上皮样肉瘤临床诊治分析

背景与目的:上皮样肉瘤是一种少见的软组织肉瘤, 多表现为肢体远端的结节或溃疡性病变,可通过腱鞘局部播散、淋巴道和血道转移.因其切除后易复发转移、有较高的淋巴结转移率而区别于其他的软组织肉瘤.本文通过分析上皮样肉瘤临床特点,治疗效果,生存情况,预后因素,旨在探讨其治疗方法及预后的影响因素等.方法:回顾我院1999-2007年收治的获得随访的18例上皮样肉瘤,对其临床表现、淋巴结转移、治疗情况、生存率及预后进行分析.结果:上皮样肉瘤5年生存率为41%,局部复发率为44.4%.即使截肢,术后仍有复发.在11例行淋巴结清扫的患者中淋巴结转移率为63.6%.结论:上皮样肉瘤是一种侵袭性较强、淋巴结转移率高、术后易复发的软组织肉瘤,手术应充分考虑切除的广泛性.     

外阴上皮样肉瘤的临床分析

目的 探讨外阴上皮样肉瘤的临床特点、诊断治疗方法及预后.方法 对我院收治的4例和文献报道资料完整的16例外阴上皮样肉瘤患者进行回顾性分析.结果 20例外阴上皮样肉瘤患者年龄23～80岁,中位年龄36岁.肿物直径为1～10 cm,中位数为5.1 cm.免疫组化检测结果显示,20例外阴上皮样肉瘤患者vimentin阳性,19例CK阳性,16例EMA阳性.10例患者行双侧或同侧腹股沟淋巴结清扫,3例未行腹股沟淋巴结清扫.术后7例患者未补充治疗,13例患者行术后放疗和(或)化疗.20例外阴上皮样肉瘤患者中,无复发及转移11例(55.0%),腹股沟淋巴结转移治疗后存活2例(10.0%),死亡7例(35.0%).早期患者(Ⅰ～Ⅱ期)的中位生存时间(21个月)高于晚期患者(6个月,P＜0.05).10例行双侧或同侧腹股沟淋巴结清扫患者的中位生存时间为11.5个月,3例未行腹股沟淋巴结清扫患者的中位生存时间为6个月,差异无统计学意义(P=0.086).结论 外阴上皮样肉瘤误诊率较高,确诊时需要结合免疫组化检查,诊断上皮样肉瘤的标记性蛋白为vimentin、EMA和CK.外阴上皮样肉瘤的有效治疗方法为局部广泛切除加双侧腹股沟淋巴结清扫,手术切缘至少2 cm,化疗和放疗的疗效尚不确切.     

乳腺癌保乳手术治疗的临床研究

目的:总结保乳手术治疗早期乳腺癌临床经验及观察近期疗效.方法: 2000年1月至2006年12月采用肿块局部广泛切除加腋淋巴结清扫或象限切除加腋淋巴结清扫治疗临床单发的、肿瘤直径小于3.0cm、无区域淋巴结转移的女性乳腺癌患者51例,手术切缘距瘤缘2.0cm～3.0cm,术后行辅助放疗、化疗及内分泌治疗.结果: 全组随访3～84个月,局部无复发或远处转移.结论: 早期乳腺癌病人接受保乳手术治疗可以取得满意的临床和美容效果.     

隐匿性乳腺癌的诊治探讨

目的探讨隐匿性乳腺癌的临床特点及诊治方法。方法对9例隐匿性乳腺癌患者的临床资料及疗效进行回顾性分析。结果术后9例患者中6例找到乳腺癌原发灶（3~8 mm）,随访1~8年,7例行乳腺癌改良根治术的患者中5例未出现局部复发或远处转移,1例术后1年发现骨转移灶,另1例术后1.5年死于脑、肺及骨转移;2例行保乳术的患者均无局部复发或远处转移。结论对有腋窝淋巴结转移腺癌而无明显原发癌灶的女性患者,应高度警惕隐匿性乳腺癌。治疗宜采用保乳术联合腋窝淋巴结清扫术,辅以全乳腺及区域淋巴结引流区放疗、化疗、内分泌治疗以及可能的靶向治疗。     

鼻咽癌放疗后颈部淋巴结残留复发的手术治疗

目的:探讨鼻咽癌放疗后颈部淋巴结残留或复发的治疗方法。方法:对我科1994~2002年21例鼻咽癌放疗后颈部淋巴结残留或复发的病人手术治疗总结,18例行改良根治性颈淋巴结清扫术,2例行扩大根治性颈淋巴结清扫术,1例行姑息性颈淋巴结切除术。结果:21例病人3年生存率52.4%,5年生存率33.3%。结论:鼻咽癌放疗后颈淋巴结残留复发手术治疗能提高患者生存率。     

隆突性皮肤纤维肉瘤诊治方法及临床预后分析

目的 探讨隆突性皮肤纤维肉瘤的诊断、治疗方法,分析影响预后的因素.方法 对35例经病理证实隆突性皮肤纤维肉瘤的诊治情况进行回顾性分析.结果 局部切除术10例,扩大切除术21例,扩大切除术+植皮或转移皮瓣4例.其中局部切除术+扩大切除术共24例,并且行术后辅助放疗,照射剂量50～70 Gy,11例未行放疗,全组复发率22.8%,5年无复发生存率71.2%,10年无复发生存率58.5%,术后放疗组与单纯手术组的5年无复发生存率分别为89.2%、59.2%,10年无复发生存率分别为81.1%、43.2%.结论 扩大切除术+辅助放疗是治疗隆突性皮肤纤维肉瘤的主要治疗方法,手术范围大小及术后辅助放疗与否是影响隆突性皮肤纤维肉瘤的独立预后因素.     

上皮样肉瘤6例诊治分析

目的 探讨上皮样肉瘤的诊断、治疗方法及预后因素.方法 通过对6例的治疗体会和国内外文献的复习,进行总结分析.结果 3例术后复发;3例发生淋巴结转移;3例发生远处转移.中位生存期21月,1、2、5年生存率分别为66.7%、33.3%、16.7%,最终死亡率为100%.结论 上皮样肉瘤临床罕见,确诊依靠病理学检查,局部广泛切除加辅助性放疗及化疗为主要治疗方法,性别、病程长短、肿瘤大小及部位、术式等因素均影响预后.     

乳腺癌保乳手术治疗的临床研究

目的：总结保乳手术治疗早期乳腺癌临床经验及观察近期疗效。方法：2000年1月至2006年12月采用肿块局部广泛切除加腋淋巴结清扫或象限切除加腋淋巴结清扫治疗临床单发的、肿瘤直径小于3．0cm、无区域淋巴结转移的女性乳腺癌患者51例，手术切缘距瘤缘2．0cm～3．0cm，术后行辅助放疗、化疗及内分泌治疗。结果：全组随访3～84个月，局部无复发或远处转移。结论：早期乳腺癌病人接受保乳手术治疗可以取得满意的临床和美容效果。     

腺泡状软组织肉瘤的临床和预后分析

目的探讨腺泡状软组织肉瘤(ASPS)的临床特点、治疗方法和预后情况。方法58例ASPS患者除6例就诊时即发现远处转移未行手术治疗外,其余52例均行手术治疗,其中19例行局部切除术,33例行扩大切除术。19例术后接受辅助性放疗或化疗。58例患者均获得随访,中位随访时间为52个月。结果50例肿瘤完整切除患者中,11例(22.0%)出现局部复发。全组58例患者中,31例(53.4%)发生肺转移。全组总的3、5和10年生存率分别为89.5%、74.1%和57.7%。中位生存时间为125个月。男性患者的3、5和10年生存率分别为79.6%、67.2%和49.7%,女性患者分别为100.0%、81.6%和65.3%(P=0.026)。结论ASPS恶性程度不高,肿瘤生长较为缓慢,局部复发率不高,但远处转移较为常见,肺是其最常见的转移器官。手术切除仍是目前治疗局限期ASPS的惟一有效手段。术后辅助性放化疗对控制局部复发和远处转移效果并不令人满意。女性患者的预后好于男性。     

上皮样肉瘤的诊治分析

目的 分析上皮样肉瘤(ES)的临床特点、治疗模式及预后.方法 回顾性分析1995年3月至2008年12月间天津医科大学附属肿瘤医院收治的13例ES患者的临床资料、治疗和随访情况.其中男性10例,女性3例,平均年龄41.5岁.肿瘤发生于上肢6例,下肢4例,腰背部3例.全组除1例患者因远处血行转移行姑息化疗外,其余12例均行手术治疗.术后单纯放疗4例,单纯化疗5例,放疗+化疗1例.采用SPSS 16.0统计软件包,以Kaplan-Meier法进行生存分析.结果 13例ES患者中,12例为单发肿物,肿物平均直径为(6.07±1.34) cm;有6例(46.2％)患者发现区域淋巴结转移.10例随访患者中,5例(50.0％)出现肿瘤局部复发,3例(30.0％)出现远处转移,其中以肺转移最常见;4例患者于术后2年内死亡.全组患者术后l、2、5、10年生存率分别为72.7％、54.5％、27.3％和9.1％,中位生存时间为27个月.结论 ES临床罕见,易发生局部复发、淋巴结扩散和(或)远处转移,患者预后不佳.明确诊断需病理学检查,手术切除仍是ES的主要治疗方式.     

Epithelioid sarcoma: Still an only surgically curable disease

BACKGROUND: Epithelioid sarcoma is a rare soft tissue sarcoma with a known high propensity for locoregional recurrence and distant metastases. The clinical behavior and prognostic factors that influence the survival of patients with epithelioid sarcoma were studied. METHODS: Twenty-three patients, including 16 men (70%) and 7 women (30%), who were treated for epithelioid sarcoma between 1979-2003 at the University Medical Center Groningen and Radboud University Nijmegen Medical Center, were reviewed retrospectively. The median age at diagnosis was 22 years (range, 1-54 years). At the time of diagnosis, 11 patients (48%) had metastases. Six patients with distant metastasis and 1 patient with an unresectable tumor received palliative treatment (30%). The remaining 16 patients underwent surgical treatment of local disease (11 patients) or locoregional disease (5 patients). Five patients in that group received isolated limb perfusion with tumor necrosis factor and melphalan. RESULTS: The 5-year and 10-year disease-free survival rates for all patients were 34% and 17%, respectively; for the 16 patients who received curative treatment, both rates were 56%. In the latter group, 8 patients developed local recurrence (50%) after a median follow-up of 4 months (range, 1-14 months). Nine patients were disease free after a median follow-up of 50 months (range, 6-290 months). Tumor size >5 cm (P < .0026) at diagnosis and local recurrence (P < .0008) were significant predictors of survival. CONCLUSIONS: The prognosis for patients with epithelioid sarcoma is poor, because a substantial number of patients present with extensive disease, lymph node metastases, and/or distant metastases. Treatment consists of radical surgical excision of the tumor and, if indicated, therapeutic lymph node dissection. In patients who have large tumors, isolated limb perfusion may be useful.     

Sentinel node biopsy in soft tissue sarcoma subtypes with a high propensity for regional lymphatic spread—results of a large prospective trial

BackgroundThe role of sentinel lymph node biopsy (SLNB) in soft tissue sarcoma patients has yet to be determined. We sought to evaluate the role of SLNB in the treatment of patients with clear cell sarcoma (CCS), synovial sarcoma (SS), epithelioid sarcoma (ES) and rhabdomyosarcoma (RMS).Patients and methodsSixty-two consecutive patients without history of regional lymphatic spread or evidence of distant metastases underwent SLNB.ResultsPositive sentinel nodes were identified in 2 out of 42 patients with SS and in 6 out of 12 patients with CCS. Only two CCS patients had further metastatic nodes in regional dissection. Both of these patients, along with another CCS patient, developed distant metastases and ultimately died of disease. The remaining three CCS patients are disease-free in follow-up. One patient with SS and another with ES developed regional lymph node metastases following a negative SLNB, while a further patient with RMS developed distant metastases followed by a local recurrence with regional metastases shortly after.ConclusionsSLNB is an important diagnostic tool for patients with CCS, who appear to have a high rate of clinically occult regional lymph node metastases at diagnosis. For SS patients, SLNB appears to be of very little relevance.     

Low rates of loco-regional recurrence following extended lymph node dissection for gastric cancer

Aim

The study by MacDonald et al. [Chemoradiotherapy after surgery compared with surgery alone for adenocarcinoma of the stomach or gastroesophageal junction. N Engl J Med 2001;345:725–30] has reported low loco-regional recurrence rates (19%) after gastric cancer resection and adjuvant radiotherapy. However, the lymph node dissection was often “inadequate”. The aim of this retrospective study is to analyse if an extended lymph node dissection (D2) without adjuvant radiotherapy may achieve comparable loco-regional recurrence rates.

Methods

A prospective database of 200 patients who underwent a curative resection for gastric carcinoma from January 2000 to December 2006 was analysed. D2 lymph node dissection was standard. Recurrences were categorized as loco-regional, peritoneal, or distant. No patients received neoadjuvant or adjuvant radiotherapy.

Results

The in-hospital mortality rate was 1% (2 patients). The mean number of dissected lymph nodes was 25.9. Overall and disease-free survival at 5 years were 60.7% and 61.2% respectively. During the follow-up, 60 patients (30%) have recurred at 76 sites: 38 (50%) distant metastases, 25 (32.9%) peritoneal metastases, and 13 (17.1%) loco-regional recurrences. The loco-regional recurrence was isolated in 6 patients and associated with peritoneal or distant metastases in 7 patients. The mean time to the first recurrence was 18.9 (95% confidence interval: 15.0–21.9) months.

Conclusions

Extended lymph node dissection is safe and warrants low loco-regional recurrence rates.     

Dermatofibrosarcoma protuberans: treatment and prognosis.

Dermatofibrosarcoma protuberans (DFSP) is a rare tumor of the skin with a strong tendency to recur locally. Nineteen cases of DFSP are presented. In eight of them a total of 20 local recurrences occurred, in five after irradical and in three after 'narrow' excisions. After wide excision (greater than 2 cm) for primary or wide re-excision for recurrent tumor, all patients remained free of tumor with a mean follow-up of 13.2 years (range 2-28 years). An extensive literature review revealed 913 cases of DFSP. The overall recurrence rate is about 50%; after adequate wide excision, 13%. Recurrent tumor is safely treated by wide re-excision and the recurrence rate is then 12%. Regional and distant recurrences are infrequent. Eleven cases (1%) were reported to have regional lymph node metastases and 37 (4%, 17 of whom were histologically confirmed) distant metastases, principally in the lung. The prognosis after appearance of regional or distant recurrence is bad. The role of radiotherapy in the management of this tumor is unclear. Primary or recurrent DFSP is best treated by surgical excision with a minimal margin of 2- preferably 3-cm of surrounding skin including the underlying fascia. Elective lymph node dissection is not advised.     

Adjuvant irradiation for cervical lymph node metastases from melanoma

BACKGROUND: The risk of regional disease recurrence after surgery alone for lymph node metastases from melanoma is well documented. The role of adjuvant irradiation remains controversial. METHODS: The medical records of 160 patients with cervical lymph node metastases from melanoma were reviewed retrospectively. Of these, 148 (93%) presented with clinically palpable lymph node metastases. All patients underwent surgery and radiation to a median dose of 30 grays (Gy) at 6 Gy per fraction delivered twice weekly. Surgical resection was either a selective neck dissection in 90 patients or local excision of the lymph node metastasis in 35 patients. Only 35 patients underwent a radical, modified radical, or functional neck dissection. RESULTS: At a median follow-up of 78 months, the actuarial local, regional, and locoregional control rates at 10 years were 94%, 94%, and 91%, respectively. Univariate analysis of patient, tumor, and treatment characteristics failed to reveal any association with the subsequent rate of local or regional control. The actuarial disease-specific (DSS), disease-free, and distant metastasis-free survival (DMFS) rates at 10 years were 48%, 42%, and 43%, respectively. Univariate and multivariate analyses revealed that patients with four or more involved lymph nodes had a significantly worse DSS and DMFS. Nine patients developed a treatment-related complication requiring medical management, resulting in a 5-year actuarial complication-free survival rate of 90%. CONCLUSIONS: Adjuvant radiotherapy resulted in a 10-year regional control rate of 94%. Complications for all patients were rare and manageable when they did occur. The authors recommend adjuvant irradiation for patients with extracapsular extension, lymph nodes measuring 3 cm in size or larger, the involvement of multiple lymph nodes, recurrent disease, or any patient having undergone a selective therapeutic neck dissection.     

Merkel cell carcinoma

Opinion statement Merkel cell carcinoma is a rare cutaneous neoplasm most commonly affecting the head and neck of elderly white patients. Even with treatment, Merkel cell carcinoma has a strong propensity toward local recurrence, lymphatic spread, and distant metastasis. Because of its rarity and the subsequent lack of well-controlled clinical trials, no single standard of care exists for the treatment of this aggressive tumor. In our institution, primary lesions are excised with wide margins or by Mohs’ micrographic surgery. After local removal, the excision site is treated locally with external radiation therapy. Sentinel lymph node mapping and biopsy are performed. Patients with tumor within a sentinel lymph node undergo lymph node dissection and radiation to the lymphatic basin. Adjuvant chemotherapy is offered to high-risk patients with local disease and to patients with metastases. Patients with distant metastases are treated with a combination of salvage chemotherapy and radiation therapy.     

Breast sarcoma. A clinicopathologic review of 25 cases

Sarcoma of the breast represents less than 1% of primary mammary malignancies; this study reports 25 such cases. The largest group had malignant fibrous histiocytoma (44%), followed by liposarcoma (24%) and fibrosarcoma (16%). Also represented were clear cell sarcoma, neurogenic sarcoma, leiomyosarcoma, and alveolar soft part sarcoma (4% each). Of 19 patients treated by wide local excision or simple mastectomy with or without adjuvant radiotherapy, 11 had local recurrence develop, of which one patient died and nine of the remaining ten had metastases develop. Of the remaining eight patients in this group with no local recurrence, only two had metastases develop. Of the six patients treated by radical or Patey mastectomy, none had local recurrence develop, but two died of metastases. No patient had metastases develop more than 5 years after diagnosis. Regional lymph node involvement with tumor was observed in only one patient (with malignant fibrous histiocytoma) despite regional lymphadenopathy in seven. The overall mortality at 5 years is 64% but does not increase thereafter. The authors' findings suggest that failure to establish local control is associated with a poor prognosis and that wide local excision or simple mastectomy does not provide sufficient clearance to be used as first-line treatment. Excision of the axillary lymphatics and adjuvant radiotherapy are unlikely to be beneficial.