Hungarian Radiology

[Éva Kis (editor): Cases of Pediatric Radiology]

MOHAI Gabriella

FEBRUARY 15, 2005

Hungarian Radiology - 2005;79(01)

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Hungarian Radiology

[2005, International Year of Physics]

LOMBAY Béla

Hungarian Radiology

[Gastrointestinal stromal tumors]

BAHÉRY Mária

[Gastrointestinal stromal tumors are the most common mesenchymal neoplasm of the gastrointestinal tract. Gastrointestinal stromal tumors are characterized by remarkable variability in their differentiation potential. They are defined by their expression of KIT (CD117), a tyrosine kinase growth factor receptor. The expression of KIT is important to distinguish gastrointestinal stromal tumors with immunohistochemically method from other mesenchymal neoplasms such as leiomyomas, leiomyosarcomas, leiomyoblastomas and schwannomas. Pathologically proved gastrointestinal stromal tumors are appropriate KIT-inhibitor therapy. Gastrointestinal stromal tumors arise with variable frequency throughout the gastrointestinal tract, they most frequently occur in the stomach (40-70%), followed by the small intestine (20-30%), colorectum (5- 15%) and esophagus (<5%). The most gastrointestinal stromal tumors arise within the muscularis propria, they most commonly have an exophytic growth pattern and manifest as dominant extraluminal masses. Radiologic features of gastrointestinal stromal tumors vary depending of tumor size and organ of origin. They are characteristically well circumscribed, sharply defined and have hemorrhage, necrosis or cyst formation. The radiologic features of gastrointestinal stromal tumors are often distinct from those of epithelial tumors. There are no specific radiologic features to separate gastrointestinal stromal tumors from other mesenchymal tumors, yet.]

Hungarian Radiology

[Esophageal diverticula in mixed connective tissue disease]

SZÁNTÓ Dezső, SZŰCS Gabriella, DITRÓI Edit

[INTRODUCTION - The functional and morphological changes of oesophagus occur in two third of mixed connective tissue disease patients according to the literature. CASE REPORT - We report three cases of 27, 39 and 48 year old women suffering of lateral pharyngoesophageal, epibronchial and epiphrenic diverticula associated with connective tissue disease. Diverticula had an average diameter of 3.8 cm (maximal diameters: 7.2-8 cm). The esophageal pouches produced dysphagia, dystonia, motility disorders, food stagnation and vomiting, retrosternal burning sensation and tachyarrythmia after 5-16 month's latency period. In one patient pneumoesophagus also evolved. The high serum enzyme levels and proximal electromyogram proved the presence of polymyositis. CONCLUSION - The localization, number and the size of esophageal diverticula are determined by the interstitial myopathy.]

Hungarian Radiology

[Basics and methods of radiostereometric analysis in orthopaedic practice]

ILLYÉS Árpád, KISS Rita M., KÖLLŐ Katalin, KISS Jenő

[Photogrametry is a discipline processing geometric information of images according to the general image concept. Radiostereometric analysis (Roentgen stereophotogrametric analysis) is the most dynamically developing area of the orthopaedic photogrametry. The aim of the authors was to summarize the different methods of radiostereometric analysis in use. Two basically different methods are used: marker-based and model-based radiostereometric analysis. The authors summarize and compare the steps, the advantages and the disadvantages of either method. Radiostereometric analysis is because of its high precision appropriate as a tool in scientific research and diagnostic in orthopaedics, such as detecting the 3D micromotion of different components of implants, the wear of polyethylene inserts or determining anterior-posterior and rotational movements of the knee. Analyzing the reproducibility and accuracy of the reconstruction methods one can establish that the accuracy of analytical methods in use is behind the accuracy of digital automated software. The precision of the model-based radiostereometry can never achieve the accuracy of the marker based radiostereometry because of the inaccuracy of the initial input model of implants, although the one of the method of reversed engineering - one of model-based radiostereometric analysis - can approach the accuracy of marker-based radiostereometry.]

Hungarian Radiology

[Solitary fibrous tumor of the chest]

AGÓCS Ágnes, KISHINDI KISS Katalin, PENCS Mónika, TÓTH Tivadar

[INTRODUCTION - Solitary fibrous tumor is a rare, in most of the cases benign tumor of mesenchymal origin arising from the pleura. CASE REPORT - Significantly raised right hemidiaphragm was noted on the chest X-ray of an elderly female patient with good general condition. A large solid mass lesion was seen above the normally positioned right hemidiaphragm on ultrasound and chest CT examinations. The mass was removed by surgical resection and a solitary fibrous tumor was confirmed by histological and immunhistochemical examinations. CONCLUSION - Solitary fibrosus tumor is a rare and histologically benign tumor. Occasionally it may enlarge rapidly and transform into malignant variant after several years. Therefore complete surgical resection and long term follow up is needed in all cases.]

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Cases of inborn errors of metabolism diagnosed in children with autism

CAKAR Emel Nafiye, YILMAZBAS Pınar

Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

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Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

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[Diagnosis and treatment of microvascular coronary heart disease. Specialities of conditions in Hungary]

SZAUDER Ipoly

[Invasive investigations show that in two-thirds of patients the myocardial ischaemia persists without obstructive coronary disease and any other heart conditions (INOCA). The underlying cause may be microvascular dysfunction (CMD) with consecutive microvascular coronary disease (MVD) and microvascular or epicardial vasospastic angina (MVA). The modern practice of clinical cardiology while using the developed non-invasive cardiac imaging permits exact measuring of the coronary flow with its characteristic indices. All of these improve the diagnosing of CMD-induced myocardial ischemia and provide opportunity to determine primary MVD cases. Since the recognition and treatment of MVD is significantly underrep­resented in the Hungarian medical care, the primary stable microvascular angina (MVA) is described in detail below with its modern invasive and non-invasive differential diagnosis and treatment, concerning especially its frequency provoked by high blood pressure and female coronary heart diseases. There are highlighted all recommended diagnostic procedures available under domestic conditions.]

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[Is the implementation of Vojta therapy associated with faster gross motor development in children with cerebral palsy? ]

SANZ-MENGIBAR Jose Manuel , MENENDEZ-PARDIÑAS Monica , SANTONJA-MEDINA Fernando

[Vojta therapy has been reported as clinically beneficial for strength, movement and gross motor activities in individual cases and is being included within the second of three levels of evidence in interventions for cerebral palsy. The goal of this study is to understand the effect of Vojta therapy on the gross motor function. Our clinical trial followed a one group, pre-post design to quantify rates of changes in GMFM-88 after a two-months period undergoing Vojta therapy. A total of 16 patients were recruited. Post-intervention acceleration rates of GMFM-88-items acquisition (0.005; p<0.001) and Locomotor Stages (1.063; p<0.0001) increased significatively following Vojta the­rapy intervention. In this study, Vojta therapy has shown to accelerate the acquisition of GMFM-88-items and Loco­motor Stages in children with cerebral palsy younger than 18 months. Because functional training was not utilised, and other non-Vojta therapy intervention did not influence the outcome, Vojta therapy seems to activate the postural control required to achieve uncompleted GMFM-88-items. ]

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Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.