Lege Artis Medicinae

[Plasmocytoid myoepithelioma of the parotid gland]

HAJDU Mária, KRUTSAY Miklós

JUNE 20, 2013

Lege Artis Medicinae - 2013;23(05-06)

COMMENTS

0 comments

Further articles in this publication

Lege Artis Medicinae

[The place of fulvestrant in the treatment of metastatic breast cancer]

BÁNHEGYI Róbert János, FÜLÖP Ferenc, MELLÁR Erzsébet, PIKÓ Béla

Lege Artis Medicinae

[Optimizing the efficacy of triple combination therapy of chronic hepatitis C]

TORNAI István

[The outcome of chronic hepatitis C (CHC) therapy has been improved significantly. If sustained virologic response (SVR) is achieved, then it may prevent the occurrence of liver failure and hepatocellular carcinoma. With the currently used double combination therapy (peginterferon and ribavirin) SVR can be achieved in 40-50% of patients with genotype 1. In treatment naive patients, triple combination with protease inhibitors can result in 70-75% SVR. In treatment experienced patients, however, the result of the previous therapy, which mostly depends on the reaction to interferon (IFN), has a significant influence on the outcome of triple combination. INF sensitivity is the highest in relapsers, triple combination can achieve about 85% SVR, while in null responders this is only 30%. Viral resistance is a new phenomenon during triple combination therapy of CHC. In poorly IFN responsive patients the virus is effectively exposed to protease inhibitor functional monotherapy, leading to the rapid emergence of resistant virus. IFN sensitivity is well represented by the on-treatment viral response, therefore the knowledge of the previous viral response, relapse, a partial response or a null response is absolutely important. Optimization of triple therapy is crucial, since for a lot of patients with advanced liver disease it might be the last chance to achieve an SVR. The selection of the patients seems very important. Relapsers are the best candidates, there is no doubt with the indication. However, there are many debates for cirrhotic nullresponders, since the most virological failures are expected in this group. Prevention of viral resistance is crucial. PegIFN and ribavirin suppress both wild-type and resistant virus. PegIFN α-2a based treatment proved to be the most effective backbone for triple combination. This combination should be preferred especially for treatment experienced patients. Adherence to therapy is also critically important to prevent resistance. If resistant mutants appear, treatment should be stopped promptly.]

Lege Artis Medicinae

[A Teacher who Created a School ]

BEDROS J. Róbert

Lege Artis Medicinae

[Development of the diagnostic criteria of Alzheimer’s disease]

KOVÁCS Tibor

[Alzheimer’s disease is a new endemic of the 21st century, which is going to become the biggest health and social problem of the ageing societies in the next few decades. Significant discoveries have been made by structural and functional imaging and biochemical (especially CSF) and genetic tests regarding the diagnosis of Alzheimer’s disease. The modern diagnostic results were recently incorporated into the diagnostic criteria. This review summarises these diagnostic results.]

Lege Artis Medicinae

[Summer Exhibitions at the Museum of Fine Arts ]

NAGY Zsuzsanna

All articles in the issue

Related contents

Clinical Neuroscience

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.

Clinical Neuroscience

A rare entity of acquired idiopathic generalised anhidrosis which has been successfully treated with pulse steroid therapy: Does the histopathology predict the treatment response?

ÖKTEM Özdemir Ece, ÇANKAYA Şeyda, UYKUR Burak Abdullah, ERDEN Simsek Nazan, YULUG Burak

Acquired idiopathic generalised anhidrosis is an uncommon sweating disorder characterized by loss of sweating in the absence of any neurologic, metabolic or sweat gland abnormalities. Although some possible immunological and structural mechanisms have been proposed for this rare entity, the definitive pathophysiology is still un­clear. Despite some successfully treated cases with systemic corticosteroid application, the dose and route of steroid application are controversial. Here, we present a 41-year-old man with lack of genera­lised sweating who has been successfully treated with high dose pulse intravenous prednisolone. We have discussed his clinical and histopathological findings as well as the treatment options in view of the current literature.

Hungarian Radiology

[A rare pancreatic mass in childhood]

TORDAS Adél, PALOTAI Andrea, KISS Imre, KIS Éva

[INTRODUCTION - Malignant pediatric pancreas tumors are rare in the pediatric age group. Among these tumors the malignant hemangiopericytoma is an even more rare condition. CASE REPORT - We have diagnosed this soft tissue sarcoma in a three month old infant during a screening abdominal ultrasound examination. The examination showed a space-occupying lesion in the region of the pancreas and the adrenals. Following further diagnostic imaging, a complete surgical resection was performed. Histology showed malignant hemangiopericytoma. The child received a 5 month long, successful adjuvant chemotheraphy. CONCLUSIONS - Malignant hemangiopericytoma belongs to the non-rhabdomyosarcoma group of diseases. Two subtypes have been described: infantile-type ( hemangiopericytoma) in infants under 1 year, and the adult-type disease in children over 1 year of age. About one third of the infantile subtype are considered congenital. Most common anatomic locations are the retroperitoneum, the pelvis, the extremities, the head and neck region. Prognosis is favorable, the 10-year-survival rate is 80%. Differential diagnosis includes other tumors of the region, such as lesions of the adrenal gland, kidney, stomach and pancreas.]

Hungarian Immunology

[Ocular myositis]

KISS Emese, FACSKÓ Andrea, DÉVÉNYI Katalin, DANKÓ Katalin, ZEHER Margit

[INTRODUCTION - Dermato-/polymyositis is an autoimmune disorder, which belongs to the idiopathic inflammatory myopaties. It involves skeletal muscles in form of weakness and inflammatory infiltrates. Characteristic skin lesions are present in dermatomyositis. Other organs may also be affected mainly in the presence of myositis specific autoantibodies. The inflammation usually involves the proximal muscles of extremities. CASE REPORT - In the present work we report the case of a 52-year-old woman. In the previous history the removal of rectal adenocarcinoma was remarkable in 1994. After that she received chemotherapy. She complied for severe headache and pain in the right eye in 2000 October, therefore a skull CT was performed, indicating thickening of rectus medalis muscle within orbital cavity. There was an enhancement of contrast material in the muscle. Glaucoma was excluded. Neurologist suspected the presence of myositis and indicated 0.5 mg/kg corticosteroid therapy. Soon after the left eye became painful, but due to the corticosteroid treatment both eyes became painless. A control orbital CT was completely negative in 2000 November. Immunology consultancy revealed a mild proximal muscle atrophy in both lower extremities, but CPK and LDH enzyme levels were normal, EMG was characteristic for mild chronic nerve lesion. The biopsy, taken from the involved proximal muscle of lower extremity, did not show inflammatory infiltration. Complete screening for cancer was negative. Thyroid gland disease could be excluded. Immune laboratory data were negative, autoantibodies, including anti-Jo1, could not be detected. Based on the results a rare disease, ocular myositis was diagnosed. Considering the clinical improvement, the withdrawal of corticosteroid therapy was offered. Stringent immunology and oncology follow-up is required. CONCLUSION - In relation to our case report, we discuss clinical symptoms of orbital myositis, diagnostic procedures to identify the disease and also differential diagnostic considerations.]

Lege Artis Medicinae

[The complexity of hyper-lipidaemia’s follow-up in a polymorbid patient diagnosed with newfound Parkinson’s disease]

HANG Dóra

[The 73-year-old polymorbid man has been examined because of above normal blood pressure and increased serum lipid levels. From his already known diseases subclinic hypothyreosis should be highlighted. Because of increased serum cholesterol (6.7 mmol/l) and LDL levels (4.91 mmol/l) 40 mg atorvastatin has been adjusted besides 10 mg amlodipine against his high blood pressure. Subsequently selegiline and levodopa/benserazid therapy have been adjusted caused by newly diagnosed Parkinson’s disease. Parallel in time decreased dosage of atorvastatin (20 milli- gramms) has been enabled to adjust as well, the pill taken by the patient is combinated with 10 milligramms of amlodipine and called Amlator®. The improvement of lipid levels might be due to the trasformation of subclinic hypothyroidism to euthyroid state of the thyroid gland. Levodopa might plays role in this improvement resulting in an inhibition of TSH release because of demonstrated dopamine-receptors in the pituitary gland.]