髓外浆细胞瘤和骨的孤立性浆细胞瘤9例

在浆细胞瘤中，多发性骨髓瘤（mm）常见，而髓外浆细胞瘤（EMP）及骨的孤立性浆细胞瘤（SPB）则罕见。1985—1995年间，作者治疗髓外浆细胞瘤7例，骨的孤立性浆细胞瘤2例。单纯放疗4例，手术加放疗5例。全组3年生存率6／8（7％）。1例SPB在治疗后2年发展为mm，而无1例EMP发生。EMP及SPB治疗后，预后较好。对于预测发展为mm的指标，尚需进一步研究。     

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。     

原发髓外浆细胞瘤的研究进展

髓外浆细胞瘤(EMP)是一种罕见肿瘤,特征是浆细胞单克隆增殖,常好发于头颈部,其次是胃肠及皮肤,活检是唯一准确可靠的诊断依据,并且需要与表现相关疾病相鉴别.因EMP有较高的放疗敏感性,故目前以放疗为首选,也可因病情选择综合治疗,而造血干细胞移植因其移植排斥反应,不作为优先选择治疗方案.     

头颈部髓外浆细胞瘤的放射治疗(附15例报告)

髓外浆细胞瘤(EMP)又称原发性软组织浆细胞瘤,约占浆细胞瘤的4％。国外文献记载近250例左右,国内有近60例的报告。本症属低度恶性肿瘤,多采用手术加放射治疗或单纯放射治疗。我院曾诊治头颈部EMP15例,现报告如下。     

髓外浆细胞瘤与骨单发性浆细胞瘤17例

1977年1月至1988年1月治疗12例髓外装细胞瘤(EMP),5例骨单发性浆细胞瘤(SPB)。单纯放疗10例,手术加放疗5例,手术加化疗2例。照射剂量41～70Gy.60％的SPB在治疗后平均22.3个月发展为多发性骨髓瘤,EMP无发生。16.7％的EMP疗后局部复发,25％的EMP和20％的SPB有区域淋巴结转移。全组3年生存率13/16(81％),EMP与SPB生存率无差异。     

孤立性浆细胞瘤12例

目的 探讨孤立性浆细胞瘤(SP)的临床特点及诊治方法.方法 回顾分析2007年1月至2014年6月收治的12例SP的临床资料.结果 12例患者中6例患者为骨孤立性浆细胞瘤(SBP),6例患者为髓外浆细胞瘤(EMP).12例患者中位年龄为52.5岁(31～ 78岁),男性6例,女性6例;6例EMP中I期1例,Ⅱ期5例;12例患者行手术+化疗+放疗l例,手术+放疗6例,化疗+放疗2例,单纯放疗3例.12例患者近期疗效完全缓解7例,部分缓解4例,稳定l例,总有效率为92 ％;1、3、5年总生存分别为8、3、2例,死亡6例,其中肺部感染死亡3例,肿瘤进展死亡2例,死亡原因不详1例,4例随访期间未出现病情进展,3例发展为多发性骨髓病(MM).结论 SP的整体治疗效果好,但部分患者因发展为MM或者发生肺部感染等原因导致生存率降低.     

气管浆细胞瘤的手术治疗及文献复习

目的 :探讨气管内恶性肿瘤的诊断方法、手术治疗要点、并发症的预防以及髓外浆细胞瘤的诊治特点。方法 :回顾性分析了 1例气管内髓外浆细胞患者的临床资料及相关文献。结果 :本例气管内浆细胞瘤获得了成功切除 ,无任何并发症。结论 :术前对气管内肿瘤的明确定位、术中麻醉插管维持通气及气管的低张力吻合是保证手术成功的关键。髓外浆细胞瘤的发病率较低 ,但可发生于多个器官 ,手术和放疗是其主要的治疗手段     

多发、巨大髓外浆细胞瘤1例

髓外浆细胞瘤（extramedullary plasmacytoma,EMP）是一种少见的浆细胞肿瘤,仅占全身浆细胞瘤的1%～2%,可发生于任何髓外组织或器官,约80%患者发生于上呼吸道,常见于鼻腔鼻窦、鼻咽部、口腔[1]。文献报道的EMP多以单发为主,多发、巨大的髓外浆细胞瘤较罕见,现将我院收治的1例多发、巨大的髓外浆细胞瘤报告如下。     

髓外浆细胞瘤8例临床分析及文献复习

[目的]探讨髓外浆细胞瘤（EMP）的临床特点与治疗方法。[方法]回顾性分析8例EMP的临床资料,并复习相关文献。[结果]EMP好发于头颈部,男性多见,预后相对较好,较易转化为多发性骨髓瘤。[结论]EMP是罕见的低度恶性肿瘤,确诊依靠病理并排除多发性骨髓瘤等,局部治疗是其主要的治疗手段,化疗对于肿瘤分化较差及复发患者有一定的作用。     

髓外浆细胞瘤四例报道并文献复习

 目的 探讨髓外浆细胞瘤的诊断与治疗。方法 回顾分析4例髓外浆细胞瘤的临床资料及复习相关文献。结果 髓外浆细胞瘤预后相对较好。结论 手术加放疗为有效的治疗手段，有不良预后因素者应予以辅助性化疗。     

Extramedullary plasmacytoma of the head and neck region: clinicopathological correlation in 25 cases.

Extramedullary plasmacytomas (EMP) of head and neck are rare tumours. Between 1972 and 1993, 25 cases of EMP of head and neck were seen at our institute. The clinical and pathological features and response to treatment are presented. At initial presentation, 23 (92%) patients presented with disease confined to a single extramedullary site only and two patients had in addition clinical involvement of cervical lymph nodes. All except these two patients received radiotherapy to the primary site only as initial treatment. Initial primary control of local disease was obtained in 16 of 24 (67%) patients treated with radical intent. With salvage treatment of further radiotherapy and/or chemotherapy, local disease control was achieved in 21 of 24 (88%) patients. One patient was treated with palliative intent. Conversion to multiple myeloma was seen in two patients (8%). Pathologically, the tumours were classified into low, intermediate and high grade, which correlated closely with outcome. This classification has been used for the first time in extramedullary plasmacytomas and is based on the multiple myeloma grading criteria devised by Bartl et al (1987). Fifteen of eighteen (83%) low-grade tumours and only one of six (17%) intermediate- and high-grade tumours were locally controlled after primary radiotherapy. This is statistically significant for local control (P= 0.0019) but not for overall survival (P= 0.12). The median survival and 5-year overall survival is 68 months and 58.9% respectively. We recommend consideration of adjuvant chemotherapy in patients with higher grade disease.     

Adjuvant radiotherapy after transoral laser microsurgery for advanced squamous carcinoma of the head and neck

PURPOSE: To evaluate the efficacy of an adjuvant radiotherapy after transoral laser microsurgery for advanced squamous cell carcinoma of the head and neck and to show that a less invasive surgery with organ preservation in combination with radiotherapy is an alternative to a radical treatment. PATIENTS AND METHODS: Between 1987 and 2000, 208 patients with advanced squamous cell carcinoma of the head and neck were treated with postoperative radiotherapy after surgical CO2 laser resection. Primary sites included oral cavity, 38; oropharynx, 88; larynx, 36; hypopharynx, 46. Disease stages were as follows: Stage III, 40 patients; Stage IV, 168 patients. Before 1994, the treatment consisted of a split-course radiotherapy with carboplatinum (Treatment A). After 1994, the patients received a conventional radiotherapy (Treatment B). RESULTS: Patients had 5-year locoregional control and disease-specific survival (DSS) rates of 68% and 48%, respectively. The 5-year DSS was 70% and 44% for Stages III and IV, respectively (p = 0.00127). Patients treated with a hemoglobin level greater or equal to 13.5 g/dL before radiotherapy had a 5-year DSS of 55% as compared with 39% for patients treated with a hemoglobin level greater than 13.5 g/dL (p = 0.0054). CONCLUSION: In this series of patients with advanced head-and-neck tumors, transoral laser surgery in combination with adjuvant radiotherapy resulted in locoregional control and DSS rates similar to those reported for radical surgery followed by radiotherapy. Treatment B has clearly been superior to Treatment A. A further improvement of our treatment regimen might be expected by the combination of adjuvant radiotherapy with concomitant platinum-based chemotherapy.     

Clinical course of solitary extramedullary plasmacytoma.

BACKGROUND AND PURPOSE: Solitary extramedullary plasmacytoma (EMP) represents a rare category of malignant disease on which there are limited data in regard to diagnosis, staging and natural history. This study attempted to clarify the clinical course of solitary extramedullary plasmacytoma after radiation or surgical therapy given with curative intent. MATERIALS AND METHODS: The diagnosis was based on a mass of clonal plasma cells separate from bone or bone marrow without evidence of occult disease elsewhere. Between 1963 and 1996, 22 previously untreated patients with an EMP were diagnosed. Disease presented in the head or neck in 86%, usually in the nasal cavity (NC) or maxillary sinus (MS), and in these areas local bone destruction was found in 10 of 11 patients. Among all patients, serum myeloma protein was present in three patients (14%) and Bence Jones protein alone was found in two patients (9%). Radiation therapy was the sole treatment for 18 of 22 patients, and the median radiotherapy dose was 50 Gy (range, 40-60 Gy); five of seven patients with an EMP of oral cavity (OC), oropharynx (OP), nasopharynx (NP), parotid or larynx also received elective neck irradiation. Two patients underwent surgery plus postoperative irradiation of a plasmacytoma of the sigmoid colon or pleura, and two patients had resection alone of a plasmacytoma of the colon or cervical lymph node. RESULTS: Local control was achieved in 21 of 22 patients (95%), and disease never recurred in regional nodes. Disappearance of myeloma protein occurred in three of five patients with an evaluable abnormality. Multiple myeloma developed in seven patients (32%), all within 5 years. The 5-year rate of freedom from progression to multiple myeloma was 56% and the median survival was 9.5 years. CONCLUSION: Radiation therapy achieved excellent locoregional control of EMP with an approximate cure fraction of 50%.     

Effect of definitive radiotherapy on the long‐term outcome in patients with solitary extramedullary plasmacytoma

Extramedullary solitary plasmacytoma (EMP) is a rare type of malignancy. This paper presents a retrospective review of the experience with EMP at the Krakow Oncology Center. Records of 17 patients with head and neck EMP, treated with definitive radiotherapy between 1976 and 2009, were analyzed. The total tumour dose ranged from 45 to 70 Gy (median 56 Gy). In four patients with partial response after radiotherapy, adjuvant melphalan‐based chemotherapy was applied. The median follow‐up period was 8.6 years. The treatment was well tolerated. The estimated 10‐year overall survival, disease‐free survival, and multiple myeloma‐free survival were 68.4%, 49.3%, and 55%, respectively. The 10‐year local control rate was 90.9%. No in‐field local recurrence was observed. During the follow‐up, progression into multiple myeloma was observed in five patients, with a mean time to conversion of 24 months. The only factor adversely affecting overall survival on univariate analysis was the age >56 years, whereas a complete tumour regression after radiotherapy was associated with a significant improvement in both disease‐free survival and multiple myeloma‐free survival. Despite the high effectiveness of local radiotherapy, there is still a significant treatment failure risk due to the EMP conversion into generalized disease. An attempt to identify prognostic factors may facilitate selection of patients with a high risk of progression to multiple myeloma. Copyright © 2015 John Wiley & Sons, Ltd.     

Desmoplastic neurotropic melanoma: a clinicopathologic analysis of 128 cases

BACKGROUND: Several studies have suggested that desmoplastic neurotropic melanoma (DNM) is associated with higher local recurrence rates than other types of melanoma. The authors investigated the local recurrence rates for patients with DNM after surgery alone or surgery followed by radiotherapy (RT). METHODS: One hundred twenty-eight patients with DNM were treated at the Sydney Melanoma Unit and the Sydney Cancer Center from 1996 to 2007. All patients underwent local excision, 27 patients also received RT. For both groups, clinical and pathologic features, treatment details, and local recurrence data were analyzed. RESULTS: The median age at diagnosis was 65.5 years. The ratio of men to women was 2.7:1. The head and neck was the most common location (51%). The median Breslow thickness was 4 mm, and 99% of patients had Clark Level IV or V primary tumors. Patients who received adjuvant RT had thicker tumors (P = .003), deeper Clark level invasion (P < .001), and narrower excision margins (P < .001). There were 8 local recurrences, including 6 (6%) in the surgery only group and 2 (7%) in the adjuvant RT group. A positive margin (P < .001) and head and neck location (P = .03) were significant predictors of local recurrence. CONCLUSIONS: The local recurrence rate in this series was lower than the rates reported in historic control groups and in the authors' previous temporal cohort. The results indicated that clear surgical margins are of paramount importance in minimizing local recurrence; when margins are compromised, the addition of RT may reduce local recurrence rates compared with historic controls. A prospective randomized trial is needed to quantify the risk reduction with adjuvant RT.     

Radiotherapy for head and neck cancer in elderly patients.

BACKGROUND AND PURPOSE: Elderly patients with head and neck cancer may not be treated aggressively with radiotherapy, due to concerns regarding tolerance of treatment and toxicity. A retrospective study was undertaken of patients aged 80 years and over, treated by definitive radiotherapy for head and neck cancer. MATERIAL AND METHODS: 98 patients aged 80-92 received radiotherapy for carcinoma of the head and neck between 1991 and 1995. All patients received beam directed radiotherapy with radical intent using an immobilisation shell. RESULTS: Cancer specific survival was 59% and overall local control was 70% at 5 years. Both were significantly affected by T stage and site of disease. Cancer specific survival was comparable to that of patients aged below 80 years. Seven patients died within 6 months of the treatment. Three patients developed severe late toxicity. Metastatic disease occurred in eight patients. CONCLUSIONS: Radiotherapy is a beneficial and well tolerated treatment in elderly patients with carcinoma of the head and neck.     

Soft tissue angiosarcomas

From 1949 to 1979, 12 patients with soft tissue angiosarcoma received radiotherapy (alone or in combination with other modalities of treatment) with curative intent at The University of Texas M.D. Anderson Hospital and Tumor Institute. The primary site was the head and neck in six patients (scalp, four; maxillary antrum, one; and oral tongue, one), the breast in four patients, and the thigh in two patients. All four patients with angiosarcoma of the scalp had advanced multifocal tumors, and two of them had clinically positive neck nodes. None of these tumors were controlled locally, and local recurrences occurred within and/or at a distance from the generous fields of irradiation. The remaining two patients with head and neck lesions had their disease controlled by surgery and postoperative irradiation. Three of the four angiosarcomas of the breast were primary cases which were treated by a combination of surgery (excisional biopsy, simple mastectomy, radical mastectomy) and postoperative irradiation. One patient also received adjuvant chemotherapy. The fourth patient was treated for scar recurrence after radical mastectomy. All four patients had their disease locally controlled, and two of them have survived over 5 years. The two patients with angiosarcoma of the thigh were treated by conservative surgical excision and postoperative irradiation. One patient had her disease controlled; the other had a local recurrence requiring hip disarticulation and subsequent hemipelvectomy for salvage.     

头颈部恶性纤维组织细胞瘤21例临床分析

背景与目的;恶性纤维组织细胞瘤是一类组织结构较复杂的多形性肿瘤,其组织来源和是否应定为独立的疾病尚存在争议,既往文献常将其混同于其他类型恶性肿瘤。本文旨在研究恶性纤维组织细胞瘤的临床及病理特点,以探讨其最佳诊疗方法。方法：回顾性分析我科1984年6月至1999年6月收治的经病理检查证实的头颈部恶性纤维组织细胞瘤患者21例的病例资料。结果：本研究的病例中除有1例失访(按死亡计算)外,其余均已随访3年以上,3年生存率为42．9％(9／21)。采用非手术治疗的2例,因就诊时即已相对晚期,疗效不佳,分别在就诊后2个月、5个月死亡;采用手术治疗的19例,6例术后3年内无复发,13例术后2年内局部复发,9例发生转移,其中7例经再次手术,3例已生存3年以上。21例中,12例术后证实有颈淋巴结转移,颈部淋巴转移率为57．1％。结论：头颈部恶性纤维组织细胞瘤是一类复发率较高的恶性肿瘤,治疗以扩大根治术为首选方法,单独放疗或化疗效果不佳。对术后复发的病例,不应放弃再次手术的机会。     

Treatment results of regional metastasis from cutaneous head and neck squamous cell carcinoma.

AIMS: To investigate the results of our treatment policy, we present our institutional experience in the management of regional neck node metastases of cutaneous head and neck squamous cell carcinoma (CHNSCC). METHODS: Between 1977 and 1997, 343 patients presented with CHNSCC, of whom 41 were treated for regional metastases, with parotidectomy and/or (selective) neck dissection. Histological analysis was performed on all operation specimens. Patients with multiple nodes and/or extra capsular rupture, at risk for recurrence, received adjuvant radiotherapy. RESULTS: Seventy-six percent of the regional metastases occurred within the first 2 years, but a delay of more than 5 years was also observed. Parotid gland (56%), neck levels II (39%) and V (22%) were most frequently involved. Twenty-four percent of patients treated with curative intent failed at the regional site. Five years overall survival was 46%, with a median survival of 49 months. No survival differences emerged between patients treated by surgery alone and patients receiving adjuvant radiotherapy (P=0.14). Five patients died of disease, of whom three with distant metastases. CONCLUSION: Regionally metastasized CHNSCC is a serious disease with a high risk of regional recurrence, calling for combined surgery and adjuvant radiotherapy. A small subset of patients with a long interval between completion of primary treatment and presence of regional nodes justifies a long follow-up.     

36例下咽后壁鳞状细胞癌的临床分析

背景与目的:下咽后壁鳞状细胞癌临床少见。本研究旨在探讨下咽后壁鳞状细胞癌的治疗方式及下咽部的一期修复与功能恢复方法。方法:回顾性分析中国医学科学院肿瘤医院头颈外科与放射治疗科1967年6月～2000年1月收治的36例下咽后壁鳞状细胞癌(1997年UICC分期:I期4例;II期4例;Ⅲ期9例;Ⅳ期19例)的临床资料,其中男性24例,女性12例。采用综合治疗(手术加放射)20例,根治性放疗10例,单纯手术3例,根治性放疗后挽救手术3例(2例颈部、1例颈部与下咽)。一期修复所用组织有胃、空肠、结肠、胸大肌、前臂皮瓣、残喉、胃网膜、颈阔肌皮瓣、斜方肌皮瓣等器官与皮瓣。结果:综合治疗、根治性放疗的5年生存率分别为40%(8/20)、30%(3/10)。单纯手术3例3年内2例死亡,1例失随。挽救手术3例均3年内死亡。对原发灶24例手术治疗患者中留喉下咽后壁局部切除4例(16.7%);一期修复20例(83.3%),其中利用胃、空肠器官修复11例(55%),其它修复9例(45%)。一期修复手术无围手术期死亡,并发症发生率为15%(3/20),修复有效率为85%(17/20),平均进食时间为2～3周。修复患者中局部复发3例。结论:综合治疗在下咽后壁鳞状细胞癌中疗效肯定,治疗的重点为下咽吞咽功能的恢复。手术一期重建下咽部,恢复患者吞咽功能,不增加患者预后的风险。胃、空肠代下咽是重要的修复手段。部分患者可选择局部切除,保留喉功能。