The surgical challenge of papillary neoplasia of the biliary tract.

This study is a case report and literature review of the surgical approach to papillary lesions of the biliary tract exclusive of the ampulla of Vater. Papillary lesions of the bile ducts, exclusive of the ampulla of Vater, are distinctly uncommon but, because of their unpredictable and aggressive behavior, pose challenging problems for the surgeon. Including the present illustrative case description, an English language literature review was conducted to determine the number and clinical behavior of papillary lesions of the bile ducts, particularly the propensity for malignant transformation, and the most favorable surgical approach. In addition to the present case, 29 patients with papillary biliary lesions were found in the literature. Twenty-two patients had tumors in multiple locations in the biliary tract, 6 had isolated lesions in one hepatic duct, and 3 had diffuse papillomatosis. Overall, 15 of 30 patients (50%) died of their disease. Patients with solitary tumors faired best (5 of 6 long-term survivors), and patients with diffuse papillomatosis did worse (all 3 died). Characteristics of these lesions include a propensity for recurrence (15 of 30 patients), an abundant mucin production, and a tendency toward malignant change (eight of 30 patients). Papillary lesions prove difficult to treat because of their frequent multifocality, propensity to recur after surgical extirpation, and malignant potential. Surgical strategy should include liver resection for isolated tumors, close monitoring for recurrence, and reoperations as required to control tumor growth.

Collagenous enterocolitis: a manifestation of gluten-sensitive enteropathy.

We report coexistent collagenous colitis and collagenous sprue in a 62-year-old woman with diarrhea. Investigations suggested malabsorption, and small intestinal biopsies demonstrated a flattened mucosa with subepithelial collagen deposition. Colonic biopsies also showed a thickened subepithelial collagen band as well as a striking lamina propria inflammatory cell infiltrate. Symptomatic remission was induced with a gluten/lactose-free diet, oral prednisone, and sulfasalazine and has been maintained with gluten restriction alone. Repeat biopsies after 2 months demonstrated restoration of normal small intestinal and colonic collagen bands; only a chronic inflammatory cell infiltrate (consistent with microscopic/lymphocytic colitis) persisted in colonic biopsies. We propose that, in this instance, collagenous enterocolitis represented a diffuse manifestation of gluten sensitivity.

Epstein-Barr virus-associated lymphoproliferative disorders.

Herein we have provided a panorama of the clinical, histopathologic, and molecular biologic mechanisms of EBV-induced LPD particularly in immunosuppressed individuals. A listing of EBV-related diseases is shown in Table 4. We have stressed the frequent need to use multiple diagnostic methods for detecting EBV genome, particularly in immunodeficient patients who may fail to mount antibody responses to EBV. Given that we now recognize some of the immunocompromised patient populations at high risk for EBV-induced LPD, and have developed techniques for detecting EBV genome and early LPD, we may eventually prevent the occurrence of some of these life-threatening diseases. For example, we have learned to recognize and distinguish hepatic allograft rejection from EBV-induced LPD in hepatic biopsies (154). A periportal and sinusoidal infiltrate of small and large lymphoid cells, immunoblasts, and plasma cells, alert us to stain frozen liver sections for EBNA. Finding EBV guides the clinicians to reducing immunosuppression which then allows the restoration of immunosurveillance against the EBV-infected B cells. Whether an EBV vaccine can be successful in immunosuppressed individuals remains to be seen. As for other vaccines, many logistical problems prevail, such as the early occurrence of EBV infection during infancy in regions where BL is endemic. Surely, with the menacing threat that approximately 10% of patients with AIDS will develop NHL, new anti-viral therapy against EBV and the causative agent of AIDS and HIV, will be developed. The pathologist and virologist play essential roles in the recognition of EBV infection by performing clinical laboratory determinations. The characteristic histopathologic features of EBV-induced LPD are now recognized and when confirmed with molecular hybridization and immunofluorescent techniques will provide a solid diagnostic approach and, thus, a foundation for developing a sound therapeutic strategy.

Immunoblastic lymphoma with abundant clear cytoplasm. A comparative study of B- and T-cell types.

The morphologic, phenotypic, molecular genetic, and clinical features of 34 cases of clear-cell immunoblastic lymphoma (IBLC) are described. Sixteen cases were of B-cell type (IBLC-B) and 18 cases were of T-cell type (IBLC-T). There were no significant differences in the morphologic characteristics of the neoplastic cells in the two types, although IBLC-B was less likely to be polymorphic than IBLC-T. Interfollicular proliferation, a higher mitotic rate, infiltration by eosinophils, and an increase in capillary-sized blood vessels were also features of IBLC-T, whereas necrosis and fibrosis were more extensive in IBLC-B. Patients with IBLC-B were predominantly female, whereas those with IBLC-T were predominantly male. The mean age was 62 years for those with IBLC-B and 46 years for those with IBLC-T. Patients with IBLC-B usually had lower-stage disease, but there was no significant difference in survival rate between those with IBLC-B and those with IBLC-T. Although most cases of IBLC have been considered to be of peripheral T-cell origin, the authors conclude that IBLC-B is more common than previously considered and that clear-cell morphologic characteristics are not a reliable indicator of T-cell type.

Nerve growth factor receptor expression on dendritic reticulum cells in follicular lymphoid proliferations.

Using an antibody to the nerve growth factor receptor (NGFR), we examined dendritic reticulum cells (DRCs) immunohistochemically in 62 formalin-fixed, paraffin-embedded lymph nodes from patients with reactive follicular hyperplasia or with various types of lymphoma. A dendritic staining pattern within germinal centers was present in 25 of 26 routinely processed lymph nodes with reactive follicular hyperplasia. In contrast, dendritic staining with anti-NGFR was present within neoplastic follicles in only three of 28 follicular lymphomas. Staining of benign, residual germinal centers with anti-NGFR was present in mantle zone lymphoma and Hodgkin's disease. These findings suggest a possible role for the NGFR in the maturation and/or activation of normal DRCs. The loss of NGFR expression in most follicular lymphomas indicates that DRCs are altered as part of the neoplastic process. The possibility that DRCs may play a role in the pathogenesis of follicular lymphoma is suggested.

Neural patterns in inflammatory bowel disease: an immunohistochemical survey.

The differential diagnosis of inflammatory bowel disease remains a significant diagnostic problem for surgical pathologists. Neural abnormalities, such as hypertrophy of nerve plexi, hyperplasia of ganglion cells, and ultrastructural axonal degeneration have been described in patients with regional enteritis. We performed an immunohistochemical survey of forty cases of regional enteritis, ulcerative colitis, nonspecific colitis, and normal colon. A panel of antibodies, directed against neuron-specific enolase, S-100 protein, synaptophysin, neurofilament protein, and nerve growth factor receptor, was utilized to evaluate the distribution of nerve fibers in paraffin-embedded tissue. Anti-synaptophysin and anti-nerve growth factor receptor highlighted small, arborizing nerve fibers in the mucosa, not apparent in the routinely stained sections. Intense staining of these fibers was observed in regional enteritis with antinerve growth factor receptor. This antibody may aid the discrimination of inflammatory bowel disease from other causes of colonic inflammation and facilitate the identification of regional enteritis in endoscopic biopsies.

The value of the physical examination in the diagnosis of anemia. Correlation of the physical findings and the hemoglobin concentration.

Certain clinical findings, such as pallor of the conjunctivae, nail beds, lips, oral mucosa, and palmar creases have traditionally been used by physicians in the diagnosis of anemia. We prospectively examined 50 patients to determine whether there was any correlation between these clinical findings and hemoglobin concentration. We noted a statistically significant correlation between hemoglobin concentration and the following: color tint of the lower eyelid conjunctiva, nail-bed rubor, nail-bed blanching, and palmar crease rubor. Results from our study support the contention that the presence and degree of anemia can be estimated clinically by careful physical examination.