Good outcome of liver transplantation in patients with pre-existing renal cell carcinoma.

The presence of a pre-existing or recent extra-hepatic solid tumor was considered for a long time as an absolute contraindication to liver transplantation, by fear of futility with an unacceptable increase in non-liver-related mortality. However, cancer-related mortality in solid malignancies is heterogeneous, and experts suggest that case-by-case multidisciplinary decisions should be made. Here, we report the cases of 3 patients with favorable oncological and liver outcome in patients with renal cell carcinoma detected during pre-transplant evaluation that nonetheless underwent liver transplantation.

[Pilonidal cyst of the scalp following a single minor trauma]. / Kyste pilonidal du scalp secondaire à un traumatisme inaperçu : à propos d'un cas.

The pilonidal cyst is an inflammatory disease occurring when a hair follicle penetrates inside a surgical wound. Uncommon in the scalp region, the pilonidal sinus is typically encountered in the sacrococcygeal regions. A single minor trauma of the scalp can be an inductor factor. The surgical treatment includes one-piece excision of the cyst followed by direct closure. We report a case of a 4-year-old child presenting a pilonidal sinus of the scalp following a single minor trauma.

Traitement Chirurgical Des Brides Cervicales Post Brûlure: À Propos De 47 Cas.

When their treatment is not optimal, deep cervical burns often develop retractions, ranging between simple vertical retractions to major global ones. They generate functional, aesthetic and psychological sequelae. Surgical treatment, adapted to both the patient and the type of retraction, has several goals including restoring the neck-chin angle, restoring a correct aesthetic aspect, and allowing social rehabilitation. In our unit, we managed 47 patients (mean age 22) with neck contractures over 8 years, from 2012-2020. Scald is more frequent in children while flame burns prevail in adults. Most (58%) patients healed spontaneously. Moderate and severe contractures (Achauer's classification) accounted for 30.6 and 38.8% respectively. In most (94%) patients, the contracture leads to a distortion of the neck-chin angle and limits the extension of the neck. Surgery techniques were skin grafts (67.3%), local plasties (24.5%) and flaps (8.2%). During the follow-up, results were considered as good in 83%, acceptable in 8.5%, while 8.5% needed another surgery. Plasties (Z, IC, VY…) and local flaps (with or without skin expansions) are indicated in minor contractures. Authors still debate on the best technique (graft or flap) for severe and major ones. Post-operative rehabilitation is a cornerstone for good results.

Management of pancreatic ascites complicating alcoholic chronic pancreatitis.

INTRODUCTION: Pancreatic ascites (PA) is an unusual and little studied complication of chronic alcoholic pancreatitis. Management is complex and is based mainly on empirical data. The aim of this retrospective work was to analyse the management of PA at our centre. PATIENTS AND METHODS: A total of 24 patients with PA complicating chronic alcoholic pancreatitis were managed at the Lille University Hospital between 2004 and 2018. Treatment was initially medical and then, in case of failure, interventional (endoscopic, radiological and/or surgical). Data regarding epidemiology, therapeutic and follow-up data were collected retrospectively. RESULTS: Twenty-four patients were analysed; median follow-up was 18.5 months [6.75-34.25]. Exclusively medical treatment was effective in three of four patients, but, based on intention to treat, medical therapy alone was effective in only two out of 24 patients. Of 17 patients treated endoscopically, treatment was successful in 15 of them. Of the 15 who underwent surgery, external surgical drainage was effective in 13. Multimodal treatment, initiated after 6.5 days [4-13.5] of medical treatment, was effective in 12 out of 14 patients. In total, 21 patients were successfully treated (87%) with a morbidity rate of 79% and a mortality rate of 12.5% (n=3). CONCLUSION: PA gives rise to significant morbidity and mortality. Conservative medical treatment has only a limited role. If medical treatment fails, endoscopic and then surgical treatment allow a favourable outcome in more than 80% of patients.

Distal pancreatectomy with superior mesenteric vein resection without reconstruction for pancreatic neuroendocrine tumor.

Mesenteric and portal vein involvement is seen frequently in pancreatic neoplastic disease and requires venous resection to obtain clear surgical margins. If sufficient collateral circulation is present, venous resection can be performed without reconstruction and without substantial impact on venous drainage.

Predictive factors for utilization of a low-volume center in pancreatic surgery: A nationwide study.

STUDY OBJECTIVE: It has been demonstrated that mortality following pancreatectomy is correlated with surgical volume. However, up until now, no French study has focused on predictive factors to undergo pancreatectomy in low-volume centers. The objective of this study is to analyze the clinical characteristics, socio-economic status and medical density according to surgical volume and to analyze predictive factors for undergoing pancreatectomy in low-volume centers. PATIENTS AND METHODS: All patients who underwent pancreatectomy in France from 2012 to 2015 were identified fromthe PMSI database. Hopsitals were classified as low, intermediate and high volume (<10, 11-19, ≥20 resections/year, respectively). Clinical and socioeconomic data, travel distance and rurality were assesed to identify factors associated with undergoing pancreatectomy at low-volume hospitals. RESULTS: In overall, 12,333 patients were included. Those who underwent pancreatectomy in low-volume centers were more likely older, had high Charlson comorbidity index (CCI), had low socioeconomic status, and resided in rural locations.distance traveled by patients operated on in low-volume centers was significantly shorter (23 vs. 61km, P<0.001). In multivariable analysis, older age (P=0.04), CCI≥4 (P=0.008), short travel distance (P<0.001), low socio-economic status (P<0.001) and rurality (P<0.001) were associated withundergoing pancreatectomy in low-volume centers. CONCLUSION: Patients continue to undergo pancreatectomy at low-volume hospitals is due not only to clinical parameters, but also to socioeconomic and environmental factors. These factors should be taken into account in process of pancreatic surgery centralization.

The management of gallbladder polyps.

Neoplastic gallbladder polyps (NGP) are rare; the prevalence in the overall population is less than 10%. NGP are associated with a risk of malignant degeneration and must be distinguished from other benign gallbladder polypoid lesions that occur more frequently. NGP are adenomas and the main risk associated with their management is to fail to detect their progression to gallbladder cancer, which is associated with a particular poor prognosis. The conclusions of the recent European recommendations have a low level of evidence, based essentially on retrospective small-volume studies. Abdominal sonography is the first line study for diagnosis and follow-up for NGP. To prevent the onset of gallbladder cancer, or treat malignant degeneration in its early phases, all NGP larger than 10mm, or symptomatic, or larger than 6mm with associated risk factors for cancer (age over 50, sessile polyp, Indian ethnicity, or patient with primary sclerosing cholangitis) are indications for cholecystectomy. Apart from these situations, simple sonographic surveillance is recommended for at least five years; if the NGP increases in size by more than 2mm in size, cholecystectomy is indicated. Laparoscopic cholecystectomy is possible but if the surgeon feels that the risk of intra-operative gallbladder perforation is high, conversion to laparotomy should be preferred to avoid potential intra-abdominal tumoral dissemination. When malignant NGP is suspected (size greater than 15mm, signs of locoregional extension on imaging), a comprehensive imaging workup should be performed to search for liver extension: in this setting, radical surgery should be considered.

[Risk factors for progression of coronary artery calcification over 5 years in hemodialysis patients]. / Facteurs de risque de progression des calcifications des artères coronaires après 5 ans d'évolution en dialyse.

BACKGROUND: Although progression of coronary artery calcification (CAC) has been established as an important marker for cardiovascular morbidity, very few studies have studied it in end-stage renal disease patients. Thus we examined and evaluate risk factors of calcification changes in dialysis patients. METHOD: Among 28 hemodialysis (HD) patients, CAC was measured in Agatston units at baseline and after five years using the 64 multi-slice ultra-fast CT. The HD patients were classified as progressors or no progressors according to the change in the CAC score across these 2 measurements. RESULTS: Over an average 63 months follow-up, participants without CAC at baseline had no incident CAC. The progression of CAC was slow and was found only in 6 patients (21.4%). It was significantly associated with several cardiovascular risk factors, namely, older age (P=0.03), diabetes (P=0.05), male sex (P=0.02), hypercholesterolemia (P=0.05), anemia (P=0.017), inflammation (P=0.05), and hyperphosphataemia (P=0.012). However, calcemia, parathormone levels, dialysis duration, tobacco, high blood pressure and dialysis dose did not seem to influence the progression of CAC in our series. A strong association was found between basal calcification scores and Delta increment at 5 years. CONCLUSIONS: Our study suggests that CAC progression in dialysis is a complex phenomenon, associated with several risk factors with special regard to elevated basal scores. This progression can be avoided or slowed with appropriate management, which must begin in the early stages of chronic kidney disease.

Impact of cirrhosis in patients undergoing laparoscopic liver resection in a nationwide multicentre survey.

BACKGROUND: The aim was to analyse the impact of cirrhosis on short-term outcomes after laparoscopic liver resection (LLR) in a multicentre national cohort study. METHODS: This retrospective study included all patients undergoing LLR in 27 centres between 2000 and 2017. Cirrhosis was defined as F4 fibrosis on pathological examination. Short-term outcomes of patients with and without liver cirrhosis were compared after propensity score matching by centre volume, demographic and tumour characteristics, and extent of resection. RESULTS: Among 3150 patients included, LLR was performed in 774 patients with (24·6 per cent) and 2376 (75·4 per cent) without cirrhosis. Severe complication and mortality rates in patients with cirrhosis were 10·6 and 2·6 per cent respectively. Posthepatectomy liver failure (PHLF) developed in 3·6 per cent of patients with cirrhosis and was the major cause of death (11 of 20 patients). After matching, patients with cirrhosis tended to have higher rates of severe complications (odds ratio (OR) 1·74, 95 per cent c.i. 0·92 to 3·41; P = 0·096) and PHLF (OR 7·13, 0·91 to 323·10; P = 0·068) than those without cirrhosis. They also had a higher risk of death (OR 5·13, 1·08 to 48·61; P = 0·039). Rates of cardiorespiratory complications (P = 0·338), bile leakage (P = 0·286) and reoperation (P = 0·352) were similar in the two groups. Patients with cirrhosis had a longer hospital stay than those without (11 versus 8 days; P = 0·018). Centre expertise was an independent protective factor against PHLF in patients with cirrhosis (OR 0·33, 0·14 to 0·76; P = 0·010). CONCLUSION: Underlying cirrhosis remains an independent risk factor for impaired outcomes in patients undergoing LLR, even in expert centres.

ANTECEDENTES: El objetivo de este estudio fue analizar el impacto de la cirrosis en los resultados a corto plazo después de la resección hepática laparoscópica (laparoscopic liver resection, LLR) en un estudio de cohortes multicéntrico nacional. MÉTODOS: Este estudio retrospectivo incluyó todos los pacientes sometidos a LLR en 27 centros entre 2000 y 2017. La cirrosis se definió como fibrosis F4 en el examen histopatológico. Los resultados a corto plazo de los pacientes con hígado cirrótico (cirrhotic liver CL) (pacientes CL) y los pacientes con hígado no cirrótico (non-cirrhotic liver, NCL) (pacientes NCL) se compararon después de realizar un emparejamiento por puntaje de propension del volumen del centro, las características demográficas y del tumor, y la extensión de la resección. RESULTADOS: Del total de 3.150 pacientes incluidos, se realizó LLR en 774 (24,6%) pacientes CL y en 2.376 (75,4%) pacientes NCL. Las tasas de complicaciones graves y mortalidad en el grupo de pacientes CL fueron del 10,6% y 2,6%, respectivamente. La insuficiencia hepática posterior a la hepatectomía (post-hepatectomy liver failure, PHLF) fue la principal causa de mortalidad (55% de los casos) y se produjo en el 3,6% de los casos en pacientes CL. Después del emparejamiento, los pacientes CL tendieron a tener tasas más altas de complicaciones graves (razón de oportunidades, odds ratio, OR 1,74; i.c. del 95% 0,92-0,41; P = 0,096) y de PHLF (OR 7,13; i.c. del 95% 0,91-323,10; P = 0,068) en comparación con los pacientes NCL. Los pacientes CL estuvieron expuestos a un mayor riesgo de mortalidad (OR 5,13; i.c. del 95% 1,08-48,6; P = 0,039) en comparación con los pacientes NCL. Los pacientes CL presentaron tasas similares de complicaciones cardiorrespiratorias graves (P = 0,338), de fuga biliar (P = 0,286) y de reintervenciones (P = 0,352) que los pacientes NCL. Los pacientes CL tuvieron una estancia hospitalaria más larga (11 versus 8 días; P = 0,018) que los pacientes NCL. La experiencia del centro fue un factor protector independiente de PHLF (OR 0,33; i.c. del 95% 0,14-0,76; P = 0,010) pacientes CL. CONCLUSIÓN: La presencia de cirrosis subyacente sigue siendo un factor de riesgo independiente de peores resultados en pacientes sometidos a resección hepática laparoscópica, incluso en centros con experiencia.

Do complications following pancreatic resections impact hospital costs in France: Medico-economic study on 127 patients.

OBJECTIVE: To define the cost of pancreatectomies and to identify factors associated with increased hospital costs after pancreatic resection. METHODS: All patients undergoing pancreatic surgery in our department between January 2008 and December 2014 were included. All complications occurring during hospitalization or in the 90-day period after discharge were documented. The hospital costs were analyzed and predictive factors of increased hospital costs were determined. RESULTS: One hundred and twenty seven patients were identified. Most patients underwent pancreatectomy for malignant tumors (70%). Median hospital costs were 21,392 [15,998-29,667] euros. Age (P=0.011) and preoperative jaundice (P<0.001) were associated with higher hospital costs. Intraoperative surgical time and blood loss were correlated with increased costs (P=0.001 and P=0.002, respectively). Pancreatoduodenectomy was associated with statistically significantly higher costs compared to distal pancreatectomy (21,770 vs. 15,422 euros, P=0.001). Severe postoperative complications (Clavien-Dindo grade≥3) (P=0.001), septic complications (P=0.002) and hemorrhage (P=0.001) statistically significantly increased costs. In multivariate analysis, septic (P=0.003) and severe complications (P=0.01) were statistically significantly associated with increased hospital costs. CONCLUSION: Pancreatic surgery is associated with high hospital costs, essentially related to postoperative complications.

Evaluation of a novel multiplex RT-qPCR assay for the quantification of leukemia-associated BCR-ABL1 translocation.

Although monitoring of BCR-ABL1 translocation has become an established practice in the management of chronic myeloid leukemia (CML), the detection limit of the BCR-ABL1 transcripts needs more standardization. The aim of the present study was to evaluate the clinical performances of a novel assay for the quantification of BCR-ABL1 fusion transcripts (e13a2 and e14a2) and ABL1 in a single reaction. This assay is based on the real-time reverse transcription polymerase chain reaction (RT-qPCR) in multiplex format. In a retrospective comparative clinical study performed in a reference laboratory, RNA was extracted from 48 CML patient blood samples with various BCR-ABL1/ABL1 ratios and RT-qPCR was performed using either MAScIR assay or the RT-qPCR simplex reference assay used in routine clinical testing. The comparative clinical results showed high qualitative and quantitative concordance (correlation coefficient >0.95) between MAScIR and the reference assays. The present study illustrates the utility of MAScIR assay as a sensitive, rapid, and cost-effective quantitative device to monitor the BCR-ABL1 ratios by RT-qPCR on whole blood of diagnosed Philadelphia chromosome-positive (Ph+) leukemia patients. This test could be used as an aid in the assessment of molecular response to available treatments.

Quantitative real-time polymerase chain reaction as an efficient molecular tool for detecting minimal residual disease in Moroccan chronic myeloid leukemia patients.

Chronic myeloid leukemia (CML) is characterized by BCR-ABL translocation and an increased number and migration of immature myeloid cells into the peripheral blood. The detection limit of the BCR-ABL transcript, particularly after treatment, is controversial. In the present study, we used quantitative real-time reverse transcription-polymerase chain reaction (RT-qPCR) to monitor BCR-ABL expression in Moroccan CML patients undergoing imatinib treatment, and compared the results with those of conventional PCR and fluorescence in situ hybridization (FISH). The aim of this study was to establish a new molecular tool for in vitro diagnosis of CML. In a retrospective comparative analysis, 20 CML Moroccan patients who had received imatinib treatment (N = 20) were analyzed by real-time PCR, conventional RT-PCR, and FISH. Half of the samples analyzed (N = 10) were positive for BCR-ABL gene expression, while the other half (N = 10) were negative according to conventional PCR. Interestingly, 5 of the 10 samples shown to be negative by conventional PCR showed positive expression of the BCR-ABL gene according to RT-qPCR. The RT-qPCR results were confirmed by FISH, which revealed a high concordance (100%) rate. We found that real-time RT-qPCR is more reliable and should be used in Moroccan biomedical analysis laboratories to monitor CML progression, particularly for minimal residual disease, following imatinib treatment.

[Screening and risk factors of cardiac calcification in hemodialysis: contribution of ultra-fast multi-slice scanner and transthoracic echocardiography]. / Dépistage et facteurs de risque des calcifications cardiaques chez l'hémodialysé : apport du scanner multi-coupe ultra-rapide et de l'échocardiographie transthoracique.

INTRODUCTION: Cardiovascular disease is the first leading cause of death in hemodialysis patients. In this population, cardiovascular calcifications occur at an earlier age and progress faster than in general population. PATIENTS AND METHODS: In order to determine the prevalence and risk factors of cardiac calcifications, 49 patients on chronic hemodialysis were screened in the coronary arteries and cardiac valves by the 64 multi-slice ultra-fast CT and the transthoracic echocardiography. Different clinical and biological parameters were studied by the SPSS 10.0 statistical software to determine risk factors. RESULT: Cardiac calcifications were identified in 81.6% of cases in at least one of the two studied sites. The coronary artery involvement was more common than valvular and concerned 69.4% of cases. The mean Agatston coronary artery calcium score (ACACS) was 331.1 and 522.2 in coronary patients and was correlated to alteration of systolic function of LV (r=-0.287, P=0.045). The severity of CACS was positively correlated with age (r=0.332, P=0.02). Coronary calcifications were associated with cardiovascular risk common to those of the general population (age, male sex, systolic blood pressure, diabetes, history of ischemic heart disease), but also to a lesser quality of dialysis. Valvular calcifications were present in 49% of cases and were correlated with left ventricular hypertrophy (P=0.006). The exclusive involvement of the aortic valve was the most common valvular abnormality. Phosphocalcic and lipid parameters, levels of hemoglobin, CRP and uric acid did not predisposed to cardiac calcifications in our patients. DISCUSSION: In hemodialysis patients, the pathogenesis of cardiovascular calcification is complex and cannot be attributed to a passive process. This process involves several factors that can promote or inhibit calcification. The new multi-slice ultra-fast scanner is a very sensitive method for topographic and quantitative assessment of coronary calcification and is a better alternative to invasive techniques. CONCLUSION: Our study confirms the high prevalence of cardiac calcification in hemodialysis, and highlights the importance of early screening and treatment of predisposing factors.

Predictors of paravalvular aortic regurgitation following self-expanding Medtronic CoreValve implantation: the role of annulus size, degree of calcification, and balloon size during pre-implantation valvuloplasty and implant depth.

OBJECTIVES: We sought to investigate the role of balloon size during pre-implantation valvuloplasty in predicting AR and optimal Medtronic CoreValve (MCS) implantation depth. BACKGROUND: Paravalvular aortic regurgitation (AR) is common following MCS implantation. A number of anatomical and procedural variables have been proposed as determinants of AR including degree of valve calcification, valve undersizing and implantation depth. METHODS: We conducted a multicenter retrospective analysis of 282 patients who had undergone MCS implantation with prior cardiac CT annular sizing between 2007 and 2011. Native valve minimum (Dmin), maximum (Dmax) and arithmetic mean (Dmean) annulus diameters as well as agatston calcium score were recorded. Nominal and achieved balloon size was also recorded. AR was assessed using contrast angiography at the end of each procedure. Implant depth was measured as the mean distance from the nadir of the non- and left coronary sinuses to the distal valve frame angiographically. RESULTS: 29 mm and 26 mm MCS were implanted in 60% and 39% of patients respectively. The majority of patients (N=165) developed AR <2 following MCS implantation. AR ≥3 was observed in 16% of the study population. High agatston calcium score and Dmean were found to be independent predictors of AR ≥3 in multivariate analysis (P<0.0001). Nominal balloon diameter and the number of balloon inflations did not influence AR. However a small achieved balloon diameter-to-Dmean ratio (≤0.85) showed modest correlation with AR ≥3 (P=0.04). This observation was made irrespective of the degree of valve calcification. A small MCS size-to-Dmean ratio is also associated with AR ≥3 (P=0.001). A mean implantation depth of ≥8+2mm was also associated with AR ≥3. Implantation depth of ≥12 mm was associated with small MCS diameter-to-Dmean ratio and increased 30-day mortality. CONCLUSION: CT measured aortic annulus diameter and agatston calcium score remain important predictors of significant AR. Other procedural predictors include valve undersizing and low implantation depth. A small achieved balloon diameter-to-Dmean ratio might also predict AR ≥3. Our findings confirm that a small achieved balloon size during pre-implantation valvuloplasty predicts moderate-severe AR in addition to previously documented factors.

Specific BRCA1 gene variations amongst young Moroccan breast cancer patients.

Germline mutations in the BRCA1 gene are known predictive markers for the development of hereditary breast cancer. Nevertheless, no comprehensive study has been performed targeting the presence and relevance of BRCA1 mutations in Moroccan breast cancer patients. We here present an analysis of BRCA1 gene regions (exon 2 and exon 11a/b) of 50 female Moroccan breast cancer patients with early disease onset (≤ 40 years) or familial disease backgrounds. Results showed that no mutation was present in either exon 2 or exon 11a of the BRCA1 gene in any of the 50 patients analysed. However, in exon 11b, a mutation generated by a nucleotide exchange was detected in 8% of patients, most of whom were young women (≤ 40). This mutation leads to substitution of the amino acid glutamine by an arginine at position 356 of the polypeptide sequence (Q356R). Although this mutation was previously characterised at a lower frequency in western populations, our study is the first to describe it in a young Moroccan population. Furthermore, another mutation was detected with a high frequency (4%) on exon 11b of the BRCA1 gene in exclusively young patients (≤ 40). This mutation was silent, encoding the same threonine residue at position 327 (T327T) as the wild type. The present study is the first to describe this mutation as well, particularly in a young Moroccan population. Analysis of a larger population is required in order to highlight the relevance of the Q356R and T327T mutations in young Moroccan breast cancer patients.

Bilateral ewing sarcoma/primitive neuroectodermal tumor of the breast: a very rare entity and review of the literature.

Peripheral primitive neuroectodermal tumors (PNET) are rare malignant tumors, affecting mostly children and adolescents and have been described in breast in eight case reports only. In this paper, we present a case of bilateral mammary ES/PNET where distinction between primary and metastatic diseases was discussed through a literature review. The aim of this work is to demonstrate that although rare, the possibility of PNET should be kept in mind while evaluating a palpable breast abnormality in a young female.

Metastatic desmoplastic small round cell tumor controlled by an anthracycline-based regimen: review of the role of chemotherapy.

Desmoplastic small round cell tumors (DSRCT) are rare and aggressive malignant neoplasms that usually occur in young males. They usually present intraabdominally with widespread peritoneal involvement at diagnosis and are sometimes associated with lymphadenopathies and liver metastases. Intraabdominal DSRCT is usually disseminated; no curative outcome has yet been achieved. We report a case of metastatic intraabdominal DSRCT controlled by an anthracycline-based regimen. A literature review of the treatment options, mainly chemotherapy, available for this unusual neoplasm, will be discussed. In patients with unresectable or metastatic disease, symptom control is most important because treatment modalities minimally impact survival. Palliative chemotherapy (mainly monotherapy) is preferable.

[Primary malignant schwannoma of the buccal branch of facial nerve]. / Schwannome malin primitif du rameaux buccal supérieur du nerf facial.

INTRODUCTION: Primary malignant schwannomas are rare neoplasms of nerve sheath origin, especially in the location of the head and neck where few cases are described in the literature. REPORT CASE: We report the case of a 65-year-old male diagnosed with malignant schwannoma in the left cheek. The patient underwent surgery with wide local excision, reconstruction were made later by skin graft. DISCUSSION: The treatment of choice is radical excision of the lesion with wide margins. In fact, to reduce local tumor recurrence, the use of adjuvant radiation or chemotherapy is still controversial.

Growing teratoma syndrome and peritoneal gliomatosis.

The growing teratoma syndrome (GTS) is defined as a detection of an enlarged mass during or after chemotherapy treatment for germ cell tumor. We report a case of an 18-year-old girl treated for growing teratoma syndrome after chemotherapy for malignant germ cell tumor of the ovary associated with peritoneal gliomatosis. Chemotherapy induced normalisation of alpha-fetoprotein rate whereas there was an enlargement of the mass. Subsequent complete resection was performed, and the patient remained in good control for 60 months. This clinical picture suggested the diagnosis of "GTS". This syndrome can lead to confusion with progression or relapse of a germ cell tumour because of increase in tumour volume during chemotherapy, so it is important to recognize it.

[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.