112例非霍奇金恶性淋巴瘤的临床特征

Objective:To study clinical characteristics of non-Hodgkin's lymphoma (NHL) in order to diagnose and treat eady and improve prognosis.Methods:112 cases with NHL were pathologically diagnosed and treated in 1992-2005.Results:Among all cases,30 were not treated previously,79 were involved in peripheral lymph nodes,55 deep lymph nodes,18 Waldeyer's ring,25 respiratory system,26 digestive system,26 spleen,13 bone,13 skin and subcutaneous,4 urinogenital system,13 other sites.Among 30 previously untreated patients,there were 4 staged Ⅰ,3 ⅠE,5Ⅱ,1 ⅡE,9 Ⅲ,1 Ⅲs and 7 Ⅳ.Among 82 patients with prior treatment,29 were involved in lymph nodes only,53 involved in different organs and tissues.In our series,there were 38 cases and 74 cases belonged to subtype A and subtype B respectively.Conclusion:The enlargement of peripheral lymph nodes is main clinical characteristic in NHL,but lesions also involve different organs and tissues.The diagnosis of NHL depends upon pathological examination.     

骨朗格汉斯细胞组织细胞增生症25例临床分析

 目的　分析骨朗格汉斯细胞组织细胞增生症（LCH）的临床特点,总结LCH诊断和治疗的方法。方法　回顾性分析2004年2月至2012年2月收治的25例经病理证实的LCH患者的临床资料及随访结果。结果　25例患者中男18例,女7例;中位年龄17岁。单发病灶17例,其中颅骨11例,多发病灶6例。首发症状多为疼痛和局部肿块,全身症状少见。主要症状多为局部疼痛,影像学表现为溶骨性改变,12例伴周围软组织肿胀。病理表现为分化好的组织细胞增生及大量嗜酸性粒细胞浸润,CD1a、S100、Vimentin、CD68免疫组织化学阳性率高。单发病例采用手术治疗为主,辅以放疗或化疗。多发病例以化疗为主,辅以放疗。仅累及骨的患者疗效满意,累及其他脏器的2例患者死亡。结论　骨LCH男性发病明显高于女性,好发于儿童和青少年,以单发病灶为多,颅骨侵犯多见。临床表现主要为局部疼痛和肿块,病理活组织检查是确诊的首选方法,治疗宜采用综合疗法。预后与骨病损范围和病理类型以及其他脏器受累情况,大部分病例预后好。     

Malignant histiocytosis (histiocytic medullary reticulosis). I. Clinicopatholigic study of 29 cases.

The clinical records and histologic material from 29 cases of malignant histiocytosis (MH) have been reviewed, as well as autopsy findings in 14 cases. The mean age was 31 years, with a 2.2:1 male to female preponderance. Major physical findings included temperature elevation, lymphadenopathy, hepatomegaly, splenomegaly, and preterminal jaundice. Common laboratory findings were anemia, leukopenia, and thrombocytopenia. The median survival was 6 months, the mean 14 months, and the range from 1 month to 8 years. The histologic features observed in lymph nodes, liver, spleen, and bone marrow have been emphasized, as well as features useful in differential diagnosis. In contrast to our experience with the non-Hodgkin's lymphomas, bone marrow aspiration was superior to biopsy in assessing marrow involvement. Unusual manifestations included soft tissue infiltration in 5 cases; 2 of these patients presented with a soft tissue mass. The distinctive clinical as well as histologic findings warrant recognition and separation of MH from other hematopoietic disorders.     

Multicentric giant lymph node hyperplasia. A report of seven cases

Multicentric giant lymph node hyperplasia (MGLH) is a distinct lymphoproliferative disorder, which may terminate in malignant lymphoma. The clinical features and laboratory findings of seven cases are reported here. The histologic changes in lymph nodes were those of giant lymph node hyperplasia, plasma cell type. Immunoperoxidase staining showed intracytoplasmatic polyclonal immunoglobulins. Malignant lymphoma supervened in two cases, one of which was proved by autopsy; in the other there was transformation of a polyclonal gammopathy into a monoclonal one. Two of the patients also developed Kaposi's sarcoma.     

癌瘤转移规律的探讨

在 4 0 0例恶性肿瘤尸解中 ,32 1例 (80 3% )癌 ,79例肉瘤 ,其中 6 5例淋巴瘤 ,14例 (3 5 % )为软组织及骨肿瘤 ,肿瘤转移到肝及肺最常见。有 16 3例转移到肺及肝 ,各占 4 0 5 %。肝的转移瘤依次主要来自乳腺、大肠、卵巢、胃及非何杰金淋巴瘤 (NHL)。肺的转移瘤主要来自乳腺、肝、NHL ,胃及卵巢。淋巴结转移主要累及颈部、纵隔及主动脉周围淋巴结。广泛转移的肿瘤是肺癌、胃癌、乳腺癌和淋巴瘤。尸检材料显示 ,宫颈癌、膀胱癌、咽癌及睾丸肿瘤主要是局部侵犯 ,转移并不广泛。本研究显示 ,恶性肿瘤的扩散与转移基本有下列方式 :直接侵袭 :很多肿瘤可能直接累及周围组织。例如胃肠癌 ,癌细胞沿着肌组织间隙进入临近器官。淋巴管扩散 :任何器官或组织的肿瘤细胞可能进入淋巴管而转移到局部或远处淋巴结 ,它是癌的主要转移方式。血道转移 :远处转移在尸检材料中很常见 ,最常累及的器官是肝和肺 ,特别是软组织肉瘤 ,但晚期癌也很常见血道转移。种植性转移 :这也是很常见的转移方式 ,特别是胃癌、大肠癌、卵巢癌等。本研究也显示与癌瘤手术标本对比研究、肿瘤转移与临床分期、肿瘤部位、组织学类型及分化程度等有密切关系     

朗格汉斯细胞组织细胞增生症3例临床分析

目的探讨朗格汉斯细胞组织增生症的临床特点及治疗方法。方法回顾分析3例朗格汉斯细胞组织细胞增生症并复习相关文献。结果 2例患者以头部肿物为首发症状,手术切除病灶后经过化疗及免疫治疗存活至今,另1例患者经过颈椎穿刺活检确诊,未行化疗,死于并发症。结论朗格汉斯细胞组织细胞增生症是一组原因不明的罕见疾病,其病理基础是单核巨噬细胞系统中朗格汉斯细胞增殖,由于病变年龄、细胞增生程度、累及组织和器官的不同,其症状表现不一样,预后也不一样。     

Hodgkin's disease developing in a patient with angioimmunoblastic lymphadenopathy with dysproteinemia--a case report

The case of a woman presenting the clinical and pathologic phenomena of angioimmunoblastic lymphadenopathy (AILD) with dysproteinemia is reported. The patient developed lesions in the lymph nodes, skin, lungs, liver and spleen, and her response to steroid and cyclophosphamide therapy was poor. At autopsy, microscopic findings in the mediastinal and abdominal lymph nodes were consistent with the diagnosis of Hodgkin's disease. Whereas the development of immunoblastic lymphoma is frequent in AILD, Hodgkin's disease is far less common. It is argued that malignant lymphoma in AILD may be the consequence of chronically depressed lymphocyte functions.     

2-(fluorine-18)fluoro-2-deoxy-D-glucose positron emission tomography in the detection and staging of malignant lymphoma. A bicenter trial

BACKGROUND: The authors undertook a prospective evaluation of the clinical value of 2-fluoro [18-]-2-deoxyglucose positron emission tomography (FDG-PET) in the detection and staging of malignant lymphoma compared with computed tomography (CT) and bone marrow biopsy (BMB). METHODS: Fifty-two consecutive patients with untreated malignant lymphoma were evaluated prospectively in a bicenter study. FDG-PET, CT, and BMB were performed for investigating lymph node/extranodal manifestations and bone marrow infiltration. Thirty-three percnt of the discrepant results were verified by biopsy, magnetic resonance imaging, or clinical follow-up (range, 4-24 month). RESULTS: Altogether, 1297 anatomic regions (lymph nodes, organs, and bone marrow) were evaluated. FDG-PET and CT scans were compared by receiver operating characteristic (ROC) curve analysis. The area under the ROC curve were as follows: lymph nodes, 0.996 (PET) and 0.916 (CT); extranodal, 0.999 (PET) and 0.916 (CT); supradiaphragmatic, 0.996 (PET) and 0.905 (CT); and infradiaphragmatic, 0.999 (PET) and 0.952 (CT). In these analyses, FDG-PET was significantly superior to CT (P < 0.05), except in infradiaphragmatic regions, in which the two methods produced equivalent results. In detecting bone marrow infiltration, FDG-PET was superior to CT and was equivalent to BMB. In 4 of 52 patients (8%), FDG-PET led to an upstaging and a change of therapy. CONCLUSIONS: Noninvasive FDG-PET is very accurate in the staging of malignant lymphoma. Compared with standard staging modalities (CT and BMB), PET was significantly superior and led to changes in the therapy regimen for 8% of patients.     

A case report of multicentric Castleman's disease with simultaneous Epstein-Barr virus infection.

Castleman's disease is a rare B-cell lymphoproliferative disorder of unknown etiology. In this report we describe a 54-year-old woman with a 10-year history of asymptomatic bilateral, multiple cervical lymph node enlargements. She was not evaluated by lymph node biopsy during this period. She had been well until four months previously. The patient presented with multiple enlarged lymph nodes and systemic symptoms including fever, sweats, weight loss, and anorexia. Two lymph nodes were biopsied, yielding a diagnosis of multicentric Castleman's disease (MCCD) of mixed hyaline-vascular and plasma cell type histology. Serologic studies revealed the simultaneous presence of an acute Epstein-Barr virus (EBV) infection. She experienced an aggressive clinical course with a fatal outcome.     

趋化因子受体4和间质细胞衍生因子1α与鼻咽癌器官特异性转移的相关性研究

目的 研究趋化因子受体4(CXCR4)在鼻咽癌细胞中的表达,间质细胞衍牛因子1α(SDF-1α)在鼻咽癌远处靶器官中的表达,探讨CXCR4和(或)SDF-1α在鼻咽癌器官特异性转移中的作用.方法 应用逆转录聚合酶链反应(RT-PCR)和免疫组织化学法分析30例鼻咽癌、15例正常鼻咽组织中CXCR4 mRNA和蛋白的表达及其同临床病理学因素之间的相关性,应用免疫组织化学法分析鼻咽癌患者的正常颈部淋巴结(包括颈深上和颈深下淋巴结)、骨髓、肺、肝脏和肾脏、结肠(各5例)中SDF-1α蛋白的表达.结果 RT-PCR检测结果显示,鼻咽癌组织中CXCR4 mRNA相对表达强度(0.71±0.22)显著高于正常鼻咽组织(0.14±0.07;F=27.94,P<0.05);免疫组织化学检测结果显示,鼻咽癌组织中CXCR4蛋白的表达(1.58±0.59)显著高于正常鼻咽组织(0.51±0.22;F=17.75,P<0.05).鼻咽癌组织中CXCR4 mRNA和蛋白的表达与临床分期、淋巴结转移、细胞分化程度显著相关(均P<0.05).SDF-1α蛋白在鼻咽癌患者的颈深上淋巴结、骨髓、肺、肝脏中表达较高(2.35±0.67),而在颈深下淋巴结、肾脏和结肠中表达较弱(0.68±0.23),差异有统计学意义(t=10.13,P<0.01).结论 CXCR4的表达与鼻咽癌的转移密切相关,CXCR4和(或)SDF-1α在鼻咽癌器官特异性转移中可能发挥重要作用.     

转移灶穿刺活检在肺癌诊断中的应用

目的:探讨肺部病灶活检难度大或者肺部活检组织较少等情况下,转移灶穿刺活检的意义。方法:回顾性分析了自2013年10月至2016年12月于南京大学医学院附属鼓楼医院肿瘤中心接受淋巴结、骨、肝脏等转移灶穿刺活检的20例肺癌患者。对转移灶活检的必要性、转移灶活检的手段、准确性、标本质量、病理诊断及相关副作用进行分析。结果:从2013年10月至2016年12月,共20例患者于肿瘤中心行转移灶穿刺,其中行肾上腺转移灶穿刺1例、骨转移灶穿刺3例、肝脏穿刺4例、皮下包块穿刺5例、淋巴结穿刺7例,结合临床及免疫组化均考虑肺来源（其中肺腺癌13例、肺鳞癌2例、肺小细胞癌4例、1例最终只能确定为肺低分化癌）。对非小细胞肺癌患者行分子检测,其中3例患者因穿刺组织较少难以行分子检测,余12例患者分子检测结果显示EGFR敏感突变阳性率75%（9/12）、ALK融合基因阳性率16.7%（2/12）。所有转移灶穿刺患者均未见严重出血、感染或针道种植转移等并发症。结论:对临床高度怀疑肺癌并且伴有远处转移的患者,在肺部病灶活检难度大或活检组织取材不能满足分子检测时,转移灶穿刺活检可作为重要补充手段,达到较好的诊断效能。     

Metastatic patterns of prostatic cancer. Correlation between sites and number of organs involved

Prostatic cancer in 1885 autopsy cases was classified according to the number of organs involved in metastasis, and a comparison was made concerning the frequency of metastasis to the various organs. The frequencies of metastasis to the lungs and para-aortic lymph nodes were low in cases with single-organ involvement (4.6% and 2.3%, respectively), but increased rectilinearly in accordance with the number of organs involved and became high in cases with metastasis to three or more organs (49.1% and 21.8% in total, respectively). On the other hand, the frequencies of local extension to the bladder and invasion of the pelvic lymph nodes were high even in cases with single-organ involvement (34.5% and 9.2%, respectively) and were not significantly changed regardless of the number of organs involved. No significant correlation was seen between pelvic and para-aortic lymph node involvement. In cases with single-organ involvement, metastasis to the lumbar spine occurred frequently, but those to the ribs, sternum, and ilium occurred less frequently. There may be multiple metastases in cases with metastases to the para-aortic lymph nodes, sternum, and ilium. The number of metastatically involved organs is useful in analyzing the mode of metastasis.     

以尿崩症为首发症状的多系统郎格汉斯细胞组织细胞增生症一例并文献复习

目的 探讨郎格汉斯细胞组织细胞增生症（LCH）的临床特点、诊断及治疗,进一步提高对LCH的认识.方法 回顾分析1例以中枢性尿崩症为首发症状的多系统LCH患者的临床症状、实验室检查、影像学检查及病理和免疫组织化学结果,并复习相关文献.结果 患者早期以中枢性尿崩症起病8年,数年间逐步发展为肋骨、肢体长骨多发损害,肝、脾、淋巴结侵犯,通过胫骨及淋巴结活组织检查示正常组织结构破坏,具有典型形态学特征的郎格汉斯细胞局灶性增生,该细胞核型不规则,扭曲、分叶,可见特征性的核沟纹（咖啡豆样核）,免疫组织化学示表达S-100、CD1a、Langerin,明确诊断为多系统LCH.结论 LCH临床表现及生物学行为呈明显的异质性,既可以表现为孤立的溶骨性损害（如嗜酸性肉芽肿）,也可表现为白血病样侵袭性的病程.LCH损害可累及全身所有器官,典型的LCH累及骨骼较为多见.以累及垂体前后叶并以中枢性尿崩症起病的多系统LCH症状较为隐匿,容易延误诊断,LCH导致的神经内分泌损害需要终生的激素替代治疗.     

Esophageal carcinoma with hypercalcemia that appeared to be caused by prostaglandin E2 produced by the tumor cells

Discussed is the case of a 50-year-old man with a well advanced esophageal carcinoma who, during his final clinical course, suddenly developed hypercalcemia (max: 15.0 mg/ml). His serum parathyroid hormone level, however, remained within normal limits. On autopsy, an extensive metastasis to many organs and lymph nodes was noted but no evidence of a bone metastasis. Nude mice bearing the same tumoral tissue were found, on autopsy, to have similarly developed hypercalcemia and cells that were cultured were found to produce an excessive amount of Prostaglandin E2 (PGE2). These findings suggest that this humoral hypercalcemia of malignancy (HHM) was caused by excessive PGE2 produced by the tumor cells, although other possible factors should be investigated.     

Transitional Cell Carcinoma of the Bladder Manifestating as Malignant Lymphoma with Generalized Lymphadenopathy

Bladder cancer usually spreads via the lymphatic and hematogenous routes, the most common sites of metastases of urinary bladder cancers being the regional lymph nodes, liver, lung, bone, peritoneum, pleura, kidney, adrenal gland and intestines. Generalized lymph node metastasis of transitional cell cancer of the bladder is extremely rare. According to our literature search, there has been no case report of transitional cell cancer of the bladder that manifests as an extensive large lymph node metastasis involving the intraparotid, supraclavicular thoracic inlet, axillary and regional abdominal and pelvic lymph nodes without bone or visceral organs involved. Such a presentation could be mistaken as malignant lymphoma and the importance of a biopsy of the lymph nodes is emphasized. The clinical course of rapid progression of the disease and the presence of wild-type p53 with rapid response to chemotherapy and a short remission may represent a unique case, which is discussed here.Key Words: Transitional cell bladder cancer, Malignant lymphoma, Generalized lymphadenopathy, Metastasis     

Extraosseus Plasmacytoma of the Pharynx with Localized Light Chain Deposition. Case Report

Light chain deposition disease (LCDD) is a rare disorder associated with a clonal proliferation of plasma cells, which synthesize abnormal monoclonal immunoglobulin light chains. It is characterized by systemic deposition of light chains in various organs, with the kidneys being most commonly affected. There have been few reports of isolated LCDD, i.e. in the brain, lungs and cervical lymph nodes. We here report on another patient with an isolated form of LCDD, which was limited to the pharyngeal mucosa and was associated with an extraosseus plasmacytoma of the pharynx, expanding the spectrum that has been recognized for LCDD. The patient was treated by local radiotherapy, with an excellent response. A less aggressive clinical course can probably be expected than in the usual form of LCDD, but a long-term follow-up is necessary to establish the clinical significance of this variant of LCDD.     

12例肾上腺淋巴瘤的影像学表现分析

目的探讨肾上腺淋巴瘤的影像学特点。方法回顾性分析12例初诊未治、有完整影像学资料及病理结果的肾上腺淋巴瘤患者的临床表现、病理类型及影像学表现。结果 12例均为非霍奇金淋巴瘤（2例原发性,10例继发性）。10例继发性病例中8例为双侧肾上腺受侵,2例为单侧受侵;8例表现为肾上腺肿物,2例为肾上腺增厚。8例边界清楚。4例伴有坏死或囊变。无伴有出血、钙化、脂肪者。增强扫描强化程度低于静脉期肝实质。8例伴多脏器受侵。9例伴淋巴结肿大,受侵淋巴结主要分布于腹腔及腹膜后。其中3例行PET-CT检查,肾上腺病变均伴有明显摄取增高,中位SUVmax为24.5。2例原发病例均为双侧肾上腺受侵,肿物密度混杂,内见囊变、坏死,增强扫描静脉期强化程度均低于肝实质。结论肾上腺淋巴瘤CT表现具有一定特征,以双侧受侵、边界清楚的低血供肿物为多见。继发病例常伴有多脏器、多组淋巴结受累。PET-CT有助于肿瘤定性诊断和观察有无淋巴结及其他脏器受累。     

Scid mice model for the in-vivo study of human oncotherapy - studies on the growth and metastasis of human lung-cancer

For the study of the growth and metastasis of human lung cancer, we established a severe combined immunodeficient (SCID) mouse model for engraftment of intact human lung-cancer tissue dissected from patient specimens. Small fragments of human lung-cancer tissues (14 cases) obtained from surgery or autopsy were implanted into the mammary fat pads of SCID mice. Seven of the fourteen cases (50%) showed an evident enlargement of the implanted lung-cancer tissue, the histopathology of which was almost identical to that of the original cancer tissues for as long as 2 months following implantation. There was slight correlation between the implantation success rate and the clinical stage of the patient at implantation. A second implantation of cancer tissues on four of these cases was successful. In contrast, no significant enlargement of the implanted tissue was observed in the cases of normal human peripheral-lung tissues (five cases), but a bronchial epidermal feature was observed in all of them. Matrigel (Collaborative Research, Bedford, MA) coating of the tissues significantly increased the growth rate of lung-cancer implants, and a high correlation (R=O.806) between the size of the implanted human lung-cancer tissues and carcinoembryonic antigen levels in the SCID mice was seen. Additionally, human lung-cancer cell lines subcutaneously injected into the backs of mice showed more metastatic lesions in the lungs and lymph nodes of SCID mice than in nude mice. Also, fresh human lung cancer metastasized to the lymph nodes and lungs of SCID mice. The results demonstrate the utility of SCID mice as recipients of human lung-cancer tissue and the applicability of this model to the in vivo study of mechanisms of human lung-cancer growth and metastasis.     

Synchronous multifocal osteosarcoma with lymphatic spread in the lung: an autopsy case report.

Synchronous multifocal/multicentric osteosarcoma (MOS) is a rare variant of osteosarcoma. We report here an autopsy case of a 15-year-old boy with MOS. Radiological examinations showed multiple sclerotic lesions in the left distal femur and in the ipsilateral proximal tibia without pulmonary metastasis at the first examination. Histological examination showed osteoblastic-type osteosarcoma. Despite high-dose chemotherapy the patient died of multiple bone and lung involvements 6 months after the initial diagnosis. Autopsy examination revealed prominent invasion of the tumor cells into lymphatic vessels and pleural dissemination without the formation of bulky, nodular metastasis in the lungs. Metastases in pulmonary hilar lymph nodes were noted without metastasis in other organs. Immunohistochemistry revealed that p53 protein was positive in most of the tumor cells. In summary, the present case was characterized by multiple bone involvement and prominent lymphatic spread of sarcoma cells in the lungs.     

18F-FDG PET-CT在非霍奇金淋巴瘤结外病灶检测及精确分期中的应用

目的 探讨18F-FDG PET-CT在检测非霍奇金淋巴瘤(NHL)结外病灶及精确分期中的应用价值.方法 回顾性分析94例初诊NHL患者的PET-CT结果,比较PET-CT与其他影像学检查对NHL结外病灶检出的一致性以及精确分期情况.结果 PET-CT检出受累病灶432处,其中淋巴组织及器官占73.8％(319/432),最大标准摄取值(SUVmax)平均13.4(3.4～33.4);结外病灶占26.2％(1 13/432),SUVmax平均13.5(3.1～55.0).PET-CT与CT对于淋巴组织及器官病灶的检出一致率为95％,而对于结外病灶的检出一致率仅为54.9％.PET-CT对于软组织、骨骼以及胃肠道病灶的检出率高于CT,但对于骨髓病灶的检出率低于骨髓细胞学检查.根据PET-CT结果再分期,29例(31.0％)调整分期,其中分期上调者占75.9％(22/29),引起分期上调的原因主要是PET-CT对于软组织、骨骼等病灶的检出率高;分期下调者占24.1％(7/29),引起分期下调的原因主要是PET-CT对于非肿瘤性原因引起的淋巴结及脾脏增大或浆膜腔积液的分辨力强.结论 18F-FDG PET-CT能够提高NHL结外病灶的检出率,特别是对于骨和软组织等弥漫性非肿块型病灶,有利于精确分期.