Lege Artis Medicinae

[RARE UTERINE MALIGNANCY: THE ENDOMETRIAL ADENOSARCOMA]

TARJÁN Miklós, SÁPI Zoltán, BENTZIK András, CSERNI Gábor

SEPTEMBER 18, 2004

Lege Artis Medicinae - 2004;14(08-09)

[INTRODUCTION - Müllerian adenosarcoma is typically composed of benign glandular epithelial elements admixed with malignant sarcomatous stroma. This rare tumour usually originates in the endometrium and grows as a polypoid mass into the endometrial cavity. CASE REPORT - We report the case of a woman aged 32, who presented with abnormal vaginal bleeding and was diagnosed with an adenosarcoma after a curettage and subsequent hysterectomy. CONCLUSION - This tumour has a relatively good prognosis among the mixed mesenchymal sarcomas of the uterus and the majority of patients with this neoplasm survive. The gynecologists and pathologists should be aware of this entity and discuss the risks and benefits of radiation and chemotherapy, which is usually indicated for malignant diseases.]

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Lege Artis Medicinae

[Abdominal compartment syndrome: the cause of postinjury multiple organ failure. Summary of the article]

BALOGH Zsolt, MCKINLEY BA, COX CS Jr, ALLENS SJ, COCANOUR CS, KOZAR RA, MOORE EE, MILLER CC, WEISBRODT NW, MOORE FA

Lege Artis Medicinae

[FIXED-COMBINATION THERAPY IN ANTIHYPERTENSIVE TREATMENT]

BARNA István

[Target blood pressure and reduction in cardiovascular morbidity and mortality have to be accomplished always with the consideration of target organ lesion. Fixed-combination antihypertensive therapy has become accepted based on the recommendations of international and Hungarian medical associations that are aware of the results of evidence based medicine. The fix combinational treatment is more favorable for patients’ compliance. The daily dosage of drugs with 24 hour effect improves the patients’ cooperativeness. During fix combinational treatment higher efficiency, smaller dose and fewer side effects were observed. In combination, the effects of the drugs on blood pressure are additive without any increase in adverse effects. Last, but not least the cost of drugs of fixed combination are lower than buying the drugs separately. After careful consideration the fixed combination therapy can be recommended to the everyday practice.]

Lege Artis Medicinae

[The diagnosis and therapy of chronic obstructive pulmonary disease - Recommendation of the 2003 consensus conference]

BÖSZÖRMÉNYI Nagy György

Lege Artis Medicinae

[RISKS AND BENEFITS OF MEDICATION IN PREGNANT WOMEN]

CZEIZEL Endre

[The Hungarian Case-Control Surveillance System of Congenital Abnormalities can provide appropriate data for the evaluation of risk and benefits of drugs and pregnancy supplements in pregnant women. Among the main principles, the importance of time factor (the first trimester concept is outdated) and the explanation of frequent false teratogenic findings (e.g. recall bias, chance effect, etc.) are discussed. The main conclusion is that at present the exaggerated teratogenic risk of drugs is much more harmful for the fetus than the rare teratogenic effect of some drugs themselves. Medical doctors and other experts therefore need more education to know the principles and findings of modern human teratology because it may help us to have a better balance between the risks and benefits of drug use during pregnancy. On the other hand, the primary prevention of neural-tube defects and some other congenital abnormalities is demonstrated by periconceptional folic acid or folic acid-containing multivitamin supplementation. Unfortunately, this new method is used rarely and inappropriately (due to the late onset of supplementation) in Hungary.]

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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.

Clinical Neuroscience

Atypical presentation of late-onset Sandhoff disease: a case report

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Clinical Neuroscience

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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.

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