Overview of histiocytic and dendritic disorders by the 5th version of WHO Classifi cation of Hematolymphoid Tumours from 2022.

BACKGROUND: Histiocytoses are rare disorders characterized by the accumulation of macrophages, dendritic cells, or monocyte-derived cells in various tissues and organs of children and adults, with a wide range of clinical manifestations, presentations, and histology. The histiocytoses are classified according to the WHO Classification, the last version of which was published in 2022, or according to the Histiocyte Society Classification, with the last version published in 2016. PURPOSE: This text provides an overview of histiocytoses as described in the WHO Classification 2022.

Therapy of Castleman's disease with siltuximab - case report and review of literature.

BACKGROUND: Idiopathic multicentric Castleman disease (iMCD) is characterized by constitutional symptoms, enlarged lymph nodes and laboratory test abnormalities, which are primarily related to the overproduction of interleukin-6 (IL-6). This form (iMCD) was treated earlier with cytostatics used for lymphoma, later with bio-logic therapy as rituximab, immunodulatory drugs and proteasome inhibitors, and in the last years with an anti-IL-6 antibody, siltuximab. Siltuximab is a human-mouse chimeric immunoglobulin G1k monoclonal antibody against human IL-6 approved in the European Union for the treatment of iMCD. In view of the limited treatment options for iMCD, this case report aimed to evaluate the efficacy and safety of siltuximab in the management of this condition. CASE: We describe a young woman with iMCD diagnosed at the age of 25 years. For first line treatment, rituximab and dexamethasone were used without any cytostatic because the patient wished to give birth to a healthy child in the future. However, the response after this first line therapy was short. In addition, after 3 years from the start of rituximab + dexamethasone therapy, it was necessary to administer treatment for the relapse of iMCD. We decided for siltuximab in this young woman, still aged < 30 years, and started administration of siltuximab in 3-week intervals. RESULTS: After administration of first two infusions of siltuximab, all inflammatory markers returned to normal value. Moreover, serum hemoglobin and albumin levels as well as C-reactive protein normalized after the first two administrations of siltuximab. The clinical response continue, siltuximab is still administered in 3-week intervals. PET/CT with fluorodeoxyglucose confirmed a very good anatomic and metabolic response to the treatment. Siltuximab demonstrated a favorable safety profile, and the prolonged treatment was well tolerated. CONCLUSION: This result is encouraging and demonstrates the potential of siltuximab as treatment of CD. As earlier published, this case confirms that significantly elevated inflammatory markers in a patient with CD predict a good response to siltuximab.

Preoperative nutritional management - compliance with current guidelines.

INTRODUCTION: Major surgery poses a significant stress to the patient. The nutritional status is one of crucial factors that have a substantial impact on the final outcome of the surgery. Preoperatively established malnutrition or an increased nutritional risk in this group of patients requires a maximum effort to minimize this negative impact as soon as the operation is scheduled. The aim of this retrospective study was to assess compliance with guidelines focused on preoperative nutrition management at our site. METHODS: Our retrospective descriptive observation was focused on the period from January 1, 2017 to June 30, 2020. All patients scheduled for major surgery were screened for nutritional status using a nutritional questionnaire, and an appropriate type of nutritional intervention was indicated based on the achieved score. RESULTS: Two hundred and forty gastrointestinal operations were performed during the study period. In total, 208 (87%) of patients were screened at the time of counselling. Diet adjustments with an increased protein intake were recommended to all 125 (100%) patients with normal nutrition status. In total, 95 patients were at nutritional risk and sipping was prescribed to all of them (100%) in the outpatient setting. All 20 malnourished patients (100%) underwent preoperative nutritional optimization as inpatients. CONCLUSION: Nutritional status of patients before major surgery is considered a predictive indicator of potential postoperative complications. Compliance with recent ERAS guidelines concerning preoperative screening and nutritional support is high in our department.

Duodenal gastrinoma - case report.

INTRODUCTION: In our text, we want to highlight a rare diagnosis. CASE REPORT: A 54-year-old obese, hypertensive male smoker had been investigated for intermittent abdominal pain for 12 years. The first gastroscopy for a bleeding ulcer was conducted in 2010. In the subsequent years, repeated gastroscopies revealed variable involvement from the esophagus to the duodenum. Capsule enteroscopy did not provide further specification of the diagnosis. The patient underwent colonoscopy and MRI enterography multiple times, with no unequivocal pathological findings. In May 2022, he was admitted to our department for abdominal pain and vomiting. This time, gastroscopy revealed multiple small ulcers in the duodenum and jejunum with clots causing a mechanical obstruction. Chromogranin A was elevated, raising suspicion of gastrinoma. However, somatostatin receptor-based imaging (Octreoscan) was negative. Only the 68Ga-DOTATOC PET (positron emission tomography with the radiopharmaceutical DOTA, labeled with gallium-68) identified a lesion in the subhepatic region, which had no correlation on CT. We concluded the diagnosis as gastrinoma with the Zollinger-Ellison syndrome. Endoscopically, a 1cm tumor was found in the duodenum. In October 2022, the patient underwent an excision of the duodenal wall, and the pathology assessment confirmed our diagnosis of gastrinoma. CONCLUSION: With this case report, we want to emphasize the importance of taking into account neuroendocrine tumors in our differential diagnostic considerations. At the same time, we want to highlight that, according to ESMO recommendations, we should preferentially use 68Ga-DOTATOC PET/CT for the diagnosis instead of scintigraphic examination (111In-Octreoscan).

Patients benefi ts from physicians empathy and results of including of empathy development into medical training.

BACKGROUND:  The extent of empathy is an individual human property, not completely dependent on cognitive intelligence. People arise with certain genetic fundament for empathy but the ability to perceive empathically develops further during the life. There has been much discussion in the medical literature about the importance of empathy and physician communication style in medical practice. Empathy has been shown to have a very real positive eff ect on patients outcomes. The literature suggests that empathy training is warranted and should be incorporated into surgical residencies through didactics, role-playing and simulations, and apprenticeship to empathic attending role models. PURPOSE: This paper reviews empathy and its importance as it pertains to the physician-patient relationship and improving patients outcomes, and the need for increased education in empathy during medical training.

Rosai-Dorfman-Destombes disease - histiocytic disorder with inflammatory manifestation.

BACKGROUND: Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytosis characterized by accumulation of activated histiocytes within affected tissues. Although the immunophenotype of this disease was described, the pathophysiology of this disease is still not sufficiently understood. Recent studies have found NRAS, KRAS, MAP2K1, and ARAF mutations in RDD lesions, raising the possibility of a clonal origin in some forms of RDD while in other cases reactive origin or association with other malignant and autoimmune disease is supposed. RDD is a widely heterogeneous entity with a range of clinical phenotypes occurring in some patients in association with autoimmune or malignant diseases. Its therapy should reflect the localization of the disease. Monotherapy with glucocorticoids is sufficient only in limited disease. In patients with advanced disease, combined nodal and extranodal forms of RDD need more intensive therapy. In older publications, antimetabolites, vinca alkaloids and prednisone were used; in recent publications, remissions after cladribine, rituximab, sirolimus, thalidomide, lenalidomide and cobimetinib were described. PURPOSE: This text summarizes current knowledge about this rare disease and reviews the therapeutic options.

Slow increase of bilirubin concentration during administration of lenalidomide, bortezomib and dexamethasone for multiple myeloma (unmasking previously undiagnosed Gilbert syndrome) and disappearance of necrobiotic xanthogranuloma after complete remission of multiple myeloma.

BACKGROUND: Lenalidomid ranks among immunomodulatory drugs. There are a few of the more common side effects, like a higher risk of venous trombembolism or diarrhea. Other side effects are rare. The hyperbilirubinemia described in this article can be assigned to them. In our case, the increase of bilirubin was associated with unrecognized Gilbert syndrome. CASE DESCRIPTION: We report a patient with multiple myeloma and necrobio-tic xanthogranuloma (NXG) of the skin and liver. After the treatment with bortezomib, lenalidomid and dexamethasone, complete remission was attained after 4 cycles with decrease of monoclonal immunoglobulin to an unmeasurable concentration. At the same time, the dis-appearance of cutaneous and hepatic lesions of NXG on FDG-PET/CT was evident. The administration of bortezomib was stopped after 8 cycles and only continued with lenalidomide as a maintenance therapy. However, after four cycles of this therapy, bilirubin increased above the upper limit and the increase continued till the 11th month of lenadomide administration, when bilirubin reached the highest concentration of 75 μmol/l (more than the three-fold of the upper limit, grade III toxicity). The patient had asymptomatic hyperbilirubinemia with no underlying liver disease or renal impairment while being on lenalidomide therapy. Genetic studies proved mutation; insertion in the promotor gene UGT1A1 typical for Gilbert syndrome. Hyperbilirubinemia may be attributed to the unmasking of previously undia-gnosed Gilbert syndrome. Therefore, the therapy with lenalidomide was interrupted after 11 months. The bilirubin level decreased after the discontinuation of the drug. CONCLUSION: NXG disappeared after fulfilling complete remission of multiple myeloma with disappearance of monoclonal immunoglobulin. This observation supports the hypothesis that monoclonal immunoglobulin has a crucial role in the ethiopathogenesis of NXG and suggests the treatment of monoclonal gammopathy if present in a patient with NXG, hoping that this will result in xantogranuloma disappearance.

Defensive medicine and the influence of litigation on doctors.

The term defensive medicine is used to describe the behavior of healthcare providers motivated by fear of litigation due to malpractice. It includes both avoidance behavior when the physician is unwilling to perform high risk procedures, as well as excessive ordering of extra tests and procedures. This leads to unnecessary diagnostic and therapeutic interventions which may be invasive and costly. Additionally, such a setting causes harms the patient-doctor relationship. A more specific legal framework, developed with respect to the innate nature of medicine, may improve the situation.

Retroperitoneal fibrosis - diagnosis and treatment.

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. Common symptoms at onset include lower back, abdominal or flank pain. Pain is frequently referred to the hip, to the groin and to the lateral regions of the leg, often with nocturnal exacerbations and not responding to position changes. The disease is commonly associated with signs of systemic inflammatory response (malaise, fever, and anorexia and weight loss). Glucocorticoids are considered the cornerstone of the therapy. The use of other immunosuppressive agents, including cyclophosphamide, azathioprine, methotrexate, mycophenolate mofetil and biological agents such as rituximab, tocilizumab and infliximab have been reported as a valuable option mostly in case reports, cases series and small studies. These agents allowed to reduce cumulative dose of glucocorticoids and their adverse effects. Combined therapy is preferable for all patients who suffer from significant glucocorticoid- related toxicity or in cases where glucocorticoids alone are insufficient to treat the condition.

Basic information for identifying psychological disorders caused by malignant disease.

BACKGROUND: Improvements in cancer dia-gnosis and treatment explain a substantial increase in the number of patients chronically affected by or recovering from cancer. This is a fragile population, physically, psychologically and socially affected by the consequences of the disease and the associated treatment. The National Comprehensive Cancer Network (NCCN) reacted to this fact, creating the NCCN guidelines for survivorship. They provide screening, evaluation and treatment recommendations for the consequences of cancer and cancer treatment. PURPOSE: Inspired by this NCCN recommendation, we drew up this article pointing out the psychological issues like anxiety, depression and fatigue in order to help the physicians refer their patients timely to psychologic or psychiatric care.

Colonic perforation in patients with COVID-19 pneumonia - case reports.

INTRODUCTION: Gastrointestinal complications in critically ill patients during the COVID-19 pandemic pose a diagnostic and treatment dilemma. CASE REPORT: We present two cases of SARS-CoV-2 positive patients treated in our department for colon perforation. One patient was operated for a diastasic right colon perforation due to acute over distension of the bowel. The perforation in the second case was associated with chronic diverticulitis. CONCLUSION: These cases highlight the fact that besides typical respiratory symptoms and signs of COVID-19, digestive symptoms also occur. These can caused by intestinal perforation, be it directly or due to changing homeostasis of the internal environment.

IgG4-releated disease.

BACKGROUND: IgG4-related disease (IgG4-RD) is a non-malignant, chronic, immune-related disease. It was first recognized as a distinct disease in 2012 and the first classification criteria were published in 2020. This new entity can cause fibroinflammatory lesions in nearly any organ. It often presents as a multi-organ disease and can be confused with malignancy, infection or other immune-mediated conditions. Although the disease could affect virtually any organ, there are strong predilections for certain organs: the major salivary glands, the orbits and lacrimal glands, the pancreas and biliary tree, the lungs, the kidneys, the aorta and retroperitoneum, the meninges and the thyroid gland. PURPOSE: Correlation among clinical, serologic, radiologic and pathologic data is required for establishing IgG4-RD. We sum up the newest information necessary for the dia-gnosis.

Abdominal abscess associated with salmonellosis case report.

INTRODUCTION: Although gastroenteritis is considered to be a non-surgical disease, rare complications necessitating surgical intervention may occur. CASE REPORT: We present a patient who underwent acute small bowel resection due to an abdominal abscess, which developed in association with Salmonella enteritis.

ERAS in colorectal surgery - neglected preadmission items.

The Enhanced Recovery After Surgery (ERAS) concept is a complex of strategies intended to reduce the perioperative stress response and achieve faster postoperative convalescence and rapid recovery of normal physiological functions. Adherence to ERAS should reduce the length of stay and postoperative complications, and it should improve the physical condition of the patient after dimission. This article is focused on those ERAS guidelines that apply to preadmission care.

Psychological aspects in surgical oncology.

The following review is dedicated to the impact of psychological factors on the genesis and development of malignant diseases. People suffering from stress, anxiety and depression have a higher risk of tumour occurrence and its faster growth. The prognosis is also influenced by the marital status; married patients have a better overall survival and cancer-specific survival compared to unmarried patients.

Psychological influences on wound healing.

Surgical wound complications remain a major cause of morbidity; although usually not life threatening, they reduce the quality of life. They are also associated with excessive health care costs. Wound healing is affected by many factors - wound characteristics, infection, comorbidities and nutritional status of the patient. In addition, though, psychological stress and depression may decrease the inflammatory response required for bacterial clearance and so delay wound healing, as well. Although the patient´s state of mind can be influenced only to a certain extent, we should nevertheless stick to ERAS (Enhanced Recovery After Surgery) guidelines and try to diminish fear and anxiety by providing enough information preoperatively, pay due attention to postoperative analgesia and seek to provide an agreeable environment.

Mesh fixation in laparoscopic reconstruction of inguinal hernias.

INTRODUCTION: Laparoscopic inguinal hernia repair is a widely used surgical technique, although not exempt from potential complications. The aim of our study was to compare our postoperative results according to the fixation method of the prosthetic material. METHODS: This is a retrospective study. We looked up 167 patients who underwent laparoscopic inguinal hernioplasty between 01 Jan 2015 and 31 Dec 2016 at the Surgical Department of Vsetin Hospital. The patients were invited for a follow-up visit after 46 weeks and a questionnaire was sent them after 933 months. We evaluated any development of postoperative hematoma, recurrent hernia and pain after one month and during the first year from the surgery in dependence on the fixation method (stapler Pro Thick, acrylic glue Glubran, 3D light mesh). RESULTS: In total, 120 patients were enrolled in the study, 22 in the Glubran group, 59 in the stapler group and 39 in the 3D group. A hematoma developed in 10% in the stapler group, while there was none in the other groups; the difference was statistically significant (p=0.0382). Recurrent hernia was found in 5% in the Glubran group, 3% in the stapler group, and 0% in the 3D group; no statistically relevant difference was found. No difference was demonstrated for postoperative pain, either. CONCLUSION: Long-term postoperative results after TAPP were not found to differ in recurrence rates and pain based on the used fixation material in our set of patients. The stapler was associated with a higher risk of postoperative hematoma, but it was a preferred method in all cases of large hernias.

Influence of overweight and obesity on complications in colorectal surgery.

INTRODUCTION: Overweight and obesity are recognized as risk factors for many health-related disorders like diabetes, cardiovascular disease and malignancy. On the other hand, according to recent studies, they may be a favorable prognostic factor in patients treated for various chronic diseases. Patients with increased body mass index (BMI) have a lower mortality rate, which is known as the "obesity paradox". Overweight and obese patients, as reported by several papers, also have better outcomes after surgery. The aim of our study was to evaluate this somewhat surprising phenomenon on our group of patients. METHODS: The medical records of patients diagnosed with colorectal cancer who were treated with resection and primary anastomosis from 1 January 2010 to 31 December 2017 were retrospectively analyzed. Patients after multivisceral resections or with lacking data were excluded. We did not evaluate two underweight patients. The BMI classification of the World Health Organization was used. Postoperative complications, anastomotic leak, mortality and length of stay in hospital were investigated. RESULTS: 225 patients were included. 56 (24.9%) patients had normal weight - group A, 98 (43.6%) patients were overweight - group B, 58 (25.7%) patients had class I obesity - group C, 9 (4%) patients had class II obesity and 4 (1.8%) patients class III obesity - together they form group D. The groups did not significantly differ in age, sex, comorbidities, cancer stage and locality. Complications occurred as follows: A-29%; B-25%; C-36%; D-55%. Anastomotic leak: A-9%; B-4%; C-9%; D-15%. Mortality: A-5%; B-2%; C-2%, D-0%. Average length of stay: A-11.7 days; B -11.3 days; C-12.6 days; D-47 days. We did not observe any statistically significant difference. CONCLUSIONS: Overweight surgical colorectal cancer patients had the lowest incidence of complications, anastomotic leak and the shortest length of stay. Obesity was associated with more complications but reduced inhospital mortality. Probably due to the relatively small number of patients, we did not prove any statistically significant difference. Key words: obesity paradox - BMI - colorectal cancer - resection - omplications.

[Internal hernia as a cause of small bowel obstruction]. / Vnitrní kýla jako prícina ileu tenkého streva.

Internal hernia is a rare cause of acute small bowel obstruction and may be difficult to diagnose. We would like to use two case reports to demonstrate that laparoscopy is a feasible treatment method for incarcerated internal hernias.Key words: intestinal obstruction - internal hernia - laparoscopy.

[Extraintestinal GIST--case report]. / Extraintestinální GIST--kazuistika.

Gastrointestinal stromal tumors (GISTs) represent the majority of mesenchymal neoplasms of the gastrointestinal tract. Extragastrointestinal stromal tumors (EGISTs), which are neoplasms with histological and immunohistological features overlapping with those of GISTs, are found in the abdomen outside of the gastrointestinal tract with no connection to the gastric or intestinal wall. We present a case of EGIST arising in the mesentery of a 68 years old patient.