Apoptosis in Endomyocardial Biopsies from Patients with Dilated Cardiomyopathy.

Apoptosis is an active energy-consuming mechanism of cell death, which may contribute to heart failure in patients with dilated cardiomyopathy. Dilated cardiomyopathy is a common clinical outcome of many prolonged cardiac insults, and therefore is considered as the most prevalent form of cardiomyopathy. Loss of heart mass is highly correlated with the heart failure and mortality, thus the purpose of this study was to define the apoptotic index in patients with dilated cardiomyopathy. Apoptosis was detected by the TUNEL method in 30 patients. Biopsies were obtained from the left ventricle, and at least three specimens were taken. TUNEL-positive cardiomyocytes were found in 26 of 30 cases (86.7 %) and the mean apoptotic index for the entire specimen series was 5.41 ± 1.70 %. The analysis showed that patients with dilated cardiomyopathy had significantly higher apoptotic index (P < 0.001) than healthy subjects. One subject (man, 41 years old) had a markedly elevated apoptotic index of 52.2 %. In the remaining subjects, the percentage of cardiomyocyte death ranged from 0 % to 15.5 %. The high percentage of apoptosis found in our study may be in accordance with the clinically manifested cardiac failure in patients with dilated cardiomyopathy since in most patients we recorded the left ventricular ejection fraction values below 30 %.

Large-cell neuroendocrine carcinoma of the ampulla of Vater.

Large-cell neuroendocrine carcinoma is a high-grade neuroendocrine carcinoma, originally described in the lung. The tumor rarely occurs in extrapulmonary sites like the gastrointestinal tract, and only few examples have been described in the ampulla of Vater. A new case of large-cell neuroendocrine carcinoma of the ampulla of Vater in a 60-year-old man is reported. After pancreatoduodenectomy, macroscopic examination revealed ulcerated tumor in the region of the ampulla of Vater. Microscopically, the tumor exhibited organoid, predominantly nested growth pattern, consisting of large, polygonal cells with pleomorphic nuclei. Average number of mitoses was 36 per 10 high-power fields. Small and large areas of necrosis were identified. Immunohistochemically, the tumor cells were positive for synaptophysin, chromogranin A, PGP 9.5, neuron-specific enolase, pancytokeratin, CK8 and somatostatin and negative for CK7, CK20, S-100, TTF-1, HMB-45, CD117, E-cadherin and regulatory peptides. Ki-67 proliferative index was 41%. Histone deacetylase (HDAC) analysis showed almost identical results for HDAC1, HDAC2 and HDAC3--60, 60.3 and 61%, respectively. Two months after surgery, liver metastases occurred, confirming highly aggressive behavior of large-cell neuroendocrine carcinoma.

Retroperitoneal "Triton" tumor.

Malignant Triton tumor is a malignant peripheral nerve sheat tumor with rhabdomioblastic differentiation. These tumors are frequently associated with Neurofibromatosis type 1, sporadic cases being exteremly rare. Retroperitoneal localization have the most unfavorable prognois due to delayed diagnosis but also due to relation to adjacant organs. Preoperative diagnosis is inaccurate, but core needle biopsy gives more promising results. Aggressive surgical management remains the most effective modality since adjuvant forms of treatment like irradiation or chemotherapy do not have reproducible results. We present a 60-year-old female patient in whom a retroperitoneal presacral mass was postoperatively diagnosed as Triton tumor. At time of diagnosis, no visible metastases were present. The posterior pelvic exenteresis was performed. Intended chemotherapy was never started since multiple pulmonary, hepatic and splenic metastases were diagnosed only a month after surgery, with rapid lethal outcome. This case demonstrates the bad prognosis of malignant retroperitoneal tumors. Diagnostic tools such as refined biopsy techniques or cytogenetic analysis might help in differentiating patients who will benefit from radical surgery.