The management of retroperitoneal sarcoma: The experience of a single institution and a review of the literature.

Objectives: Retroperitoneal sarcomas (RPSs) are rare mesenchymal tumors. The objective of this study was to discuss the different clinical, therapeutic and prognostic aspects of RPS in our institution. Methods: This was a retrospective study conducted at the Department of Medical Oncology in the Habib Bourguiba University Hospital in Sfax, including 19 patients who were treated for RPSs between 1999 and 2016. Results: The median age was 49 years (range: 18-83 years); 68.4% of the patients were female. The commonest symptom was abdominal pain (88%) and the median tumor size was 15 cm (range: 4-30 cm). Complete resection was achieved in only five cases (26.3%). The most common histological subtypes were liposarcoma (47.4%) and leiomyosarcoma (26.3%). Eight patients had a high-grade tumor (G2 = 2 or G3 = 6). Adjuvant radiotherapy was administered in 5 patients (26%). Seventeen patients were treated with chemotherapy; six received chemotherapy in an adjuvant treatment, three as a neoadjuvant treatment, and eight were treated during the palliative phase. Relapse was observed in 58% of cases. For all patients, the overall survival (OS) was 89.5% at 1 year and 40.3% at 5 years. Prognostic factors influencing OS were sex (p = 0.037), resection margins (p = 0.02), recurrence (p = 0.042), and radiotherapy (p = 0.023). In multivariate analysis, radiotherapy (p = 0.031) and histological subtype (p = 0.028) were independent factors influencing OS and disease-free survival (DFS) respectively. Conclusion: We showed that the treatment of RPSs relies on surgery with complete resection. Other factors, such as radiotherapy and the occurrence of relapse, also have an impact on OS. To facilitate surgery and to obtain negative resection margins, preoperative radiotherapy is indicated in selected patients with a high risk of relapse. Further prospective trials are warranted to select optimal therapies with less toxicity and better efficacy in reducing recurrences, mainly at a local level.

A bladder stone surrounding a foreign body: A rare case.

Intra-vesical foreign bodies can be of variable nature and etiology, their introduction can be voluntary or not. The motivations most frequently associated with the presence of foreign bodies within the bladder are of a sexual or erotic nature. Various objects have been inserted into the bladder and many patients fail to remove them themselves and are very embarrassed to seek medical advice, which is the origin of a clinical picture which is most often atypical which occurs in a patient particular terrain.

Renal cell carcinoma in children, report of a new case.

Renal cell carcinoma (RCC) is rare in children and is usually found in late children. We present a case of a 14 year-old boy who presented with right lumbar pain. CT-scan showed a tumor in the upper pole of the right kidney measuring 15 cm. He underwent radical nephrectomy and histopathologic examination revealed RCC. No adjuvant therapy was given. After three years and half, there is no evidence of recurrence.

Isolated shwannoma of the bladder: A rare entity.

Isolated shwannoma of the urinary bladder is a very rare entity. We report a case of a shwannoma of the bladder that was diagnosed by an MRI and confirmed by histopathology after the patient underwent TURB.

A case of a giant adrenal cyst.

Giant adrenal cysts represent rare clinical entities that are usually discovered incidentally. Here, we describe a patient who presented with nonspecific upper abdominal left pain. Imaging studies revealed a huge left adrenal cyst. Routine laboratory tests and endocrine function tests were all normal. The patient underwent surgery, and the cyst was completely removed with total adrenalectomy. Histological examination revealed a benign epithelial adrenal cyst.The postoperative course was uneventful and the patient had no evidence of recurrence during a 3-year follow-up. We discuss the diagnosis and management of adrenal cysts.

RNA receptors, TLR3 and TLR7, are potentially associated with SLE clinical features.

The influence of intracellular Toll-like-receptors (TLR), recognized as nucleic acid sensors, in the immunopathogenesis of systemic lupus erythematosus (SLE) is increasingly explored. Yet, the results of both functional and genetic studies remain conflictual. We evaluated the association between TLR3 and TLR7 genes selected variants and SLE and investigated the possible relationship with clinical and serological parameters. Then, we studied the genetic expression of these receptors, and if the TLR7 gene evades X chromosome inactivation (XCI). Our study covers 106 cases and 200 controls, genotyped using a polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) technique. TLR3 and TLR7 expression level was assessed by qPCR carried, respectively, on renal tissues and PBMC, and methylation status was evaluated by methylation-specific PCR. Results were statistically analysed using Shesis software, χ2 , and Mann-Whitney test. Significant associations with SLE susceptibility were found for the TLR3 rs3775291, rs5743305 and rs3775294 polymorphisms. Further subgroup analysis, TLR3 rs3775291 and rs3775294 polymorphisms were significantly associated with lupus nephritis (LN) and even correlate with the presence of auto-antibodies binding RNA molecules. SLE and LN were more common in men with rs3853839-G variant within TLR7 gene versus those carrying the C allele. Moreover, the role of the G allele in the TLR7 expression up-regulation was confirmed. However, gene expression analysis showed no significant differences in TLR3 and TLR7 mRNA levels between LN patient biopsies and healthy tissues (p > .05). When comparing patients and controls, no statistical difference was observed in XCI pattern. Otherwise, notable associations were raised between TLR3 and TLR7 gene variants and clinical and serological lupus features pointing towards the role of genetic background in the physiopathogenesis of the disease.

Emphysematous prostatitis: A case report.

We report the case of a 48-year-old man with ischemic stroke, diabetes and chronic renal failure admitted for fever with LUTS associated with nausea and vomiting.The physical examination showed a depressible abdomen, painful prostate on digital rectal examination.A biological inflammatory syndrome with diabetic ketoacidosis.The abdominopelvic CT scan confirmed the diagnosis by the presence of gas in the prostate,and the CBUE was positive for Enterobacter cloacae.The patient was treated with appropriate antibiotic therapy with transrectal aspiration of the prostate collection,but the evolution was marked by the worsening of the patient's condition leading to his death after two weeks of treatment.

Polymorphisms in XRCC1, ERCC2, and ERCC3 DNA repair genes, CYP1A1 xenobiotic metabolism gene, and tobacco are associated with bladder cancer susceptibility in Tunisian population.

Other than the established environmental risk factors associated with bladder cancer (BC), little is known about the genetic variations determining the individual susceptibility of this complex disease. This study aimed to investigate the relationship of BC with environmental agents and polymorphisms in XRCC1, ERCC2, and ERCC3 DNA repair genes and CYP1A1, CYP2D6, NAT1, and NAT2 xenobiotic metabolism genes through a hospital-based case-control study in Tunisia. The selection of the single nucleotide polymorphisms (SNPs) (rs25487, rs 13181, rs415407, rs446421, rs1058172, rs4921880, and rs1208) was performed using the dbSNP database. DNA genotyping was determined by PCR-RFLP after DNA extraction from whole blood. The risks of BC associated with every polymorphism as well as the studied environmental factors were estimated by multivariate-adjusted logistic regression using R software. In addition, gene-gene interactions were analyzed using generalized multifactor dimensionality reduction (GMDR) methods. Results showed that tobacco smoking and chewing parameters were significantly associated with BC risk. Single-gene variant analysis showed significant associations of the TT genotype of CYP1A1 and the rare GG genotype of ERCC2 with bladder cancer susceptibility (OR = 1.34, 95% CI 1.22-1.40, P < 0.0001). According to GMDR analysis, our findings indicated a significant association between BC and gene-gene interaction among the CYP1A1, ERCC3, and XRCC1. The present results suggest a potential role of XRCC1, ERCC2, ERCC3, and CYP1A1 besides tobacco intake in susceptibility to BC.

Primary renal angiosarcoma: Case report and literature review.

Angiosarcoma is a rare malignant tumour occurring in less than 2% of soft tissue sarcomas. Angiosarcoma involving the kidney usually represents metastasis from skin or visceral primary lesions, while angiosarcoma primarily occurring in the kidney is a very rare neoplasm. We report a case of angiosarcoma of the right kidney in a 59-year-old male. The computed tomography scan showed a solid tumour with a low increased density after administration of contrast medium. Histological examination of the piece of nephrectomy confirmed the diagnosis.

Feasibility and oncological outcomes of laparoscopic treatment for local relapse of renal cell carcinoma.

UNLABELLED: What's known on the subject? and what does the study add?: Local relapse of renal cell carcinoma following radical nephrectomy is rare, and surgical removal provides the only opportunity for cure. Open surgery has been established as the usual approach for these tumours. It is, however, associated with significant morbidity. Our study describes the largest series of laparoscopic treatment of local relapse of renal cell carcinoma with the longest follow-up. We show that the laparoscopic approach is feasible in expert centres. It provides faster recovery and fewer complications with satisfactory oncological outcomes in selected patients. OBJECTIVE: To assess the feasibility and oncological outcomes of laparoscopic treatment for local relapse of renal cell carcinoma. PATIENTS AND METHODS: Nine patients were treated by a pure laparoscopic approach for local recurrence of a renal tumour between 2005 and 2011 by a single surgeon (HB), following an initial open radical nephrectomy for the primary tumour. Clinical and histopathological data were collected prospectively and analysed retrospectively. Seven patients were treated by a transperitoneal approach and two patients had a retroperitoneal approach. RESULTS: Relapse occurred within a mean time of 83 months (7-168) following nephrectomy. Recurrent tumour size varied from 2.5 to 4.5 cm. All surgeries were performed laparoscopically without need for conversion. Mean operative duration was 144 min (40-240), mean estimated blood loss was 430 mL (50-1300) and mean hospital stay was 4.5 days (3-6). Three patients had Clavien grade I intraoperative complications. Late complications were noted in two patients (Clavien I and IIIb). Pathology confirmed clear cell carcinoma in all patients with an absence of sarcomatoid features and negative surgical margins. Three patients had neoadjuvant treatment and two patients had adjuvant treatment. In all, 67% of patients were disease free with a mean follow-up period of 3 years. CONCLUSIONS: Surgical removal of isolated local recurrence remains the only possibility of cure in patients with renal cell carcinoma. We demonstrated that the laparoscopic approach is a safe and feasible alternative treatment option for selected cases with low morbidity and satisfactory oncological outcomes.

Cystic lymphangioma of the kidney: Diagnosis and management.

INTRODUCTION: Cystic lymphangioma is a rare benign vascular tumor that may arise in various sites, revealed at any age. Abdominal locations represent less than 10% of the cases preferentially involving the mesentery. We report a case of primary lymphangioma arising from the kidney. PRESENTATION OF CASE: A 50-year-old woman was admitted for severe left lumbar pain. Abdominal ultrasonography and computed tomography revealed a 6×10 cm multiloculated cystic mass with hydronephrosis, extending along the left renal hilum. Surgical exploration revealed a retroperitoneal cystic tumor. Anatomopathologic examination concluded it to be a cystic lymphangioma. DISCUSSION: The cystic lymphangioma is a benign malformative tumor of the lymphatic system. Surgery is the best curative treatment with complete excision, the prognosis is excellent. CONCLUSION: Primary renal lymphangioma is exceedingly rare. Medical imaging has certain limits for the diagnosis which required histological confirmation. The treatment of choice is surgical.