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”]

Lege Artis Medicinae

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

Clinical Neuroscience

Acute effect of sphenopalatine ganglion block with lidocaine in a patient with SUNCT

KOCATÜRK Mehtap, KOCATÜRK Özcan

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing/short-lasting unilateral neuralgiform headache with cranial autonomic features (SUNCT/SUNA) is a rare severe headache. At the time of an attack, it can hinder a patient from eating and requires acute intervention. The sphenopalatine ganglion is an extracranial parasympathetic ganglion with both sensory and autonomic fibers. Sphenopalatine ganglion block has long been used in the treatment of headache, particularly when conventional methods have failed. Here, we present a patient who was resistant to intravenous lidocaine, but responded rapidly to sphenopalatine ganglion block during an acute episode of SUNCT/SUNA.

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.