Retinal ependymoma: an immunohistologic and ultrastructural study

Glial tumors of the retina are rare. Most are syndrome associated and include pilocytic astrocytoma in neurofibromatosis type 1 and subependymal giant cell astrocytoma in tuberous sclerosis complex. Acquired, more conventional, diffuse astrocytomas are less frequent. Ependymoma is exquisitely rare. Herein, we report the clinicopathologic features of the second case of retinal ependymoma. The tumor was sporadic in occurrence and unilateral, low grade, and of cellular type. Its chronic course and large size prompted an initial pathologic diagnosis of "massive retinal gliosis." The literature regarding retinal glial neoplasia including ependymoma as well as the so-called massive retinal gliosis is discussed.     

Allergic fungal sinusitis: an immunohistologic analysis

BACKGROUND: Allergic fungal sinusitis is a noninvasive form of fungal sinusitis that has recently been delineated as a distinct clinicopathologic entity. It is increasingly recognized as a cause of chronic sinusitis, with the primary causative agents being members of the Dematiaceae fungus family. Although its immunopathogenesis has not been elucidated, the eosinophil is a prominent inflammatory cell on histologic examination. OBJECTIVE: We sought to characterize the involvement of eosinophils in sinus tissue and accompanying mucin from patients with allergic fungal sinusitis. As a comparison, neutrophil and mast cell involvement was also evaluated in the same group of patients. METHODS: Tissue specimens from 8 patients with allergic fungal sinusitis, along with 8 nasal polyp specimens from patients without allergic fungal sinusitis, were stained by using indirect immunofluorescence for eosinophil granule major basic protein (MBP). Neutrophil elastase and mast cell tryptase staining was also performed on the same allergic fungal sinusitis and nasal polyp tissues. RESULTS: MBP was diffusely localized within the mucin, showing intense staining at the periphery and variable staining of degenerated cell clusters throughout. Extracellular MBP in the mucin was strikingly greater than intact eosinophil staining. Diffuse extracellular neutrophil elastase was also present in the mucin. Mucinous areas showed no tryptase localization. Adjacent nonmucinous areas of respiratory mucosa showed predominantly cellular staining with eosinophil MBP, neutrophil elastase, and mast cell tryptase. MBP staining of nasal polyps showed a predominantly cellular pattern with focal areas of extracellular deposition. CONCLUSIONS: Given the known toxicities of eosinophil granule MBP and neutrophil elastase, their extracellular presence supports the contribution of these proteins in the pathogenesis of allergic fungal sinusitis and further indicates that eosinophil and neutrophil activation occurs in the disease.     

Intermediate lymphocytic lymphoma: an immunohistologic study with comparison to other lymphocytic lymphomas

In an immunohistologic analysis of 13 cases of intermediate lymphocytic lymphoma (ILL), the immunophenotype of ILL was compared to the immunophenotypes of other B-lymphocytic lymphomas and the normal lymphoid follicle to determine the normal cell in the scheme of B-cell differentiation that corresponds to ILL. The characteristic immunophenotype of ILL was surface IgM +/- D+, cytoplasmic immunoglobulin -, B1+, BA1+, B2-, BA2-, B4+, Leu 14+, HLA-DR+, Leu 1+, and common acute lymphoblastic leukemia associated (CALLA) antigen -. The immunophenotype of ILL was similar to that of lymphocytes in normal primary follicles and the mantle zones of secondary follicles. The "immature" phenotype of ILL was identical to that of small lymphocytic lymphoma, which strongly supports their close lineage relationship. In contrast, the "mature" phenotypes of the follicular center cell and lymphoplasmacytoid lymphomas suggest that they correspond to normal cells at later stages of differentiation. Our findings indicate that B-lymphocytic lymphomas recapitulate the normal stages of B-cell differentiation. The cell of ILL appears to be an immature B cell that homes to, and resides in, primary follicles and the mantle zones of secondary follicles. The cytologic, architectural, immunologic, and clinical features of ILL indicate that it should be included as a separate category in the International Working Formulation.     

The macrophagic origin of multinucleated giant cells in myeloma kidney: an immunohistologic study

The cast-engulfing giant cells in a case of kappa light chain nephropathy/Bence Jones cast nephropathy were characterized with monoclonal antibodies Uro-5 (renal epithelium: Henle's loop, distal tubule, collecting duct) and Leu-M5 (macrophage/monocyte/dendritic cell specific). The giant cells were Leu-M5 positive and Uro-5 negative. There was an increased concentration of Leu-M5-positive cells surrounding cast-containing tubules, and occasional Leu-M5-positive cells were found within the tubular epithelial layer. Although there were no Uro-5-positive giant cells, an occasional Uro-5-positive cell was found within casts. The majority of casts were found within Uro-5-positive tubules. These results confirm earlier electron microscopic reports that the cell of origin of the cast-engulfing multinucleated giant cell is the macrophage (Leu-M5 positive, Uro-5 negative). These giant cells may originate from macrophages that have migrated from the interstitium through the tubular epithelium.     

Chordoma: cytologic and immunocytochemical study of four cases

We present the cytologic features and the immunocytochemical profile of four cases of chordoma on fine-needle aspiration biopsies. The physaliferous cells in signet-ring, pearl-like formations and the trabecular arrangement with rounded contours are distinctive. Other cell types and cellular arrangements are also described. The negative immunoreactivity of carcinoembryonic antigen (CEA) and the positive staining pattern for neuron-specific enolase (NSE), S-100 protein, epithelial membrane antigen (EMA), and keratin provide a profile that, in the appropriate clinical setting, can be useful in the differential diagnosis of chordoma from similar-appearing neoplasms in small biopsies and fine-needle aspirates.     

Syringoma: a clinicopathologic and immunohistologic study and results of treatment

The purpose of our study was to describe clinical and histopathological features of sixty one patients with histological diagnosis of syringoma over four year period in our dermatology clinic in Korea. Female:male ratio was 6.6:1 with onset of age during 2nd and 3rd decades in more than half of the patients in our study. The most frequently involved site was eyelids (43 cases, 70.5%) and the most common color of lesion was skin-color (30 cases, 49.2%). In 34 cases, characteristic tad-pole appearances (55.7%) were observed. Basal hyperpigmentation was observed more frequently in brown-colored lesion (p=0.005). Fibrosis was observed more frequently in erythematous lesion (p=0.033). Keratin cyst was observed less frequently in genital involved group (p=0.006). We also performed immunohistochemical stain for the presence of progesterone receptor (PR) and estrogen receptor (ER) in fifty six cases with negative results.     

Chordoma

Chordoma is characterized clinicomorphologically and epidemiologically using own observations for 1955-1995 and literature data. Chordoma is a rare tumor. Its histogenetic association with the notochorda remnants and location in the region of axial skeleton results in a grave clinical course due to alteration of nervous structures of the brain and spinal cord. The diagnosis is based on roentgenological data showing the connection of the tumor with axial skeleton (in sacral-coccygeus and occipital-basilar regions in 90% of cases) and on typical histologic structure.     

Chordoma. An immunohistochemical study of 20 cases

Twenty chordomas from 20 patients, including 17 nonchondroid and three chondroid types, were studied with a variety of antibodies directed against cytokeratin (AE-1/3), epithelial membrane antigen, carcinoembryonic antigen, S100 protein, vimentin, alpha 1-antichymotrypsin, and lysozyme. All 17 nonchondroid chordomas stained for cytokeratin, and most (16) stained for epithelial membrane antigen. In contrast, two chondroid chordomas failed to stain for either cytokeratin or epithelial membrane antigen, while one of them did stain for both antigens. Sixteen of the 20 chordomas (80%) stained for S100 protein, including all three chondroid chordomas. Vimentin was found in six (30%), and alpha 1-antichymotrypsin in 16 chordomas (80%). Carcinoembryonic antigen and lysozyme were each found in two specimens (10%). While these findings basically agree with the immunohistochemical studies of other investigators, there are a few discrepancies. Most significant is the lack of epithelial markers in two of three chondroid chordomas located at the base of the skull. Possible reasons for the discrepancies are discussed.     

Chordoma. A clinicopathologic study of metastasis.

Thirty cases of chordoma were reviewed with respect to the incidence of distant metastases. Follow-up information was obtained in 27 (90%), and the incidence of metastases was approximately 30%. The sites of the metastasizing primary tumors were predominantly sacral and vertebral. The sites of the metastases were predominantly skin and bone, although metastases were found in the lungs and lymph nodes. In two of the three patients with dermal metastases, the metastases were present prior to the diagnosis of the primary lesions. All three dermal metastases were initially diagnosed as mixed tumors of the skin, and all three patients had at least four such lesions of the skin. Accurate prediction of which chordomas will eventually metastasize is difficult. Clinically, local aggressiveness and radiotherapy were positively correlated with this ability; histologically, more anaplastic chordomas were more likely to metastasize.     

Recurrent "syncytial variant" of Hodgkin's disease: an immunohistologic diagnosis

A case of recurrent Hodgkin's disease of the "sarcomatoid" or "syncytial variant" type was seen that occurred as an extension from the mediastinum to a previously uninvolved extranodal site (breast) and pericardium after treatment of classical nodular sclerosing Hodgkin's disease based in the lymph nodes. This histologic variant was composed of sheets of large, undifferentiated neoplastic cells with few, if any, diagnostic features of nodular sclerosing Hodgkin's disease. For this reason, the differential diagnosis of this variant was difficult and included non-Hodgkin's lymphoma (peripheral T-cell lymphoma), Ki-1-positive lymphoma, medullary carcinoma, metastatic carcinoma, melanoma, and granulocytic sarcoma. Immunologic analysis by immunoperoxidase technique showed a phenotype consistent with "syncytial variant" Hodgkin's disease: Leu-M1+, Ki-1+, IL-2+, HLA-DR+, T11-, pan B-, K-, lambda-, cytokeratin-, S-100-, muramidase-.     

A longitudinal study of histologic and immunohistologic changes in an experimental model of sclerosing cholangitis*

Summary A longitudinal study of intra and extrahepatic bile duct injuries was performed in an animal model of secondary sclerosing cholangitis induced by formalin injection into the common bile duct. Lymphocytic infiltration inside and around the bile ducts occurred seven days after injection. The disease later evolved to a fibrous cholangitis of the small bile ducts. Septal intrahepatic and extrahepatic bile duct involvement became evident three months after formalin injection. The ductular proliferation led to a progressive biliary cirrhosis with portal to portal fibrous septa. After formalin injection, bile duct cells expressed the Ia antigen in the cytoplasm and/or on the membrane of bile duct cells. The intensity of staining did not correlate with the duration or severity of the disease. Lymphocytes infiltrating into and around the bile duct were mainly T-cells. This study suggests that a local cell-mediated immune response to the injection of a toxic agent induces pathological features similar to those of sclerosing cholangitis in man.This study was supported in part by a grant from the Research Council of the Faculté de Médecine, Université de Paris-Sud, and the Faculté de Médecine, Paris VI     

Sinonasal small cell neoplasm developing after radiation therapy for retinoblastoma: an immunohistologic, ultrastructural, and cytogenetic study.

Patients with retinoblastoma have an increased risk of developing second primary tumors. Only a few examples of sinonasal small cell neoplasms developing after radiation therapy for retinoblastoma have been reported. We report one such case that developed 18 years after treatment for retinoblastoma. Histologic examination revealed a small, blue, round cell tumor without rosettes or cytoplasmic glycogen. Immunohistochemically, the tumor cells were positive for neuron-specific enolase, synaptophysin, and S-100 protein, but negative for epithelial and mesenchymal markers, suggesting that this was a primitive neuroectodermal tumor. Cytogenetic studies of this tumor failed to reveal the chromosome 13 abnormality typical of retinoblastoma and the t(11:22) translocation typical of the group of peripheral neuroepitheliomas.     

Thymic epithelium in AIDS. An immunohistologic study.

The authors investigated by immunofluorescence the thymic epithelium from AIDS patients, using several structural and functional markers of this tissue. In AIDS thymus, the epithelial reticular pattern was lost; and instead, clusters of round or spindle-shaped cells (assessed by their keratin content) were found. In addition, regions of epithelial necrosis, with loss of cell limits, were frequently observed. Thymulin content was decreased, and a partial loss of differentiation antigens of the thymic epithelium was also detected. The authors showed the presence of immunoglobulins and elements of the complement system directly bound to AIDS thymic epithelium, but not in any of the controls studied, including thymuses from patients who died after a long period of agony. These data suggest that the thymic epithelium can be included among the target tissues in AIDS, possibly by an indirect mechanism of autoimmune destruction.     

Mammaglobin vs GCDFP-15: an immunohistologic validation survey for sensitivity and specificity

There are limited data that compare the usefulness of mammaglobin with gross cystic disease fluid protein-15 (GCDFP-15) in the identification of breast carcinomas. Whole tissue sections of 29 breast carcinomas with matched lymph node metastases and 63 breast carcinomas on tissue microarray were stained with mammaglobin cocktail and GCDFP-15 antibodies. In addition, tissue microarrays (US Biomax, Rockville, MD) containing 544 different human tumors were also stained with the mammaglobin antibody cocktail. Positive staining was seen in 67 (55.4%) of 121 breast carcinomas with mammaglobin and in 28 cases (23.1%) with GCDFP-15. In the majority of cases, the staining intensity and number of cells staining were higher with mammaglobin than with GCDFP-15. Positive mammaglobin staining was also seen in 44 (8.1%) of 544 nonbreast tumors. Mammaglobin is a more sensitive marker than GCDFP-15 for breast carcinoma; however, it lacks the specificity of GCDFP-15.     

Neuroendocrine differentiation in poorly differentiated lung carcinomas: a light microscopic and immunohistologic study

Frozen, unfixed tissue sections from 56 poorly differentiated, non-small cell primary lung tumors were examined by the immunoperoxidase technique with a panel of monoclonal antibodies to neuroendocrine (chromogranin A (CGA), synaptophysin (SYN), S-100) and intermediate filament (cytokeratin, vimentin, neurofilament) antigens. Although light microscopic features of neuroendocrine (NE) differentiation were not present, staining for CGA and/or SYN was identified in 5/17 (29%) large cell carcinomas (LCC) and 4/19 (21%) poorly differentiated adenocarcinomas (PDA). Diffuse, strong SYN staining was present in two LCC and one PDA. Heterogeneous intermediate filament expression (vimentin and/or neurofilament) was frequent (LCC, 10/17 (59%); PDA, 10/19 (53%)) and accompanied NE markers in 8/9 (89%) cases. Poorly differentiated squamous carcinomas were more homogeneous, with focal SYN in only 1/20 (5%) and focal presence of intermediate filaments other than cytokeratin in only 2/20 (10%). We conclude: (a) immunohistologic evidence of NE differentiation is present in a significant proportion of pulmonary large cell and poorly differentiated adenocarcinomas and rare in poorly differentiated squamous carcinomas; (b) NE differentiation is generally accompanied by heterogeneous intermediate filament expression; (c) divergent NE differentiation is not necessarily reflected by light microscopic features.