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病理报告黏膜相关边缘带B细胞淋巴瘤/malt淋巴瘤
就诊医院中山大学附属肿瘤医院
目前状态康复0-1年
最后登录2024-10-28
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发表于 2023-10-28 13:18:17
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@橙色雨丝 @战斗先生
时间好快,距离去年来这里已有一年之久。首先衷心祝各位老师别来无恙身体健康万事顺意。
在去年病理结论不一致以后,我2022年10月重新做了PET-CT,显示左颈部淋巴结大部分消失并无摄取值,胸腺肿物尺寸变小摄取值从19降为7。而后在中肿再做会诊,结论是免疫性非典型性增生,密切随访。
2023年8月一年复查CT,胸腺肿物尺寸又变大了,左侧脖子淋巴较2022年9年增多增大,直到现在左锁骨可以摸到浅表淋巴结。我昨天又取了脖子淋巴结做活检,3个,0.5—1cm大小。目前在忍痛等结论。
我们通过医院申请了香港陈GuoZhang教授做会诊,包括把2015年胸腺手术组织、2022年淋巴结、胸腺穿刺全部送过去,刚刚收到陈教授的报告。还请大神们@橙色雨丝 @战斗先生 帮忙看看给点意见。
2015 mediastinum (thymus) excision/biopsy specimen 1512115
The thymus excision specimen shows features typical of thymic extranodal marginal zone lymphoma (MALT lymphoma). The extensive cystic transformation of the thymic epithelium, dense infiltration of small lymphoid cells and plasma cells, prominent lymphoepithelial lesions and presence of pale monocytoid cells in the vicinity of the epithelium are all characteirstic. [Unstained paraffin sections are not available to further characterize the nature of the neoplastic cells, such as IgA production.]
Comment: Thymic MALT lymphomas most typically / frequently occurs in patients with Sjogren syndrome, which this patient did have. This is a highly indolent tumor with excellent prognosis. The tumor most commonly secretes IgA, and IgA paraproteinemia may be present in some patients. (We do not have unstained paraffin sections for immunostaining to prove that the neoplastic plasma cells secrete IgA and are monotypic.).
2022 mediastinal core biopsy 2214844
The biopsy shows strips of tissue densely infiltrated by small lymphoid cells, monocytoid cells and plasma cells. There are bands of interspersed cytokeratin+ epithelium (thymic epithelium), and lymphoepithelial lesions are present. There are moderate numbers of CD20+ B cells, and many CD3+ T cells are present. We have performed immunostaining for immunoglobulins; the neoplastic cells express IgA, and exhibit kappa light chain restriction. Thus the mediastinal biopsy shows persistence or recurrence of thymic extranodal marginal zone lymphoma.
Comment: It is unclear from the clinical records whether the 2015 thymic excision was a complete excision; so the 2022 biopsy may represent either residual or recurrent thymic MALT lymphoma. This time, we manage to demonstrate that this neoplasm is IgA-kappa positive, which can help us further track the nature of other biopsies. NGS studies on this sample (1DNA) shows mutations in several genes, including EP300, which is known to be implicated in some MALT lymphomas.
2022 neck lymph node needle biopsy 2214362
The lymph node core biopsy shows strips of tissue populated mostly by small lymphoid cells, plasma cells and occasional large cells. The morphology is rather nonspecific. Immunostaining shows that there are not many CD20+ B cells, but moderate numbers of CD3+ T cells. While the findings are nonspecific (reactive versus neoplastic), we have nonetheless performed IgA, Kappa and lambda immunostains, and can convincingly demonstrate presence of IgA-kappa-restricted cells, indicating the presence of subtle involvement by the extranodal marginal zone lymphoma.
Comment: The demonstration by NGS of similar genetic alterations (specimen 2DNA) in comparison with the mediastinal biopsy further supports involvement of the lymph node by the same tumor, albeit with a lower allelic frequency (suggesting presence of tumor in a lower percentage in this specimen).
2022 neck lymph node excision 948691
The lymph node shows distorted but still preserved architecture – sinuses are intact and often distended by histiocytes and/or lymphoid cells. Lymphoid follicles are inconspicuous. The paracortex is expanded, with a spectrum of lymphoid cells (small and large) that do not show frank nuclear atypia; there are also focal collections of monocytoid cells. Scattered histiocytes and epithelioid histiocytes are present. Immunostaining shows nodular aggregates of CD20+ B cells plus small numbers of CD20+ cells in between. Many CD3+ cells are present, including small and large cells; and they represent a mixture of CD4+ and CD8+ cells. We have performed IgA, Kappa and lambda immunostains, and can demonstrate a minor population of IgA-kappa-restricted cells, indicating the presence of subtle involvement by the extranodal marginal zone lymphoma (even more subtle than in the neck lymph node needle biopsy). I believe the background lymph node shows reactive changes, presumably a reaction to the lymphoma or a manifestation of the underlying autoimmune disease (Sjogren syndrome).
Comment: NGS studies (3DNA) show similar genetic mutations, at a very low tumor allelic frequency, suggesting that the thymic MALT lymphoma cells are present as a minor population – either the disease is regressing or there is a sampling error or the excised lymph node is not the same as the biopsied lymph node. I note in one of the reports that clonal TCRG rearrangement is demonstrated in this specimen; I cannot fully explain this – there might be minor T-cell clones in the background of lymphoid hyperplasia. |
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