Primary pigmented nodular adrenocortical disease with synaptophysin immunoreactivity in two thai children.

This paper reports the cases of two Thai children diagnosed with primary pigmented nodular adrenocortical disease (PPNAD). The first was a thirteen and a half year old male who presented with Cushing syndrome for three years and severe osteoporosis. He had hypercortisolemia, a non-suppressible dexamethasone suppression test, and low serum ACTH. A CT scan showed slight enlargement of both adrenal glands. A bilateral adrenalectomy was performed. The second case was a thirteen-year old female who presented with mild Cushing syndrome for one year with paradoxical response to high-dose dexamethasone suppression test. An MRI revealed suspected microadenoma of the left adrenal gland. Blood sampling showed a higher cortisol level from the left adrenal vein than from the right. A left adrenalectomy was performed, followed by a right adrenalectomy four months later. The pathologies were compatible with PPNAD. Immunostaining for synaptophysin was done in both patients. The cells in the adrenocortical micronodules of both cases were stained intensely with antiserum for synaptophysin, whereas the surrounding adrenal cortex did not.

Epstein-Barr virus-associated extranodal non-Hodgkin's lymphoma of the sinonasal tract and nasopharynx in Thailand.

Epstein-Barr virus (EBV) infection is highly associated with specific subtypes of malignant lymphoma. In our previous report on nodal malignant lymphoma in Thailand, we found that 64% of classical Hodgkin's lymphoma (cHL), 51% of non-Hodgkin's lymphoma, T-cell (NHL-T), and 13% of non-Hodgkin's lymphoma, B-cell (NHL-B) were EBV-related. In the present research, we conducted a retrospective study of primary extranodal non-Hodgkin's lymphoma of the sinonasal tract (e-NHL-ST) and primary extranodal non-Hodgkin's lymphoma of the nasopharynx (e-NHL-NP) in Southern Thailand, between 1997 and 2004. EBV-encoded RNA (EBER) expression by in situ hybridization was performed in all cases and a T-cell receptor (TCR)-g gene rearrangement study was performed in NHL-T cases. There were 18 cases of e-NHL-ST and 42 cases of e-NHL-NP detected by histologic and immunohistochemistry examinations. The percentages of e-NHL-ST and e-NHL-NP as compared to nodal malignant lymphoma were 3.7% and 6.8%, respectively. Sixteen cases (88.9%) of e-NHL-ST and 7 cases (16.7%) of e-NHL-NP were NHL-T, and the remainder were NHL-B. All of the NHL-T cases in both sites were EBER-positive. Two (5.4%) of the NHL-B cases in the nasopharynx showed EBER positive. Monoclonal bands of the TCR-gamma gene were detected in 71.4% of the extranodal NK/T-cell lymphomas, nasal type, patients; 50.0% of peripheral T-cell lymphoma, unspecified, patients; and one case of angioimmunoblastic T-cell lymphoma. This study indicates a very strong association of NHL-T in the sinonasal tract or nasopharynx with EBV infection, the link apparently being weaker in NHL-B patients. The study also indicates that most cases of extranodal NK/T-cell lymphoma, nasal type, are not the germline configuration of the TCR genes.

Isolated corneal intraepithelial neoplasia.

OBJECTIVES: To report two cases of isolated corneal intraepithelial neoplasia. MATERIAL AND METHOD: The present study included 2 cases presenting with isolated corneal intraepithelial neoplasia. Both patients were treated by corneal epithelial scraping. RESULTS: The authors present the clinical picture and histopathological findings of 2 patients suffering from corneal intraepithelial neoplasia that did not arise from the corneoscleral limbus. Both patients showed no recurrence after treatment for a period of time. CONCLUSION: Isolated corneal intraepithelial neoplasia is rare. Corneal scraping may be an effective treatment.

Epstein-Barr virus-associated nodal malignant lymphoma in Thailand.

Specific subtypes of malignant lymphoma are highly associated with Epstein-Barr virus (EBV) infection. In the present study, the authors evaluated EBV-encoded RNA (EBER) expression by in situ hybridization in 300 cases of malignant lymphomas diagnosed by lymph node biopsy, with 100 cases of reactive lymphoid hyperplasia in lymph nodes as controls, for comparison. There were 100 consecutive cases of classical Hodgkin's lymphoma (cHL), 100 consecutive cases of non-Hodgkin's lymphoma, B cell (NHL-B), and 100 consecutive cases of non-Hodgkin's lymphoma, T cell (NHL-T). EBER expression was detected in 46% of reactive lymphoid hyperplasia cases, but the positively stained cells in those cases constituted less than 5 percent of the total cell populations. When using the presence of EBER in 5 percent or more of the cell population and/or the presence of EBER in the Hodgkin's Reed-Sternberg's cells as indicators of positivity, 64% of cHL, 13% of NHL-B, and 51% of NHL-T were found to be positive. The study indicates a strong association of cHL and NHL-T with EBV infection, the link apparently being weaker for NHL-B except for the subtypes of Burkitt's lymphoma and diffuse large B cell lymphoma.

Epstein-Barr virus-associated peripheral T-cell lymphoma with gastrointestinal tract involvement.

Peripheral T-cell lymphoma (PTCL) is a group of diseases which are common in Asia and areas of South and Central America. They are highly associated with the Epstein-Barr virus (EBV) infection. In the present study the authors evaluated patients with gastrointestinal involvement of PTCL with respect to clinical findings and outcome, pathologic features, and molecular analysis for EBV infection and the clonality of tumor cells. From January 1997 through December 2000, 7 patients with gastrointestinal tract involvement of PTCL were identified. The frequency of gastrointestinal tract involvement in the various types of PTCL was 5.4 per cent (7 of 129 cases). The pertinent clinical features were prolonged fever, weight loss, anemia, hepatosplenomegaly, lymphadenopathy, multiorgan involvement, and gastrointestinal bleeding. Laboratory results showed a significantly high serum level of alkaline phosphatase and lactate dehydrogenase, and abnormal coagulograms. Five patients died within 4 months after onset of illness, while two were in complete remission after chemotherapy. The tumor cell morphology was classified into three categories: small-sized cells, mixed medium- and large-sized cells, and large-sized cells. The antigenic phenotypes of the tumor cells were LCA+, CD3+, CD15-, CD16-, CD30-, CD45R0+, CD57-, CD68-, EMA-, betaF1-, granzyme B+, TIA-1+, and p53+. The expression of CD4, CD8, CD56 and CD20 was variable. EBV-RNA expression by in situ hybridization (EBER-ISH) study was positive and T-cell receptor (TCR) beta and/or gamma gene rearrangements were detected in all patients. DNA sequence analysis showed high identity to the human TCR germline gene. PTCL with gastrointestinal tract involvement was associated with EBV infection. The tumor cells were mature T cells with some NK-cell antigenic expression and all demonstrated TCR gene rearrangements.

Epstein-barr virus-associated non-Hodgkin's lymphoma of B-cell origin, Hodgkin's disease, acute leukemia, and systemic lupus erythematosus: a serologic and molecular analysis.

Parallel studies of (a) patients with Epstein-Barr virus (EBV)-associated peripheral T-cell proliferative disease/lymphomas and (b) a group of patients with a prolonged fever from other causes were conducted at Songklanagarind University Hospital from 1997 through 2000. (Reports on EBV-associated peripheral T-cell and NK-cell proliferative disease/lymphomas have been published elsewhere) In this study, the authors identified 58 patients; 14 were non-Hodgkin's lymphoma of B-cell origin (NHL-B), 8 were Hodgkin's disease, 6 were acute leukemia, 9 were systemic lupus erythematosus (SLE), and 21 were patients with other diseases. Serologic tests for the EBV infection, the study of EBV genome in circulating non-T-cells (CD3-cells) and T-cells (CD3+ cells), and the EBV-RNA study in the tumor cells were performed. EBV internal repeat-1 region (IR-1) in peripheral blood CD3+ cells was detected in 10 of 14 patients (71.5%) with NHL-B, 3 of 8 patients (37.5%) with Hodgkin's disease, 1 of 6 patients (16.7%) with acute leukemia, 4 of 9 patients (44.5%) with SLE, and was not detected in any of the 21 patients with other diseases. Anti-viral capsid antigen-IgG was significantly elevated in hematologic malignancy patients with EBV IR-1 genome in the peripheral blood CD3+ cells when compared to hematologic malignancy patients with a negative result, whereas there was no significant difference in anti-EBV nuclear antigen among these two groups. EBV-RNA expression in tumor cells by in situ hybridization was detected in 4 of 13 patients (31%) with NHL-B (all showed EBV IR-1 genome in peripheral blood CD3+ cells), and 3 of 5 patients (60%) with Hodgkin's disease (only two showed EBV IR-1 genome in peripheral blood CD3+ cells). These data support the theory that chronic EBV infection is often found in association with cases of NHL-B, Hodgkin's disease, acute leukemia, and SLE.

Congenital megacolon: diagnosis using size of sub-mucosal nerve trunk as a criteria.

Cholinergic hyperinnervation in submucosa of aganglionic segment of Hirschsprung's disease has been described. However, objective measurement of the nerve fibers was insufficiently reported. OBJECTIVES: To study the amount and size of hypertrophic submucosal nerve trunks in the aganglionic segment of Hirschsprung's disease, semiquantitatively and evaluate the possibility of using the size as objective diagnostic criteria. MATERIAL AND METHOD: Thirteen specimens from aganglionic segments from Hirschsprung's disease and six specimens from the age-matched control subjected without colonic innervation disorders were studied with the S-100 immunohistochemical technic. The submucosal nerve trunks were counted per ten HPF and measured in width, semiquantitatively. Fiber sizes were stratified into small (<20 micrometers), medium (20-40 micrometers), and large (>40 micrometers). Maximum fiber size in each specimen was also recorded. RESULTS: Average density of submucosal nerve trunk in the aganglionic segment and the control were 12.5 and 3.4 fibers per ten HPF, respectively. The large nerve trunks were detected in 12 of 13 aganglionic specimens and none was detected in the controls. The maximum fiber size in aganglionic segments was 78.2 micrometers, compared to 24.1 micrometers in the control group. The sensitivity and specificity to set the presence of hypertrophic nerve trunk, larger than 40 micrometers as a diagnostic criteria for Hirschsprung's disease were 92.3 and 100 per cent, respectively. The data suggest the use of this size as an objective diagnostic criterion, which may be valuable in rectal suction biopsy specimens.