Historia leczenia nowotworów wieku dziecięcego

W pracy przedstawiono historię odkryć dotyczących onkologii, w tym także onkologii dziecięcej, początki i rozwój metod terapeutycznych stosowanych w onkologii od chirurgicznego leczenia nowotworów, przez promieniolecznictwo i odkrycie pierwszych cytostatyków. Omówiono początki kompleksowego leczenia nowotworów dziecięcych, które doprowadziły do dynamicznego postępu w leczeniu tych chorób, aż do osiąganej obecnie wyleczalności chorób nowotworowych u ponad 70% pacjentów w wieku rozwojowym z tym rozpoznaniem. Zwrócono uwagę na możliwe zwiększenie intensywności leczenia dzięki stosowanym przeszczepom komórek krwiotwórczych oraz udoskonaleniu terapii wspomagającej. Omówiono też aktualnie wprowadzane nowe metody leczenia w hematoonkologii dziecięcej, które skierowane są wybiórczo na hamowanie rozwoju nowotworu i mogą stanowić cenne uzupełnienie dotychczasowych metod terapii

Localisation of focal liver lesions to specific hepatic segments - comparison of multiphase spiral CT and MR imaging

The purpose of this study was an evaluation of the ability of the mulitiphase spiral CT and MR imaging to localise focal liver lesions referring to specific hepatic segments. The authors studied prospectively 26 focal liver lesions in 26 patients who had undergone spiral CT and MRI before surgery. Multiphase spiral CT included noncontrast scans, hepatic arterial-dominant phase, portal venous - dominant phase and equilibrium phase. MRI was performed in all cases. The following sequences were performed: SE and TSE T1- and T2-weighted images, STIR and dynamic T1-weighted FFE study after i.v. administration of gadolinium (Gd-DTPA). The CT and MR scans were prospectively and independently reviewed by three radiologists for visualisation of hepatic and portal veins and segmental localisation of hepatic lesions. The authors used the right and left main portal veins along with transverse fissura, hepatic veins and gallbladder fossa as landmarks for the tumour localisation to specific hepatic segments. The primary segmental locations of the lesions were correctly determined with CT in 22 of 26 focal liver lesions (85%) and with MR imaging in 24 of 26 lesions (92%). The full extent of lesions was correctly described with sCT in 19 of 26 focal lesions and with MR in 21 of 26 tumours. MRI and CT were helpful preoperative tools for determining the segmental location of focal liver lesions and for planning the surgical approach

Capecitabine and temozolomide combination for treatment of high-grade, well-differentiated neuroendocrine tumour and poorly-differentiated neuroendocrine carcinoma — retrospective analysis

Introduction: Many retrospective studies have confirmed that capecitabine combined with temozolomide is effective in neuroendocrine neoplasms. Most of the studies focused on grade 1 and grade 2 neuroendocrine tumours, mainly of pancreatic origin. There are limited data regarding the efficacy capecitabine with temozolomide in grade 3 neuroendocrine tumours. The new World Health Organisation 2017 classification distinguished well-differentiated grade 3 neuroendocrine tumours from poorly differentiated grade 3 neuroendocrine carcinomas. Treatment options for grade 3 neuroendocrine neoplasms are limited, and the overall prognosis is better in the subgroup of patients with grade 3 neuroendocrine tumours. Material and methods: It was a retrospective study in the population of patients with diagnosed grade 3 neuroendocrine neoplasms of different origin treated with capecitabine and temozolomide. Data on clinical and demographic characteristics of the population were collected from four Polish clinical centres. This study aimed to evaluate response and survival parameters and compare outcomes of treatment of neuroendocrine tumours and carcinomas. Results: The study included 32 patients with grade 3 neuroendocrine tumours treated with capecitabine and temozolomide. The disease control rate was twice as high in the group of patient with neuroendocrine tumours in comparison to carcinomas (70 vs. 30%). The progression-free survival for patients with neuroendocrine tumours was 15.3 months (95% CI: 3.9–30.4), and for patients with neuroendocrine carcinomas it was 3.3 months (95% CI: 2.5–7.1). Median overall survival was 22 months (95% CI: 11.8–22.0) and 4.6 months (95% CI: 2.2–5.9) for patients with tumours and carcinomas, respectively. The treatment regimen was generally well tolerated. Conclusions: The combination of capecitabine and temozolomide is an effective treatment for patients with grade 3 neuroendocrine tumours with Ki-67 index ranging between 20 and 54%. The treatment did not overcome the aggressive character of neuroendocrine carcinomas and resulted in low response and survival outcomes in comparison to those achieved in tumour therapy

Dermoscopic Features of Giant Molluscum Contagio Contagiosum in a Patient with Acquired Immunodeficiency Syndrome

Giant molluscum contagiosum (MC) is a peculiar variant of the disease with the presence of multiple or single lesions larger than 5 mm. In contrast to typical molluscum contagiosum, dermoscopic features of giant lesions have been poorly described, and none of the reports included multiple giant lesions in an immunocompromised patient. We present a patient with acquired immunodeficiency syndrome diagnosed with multiple giant molluscum contagiosum along with the dermoscopic features of this entity

Pulmonary actinomycosis a case report

Promienica płuc jest rzadką chorobą wywołaną przez bakterie z rodzaju Actinomyces. Objawy kliniczne i wyniki badań obrazowych są w tej chorobie mało charakterystyczne, co utrudnia ustalenie właściwego rozpoznania. Przedstawiony przypadek dotyczy 59- letniego mężczyzny z promienicą płuc spowodowaną niewłaściwą higieną jamy ustnej. Proces chorobowy obejmował miąższ płucny i naciekał ścianę klatki piersiowej powodując osteolizę mostka i powstanie przetok skórnych. Wyniki badań obrazowych sugerowały proces rozrostowy. Ostateczne rozpoznanie ustalono na podstawie wyniku badania histopatologicznego wyskrobin z przetoki skórnej, w którym wykryto kolonie promieniowców. Po sześciu miesiącach antybiotykoterapii stan chorego uległ znacznej poprawie, a zmiany skórne uległy wygojeniu. Wyniki kontrolnych badań radiologicznych wykazały regresję zmian płucnych.Pulmonary actinomycosis is a rare disease caused by Actinomyces sp. Its symptoms and radiological findings are not characteristic, so the diagnosis might be difficult to establish. We report a case of a 59 year old male, who developed bronchopulmonary Actinomycosis due to poor dental hygiene. The infectious process affected lung parenchyma and infiltrated chest wall causing multifocal sternal osteolisis and multiple cutaneous fistulas. The radiological findings sugested neoplasmatical process. The diagnosis was based on histopatological findings of fistular scrapes. The material contained Actinomyces colonies. Afler 6 months of antibiotic therapy the patient’s state improved and the cutaneous fistulas healed. Radiological finding revealed partial resolution of the lung infiltration

c.1810C>T Polymorphism of NTRK1 Gene is associated with reduced Survival in Neuroblastoma Patients

<p>Abstract</p> <p>Background</p> <p>TrkA (encoded by <it>NTRK1 </it>gene), the high-affinity tyrosine kinase receptor for neurotrophins, is involved in neural crest cell differentiation. Its expression has been reported to be associated with a favourable prognosis in neuroblastoma. Therefore, the entire coding sequence of <it>NTRK1 </it>gene has been analysed in order to identify mutations and/or polymorphisms which may alter TrkA receptor expression.</p> <p>Methods</p> <p>DNA was extracted from neuroblastomas of 55 Polish and 114 Italian patients and from peripheral blood leukocytes of 158 healthy controls. Denaturing High-Performance Liquid Chromatography (DHPLC) and Single-Strand Conformation Polymorphism (SSCP) analysis were used to screen for sequence variants. Genetic changes were confirmed by direct sequencing and correlated with biological and clinical data.</p> <p>Results</p> <p>Three previously reported and nine new single nucleotide polymorphisms were detected. c.1810C>T polymorphism present in 8.7% of cases was found to be an independent marker of disease recurrence (OR = 13.3; p = 0.009) associated with lower survival rates (HR = 4.45 p = 0.041). c.1810C>T polymorphism's unfavourable prognostic value was most significant in patients under 18 months of age with no <it>MYCN </it>amplification (HR = 26; p = 0.008). <it>In-silico </it>analysis of the c.1810C>T polymorphism suggests that the substitution of the corresponding amino acid residue within the conservative region of the tyrosine kinase domain might theoretically interfere with the functioning of the TrkA protein.</p> <p>Conclusions</p> <p><it>NTRK1 </it>c.1810C>T polymorphism appears to be a new independent prognostic factor of poor outcome in neuroblastoma, especially in children under 18 months of age with no <it>MYCN </it>amplification.</p

Hypoxic hepatitis as a complication of newly diagnosed type 1 diabetes in a teenager

Hypoxic hepatitis is a rare complication of type 1 diabetes with unknown prevalence in Pediatrics. We present a case report of an 11-year-old boy admitted to the ER in the spring of 2020 (the beginning of the COVID19 pandemic in Poland) due to nausea, abdominal pain, and weight loss. A diagnosis of type 1 diabetes accompanied by severe ketoacidosis (pH 6.9, blood glucose 632mg/dl, ketone bodies in urine – 150mg/dl) was made. The hyperglycemia, ketoacidosis, and water-electrolyte disturbances were treated in the Pediatric Intensive Care Unit. On day 4, the boy developed fulminant septic shock with high aminotransferases (AST 9026 U/l, ALT 3559 U/l). CT scan revealed hepatic enlargement and steatosis. Acute viral hepatitis was suspected. The levels of anti-CMV IgM and IgG antibodies were slightly elevated. At autopsy, the liver was enlarged, with petechial bleedings on the surface. The liver parenchyma was congested, with signs of steatosis. Microscopically, there was extensive centrilobular necrosis, acute passive sinusoidal congestion, and steatosis of hepatocytes. There were no signs of CMV infection. Based on the entire clinicopathological picture, the patient was diagnosed with hypoxic hepatitis, complicated by septic shock and multiple organ failure

Cerebral microbleeds in neurological practice: concepts, diagnostics and clinical aspects

Introduction: Due to the widespread use of magnetic resonance imaging (MRI) in neurological diagnostics, the number of patients detected as having cerebral microbleeds (CMBs) continues to increase. However, their clinical impact still remains controversial, especially the question of whether CMBs significantly increase the risk of life-threatening intracerebral haemorrhage (ICH) in patients undergoing intravenous thrombolysis (IVT) or endovascular thrombectomy (EVT), or in patients on anticoagulant therapy or statins.State of the art: The term ‘CMB’ is a radiological concept that aims to illustrate microscopic pathology of perivascular hemosiderin deposits corresponding most probably to small foci of past bleeding. MRI images in sequence T2*-GRE and susceptibility-weighted imaging (SWI) are used for a diagnosis of a CMB. This review summarises the current knowledge regarding the definition, prevalence, genetics, risk factors, radiological diagnosis and differential diagnosis of a CMB. We discuss its role as an indicator of future ischaemic or haemorrhagic events in high risk patients or those on antiplatelet or anticoagulant therapy, and its prognostic value for reperfusion strategies and for the development of dementia.Future direction: The place of CMBs in current guidelines is explored herein. It must be emphasised that the recommendations relating to CMBs are expert opinions. Therefore, at the end of this review, we pose a number of questions that future clinical trials should answer

Calcifiying Fibrous Pseudotumor of the Small Bowel Mesentery – Case Report

Calcifiyng fibrous pseudotumor (CFPT) is a benign mesenchymal tumor diagnosed in children and young adults, located in the subcutaneous tissue of the trunk and limbs. Its intraabdominal localization is a unique rarity. The Authors of the study presented a case of a 48-year old female patient with an accidentally diagnosed small bowel mesentery tumor during surger

Insight into the history of anatomopathological museums – Part 1. From casual assemblages to scientific collections

We present a short history of anatomopathological museums in Europe. In the first part we provide an insight into the beginnings from the Renaissance until the middle of the 19th century. We assess forms of acquisition and exhibition of the specimens concerning the steps of medicine and pathology development. The prototypes were “curiosities of nature” collections starting in the 15th century. The next milestone collections focusing on the human body were those of Frederik Ruysch in the Netherlands (17th century). In the 18th century teachers in surgical and anatomical schools realized the educational power of such collections. Anatomopathology as a separate medical discipline was developing in parallel. At that time museums such as the one established by Honoré Fragonard in Paris, the Hunterian in Glasgow and Narrenturm in Vienna were created. At Polish universities in Cracow and Vilnius, such museums were beginning to emerge at the beginning of the 19th century. Anatomopathological collections became more popular, gathering specimens: osteological, dry and mummified, as well as wet – embedded in alcohol, formalin, and mysterious mixtures. They provide a wealth of important data for scientific, medical, historical and even ethical areas, as well as methods and concepts of conservation and even recreation of human body parts