乳腺外佩吉特病三例报告并文献复习

目的 探讨乳腺外佩吉特病(EMPD)的临床特点以及手术、放疗及化疗在EMPD治疗中的作用.方法 回顾性分析3例EMPD,并复习国内外文献中EMPD的病例65例,共68例.结果 手术和手术加放疗组56例,随访时间6～204个月,局部复发率为39.3%,18例行术后放疗,其中1例术后3年复发、放疗后8年复发、再次放疗后生存6年,死于肺转移;6例放疗后2年未见复发,2例放疗后5年未见复发.单纯放疗者10例,随访时间6～96个月,2例放疗后12～24个月出现局部复发,其中1例再次放疗后生存26个月死亡,局部控制率为80%.单纯化疗者2例,1例完全缓解.结论 EMPD病史较长,病情发展缓慢,可局限于局部数年,易误诊,误诊时间≥1年,手术为EMPD的首选治疗方式,但局部复发率较高,配合放疗可降低其局部复发率,单纯放疗也可取得满意的局部控制率.反复复发或转移者化疗具有一定的作用.     

三维适形调强放疗同步顺铂化疗联合尼妥珠单抗治疗局部晚期鼻咽癌的临床疗效

目的 观察三维适形调强放疗同步顺铂化疗联合分子靶向药物尼妥珠单抗治疗局部晚期鼻咽癌患者的临床疗效、耐受性及安全性。方法 选取本院收治的初治局部晚期鼻咽癌患者40例,采用三维适形调强放疗同步顺铂化疗联合分子靶向药物尼妥珠单抗治疗。三维适形调强放疗靶区剂量PGTVnx及PGTVnd均为69.96~73.92 Gy、PTV-1为60~66 Gy、PTV-2为54~56 Gy,共分割33次。化疗方案：顺铂80 mg/m2,静滴,3周为1个周期,每个周期的首日使用,共2个周期;放疗的第1天给予尼妥珠单抗（泰欣生）100 mg,每周1次,共8周。随访观察患者治疗的临床疗效、耐受性及安全性。结果 40例患者均按计划完成治疗,所有患者至少随访24个月,随访率100%,在随访期内1例局部进展。治疗后6个月评价,鼻咽病灶和淋巴结的有效率均为97.5%。治疗期间发生Ⅲ级骨髓抑制9例,Ⅲ级口腔黏膜炎8例,无Ⅳ级毒副反应发生。结论 三维适形调强放疗同步顺铂化疗联合分子靶向药物尼妥珠单抗治疗局部晚期鼻咽癌的近期疗效显著,耐受性及安全性良好,值得临床推广应用。     

朗格汉斯组织细胞增生症放疗的疗效观察

目的探讨放疗对朗格汉斯组织细胞增生症的疗效及不良反应。方法回顾性分析21例经病理检查确诊的朗格汉斯组织细胞增生症患者的临床资料。结果非成人组14例(27个病变部位),中位单次放疗剂量180 cGy,中位总放疗剂量1 600 cGy;局部控制率96.3%,完全缓解率为74.1%。成人组7例(9个病灶),中位单次放疗剂量200cGy,中位总放疗剂量2 000 cGy;局部控制率100.0%,完全缓解率66.7%。放疗剂量≤2 000 cGy者与>2 000 cGy者局部控制率及完全缓解率比较,无显著性差异(P>0.05)。中位随访时间4年。随访期间无严重放疗并发症发生;无野内复发。结论放疗是朗格汉斯组织细胞增生症的有效治疗手段,其局控率高,野内复发及严重放疗并发症少见。     

西妥昔单抗联合顺铂同步三维适形调强放疗治疗初治局部晚期鼻咽癌的临床观察

目的 探讨西妥昔单抗联合顺铂同步三维适形调强放疗（IMRT）治疗初治局部晚期鼻咽癌患者的耐受性、安全性及疗效。方法 2008年8月至2009年4月,对经病理学证实的初治局部晚期鼻咽癌8例患者采用西妥昔单抗联合顺铂同步IMRT方案治疗。同步化疗方案为：顺铂80mg／m2,每3周1次,共2次;西妥昔单抗放疗前1周给予400mg／m2,之后每周250mg／m2,共7～8次。IMRT靶区剂量为69.96～73.92Gy,照射33次。随访观察患者的耐受性、安全性及疗效。结果 8例患者均按计划完成治疗,随访36个月,随访期内无1例死亡。8例患者均出现1～2级皮疹,2例3级骨髓抑制,2例3级口腔黏膜反应,无4级毒副反应发生。治疗后3个月评价,获CR 5例,PR 3例。2例出现鼻咽局部复发,经再程治疗后获CR和PR各1例;其余患者随访期内未出现复发。结论 对初治局部晚期鼻咽癌患者,采用西妥昔单抗联合顺铂同步IMRT治疗有较好的安全性和耐受性,近期疗效好,远期疗效待扩大病例进一步随访观察。     

食管癌放疗后复发再程调强放疗的临床观察

目的:探讨调强放疗(intensity modulater radiation therapy,IMRT)在食管癌首程放疗后局部复发患者中的应用及临床观察。方法:37例首程放疗后局部复发的食管鳞癌患者,采用调强放疗技术进行二程放疗,处方剂量为（50-60）Gy/［2Gy·（25-30）f］。结果:37例患者中完全缓解（complete response,CR）7例,部分缓解（partial response,PR）20例,有效率（response rate,RR）为73.0%（27/37）。本组患者1、2、3年生存率分别为59.5%、32.4%、21.1%。放射性食管炎、骨髓抑制、胃肠道反应的发生率较高。全部病例随访资料完整。截止2012年12月,25例患者已经死亡,其中死于局部复发11例、远处转移8例、食管气管瘘2例、大出血2例、其他疾病1例、自杀1例。结论:调强放疗对于食管癌首程放疗后局部复发患者是一种较好的有效的治疗方法,能取得较好的局部控制率。     

恶性纤维组织细胞瘤13例临床分析

恶性纤维组织细胞瘤较少见,作者在18年间收治13例,其中男性12例,女性1例,平均年龄43岁,发生于下肢4例,上肢3例,肩关节1例,腹股沟1例,上颌窦1例,锁骨上1例,腹膜后1例,阴囊1例。肿块小于5cm5例,5～10cm7例,大于10cm1例。截肢术3例,扩大切除2例,扩大切除+放疗+化疗1例,扩大切除+放疗1例,局部切除2例,局部切除+放疗1例,活检后行化疗3例。11例随访2年以上,2年生存率为46.1％(2例失访均认为生存期不足2年)。本组资料提示,肿瘤大小、部位及治疗方法影响预后。     

42例放疗后复发食管癌三维适形放疗的疗效分析

目的 探讨三维适形放疗(3DCRT)在食管癌首程放疗后局部复发患者中应用的可行性、疗效和放射损伤及影响因素.方法 42例首程放疗后局部复发的食管癌患者,采用3DCRT技术进行二程放疗.放疗处方剂量中位值54 Gy(50～64 Gy),1.8～2.0 Gy/次,5次/周.结果 随访截至2008年12月31日,随访率为100%,其中随访满1、2年者分别为20、22例.42例患者中完全缓解7例,部分缓解31例,无进展4例.全组患者1、2年总生存率分别为60%和24%.全组≥2级放射性食管炎15例,其中2级13例,3级2例.≥2级放射性肺炎9例,其中2级8例,3级1例.≥2级血液学副反应3例,其中2级2例,3级1例.本组共死亡28例,其中死于局部复发9例、远处转移7例、食管瘘1例、食管狭窄1例、全身衰竭4例、心脏病3例、电解质紊乱1例、死因不明1例.结论 食管癌首程放疗后局部复发采用3DCRT二程放疗是可行的,有较好临床症状缓解率和即时疗效,部分患者可延长生存期;但再程放疗并发症较高,临床应用时应严格掌握其适应证.     

序贯放化疗治疗局部晚期非小细胞肺癌

目的评价局部晚期非小细胞肺癌予NP方案序贯放化疗综合治疗的疗效及耐受性。方法92例局部晚期非小细胞肺癌均分为两组,单纯放疗组采用常规分割放疗,原发灶及转移淋巴结总剂量均为60Gy~70Gy;综合治疗组化疗采用NP方案,先予化疗2周期,随后局部放疗,放疗方法同单放组,放疗期间停止化疗,放疗结束后继续化疗2周期。结果92例均完成治疗计划,随访1年以上。放化疗组总有效率(CR PR)65.2%,CR21.7%;均较单放组高(34.8%,10.9%)。1、2年生存率放化疗组为60.9%和32.6%,而单放组为39.1%,17.4%,两组比较有显著性差异。结论序贯放化疗治疗局部晚期非小细胞肺癌的疗效优于单纯放疗,放射性食管炎、放射性肺炎及骨髓抑制两组比较差异存在显著性,但均可耐受。     

调强放疗在局部晚期及复发甲状腺低分化癌中的应用

背景与目的：甲状腺低分化癌（poorly differentiated thyroid carcinoma,PDTC）是一种罕见恶性肿瘤,易发生侵袭及转移,目前尚缺乏有效的治疗方法。本研究旨在分析调强放疗对局部晚期及复发PDTC的疗效及安全性。方法：纳入2011年2月—2014年9月在复旦大学附属肿瘤医院接受调强放疗的8例局部晚期及复发PDTC患者（均经病理及影像学确诊为T_4a-b期PDTC）。1例术后无残留病灶但包膜侵犯的患者接受处方剂量60 Gy/30次的单纯放疗,其余患者甲状腺原发灶及淋巴结转移灶的处方剂量为66 Gy/33次,并接受顺铂为基础的联合化疗。结果：患者在放疗±化疗后,2例局部区域病灶完全缓解（complete response,CR）,1例部分缓解（partial response,PR）,5例保持疾病稳定（stable disease,SD）。截止末次随访或患者死亡时,局部区域控制率为87.5%（5例CR+1例PR+1例SD）。随访期间共有4例（50%）患者死亡,死亡原因分别为原发病灶进展（12.5%）和肺转移（37.5%）。大部分治疗相关不良反应为1~2级。结论：对于局部晚期及复发PDTC,调强放疗为基础的综合治疗能控制局部区域病灶,延长生存时间,不良反应可控,是一种有效且安全的治疗手段。     

鼻咽粘液表皮样癌——附8例临床分析

目的 探讨分析鼻咽粘液表皮样癌的临床诊治及预后.本文对我院治疗过的鼻咽粘液表皮样癌病例进行综合分析.方法 回顾分析1996年12月～2006年2月收治的8例鼻咽粘液表皮样癌患者.从发病到确诊最短为2.0个月,最长为36.5个月,平均为10.4个月.确诊前5例误诊.鼻咽肿瘤以位于鼻咽侧壁、顶后壁为主,局部多侵犯鼻腔、颅底、咽旁、头长肌.颈部淋巴结转移少见,仅1例.3例采用单一治疗(1例放疗、2例手术),5例综合治疗(2例术前放疗、3例术后放疗).结果 ①近期疗效:治疗结束后3个月,6例达CR其中(5例采取综合治疗),1例达PR(单纯切除 放疗),1例SD(单独放疗);②远期疗效:随访4.2～90.9个月,中位随访期26.9个月,8例患者均存活,其中6例无瘤存活,2例原位带瘤生存.自随访以来,无一例有颈部淋巴结及远处转移.结论 鼻咽粘液表皮样癌进展慢,疾病误诊率高,临床表现以局部症状为主,颈部淋巴结和远处转移少见,治疗上应以手术为主辅以放疗.     

髓外浆细胞瘤的诊断和治疗

目的 探讨髓外浆细胞瘤（EMP）的诊断和治疗。方法 4例髓外浆细胞瘤的临床资料。结果 1例疗后失访。2例分别随访74月和56月，无局部复发、远处转移，无进展证据。1例治疗后6个月复发，18个月时死于多发性骨髓瘤（MM）。结论 EMP的诊断确立必须有组织学结果，并经系统检查除外MM；放疗和手术是EMP的主要治疗手段。     

50例孤立性浆细胞瘤临床分析

目的对孤立性浆细胞瘤(SP)的特点和预后因素进行回顾性分析,以帮助临床诊断、治疗及对预后的判断。方法入组50例SP患者,并对其进行随访,应用Kaplan-Meier法对其临床特点、治疗及预后因素进行回顾性分析。结果髓外浆细胞瘤(EMP)组与骨孤立性浆细胞瘤(SPB)组、放疗组与未放疗组、年龄﹤60岁与年龄≥60岁组的log rank检验结果提示,生存曲线差异均无统计学意义(P均>0.05)。50例SP患者与168例多发性骨髓瘤(MM)患者的生存曲线差异有统计学意义(P<0.05)。结论 SP患者中,EMP与SPB的生存状况无明显差异,SP好发于中老年人,预后较好,部分可转化为MM。     

Primary isolated extramedullary plasmacytoma of mesentry: A rare case report

Extramedullary plasmacytoma (EMP) is an uncommon entity that most commonly involves nasopharynx and upper repository tract. Involvement of GIT occurs in approximate 10% of cases. According to WHO plasma cell tumors have been classified into two main groups: Multiple myeloma and plasmacytoma. Plasmacytoma includes solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. EMP can be either primary without evidence of bone marrow involvement or may occur simultaneously with multiple myeloma representing extramedullary spread of the disease. It may occur in association with multiple myeloma and it may precede, accompany or follow the onset of multiple myeloma. Diagnosis of primary EMP requires the exclusion of associated multiple myeloma as shown by negative Bence Jones Proteins in urine, normal serum electrophoresis, normal bone marrow biopsy, normal skeletal survey and normal calcium levels. Here we present a case of 55-year male who came to Nephrology Department for urinary tract infection and pain abdomen. Patient was referred to Radiology for ultrasonography which revealed bilateral renal parenchymal disease with a well-defined mass in the mesentry which was further confirmed on computed tomography. Patient was surgically operated and diagnosis of primary EMP of mesentry was made on histopathological examination. Only three cases have been reported so far in the literature. Keywords: Extramedullary plasmacytoma, mesentry, plasma cell dyscrasias.     

Plasmacytoma of the Nasolacrimal Duct Simulating Dacryocystitis: An Uncommon Presentation for Extramedullary Relapse of Multiple Myeloma

The most common site for localized forms of plasma cell neoplasms (extramedullary plasmacytoma; EMP) is the upper respiratory tract, including the oropharynx, nasal cavities, sinuses and larynx. A 50-year-old woman with a history of myeloma in complete remission after autologous stem cell transplantation complained of two weeks of epiphora of the left eye with subsequent diplopia, bloody nasal discharge and progressive swelling around the nasolacrimal sac. A solitary mass in the left sinonasal area, extending to the nasolacrimal duct (NLD) was detected on MRI, whose histopathological examination was consistent with plasmacytoma. Further clinical investigation ruled out multiple myeloma (MM). The patient underwent debulking surgery and adjuvant chemotherapy followed by local radiotherapy in an attempt to achieve complete response. Despite being a rare entity, EMP of the NLD should be considered in the differential diagnosis of epiphora and dacryocystitis. To our knowledge, this is the first case of a plasmacytoma of the NLD presenting as isolated extramedullary relapse of MM. The follow-up in EMPs should include appropriate imaging studies, a systemic workup to rule out MM.Key words: Dacryocystitis, Multiple myeloma, Nasolacrimal duct, Plasmacytoma     

Plasmacytoma. Treatment results and conversion to myeloma.

Forty-six cases of solitary plasmacytoma were reviewed for response to radiation and progression to multiple myeloma. Cases were classified as solitary plasmacytomas of bone (SPB) (32 cases) or extramedullary plasmacytomas (EP) (14 cases). There was an overall 93% response rate of the tumor to radiation therapy: 62% had a complete response after radiation therapy, whereas 31% had a partial response. Conversion to multiple myeloma was influenced by the type of plasmacytoma; 53% of the patients with SPB converting to myeloma versus 36% of the patients with EP. Time from diagnosis to conversion for patients with SPB showed no evidence of plateau, with conversion continuing to occur even after 17 years. The median survival time for patients after conversion to myeloma was 14.5 months and was not affected by time to conversion. Serum protein level, presence of monoclonal gammopathy, and size of primary lesion were of some prognostic significance in predicting conversion to myeloma. Adjuvant chemotherapy did not affect the incidence of conversion but did appear to delay conversion to myeloma. Seven patients in whom multiple sequential solitary plasmacytomas developed formed a distinct subset, with a median time to a second plasmacytoma of 63 months. In three of these patients, conversion to myeloma occurred subsequently. This study supports the idea of EP having a lower incidence of conversion to myeloma and a different natural history from SPB, with SPB likely to be multiple myeloma in evolution.     

Dural plasmacytoma as the initial presentation of multiple myeloma: A case report and review of the literature

Dural plasmacytoma is a type of multiple myeloma of the central nervous system. Our patient presented with symptoms of headache. Imaging findings suspected glioblastoma, whereas pathological findings revealed mucosa-associated lymphoid tissue lymphoma associating with plasma cell differentiation. Further in-depth studies confirmed a diagnosis of dural plasmacytoma. This case indicates that morphological variations may occur in the extramedullary involvement of CD20-positive multiple myeloma. The multidisciplinary team contributes to the diagnosis of hematological diseases.     

Solitary plasmacytoma treated with radiotherapy: impact of tumor size on outcome

PURPOSE: Solitary plasmacytoma (SP) is a rare presentation of plasma cell neoplasms. In contrast to multiple myeloma, long-term disease-free survival and cure is possible following local radiotherapy (RT), particularly for soft tissue presentations. In this study, we attempt to identify factors that predict for local failure, progression to multiple myeloma, and disease-free survival (DFS) in patients mainly managed with local RT. METHODS AND MATERIALS: We identified 46 patients referred to the Princess Margaret Hospital between 1982 and 1993. The median age was 63 years (range 35-95), with a male:female ratio of 1.9:1. All patients had biopsy-proven SP (osseous: 32, soft tissue: 14). M-protein was abnormal in 19 patients (41%). All patients were treated with local RT (median dose 35 Gy), with 5 patients (11%) also receiving chemotherapy. Maximum tumor size pre-RT ranged from 0 to 18 cm (median 2.5). RESULTS: The 8-year overall survival, DFS, and myeloma-free rates were 65%, 44%, and 50%, respectively. The local control rate was 83%. Factors predictive of progression to myeloma (and poorer DFS) included bone presentation and older age. However, these two factors did not influence local control, which was affected by tumor size. All tumors < 5 cm in bulk (34 patients) were controlled by RT. Anatomic location did not predict outcome; however, 3 of the 5 tumors arising in paranasal sinuses did not achieve local control. Lower RT dose (< or =35 Gy) was not associated with a higher risk of local failure. CONCLUSION: Solitary plasmacytomas are effectively treated with moderate-dose RT, although osseous tumors have a high rate of recurrence as systemic myeloma. Large tumor bulk locally (> or =5 cm) predicts for local failure. Combined chemotherapy and RT should be investigated in these high-risk patients to increase the local control rate and the cure rate.     

Solitary plasmacytomas: outcome and prognostic factors after definitive radiation therapy

BACKGROUND:

The objective of this study was to review the outcome of patients with solitary plasmacytoma (SP) after definitive radiation therapy.

METHODS:

The authors retrospectively reviewed 84 patients with SP who were diagnosed and treated at The University of Texas MD Anderson Cancer Center during 1988 to 2008. The impact of tumor anatomic site, tumor size, and the presence of serum and urinary paraprotein at diagnosis was assessed on local control, survival, and the risk of developing multiple myeloma (MM).

RESULTS:

Fifty‐nine patients (70%) had bone SP, and 25 patients (30%) had extramedullary SP. Serum paraprotein was present in 39 patients (46%). The median radiation dose was 45 grays (Gy) (range, 36‐53.4 Gy). Local control was achieved in 77 patients (92%). Neither radiation dose nor tumor size predicted local control. The 5‐year rate of progression to MM was 47% and was higher for patients with bone SP (56% vs 30% for extramedullary SP; P = .021), and patients who had serum paraprotein detected at diagnosis (60% vs 39%; P = .016). On univariate analysis, patients aged <60 years and men had higher rates of progression to MM, although the differences were not significant (P = .048 and P = .29, respectively). Multivariate analysis revealed that bone location and serum protein at diagnosis were associated statistically with progression to MM. The 5‐year overall survival rate for the entire patient cohort was 78%, and no difference was observed between patients who had bone SP versus extramedullary SP (76% vs 85%, respectively; P = .274).

CONCLUSIONS:

The current results indicated that definitive radiation therapy for SP can provide excellent local control. Progression to MM remains the main problem and is more common among patients with bone SP and those who have serum paraprotein detected at diagnosis. Cancer 2011;. © 2011 American Cancer Society.     

Renal extramedullary plasmacytoma—One case report

Extramedullary plasmacytoma (EMP) is rare in multiple myeloma, especially kidneys are involved. We report one case, including diagnosis and treatment. The purpose of this paper is to provide an attention on the extramedullary plasmacytoma disease, reducing the misdiagnose with this disease.     

Plasma cell myeloma of the skull base: Report of two cases

Plasma cell myeloma (PCM) of the skull base is rarely encountered in neurosurgical practice. PCM has a wide spectrum of pathology, including a quite benign, solitary plasmacytoma (SPC), and an extremely malignant, multiple myeloma (MM) at the two ends of the spectrum. We have described two patients with PCM of the skull base, of which one harbored SPC, and the other progressed to MM. In case 1, a 46-year-old man presented with left multiple cranial nerve impairments and had a large skull base tumor. Subtotal removal was performed. The specimen and general examination, including bone marrow aspiration, revealed SPC. Postoperatively 50 Gy of external radiotherapy was administered. The patient has no manifestation of MM 24 months after the initial presentation. In case 2, a 53-year-old woman presented with left abducens palsy and had a left petroclival osteolytic mass. Gross total resection was performed. The specimen revealed a plasmablastic tumor, i.e., myeloma. General examination established the diagnosis of MM. She was administrated adjuvant chemotherapy and autologous bone marrow transplantation. She is alive without local recurrence 30 months after the initial presentation.