原发乳腺非霍奇金淋巴瘤6例临床分析

目的 分析原发乳腺非霍奇金淋巴瘤的临床特点,探讨诊断、分期和治疗方法及其预后的影响因素。方法 回顾性分析6例原发乳腺非霍奇金淋巴瘤的临床资料。6例病例均获随访,均于手术局部切除后辅助化疗和（或）放疗。结果 6例的中位生存期为53．5个月,均无瘤生存。结论 与原发乳腺非霍奇金淋巴瘤预后密切相关的因素依次是临床分期、病理类型和原发肿瘤大小、对原发乳腺非霍奇金淋巴瘤行综合治疗,可取得满意疗效。     

Estrogen Receptor Expression and Estrogen Receptor-independent Cytotoxic Effects of Tamoxifen on Malignant Rhabdoid Tumor Cells in vitro

Recent studies have shown that the antiestrogen tamoxifen (TAM) can be used in the treatment of malignant neoplasms other than breast cancer. In the present study, we investigated the expression of estrogen receptor (ER) in six malignant rhabdoid tumor (MRT) cell lines. Alterations in MRT cell growth in response to estrogen or antiestrogens (4-hydroxytamoxifen (4-OHT), TAM, and ICI 182 780) were also investigated. RT-PCR and western blotting showed that ER-a was expressed in three of the six MRT cell lines. While 17-β-estradiol (E2) did not significantly alter MRT cell line proliferation, the hydroxylated tamoxifen metabolite 4-OHT significantly inhibited the growth of all 6 MRT cell lines. However, the steroidal antiestrogen ICI 182 780 did not alter the proliferation of any of the MRT cell lines. 4-OHT induced apoptosis in both ER-α-negative and ER-α-positive MRT cell lines, as assessed by nuclear morphology and DNA fragmentation. Neither growth inhibition nor induction of apoptosis due to 4-OHT was blocked by the addition of excess E2. Our data suggested that 4-OHT induced cytotoxic effects against MRT cells, and that these effects were independent of ER expression.     

Malignant Rhabdoid Tumor in a Pregnant Adult Female: Literature Review of Central Nervous System Rhabdoid Tumors

Summary Rhabdoid tumors of the central nervous system are uncommon, aggressive childhood malignancies. The 13 described adult cases comprise both primary CNS tumors and malignant transformation of previously existing gliomas, meningiomas, and astrocytomas. Central nervous system rhabdoid lesions of adults have been diagnosed as primary malignant rhabdoid tumors, atypical teratoid/rhabdoid tumors, and more recently, rhabdoid glioblastomas. We report a case of a 20-year-old woman in her 30th week of pregnancy who presented with headache, nausea and blurry vision. MRI revealed a large rim-enhancing mass of the right occipital lobe. Gross total resection was achieved via a right parietal-occipital craniotomy. Pathologic evaluation revealed histology, electron microscopy and immunohistochemistry consistent with the diagnosis of malignant rhabdoid tumor. FISH studies were negative for the INI-1 genetic mutations and chromosome 22q deletion associated with childhood atypical rhabdoid/rhabdoid tumor in 75% of cases. The patient delivered her infant via caesarian section prior to initiating further therapy. We briefly describe the characteristics and current understanding of rhabdoid tumors, and review the literature comparing the 12 other cases of central nervous system rhabdoid tumors in adults. Furthermore, we consider and discuss the implications of this case being the second presentation of MRT during pregnancy in only six adult female patients.     

Malignant Peripheral Nerve Sheath Tumor of the Liver: A Case Report

A large, rapidly growing malignant peripheral nerve sheath tumor (MPNST) of the liver in a young female patient, not associated with von Recklinghausen’s disease, is presented. Diagnosis was based on detailed immunohistochemical and electromicroscopic examination beside the characteristic H&E picture. As far as we know, this is the first reported, unambiguously proven “de novo” MPNST in the liver. Differential diagnostic problems are discussed and a review of the literature is given.     

Fibromatosis of the Breast: A Case Report

Fibromatosis develops in many anatomic sites, but it rarely arises as a primary lesion in the breast. This lesion is locally invasive and frequently recurs after a local excision, but it has no potential for distant metastasis. In this report, we present a case of mammary fibromatosis which was closely similar to carcinoma in clinical, mammographic and ultrasonographic findings, thus leading us to breast conserving surgery. Despite being a rare disease, fibromatosis should be included in the differential diagnosis of younger patients (the age of the present case was 51, and the mean ages of patients with fibromatosis ranges from37 to 49) with abnormal changes on physical examinations and imaging studies.     

The Autocrine Loop of Epidermal Growth Factor Receptor-Epidermal Growth Factor/Transforming Growth Factor-α in Malignant Rhabdoid Tumor Cell Lines: Heterogeneity of Autocrine Mechanism in TTC549

To investigate the effects of the autocrine loop of epidermal growth factor receptor (EGFR)-epidermal growth factor (EGF)/transforming growth factor-α (TGF-α) on the proliferation and differentiation of malignant rhabdoid tumor (MRT), we used five MRT cell lines, TM87-16, STM91-01, TTC549, TTC642, and YAM-RTK1. RT-PCR analyses revealed expression of EGFR mRNA in all MRT cell lines. In contrast, the expression of either EGF or TGF-α mRNA was detected in all MRT cell lines. Expression of EGF, TGF-α, and EGFR as determined by immunocytochemical staining and in situ hybridization, correlated with the results of RT-PCR. Upon differentiation-induction with 12-O-tetradecanoylphorbol-13-acetate (TPA), in TTC549, showing an expression of TGF-α but not EGF initially, de novo expression of EGF mRNA appeared abruptly on day 2 of TPA treatment. To confirm the EGFR-EGF/TGF-α autocrine loop, we used TGF-α, EGF, and their antibodies in the cultures. Monoclonal antibody (mAb) to EGFR alone significantly inhibited the growth of cell line TTC549. However, mAb to EGF or TGF-α could inhibit proliferation of this cell line only when administrated together. Our findings would suggest that growth of the TTC549 cell line is constitutionally regulated by TGF-α/EGFR, but that inhibition of this autocrine mechanism results in transient activation of an autocrine loop involving EGF/EGFR. Our results may indicate the presence of two different autocrine loops of EGFR-EGF and/or EGFR-TGF-α in MRT cell lines. The heterogeneity of autocrine mechanisms found in MRT cell lines would be consistent with the multiphenotypic diversity and aggressive characteristics of this enigmatic tumor.     

原发性乳腺淋巴瘤(15例报告及文献复习)

目的：总结原发性乳腺淋巴瘤（PBL）的临床特征并探讨其预后因素。方法：回顾性分析15例PBL的临床资料并复习同期国内文献共134例报告,运用SPSS软件包分析其预后因素。结果：15例PBL均为术后明确诊断。中位年龄42岁,全部为NHL,其中DLCL型占53．33％,10例右乳首发,文献统计分析显示右乳多发（59％）,且乳房肿块大小和治疗模式是重要的预后因素,而与年龄、左或右乳首发分组其生存期无显著差别。结论：PBL以NHL占绝大多数,右乳首发多见,术前诊断困难,治疗宜选择手术联合放疗或（和）化疗的综合治疗模式。     

乳腺颗粒细胞瘤1例报告及合并文献分析

目的 分析乳腺颗粒细胞瘤的临床病理特点,并指导临床诊治.方法 回顾性分析1例乳腺颗粒细胞瘤的临床病理资料.结果 乳腺颗粒细胞瘤很少见,临床症状不典型,与乳腺癌很难鉴别,细针穿刺及术中快速冰冻病理检查无法确诊.手术治疗后,预后良好.结论 乳腺颗粒细胞瘤的诊断手术后病理诊断结果为标准,其特征性表现为胞质内存在嗜酸性颗粒.     

乳腺恶性腺肌上皮瘤5例临床病理分析并文献复习

目的: 探讨乳腺恶性腺肌上皮瘤(malignant adenomyoepithelioma,MAME)的临床病理特征、诊断、治疗、预后的特点。 方法: 对中国医学科学院肿瘤医院1991年1月至2007年12月收治的5例乳腺恶性腺肌上皮瘤的治疗结果、光镜及免疫组化特征进行了回顾性研究,并复习文献。 结果: 5例患者均为女性,发病年龄从27岁至74岁,中位年龄53岁,均行手术治疗。按照美国癌症联合委员会(AJCC)分期为Ⅰ期1例(20%),Ⅱ期4例(80%)。术后随访9～64个月,中位随访时间48个月,术后死亡1例(术后38个月死亡),其余4例仍生存,生存期9～64个月。术后出现复发转移2例,1例术后19个月出现胸壁及同侧腋下复发,其后全身骨转移;另1例为乳腺肿物扩大切除术后1个月出现切口处复发。术后平均生存时间为46个月。乳腺恶性腺肌上皮瘤组织学特点为存在上皮及肌上皮双向分化,主要为肌上皮细胞成分,同时伴有腺管状分化。免疫组化特征为肌上皮细胞对S-100阳性,Actin、SMA、Vimentin也可阳性,腺上皮细胞对CK、EMA、AE1/AE3阳性,而ER、PR、C-erbB2多为阴性。 结论: 乳腺肌上皮瘤的上皮、肌上皮成分均可发生恶变,同时恶变者罕见。以组织学结合免疫组织化学指标检测是确诊本病的关键。治疗应采取扩大手术切除,辅以放、化疗。乳腺恶性腺肌上皮瘤可局部复发和远处转移,出现局部复发患者应行根治术,转移以血行转移多于淋巴结转移,出现复发及转移者预后差。     

Fibromatosis of the Breast: A Case Report

Fibromatosis of the female breast is rare. We dealt with a recurrentcase of fibromatosis four months after a wide excision of thetumor. It was difficult to differentiate the tumor from carcinomaand only a pathological examination could lead to the finaldiagnosis. Ultrasonographic findings are so characteristic thatwe would like to emphasize the usefulness of ultrasonographynot only in distinguishing between recurrence and operativescar but also in defining the diagnosis as fibromatosis of thebreast     

电医化学治疗恶性肿瘤：（附20例分析报告）

电医化学治疗恶性肿瘤是一种局部治疗的最新方法。我院采用本法治疗恶性肿瘤20例,其中肝癌4例、肺癌10例、骨癌1例、纤维肉瘤2例、皮肤癌3例。有效率90％。肿瘤细胞比正常细胞对周围环境变化更敏感。在直流电场的作用下强制改变肿瘤细胞周围环境,在阳极pH2～3,阴极pH12以上,使肿瘤细胞退化死亡。本法对远处转移无明显治疗作用,如配合辅助化疗及放疗则效果更为理想。     

Central Nervous System Atypical Teratoid Tumor/Rhabdoid Tumor: Response to Intensive Therapy and Review of the Literature

Central nervous system atypical teratoid/rhabdoid tumor (ATT/RT) of infancy and childhood is a unique histologic entity with an extremely aggressive natural history. Standard therapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective; most children survive less than 12 months after diagnosis. Intensified therapy has been recently used for children with this disease, with promising results [1,2].We report four cases of ATT/RT in young children; all had subtotal resections and localized disease at diagnosis. One child treated prior to bone marrow transplant availability died of progressive disease 9 months after diagnosis. Another child, treated with high-dose chemotherapy and radiotherapy in preparation for bone marrow transplant, had a recurrence and died 20 months after diagnosis, without undergoing the transplant. Two children received high-dose chemotherapy and autologous bone-marrow transplant and had a good response to treatment; one survived 19 months, the other child is free of disease 46 months from diagnosis. Intensified therapy has altered the natural history of central nervous system ATT/RT.     

Primary Malignant Melanoma of the Female Urethra: A Case Report

A case of primary malignant melanoma of the female urethra ispresented. A 65-year-old Japanese woman was referred with dysuriaand urethral bleeding. A hemorrhagic blue-black tumor, 3 cmin diameter, was diagnosed as a malignant melanoma by urinarycytology and biopsy. In spite of radical surgery followed byadjuvant immunochemotherapy with beta interferon, dacarbazine,nimustine and vincristine (IFN ß-DAV), the patientdied of the disease one year after surgery because of lung metastasiswhich developed six months after diagnosis. The regional lymphnodes were not involved. In the present paper, we have brieflydiscussed the diagnostic value of cytological examination forthis condition, as well as biopsy, with regard to the risk ofhematogeneous tumor spread.     

Diagnosis and Differential Diagnosis of Rhabdoid Meningioma:One Case Report and Literature Review

Introduction Rhabdoid meningioma (RM) is a special type of meningioma. The pathologic features of RM include sheets of rhabdoid tumor cells with or without the findings typical of conventional meningiomas.     

3081例儿童肿瘤活检资料分析

目的：分析儿童肿瘤的发生率及其特点。方法：活检资料回顾性分析。结果：良性肿瘤1841例,主要为脉管瘤和畸胎瘤。恶性肿瘤1240例,主要为淋巴系统肿瘤、肾母细胞瘤、视网膜母细胞瘤等。结论：儿童良性肿瘤发生率为59.8%,恶性肿瘤发生率为40.2%。发病年龄集中在4岁以前,淋巴系统肿瘤以5-9岁年龄组最多,男多于女。     

Malignant proliferating trichilemmal tumor in the skin over the breast: a case report

A proliferating trichilemmal tumor is relatively uncommon. It is composed of multiple cysts consisting of squamous epithelium with trichilemmal keratinization without granular layer interposition. This lesion usually occurs in the scalp of elderly women. We describe a 67-year-old woman with a malignant proliferating trichilemmal tumor in the skin over the breast. We first misdiagnosed the disease as a primary squamous cell carcinoma of the breast with a metastatic lymph node in the axilla because of the disease site and our unfamiliarity with the disease. The patient underwent radical mastectomy with axillary dissection. Eight months postoperatively, a tumor appeared in her right axilla and progressively enlarged. We subsequently excised the tumor. She is healthy as of 8 months postoperatively. To the best of our knowledge, only one case of a proliferating trichilemmal tumor occurring in the skin over the breast has been reported.