Adenoid cystic carcinoma of Bartholin's gland, a case report with genomic data and literature review.

Adenoid cystic carcinoma of the Bartholin's gland (ACCBG) is a rare, slowly but aggressive malignancy. We reported the case of a 31-year-old woman who was treated by local excision and then hemi-vulvectomy, with positive margins and perineural invasion. Radiation therapy (RT) was then performed delivering 45Gy in 25 fractions in bilateral inguinal lymph nodes and 64.8Gy in 36 fractions on the vulvar area. After 30 months, there was no local relapse (LR) but the patient presented a histologically documented lung recurrence. Genomic profiling of the tumor showed a MYB-NFIB fusion transcript and a somatic mutation of PLCG1. A treatment by Lenvatinib was started. We conducted a literature review of 100 published cases. Patients were mainly treated by radical vulvectomy (30%), hemi-vulvectomy (17%), wide or local excision (21% and 24%, respectively) or other. Forty-four percent of patients received postoperative RT, more frequently in case of positive margin (71.9% versus 29.5%). RT may reduce the risk of LR regardless of margin status, with 15.4% vs. 41.9% of LR with or without RT, respectively, in patients with negative margins, and 13% vs. 33.3% of LR with or without RT, respectively, in patients with positive margins. The risk of relapse of any type was 40.9% in patients who received adjuvant RT vs. 48.2% in patients who did not. Median time to relapse was 24 months (range 6-156 months). The most frequent metastatic sites were lung (76.7%) and bone (26.7%). Optimal treatment for ACCBG is still not clearly defined but pooling the data from published case report help us better understand this rare disease and help in the therapeutic decision.

Tunneled catheters in hemodialysis: Indications and complications.

OBJECTIVE: Tunneled central venous catheters (TCVC) are a good alternative in case that an arteriovenous fistula (AVF) cannot be made, awaiting maturation or during unscheduled hemodialysis. We report the experience in placing these catheters by nephrologists and establish the various indications and complications in hemodialysis patients. We also relate the lifespan of tunneled catheters in our series. MATERIALS AND METHODS: Our study is a retrospective, descriptive and analytical, study carried out in nephrology department, including all the patients who benefited from a TCVC between June 2018 to June 2020. We included a total of 138 patients, Glomerular nephropathy was the main cause of end stage renal disease (ESRD) (34.6%). In total, 116 patients previously had an AVF. The average duration of use of TCVC was 140.62 days±106.104 [10-548 days]. The most frequent indication for placement of the tunneled catheter was pending for AVF to be made. We identified 64 (46%) cases who presented with complications per procedure (mechanical and hemorrhagic), the delayed complications were present in 12.3% of patients with 56.5% who presented with infectious complications in our series, and 43.5% of them experienced thrombotic complications. Two deaths in our series were related to a septic shock and a hemothorax. Mechanical and thrombotic complications occurred more frequently in women (P=0.032, P=0.018, respectively). For bleeding complications, the significant risk factor during our study was thrombocytopenia (P=0.027). Infectious complications, were linked to seniority in hemodialysis (P=0.014). Thrombotic complications were related to history of heart disease and anemia<8g/dL (P=0.01; P=0.043, respectively). CONCLUSION: The first indication of a CVC was a pending for AVF. Even if the per procedure complications were more frequent, they were benign in most cases. The delayed complications were mostly infectious and thrombotic. The lifespan of TCVC in our series was short since the main indication of the removal was the maturation of an AVF.

Incidental diagnosis of Covid-19 pneumonia on chest computed tomography.

PURPOSE: The purpose of this study was to determine the prevalence and imaging characteristics of incidentally diagnosed COVID-19 pneumonia on computed tomography (CT). MATERIALS AND METHODS: This retrospective study was conducted between March 20th and March 31st, 2020 at Cochin hospital, Paris France. Thoracic CT examinations of all patients referred for another reason than a suspicion of SARS-CoV-2 infection were reviewed. CT images were analyzed by a chest radiologist to confirm the presence of findings consistent with COVID-19 pneumonia and quantify disease extent. Clinical and biological data (C-reactive protein serum level [CRP] and white blood cell count) of patients with CT findings suggestive for COVID-19 pneumonia were retrieved from the electronic medical chart. RESULTS: During the study period, among 205 diagnostic CT examinations, six examinations (6/205, 3%) in 6 different patients (4 men, 2 women; median age, 57 years) revealed images highly suggestive of COVID-19 pneumonia. The final diagnosis was confirmed by RT-PCR. Three inpatients were suspected of extra thoracic infection whereas three outpatients were either fully asymptomatic or presented with fatigue only. All had increased CRP serum level and lymphopenia. Disease extent on CT was mild to moderate in 5/6 patients (83%) and severe in 1/6 patient (17%). CONCLUSION: Cumulative incidence of fortuitous diagnosis if COVID-19 pneumonia did not exceed 3% during the highest pandemic phase and was predominantly associated with limited lung involvement.

COVID-19 pneumonia: A review of typical CT findings and differential diagnosis.

The standard of reference for confirming COVID-19 relies on microbiological tests such as real-time polymerase chain reaction (RT-PCR) or sequencing. However, these tests might not be available in an emergency setting. Computed tomography (CT) can be used as an important complement for the diagnosis of COVID-19 pneumonia in the current epidemic context. In this review, we present the typical CT features of COVID-19 pneumonia and discuss the main differential diagnosis.

Cumulative radiation dose after lung transplantation in patients with cystic fibrosis.

PURPOSE: The purpose of this study was first to evaluate the imaging-related cumulative post-transplantation radiation dose in cystic fibrosis (CF) lung transplantation (LT) recipients and second, to identify the occurrence and type of malignancies observed after LT. MATERIALS AND METHODS: A total of 52 patients with CF who underwent LT at our institution between January 2001 and December 2006 with at least 3 years of survival were retrospectively included. There were 27 men and 25 women with a mean age of 24.4±9.2 (SD) years (range: 7.6-52.9 years) at the time of LT. Calculation of cumulative effective and organ doses after LT were based on dosimetry information and acquisition parameters of each examination. Cumulative radiation doses were calculated until June 2016, but stopped at the time of de novomalignancy diagnosis, for patients developing the condition. RESULTS: Patients received a mean cumulative effective dose of 110.0±51.6 (SD) mSv (range: 13-261.3 mSv) over a mean follow-up of 8.1±3.6 (SD) years (range: 0.5-13.5 years), with more than 100mSv in 5 years in 19/52 patients (37%). Chest CT accounted for 73% of the cumulative effective dose. Mean doses to the lung, breast and thyroid were 152.8±61.1 (SD) mGy (range: 21.2-331.6 mGy), 106.5±43.2 (SD) mGy (range: 11.9-221.4 mGy) and 72.7±31.8 (SD) mGy (range: 9.5-165.0 mGy), respectively. Nine out of 52 patients (17%) developed a total of 10 de novo malignancies, all but one attributable to immunosuppression after a mean post-transplantation follow-up period of 11.1±3.5 (SD) years (range: 3.7-16.3 years). Six-month cumulative effective dose was not greater in patients with de novomalignancies than in those without de novomalignancies (28.9±14.5 (SD) mGy (range: 13.0-53.4) vs 25.6±15.3 (range: 5.0-69.7), respectively, P>0.05). CONCLUSION: The cumulative effective dose exceeded 100 mSv in 5 years in 37% of LT recipients, the reason why continuous efforts should be made to optimize chest CT acquisitions accounting for 73% of the radiation dose.

[Bronchiectasis imaging]. / Imagerie des dilatations des bronches.

Bronchiectasis are defined as an irreversible focal or diffuse dilatation of the bronchi and can be associated with significant morbidity. The prevalence is currently increasing, probably due to an increased use of thoracic computed tomography (CT). Indeed, the diagnosis relies on imaging and chest CT is the gold standard technique. The main diagnosis criterion is an increased bronchial diameter as compared to that of the companion artery. However, false positives are possible when the artery diameter is decreased, which is called pseudo-bronchiectasis. Other features such as the lack of bronchial tapering, and visibility of bronchi within 1cm of the pleural surface are also diagnostic criteria, and other CT features of bronchial disease are commonly seen. Thoracic imaging also allows severity assessment and long-term monitoring of structural abnormalities. The distribution pattern and the presence of associated findings on chest CT help identifying specific causes of bronchiectasis. Lung MRI and ultra-low dose CT and are promising imaging modalities that may play a role in the future. The objectives of this review are to describe imaging features for the diagnosis and severity assessment of bronchiectasis, to review findings suggesting the cause of bronchiectasis, and to present the new developments in bronchiectasis imaging.

[New TNM classification of non-small cell lung cancer]. / Nouvelle classification TNM des cancers du poumon non à petites cellules.

Initial staging is a key part of the initial evaluation of non-small cell lung cancer. It relies on the 7th edition of the TNM classification. Proposals have been recently developed for the 8th edition of the classification, which is due to be enacted in early 2017. Among these proposals, the weight of tumor size has been increased and new N descriptors have been introduced to further describe N category depending on the number station involved. Regarding M descriptors, oligometastatic disease is distinguished from multiple distant extrathoracic metastases.

[Computed tomography imaging of non-small cell lung cancer]. / Imagerie par tomodensitométrie du cancer bronchique non à petites cellules.

Computed tomography (CT) plays a key role in the initial evaluation of non-small cell lung cancer. It allows initial staging and helps targeting lesions for pathological analysis. The aim of initial imaging work-up is to differentiate between localized disease, eligible to a local treatment, and advanced disease requiring medical treatment. CT is very useful for the assessment of local extension but is less accurate than positron emission tomography (PET)-CT for the assessment of lymphatic and metastatic spread. However, initial staging should include CT examination of the brain and upper abdomen, and PET-CT should be only be performed in patients eligible to a local treatment after initial CT assessment. Propositions for the 8th edition of lung cancer TNM bring several changes for T staging. In particular, the weight of lesion size is increased. Similarly, N1 and N2 stages are now divided in subgroups according the number of involved stations.

The immunophenotype of adult T acute lymphoblastic leukemia in Morocco.

BACKGROUND: There is paucity of detailed studies of adult T cell acute lymphoblastic leukemia (T-ALL) in developing countries reflecting the condition of these patients including clinical and biological features. OBJECTIVE: This study was carried out to analyze the immunophenotypic characteristics of 40 Moroccan patients with T-ALL and its association with biological and clinical features. PATIENTS AND METHODS: Between 2006 and 2009, 130 adult patients diagnosed with acute lymphoblastic leukemia (ALL) were immunophenotyped by 3-color flow cytometry using a panel of monoclonal antibodies. Cases presenting features of a T-lineage phenotype were subjected to detailed analysis including immunophenotypic, clinical and biological parameters. RESULTS: Proportion of T-ALL among ALL Moroccan patients was 31.0%. Median age of patients was 28 years. Twenty-nine patients were females and 11 were males. 45.0% of patients (18/40) had features of immature T-ALL stages (pro-T and pre-T ALL), 30.0% (12/40) of CD1a+ cortical T-ALL stage and 25.0% (10/40) had a characteristic phenotype of medullary T-ALL. The frequencies of progenitor cell markers CD10, CD34 and TdT expression were 14.0; 57.5% and 50.0% respectively. The aberrant expression of B lineage associated antigen CD79a were positive in 20.5% of the cases and the aberrant expression of myeloid antigens CD13 and/or CD33 was found in 22 (55.0%) cases. No significant association was encountered between TdT, CD34 or myeloid antigens positivity and high risk features at presentation as age, sex, and white blood cells. However, myeloid antigens (CD13 and/or CD33) was significantly associated with T-cell maturation stages (p = 0.009). CONCLUSION: To the best of our knowledge, this is the first report from North Africa of immunophenotypic study on adult T-ALL. Our findings indicate that the proportion of T-ALL among ALL in Morocco is similar to that reported in others Mediterranean countries like France and Italy and that myeloid-associated antigens expression is frequently associated with immature immunophenotype.

Clinical importance of myeloid antigen expression in Moroccan patients with adult B-lineage acute lymphoblastic leukemia.

The prognostic significance of myeloid antigen (MyAg) expression in acute lymphoblastic leukemias (ALL), especially in adult patients, is still controversial. In the present report, frequency and clinical significance of MyAg (CD13 and/or CD33) in blast cells were assessed in 80 consecutive adult (≥18 years) patients with B-lineage acute lymphoblastic leukemia (B-ALL), representing 66.7% of 120 patients diagnosed as having ALL during the study period. Immunophenotyping was used to classify leukemic cells as Bor Tlymphoblasts and to identify the aberrant expression of myeloid-associated antigens. MyAg expression was documented in 52.5% of the 80 B-ALL cases analyzed. CD13 was the most commonly antigen expressed (36.3%) followed by CD33 (28.8%). No significant associations were found between the expression of MyAg and the presence of known adverse prognostic features (eg: age>30 years, male gender, high WBC count and Philadelphia positivity). Also, we failed to observe any statistically significant difference between MyAg-positive and MyAg-negative patients in terms of achievement of complete remission and overall survival at 3 years. This study demonstrates that the presence of MyAg on lymphoblastic cells lacks prognostic value In Moroccan patients with adult B-ALL.

Tumeurs des deux surrenales. A propos d'un cas

Les tumeurs surrenaliennes bilaterales sont rares. Nous rapportons un cas de pheochromocytome surrenalien associe a un corticosurrenalome controlateral chez une patiente de 65 ans. L'association d'une hypertension arterielle a des cephalees; sueurs; et palpitations a oriente vers le diagnostic de pheochromocytome. Le bilan biologique base sur le dosage urinaire des derives methoxyles a confirme ce diagnostic. L'echographie et le scanner ont montre la presence de deux masses surrenaliennes bilaterales de structure tissulaire. Les deux masses sont discretement rehaussees apres injection du produit de contraste. Devant ce tableau clinique; biologique et morphologique; le diagnostic de pheochromocytome surrenalien bilateral est retenu. Par consequent une surrenalectomie bilaterale par voie bi-sous-costale a ete realisee. L'examen anatomopathologique a revele la coexistence d'un pheochromocytome sur la piece de surrenalectomie droite et d'un carcinome corticosurrenalien sur la piece de surrenalectomie gauche; ce qui etait imprevu. Les suites operatoires ont ete bonnes; sans recidive; avec un recul de 4 ans. A la lumiere de cette observation et d'une revue de la litterature; nous discutons les differentes caracteristiques de cette association inhabituelle

[Factor VIII hyperactivity: an arterio-venous thrombogenic factor. Case report]. / Hyperactivité du facteur VIII: un facteur thrombogène artério-veineux. A propos d'un cas.

Thrombosis is the principal mechanism in vascular pathology, whether cerebral, coronary or peripheral. During the initial stages of infarction, coagulation contributes to vascular occlusion, the haemostatic factors playing a determining role in the development of atherothrombotic lesions. An increase in a coagulation protein, besides any lowering of anticoagulation protein levels, is a risk factor for thrombosis. Among these pro-coagulant factors, the pro-thrombogenic action of factor VIII has without doubt been studied the least. We report the case of a 62 year old patient with a personal and family history of many previous thrombotic episodes, both arterial and venous, in whom factor VIII hyperactivity was discovered after a myocardial infarction. This case underlines the association of the factor VIII complex with thrombosis, and its clinical repercussions, especially the incidence of coronary pathology.

[Hydatic kidney cyst: 90 case reports]. / Kyste hydatique du rein: à propos de 90 cas.

The hydatid cyst of kidney is rare, it ranks third among all visceral localisations. The authors report a series of 90 cases renal hydatid cyst from 1972 to 2000. The middle age is 36 years with female predominance. Renal hydatid cyst often has a suggestive clinical presentation; flank mass in 84%, pain in 74% and sometimes a specific presentation hydaturia in 29%. The hydatid serology is positive in 55% and preferring ultrasonography and computed tomography in diagnosis of renal hydatid cyst. Surgical treatment is now well defined. Conservative treatment occupes a predominant place 84% and resection of the proeminent dome is usually efficient. Total nephrectomy should only be considered in the case of a completely destroyed kidney (16%) of cases. Post-operative course is generally uneventful and reexpansion of renal parenchyma is observed in 93% indicating the benign nature of this disease.

A baseline-controlled, open-label, flexible dose-escalation study to assess the safety and efficacy of sildenafil citrate (Viagra) in patients with erectile dysfunction.

Although sildenafil citrate (Viagra) has demonstrated effectiveness in the treatment of erectile dysfunction (ED), the dosing regimens often used in clinical trials may not always match those employed in clinical practice. This study was undertaken to further assess the efficacy and safety of sildenafil taken as required in male outpatients 18 years of age and older with ED (n=71). It was conducted as a placebo-baseline-controlled, open-label, flexible dose-escalation study, with sildenafil (25,50, or 100 mg) administered for 8 weeks following a 4-week placebo run-in. Efficacy variables included questions 3 and 4 of the International Index of Erectile Function (IIEF), other IIEF domains, patient event logs, and quality-of-life (QOL) assessments. Treatment with sildenafil resulted in improvements from baseline in all IIEF domains analyzed (all P<0.0001), as well as overall QOL and amelioration of specific sexual and social relationships (all P&<0.0001). Sildenafil was well tolerated. One participant discontinued treatment because of adverse events. Results suggest that flexible dosing with oral sildenafil is safe and has beneficial effects on all indices of erectile function and QOL.

[Emphysematous pyelonephritis: review of the literature concerning a case report]. / La pyélonéphrite emphysémateuse: revue de la littérature à propos d'un patient décédé.

Emphysematous pyelonephritis is defined as the presence of gas-producing bacteria in the kidney and in peri-nephretic areas. Even if it is rare, the mortality rate of this affection is between 50% and 90%. The E. coli is responsible in 60% of the cases. We report a case of a 50 years old male patient, with under diagnosed diabetics, how is admitted with pains in the left flank, fever, troubled urine, hematuria and worsening of general state. The diagnostic of emphysematous pyelonephritis was confirmed by CT Scan. In spite of adapted antibiotherapy to the renal function, insulinotherapy and correction of hydro-electrolytic troubles, the patient died with septic shock associated to digestive bleeding. Based on this case and a review of the literature, the authors describe the different features of this disease. Only an urgent nephrectomy after a short reanimation can improve the prognostic.

[Retroperitoneal fibrosis with pelvic extension: a case report]. / La fibrose rétropéritonéale à extension pelvienne: à propos d'un cas.

The retroperitoneal fibrosis is a rare pathology, whose physiopathologic mechanism is poorly known. It characterizes by the training of a benign fibrous prevertebral plate. This plate is in rule limited between the renal pedicle in high and the promontory anointed down. However, extensions to the great peritoneal cavity, the mediastinum and especially the pelvis have been brought. Authors bring a case of retroperitoneal fibrosis at a woman of 54 years, that spreads to the pelvic region complicated of an obstructive renal insufficiency. The ureteral catheterism has allowed the standardization of the renal function. The uroCT-scan has allowed to make a complete lesion statement. The patient has been operated on with realization of ureterolysis and ureteral intraperitonization. Through this observation, therapeutic and diagnostic aspects will be discussed.

[Bladder stone surrounding a foreign body: a case report]. / Lithiase vésicale englobant un corps étranger: à propos d'un cas.

The bladder can be the site of various foreign bodies. We report one case of bladder stone including a foreign body in a 24 years old man with a psychomotor deficiency who was admitted for pyuria, block miction and bladder symptoms. The pelvic X-Ray film showed a bladder stone including a sewing needle. We analysed the diagnosis, aspect and therapeutic management of this case.

[Incidence, causes and prognostic factors of hyponatremia in intensive care]. / Incidence, étiologies et facteurs pronostiques de l'hyponatrémie en réanimation.

PURPOSE: The incidence of hyponatremia is unknown, their causes are multiple. The higher mortality, especially in intensive care units, is currently unexplained. The objective of this article is to evaluate the incidence of hyponatremia, to assess their causes and to identify predictors of prognosis in intensive care units. METHODS: We included retrospectively all patients admitted at department of medical intensive care unit between January 1996 and February 2001, who presented at the admission, an hyponatremia (< 130 mmol/l). We excluded all patients who presented a hospital acquired hyponatremia, or hyponatremia associated with hyperglycemia > 13 mmol/l or with mannitol administration. Data were analysed by univariate methods, then by multivariate analysis. RESULTS: During the study period, 300 patients were identified among 2188: the incidence was 13.7% with 95% confidence interval (95% CI) between 9.8 % and 16.7%. Hypovolemic hyponatremia was observed in 25.7%, hypervolemic in 23.7% and normovolemic in 50.6%. In-hospital mortality was 37.7% (95% CI: 31.8% - 42.3%). Nine data were significantly associated with higher mortality in univariate analysis, but only 5 were identified as independant predictors of hospital mortality in multivariate analysis: hyponatremia < 125 mmol/l with a significant relative risk (RR) (RR = 2.10; 95% CI: 1.43-3.08; p < 0.001), Glasgow score < 9 (RR = 2.66; 95% CI: 1.25-5.66; p = 0.01), Glasgow score between 9 and 14 (RR = 1.94; 95% CI: 1.31-2.88; p < 0.001), shock (RR = 1.80; 95% CI: 1.10-3.05; p = 0.02) and blood urea concentration > 10 mmol/l (RR = 1.59; 95% CI : 1.08-2.34; p = 0.02). CONCLUSION: The frequency of hyponatremia is high; the normovolemic type represented 50%. Mortality is linked, in greater part, to organs dysfunction, but the severity of hyponatremia remained a significant predictor of mortality.

[Postpartum cardiomyopathy revealed by acute lower limb ischemia]. / Cardiomyopathie du péripartum révélée par l'ischémie aiguë d'un membre inférieur.

Peripartum cardiomyopathy is an uncommon disease defined as a dilated cardiomyopathy during puerperium, with left ventricular dysfunction (ejection fraction < 45%) without any other etiology. The etiology of this disease remains uncertain and it can be revealed in a variety of ways. Thrombo-embolic complications may be, although infrequently, the initial manifestation of peripartum cardiomyopathy, which is usually an intracardiac thrombosis. Lower extremity embolism is uncommon. The case reported is about a 39-year-old woman, multiparous, who presented, 40 days after delivery, a global heart failure with atrial fibrillation, revealed by left lower extremity thromboembolism. After echocardiographic and etiologic examinations, the diagnosis was established as peripartum cardiomyopathy. It evolved favourably after 2 months of medical treatment: the symptoms and cardiomegaly decreased, left ventricular systolic function was improved.

[Local recurrence of paratesticular rhabdomyosarcoma]. / Récidive locale d'un rhabdomyosarcome paratesticulaire.

We report an uncommon case of scrotal recurrence of embryonal paratesticular rhabdomyosarcoma in 19 year old man after 3 years later. The diagnosis was suspected clinically and confirmed by histopathology study after resection of the scrotal tumor. About this case, the authors discuss the diagnosis and the management of this tumor.