginal melanosis. We suggest that the three disorders be grouped as essential melanotic hyperpigmentation of mucosa.

5 cases of angiolymphoid hyperplasia with eosinophilia of the skin are reported. This rare condition was initially described in 1969 by Wells and Whimster. The microscopical pattern is characterised by an atypical vascular proliferation associated with an eosinophilic and lymphoplasmocytic granuloma. In our series, immunohistological study demonstrated the polyclonality of the plasmacytoid component. Electron microscopy performed in one case shows endothelial features even in cells forming clusters without well-defined lumen. These results support the endothelial origin of angiolymphoid hyperplasia with eosinophilia.

Cutaneous primary lymphomas excluding mycosis fungoides and Sezary Syndrome represent a rare condition. Few papers are published on the subject. The authors report a retrospective study of 16 cases concerning only these primary lymphomas. It comprises 10 adults and 6 children. In 13 cases the cutaneous tumor was solitary without extra-cutaneous lesion, in 3 cases there was a satellite simultaneous lymph node. The most frequent location was head and neck region (7 cases with 4 cases from children); limbs were concerned too (5 cases/16); more rarely trunk. Our cases were classified with reference to lennert's classification. We found that large cells lymphomas were predominant: centroblastic (4 cases), lymphoblastic (4 cases), immunoblastic lymphomas (6 cases). The clinical course was unfavourable in 50% of our cases in less than 3 years: 6 lethal cases, and 2 metastatic disseminations. These 8 cases belonged to the pejorative group of lymphomas (large cells type). The 2 cases with small cells population (centrocyto-centroblastic and lymphoplasmocytoïd type) were free of disease for 5 and 2 years after diagnosis. Thus, our experience suggests that there is a relations hip between the histologic type of cutaneous lymphoma and the course of the disease. In disseminated cases, the extra-cutaneous extension was located in lymph nodes, bone marrow, as it was mentioned in literature.

A monoclonal gammopathy was observed in 3 adult patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. The monoclonal immunoglobulin was in every case of the IgG-kappa type. Bence Jones proteinuria was noted in 1 case (kappa). Deposition of monoclonal IgG-kappa in the skin was not detected by immunofluorescence microscopy. These findings and previous reports of 5 cases suggest that diffuse scleredema may be frequently associated with paraproteinemia, but the role of monoclonal immunoglobulins in the pathogenesis of the disease remains to be clarified.