Metanephric adenoma diagnosed on biopsy in an infant: a case report.

BACKGROUND: Metanephric adenoma is a rare benign renal tumor of the kidney, uncommonly observed in children. It is often misdiagnosed preoperatively as a malignant neoplasm, leading to an unnecessary nephrectomy. The challenge is to make the right diagnosis preoperatively and therefore manage it with conservative surgery. We report a case of a child with metanephric adenoma who underwent nephron-sparing surgery. CASE PRESENTATION: A renal tumor was discovered fortuitously in an 18-month-old Caucasian girl with several congenital malformations. Investigations showed a 28 × 27 × 27 mm left renal mass centrally located, well defined, nonvascularized, with no calcifications and which compressed the adjacent renal tissue. Furthermore, there were no signs of metastasis. The decision of a multidisciplinary meeting was to perform a computed tomography (CT)-scan-guided biopsy. Histologic examination concluded it was a metanephric adenoma. We performed a left open partial nephrectomy via a flank retroperitoneal incision. The final histopathological examination confirmed the diagnosis. The postoperative course was uneventful. CONCLUSION: Preoperative diagnosis of metanephric adenoma is challenging. Because of the high probability of unnecessary radical nephrectomy, preoperative biopsy can be safe and determining to guide a more conservative approach so nephron-sparing surgery can be performed.

[Sudden cardiovascular death in adults: Study of 361 autopsy cases]. / La mort subite cardiovasculaire de l'adulte : étude autopsique de 361 cas.

AIMS OF THE STUDY: To describe epidemiological aspects of sudden cardiovascular death and to specify the etiopathogenic characteristics. PATIENTS AND METHOD: Our study is retrospective and descriptive. It included 361 cases of sudden cardiovascular death, which underwent autopsy in forensic medicine department of Monastir during eight years, from 1st January 2004 to 31st December 2011. RESULTS: The incidence of sudden cardiovascular death was 9 per 100,000 person. A marked male predominance was noted. The mean age was 55.75 years. In our series, myocardial infarction represents the leading cause of sudden cardiovascular death, 57.8% of cases. Other etiologies were hypertrophic cardiomyopathy (4.7%), heart failure (1.9%), arrhythmogenic right ventricular dysplasia (2.8%), valvular disease (2%), cardio-myo-pericarditis (1.9%), hydatid cyst of the heart (0.8%), ruptured aneurysm (2.5%), pulmonary embolism (1.9%) and aortic dissection (1.3%). A sudden cardiovascular death at work was found in 25 cases. These cases pose essentially a problem of imputability. CONCLUSION: Sudden cardiac death is usually the complication of underlying heart disease, sometimes overlooked. Several risk factors are involved. Sudden cardiac death in healthy heart or death caused by arrhythmia is an important entity seeking the intervention of several actors (forensic doctor, cardiologist, geneticist, media…) for prevention.

Human papillomavirus genotypes and HPV-16 variants distribution among Tunisian women with normal cytology and squamous intraepithelial lesions.

BACKGROUND: Little is known about the epidemiological characteristics of papillomavirus (HPV) infection among North African countries. Herein, we conducted a molecular epidemiological study to investigate prevalence of HPV type and HPV-16 variants among cervical-screened unvaccinated Tunisian women. METHODS: Cross-sectional study was performed on 494 Tunisian women visiting Women's Healthcare Centers. HPV-DNA detection was carried out on cervical samples using real-time polymerase chain reaction. HPV genotyping and HPV-16 variants were characterized by direct sequencing of L1 viral capsid gene. RESULTS: The overall HPV prevalence was 34% (95% CI: 30-38%) with significantly higher prevalence among women with squamous intraepithelial lesions (SIL) than those with no intraepithelial lesions (NIL) 84% (95% CI: 76-92%) and 24.5% (95% CI: 20-29%) respectively. The distribution of HPV prevalence according to women's age shows a U-shaped curve and the highest HPV prevalence rates were observed among the youngest (≤25 years; 51.2%, 95% CI: 37-67%) and the oldest women (>55 years; 41.7%, 95% The HPV-16 prevalence was 32.8% (95% CI: 22-45%) among women with SIL and 9.2% (95% CI: 6-12%) among women with NIL. Whereas, the HPV-18 prevalence was 1.3% (95% CI: 0-5%) among women with SIL and 0.3% (95% CI: 0-1%) among women with NIL. Among HPV-16 positive women, European lineage (E) was identified as the predominant HPV-16 variant (85.7%, 95% CI: 76-95%). The frequency of E variant was lower among SIL than among NIL women (81%, 95% CI: 64-99%, and 88%, 95% CI: 77-100%, respectively). Conversely, the African-2 variant frequency was higher among SIL than among NIL women (18%, 95% CI: 1-36% and 6%, 95% CI: 2-14%, respectively). In multivariate analysis, young age was the only risk factor that is independently associated with HPV infection. Moreover, HPV infection and menopause were both found to be independently associated with SIL and HSIL. CONCLUSION: HPV DNA testing should be proposed to young and menopausal Tunisian women. Considering HPV prevalence, only 13% of the Tunisian women could be protected by the bivalent HPV vaccine. These results may be helpful for designing an adapted HPV testing and vaccination program in Tunisia.

In vivo behavior of MIL-100 nanoparticles at early times after intravenous administration.

Metal-organic frameworks have shown interesting features for biomedical applications, such as drug delivery and imaging agents. The benchmarked mesoporous iron(III) trimesate MIL-100 MOF nanocarrier combines progressive release of high drug cargoes with absence of visible in vivo toxicity. Although in a previous study pharmacokinetics and biodistribution of MIL-100 nanoparticles were evaluated in the long term (from 24h to 1 month), the crucial times for drug targeting and delivery applications are shorter (up to 24h). Thus, this work aims to study the blood circulating profile and organ accumulation of MIL-100 nanocarrier at early times after administration. For this purpose, after intravenous administration to rats, both constitutive components of MIL-100 (trimesate and iron) were quantified by high performance liquid chromatography and a spectrophotometric method, respectively. The pharmacokinetic profile suggested that the nanoparticles act as a depot in the blood stream during the first hours before being cleared. Accumulation took mainly place in the liver and, in some extent, in the spleen. Nevertheless, histological studies demonstrated the absence of morphological alterations due to the presence of the particles in these organs. Liver function was however slightly altered as reflected by the increased plasma aspartate aminotransferase concentrations. Finally trimesate was progressively eliminated in urine.

Molecular genotyping of Echinococcus granulosus using formalin-fixed paraffin-embedded preparations from human isolates in unusual tissue sites.

Cystic echinococcosis (CE) caused by Echinococcus granulosus remains a serious problem worldwide for issues relating to public health and the economy. The most predominantly affected sites are the liver and the lungs, but other organs such as the heart, the spleen and the peritoneum can also be infected. Access to cysts from uncommon sites has limited genomic and molecular investigations. In the present study, genotypes of E. granulosus sensu lato were identified from formalin-fixed paraffin-embedded tissues (FF-PETs) implicated in human CE. Tissue samples were obtained from 57 patients with histologically confirmed CE. DNA samples were analysed using Egss 1 polymerase chain reaction (PCR) specific to the mitochondrial 12S rRNA gene of E. granulosus sensu stricto. All cysts were typed as E. granulosus sensu stricto with up to 35% of the liver and 16.6% of lungs being the most frequently infected, and up to 48.4% of samples being from rare sites. No correlation was found between cyst site and either the gender or the age of patients. This study demonstrates the possibility of exploiting atypical cysts using FF-PET samples and highlights the predominance of E. granulosus sensu stricto species in the Tunisian population, even in unusual infection sites.

A rare cause of death in infancy: idiopathic infantile arterial calcification.

The aim of this paper is to report the autopsy findings of an Idiopathic Infantile Arterial Calcification-new-born male and describe its follow-up. Y.R, a 23-days-old male, hasn't any relevant personal past medical or family history. The baby was weighing 3.2 kg at birth. He was breast fed and appeared to be perfectly normal. In the last 24 hours, he presented to the family doctor with vomitis, refuse of feeds without fever or diarrhea. He was diagnosed as having gastroenteritis and was medicated accordingly. A few hours later, he had an hematemese episode associated with facial cyanosis. Death occurred despite cardio-pulmonary resuscitation. Forensic autopsy was required. The macroscopic examination showed a bilateral pleural liquid effusion without any other abnormalities. Microscopic investigation revealed a generalized arterial calcification of all organs. Idiopathic arterial calcification is primarily a disease of infancy. It is characterized pathologically by generalized arterial calcification within the internal elastic lamina, associated with intimal fibrous proliferation. Death occur often in the first sixth months due to heart failure.

[Arrhythomgenic right ventricular dysplasia and sudden death: An autopsy and histological study]. / Dysplasie arythmogène du ventricule droit et mort subite : étude autopsique et histologique.

Arrhythmogenic right ventricular dysplasia (ARVD) is cardiomyopathy where normal myocardial tissue is replaced with fibrofatty tissue. Histological examination performed on myocardial biopsy or on autopsy samples are used to confirm the diagnosis. However, in many cases, the diagnosis cannot be made on a simple macroscopic and histological study and requires genetic analysis and molecular biology. In this work, we propose to describe the main macroscopic and histological findings of ARVD through the study of an autopsy series. We report 12 autopsy cases of sudden death in ARVD collected in the Department of Forensic Medicine of the University Hospital Fattouma Bourguiba Monastir (Tunisia) during a period of 20years. Microscopic examination was performed on 5microns thick histological sections. All slides were reviewed by two operators in a double blind (physician pathologist, pathologist) and in each, the percentage of adipose tissue, fibrosis and infarction in the right ventricle, left ventricle and interventricular septum, the presence or absence of inflammatory infiltrate, the presence or absence of signs of degeneration of myocytes were noticed. ARVD was found in 12 cases (1.8% of sudden cardiac death). The age ranged between 13 and 67years (mean age: 45.3years). The death occurred in half of the cases during exercise. Macroscopic examination of the RV showed the presence of a wall thinning (thickness<3mm) in 9 cases. Histological study highlight RV adipose infiltration in all cases with a percentage between 15% and 60%, fibrotic lesions were observed in only 9 cases with an average percentage of 10.25% and signs of degeneration of myocytes were noted in 10 cases. In concordance with what has been reported in the literature, there is still no consensus regarding the criteria to be adopted to pose with certainty the diagnosis of ARVD and the presence of adipose tissue remains the criterion more suggestive.

[Cervical teratoma in a child]. / Tératome cervical chez l'enfant.

Teratomas are unusual tumors derived from all 3 germs cells layers: endoderm, mesoderm, and ectoderm, with varying proportions. The cervical area is exceptionally affected. We report 4 cases of cervical teratoma. The clinically and radiologically suggested diagnosis was confirmed by histology. We describe herein the main clinical, radiological, and histological aspects and outcomes of this disease. Despite its most often benign histologic nature, cervical teratoma may threaten newborn infants' life due to airway compression. A multidisciplinary approach to the disease starting at delivery is required to improve the prognosis.

The protective effect of recombinant human erythropoietin against cisplatin-induced renal and hepatic dysfunctions in Wistar rats.

Cisplatin (Cisp) is one of the most effective chemotherapeutic drugs. However, the dose of Cisp is greatly limited by its toxicity. Recombinant human erythropoietin (rhEPO), a hormone that regulates hematopoiesis, has also been shown to exert tissue-protective effects. The purpose of this study was to explore the protective effect of rhEPO against Cisp-induced renal and liver dysfunctions. Adult male Wistar rats were divided into six groups of six each: control, rhEPO-alone group, Cisp-alone group and rhEPO + Cisp group (pretreatment, cotreatment and posttreatment conditions). Our results showed that Cisp-induced a marked renal and liver failure characterized by a significant decrease in body weight, organ weight and organ ratio and a significant increase in creatinine, blood urea nitrogen, alanine aminotransferase, aspartate aminotransferase, G-glutamyl transferase, alkaline phosphatase, bilirubin conjugated and bilirubin total levels in serum. Histological examination showed that Cisp caused kidney alterations. rhEPO treatments restored body weight, organ weight and organ ratio as well as serum biochemical parameters changed due to Cisp exposure.

Giant cell tumour of the flexor tendon sheath of the hand: analysis of 27 cases.

Giant cell tumour of the tendon sheath (GCTTS) is a slowly progressing benign tumour arising from synovial cells of tendon sheaths. It is one of the most common soft tissue tumours in the hand. We report a retrospective study of 27 proven GCTTS of the hand. The mean length of follow-up was 4.5 years (17 months-8.5 years). Radiographic findings are useful and may prove of great diagnostic value. The positive diagnosis was provided by the pathology examination after complete excision that was performed in all patients. The recurrence was noted in two surgically managed cases. The excision should be meticulous and complete in order to avoid recurrence.

Erythema nodosum revealing parathyroid carcinoma.

INTRODUCTION: Parathyroid carcinoma (PTC) is a rare parathyroid tumor. We report a case of PTC revealed by erythema nodosum (EN). CASE STUDY: A 53-year-old woman was admitted for EN exploration. Biology found hypercalcemia (3 mmol/L) and 184.89 ng/L parathormonemia. Histology following cervicotomy diagnosed PTC. Postoperative course was free of complications. At eight months' follow-up, there was no recurrence of EN. DISCUSSION/CONCLUSION: PCT is rare and life threatening. Paraneoplastic EN is rare, and has not previously been reported in association with PCT. Solid neoplasia and hemopathy should be systematically explored for in case of recurrent EN or resistance to conventional treatment.

[Malignant recurrence of "mature" sacrococcygeal teratoma. A clinicopathological study of 4 cases]. / Récidive maligne du tératome sacrococcygien "différencié". Etude anatomoclinique de 4 cas.

Sacrococcygeal teratomas (SCT) are uncommon neonatal tumours which are usually benign. Our interest was aroused by encountering patients with reportedly benign SCT which later, after surgery, recurred as malignant tumours. We conducted a retrospective study of 17 patients treated for benign SCT during a period of 9 years. Of these patients, 4 developed malignant recurrence (3 females and 1 male) with a mean age of 19 months. The average time to recurrence was 17 months. Recurrence presented as a gluteal mass in one case, urinary and digestive compression signs in another case and as elevated alpha-fetoprotein levels in the two remaining cases. Histologically, one of the original tumors included tiny immature foci but none contained a malignant component after reading slides. Recurrences were as endodermal sinus tumour in all cases. After surgery and adjuvant chemotherapy, only one child died from complications related to chemotherapy. The other three were alive and well at mean follow-up of 5 years. These results emphasise the need for close clinical and biological follow-up for at least 2 years in all patients who had undergone excision of a neonatal SCT.

Benign osteoblastoma in an unusual mastoid location.

INTRODUCTION: Benign osteoblastoma (OB) is an unusual primary bone tumor. The preferred locations are the posterior arch of vertebrae and long bones. We report herein an extremely rare location of an OB in the mastoid process of the temporal bone. CASE REPORT: A 22-year-old woman presented with painful left retro-auricular swelling. Computed tomography features were suggestive of an aggressive osteolytic lesion of the left mastoid. The pathologic examination of bone curettage material revealed a benign OB. A complete resection of the tumor was performed later, with no evidence of recurrence at 1 year. DISCUSSION/CONCLUSION: To our knowledge, this is the 14th reported case of OB confined to the mastoid process of temporal bone. Its histological diagnosis can be difficult and osteosarcoma is its principal differential diagnosis. Although generally regarded as benign, OB has potential for recurrence and local invasion. As such, complete resection, whenever possible, is preferred over conventional curettage.

[Pseudosarcomatous myofibroblastic proliferation of the bladder]. / Prolifération myofibroblastique pseudosarcomateuse de la vessie.

Myofibroblastic proliferations of the bladder in adults are unusual lesions with a benign course. These proliferations, whether spontaneous or secondary to instrumentation, have identical morphology and behaviour. Histologically, similar lesions have been reported in the literature using different names, such as inflammatory pseudotumour, pseudosarcomatous fibromyxoid tumour, nodular fasciitis, postoperative spindle cell nodule... Recently, some authors proposed that these lesions are similar enough to be considered the same entity, designated as "pseudosarcomatous myofibroblastic proliferation" and insisted on the necessity to distinguish them from the inflammatory myofibroblastic tumour of the childhood. The latter, recently recognized as tumour, has a malignant potential and is capable of giving metastases. We describe the case of a 17-year-old man who presented a vesical mass. The histopathological study concluded to a pseudosarcomatous myofibroblastic proliferation. We discuss, through a literature review, the relationship of this lesion with inflammatory myofibroblastic tumour and its main differential diagnoses.