[Rare case of primary pulmonary myxoid sarcoma]. / Primer myxoid tüdosarcoma ritka esete.

Mesenchymal tumors of the lungs are rare, mostly aggressive, with a high metastatic rate, representing only 0.013-1.1% of all pulmonary malignancies. Primary pulmonary myxoid sarcoma is an extremely rare type of lung sarcoma and stands as a separate entity in the 2015 WHO classification, characterized by EWSR1-CREB fusion gene. So far, 37 myxoid sarcoma cases have been reported. We offer an overview of the most important characteristics of pulmonary myxoid sarcoma and differential diagnosis, while reviewing the reported cases. We present the case of a 47-year-old patient with pulmonary myxoid sarcoma, who was diagnosed with a right central pulmonary mass, showing rapid endobronchial progression, complicated by empyema. EWSR1 gene translocation could not be detected. During chemotherapy, tumor progression occurred. Molecular genetic examinations revealed MET gene exon 14 skipping mutation, based on which tyrosine-kinase inhibitor treatment was administered. Pulmonary myxoid sarcoma can be classified as a nonvascular, spindle cell entity of mesenchymal tumors, with the characteristic EWSR1-CREB1 gene translocation. The male-female ratio is similar, with a slightly higher incidence in middle-aged women (1.5 : 1). Patients' average age is 44 years; with predilection in the right upper lobe (62%), or endobronchially (85%). Without specific symptoms, diagnosis is often cumbersome. Immunohistochemical methods, typical hystological image and molecular genetic tests confirm the diagnosis. Pulmonary myxoid sarcoma is a rare entity, without specific symptoms. In our case, myxoid sarcoma was complicated by empyema, which was drained. Because of advanced stage, surgical resection was not an option. Radical surgery offers the best results, in inoperable cases therapeutic recommendations for sarcomas are the guiding principles. Our case belongs to the rare group of myxoid sarcomas, where MET activating mutation was detected, making it eligible for targeted treatment. Orv Hetil. 2023; 164(27): 1077-1083.

[CUP in the liver]. / CUP in der Leber.

Hepatic involvement is one of the most common manifestations in cancer of unknown primary (CUP) syndrome. The most frequent secondary neoplasms of the liver are carcinomas and malignant melanomas. Most common carcinoma metastases are adenocarcinomas originating from the digestive system or metastases of breast and lung carcinomas. Therefore, hepatic CUP syndrome is an exclusion diagnosis. Immunohistochemistry and molecular examinations are an important part of histopathological diagnosis. They do not only serve to identify the tissue of histologically origin or possible primary tumor, but also contribute to the selection of a personalized targeted therapy by detecting so-called druggable targets in the interdisciplinary management.

Molecular mechanisms underlying the effects of temperature increase on Mytilus sp. and their hybrids at early larval stages.

The present work aims to investigate the effects of water temperature increase on Mytilus galloprovincilis and Mytilus edulis pure larvae (PG, PE) and their hybrids (HFG, HFE). D-larvae were maintained at 18 °C or exposed to a higher temperature of 22 °C for 48 h. Initially, Embryotoxicity test was evaluated. Second, a transcriptomic analysis using a recently developed microarray platform was applied to determine the main biological processes involved in early life stages responses to temperature increase. Finally, an immunofluorescence investigation was performed to bridge the gap between transcriptomic regulation and the real changes at cellular/tissue levels. Embryotoxicity test revealed a higher sensitivity of M. edulis (PE) D-larvae as well as hybrids from females M. edulis (HFE) to temperature increase, with the highest rate of larval malformations. Transcriptomic results indicated a lack of an adequate heat shock protein (Hsp) response in PE and HFE larvae (the high expression was observed in PG larvae); the differential expression of gene involved in translation, energy metabolism and oxidative stress response may contribute to explain the observed complex alterations in the studied conditions. As revealed by immunohistochemistry, cytoskeleton proteins changes associated with a drastic decrease of Histidine-Rich Glycoprotein (HRG) may elucidate the larval abnormalities in shell development observed for PE and HFE larvae. Overall, the results indicate that each type of pure larva (PG and PE) and their respective female hybrid (HFG and HFE) react similarly to the temperature increase. Our data should be carefully considered in view of the water temperature increase in marine ecosystems and especially for the mussel's species in confluence zones.

Masters of communication: The brain of the banded cleaner shrimp Stenopus hispidus (Olivier, 1811) with an emphasis on sensory processing areas.

The pan-tropic cleaner shrimp Stenopus hispidus (Crustacea, Stenopodidea) is famous for its specific cleaning behavior in association with client fish and an exclusively monogamous life-style. Cleaner shrimps feature a broad communicative repertoire, which is considered to depend on superb motor skills and the underlying mechanosensory circuits in combination with sensory organs. Their most prominent head appendages are the two pairs of very long biramous antennules and antennae, which are used both for attracting client fish and for intraspecific communication. Here, we studied the brain anatomy of several specimens of S. hispidus using histological sections, immunohistochemical labeling as well as X-ray microtomography in combination with 3D reconstructions. Furthermore, we investigated the morphology of antennules and antennae using fluorescence and scanning electron microscopy. Our analyses show that in addition to the complex organization of the multimodal processing centers, especially chemomechanosensory neuropils associated with the antennule and antenna are markedly pronounced when compared to the other neuropils of the central brain. We suggest that in their brains, three topographic maps are present corresponding to the sensory appendages. The brain areas which provide the neuronal substrate for these maps share distinct structural similarities to a unique extent in decapods, such as size and characteristic striated and perpendicular layering. We discuss our findings with respect to the sensory landscape within animal's habitat. In an evolutionary perspective, the cleaner shrimp's brain is an excellent example of how sensory potential and functional demands shape the architecture of primary chemomechanosensory processing areas.

First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions.

IgG4-related disease is a fibro-inflammatory disorder characterized by swelling of tissues and affected organs accompanied by the development of scar tissue (fibrosis) and infiltration by IgG4 positive plasma cells. Almost any organ can be affected, including, but rarely, bone marrowinvolvement. Here we present a case of a 76-year-old male with IgG4-related disease presenting primarily with vertebral bone marrow lesions. Histopathology showed the typical features of storiform fibrosis, and increased IgG4 positive plasma cells. Treatment with corticosteroids significantly improved wellbeing and resolved lesion size on MRI.

Comparative study on serum-induced arthritis in the temporomandibular and limb joint of mice.

INTRODUCTION: The subject of the present study was a systematic comparative analysis of the rheumatoid arthritis (RA)-induced pathomechanisms in the temporomandibular joint with those of the limb joints using the serum-induced arthritis K/BxN model. METHODS: In 18 BALB/c mice the induction of RA was performed according to the Kouskoff method. Another healthy cohort served as controls (n = 12). Joint swelling of the paws was measured using a micrometer. Functional data were obtained analyzing locomotion. Three-dimensional examination of the temporomandibular joint was performed with micro-computed tomography imaging, followed by histological evaluation of the extremity joints and the temporomandibular joint. Additionally, immunohistochemical investigations were carried out to evaluate inflammatory and immunological changes. RESULTS: Measurement of joint swelling showed a significant increase in the diameter of the paws, as well as a decrease in locomotor activity compared to control animals and the time before arthritis induction. Histological and immunohistochemical investigations showed clear signs of inflammation in the extremity joints. In contrast, no histological or immunohistochemical indications of an inflammatory process were detectable in the temporomandibular joint. In addition, the three-dimensional analysis by micro-computed tomography of the temporomandibular joints did not show any obvious morphological changes. CONCLUSION: For the first time, using the K/BxN model we could demonstrate that, due to its anatomical and mechanical conditions, the temporomandibular joint seems to be less susceptible to the initiation of RA compared to limb joints. Therefore, additional investigations are needed on other arthritis models as well, in order to further improve our understanding of the pathogenesis and defense mechanisms of the disease.

[Staging of oropharyngeal carcinomas : New TNM classification as a challenge for head and neck cancer centers]. / Staging von Oropharynxkarzinomen : Neue TNM-Klassifikation als Herausforderung für Kopf-Hals-Tumorzentren.

BACKGROUND: The TNM (tumor, nodes, metastasis) classification is updated periodically according to the literature and international recommendations. With the 8th edition, notable changes have been developed especially with regard to oropharyngeal cancer. MATERIALS AND METHODS: The modifications as well as the practicability of the classification for staging of oropharyngeal cancers are demonstrated on the basis of cases from the tumor database. RESULTS: The latest edition of the TNM classification realizes requirements to differentiate between human papilloma virus (HPV) positive and HPV-negative tumors during staging. Furthermore, the prognostic relevance of extranodal extension of lymph node metastases was integrated into the classification. While downstaging is performed regarding N category and Union Internationale Contre le Cancer (UICC) stage in many p16-positive tumors, for p16-negative tumors, extranodal spread mostly leads to a notable upstaging. Due to limited specificity of the p16 immunostaining, the relevance of false positive results has to be underlined. Missing integration of smoking behavior, limited standardization of the extranodal extension examination technique, as well as high demands on the documentation quality should be kept in mind. CONCLUSION: Clinical trials will have to show whether deescalation strategies regarding p16-positive carcinomas are supported by the changes made in the TNM staging system. A prospective multicenter study should examine the universal applicability, the appropriateness for all sublocations, as well as the prognostic significance of the current edition.

Prognostic significance of Tspan9 in gastric cancer.

Tetraspanins are a large superfamily of glycoproteins, which are engaged in a wide range of specific molecular interactions by forming tetraspanin-enriched microdomains. Tetraspanin 9 (Tspan9) is a previously poorly studied tetraspanin gene, which was predominantly identified as an amplified gene in serous Fallopian tube carcinoma. However, the expression and role of Tspan9 in gastric cancer have yet to be fully elucidated. The aim of the present study was to evaluate the expression and clinical significance of Tspan9 in gastric cancer. In the present study, 105 gastric cancer tissue samples and corresponding adjacent normal samples were detected for Tspan9 expression using immunohistochemistry; furthermore, the association between clinical characteristics and Tspan9 expression was also analyzed. Tspan9 expression was determined to be significantly lower in cancer samples compared with those in corresponding adjacent normal samples (P<0.001). However, its increased levels of expression in cancer samples appeared to demonstrate a poorer prognostic tendency, which is associated with deeper tumor depth (P=0.025), more nodal involvement (P=0.01), more advanced tumor/lymph node/metastasis (TNM) stages (P=0.017) and a larger tumor size (P=0.026). Additionally, multivariate analysis demonstrated that high expression of Tspan9 was an independent prognostic factor for poor overall survival (P<0.01). These results suggested that Tspan9 may be used as a potential prognostic factor in gastric cancer.

Expression of P33(ING1b) Protein in Colorectal Cancer.

BACKGROUND Colorectal cancer (CRC) is the second most common malignancy in the world. However, its mortality rate can be reduced if diagnosed early. P33ING1b is a tumor suppressor protein, which plays a role in growth control and apoptosis. Suppression of p33(ING1b) is associated with the loss of cellular growth control. However, p33 (ING1b) expression in CRC and its correlations with clinicopathological factors have been less studied. The aim of this study was to examine p33(ING1b) expression in patients with CRC and evaluate its potential correlations with clinicopathological factors. METHODS P33(ING1b) protein expression was examined in 70 cases of CRC tissue samples and their corresponding neighboring normal tissues by immunhistochemistry. Moreover, p33(ING1b) expression in CRC and its correlations with clinicopathological variables including patients' sex and age, tumor type, location, stage, and differentiation grade were examined. RESULTS P33(ING1b) expression was significantly lower in tumor samples compared with the normal adjacent samples (p<0.002). CONCLUSION Low expression of P33(ING1b) in patients with colorectal cancer, may be an important molecular event in the pathogenesis of colorectal cancer. Our data suggest that reduced expression of p33(ING1b) may be contribute to tumor genesis and accompanied by the loss of cellular growth control. In fact cell growth is out of control in lower expression of P33 and dysfunctional program cell death. P33 expression might explain the etiology of CRC for reducing the expression of tumor suppressor proteins.

Low-grade fibromyxoid sarcoma of anterior abdominal wall.

Low grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor, having metastatic potential and high local recurrence rate despite its low grade histologic findings. This tumor of deep and subcutaneous soft tissues occurs in 3rd and 5th decade of life. Diagnosis of LGFMS remains problematic because of its bland looking histologic features that can be potentially confused with other benign or low grade soft tissue tumors. We report here a rare case report of low grade fibromyxoid sarcoma of anterior abdominal wall.