Discal cysts and pseudocysts: Single center experience
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ABSTRACT:
SUBMITTER: Gorolay V
PROVIDER: S-EPMC11372899 | biostudies-literature | 2023 Sep
REPOSITORIES: biostudies-literature
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Project description:Due to its rarity, male breast cancer remains a poorly characterized disease. The present study obtained retrospective clinicopathological data, treatment patterns and outcomes for all male patients diagnosed with breast cancer in the Oncology Department, Faculty Hospital Tren?ín (Tren?ín, Slovakia) over the last 20 years from January 1995 to December 2015. A total of 21 patients with male breast cancer were analyzed, with a median patient age of 65.6 years. Two patients were diagnosed with lobular invasive cancer; all others were diagnosed with cancer of a ductal origin. One patient presented with metastatic disease in the pleural cavity. The primary tumors in 8 patients were staged as pT1, whilst 6 patients were staged as pT2 and 7 as pT4. Axillary lymph node involvement was present in 11 patients (52%) and 15 patients were hormone receptor-positive (83%). All but 1 patient underwent mastectomy and surgical staging of the axilla. Adjuvant chemotherapy, radiotherapy and hormone treatment was administered in the same manner as breast cancer treatment in female patients. The median follow-up time was 4.5 years. The 5- and 10-year overall survival rates were 87 and 74%, respectively, and the estimated median disease-free survival for the same population was 9.5 years (95% confidence interval, 6.2-14.6). The survival rates reported in the present retrospective study are comparable with previously published studies. In addition, the current study reported predominant hormone-positive characteristics and rare expression of human epidermal growth factor receptor 2. However, further multi-institutional trials are required to allow for informed treatment decisions in this uncommon disease.
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Project description:Context:Persistent Mullerian duct syndrome (PMDS) is a rare disorder. It is a type of male pseudohermaphroditism, usually presenting as "Hernia Uteri Inguinalis". Aims:This study aims to present our experience of PMDS, over a 7-year period. Settings and Design:Our center is a tertiary care facility, situated in Tamil Nadu, a southern state of India. Subjects and Methods:This is a retrospective study. The study period was from 2007 to 2015. Seven cases presented during that period. The difficulties in diagnosis, treatment options discussed, along with a review of literature are presented. Results:Seven cases of PMDS presented over 8 years. Only four were diagnosed preoperatively. Mullerian remnants were excised in five cases. Conclusions:PMDS is rare. Orchiopexy should be the goal of treatment.
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Project description:IntroductionPancreatic pseudocysts (PPCs) and walled-off necrosis (WON) in children following acute pancreatitis are uncommon. The various modalities of therapy possible are conservative treatment, external drainage, endoscopic stenting, and internal surgical drainage procedures. There are no existing guidelines for the management of PPC in children. We evaluate the outcomes of laparoscopic cystogastrostomy (LCG) performed at our center.Materials and methodsEight children (median age: 10 years) underwent LCG for large PPC (median size: 12.5 cm). There were seven patients with PPC and one with WON. Seven underwent LCG by a transgastric approach and one underwent LCG by a retrogastric approach.ResultsSeven out of the eight patients had complete resolution of symptoms and the PPC. The median follow-up period was 32 months (interquartile range: 9.5-55.5 months). There were no conversions. There was one patient with a WON who developed a recurrence.ConclusionLCG is a safe and effective treatment option for large PPC/WON in children. A posterior retrogastric approach, when indicated, is a safe approach with a comparable outcome.
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Project description:Eight patients of choledochal cyst have been diagnosed and six have been managed surgically. There were two children and six adults. Both the children had Type I cyst. They were managed by excision of the cysts. One lady with associated stone and carcinoma of the gall bladder underwent radical cholecystectomy with excision of segments IV and V of liver. Second lady with intrahepatic porcelain gall bladder had a choledochocele and was treated by transduodenal sphincteroplasty. Fifth patient, an 82 years old man with symptomatic gall stone with Type I choledochal cyst was subjected to just a cholecysto-jejunostomy. A 54 years old man with a Type IV cysts has undergone excision of the cyst. In patients undergoing excision of choledochal cysts, the biliary tract was reconstructed by a Roux-en-Y hepaticojejunostomy. Two of our patients never turned up for surgery. All the operated patients were asymptomatic during the follow-up period of 6 months to 24 months.
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Project description:PurposeThe number of newly diagnosed inflammatory bowel disease (IBD) cases such as ulcerative colitis (UC), Crohn's disease (CD), and indeterminate colitis (IC) is rapidly increasing in Gulf countries and Saudi Arabia. The aim of this study was to investigate the incidence and prevalence of IBD in patients who have attended the Salmaniya Medical Complex, Bahrain, between the years 1984 and 2014.Patients and methodsAll patients who had attended the Salmaniya Medical Complex, Bahrain, and had been diagnosed with UC, CD, or IC, between the years 1984 and 2014, were included in the analysis. Data collected were: patient demographics, symptoms, clinical signs, complications, surgical interventions, extent of disease, endoscopic findings, histopathology, and lab measurements.ResultsA total of 187 cases were included; 61 had CD, 123 had UC, and a further 3 cases presented with IC. A clear increase in the incidence and prevalence of IBD can be seen in this cohort. The prevalence of IBD was calculated to be 26.25/105 cases. The average number of IBD cases increased from 3 cases (average for the years 1984-2001) to 12 cases (average for the years 2002-2014). A number of factors correlate positively or negatively with CD and UC. In the current study, a link between gastrointestinal complications in CD cases and the use of steroids as a treatment was noted (p-value -0.02). Age also had a significant influence on the need for surgery in CD cases (p-value -0.04), and a family history of UC was statistically linked to surgical intervention (p-value -0.05).ConclusionsIBD can no longer be considered a rare disease in Bahrain. The incidence of both UC and CD is steadily increasing. There is a need for increasing awareness of the Bahraini public to IBD in order for proper medical care to be given.
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Project description:We investigated the surgical outcomes of patients who underwent therapeutic surgery for malignant pleural mesothelioma (MPM) at a single center.A retrospective review of 21 patients who underwent therapeutic surgery for MPM from January 2001 to June 2015 was conducted to assess their outcomes. The patients' characteristics and postoperative course, including complications, mortality, overall survival, and recurrence-free survival, were analyzed.Of the 21 patients who underwent therapeutic surgery, 15 (71.4%) underwent extrapleural pneumonectomy, 2 pleurectomy (9.5%), and 4 excision (19.1 %). The median age was 57 years (range, 32-79 years) and 15 were men (71.4%). The mean hospital stay was 16 days (range, 1-63 days). Median survival was 14.3 months. The survival rate was 54.2%, 35.6%, and 21.3% at 1, 3, and 5 years, respectively. In patients' postoperative course, heart failure was a major complication, occurring in 3 patients (14.3%). The in-hospital mortality rate was 2 of 21 (9.5%) due to a case of severe pneumonia and a case of acute heart failure.A fair 5-year survival rate of 21.3% was observed after surgical treatment. Heart failure was a major complication in our cohort. Various surgical methods can be utilized with MPM, each with its own benefits, taking into consideration the severity of the disease and the comorbidities of the patient. Patients with local recurrence may be candidates for surgical intervention, with possible satisfying results.
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Project description:ObjectiveThis study aims to investigate the imaging features of epidermoid cysts within intrapancreatic accessory spleen (ECIPAS) to benefit the differential diagnosis of pancreatic cystic lesions.MethodsWe retrospectively reviewed the clinical, radiological and pathological data of 24 patients with pathologically confirmed ECIPAS. All cases underwent abdominal contrast-enhanced computed tomography (CE-CT) scans, with 4 cases further undergoing magnetic resonance cholangiopancreatography (MRCP). The imaging features of the lesions were analyzed.ResultsThe majority of the patients were young to middle-aged with no apparent clinical symptoms. Over half of the masses exhibited circular, oval or unilocular architectures. On plain CT, the masses primarily displayed indistinct margins with an average diameter of 3.0 ± 1.1 cm. Nineteen lesions were situated in the pancreatic tail, 1 in the head, 1 in the head-body junction, and 3 in the body. In the arterial phases of enhanced CT scans, the lesions exhibited varying degrees of enhancement: slight enhancement (n = 11), significant enhancement (n = 5), and no discernible enhancement (n = 8) compared to pancreatic parenchyma. On MRCP scan, all four lesions showed hyperintense on T2-weighted imaging (T2WI).ConclusionECIPAS is a rare benign lesion, with nearly half exhibiting slightly uneven enhancement on contrast-enhanced CT scans. Usually, the solid-portion-enriched lesions show similar enhancement to that of the spleen, aiding in the precise preoperative diagnosis. ECIPAS can manifest in various locations within the pancreas, including the tail, head, and body. Patients may benefit from improved quality of life by avoiding unnecessary surgeries.
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Project description:Pheochromocytoma and paragangliomas (PPGLs) are rare neuroendocrine tumors carrying 25-40% pathogenic germline gene variants (PGVs). We evaluated clinical, laboratory, and germline molecular profile of 115 patients with pathologic (14 patients were relatives from 8 different families recruited for genetic survey) confirmed PPGL followed in our institution. Patients with classic MEN2A/MEN2B phenotypes and at-risk relatives underwent direct analysis of RET proto-oncogene, and the remaining had samples submitted to complete next-generation sequencing aiming 23 PPGL-related genes: ATM, ATR, CDKN2A, EGLN1, FH, HRAS, KIF1B, KMT2D, MAX, MDH2, MERTK, MET, NF1, PIK3CA, RET, SDHA, SDHAF2, SDHB, SDHC, SDHD, TMEM127, TP53, and VHL. We also developed a clinical judgment score (CJS) to determine the probability of patients having a potentially hereditary disease. The resulting genetic landscape showed that 67 patients (58.3%) had variants in at least one gene: 34 (50.7%) had exclusively pathogenic or likely pathogenic variants, 13 (19.4%) had pathogenic or likely pathogenic variants and variant of undetermined significance (VUS), and 20 (29.8%) carried only VUS. PGVs were found in RET (n = 18; 38.3%), VHL (n = 10; 21.3%), SDHB and NF1 (n = 8; 17% each), and MAX, SDHD, TMEM127, and TP53 (n = 1; 2.1% each). Direct genetic testing disclosed 91.3% sensitivity, 81.2% specificity, and 76.4% and 93.3% positive predictive value (PPV) and negative predictive values (NPV), respectively. The CJS to identify patients who would not benefit from genetic testing had 75% sensitivity, 96.4% specificity, and 60% and 98.2% PPV and NPV, respectively. In summary, the landscape of PPGL germline gene variants from 115 Brazilian patients resulted in slightly higher prevalent pathogenic and likely pathogenic variants, especially in the RET gene. We suggest a CJS to identify PPGL patients who would not require initial genetic evaluation, improving test specificity and reducing costs.
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Project description:BackgroundPancreatic neuroendocrine tumors (PNET) are rare, with a significant malignant potential. This study aimed to determine outcomes of patients with resected PNETs according to the cystic component and confirm the accuracy of preoperative staging.MethodsFrom 1997 to 2016, 106 patients underwent resection of PNETs, including 73 purely solid (S-PNETs, 69%), 21 mixed (M-PNETs, 20%), and 12 purely cystic lesions (C-PNETs, 11%). To ensure consistent comparisons of overall (OS) and disease-free (DFS) survival outcomes between the 3 groups, the patients were matched according to the World Health Organization (WHO) grade and tumor height.ResultsOverall, the rate of correlation between the preoperative and pathological diagnoses was low in the C-PNET group (33%, P = 0.03). None of the 24 patients (23%) with metastatic disease at the time of surgery were in the C-PNET group. Furthermore, significantly more parenchyma-sparing resections (P = 0.039) and fewer enlarged resections (P = 0.019) were achieved in the C-PNET group. C-PNET group had a significantly lower node invasion rate than the S-PNET and M-PNET groups (8% vs. 41% and 24%, P = 0.004). Although median OS was comparable in all 3 groups before (P = 0.3) and after (P = 0.18) matching, higher median DFS was observed in the C-PNET group than in the other groups after matching (P = 0.038).ConclusionC-PNET was associated with a better prognosis than PNET with a solid component. The results support a wait-and-see policy in cases wherein a reliable preoperative diagnosis remains challenging.
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Project description:GATA2 deficiency is one of the most common predisposing conditions for MDS in young individuals. It is characterized by autosomal dominant inheritance and a high rate of de novo mutations. Here we describe the clinical phenotype and hematological presentation of 10 pediatric patients with GATA2 deficiency presented to the Dmitry Rogachev Center between 2013 and 2020. All patients had been referred for neutropenia or suspected aplastic anemia. While some patients presented with an immunological phenotype, others displayed monosomy 7 and MDS. The clinical presentation with MDS in infancy and the constitutional phenotypes in our patients underline the great variability in clinical manifestation. Careful description of cohorts with GATA2 deficiency from different countries and genetic backgrounds will help to unravel the enormous heterogeneity of this recently discovered genetic disorder.
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