The American journal of surgical pathology
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Am. J. Surg. Pathol. · Mar 2010
Comparative StudyB-cell lymphomas with concurrent IGH-BCL2 and MYC rearrangements are aggressive neoplasms with clinical and pathologic features distinct from Burkitt lymphoma and diffuse large B-cell lymphoma.
B-cell lymphomas with concurrent IGH-BCL2 and MYC rearrangements, also known as "double-hit" lymphomas (DHL), are rare neoplasms characterized by highly aggressive clinical behavior, complex karyotypes, and a spectrum of pathologic features overlapping with Burkitt lymphoma (BL), diffuse large B-cell lymphoma (DLBCL) and B-lymphoblastic lymphoma/leukemia (B-LBL). The clinical and pathologic spectrum of this rare entity, including comparison to other high-grade B-cell neoplasms, has not been well defined. We conducted a retrospective analysis of clinical and pathologic features of 20 cases of DHL seen at our institution during a 5-year period. ⋯ DHL is a high-grade B-cell neoplasm with a poor prognosis, resistance to multiagent chemotherapy, and clinical and pathologic features distinct from other high-grade B-cell neoplasms. Familiarity with the morphologic and immunophenotypic spectrum of DHL is important in directing testing to detect concurrent IGH-BCL2 and MYC rearrangements when a karyotype is unavailable. The aggressive clinical behavior and combination of genetic abnormalities seen in these cases may warrant categorization as a separate entity in future classifications and call for novel therapeutic approaches.
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Am. J. Surg. Pathol. · Dec 2009
Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4.
The possible involvement of immunoglobulin G4 (IgG4) in the pathogenesis of idiopathic sclerosing lesions has been suggested. In this study, a clinicopathologic analysis was performed to reveal characteristics of retroperitoneal fibrosis relating to IgG4. The study involved 17 patients with retroperitoneal fibrosis. ⋯ The only major clinical difference was the striking male predominance in IgG4-related cases. In conclusion, this study revealed that retroperitoneal fibrosis could be classified as IgG4-related or not. This distinction seems important to help better characterize the biology/pathogenesis of both groups and better predict the possibility of other IgG4-related processes at other anatomic sites.
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Am. J. Surg. Pathol. · Dec 2009
Pathologic patterns and survival in chronic hypersensitivity pneumonitis.
The objective of the study was to examine the relationship of pathologic pattern and prognosis in hypersensitivity pneumonitis (HP). We analyzed 24 cases of subacute (cellular, nonfibrotic) and 25 cases of chronic (fibrotic) HP. Nineteen (79%) of the subacute cases showed a pattern of bronchiolocentric interstitial pneumonia and 5 (21%) a pattern mimicking cellular nonspecific interstitial pneumonia (NSIP). ⋯ Patients with a pattern of only peribronchiolar fibrosis had a median survival of 11.3 years. These data confirm that the presence of fibrosis is associated with a generally poor prognosis in patients with HP, and suggest that pure peribronchiolar fibrosis may portend a longer survival than does a UIP-like or fibrotic NSIP-like pattern of fibrosis. Most cases of chronic HP have distinctive pathologic features, but a small percentage of cases cannot be pathologically distinguished from UIP.
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Am. J. Surg. Pathol. · Nov 2009
Papillary squamous cell carcinoma of the head and neck: frequent association with human papillomavirus infection and invasive carcinoma.
Papillary squamous cell carcinoma (SCC) is an uncommon variant of SCC in the upper aerodigestive tract. It is most frequently located in the larynx, oropharynx, and sinonasal tract, and is more common in older men. Because of its complex exophytic papillary architecture, histologic assessment of underlying invasion can be challenging. ⋯ In our study, more than two-thirds of papillary SCCs in the upper aerodigestive tract were immunoreactive with antibody to p16 and 68% of those lesions had identifiable high-risk HPV by in situ hybridization. As with other HPV-associated SCCs of the upper aerodigestive tract, the majority of HPV-associated papillary SCCs are oropharyngeal (base of tongue and palatine tonsils), although both sinonasal and laryngeal tumors were also associated with infection (67% and 33% of cases, respectively). Given the better prognosis of HPV-associated SCCs of the upper aerodigestive tract, it may be prudent to report the p16 and HPV status of these tumors when they are encountered.