Fetal Inguinal Hernia: Case Report and Review of the Literature.

Fetal inguinal hernia (FIH) is a rare event and only few cases were published in the medical literature. In the present study, we aimed to characterize the sonographic features, clinical presentation, management, outcomes, and differential diagnoses of FIH. Accordingly, we reviewed all 17 cases of FIH published in the medical literature, including one new case evaluated by our group. All 17 cases (100%) were male, and FIH is presented as a scrotal mass with a mean diameter of 38 ± 9.5 mm. The right side was dominant (62%). Peristalsis was reported in 80% of the cases, and blood flow was reported in two-thirds. Most cases were diagnosed in the third trimester (88%) at a mean gestational age (GA) of 33.1 ± 5.2 weeks. 60% of the cases had isolated FIH, and 40% had another sonographic or genetic abnormality. Three cases (18%) were syndromic with multiple malformations: trisomy 18, skeletal anomalies due to Jarcho-Levin syndrome, and undefined multiple joint contractures. Two cases (12%) had copathologies in the gastrointestinal tract: one had an echogenic bowel due to homozygosity for cystic fibrosis, and the other had low anorectal malformation. Bowel loop dilatation was observed prenatally in both cases and in another one isolated case (18%). GA at delivery was 38 ± 1.8 weeks, and the median time between diagnosis and delivery was 3 weeks. All three cases of neonatal death occurred in syndromic fetuses. All patients with nonsyndromic inguinal hernias underwent definitive surgical repair at a median of 13 days postpartum. No signs of strangulation and only one case of edematous bowel without necrosis have been reported. In conclusion, FIH should be suspected in male fetuses when an intrascrotal mass with peristalsis is diagnosed during the third trimester. Close follow-up until term in the absence of signs of bowel obstruction is reasonable, and in isolated FIH, the prognosis is favorable.

Gallbladder Pancreatic Heterotopia-The Importance of Diagnostic Imaging in Managing Intraoperative Findings.

Pancreatic heterotopy is a rare entity defined as the presence of abnormally located pancreatic tissue without any anatomical or vascular connection to the normal pancreas. Heterotopic pancreatic tissue can be found in various regions of the digestive system, such as the stomach, duodenum, and upper jejunum, with the less commonly reported location being the gallbladder. Gallbladder pancreatic heterotopia can be either an incidental finding or diagnosed in association with cholecystitis. Pancreatitis of the ectopic tissue has also been described. In this context, we report three cases of heterotopic pancreatic tissue in the gallbladder with different types of pancreatic tissue according to the Heinrich classification. One patient was a 24-year-old male who presented with acute pancreatitis symptoms and an ultrasonographical detected mass in the gallbladder, which proved to be heterotopic pancreatic tissue. The other two cases were female patients aged 24 and 32, respectively, incidentally diagnosed on histopathological examination after cholecystectomy for symptomatic cholelithiasis. Both cases displayed chronic cholecystitis lesions; one of them was also associated with low grade dysplasia of the gallbladder. Although a rare occurrence in general, pancreatic heterotopia should be acknowledged as a possible incidental finding in asymptomatic patients as well as a cause for acute cholecystitis or pancreatitis.

Methods of postural analysis in connection with the stomatognathic system. A systematic review.

This systematic review aimed to identify the main tools used to analyze the relationship between the postural and stomatognathic systems. The study followed the PRISMA guidelines, and data were collected from Science Direct and PubMed databases to identify articles published until December 2022. After applying inclusion and exclusion criteria, 26 articles were selected from the initial 903 articles. The selected articles were full-text studies in English or Romanian, examining the relationship between dental occlusion and posture, measuring postural parameters using various tools, implementing occlusal changes, evaluating patients with permanent dentition, or analyzing the connection between occlusion and posture in a unidirectional manner. The findings indicate that orthognathic surgery and orthodontic mouthguards can significantly enhance postural balance and athletic performance. In addition, 63% of the studies concluded that varying modifications and occlusal conditions impact posture. Notable differences exist concerning posture and Angle dental occlusion classes, and different occlusal devices used to simulate malocclusion can affect patients' postural systems in response to external stimuli. The stabilometry platform is the predominant method for measuring postural parameters; however, other researchers have employed raster stereography, photogrammetry, mobile phone apps, and the Fukuda-Unterberger test. Consequently, interventions targeting the stomatognathic system should consider potential variations in the postural system.

Musicogenic seizures in temporal lobe epilepsy: Case reports based on ictal source localization analysis.

Musicogenic epilepsy is a rare form of reflex epilepsy in which seizures are provoked by music. Different musicogenic stimuli have been identified: pleasant/unpleasant music or specific musical patterns. Several etiologies have been uncovered, such as focal cortical dysplasia, autoimmune encephalitis, tumors, or unspecific gliosis. In this article, we report two patients with musicogenic seizures. The first patient was diagnosed with structural temporal lobe epilepsy. Her seizures were elicited by music that she liked. Interictal and ictal video-electroencephalography (video-EEG) and signal analysis using independent component analysis revealed the right temporal lobe seizure onset extending over the neocortical regions. The patient underwent right temporal lobectomy (including the amygdala, the head, and the body of the hippocampus) and faced an Engel IA outcome 3 years post-surgery. The second patient was diagnosed with autoimmune temporal lobe epilepsy (GAD-65 antibodies). Her seizures were triggered by contemporary hit radio songs without any personal emotional significance. Interictal and ictal video-electroencephalography (video-EEG) and independent component analysis highlighted the left temporal lobe seizure onset extending over the neocortical regions. Intravenous immunoglobulin therapy was initiated, and the patient became seizure-free at 1 year. In conclusion, musicogenic seizures may be elicited by various auditory stimuli, the presence or absence of an emotional component offering an additional clue for the underlying network pathophysiology. Furthermore, in such cases, the use of independent component analysis of the scalp EEG signals proves useful in revealing the location of the seizure generator, and our findings point toward the temporal lobe, both mesial and neocortical regions.

The Pathological Links between Adiposity and the Carpal Tunnel Syndrome.

An association between obesity and carpal tunnel syndrome is found in many epidemiological studies. Therefore, there is a need to evaluate the physiopathological links that could explain the association between these two entities. Ectopic adipose tissue is responsible for metabolic syndrome and inflammation, and is a major risk factor for diabetes and cardiovascular diseases. Taking these elements into consideration, we conducted an extensive literature revision of the subject, considering as ectopic fat-related mechanisms the following: (a) the direct compression and the association with the metabolic syndrome of the fat deposition around the wrist, (b) the insulin resistance, dyslipidemia, inflammatory, and oxidative mechanisms related to the central deposition of the fat, (c) the impaired muscle contraction and metabolism related to myosteatosis. Each section presents the cellular pathways which are modified by the ectopic deposition of the adipose tissue and the impact in the pathogeny of the carpal tunnel syndrome. In conclusion, the experimental and clinical data support the epidemiological findings. Efforts to reduce the obesity epidemics will improve not only cardio-metabolic health but will reduce the burden of the disability-free life expectancy due to the carpal tunnel syndrome.

Continuous glucose monitoring devices: A brief presentation (Review).

As diabetes prevalence is continuously increasing, better management is needed to achieve blood glucose control, in order to prevent complications and lessen the burden of this disease. Since the first measurement of glycosuria at the beginning of the 1900s', huge advances were made in monitoring glycemia. Continuous glucose monitoring systems revolutionized diabetes management, especially for patients with type 1 diabetes. Avoiding glycemic variability and maintaining optimal glycemic control is crucial for the evolution of patients with type 1 diabetes. The usefulness of glycemic monitoring devices can be extended to patients with type 2 diabetes. It is also important to note that in those patients at risk of developing high glycemic variability (e.g. patients with advanced chronic kidney disease), continuous glycemic monitoring may improve their prognosis. These monitoring systems can be classified according to the analytical method, the degree of invasiveness, the data availability and the mode of usage. The technology is constantly improving in bioanalytical performance, biocompatibility, length of wearing time, safety and clinical features. The aim of this review was to briefly present the main characteristics of glucose biosensors, glucose monitoring systems and their clinically utility.

New evidence from hyperspectral imaging analysis on the effect of photobiomodulation therapy on normal skin oxygenation.

The aim of this study was to assess the changes induced by photobiomodulation therapy in oxygenation of normal skin and underlying tissue using hyperspectral imaging combined with a chemometric regression approach. Eleven healthy adult volunteers were enrolled in this study. The dorsal side of the left hand of each subject was exposed to photobiomodulation therapy, while the correspondent side of the right hand was used as a control (placebo effect). Laser irradiation was performed with a laser diode system (635 nm, 15mW, 9 J/cm2) for 900 s. Changes in skin oxygenation were assessed before and after applying the photobiomodulation therapy and placebo using the hyperspectral imaging. Hyperspectral data analysis showed that variations of oxyhemoglobin and deoxyhemoglobin concentrations had no statistical significance in both groups. In conclusion, photobiomodulation therapy does not induce changes in oxyhemoglobin and deoxyhemoglobin concentrations in the normal skin measured from hyperspectral images, at least at λ = 635 nm and 900-s exposure time.

Overview of the anatomical basis of the piriformis syndrome-dissection with magnetic resonance correlation.

The piriformis syndrome is one of the most commonly misdiagnosed causes of lower back and gluteal pain caused by the compression of the sciatic nerve and the internal pudendal neurovascular bundle by the piriformis muscle. Although this syndrome was first suggested over 90 years ago, its diagnosis still represents a challenge for clinicians. In the present study, dissection was used to determine the intra- and extrapelvic anatomical course of the internal pudendal nerve and the data were compared with the information obtainable through MRI examination, in order to identify the piriformis syndrome and to differentiate it from other causes of internal pudendal neuralgia. Thorough dissections of the pelvis and deep gluteal region were conducted on female cadavers, which were correlated with MRI scans, in order to describe the course of the internal pudendal nerve in contact with the piriformis muscle. The dissection findings and MRI scans obtained allowed us to describe and demonstrate the compression points along the course of the sciatic nerve and the internal pudendal bundle, the anatomical correlations between the piriformis muscle and the nervous structures around it, emphasizing the areas most susceptible to possible nerve impingement syndromes. In the anatomic trajectory of the sciatic nerve and the internal pudendal bundle there are multiple contact points with anatomical structures that may lead to compression of the nerve structures, generating symptoms that comprise the piriformis syndrome. The present study sought to establish clear osseous landmarks that may help evaluate these associations and possible nerve compressions on pelvic MRI examination.

Impact of adipose tissue in chronic kidney disease development (Review).

Obesity is a worldwide pandemic health issue. Obesity is associated with the pathogenesis of type 2 diabetes, hypertension, dyslipidemia, cardiovascular diseases, cancer, and kidney diseases. This systemic disease can affect the kidneys by two mechanisms: Indirectly through diabetes mellitus (DM) and hypertension and directly through adipokines secreted by adipose tissue. Obesity is a risk factor for chronic kidney disease (CKD), which is associated with an increased risk of morbidity and mortality among the adult population. Increased visceral adipose tissue leads to renal glomerular hyperfiltration and hyperperfusion, which may lead to glomerular hypertrophy, proteinuria, and CKD development. Adipokines are hormones produced by fat tissue. They are involved in energy homeostasis, sugar and fat metabolism, reproduction, immunity, and thermogenesis control. Hormones and cytokines secreted by adipose tissue contribute to the development and progression of CKD. Decreased serum or urinary adiponectin levels are specific in diabetic and non-diabetic CKD patients, while leptin presents increased levels, and both are associated with the development of glomerulopathy. Excessive adipose tissue is associated with inflammation, oxidative stress (OS), insulin resistance and activation of the renin angiotensin-aldosterone system (RAAS). Therefore, adipose tissue dysfunction plays an important role in the development of CKD.

PI3K/AKT/mTOR signalling pathway involvement in renal cell carcinoma pathogenesis (Review).

Renal cell carcinoma (RCC) accounts for over 90% of all renal malignancies, and mainly affects the male population. Obesity and smoking are involved in the pathogenesis of several systemic cancers including RCC. The phosphoinositide 3-kinase (PI3K)/protein kinase B (AKT)/mammalian target of rapamycin (mTOR) signalling pathway regulates cell growth, differentiation, migration, survival, angiogenesis, and metabolism. Growth factors, hormones, cytokine and many extracellular cues activate PI3K/AKT/mTOR. Dysregulation of this molecular pathway is frequently reported in human cancers including RCC and is associated with aggressive development and poor survival rate. mTOR is the master regulator of cell metabolism and growth, and is activated in many pathological processes such as tumour formation, insulin resistance and angiogenesis. mTOR inhibitors are used at present as drug therapy for RCC to inhibit cell proliferation, growth, survival, and the cell cycle. Temsirolimus and everolimus are two mTOR inhibitors that are currently used for the treatment of RCC. Drugs targeting the PI3K/AKT/mTOR signalling pathway may be one of the best therapeutic options for RCC.

Not only appendicitis: rare appendix disorders manifesting as surgical emergencies in children.

Acute appendicitis is the most common cause of acute abdominal pathology in children. However, other rare non-inflammatory non-neoplastic disorders involving the appendix may manifest as surgical emergencies. This study aimed to describe these atypical entities and present representative cases. The database of a tertiary children's medical center was reviewed for all the patients aged 0-18 years who underwent urgent appendectomy between June 2014 and December 2019, for rare disorders of the appendix unrelated to inflammatory or neoplastic processes. Of 1367 patients who underwent appendectomy, 1345 were operated urgently or emergently. Of these, six, all males, mean age 32.6 months (range 0.7-76), underwent appendectomy for rare surgical complications that involved the appendix. These included torsion of the appendix (2), a strangulated internal hernia through an appendicular ring (1) or through a mesoappendix gap (1), an incarcerated appendix in an acute hernia sac (1), and appendiceal intussusception (1). In all cases, the role of the appendix in the pathologic process was unexpected and came as a surprise to the surgeon. During a median follow-up of 4.2 months (range 1-8 months), one patient underwent relaparotomy for small bowel obstruction 4 weeks after the original procedure.Conclusion: The appendix in children can be the source of rare pathological disorders that present as surgical emergencies. Familiarity with these entities may aid in achieving accurate preoperative diagnosis and contribute to surgical team orientation on exploratory laparotomy. However, correct diagnosis is often only established during timely surgical intervention.

Cingulate cortex function and multi-modal connectivity mapped using intracranial stimulation.

The cingulate cortex is part of the limbic system. Its function and connectivity are organized in a rostro-caudal and ventral-dorsal manner which was addressed by various other studies using rather coarse cortical parcellations. In this study, we aim at describing its function and connectivity using invasive recordings from patients explored for focal drug-resistant epilepsy. We included patients that underwent stereo-electroencephalographic recordings using intracranial electrodes in the University Emergency Hospital Bucharest between 2012 and 2019. We reviewed all high frequency stimulations (50 â€‹Hz) performed for functional mapping of the cingulate cortex. We used two methods to characterize brain connectivity. Effective connectivity was inferred based on the analysis of cortico-cortical potentials (CCEPs) evoked by single pulse electrical stimulation (SPES) (15 â€‹s inter-pulse interval). Functional connectivity was estimated using the non-linear regression method applied to 60 â€‹s spontaneous electrical brain signal intervals. The effective (stimulation-evoked) and functional (non-evoked) connectivity analyses highlight brain networks in a different way. While non-evoked connectivity evidences areas having related activity, often in close proximity to each other, evoked connectivity highlights spatially extended networks. To highlight in a comprehensive way the cingulate cortex's network, we have performed a bi-modal connectivity analysis that combines the resting-state broadband h2 non-linear correlation with cortico-cortical evoked potentials. We co-registered the patient's anatomy with the fsaverage FreeSurfer template to perform the automatic labeling based on HCP-MMP parcellation. At a group level, connectivity was estimated by averaging responses over stimulated/recorded or recorded sites in each pair of parcels. Finally, for multiple regions that evoked a clinical response during high frequency stimulation, we combined the connectivity of individual pairs using maximum intensity projection. Connectivity was assessed by applying SPES on 2094 contact pairs and recording CCEPs on 3580 contacts out of 8582 contacts of 660 electrodes implanted in 47 patients. Clinical responses elicited by high frequency stimulations in 107 sites (pairs of contacts) located in the cingulate cortex were divided in 10 groups: affective, motor behavior, motor elementary, versive, speech, vestibular, autonomic, somatosensory, visual and changes in body perception. Anterior cingulate cortex was shown to be connected to the mesial temporal, orbitofrontal and prefrontal cortex. In the middle cingulate cortex, we located affective, motor behavior in the anterior region, and elementary motor and somatosensory in the posterior part. This region is connected to the prefrontal, premotor and primary motor network. Finally, the posterior cingulate was shown to be connected with the visual areas, mesial and lateral parietal and temporal cortex.

Synchronous Multiple Breast Cancers-Do We Need to Reshape Staging?

Background and Objectives: Current recommendations and treatment regimens in breast cancer are a reflection of its heterogeneity on multiple levels including histological subtypes, grading, molecular profiling, and numerous prognostic indices. Although based on extensive research, current guidelines are not explicit in the case of surgical specimens showing various degrees of mismatch between different parts of the same tumor and even more so between multicentric lesions. Synchronous breast cancer is the ideal prototype for studying inter- and intra-tumoral heterogeneity, therefore we envisaged that a study on patients with multicentric and multifocal lesions could contribute to the reshaping of the staging, prognosis, and treatment of breast malignancies. Material and Methods: A prospective observational study was conducted between January 2013 and May 2017 on 235 patients diagnosed with breast cancer (BC) and surgically treated at Emergency University Hospital, Bucharest. Thirty-seven patients had multiple breast tumors and were eligible for assessment of the heterogeneity of their lesions. Results: 6 were multicentric and 31 multifocal. The number of foci varied from 2 to 11. We encountered numerous mismatches between the index and the secondary tumors, as follows: 3 cases (8.1%) with histopathological mismatch, 13 (35.1%) with different grades of differentiation, 11 (29.8%) with ER (Estrogen Receptors) status mismatch, 12 (32.4%) with PR (Progesterone Receptors) status mismatch, 8 (21.6%) with molecular phenotype mismatch, and 17 (45.9%) cases with variable Ki-67. After careful analysis of index and secondary tumors, apart from the mismatches reported above, we discovered that the secondary tumors were actually dominant in 5 cases (13.5%), and therefore at least those cases had to be reclassified/restaged, as the supplementary data commanded changes in the therapeutic decision. Conclusions: For synchronous breast tumors, the current Tumor-Node-Metastasis (TNM) staging system ignores not only the histopathological and immunohistochemical characteristics of the secondary foci, but also their size. When secondary lesions are more aggressive or their cumulative mass is significantly bigger than that of the index tumor, the treatment plan should be adapted accordingly. We believe that information obtained from examining secondary foci in synchronous breast cancer and assessment of the cumulative tumoral mass should be reflected in the final staging and definitive treatment. The clinical benefit of staging the patients based on the most aggressive tumor and the cumulative tumoral burden rather than according to the biggest single tumor, will avoid under-treatment in cases with multifocal/multicentric BC displaying intertumoral mismatch.

Fatal outcome of gastric perforation due to infection with Sarcina spp. A case report.

Sarcina ventriculi is an extremely rare pathogen. These gram-positive cocci bacteria are rarely identified in gastric biopsies and usually described in the scientific literature as an incidental finding, particularly in patients with delayed gastric emptying, gastroparesis, emphysematous gastritis or gastric perforation. It occurs most commonly in adult women and can be identified easily by its distinctive morphologic features, such as basophilic staining, cuboidal shape, tetrad arrangement, red blood cell-sized packets, flattened cell walls, and refractile nature in bright field microscopy. Although the pathogenesis of the microorganism is highly debated in humans, this bacterium is a well-known pathogen in livestock. Fewer than 30 cases of human infection have been described in the scientific literature so far, but none mentioned this micro-organism as a potential cause of death. We report the case of a 76-year-old patient with gastric perforation due to massive infection with Sarcina ventriculi. To date, this is the first report of human infection with Sarcina ventriculi in Romania.

Correlation between Pregnancy Related Weight Gain, Postpartum Weight loss and Obesity: a Prospective Study.

Weight gain during pregnancy can be a real risk factor for long-term obesity which has implications in all areas of medicine. This study is designed to assess pregnancy-related weight gain and postpartum weight loss, to identify a possible correlation between weight gain during pregnancy and the risk of obesity in the late postpartum period. The batch comprised 306 women, hospitalized in the Obstetrics and Gynecology Section of the "Nicolae Malaxa" Clinical Hospital between June - November 2017. During this study, we assessed the weight status using the Weight, Body Mass Index, Height, and Abdominal Circumference. These parameters were clinically assessed in three periods pre-pregnancy, early postpartum period, late postpartum period. We also collected data on the evolution of the pregnancy using the anamnesis and the personal pregnancy monitoring sheet. Pregnancy and postpartum period represent a key moment in women's lives in which the risk of obesity is real. Understanding women experiences with weight changes during pregnancy and postpartum period can improve the management of losing weight following pregnancy, avoid long-term weight gain and so reduce the risk for obesity. Also, the correct management of obesity should include the assessment of somatic disorders that may cause major dysfunction, requiring complex rehabilitation programs.

Fractionated Irradiation in Photobiomodulation Therapy of Ankle Sprain.

OBJECTIVE: The aim of this study was to test whether fractionated irradiation in photobiomodulation therapy enhances short-term recovery in patients with moderate severity ankle sprain. DESIGN: Nineteen patients were randomly assigned to one of two groups: one group receiving photobiomodulation therapy combined with the standard rest, icing, compression, and elevation treatment, or a group receiving only standard treatment. Group 1 patients were irradiated using a laser system (635 nm, 15 mW) according to a double-fractionated irradiation scheme consisting of two sessions (4.5 and 9 J/cm) separated by a 30-min time interval. Short-term outcomes (reflectance, pain, and clinical outcomes) were assessed at baseline, 10 days, and 6 wks after treatment. RESULTS: Reflectance data analysis showed significant changes in group 1 (P = 0.027). There was also an ankle function score improvement more in group 1 than in group 2, with a significant short-term effect (P = 0.011) but without significant long-term effects (P = 0.178). Compared with group 2, group 1 had an immediate effect on pain reduction, but no long-term effect (P = 0.074). CONCLUSIONS: Combined with standard treatment, fractionated irradiation photobiomodulation therapy has been shown to have favorable short-term effects on the recovery of patients with ankle sprains, but its long-term effects should be improved.

Appendiceal Mass - Dilemmas Regarding Extension of the Resection.

Starting from a typical case, we try to highlight the main critical elements, both in the diagnostic algorithm and in the therapeutic one. We hereby present a 54-year old patient, known with hepatic steatosis and renal microlithiasis, which undergoes an ultrasonography in ambulatory, on the background of unsystematised abdominal discomfort; the ultrasound reveals the presence of a tumoral mass in the right iliac fossa.

New insights in the molecular pathways linking obesity, type 2 diabetes and cancer.

Steadily, cancer is becoming the first cause of mortality, with over 9 million deaths estimated in 2018. Increasing evidence supports a direct association between obesity, type 2 diabetes mellitus (T2DM) and cancer, with a higher risk of cancer mortality especially for some of the most common malignancies, such as breast, colon, and rectal cancers. So far, several mechanisms underlying the cancer-diabetes relationship have been investigated revealing dysregulations of the insulin-insulin-like growth factor (IGF) system as the most important paradigm. Other molecular mechanisms that seem to play a role in the association cancer-T2DM consist of alteration of the signaling pathways activated by inflammatory cytokines, adipocytokines or adhesion molecules. The overall aim of this review is to provide an overview of the molecular mechanisms linking obesity, T2DM and cancer, as related to the receptors and signaling pathways involved in these associations.

Splenectomy in Lymphoproliferative Disorders: A Single Eastern European Center Experience.

Background and Objectives: Hematological malignancies are usually systemic diseases of life-threatening impact, and frequently require prompt and energetic therapeutic intervention. Due to systemic involvement, the role of surgery is generally limited to diagnostic approaches, and it is very rarely employed as a therapeutic modality. Splenectomy represents an exception to this paradigm, being used both as a diagnostic and tumor debulking procedure, notably in splenic lymphomas. Materials and Methods: We investigated the role of splenectomy in a single center prospective study of splenectomy outcome in patients with splenic involvement in the course of lymphoproliferative disorders. In the present study, we included all patients treated in our department for lymphoid malignancies over a period of six years, who underwent splenectomy as a diagnostic or debulking procedure after referral and workup, or had been referred to our department after first being splenectomized and diagnosed with splenic lymphoma. Patient characteristics and treatment outcome were investigated. Results: We enrolled 54 patients, with 34 (63%) splenectomized patients: 12 splenectomies (22.2%) for diagnostic purposes and 22 (40.7%) for treatment. Special attention was given to the 28 (51.85%) patients diagnosed with splenic marginal zone lymphoma (SMZL), a subtype with a clear therapeutic indication for splenectomy. Average age of patients was 57.5 (±13.1) years with a higher prevalence of feminine gender (66.67%). Age above 60 years old (p = 0.0295), ECOG (Eastern Cooperative Oncology Group) > 2 (p = 0.0402) and B-signs (p nonsignificant (NS)) were most frequently found in SMZL patients. Anemia, and notably autoimmune anemia, was more frequent in SMZL versus other small-cell lymphomas and also in splenectomized patients, as was leukocytosis and lymphocytosis. Treatment of patients with lymphoproliferative disorders consisted of chemotherapy and/or splenectomy. Most SMZL patients received chemotherapy as first line treatment (61.5%) and had only partial response (57.7%). Second treatment line was splenectomy in 80% of patients who required treatment, followed by a 60% rate of complete response (CR). Splenectomy offered a higher complete response rate (twice as high than in non-splenectomized, regardless of histology type, p = NS), followed by a survival advantage (Overall Survival (OS)~64 versus 59 months, p = NS). Particularly, SMZL patients had a 4.8 times higher rate of CR than other non-Hodgkin lymphoma (NHL) patients (p = 0.04), a longer progression free survival (73 months vs. 31 months for other small-cell NHLs p = NS) and a 1.5fold lower death rate (p = NS). The procedure was rather safe, with a 38.5% frequency of adverse reactions, mostly minor and manageable. Conclusions: Our data suggest that splenectomy is an effective and safe therapeutic option in patients with lymphoid malignancies and splenic involvement, particularly splenic marginal zone lymphoma.

Emerging concepts and latest advances regarding the etiopathogenesis, morphology and immunophenotype of basal cell carcinoma.

Basal cell carcinoma (BCC) represents the most common skin malignancy, which has been linked over time to multifactorial causes. It usually occurs on sun-exposed areas in people with fair skin and with predilection for men. BCC usually affects elderly patients and has an extremely wide range of histological subtypes, which can present clinically under different variants, some with really challenging differentials. Although BCC may occur in familial settings, such as nevoid BCC syndrome, Rombo or Bazex-Dupré-Christol syndromes, nonsyndromic multiple BCCs have also been described in the scientific literature. In this review, we discuss in detail the latest scientific concepts regarding BCC, its etiology, pathogenesis, genetic bases of disease, morphology and immunophenotype, as well as the currently known genetic syndromes, which may lead to development of multiple BCCs, especially in young adults.