Anaplastic astroblastoma of childhood: aggressive behavior.

OBJECTIVE: Astroblastoma is an unusual brain tumor in childhood. Tumor usually arises from cerebral hemispheres. This large tumor is well-demarcated, lobulated, solid or cystic, and therefore, may resemble glioblastoma. Histopathologically, there are two types of astroblastoma: well-differentiated (low-grade) or anaplastic (high-grade). In low-grade astroblastoma, treatment of choice is complete excision. This type of tumor usually does not recur. However, anaplastic tumors can recur despite surgery, radiation, and chemotherapy and may be problematic for clinician. CASE REPORT: A 7-year-old female patient presented with an acute onset of vomiting and seizure. Magnetic resonance imaging study revealed a large mass in the left parieto-occipital region. She underwent total excision of the tumor. Histopathologically, the tumor was an anaplastic astroblastoma. Her adjuvant treatment was planned to consist of radiation therapy and cisplatin-based chemotherapy. However, the tumor recurred early in the course, and she died 18 months after diagnosis. CONCLUSION: High-grade astroblastomas behave like glioblastoma, as emphasized in this case report. Local control of this type of tumor seems difficult despite surgery, radiation therapy, and cisplatin-based chemotherapy.

Intrahepatic cholangiocarcinoma arising in chronic viral hepatitis-associated cirrhosis: two transplant cases.

Hepatitis C virus (HCV) or hepatitis B virus (HBV)-related cirrhosis is known to be a risk factor for hepatocellular carcinoma (HCC). Recently, these viruses have been reported to have an etiologic role in the development of intrahepatic cholangiocarcinoma (ICC). Herein we have reported two cases of HCV- and HBV-related cirrhosis with ICC in whom the pretransplant diagnosis was HCC. The patient with HCV cirrhosis, was a 47-year-old woman with a large nodule in the right lobe. The patient with HBV cirrhosis was a 45-year-old man with two nodules. Serum tumor marker levels, carcinoembryonic antigen (CEA), alphafetoprotein (AFP), and carbohydrate antigen 19-9 (CA 19-9) were determined before live donor liver transplantation (LDLT). The patient with HCV cirrhosis showed mildly elevated serum levels of AFP. The patient with HBV cirrhosis showed an elevated CA 19-9 level. On microscopic examination, all nodules exhibited typical morphological findings of adenocarcinoma. The patient with HCV cirrhosis developed brain metastases 4 years after LDLT. The patient with HBV cirrhosis is disease-free at 18 months after transplantation. In cirrhotic patients with active malignancy who are candidates for LDLT, ICC should be considered in the differential diagnosis. Although the literature is limited, selected patients with ICC may benefit from LDLT.

The prevalence of RET/PTC mutations in papillary thyroid cancers in Turkish population and its relation between tumor histopathology and prognostic factors.

OBJECTIVE: In recent years, thyroid cancer has been at the forefront of molecular pathology as a result of the consequences of the Chernobyl disaster and the recognition of the role of RET/PTC rearrangements in papillary thyroid carcinomas (PTCs). Correlation of RET/PTC expression with clinical outcome is controversial. This study aims to identify the prevalence of RET/PTC oncogene expression in Turkey, and to investigate the correlation between RET/PTC oncogene expression and the known prognostic factors of PTC in 101 patients. METHODS: The RET rearrangements were examined by means of reverse transcriptase-polymerase chain reaction analysis, with primers flanking the chimeric region. Statistical evaluation was performed by using Independent samples t-test, One-sample Chi-square test and Pearson Chi-square or Fisher's Exact Test. RESULTS: RET/PTC was determined positive in 67(66.3%) of totally 101 patients (p<0.001). RET/PTC1 in 32(31.7%), RET/PTC3 in 21(20.8%), RET/PTC1+RET/PTC3 both in 10(9.9%) patients were found to be positive. There was RET/PTC2 positiveness in two patients, RET/PTC2,3 positiveness in one patient, and RET/PTC1,2,3 positiveness in one patient. No statistical difference was found between RET/PTC1 and RET/PTC3. None of genetico-clinical analyses showed any significant association between RET/PTC expression and the clinical and pathological features of the cancers. CONCLUSION: While this prevalence of the RET/PTC is less than RET/PTC frequency seen after Chernobyl in Belarus, its prevalence in our region is also high (66.3%). As a result, no significant correlation was found in between prognosis and RET/PTC frequency.

Fas/Fas ligand gene polymorphism in patients with papillary thyroid cancer in the Turkish population.

OBJECTIVE: Fas ligand (FasL) is an apoptotic agent and a member of tumor necrosis factor (TNF) family. FasL exists in cytotoxic T lymphocyte (CTL) and natural killer (NK) cells, and it is increased in tumor cell membrane. On the contrary, CTL and NK are bound to Fas on the surfaces of cell membrane; this triggers apoptosis in cytotoxic cells and leads to their death. This system plays an important role in eliminating viral infections and cancer cells. Malfunction of this system results in the development and spread of the malignancy. This study aims at evaluating the influence of Fas and FasL gene polymorphism in papillary thyroid cancer (PTC) in the Turkish population. RESEARCH DESIGN AND METHODS: Forty-five patients with PTC and 100 healthy controls were included in this study. The diagnosis of PTC was confirmed by histopathologic examination after surgery. The evaluation of genotype for Fas 670 A/G and FasL 843 C/T gene polymorphism was performed using the PCR-restriction fragment length polymorphism (RFLP) method. RESULTS: The evaluation of Fas/FasL genotype and gene allele frequency did not show statistically significant differences between the patient and control group (p>0.05). In addition, the univariate analysis did not reveal a statistically significant relationship between the size of the nodule and the Fas/FasL gene polymorphism in patients with PTC. CONCLUSIONS: As in other types of malignancy, genetic factors in the pathogenesis of PTC may also show changes in different populations. Fas/FasL gene polymorphysms are possible that different mechanisms function in apoptosis balance in PTC development.

Primary hepatic marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type in a liver transplant patient with hepatitis B cirrhosis.

We describe the clinical, histological, and immunohistochemical features of primary hepatic low grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) in a liver transplant recipient with hepatitis B cirrhosis. MALT lymphomas arise in organs normally devoid of lymphoid tissue, which accumulates as a consequence of chronic antigenic stimulation associated with chronic infection or autoimmune disease. Primary hepatic MALT lymphoma is extremely rare; 13 cases have been reported worldwide to date. Our patient is the first case of primary hepatic MALT lymphoma associated with hepatitis B cirrhosis who was treated with orthotopic liver transplantation.

The Differential Diagnosis of Lymphocyte-rich Classical Hodgkin's Lymphoma and Lymphocyte Predominant Hodgkin's Lymphoma Using the R.E.A.L. Criteria. An Immunohistochemical Study on 45 Cases.

In the present study, the two types, lymphocyte-rich classical HL (LRCHL) and nodular lymphocytepredominant type (NLPHL), which were grouped together before the R.E.A.L. classification, were questioned on the basis of differential criteria and 45 cases were retrieved from last ten years' archival material. On histopathological examination, nodular pattern, the cytological features and intensity of Reed- Sternberg (RS) cells, the pattern and intensity of histiocytes, the presence of germinal centers with progressive transformation were analysed. An immunohistochemical study was performed using antibodies against CD20, CD45RO, CD3, CD30, CD15 antigens and streptavidin-biotin procedure. The cases were classified into three groups according to the histologic pattern and immunophenotypical features ofthe RS cells: I) diffuse, LRCHL (CD20-, CD30+/-, CD15+/-): n= 28; II) NLPHL (CD20+, CD30-, CD15-): n= 11; III) cases which could not be evaluated in former groups: n= 6. Four cases in the latter group showed a nodular pattern with RS cells negative for all markers, except for one case, which expressed both CD20 and CD15. The remaining two cases exhibited a diffuse pattern and the RS cells were CD20+, coexpressing CD30 in one. These findings suggest that, differential diagnosis according to the R.E.A.L. criteria is not distinctive between the two categories of HL in about 13% of cases, and further criteria need to be established to define the grey zone between the two entities which might lead to further therapeutic trials.