Secondary myeloid neoplasms: bone marrow cytogenetic and histological features may be relevant to prognosis.

Background: Secondary myeloid neoplasms comprise a group of diseases arising after chemotherapy, radiation, immunosuppressive therapy or from aplastic anemia. Few studies have addressed prognostic factors in these neoplasms.

Method: Forty-two patients diagnosed from 1987 to 2008 with secondary myeloid neoplasms were retrospectively evaluated concerning clinical, biochemical, peripheral blood, bone marrow aspirate, biopsy, and immunohistochemistry and cytogenetic features at diagnosis as prognostic factors.

Predictive features for histology of gastric subepithelial lesions.

Background: - Gastric subepithelial lesion is a relatively common diagnosis after routine upper endoscopy. The diagnostic workup of an undetermined gastric subepithelial lesion should take into consideration clinical and endoscopic features.

Objective: - We aimed to investigate the association between patients' characteristics, endoscopic and echographic features with the histologic diagnosis of the gastric subepithelial lesions.

Splenic diffuse red-pulp small B-cell lymphoma associated with hepatitis B virus: a report of two cases.

Context: Splenic diffuse red-pulp small B-cell lymphoma is a rare disease, representing less than 1% of all non-Hodgkin lymphomas (NHL). This entity is characterized by involvement of bone marrow sinusoids and peripheral blood. The majority of cases are at an advanced stage when diagnosed.

Quantitative analysis of lymph nodes in neck dissection specimens. Morphologic study.

Purpose: To quantify the amount of lymph nodes harvested in modified radical neck dissection.

Methods: Cross-sectional anatomical study conducted in 28 non-preserved cadavers.

Results: The mean number of lymph nodes found in each nodal level of the 56 modified radical neck dissections performed were: level IA - 1.

Advanced chronic lymphoid leukemia with severe bronchopneumonia: an Autopsy Case Report.

Chronic lymphocytic leukemia (CLL) is a lymphoid neoplasia with the B immunophenotype, which corresponds to the leukemic form of lymphocytic lymphoma. This entity is characterized, in most cases, by immunosuppression due to impaired function of immune cells, hypogammaglobulinemia, bone marrow infiltration, and immune dysfunction due to the neoplasia and the chemotherapy, when prescribed. We describe the case of a 63-year-old woman with a previous diagnosis of advanced CLL, refractory to treatment, who presented respiratory failure at the emergency department and died soon after hospital admission.

Gastric metastasis as the first manifestation of an invasive lobular carcinoma of the breast.

Gastrointestinal metastases from breast cancer are rare and generally occur several years after the diagnosis of the primary lesion. The diagnosis of gastric metastasis as the initial presentation of breast cancer is even rarer and can potentially mimic gastric carcinoma. We report the case of a 66-year-old female patient submitted to a total gastrectomy because of the histological diagnosis of undifferentiated gastric carcinoma.

Differential expression of genes encoding proteins of the HGF/MET system in insulinomas.

Background: Insulinomas are the most common functional pancreatic neuroendocrine tumors, whereas histopathological features do not predict their biological behaviour. In an attempt to better understand the molecular processes involved in the tumorigenesis of islet beta cells, the present study evaluated the expression of genes belonging to the hepatocyte growth factor and its receptor (HGF/MET) system, namely, MET, HGF; HGFAC and ST14 (encode HGF activator and matriptase, respectively, two serine proteases that catalyze conversion of pro-HGF to active HGF); and SPINT1 and SPINT2 (encode serine peptidase inhibitors Kunitz type 1 and type 2, respectively, two inhibitors of HGF activator and of matriptase).

Methods: Quantitative real-time reverse transcriptase polymerase chain reaction was employed to assess RNA expression of the target genes in 24 sporadic insulinomas: 15 grade 1 (G1), six grade 2 (G2) and three hepatic metastases.

Hepatocellular carcinoma may display elevated nestin expression in endothelial cells: experimental study.

Context And Objective: Nestin, a class VI intermediate filament protein, is highly expressed in the portal mesenchyme and sinusoidal endothelium of the human fetal liver, but scarcely expressed in adult portal vessel endothelium. During experimental liver regeneration, an increased number of nestin-positive parenchymal cells have been observed in the zone adjacent to the Hering canals. These parenchymal cells are regarded as hepatic stem cells or hepatoblasts, which may be involved in hepatocellular carcinogenesis.

Dealing with bone marrow biopsies in the staging of classical Hodgkin lymphoma: an old issue revisited in the (18)F-fluorodeoxyglucose-positron emission tomography era.

Bone marrow biopsy is recommended for staging of classical Hodgkin lymphoma. The aim of this study was to compare bone marrow evaluation by histology with that obtained by (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET). One hundred and three cases of Classical Hodgkin Lymphoma were reviewed.

Penetrance and clinical features of pheochromocytoma in a six-generation family carrying a germline TMEM127 mutation.

Context: The phenotype of familial pheochromocytoma (PHEO) associated with germline TMEM127 mutations (TMEM127-related PHEO) has not been clearly defined.

Objective: This study aimed to investigate the penetrance, full phenotypic spectrum and effectiveness of clinical/genetic screening in TMEM127-related PHEO.

Design, Setting, And Participants: Clinical and genetic screening, and genetic counseling were offered to 151 individuals from a six-generation family carrying a TMEM127 germline mutation in a referral center.

Comparison of two methods of RNA extraction from formalin-fixed paraffin-embedded tissue specimens.

The present study aimed to compare two different methods of extracting RNA from formalin-fixed paraffin-embedded (FFPE) specimens of diffuse large B-cell lymphoma (DLBCL). We further aimed to identify possible influences of variables--such as tissue size, duration of paraffin block storage, fixative type, primers used for cDNA synthesis, and endogenous genes tested--on the success of amplification from the samples. Both tested protocols used the same commercial kit for RNA extraction (the RecoverAll Total Nucleic Acid Isolation Optimized for FFPE Samples from Ambion).

Association between the p27 rs2066827 variant and tumor multiplicity in patients harboring MEN1 germline mutations.

Objective: To date, no evidence of robust genotype-phenotype correlation or disease modifiers for multiple endocrine neoplasia type 1 (MEN1) syndrome has been described, leaving the highly variable clinical presentation of patients unaccounted for.

Design: As the CDKN1B (p27) gene causes MEN4 syndrome and it is transcriptionally regulated by the product of the MEN1 gene (menin), we sought to analyze whether p27 influences the phenotype of MEN1-mutated patients. The cohort consisted of 100 patients carrying germline MEN1 gene mutations and 855 population-matched control individuals.

Existence of a potential neurogenic system in the adult human brain.

Background: Prevailingly, adult mammalian neurogenesis is thought to occur in discrete, separate locations known as neurogenic niches that are best characterized in the subgranular zone (SGZ) of the dentate gyrus and in the subventricular zone (SVZ). The existence of adult human neurogenic niches is controversial.

Methods: The existence of neurogenic niches was investigated with neurogenesis marker immunostaining in histologically normal human brains obtained from autopsies.

ERCC1 in advanced biliary tract cancer patients treated with chemotherapy: prognostic and predictive roles.

Background: In oncology, we tend to look for factors that reflect better prognosis or predict response to treatments in order to make a selection from which patients will derive the benefit, avoiding futile therapies and/or toxicities. Definitive prognostic and predictive factors in advanced biliary cancer remain unknown.

Methods: We retrospectively analyzed all consecutive patients in our institution with advanced biliary tract cancer treated with palliative cisplatin plus gemcitabine.

Penetrance of functioning and nonfunctioning pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1 in the second decade of life.

Context: Data are scarce on the penetrance of multiple endocrine neoplasia type 1 (MEN1)-related nonfunctioning pancreatic neuroendocrine tumors (NF-PETs) and insulinomas in young MEN1 patients. A potential positive correlation between tumor size and malignancy (2-3 cm, 18%; >3 cm, 43%) has greatly influenced the management of MEN1 adults with NF-PETs.

Objective: The aim of the study was to estimate the penetrance of NF-PETs, insulinomas, and gastrinomas in young MEN1 carriers.

Sudden coronary death due to IgG4-related disease.

A 54-year-old male entered the emergency room in cardiorespiratory arrest after syncope at home. Resuscitation was attempted, but the patient died a few hours later. At necropsy, aneurysms were found at the right and left anterior descending coronary arteries.

Development and internal validation of an adrenal cortical carcinoma prognostic score for predicting the risk of metastasis and local recurrence.

Objective: To develop and internally validate a prognostic score to predict the risk of metastases or recurrence in patients with adrenal cortical carcinomas (ACC).

Design: Clinical, laboratory and pathological data from 129 ACC patients, treated in a tertiary centre, were retrospectively reviewed.

Results: Using a multivariate binary logistic regression analysis, we developed a prognostic score with five covariates: a functional pattern other than isolated hyperandrogenism, a tumour size >7·5 cm, a primary tumour classified as T3/T4, the presence of microscopic venous invasion and a mitotic index >5/50 high-power fields.

Pathophysiology and molecular aspects of diffuse large B-cell lymphoma.

Diffuse large B-Cell lymphoma is the most common subtype of non-Hodgkin lymphoma in the West. In Brazil, it is the fifth cause of cancer, with more than 55,000 cases and 26,000 deaths per year. At Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo - HCFMUSP, diffuse large B-Cell lymphoma represents 49.

Optic nerve sheath meningioma in the first decade of life: case report and review of the literature.

Background/aim: Tumors of the optic nerve are mostly either optic nerve gliomas or optic nerve sheath meningiomas (ONSMs). While gliomas occur in children, most meningiomas are slow-growing tumors affecting middle-aged individuals with progressive visual loss, proptosis, disc edema and optociliary veins on fundus examination. ONSMs are extremely rare in children, with only 14 cases reported in children under the age of 10 years.

The interference of cold ischemia time in the quality of total RNA from frozen tumor samples.

Tumor Banks were created to organize the collection, storage and distribution of biological samples from oncological patients, facilitating its use in cancer research. To ensure the quality of the samples from our bank, we implemented standard operating procedures international. In order to evaluate the influence of cold ischemia time (time between surgical removal of the specimen and the snap freezing of the sample) on the quality of the samples (evaluated by measurement integrity of their RNA), collected during 10 months two tumor samples from each donor, one with up to 30 min of cold ischemia and other with exact 45 min, totaling 100 different donors and 200 samples, 40 from each of the following organs: breast, thyroid, stomach, lung and colorectum.

From conventional fluid cytology to unusual histological diagnosis: report of four cases.

Malignant small round cell tumors represent a diagnostic challenge for cytologists and pathologists. This case series describes four cases of unusual metastasis of small round cell tumors subtypes into body cavities generating effusions in which fluid cytological examination suggested the neuroendocrine origin of the tumors. Tumor diagnosis (Ewing sarcoma/primitive neuroectodermal tumor and desmoplastic small round cell tumors) were unknown at the cytological evaluation.

Disease progression after R-CHOP treatment associated with the loss of CD20 antigen expression.

A case of a follicular lymphoma transformed into a CD20(+) is described which progressed with the loss of CD20 expression after 8 cycles of R-CHOP. This phenomenon is not a rare event and has shown poor prognosis. Our purposes are to describe this event and suggest biopsy in relapsed or progressive disease.