[Mild clinical course of left ventricular pseudoaneurysm]. / Tetniak rzekomy lewej komory serca o lagodnym przebiegu klinicznym.

Left ventricular pseudoaneurysm from myocardial infarction is rare but is associated with a high risk of rapid enlargement and rupture. This report describes a 57 year old woman with left ventricular pseudoaneurysm and delayed surgical repair. Seven weeks after myocardial infarction control transthoracic echocardiography revealed a inferolateral left ventricular pseudoaneurysm. Early surgical intervention was recommended but the patient refused hospitalization and surgical repair and continued every day home activity. The symptoms and signs were so mild that she was qualified to 1st class of NYHA classification and 1st class of CCS classification. Six weeks later-ventriculography confirmed the primary diagnosis. During heart surgery the pseudoaneurysm was incised, its fibrous wall with thrombus was resected and the ruptured left ventricular myocardium was satured. She had an uneventful postoperative course. Definitive diagnosis of the pseudoaneurysm was confirmed by histopathological evaluation of the excised left ventricular wall.

Photodynamic therapy with 5-aminolevulinic acid and diamino acid derivatives of protoporphyrin IX reduces papillomas in mice without eliminating transformation into squamous cell carcinoma of the skin.

Photodynamic therapy (PDT) is used to treat malignant and nonmalignant diseases. It is also used for cosmetological skin treatment. PDT is generally considered to have a low risk of carcinogenicity. However, instances of nonmalignant human tumors turning malignant have been linked to PDT. In this study, we used 5-aminolevulinic (ALA) acid and 3 water soluble photosensitizers-PP(Arg)(2), PP(Ser)(2)Arg(2), PP(Ala)(2)Arg(2), all diamino acid derivatives of protoporphyrin IX-to treat benign papillomas in FVB/N mice induced by 7,12-dimethylbenz(a)anthracene (DMBA)-12-O-tetradecanoyl-phorbol-13-acetate (TPA). Of these drugs, ALA and PP(Arg)(2) were found the most efficient. PDT reduced the number of papillomas, but with increasing effectiveness of the drugs, the risk of malignant transformation of the papillomas into squamous cell carcinomas increased. The underlying mechanisms are not clear and further investigations are needed.

Prognostic value of the apoptotic index analysed jointly with selected cell cycle regulators and proliferation markers in non-small cell lung cancer.

In a previous small series of surgically treated non-small cell lung cancer patients (NSCLC), we found that higher apoptotic index (AI) negatively influenced survival (Dworakowska D, Jassem E, Jassem J, Karmolinski A, Dworakowski R, Wirth T, et al. Clinical significance of apoptotic index in non-small cell lung cancer: correlation with p53, mdm2, pRb and p21WAF1/CIP1 protein expression. J Cancer Res Clin Oncol 2005; 131:617-623.). In this study we attempted to verify our previous finding in larger group of 170 NSCLC cases, additionally correlating AI to selected cell cycle regulators as well as a proliferation marker. Apoptosis was assessed with the use of the TUNEL technique, whereas the expression of p53, pRb, mdm2, p21(WAF1/CIP1), cyclin D1 and PCNA were assessed immunohistochemically. The mean and the median AI was 12 and 8, respectively. The expression of p53, pRb, mdm2, p21(WAF1/CIP1) proteins and cyclin D1 was found in 47%, 71%, 37%, 65% and 40% of cases, respectively. The mean and the median PCNA labeling index (PCNA LI) was 34 and 35, respectively. AI was not correlated with any patient characteristic or other tumor markers. In uni- and multivariate analysis AI, analysed separately or jointly with cell cycle regulators and PCNA LI, did not influence disease-free or over-all survival. However, patients with "very high AI/very high PCNA LI" had a particularly poor prognosis (P=0.001). Patients with "very low AI/negative pRb" phenotype survived for a shorter time in comparison to others (P=0.04). In addition, patients with the highest PCNA LI had a worse outcome in comparison to patients with the lowest PCNA LI (P=0.04), especially those with concomitant p53 protein expression (P=0.026) or lacking pRb protein expression (P=0.04). This study demonstrates that joint analysis of several factors involved in apoptosis, proliferation and cell cycle regulation, but not AI alone, might provide additional prognostic information in NSCLC patients.

Pancreatic duct diversity.

OBJECTIVES: The formation of the pancreatic duct system is the result of the fusion of 2 embryonic buds, the ventral and dorsal primordia. Frequently, this fusion process is localized in the pancreatic head; variations, however, may account for the structural diversity of the duct system. Pancreatic duct anomalies and diversity of body and tail are thought to be casuistic. METHODS: Ninety-nine consecutive adult autopsies with reference to macroscopic anomalies in the distal part of the gland were evaluated. Pancreatograms were performed after large duodenal papilla cannulation. Ducts parallel to gland axis with a diameter of at least one third of the main pancreatic duct at the junction point and aberrant duct with different shapes and/or abnormal third-degree ductuli architecture were noted. RESULTS: Our study revealed a 9.9% frequency of main pancreatic duct diversity in the pancreatic corpus and tail. Eleven atypical ducts were visible, 9 cranially and 2 caudally from the main pancreatic duct. CONCLUSIONS: The pancreatic duct system in the body and the tail presents abnormal configuration not described in the past.

Could iron deposits in hepatocytes serve as a prognostic marker of HFE gene mutations?

BACKGROUND/AIMS: The diagnosis of hereditary hemochromatosis (HH) is based on qualitative measurement of tissue iron concentration and genetic tests. The aim of this study was to evaluate the correlation between the presence of iron deposits in the liver and the HFE gene mutations in patients with chronic liver diseases (CLD). METHODOLOGY: The 182 patients, age range 18-71 years, were hospitalized in Gdansk because of CLD. The C282Y, H63D and S65C HFE mutations were screened by PCR-RFLP analysis. Liver function tests, serological examinations for viral hepatitis, serum iron and ferritin concentration and semiquantitative assessment of liver iron were done in all subjects. Patients were divided into Group A without iron deposits in the liver, and Group B with deposits. The most frequent etiology of CLD was chronic hepatitis C. RESULTS: Biochemical parameters indicating iron storage and ALT activity were significantly higher in Group B. Either typical for diagnosis HH homozygotes C282Y/C282Y and combined heterozygotes C282Y/H63D or carriers of other HFE gene mutations were found significantly more frequently in Group B. CONCLUSIONS: The finding of iron deposits in routinely obtained liver specimen correlates with occurrence of the different HFE gene mutations.

Clinicopathological considerations on angiogenic potential in neuroblastoma Schwannian stroma - poor tumours.

The aim of the study was to determine microscopic angiogenic parameters of neuroblastoma (NB) Schwannian stroma-poor tumours. Furthermore the associations between vascular parameters and clinicopathological features of tumours and basic prognostic factors were analysed. Examined tissue samples from 62 NB came from 39 untreated and 23 chemotherapy pretreated tumours. The clinicopathological data comprised: patients' age, gender, survival, tumour site and stage, tumour histology and MYCN status.The morphological analysis of the angiogenic potential concentrated on examination of vascular patterns - classical type or pathological angiogenesis with mural microvascular proliferation (MVP). The quantitative study included semi-automatic assessment of vascular density (VD) in CD34 stained tumour sections. Pathologic angiogenesis with MVP, including simple and/or glomeruloid type, was encountered in 25 cases and was more frequent in differentiating histology subtype and extraadrenal tumours. VD value ranged from 56 to 385 vessels/mm2 (median 149). Higher VD was connected with younger patient's age. In untreated tumours VD was significantly higher in infants than in children over one year of age. Pathologic type angiogenesis and lower VD were found to be associated with shorter survival. Our study confirmed high vascularization of NB and revealed common occurrence of vascular pattern with MVP. Angiogenic potential in the analysed group showed diversity related to some clinicopathological tumour features. This points toward heterogeneity of NB tumours in vascular aspects, possibly affecting tumours' reactivity to antiangiogenic therapy.

Pancreatic cross-section: an elliptical model.

BACKGROUND: Pancreatic surgery has developed over the last 60 years. A pancreaticoduodenectomy of the pancreatic head and duodenal papilla is the most common procedure. The most popular pancreatic cross-section site of a pancreaticoduodenectomy is the isthmus of the gland. The pancreatic isthmus anatomical configuration is still being considered. STUDY DESIGN: The authors propose a new parametric descriptive system of pancreatic cross-section based on an elliptical model. RESULTS: The pancreatic cross-section was evaluated as a geometric ellipse and the location of the pancreatic duct was based on coordinates. When analyzing the cross-section plane of the pancreatic isthmus, the mean size of the pancreatic isthmus cross-section was 10.46+/-2.34 mm in width (mean+/-SD) and 25.55+/-4.56 mm in length. The mean vertical/horizontal distance ratio was 0.42+/-0.12. The mean size of the main pancreatic duct was 1.02x2.46 mm. The mean distribution coordinates of the main pancreatic duct were: X 51.23; Y 50.60. CONCLUSIONS: A proportional model of pancreatic isthmus cross-section analysis is easy and effective, and could become a valuable tool in future anatomical studies. The system described allows us to analyze data acquired from several investigators.

[Encapsulating peritoneal sclerosis--a serious complication of peritoneal dialysis with not characteristic symptoms]. / Stwardniajace zapalenie otrzewnej--ciezkie powiklanie dializy otrzewnowej o nietypowym przebiegu.

Encapsulating peritoneal sclerosis (EPS) is a serious complication in patients on peritoneal dialysis (PD). We report the case of a 45 years old woman who developed EPS five years after being placed on CAPD. We did not observe any typical abdominal symptoms. Recurrent cloudy and blood stained dialysate as well as deterioration of adequacy were noted few month before histological verification. The results of radiological findings did not allow for precise diagnosis. A patient had undergone an operation because of massive bleeding to the renal cyst during which a completely walled up peritoneal cavity was revealed (the bowel loops were cocooned in thickened peritoneum). Histological examination of peritoneal specimen confirmed the diagnosis. The patient died due to inflammatory and bleeding complications one month after an operation.

The "sugar" clear cell tumor of the lung-clinical presentation and diagnostic difficulties of an unusual lung tumor in youth.

A case of the "sugar" clear cell tumor of the lung in a 16-year-old boy is presented. The course of the disease with general symptoms, never reported before, highlights diagnostic difficulties of an extremely rare lung tumor in youth. The boy presented with daily spikes of unexplained high fever of 6 weeks' duration with features of hypochromic microcytic anemia, elevated erythrocyte sedimentation rate, C-reactive protein, alpha(2)- and beta-globulins, and elevated platelet count. The lung tumor was a yellow, circumscribed mass confined to the sixth segment of the left lung. Histological examination revealed the tumor composed of cells with clear cytoplasm with large content of glycogen, with no signs of necrosis, and immunoreactive for HMB-45, but not for cytokeratin, LCA, CD34, and CD68. The performed thoracotomy and segmentectomy were both diagnostic and curative.

Intraocular malignant teratoid medulloepithelioma in an adult: clinicopathological case report and review of the literature.

PURPOSE: To report a case of intraocular medulloepithelioma, an embryonal tumour with extremely rare presentation in adults. METHOD: The case of a 44-year-old man with intraocular malignant teratoid medulloepithelioma, primarily diagnosed as intraocular teratoma, is described and the literature on this subject is reviewed. RESULTS: The patient presented with progressive proptosis caused by a tumour in the left eyeball. He had a 28-year history of loss of vision in the left eye. Histopathological examination of the enucleated eye demonstrated an intraocular teratoma. No adjuvant treatment was given. Six months later the patient presented with massive progression in the left orbit and intracranial invasion. Cisplatin-based chemotherapy was administered, but discontinued after two cycles due to poor tolerance and lack of response. At subsequent pathology review, a final diagnosis of malignant teratoid medulloepithelioma was made. Salvage radiotherapy (60 Gy in 30 fractions) resulted in partial response of the intracranial lesion. However, the patient died 6 months later due to intracranial tumour progression. CONCLUSION: Medulloepithelioma should be considered in the differential diagnosis of intraocular tumours in adults, especially in the case of coexisting, long-standing ocular symptoms. In some cases this disease is very aggressive.

Epithelioid malignant peripheral nerve sheath tumor involving maxillary sinus.

We present a case of epithelioid malignant peripheral nerve sheath tumor (MPNST) located in the maxillary sinus region in a young man. The clinical history was short, but at admission the neoplastic infiltration was so extensive that only diagnostic biopsy was performed. The patient received palliative treatment and died 6 months later. Histologically, the neoplasm had a predominant epithelioid component. Neoplastic tissue was vimentin, S-100, Cam 5.2 and neuron-specific enolase positive. P53 protein reaction was found in 6% of the cells and the proliferation index assessed with Ki-67 was 52%. An appropriate immunohistochemical panel was essential for the final diagnosis of this epithelioid malignant tumor, with the location rather unusual for MPNST.

Prognostic value of cyclin D1 overexpression in correlation with pRb and p53 status in non-small cell lung cancer (NSCLC).

PURPOSE: The aim of this study was to assess the impact of cyclin D1 overexpression (considered separately or jointly with previously assessed p53 and pRb statuses) on survival in a group of 111 surgically treated non-small cell lung cancer patients (NSCLC). METHODS: Cyclin D1 accumulation was assessed immunohistochemically, with the use of monoclonal antibody (DCS-6, DakoCytomation) and the alkaline phosphatase-anti-alkaline phosphatase (APAAP) technique. RESULTS: Overexpression of cyclin D1 was found in 55 samples (49%), whereas the altered phenotypes cyclin D1+/p53+ or cyclin D1+/pRb- were found in 23 (22%) and 9 samples (9%), respectively. Statistical analysis was performed for different cut-off values and the only significant differences were found if samples with some expression of each protein were considered positive. There was no relationship between cyclin D1 overexpression and major clinicopathological factors, including p53 expression; however, there was a direct correlation between cyclin D1 and pRb protein expression (p=0.007). Cyclin D1 accumulation did not influence patients' survival. Of all possible cyclin D1/p53, cyclin D1/pRb and cyclin D1/p53/pRb phenotypes, patients with cyclin D1-/p53+ phenotype had shortened overall survival compared to other patients (p=0.027, HR=1.8). In the multivariate analysis, the only variable associated with shortened overall and disease-free survival was the stage of disease (p<0.001). CONCLUSIONS: These results suggest the lack of prognostic value of cyclin D1 overexpression in NSCLC patients.

Absence of prognostic significance of p21(WAF1/CIP1) protein expression in non-small cell lung cancer.

Prognostic value of p21WAF1/CIP1 expression in non-small-cell lung cancer patients (NSCLC) remains unclear. In this study the authors investigated the clinical significance of p21WAF1/CIP1 expression in a group of 117 NSCLC patients, who underwent curative pulmonary resection. Expression of p21WAF1/CIP1 protein was assessed immunohistochemically and samples showing>5% of positive tumor cells were considered positive. Seventy-six samples (65%) showed positive nuclear p21WAF1/CIP1 protein expression. There was no relationship between the expression of p21WAF1/CIP1 protein and major clinico-pathological factors, and neither there was an impact of p21WAF1/CIP1 protein expression on disease-free and overall survival. p21WAF1/CIP1 protein occurrence was not correlated with previously determined p53 protein expression and there was also no relationship between all possible p21WAF1/CIP1/p53 phenotypes and survival. In uni- and multivariate analysis only stage of disease was independent prognostic factors. These results suggest the lack of prognostic relevance of p21WAF1/CIP1 expression (analyzed separately or jointly with p53 protein) in surgically treated NSCLC patients.

Enhanced citrate synthase activity in human pancreatic cancer.

OBJECTIVES: Assuming that a high flux of carbohydrate is strictly connected with lipid synthesis in neoplastic cells, one can hypothesize that the activity of citrate synthase, which plays an important role in glucose to lipid conversion, is enhanced in pancreatic cancer. The aim of the present study was to verify this hypothesis. METHODS: The activity of citrate synthase (as well as lactate and glucose 6-phosphate dehydrogenases) was measured using tissue extract prepared from specimens (pancreatic cancer and control specimens taken from the adjacent pancreatic normal tissue) obtained from 24 patients with ductal carcinoma who underwent pancreatoduodenectomy or total pancreatomy. RESULTS: The average of citrate synthase activity in human pancreatic ductal carcinoma is significantly higher comparing with adjacent nonneoplastic tissue: 40.2 +/- 27.2 and 18.3 +/- 13.6 nmole/min/mg protein, respectively (P = 0.001). The lactate dehydrogenase and glucose 6-phosphate dehydrogenase activity in human pancreatic ductal carcinoma were also higher than in adjacent nonneoplastic tissues. CONCLUSION: It is likely that enhanced citrate synthase activity contributes to the conversion of glucose to lipids in pancreatic cancer providing substrate for membrane lipids synthesis.

Prognostic relevance of altered pRb and p53 protein expression in surgically treated non-small cell lung cancer patients.

OBJECTIVE: The prognostic value of pRb and p53 altered expression in non-small cell lung cancer (NSCLC) remains debatable. We assessed the occurrence of altered pRb and p53 protein expression, and the prognostic value of these assays considered as separate and combined variables in operable NSCLC. The study group included 195 NSCLC consecutive patients from one institution who underwent curative pulmonary resection between 1994 and 1999. METHODS: Expression of pRb and p53 was assessed immunohistochemically with the use of monoclonal antibodies (LM95.1 and Pab 1801, Oncogene Science, respectively). RESULTS: A lack of pRb and abnormal p53 protein expression were found in 57 (29%) and 92 samples (47%), respectively, whereas both abnormalities (pRb-/p53+) occurred in 24 samples (12%). There was no relationship between altered pRb/p53 expression and major clinico-pathological characteristics, neither was there a significant difference in disease-free and overall survival between particular groups of patients with tumors carrying four possible pRb/p53 phenotypes. In uni- and multivariate analysis, the only variable associated with shortened disease-free and overall survival was stage of disease (p < 0.001) and degree of tumor differentiation (p = 0.005). CONCLUSION: These results suggest that altered pRb and p53 expression does not provide prognostic information in operable NSCLC patients.

[Malignant vascular tumours in children -- report from the Polish Pediatric Rare Tumors Study]. / Charakterystyka kliniczna naczyniopochodnych nowotworów zlosliwych u dzieci -- raport Programu Guzów Rzadkich PPGGL.

UNLABELLED: Malignant vascular tumours represent a rare group of neoplasms, usually treated according to protocols for soft tissue sarcomas. THE AIM OF THE STUDY: To assess the clinical characteristics, disease course and outcome in Polish children with malignant vascular neoplasms. MATERIAL AND METHODS: The Polish Paediatric Rare Tumours Study retrospectively analysed multicentre data on 32 children with vascular tumours, registered between 1992 and 2002. On the basis of the histological type of the neoplasm these patients have been divided into three groups: group I -- 10 patients with angiosarcoma (ASA), group II -- 7 children with haemangioendothelioma (HE) and group III- 15 patients with haemangiopericytoma (HP), of both infantile (7 children) and adult-types (8 patients). RESULTS: Group I: 5 patients presented with local, 3 with systemic and 2 with regional disease. Primary complete resection (PRC) was performed in 7 patients, not resulting in local control in any of them. Radiotherapy (RTX) was administered in 5 patients, chemotherapy (CHT) in all. 7 patients relapsed, 3 never entered clinical remission. 9 of the 10 patients of group I, died of disease progression. Group II: PRC was performed in 5 patients and led to local control in 2. Adjuvant RTX was used in 2 and CHT in 4 patients. 2 children relapsed and died of the disease. Infantile HP: PRC was performed in 3 children, remaining 4 patients received adjuvant CHT All children entered complete remission and are disease-free. Adult-type HP: PRC was performed in 5 patients, resulting in local control in 4. Five children were given adjuvant RTX and six CHT Three patients relapsed and died of disease progression. CONCLUSIONS: The effectiveness of primary complete resection in all groups was doubtful. The high rate of metastatic relapses suggests that the currently given systemic therapy is not satisfactory. The only tumour with excellent prognosis was infantile type HP (all patients are alive and free of disease). Adequate treatment for children with angiosarcoma remains still unknown -- 9 of 10 patients died of disease progression. Prognosis in patients with haemangioendothelioma is intermediate, however the role of immunotherapy should be further investigated.

Microvascular density in chronic pancreatitis and pancreatic ductal adenocarcinoma.

Chronic pancreatitis and pancreatic adenocarcinoma represent two pathologic phenomena with marked production of connective tissue stroma containing numerous small blood vessels. The aim of this study was to characterise quantitatively the vascular supply of pancreatic adenocarcinoma and fragments of the periductal tissue collected from patients with chronic pancreatitis. The study material included 18 cases of pancreatitis and 22 cases of pancreatic ductal adenocarcinoma. Microvessels were marked using monoclonal anti-CD34 antibodies. The number of blood vessels in the fibrous stroma was significantly higher in the chronic pancreatitis samples compared to the pancreatic carcinoma group (mean vessel count 298 and 194 vessel/mm2; median 251 and 187 vessel/mm2 respectively; p<0.01). Distributions of the vascular diameter in both studied groups were very similar. The obtained results suggest that the development of vascular network accompanying chronic pancreatitis is more effective in some parts of pancreas compared to angiogenic intensity in pancreatic adenocarcinoma.

[Cyclosporine in the treatment of atypical celiac sprue. A case report]. / Zastosowanie cyklosporyny w leczeniu atypowej postaci choroby trzewnej. Opis przypadku.

We present a 45-year-old woman with atypical celiac sprue who was unresponsive to gluten-free diet. Cyclosporine therapy was applied (5 mg/kg body weight daily for 2 months). The patient demonstrated a remarkable clinical and histological response to this drug.