We report nine cases of micronodular thymoma with lymphoid B-cell hyperplasia and one case of micronodular thymic carcinoma with lymphoid hyperplasia from our institution. For a better understanding of these rare tumors, clinical records, and histological features of these cases were reviewed, with detailed review of additional 64 literature cases of micronodular thymic neoplasms. The joint analysis identified 64 cases of micronodular thymoma with lymphoid B-cell hyperplasia and 9 cases of micronodular thymic carcinoma with lymphoid hyperplasia. Both groups revealed slight male predilection, with male:female ratio of 1.3:1 and 5:4, and occurred at 440 years of age, with a mean of 64 (41-83) and 62 (42-78) years, respectively. Myasthenia gravis was noted in 3/64 (5%) and 1/9 (11%) patients, respectively. Other systemic, disimmune, or hematologic disorders were noted in 6/64 (9%) and 1/9 (11%) patients, respectively. Components of conventional thymoma were reported in 11/64 (17%) micronodular thymomas with lymphoid B-cell hyperplasia, with transitional morphology between the two components in most of them. Cellular morphology was predominantly spindle in micronodular thymoma with lymphoid B-cell hyperplasia when specified (30/43), and epithelioid in micronodular thymic carcinoma with lymphoid hyperplasia (6/9), and cytological atypia was more encountered in the latter. Dedifferentiation/transformation from micronodular thymoma with lymphoid B-cell hyperplasia to micronodular thymic carcinoma with lymphoid hyperplasia seems to occur in a small subset of cases. Three cases of micronodular thymomas with lymphoid B-cell hyperplasia were described with co-existent low-grade B-cell lymphomas. Follow-up data were available for 30 micronodular thymomas with lymphoid B-cell hyperplasia and 6 micronodular thymic carcinomas with lymphoid hyperplasia, with a mean of 47 (0.2-180) months and 23 (3-39) months, respectively. Patients were alive without disease, except for five micronodular thymoma with lymphoid B-cell hyperplasia patients (dead from unrelated causes), and one micronodular thymic carcinoma with lymphoid hyperplasia patient (dead of disease).
The risk scoring systems proposed by Tapias et al. and Demicco et al. were both predictive of OS and PFS. The Demicco system has the advantages of simplicity and applicability to SFTs from other sites, as well as provision of the best discrimination for PFS, and thus may be the best system to apply in general practice.
Myoepithelial carcinoma (MECA) is an underrecognized challenging entity with a broad morphologic spectrum. Misinterpreting MECA is not uncommon as distinguishing it from its mimics, especially cellular myoepithelial-rich pleomorphic adenoma (PA), can be difficult. We described 21 histologically challenging cases of MECAs (16 MECA ex-PA and 5 MECA de novo). All MECAs ex-PA were intracapsular or minimally invasive except for 3 cases. Eighteen (86%) were initially misinterpreted as benign neoplasms, including PA (10), atypical PA (5), and myoepithelioma (3). The remaining 3 were initially diagnosed as malignant (MECA ex-PA) but were histologically challenging. Histologic features that were found most helpful in recognizing the malignant nature of MECA included: uniformly cellular myoepithelial proliferation with an expansile nodular lobulated pattern (all cases) and alternate hypocellular and hypercellular zonal distribution (76% of cases). Among the 16 MECA patients with follow-up, 14 (87.5%) progressed: 10 developed local recurrence and 5 distant metastases. In contrast, only one of 33 patients with cellular PA (control group) recurred locally. Ten of the 14 MECAs that progressed were MECA ex-PA, and 12 (85%) had an initial benign diagnosis. Two patients with MECA ex-PA died of their disease; one had an initial diagnosis of PA. MECA is a histologically challenging entity that closely mimics PA and seems to carry a significant risk of recurrence. Areas of clonal appearing cellular myoepithelial growth with an expansile nodular lobulated pattern and zonal cellular distribution distinguish the majority of MECAs and may serve as useful diagnostic histologic features to differentiate MECA from its benign mimics.
The differential diagnosis in cellular effusions with cytological atypia often includes malignant mesothelioma (MM), reactive mesothelial proliferation, and malignancies of metastatic origin, particularly carcinomas. The International Reporting System for Serous Fluid recently established guidelines for reporting MM. In conjunction with the cytomorphologic evaluation, the role of immunochemistry (IC) was emphasized as a very useful tool in the workup of serous fluids, especially with the availability of novel markers. Utilizing a panel of markers, IC allows the characterization of the cells, whether mesothelial or not, and when mesothelial origin is established, IC can frequently assist in delineating its benign or malignant nature. IC can also confirm metastatic disease, allowing the identification of the primary origin in most cases. This review summarizes the current status of IC and its role in the diagnosis of MM and its differential diagnosis in serous fluids.
The transgastric approach is a novel method for obtaining liver biopsies in patients undergoing upper gastrointestinal endosonography. Avoidance of vascular puncture and ability to acquire tissue in patients with obesity or ascites offers a practice niche for this technique. Although several series have reported on specimen adequacy, biopsy core length, and number of portal tracts, none has addressed the diagnostic challenges presented by the fragmented nature of these specimens. We systematically evaluated 113 transgastric liver biopsies obtained for diagnosis of parenchymal liver disease by 3 needle types and compared them with 100 percutaneous and 100 transjugular liver biopsies, respectively. Parameters recorded were number of tissue cores, sizes of longest and shortest cores, numbers of complete and incomplete portal tracts, morphologic characteristics, and adequacy of specimen for diagnosis and staging. In contrast to percutaneous and transjugular liver biopsies, transgastric biopsies often contained>10 tissue fragments and smaller tissue cores. In addition, 2 of the 3 types of transgastric needles obtained less numbers of complete portal tracts. Transjugular biopsies were also smaller and contained less number of complete portal tracts than percutaneous specimens but, unlike transgastric biopsies, only rarely contained >10 tissue fragments. Specimen adequacy for diagnosis and staging was 80%, 100%, and 98% for transgastric, percutaneous, and transjugular biopsies, respectively. Difference in specimen adequacy is related to tissue fragmentation of transgastric liver biopsies rather than biopsy core length or numbers of complete portal tracts. Tissue fragmentation is particularly challenging for staging chronic liver disease.
Spinal primary dural lymphoma (PDL) is uncommon with a total of 37 previous well-documented cases reported, including one diagnosed in the authors' institution. More recently we encountered an additional case of spinal PDL that, similarly to our previous case, was grade 1-2 follicular B-cell PDL. Our two cases were diagnosed over a 3-year interval in a 72-year-old female and a 74-year-old male, respectively. An exhaustive literature review on PDL was performed consequently to reveal that: (i) spinal and cerebral sites of involvement by PDL are constantly mutually exclusive; and (ii) unlike cerebral PDL, which is usually of marginal zone B-cell type, only two of the 38 cases of spinal PDL were diagnosed as such, diffuse large B-cell lymphoma being the most commonly encountered type in the spine. This divergence infers that, in contrast to the prevailing concept that PDL is a unique disease group, PDL appears to be rather heterogeneous with a difference in predilection of lymphoma type for the anatomical site of dural involvement. Such a site-specific lymphoma-type predilection phenomenon, well-recognized in other organ systems, has not been acknowledged in PDL. This report brings new insights into PDL, and may contribute to a better understanding of nervous system pathophysiology and lymphoma classification.
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