Vascular lesions of the sinonasal tract are rare. These lesions do not have typical signs or symptoms. They may present insidiously with minimal symptoms. A high index of suspicion and a good preoperative evaluation are needed for diagnosis. No standard surgical approach is indicated. We report a case of cavernous hemangioma of the maxillary sinus in an adult male. We present the diagnostic work-up and discuss the differential diagnosis and potential therapeutic approaches.
Waldenström macroglobulinemia (WM) is currently defined as lymphoplasmacytic lymphoma involving bone marrow (BM) associated with a serum IgM paraprotein. WM is typically composed of small lymphocytes, plasmacytoid lymphocytes, and plasma cells in variable proportions, which can change after therapy. In this study, we assessed 41 WM cases that required chemotherapy, 39 showing persistent disease in restaging BM specimens. In 10 cases, there was persistent monotypic plasmacytosis in BM in the absence of demonstrable monotypic B cells. The monotypic plasma cells represented 0.5% to 46% of the cellularity and persisted 1 to 50 months after the last course of chemotherapy. The plasma cells were best quantified by immunohistochemical analysis on paraffin sections. We conclude that WM can persist as a pure plasma cell population after therapy. This finding has implications for the immunophenotypic assessment of WM after therapy and may explain persistent IgM paraproteinemia in patients with WM with no evidence of a clonal B-lymphocyte population.
These findings are similar to most published data. FS is a reliable method for evaluating SLN. The most common cause of false negative diagnosis is sampling error. More attention should be paid to low-grade tumors. Moreover, in FS analysis, we recommend to totally submit SLNs that are less than 5 mm in diameter, bisecting them if possible, and to serially section SLNs that are at least 5 mm at 2-mm intervals.
Context.—Nucleophosmin 1 (NPM1) is the most commonly mutated gene in acute myeloid leukemia. Detection of NPM1 mutations is useful for stratifying patients for therapy, predicting prognosis, and assessing for minimal residual disease. Several methods have been developed to rapidly detect NPM1 mutations in genomic DNA and/or messenger RNA specimens. Objective.—To directly compare a quantitative real-time polymerase chain reaction (qPCR) assay with a widely used capillary electrophoresis assay for detecting NPM1 mutations. Design.—We adopted and modified a qPCR assay designed to detect the 6 most common NPM1 mutations and performed the assay in parallel with capillary electrophoresis assay in 207 bone marrow aspirate or peripheral blood samples from patients with a range of hematolymphoid neoplasms. Results.—The qPCR assay demonstrated a higher analytical sensitivity than the capillary electrophoresis 1/1000 versus 1/40, respectively. The capillary electrophoresis assay generated 10 equivocal results that needed to be repeated, whereas the qPCR assay generated only 1 equivocal result. After test conditions were optimized, the qPCR and capillary electrophoresis methods produced 100% concordant results, 85 positive and 122 negative. Conclusions.—Given the higher analytical sensitivity and specificity of the qPCR assay, that assay is less likely to generate equivocal results than the capillary electrophoresis assay. Moreover, the qPCR assay is quantitative, faster, cheaper, less prone to contamination, and well suited for monitoring minimal residual disease.
Background: Thymolipoma is a rare benign thymic tumor that arises in the anterior mediastinum. It presents with non-specific symptoms such as chest pain and dyspnea due to compression of the tumor on surrounding structures. In addition, this tumor is associated with paraneoplastic syndromes, including myasthenia gravis. Such a relationship is still not understood and requires investigation. Objective: Investigate the significance of myasthenia gravis in thymolipoma patients. Methods: We present a series of 16 thymolipoma cases from multiple medical centers. Data extraction included demographic, diagnostic, radiological and laboratory, and clinical outcome variables. We also used the modified Osserman score to assess the severity of myasthenia gravis. Results: Ten patients were males, and six were females; the sample mean age was 39.9 years (SD = 16.7). Upon presentation, 7/16 patients were asymptomatic, 4/16 patients had chest pain, and 2/16 had dyspnea. Seven patients were having myasthenia gravis; they experience generalized weakness (3/7), diplopia (2/7), ptosis (1/7), and bulbar weakness (1/7). Serum acetylcholine receptor antibody (AChRAb) was positive in 4 patients. All patients underwent thymectomy either via sternotomy or thoracotomy. After proper follow-up, only 2/7 of myasthenic patients had a complete remission of symptoms, no tumor recurrence was observed. Conclusion: Although the effect of using steroids in myasthenic patients on thymolipomic transformation still needs confirmation, diagnosis of thymolipoma should be kept in mind in myasthenic patients presenting with relevant symptoms and taking steroids. Furthermore, complete remission of myasthenic symptoms after removal of thymolipoma needs further investigation.
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