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Warsaw, Poland

Lipka B.,Klinika Niemowleca | Milewska-Bobula B.,Klinika Niemowleca | Cielecka-Kuszyk J.,Zaklad Patologii | Pakula-Kosciesza I.,Pracownia Rezonansu Magnetycznego
Pediatria Polska

The main cause of salivary glands swelling in children is acute inflammation (usually viral), sialolithiasis and tumors. The most common viral infection is parotitis epidemica caused by paramyxoviruses, then cytomegalovirus infection. Cytomegalovirus is the major cause of congenital viral infections, but isolated inflammatory salivary gland disease in the course of intrauterine cytomegalovirus infection is very rare. The article discusses the clinical course of cytomegalovirus infection in two patients with congenital enlargement of parotid gland, in which despite the use of antiviral treatment the local improvement was not achieved. © 2011 Polish Pediatric Society. Source

Taybert J.,Klinika Chorob Metabolicznych | Pronicki M.,Zaklad Patologii | Bednarska-Makaruk M.,Zaklad Genetyki | Sykut-Cegielska J.,Klinika Chorob Metabolicznych
Przeglad Pediatryczny

Cholesterol ester storage disease (CESD) is a recessive disorder due to deficient activity of lysosomal acid lipase leading to lysosomal accumulation of cholesterol esters and triglycerides. Its prevalence in the Polish population is unknown. Clinical manifestation includes hepatomegaly (usually mild at onset) with hypercholesterolemia (hyperlipidemia) and hypertransminasemia. Liver insufficiency or cirrhosis may not appear until adulthood. Microscopic examination of liver biopsy shows relatively typical changes. Liver lipids chromatography reveals storage of cholesterol esters, which suggests diagnosis. Demonstration of acid lipase deficiency in leukocytes confirms the diagnosis. Hypercholesterolemia is often the predominant sign in cases with slight hepatic manifestation. Contrary to familial hypercholesterolemia (FH) of autosomal predominant inheritance (heterozygosity 1:500), in patients with CESD family history is negative (except when FH coexists). Differential diagnosis does not reveal hypoglycaemia typical for glycogen storage diseases or severe cholestasis. Up to now there is no specific treatment. Management includes dietary treatment of hypercholesterolemia, monitoring of liver function, and in individual cases statins are ordered. Enzymatic replacement therapy is in the course of trials. Genetic counseling should be provided. Risk of occurrence of CESD is different than in FH. To sum up, precise family history taking, palpable examination of abdomen and basic laboratory liver function tests are helpful for preliminary differential diagnosis in children with hypercholesterolemia. Source

Klimczak A.,Klinika Nowotworow Tkanek Miekkich | Pekul M.,Zaklad Patologii | Wiater K.,Klinika Nowotworow Tkanek Miekkich | Rutkowski P.,Klinika Nowotworow Tkanek Miekkich

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor composed mostly of perivascular epithelioid cells (PEC). The PEComa related tumors encompass angiomyolipoma, clear cell "sugar" tumor of the lung, lymphangioleiomyomatosis (AML) and clear cell myomelanocytic tumor (CCMT). Tumors of the PEComa family are rare and usually occur sporadically. LAM and AML are also frequently observed in patients with tuberous sclerosis simplex complex (TSC). Most PEComas are benign and do not recur after complete surgical resection. However, a subset of PEComas exhibit malignant behaviour. There is no known effective systemic therapy for PEComa. Recently published studies have shown that inhibition of the mTOR signaling pathway might constitute a promising treatment modality in patients with PEComa. Source

Koc Z.,Poradnia Specjalistyczna Chirurgii Onkologicznej | Latka D.,Oddzial Neurochirurgii | Sachanbinski T.,Oddzial Chirurgii Onkologicznej | Nasierowska-Guttmejer A.,Zaklad Patologii | Szawlowski A.W.,Centrum Onkologii Instytut Im. Marii Sklodowskiej Curie

Primary lepto-meningeal melanoma represents less than 0.1% of all tumours of the central nervous system. It occurs in two different forms: as a primary malignant lepto-meningeal tumour or as a primary lepto-meningeal melanomatosis. Current review of medical literature showed only 45 such case reports. Due to the lack of characteristic symptoms, each case was discovered by chance during surgery or at the time of autopsy. The histogenesis of primary melanoma of the CNS is still unknown issue. For the time being, many different theories have been proposed but have not become fully proven. In this paper the case of a 66-year old male patient with primary melanoma of lepto-meningeal space cone vertebral core was presented. Source

Background. Lobular neoplasia is a hyperplastic breast lesion - a borderline type of lesion with a high risk of subsequent development of invasive carcinoma. In case of radial scar diagnosis the risk of invasive carcinoma increases twice in comparison with healthy patients population while in the case of lobular neoplasia diagnosis within radial scar this risk increases 8 to 10 times. Basing on the presented case we analyse and review the literature regarding the clinical, radiological and pathological aspects of lobular neoplasia of the breast. Case report. A 67-yeas old patient was hospitalised in 1995 in order to consult the results of mammography, which revealed a "radial scar" lesion of the right breast. The patient was then referred to undergo wide local excision. Pathological examination showed dysplastic changes of the "radial scar"type with 1 cm multifocal lobular neoplasia. The patient was systematically followed clinically and radiologically during the decade 1996-2006 and all examination results were normal. In 2008, an ill-defined nodule appeared within the scar on mammography examination. This was categorized as BI-RADS 4C. The mammotomic biopsy performed under ultrasonography control revealed invasive lobular carcinoma. The tumor was totally locally excised and the sentinel node was histologically verified. The histopathological examination revealed a 1.6 cm focus of invasive lobular carcinoma and the sentinel node was negative. Conclusion. Patients with detected lobular neoplasia should be treated as a risk group of invasive breast cancer development (30-40% vs 10% in a healthy population during the entire life period). Systematic clinical and radiological follow-up should be mandatory. Source

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