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Diffuse ganglioneuromatosis (DG) of the gastrointestinal tract is a rare condition that is closely associated with neurofibromatosis type 1 and multiple endocrine neoplasia type 2B. The occurrence of DG with multiple schwannomas, which, of the GI tract, usually affect the stomach, is considerably more rare. The present study describes the case of a 54-year-old male with indolent DG, principally involving the small intestine and colon, associated with multiple schwannomas in the subserosa. The patient was treated with surgery. A brief overview of intestinal ganglioneuromatous lesions and the associated conditions is additionally presented.

To study the relationship between immunoglobulin variable heavy chain (IgVH) gene mutation status and clinical features, pathologic findings and biologic behavior of mantle cell lymphoma (MCL).IgVH gene was amplified in 60 cases of MCL with FR1-JH and FR2-JH primers in BIOMED-2. The sequence was determined by cloning. The IgVH somatic mutational status was analyzed using NCBI's Ig-Blast tool. The relationship between IgVH gene mutation status and clinicopathologic features was also analyzed.Forty percent (24 cases, 28 functional Ig genes) of the MCL cases displayed somatically mutated VH genes (defined as2% mutated), whereas 60.0% (36 cases, 40 functional Ig genes) showed unmutated VH genes. The most widely used genes were VH3-21 (27.9%) and VH4-34 (19.1%). The former were mainly used by unmutated cases, while the later mainly by mutated cases.Intraclonal heterogeneity was noted in 19 cases. There was no correlation of VH mutation status and specific VH gene with survival (P0.05).MCL comprises at least two subsets that do not correlate with morphology: one with unmutated VH genes and one with mutated VH genes. The biased use of VH3-21 and VH4-34 is noted. The nonrandom usage of IgVH segments suggests specific antigens may play a role in the pathogenesis and progression of MCL subsets. There is no correlation of IgVH mutation status and specific VH gene with survival.

To study the clinicopathologic features, immunophenotype, histological diagnosis and prognosis of hepatic epithelioid angiomyolipoma.Clinical data of 25 cases of hepatic epithelioid angiomyolipoma were collected along with follow-up study of the patients. The pathological features were documented and immunohistochemical study of various markers was performed with an emphasis on diagnosis and differential diagnosis.Hepatic epithelioid angiomyolipoma was more commonly found in young women without characteristic clinical symptoms. Its morphological features were characterized by marked cytological atypia, relatively rare mitotic figures; radial distribution of tumor cells around the thin-walled blood vessels or muscular vessels; and the presence of common multinucleated giant cells and large ganglion-like tumor cells. The tumor cells expressed both melanoma cell markers (HMB45, MART-1) and smooth muscle cell markers (SMA). Tumor cells expressed various other markers including ER 16% (4/25), PR 32% (8/25), TFE3 24% (6/25) and p53 60% (15/25).Hepatic epithelioid angiomyolipoma has variable morphological features and characteristic immunohistochemical phenotype. The differential diagnoses include a variety of tumors. The biological behavior of the tumor tends to be benign.

We report an uncommon 22-year-old male Pulmonary Langerhans Cell Histiocytosis (PLCH) case which co-existed with pulmonary tuberculosis (TB). Unlike the common PLCH cases, this PLCH case has cervical lymph node involvement and right pneumothorax. The diagnosis was established by the imaging of lung and the biopsies of the lung and left neck lymph node. Imaging of the chest showed characteristic small nodules and thin-walled cysts and right pneumothorax. The LCH cells in the lung and left neck lymph node were characterized by large convoluted nuclei with cerebriform indentations of the nuclear envelope and longitudinal grooves. The nuclei contained small eosinophilic nucleoli and moderate amount cytoplasm. Immunohistochemically, the histiocytoid cells were positive for Langerin, CD1a and S-100. Acid-fast bacilli were found in sputum and lung biopsy tissue. To the best of our knowledge, this is the first case of PLCH with cervical lymph node involvement, and coexisted with pulmonary tuberculosis, right pneumothorax. A contribution of this case and review three of the five cases of PLCH with extrapulmonary involvement to lymph nodes resolved spontaneously after smoking cessation constitute a novel addition that it is inappropriate to regard pulmonary/nodal LCH as multi-organ or disseminated disease, and the treatment methods are the same whether the PLCH patient with lymph node involvement or not.

To analyze the clinicopathological features of pancreatic neuroendocrine neoplasms (P-NENs).From January 2006 to December 2010, 64 patients with P-NENs were diagnosed in the Department of Pathology, West China Hospital, Sichuan University. Immunohistochemical staining of neuroendocrine markers, synaptophysin (Syn) and chromogranin A (CgA), were first made to determine whether the tumor had neuroendocrine properties, then the P-NENs were classified as neuroendocrine tumor (NET), neuroendocrine carcinoma (NEC) and mixed adenoneuroendocrine carcinoma (MANEC, G3) according to the morphological changes and proliferative activity (Ki 67 expression).Of all the 64 cases detected, 60 were NETs and four were NEC. Most of the tumors were single solitary masses, and more than half of the tumors arose in the head of the pancreas, while about one third in the tail. The positive rates of CgA and Syn immunostaining were 96.9% and 95.3%, respectively. The tumor stages of the 64 patients were as follows: stage I, 44 cases; stage II, 11 cases; stage III, one case; and stage IV, 8 cases. The median age of patients in the study was 45.56 years. Of all the P-NENs, 38 cases were functional ones, presenting with characteristic clinical syndrome owing to hormone hypersecretion, while 26 cases were nonfunctional ones with no distinct clinical syndrome. 58 patients underwent surgical operation. The 5-year progression-free survival rate was 91.4%.P-NENs may occur anywhere in the pancreas, and the clinical manifestations may not be easy to distinguish from other diseases. Diagnosis depends on pathological examination. Surgery is the major approach option, and the clinical prognosis is rather good. The tumor histological grade and distant metastasis are independent prognostic factors in P-NENs.

The purpose of this study was to test the hypothesis that cooperative interaction between cutaneous fatty acid-binding protein (C-FABP) and peroxisome proliferator-activated receptors (PPAR) promotes the malignant progression of human prostate cancer. The expression of C-FABP, PPARβ/δ and PPARγ was measured by western blot analysis in prostate cell lines and by immunohistochemical staining in tissue sections of benign prostatic hyperplasia (BPH) and prostatic carcinomas. The correlation between the expression of PPARs and C-FABP was assessed. The significance of increased expression of these proteins was analysed with respect to prognosis and compared with those of alternative biomarkers. The expression levels of C-FABP and PPARγ in prostate cancer cell lines and the cytoplasm and nuclei of carcinoma tissues were significantly (Student's t-test, p

Primary intestinal NK/T cell lymphoma is extremely rare and early diagnosis is frequently difficult. The aim of this study is to investigate the clinicopathological findings, immunophenotype, and T cell receptor (TCR) γ gene rearrangement of primary intestinal NK/T cell lymphomas in 25 Chinese cases.Clinical data of the 25 cases were analyzed. Immunohistochemistry for immunophenotype, in situ hybridization for EBER, and polymerase chain reaction for TCR γ gene rearrangement were investigated. Survival curves according to clinical characteristics were analyzed.The median age was 33 years and the median survival was 7 months. The common symptoms consisted of abdominal pain, fever, marasmus, diarrhea, and hematochezia. Endoscopically, the tumors were mainly featured by focal, multifocal or diffuse irregular ulcers, which most frequently emerged in the ascending colon. Histologically, the tumors were characterized by the proliferation of pleomorphic atypical lymphoid cells (ALCs), necrosis, lympho-epithelial lesions, and mixed inflammatory infiltration. The positive frequency of CD3ε was 88.2%, of CD56 was 84%, granzyme B was 90%, and EBER was 84.2%. A total of 12 out of 14 cases (85.7%) highly expressed Ki67. The negative prognostic factors for survival were Ann Arbor stage IIIE or IVE (P = 0.039) and more than one extranodal site of disease (P = 0.019).Primary intestinal NK/T cell lymphomas most frequently favor young people and have a poor prognosis. Due to the nonspecific clinical and endoscopic findings, it is difficult to distinguish intestinal NK/T cell lymphomas from inflammatory and infectious disorders. Histopathology, immunophenotype, and DNA study play key roles in differential diagnosis.

Polymorphic reticulosis (PR) or midline malignant reticulosis (MMR) is considered to be malignant, or at least pre-malignant T-cell proliferations of the nose or midline area. Recent reports of small series of nasal T-cell lymphomas have shown a strong association with Epstein-Barr virus (EBV). Furthermore, a peculiar phenotype is described, with expression of CD56 and not of CD3, suggesting a possible origin from natural killer (NK) cells. We have analysed a series of 38 cases of PR/MMR for the presence of EBV by in situ hybridization (ISH) of the EBV-encoded RNAs 1 and 2 (EBER). Twenty cases were tested for expression of EBV-encoded latent membrane protein 1 (LMP-1). Special attention was also paid to the expression of CD3 and the NK cell-related marker CD56. Thirty-two cases (84 per cent) showed positive EBER ISH. In 5 of 20 cases, LMP-1 expression was detected. In three cases, a few scattered cells were positive, and in two cases, LMP-1 was detected in clusters of atypical cells. Most of the neoplasms showed expression of CD3 (89 per cent) and in 27 cases (71 per cent), CD56 was detected. These results are consistent with an aetiopathogenetic role for EBV in most, but not all, cases of PR/MMR. Our findings are less supportive of a major role for LMP-1 in tumour genesis. CD3 expression in most of the cases of PR/MMR underlines the T-cell origin of these neoplasms, often with aberrant expression of CD56.

To study the clinicopathologic and immunohistochemical features of primary pulmonary extranodal marginal zone B-cell lymphomas (MALT lymphomas).Immunohistochemical staining for LCA, CD20, CD45RO, CD5, cyclinD1, Ki-67, immunoglobine light chain κ and λ, CK and NSE was carried out in 9 patients with primary pulmonary extranodal marginal zone B-cell lymphomas by the S-P methods.According to the histological pattern and immunohistochemical features, all patients were diagnosed as primary pulmonary extranodal marginal zone B-cell lymphomas. There were four males and five females with a median age of 51.5 years (range from 37 to 64 years). The tumor cells were positive for LCA and CD20, however, the positive rates of Ki-67 were low. The tumor cells expressed immunoglobine light chain restriction λ in 5 cases , κ in 1 case, both λ and κ in 1 case, and neither of them in 2 cases. The tumor cells were negative for CD45RO, CD5, cyclineD1, CK, NSE in all patients.Primary pulmonary extranodal marginal zone B-cell lymphomas are inert lymphomas which are easily misdiagnosed. The evaluation of pathologic features and immunohistochemistry are useful and practical in the diagnosis and differential diagnosis of primary pulmonary extranodal marginal zone B-cell lymphomas.

The expression of p53 protein in 66 cases of colorectal cancer and its relationship to cell porliferative activity, lymph node metastasis as well as prognosis were investigated by means of AB-PAP immunohistochemical technique. The results showed that 62.1% of colorectal cancer was positive of p53 expression. The cell proliferative activity and the frequency of lymph node metastasis in p53- positive tumors were significantly higher than those in p53-negafive tumors (P

To evaluate the potential diagnostic role of A103 and inhibin alpha in adrenocortical adenoma and the feasibility of tissue microarray/tissue chips.A series of adrenal gland tissue chips were prepared, including 79 cases of formalin-fixed, paraffin-embedded tissue from 3 normal adrenal glands, 66 adrenocortical adenomas, 5 cases of metastatic carcinoma and 5 pheochromocytomas. A103 and inhibin alpha were detected with Envision immunohistochemical staining methods.Positive staining was seen in 3 of 3 (100%) adrenal cortices and in 55 of 61(90.2%) adrenocortical adenomas but in none of the pheochromocytomas or adrenal metastatic carcinomas. In the 3 of 3(100%) adrenal cortices, inhibin alpha immunoreactivity was limited to the zona reticularis and the innermost zona fasciculata. 51 of 61(83.6%) adrenocortical adenomas reacted with inhibin alpha. None of the pheochromocytomas or metastatic carcinomas reacted with inhibin alpha.High-throughput tissue microarray/tissue chip offers a powerful strategy for rapid identification in situ. It is feasible to utilize tissue microarray for a rapid, economic and accurate screening of tissue specimens on a large scale. The combined use of A103 and inhibin alpha is valuable in distinguishing adrenocortical adenoma from pheochromocytoma and other metastatic carcinomas.

To investigate the cell lineage and Epstein-Barr virus infection in previously diagnosed cases of malignant histiocytosis (MH) with tissue microarray technique.Using tissue-chips, immunohistochemical staining, in situ hybridization and PCR to analyze 5 autopsy cases of MH.(1) In all 5 cases, positive reactions of CD45RO, CD3 epsilon, TIA-1, Granzyme B were detected in the neoplastic cells, whereas negative reactions were found with CD30, CD20, CD56. (2) All cases revealed EBER1/2 positivity in neoplastic cells.Previously diagnosed malignant histiocytosis is an EBV-associated aggressive NK/T cell lymphoma.

To investigate the roles of different clinicopathological features and expression of EBV genome in prognosis of intestinal T-cell lymphoma (ITCL).Polymerase chain reaction for TCR-gamma gene rearrangement, in situ hybridization for EBER1/2 and immunohistochemical staining for CD4, CD8, CD45RO, CD56, TIA-1 were investigated and all patients followed-up. The LMP-1 expression was determined in forty-two ITCLs cases. The relationship between clinical data, different clinicopathological features, expression of EBV genome and prognosis were analyzed by SPSS10.0 program.(1) All 42 cases of ITCL had an extremely poor prognosis with a median survival of 3.0 months, of which the one year survival rate and two year survival rate being 30% and 22% respectively. (2) The patients without TCR-gamma gene rearrangements showed poorer prognosis than those with TCR-gamma gene rearrangements, and the patients who received operation and chemotherapy showed better prognosis than those who only received operation (P0.05). (3) No significant prognostic factor for ITCLs was determined.The special clinicopathological features of ITCL could be due to the cytotoxic function and the role of EBV infection in the pathogenesis of ITCL.

To investigate the clinicopathologic features of mantle cell lymphoma (MCL) and the significance of immunostaining for cyclin D1 in diagnosis.Clinicopathologic observation and immunohistochemical staining for CD20, CD45RO, cyclin D1, bcl-2, Ki-67, CD5 for 8 cases of mantle cell lymphoma were performed.The 8 cases of mantle cell lymphoma consisted of 6 males and 2 females, aged from 43 to 78 years (mean 57 years). Histopathologically, MCL demonstrated architectural destruction by a vaguely nodular monomorphic lymphoid proliferation with vaguely nodular, diffuse or mantle zone growth patterns. Analogous to centrocytes, the lymphoma cells with slightly to markedly irregular nuclear contours showed moderately dispersed chromatin and a low mitotic figure. Three cases were transformed into highly aggressive blastoid variants. The tumor cells were positive for CD20, CD5, bcl-2 and cyclinD1 in all 8 cases and negative for CD45RO.The clinicopathological features and special immunophenotypes were present in mantle cell lymphoma. This tumor can be differentiated from other small B-cell lymphomas on the basis of histopathologic features and positive cyclin D1 immunophenotype. The blastoid variant should also be differentiated from other variants.

To explore the clinical and laboratory features of disseminated Penicillium marneffei infection in patients with AIDS.The HIV antibody in serum was assayed by both enzyme immunoassay (EIA) and Western immunoblot (WIB) methods. Morphology of the pathogenic fungus in smear and biopsy specimens of bone marrow was observed. The fungus was isolated from the patient's skin lesion and inoculated into the abdominal cavities of 2 rats and 2 mice. Twenty days later the rats and mice were killed and their viscera were taken out. Blood from the organs were cultured in Sabourand glucose agar at 25 degrees C and 37 degrees C. The colonies were observed. The morphology of the fungus was observed by microscopy and scanning electron microscopy.The most common clinical manifestations of Penicilium marneffei infection were fever, weight loss, anemia, papular skin lesion, hepatosplenomegaly, and lymphadenectasis. Yeast-like cells were found in the culture at 37 degrees C or in tissues. The fungi outside the host cells were elongated, often curved, sausage-like and with clear central septi. When cultured at 25 degrees C, the fungus was mycelia-like and produced a characteristic red pigment, diffusing into the medium.Disseminated Penicilliosis marneffei is one of the most important opportunistic infections in patients with AIDS in Southeast Asia and the southern part of China. Since there is no specific clinical manifestation for Penicillium marneffei infection, it is often misdiagnosed. Definite diagnosis requires culture of the pathogenic fungus from clinical specimens. The fungus is thermally dimorphic, produces red pigment, and is sausage-form with clear central septum outside the host cell. Amphotericin B and itraconazole are effective in treating Penicilliosis marneffei.

Dear Editor, A 75-year-old man visited the outpatient department in our hospital in November 2007 presenting with tender and swollen bilateral metacarpophalangeal joints and proximal interphalangeal joints with low fever. Serological tests revealed positive antinuclear antibody (ANA) of 1:320 (speckled), anti-SSA antibody (2+), positive rheumatoid factor of 53.60 IU/ml, and anti-CCP antibody of 21.54 RU/ml (positive, >5 RU/ml). He was given a presumptive diagnosis of early rheumatoid arthritis. Nine months later, the patient was admitted to the rheumatology department complaining of fever with myalgia. No tender and swollen peripheral joints were palpable. Physical examination for his lung, heart, and abdomen was unremarkable. He had normal muscle strength except dorsiflexion weakness in his left foot. Neurological examination did not show obvious abnormality. Routine laboratory results showed normochromic, normocytic anemia (Hb=79 g/L), mild proteinuria (0.6 g/day) without active urinary sediment. He did not complaint of gastrointestinal disorder and his stool was normal and negative for occult blood. An obviously elevated erythrocyte sedimentation rate at 130 mm/1 h (