Hungarian Radiology

[A rare pancreatic mass in childhood]

TORDAS Adél1, PALOTAI Andrea2, KISS Imre3, KIS Éva3

APRIL 07, 2009

Hungarian Radiology - 2009;83(01)

[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.]

AFFILIATIONS

  1. Budai Irgalmasrendi Kórház
  2. Huniko Kft., Flór Ferenc Kórház
  3. Semmelweis Egyetem, I. Sz. Gyermekklinika, Budapest

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Hungarian Radiology

[With renewed outlook and new fortitude]

LOMBAY Béla

Hungarian Radiology

[Self-expanding metallic stents in intrahepatic biliary strictures after liver transplantation]

DOROS Attila, NÉMETH Andrea, HARTMANN Erika, DEÁK Pál Ákos, JUHAROSI Gyöngyi, LÉNÁRD Zsuzsa, KOZMA Veronika, GÖRÖG Dénes, GERLEI Zsuzsa, FEHÉRVÁRI Imre, NEMES Balázs, KÓBORI László

[INTRODUCTION - Bile duct complications remain a key problem of liver transplantation. Two main types are recognized: anastomotic and intrahepatic. In cases of anastomotic strictures good results can be achieved with surgery or minimally invasive therapy. Intrahepatic stenosis usually requires retransplantation. In this report the results of intrahepatic metallic stent placements are analyzed. PATIENTS AND METHODS - Since 1995, 20 patients with intrahepatic bile strictures were referred for percutaneous treatment. Of 34 percutaneous transhepatic cholangiography, 33 successful drainages were performed and 58 balloon dilatations were employed to overcome. In 13 patients, 20 metallic stents were implanted. One bleeding complication was successfully treated with selective embolization. RESULTS - The average follow up time was 35 months. 14 patients have no symptoms, 12 of them after metallic stent placements and 4 of them after retransplantation (2 patients had metallic stents at retransplantation). One patient has metallic stent and an external drain waiting for retransplantation. Three patients died after 7 retransplantations. Two patients died on the waiting list, one with and one without external drain. There were no deaths after successful metallic stent placement. CONCLUSION - After meticulous preparations metallic stent placement is safe and effective in intrahepatic biliary stenosis after liver transplantation. The patients can be stabilized till the retransplantation, or it can even be avoided.]

Hungarian Radiology

[Xanthogranulomatous cholecystitis]

BERÉNYI Zsolt, MORVAY Zita, PALKÓ András

[INTRODUCTION - The xanthogranulomatous cholecystitis is a rare and benign form of lesions associated with diffuse thickening of the gall bladder wall. It is important to recognize it radiologically because it can be mistaken easily for gall bladder carcinoma. The characteristic US, CT and MR findings, however, may be helpful in the differential diagnosis. CASE REPORT - We present the cases of two middleaged female patients suffering from right upper quadrant, radiating abdominal pain for several weeks without occurrence of fever. In both patients, the ultrasound examination revealed marked thickening of the gall bladder wall containing hypoechoic nodules. Further, non-specific sign such as cholecystolithiasis and fine infiltration of the adipose tissue surrounding the gall bladder and dilatation of extrahepatic or intrahepatic bile ducts were visible. On the post contrast CT images, rim enhancement was detectable. MR/MRCP examination showed a sharp delineation of the gall bladder from the liver parenchyma. Both patients underwent cholecystectomy. The pathological examination excluded malignancy and confirmed the diagnosis of xanthogranulomatous cholecystitis. CONCLUSION - The characteristic features of xanthogranulomatous cholecystitis (hypoechoic xanthogranulomas in the markedly thickened gall bladder wall and the presence of calculi) can be detected by ultrasound examination. CT or MRI may play an important role in confirmation of the diagnosis of an inflammatory process and provide useful information in exclusion of gall bladder carcinoma.]

Hungarian Radiology

[Transjugular intrahepatic portosystemic shunt implantation in a patient with severe dilatative cardiomyopathy]

SZALÁNCZY Katalin, LÁZÁR István, STEFÁN János, KALÓ Emil

[INTRODUCTION - Indications for TIPS (transjugular intrahepatic portosystemic shunt) are usually portal hypertension induced by alcoholic or viral cirrhosis. Reported patient underwent TIPS because of a rare indication where his rapidly progressing heart failure lead to rapid deterioration of the splanchnic hypertension. CASE REPORT - A 51 years old male was admitted with severe dilatative cardiomyopathy and atrial fibrillation, generalized edema refractory to conservative treatment, and rapidly worsening hepatic laboratory test results. TIPS implantation achieved improvement of all clinical signs, decreased edema and the patient could finally be discharged. CONCLUSION - TIPS can result in improved quality of life not only in primary hepatic cirrhosis but in other clinical circumstances with portal hypertension.]

Hungarian Radiology

[Difficulties in the diagnosis of ectopic ureter]

KONCZ Júlia, RÉTI Gyula, NYÁRI Edit, SHAIKH M. Shoaib

[INTRODUCTION - Ureter ectopy refers to the distal opening of the ureter at the site of the bladder neck or lower. 70-80% of the ectopic ureters are associated with pyelectasia and duplicated ureters. The incidence of this is 2-3 times higher in females. CASE REPORT - Following is a case report of a boy who was diagnosed with left-sided pyelectasia during a prenatal ultrasound scan. The postnatal ultrasound revealed a duplicated pelviceal cavity and ureter. The upper pole ureter and the pelvis demonstrated dilatation. At 8 months of age a left side heminehprectomy was performed. Two years postoperatively a follow-up ultrasound revealed a dilatation of the ureteral stump on the left side, which progressed. On MCUG the ureteral stump was identified inserting on to the proximal urethra. Cystography and MR urography demonstrated a ureteral stump which inserted on to the urethra. A repeat surgery was performed to remove the stump. Patient is symptom-free ever since. CONCLUSION - In ectopic, non-refluxing ureters long-term follow-up is necessary following heminephrectomy. A ureter stump besides the bladder can cause serious diagnostical difficulties. Also, it is possible that a dilating stump may lead to a reflux not identified earlier. MCUG and MR urography can help to clear delineate the pathology.]

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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.

Lege Artis Medicinae

[The author’s response to the comment on “Exploratory study of outcomes of blood sample mass examinations by rank correlations”]

Clinical Neuroscience

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

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.

Lege Artis Medicinae

[Comment to the article titled “Exploratory study of outcomes of blood sample mass examinations by rank correlations”]

Clinical Neuroscience

[Consensus statement of the Hungarian Clinical Neurogenic Society about the therapy of adult SMA patients]

BOCZÁN Judit, KLIVÉNYI Péter, KÁLMÁN Bernadette, SZÉLL Márta, KARCAGI Veronika, ZÁDORI Dénes, MOLNÁR Mária Judit

[Background – Spinal muscular atrophy (SMA) is an autosomal recessive, progressive neuromuscular disorder resulting in a loss of lower motoneurons. Recently, new disease-modifying treatments (two drugs for splicing modification of SMN2 and one for SMN1 gene replacement) have become available. Purpose – The new drugs change the progression of SMA with neonatal and childhood onset. Increasing amount of data are available about the effects of these drugs in adult patients with SMA. In this article, we summarize the available data of new SMA therapies in adult patients. Methods – Members of the Executive Committee of the Hungarian Clinical Neurogenetic Society surveyed the literature for palliative treatments, randomized controlled trials, and retrospective and prospective studies using disease modifying therapies in adult patients with SMA. Patients – We evaluated the outcomes of studies focused on treatments of adult patients mainly with SMA II and III. In this paper, we present our consensus statement in nine points covering palliative care, technical, medical and safety considerations, patient selection, and long-term monitoring of adult patients with SMA. This consensus statement aims to support the most efficient management of adult patients with SMA, and provides information about treatment efficacy and safety to be considered during personalized therapy. It also highlights open questions needed to be answered in future. Using this recommendation in clinical practice can result in optimization of therapy.]