Kimura Disease: A Rare and Difficult to Diagnose Entity.

Kimura disease (KD) is a rare inflammatory disorder which involves the head and neck. Due to its rarity and various findings, definitive diagnosis can be difficult to ascertain. Kimura disease is distinguished from other conditions, including angiolymphoid hyperplasia, by histopathological features including follicular hyperplasia, reactive germinal centers, abundant eosinophilia, eosinophilic microabscesses, preserved nodal architecture, Warthin-Finkeldy polykaryocytes, and capsular fibrosis. Herein, we describe the clinical presentation, pathology, and diagnosis of a single case of a 39-year-old treated at an academic center in Texas.

Multifocal sinonasal inverted papilloma with middle ear involvement.

Inverted papilloma of the nasal cavity is a benign neoplasm, although it can be locally invasive and has the potential for malignant degeneration. Inverted papilloma of the temporal bone is extremely rare. We describe a case of a 44-year-old woman who was treated for nasal inverted papilloma and was later found to have inverted papilloma of her temporal bone. The patient required several procedures to remove the inverted papilloma from the nasal cavity and temporal bone, and she is currently free of recurrence.

Marginal zone B cell lymphomas with extensive plasmacytic differentiation are neoplasms of precursor plasma cells.

Occasional marginal zone lymphomas have extensive plasmacytic differentiation (P-MZL). We present an argument based on our data and previous published observations that P-MZL represent neoplasms of precursor plasma cells. Five P-MZL were analyzed by flow cytometry and the phenotype was compared with conventional MZL, plasma cell myeloma (PCM), reactive plasmacytoses (RP), and normal marrow plasma cells. The clonal cells in four of five P-MZL were CD19+, CD20(dim/⁻), CD38(bright), CD45++, CD56⁻, CD138⁻, and surface light chain(dim/⁻). One case was CD56+, CD138+, and CD19⁻. A separate clonal mature B cell population [CD20+, CD79b+] was not detected in any of these tumors. Hierarchical clustering demonstrated similarity between the neoplastic cells in four of the five P-MZL with plasma cells in RP comprised of precursor plasma cells. Analysis of normal bone marrow plasma cells revealed a CD45++CD38(bright)CD19+CD138⁻/+ precursor plasma cell population similar to the plasma cells in RP and P-MZL and a very small population of mature CD45+/⁻CD38(bright)CD138+CD19⁻ plasma cellsresembling the neoplastic cells in PCM. The findings suggest that P-MZL represents a malignancy of a plasma cell stage of differentiation distinct from the plasma cell stage corresponding to PCM. We propose the term precursor plasma cell neoplasm for these tumors.

Evidence for common clonal origin of multifocal lung cancers.

BACKGROUND: Lung cancer is the most common cause of cancer death in the United States. Multiple anatomically separate but histologically similar lung tumors are often found in the same patient. The clonal origin of multiple lung tumors is uncertain. METHODS: We analyzed 70 lung tumors from 30 patients (23 females and seven males) who underwent surgical resection for lung epithelial tumors, of whom 26 had non-small cell carcinomas and four had carcinoid/atypical carcinoid tumors. All patients had multiple tumors (two to five) involving one or both lungs. Genomic DNA was extracted from paraffin-embedded tissue sections using laser capture microdissection and analyzed for loss of heterozygosity, TP53 mutations, and X-chromosome inactivation status. The percentage (95% confidence interval [CI]) of patients in whom there were concordant patterns of genetic alteration was calculated. RESULTS: All 30 case subjects showed loss of heterozygosity (LOH) in at least one and at most four of the six polymorphic microsatellite markers. Completely concordant LOH patterns between synchronous and metachronous cancers in individual patients were seen in 26 (87%) of 30 informative patients (95% CI = 75% to 99%). Identical point mutations were present in eight of 10 patients who exhibited TP53 mutation by sequencing. Tumors in 18 (78%) of 23 female patients (95% CI = 67% to 98%) showed identical X-chromosome inactivation patterns. Combining the results of LOH studies, TP53 mutation screening analyses, and X-chromosome inactivation data, we demonstrated that the multiple separate tumors in 23 (77%) of 30 patients (95% CI = 62% to 92%) had identical genetic changes, consistent with monoclonal origin of the separate tumors. CONCLUSIONS: Our data indicate that the great majority of multifocal lung cancers have a common clonal origin and that multifocality in lung cancer represents local and regional intrapulmonary metastasis.