原发性乳腺淋巴瘤27例临床分析并文献复习

背景与目的:原发性乳腺淋巴瘤(primary breast lymphoma,PBL)发病率低,预后较差.本研究旨在分析该病例的临床和病理特征,从而探讨PBL的合理治疗模式.方法:收集并回顾性分析1976年到2005年间在中山大学肿瘤防治中心诊断为PBL,并接受治疗的27例患者的临床资料和治疗情况.结果:27例中有26例女性和1例男性患者;年龄12～84岁;90%的患者为Ⅰ E期或ⅡE期.按照WHO 2001淋巴瘤病理分类系统,有22例B细胞性淋巴瘤(17例弥漫大B细胞性淋巴瘤,2例黏膜相关性淋巴瘤,1例边缘区淋巴瘤,2例未能分类),3例外周T细胞性淋巴瘤,2例患者的病理类型未能分类.初始治疗时有20例患者接受了综合治疗,其中8例患者为根治术加术后化疗,12例患者为肿物切除术后加全身化疗,两组的5年生存率分别为23.0%和58.0%(P=0.006);其余有5例患者仅接受全身化疗,2例患者仅接受肿物切除手术.24例患者在初始治疗后取得完全缓解,1例患者部分缓解,2例患者疾病进展.随访时间1个月～10年,中位随访时间38个月.全组患者的5年总生存率和无病生存率分别是47.0%和23.0%;其中20例中高度恶性淋巴瘤患者(17例弥漫大B细胞性淋巴瘤和3例外周T细胞性淋巴瘤)的5年总生存率和无病生存率分别是48.0%和27.0%;随访中有16例复发,部位见于同侧乳腺6例、对侧乳腺4例、中枢神经系统(central nervous system,CNS)3例、骨髓1例和淋巴结侵犯2例.结论:PBL的病理类型以中高度恶性淋巴瘤为主;根治性手术在其治疗中作用有限,肿物切除术加术后化疗和放疗的效果较好.PBL患者易发生CNS复发,在随访中应定期进行颅脑CT或MR检查.     

24例原发纵隔大B细胞淋巴瘤的临床分析并文献复习

背景与目的:原发纵隔大B细胞淋巴瘤(primary mediastinal large B-cell lymphoma,PMBCL)是弥漫大B细胞淋巴瘤的一种亚型,发病率较低。本研究旨在分析其临床特征,探讨合理的治疗模式及预后因素。方法:收集1995年5月至2005年9月于福建省肿瘤医院确诊并接受治疗的24例PMBCL患者的临床资料并行回顾性分析,同时结合文献加以讨论。结果:24例患者中男性16例,女性8例,年龄12～81岁,30岁以下13例。临床Ⅰ Ⅱ期20例,Ⅲ期1例,Ⅳ期3例。有纵隔巨块13例,伴上腔静脉综合征10例,邻近器官受侵14例,乳酸脱氢酶升高15例。初治时11例采用联合化放疗,10例采用单纯化疗,3例采用单纯放疗,完全缓解率41.7%,总有效率91.7%,中位生存期89个月,3年总生存率68.8%,其中初治时达完全缓解者至随访结束时均生存,国际预后指数(international prognostic index,IPI)在本组中未显示预后,多因素分析提示纵隔巨块与预后相关。结论:PMBCL在本组中男性多见,临床表现凶险,须尽快明确诊断。蒽环类为主的化疗联合放疗可取得较好疗效,初治时获得完全缓解尤为关键,伴有巨块者预后差。     

Clinicopathologic features and treatment outcome of primary breast diffuse large B-cell lymphoma

We studied the clinicopathologic features and treatment outcome of patients with breast diffuse large B-cell lymphoma. As to the cellular immunophenotype, CD5 was detected in two patients, CD10 in 4, BCL2 in 20, BCL6 in 11, and MUM-1 in 17. The 5-year progression-free survival was 77% and the 5-year overall survival was 87%. Patients with the germinal center B-cell (GCB) type had a significantly better prognosis than those with the non-GCB type. The combination of anthracycline-containing chemotherapy and/or involved-field radiotherapy produced a relatively good prognosis. However, it is a heterogeneous disease with regard to histological type and pathological state.     

以原发性脾淋巴瘤为表现的弥漫性大B细胞淋巴瘤1例并文献复习

目的：探讨原发性脾淋巴瘤（PSL）的诊断以及原发性脾弥漫性大B细胞淋巴瘤的治疗。方法：报告1例以PSL为表现的弥漫性大B细胞淋巴瘤并结合文献进行复习。结果：患者表现为左上腹疼痛,无发热、盗汗,无浅表淋巴结肿大,CT证实脾脏内有占位性病变,脾脏病理示弥漫性大B细胞淋巴瘤,外周血和骨髓检查正常,脾切除术后PET/CT未见明显与淋巴瘤相关的18F-脱氧葡萄糖摄取异常增高表现,后给予联合化疗,随访8月余仍处于完全缓解期。结论：PSL是一少见肿瘤,采取脾切除后继以CHOP±利妥昔单抗治疗原发性脾弥漫性大B细胞淋巴瘤能够达到长期生存的目的。     

利妥昔单抗应用于临床后原发纵隔B细胞淋巴瘤治疗进展

原发性纵隔B细胞淋巴瘤(PMBCL)形态学上与弥漫性大B细胞淋巴瘤(DLBCL)和结节性硬化霍奇金淋巴瘤相似。多数 PMBCL 患者接受化疗后加巩固性累及野放疗治疗方式,认为巩固放疗能提高PMBCL的有效率和PFS,化放疗结合取得了较好疗效。最近临床研究结果显示,利妥昔单抗和蒽环类化疗方案提高了PMBCL治疗效果,能降低早期治疗失败,提高PFS和OS,改善预后。利妥昔单抗结合部分高强度化疗后不放疗也取得了较好疗效,但多数文献仍在支持免疫化疗后巩固性纵隔放疗。基于病例数较少的研究结果,结合PET进展高强度免疫化疗后PET评价达到完全代谢缓解(CMR)的患者,或许可以不行巩固纵隔放疗。然而,这些结果需要一系列更大样本的多中心临床试验证实,患者伴预后不良因素或PET评价分值高于3分时建议纵隔巩固性放疗。     

Clinical Features,Management, and Prognosis of an International Series of 161 Patients With Limited‐Stage Diffuse Large B‐Cell Lymphoma of the Bone (the IELSG‐14 Study)

Introduction.

The clinical features, management, and prognosis of stage I–II diffuse large B-cell lymphoma of the bone (PB-DLBCL) included in an international database of 499 lymphoma patients with skeletal involvement were reviewed.

Methods.

HIV-negative patients (n = 161) with diffuse large B-cell lymphoma of the bone (PB-DLBCL) after complete staging workup were considered. The primary objective of this study was to identify the most effective treatment modality; the secondary objectives were to define the contribution of irradiation fields and doses and the pattern of relapse.

Results.

Median age was 55 years (range, 18–99 years), with a male/female ratio of 1:2; 141 (87%) patients had stage I, 14 (9%) had B symptoms, 37 (23%) had bulky lesion, 54 (33%) showed elevated lactate dehydrogenase serum levels, and 25 (15%) had fracture. Thirteen (8%) patients received chemotherapy alone, 23 (14%) received radiotherapy alone, and 125 (78%) received both treatments. The response to the first-line treatment was complete in 131 of 152 assessed patients (complete response rate, 86%; 95% confidence interval [CI], 81%–91%) and partial in 7, with an overall response rate of 91% (95% CI, 87%–95%). At a median follow-up of 54 months (range, 3–218), 107 (67%) patients remained relapse-free, with a 5-year progression-free survival of 68% (SE: 4). Four (2.5%) patients had meningeal relapse; 119 patients were alive (113 disease-free), with a 5-year overall survival of 75% (SE: 4). Patients managed with primary chemotherapy, whether followed by radiotherapy or not, had a significantly better outcome than patients treated with primary radiotherapy, whether followed by chemotherapy or not. The addition of consolidative radiotherapy after primary chemotherapy was not associated with improved outcome; doses >36 Gy and the irradiation of the whole affected bone were not associated with better outcome.

Conclusion.

Patients with PB-DLBCL exhibit a favorable prognosis when treated with primary anthracycline-based chemotherapy whether followed by radiotherapy or not. In patients treated with chemoradiotherapy, the use of larger radiation fields and doses is not associated with better outcome. Central nervous system dissemination is a rare event in PB-DLBCL patients.     

Sequential development of peripheral t-cell lymphoma post immunochemotherapy of diffuse large B cell lymphoma

Reports of sequential occurrence of two or more types of lymphoma are rare, especially when they involve different cell lineages. Herein, we report a rare case of sequential development of peripheral t-cell lymphoma following treatment of diffuse large B cell lymphoma. In a 73-year-old Chinese male patient, diffuse large B-cell lymphoma (DLBCL) was diagnosed in September 2011 based on the result of a tongue biopsy. Afterwards, he received rituximab combined with chemotherapy and local radiotherapy. Though he achieved completed remission, he had a new symptom of one enlarged left inguinal lymph node in November of 2015. A new biopsy was then performed. Immunohistochemistry and polymerase chain reaction (PCR) for gene rearrangements proved monoclonal T-cell lymphoma. We didn't detect EBV infection in either of two biopsies, nor any evidence of immune dysfunction complications. Sequential development of B-cell and T-cell malignancy in this patient maybe an example of treatment-related secondary lymphoma.     

14例原发性鼻窦非霍奇金淋巴瘤的临床分析

背景与目的:原发性鼻窦非霍奇金淋巴瘤(primary paranasal sinus lymphoma,PPSL)临床上较罕见,其生物学行为较独特.本文总结和分析PPSL的临床病理特点和治疗情况,以期增加对此病的认识.方法:回顾性分析1994～2006年中山大学肿瘤防治中心收治的14例PPSL,分析其临床和病理特点、治疗经过和随访结果.结果:14例中原发于上颌窦者11例,筛窦2例,蝶窦1例.根据淋巴瘤Ann Arbor分期,所有患者均为Ⅰ～Ⅱ期.根据美国癌症联合委员会(AJCC)的鼻窦实体瘤TNM分期标准,大部分患者(11/14)属于局部晚期T3～T4.病理类型:B细胞性12例(85.7%),其中弥漫大B细胞性占42.9%(6例);T细胞性1例,非B非T细胞性1例.2例接受上颌窦根治术,12例为肿物切除或活检;14例全部接受化疗,6例化疗后接受局部放疗.中位生存期59.5个月,全组5年总生存率和无事件生存率均为78.6%.结论:PPSL发病部位以上颌窦多见,病理类型以B细胞性为主,其中弥漫大B细胞性最常见,肿瘤局部侵犯广泛为其重要特征;采用全身化疗和局部放疗联合治疗,患者总体预后相对较好.     

原发性肝脏淋巴瘤一例诊治讨论

原发性肝脏淋巴瘤(PHL)是一种极其罕见的结外淋巴瘤,目前尚无统一的诊治标准.其临床表现无特异性,容易与肝炎、肝脏原发和继发性肿瘤混淆,病理为诊断的金标准.通常认为该病的治疗与其他部位的结外淋巴瘤相似,应作为全身疾病来考虑,宜采用包括手术、化疗、放疗等在内的综合治疗模式.2014年2月山西医科大学附属肿瘤医院血液病诊疗中心收治1例33岁男性原发性肝脏弥漫大B细胞淋巴瘤患者,经8个周期化疗达完全缓解,至截稿时仍无病生存.     

The rationale and role of radiation therapy in the treatment of patients with diffuse large B-cell lymphoma in the Rituximab era

Developments in the evaluation and systemic management of diffuse large B-cell lymphoma (DLBCL) require ongoing assessment of the role of external beam radiotherapy in management. This review assesses data regarding the use of radiotherapy in the initial management of early stage and advanced DLBCL, and considers the implications of bulky and residual disease, and the contribution of PET scanning, to decisions regarding the use of radiotherapy after chemotherapy. Limited R-CHOP plus radiotherapy, or full dose R-CHOP alone, are both likely to cure approximately 90% of patients with low risk early stage disease. The choice of therapy will depend on considerations of acute and late toxicity of the two approaches, taking into account individual patient risk profiles and preferences. Unfavorable early-stage and advanced-stage disease require treatment with full dose R-CHOP. The presence of bulky disease predicts for a higher risk of relapse, which may be partly ameliorated by the addition of radiotherapy. The rapidity of response on PET scanning, the presence of a post-therapy residual mass, the potential toxicity of radiotherapy and the available salvage options all need to be considered on a patient by patient basis, when considering the use of radiotherapy for advanced disease.     

培美曲塞为主的化疗方案治疗中老年非霍奇金淋巴瘤回顾性分析CSCD

目的回顾性分析培美曲塞为主的化疗方案治疗中老年非霍奇金瘤患者的安全性及有效性。方法回顾性收集2016年11月至2018年10月中国医学科学院肿瘤医院收治的以培美曲塞为主的化疗方案治疗的非霍奇金淋巴瘤患者临床资料,并对相关文献进行系统性综述。结果共纳入10例非霍奇金淋巴瘤患者,7例为弥漫大B细胞淋巴瘤,2例为血管免疫母T细胞淋巴瘤,1例为结外NK/T细胞淋巴瘤。肿瘤复发或一线治疗进展后接受培美曲塞为主的化疗方案,弥漫大B细胞淋巴瘤有4例达到部分缓解,3例疾病进展,出现的不良反应主要为Ⅰ~Ⅱ度骨髓抑制及胃肠道反应。结论培美曲塞为主的化疗方案治疗弥漫大B细胞淋巴瘤患者,尤其是原发性中枢神经系统淋巴瘤,可能有一定的有效性,且安全性可控。     

Primary bladder lymphoma: management and outcome of 12 patients with a review of the literature

Primary bladder non-Hodgkin's lymphoma (NHL) is rare. Optimal management remains controversial. Using the Scotland and Newcastle lymphoma group database, 12 patients with primary bladder lymphoma were identified between 1980 and 2001, the largest single group of patients available to date. Histology and immunocytochemistry was reviewed in 9 of the 12 cases. Six cases were low-grade extranodal marginal zone lymphoma, 4 diffuse large B-cell lymphoma, one an ALK 1 positive anaplastic large cell lymphoma (ALKoma) and one a low-grade lymphoma unspecified. Two patients (low-grade NHL) were treated with oral antibiotics (n = 1) or diathermy (n = 1) alone with complete resolution of disease. One patient with high-grade NHL gained complete remission without conventional therapy. Nine patients were treated with single or combined modality surgery, chemotherapy and/or radiotherapy. Overall survival was 75%, mean follow up of 4.8 (range 1 - 10) years. A review of 88 additional cases in the literature support the findings that primary bladder lymphoma is associated with a good prognosis. Patients with low-grade extranodal marginal zone lymphoma may respond well to simple therapies. Patients with diffuse large B-cell lymphoma respond well to first-line chemotherapy regimens. Ureteric obstruction and acute renal failure are serious complications. Repeat cystoscopy is mandatory for follow-up.     

原发乳腺淋巴瘤14例临床分析并文献回顾

目的：探讨原发乳腺淋巴瘤（primary breast lymphoma,PBL）的治疗方法及预后。方法：对军事医学科学院附属医院淋巴瘤科2005-07-15-2011-01-20收治的14例PBL患者的临床特征、病理特点、治疗方法及预后进行回顾性分析。结果：14例PBL均为女性,中位年龄48岁;ⅠE及ⅡE期各7例;病理类型均为B细胞淋巴瘤。其中3例行患侧全乳切除并腋窝淋巴结清除术,9例行乳腺肿物切除术,2例行乳腺肿物核芯针穿刺活检术。10例接受化放疗,4例单纯化疗,4例同时行中枢预防性鞘内注射;其中一线化疗完全缓解率为86%。中位随访53.5个月,7例复发,3例死亡;5年无进展生存率为39%,5年总生存率为72%。结论：全乳切除未使PBL患者获益。乳腺肿物切除明确病理、以蒽环类为主的化疗联合受累野放疗可为适宜的选择。由于PBL中枢复发率较高,故建议中枢预防鞘内注射。     

Primary non-Hodgkin’s lymphoma of the bone

Primary non-Hodgkin’s lymphoma of the bone is an unusual entity. Twenty-five patients with diffuse large cell lymphoma of the bone were registered at the Tata Memorial Hospital (TMH) from August, 1991, to May, 2002. Pain at the local site and soft tissue swelling were the commonest symptoms. Involvement of the bones in the lower half of the body was more frequent than the bones in the upper half. Osteolytic lesions and an associated soft tissue mass were the common radiological findings. Nineteen patients received CHOP chemotherapy and five received COP chemotherapy. Twenty-three patients received involved field radiotherapy. The overall response to therapy was 96%. On follow-up, two patients had a nodal relapse. One patient died of progressive disease, and one patient died of cryptococcal meningitis. There were no deaths due to treatment-related toxicity. The mean progression free survival was 9.39 yr and the overall survival was 11.66 yr. The median overall survival has not been reached. At last follow-up, 21 patients were being following up at TMH and are free of disease. Conclusion: Primary bone lymphoma is a malignancy that is highly curable with a combination of chemotherapy and radiotherapy.     

A clinicopathological retrospective study of 131 patients with primary bone lymphoma: a population-based study of successively treated cohorts from the British Columbia Cancer Agency.

BACKGROUND: Primary bone lymphoma (PBL) is a distinct clinicopathological entity. Although PBL has been reviewed in several small studies, few reflect recent improvements in primary treatment. METHODS: We used the British Columbia Cancer Agency Lymphoid Cancer Database to identify all patients with PBL (1983-2005). All were staged in a uniform manner and treated with era-specific protocols. RESULTS: We identified 131 patients with a median age of 63 years (18-87). One third had disease in long bones and another one third had disease in the spine, of which half presented with spinal cord compression. Patients with diffuse large-cell lymphoma (DLCL) (n=103, 79%) had 5- and 10-year overall survivals (OS) of 62% and 41%, respectively. Multivariate analysis identified three prognostic groups: age<60 with International Prognostic Index (IPI) 1-3 (n=43), age>or=60 with IPI 0-3 (n=23) and age>or=60 with IPI 4-5 (n=33), with markedly different 5-year OS of 90%, 61% and 25%, respectively (P<0.0001). Neither primary site nor pathological fracture at presentation had an impact on OS. The 3-year progression-free survival in patients who received rituximab plus combination chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOPR) chemotherapy was 88% compared with 52% in those who received CHOP-like chemotherapy without rituximab (P=0.005). The 10-year OS for those with advanced-stage disease who received irradiation plus chemotherapy was 25% versus 56% for those who received chemotherapy alone (P=0.025). Patients received irradiation if spinal cord compression was present or residual disease at the end of chemotherapy was thought to require it. CONCLUSIONS: PBL is usually of DLCL type and has an improved outcome with CHOPR. Younger patients with good IPI score have a favorable prognosis.     

Outcomes of Transplant-Eligible Patients With Relapsed or Refractory Diffuse Large B-Cell Lymphoma After Second-Line Salvage Chemotherapy: The Gustave Roussy Experience

IntroductionAfter failure of frontline therapy, patients with relapsed/refractory diffuse large B-cell lymphoma (RR-DLBCL) that does not respond to first-line salvage chemotherapy can be recommended second-line salvage chemotherapy. The available literature in this regard is weak, although many centers routinely offer this type of second-line salvage chemotherapy to their patients.Patients and MethodsThis retrospective study included transplant-eligible patients with RR-DLBCL treated at Gustave Roussy between January 2008 and April 2020. Eligible patients were those who received second-line salvage chemotherapy using R-DHAP or R-ICE in patients who experienced an insufficient partial response, stable disease, or progressive disease in response to first-line salvage chemoimmunotherapy using an alternative regimen.ResultsForty-six RR-DLBCL patients received second-line salvage regimen, which yielded an objective response rate of 33%, median progression-free survival of 2.1 months, and overall survival of 11.4 months. Twelve patients proceeded to autologous stem-cell transplantation (ASCT), of whom 70% remained alive 1 year after ASCT. To explore the impact of transplantation, a multivariate analysis (excluding response to the first-line salvage regimen because this covariate was totally embedded within the transplantation covariate), ASCT was associated with progression-free survival (hazard ratio = 0.16; 95% confidence interval, 0.06-0.42) and overall survival (hazard ratio = 0.27; 95% confidence interval, 0.08-0.88).ConclusionSecond-line salvage chemotherapy with R-DHAP or R-ICE followed by ASCT leads to a favorable outcome in almost one third of patients with RR-DLBCL and offers a median overall survival of approximately 1 year. These data support the administration of second-line salvage chemotherapy followed by ASCT.     

Primary pulmonary non-Hodgkin's lymphoma

BACKGROUND: Primary pulmonary non-Hodgkin's lymphoma is a very rare neoplasm. It is represented most commonly by marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type. Although there have been a few reviews of this lymphoma, clinical features, diagnostic procedure, optimal management and prognostic factors have not been well defined. METHODS: We reviewed the medical records of 24 patients who were pathologically and clinically diagnosed as primary pulmonary lymphoma between September 1995 and June 2003. RESULTS: There were 13 patients with MALT lymphoma and two with MALT lymphoma accompanied by large B-cell lymphoma, seven with diffuse large B-cell lymphoma and two with anaplastic large cell lymphoma. Half the patients were asymptomatic at presentation; 46% had respiratory symptoms and 16.7% had B-symptoms. Initial radiological findings were variable including nodules, masses, infiltrates or consolidation. The majority of patients (66.7%) needed surgical approaches (open thoracotomy or video-assisted thoracoscopy) for definite diagnosis. Bronchoscopy was performed in 83%, but only 30% showed a diagnostic yield. The 13 patients with MALT lymphoma were treated with a variety of modalities such as observation, surgery and single or combination chemotherapy, and combination chemotherapy was administered to 11 patients with non-MALT lymphoma regardless of surgery. The overall survival rate at 3 years for all 24 patients was 86% with a median follow-up of 32 months. CONCLUSION: Although this entity of lymphoma appears to have a good prognosis, further clinical experience and long-term follow-up are needed to identify prognostic factors.     

Gastrointestinal lymphoma

Opinion statement The ability to make treatment recommendations for patients with gastrointestinal lymphoma is hampered by a lack of prospective trials and by a lack of uniformity in classification and staging. Patients with gastric diffuse large B-cell lymphoma have traditionally been treated with surgery and many physicians continue to recommend this approach. However, recent data suggest that these patients can be treated with combination chemotherapy regimens in the same manner as patients with nodal presentations of diffuse large B-cell lymphoma. There is evidence to suggest that adjuvant radiotherapy may improve the outcome for these patients. The recognition that extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue is a distinct clinicopathologic entity and the elucidation of the pathogenic role of Helicobacter pylori has revolutionized the treatment of these gastric lymphomas. Patients with localized disease should be managed with antibiotic therapy initially. Radiation therapy is extremely effective for these patients, but it should probably be reserved for patients who fail anti-H. pylori treatment.     

原发性甲状腺恶性淋巴瘤的临床病理特点

背景与目的:原发性甲状腺恶性淋巴瘤(primary thyroidl ymphoma,PTL)是一种罕见疾病,易误诊,治疗上仍存在争议。本研究旨在分析总结PTL的临床病理特点,结合文献复习,以提高对本病的认识并探讨合适的临床治疗方法。方法:分析中国医学科学院中国协和医科大学肿瘤医院1990年1月至2004年1月收治的22例PTL患者的临床和病理资料。结果:22例患者中女性18例,男性4例;中位年龄55岁(33～80岁)。所有患者均为B细胞性NHL;按WHO分类,72.7%(16/22)为弥漫大B细胞型(DLBCL)、27.3%(6/22)为粘膜相关淋巴样组织淋巴瘤(MALT)。单纯手术5例;综合治疗17例,其中术后化疗6例,术后放疗7例,术后放、化疗4例。中位随访42个月(1～168个月),5年无复发生存率和总生存率分别为22.7%和31.8%。结论:PTL好发于女性,DLBCL与MALT型NHL为最常见类型。对ⅠE～ⅡE期PTL患者采用手术为主的综合治疗效果较好。     

原发性卵巢非霍奇金淋巴瘤1例及相关文献复习

目的:探讨原发性卵巢弥漫性大B细胞淋巴瘤的病因、临床表现、诊断、治疗及预后。方法:报道1例原发性卵巢弥漫性大B细胞淋巴瘤的临床病理资料及术后免疫组化结果,并阅读及复习国内外相关文献。结果:该例患者为（右）卵巢弥漫性大B细胞淋巴瘤,积极治疗后现考虑肿瘤复发。结论:原发性卵巢弥漫性大B细胞淋巴瘤罕见,确诊依赖于术后病理及免疫组化,预后不佳,常采用以手术为主,辅以化疗和放疗的综合治疗。