肛管直肠恶性黑色素瘤的诊治

目的 探讨肛管直肠恶性黑色素瘤的临床特点，诊断，治疗及预后。方法 对近21年来经手术及病理证实的11例肛管直肠恶性黑色素瘤临床特征，治疗方法及预后进行回顾性分析。结果 11例中7例有不同程度的便，肛周疼痛等肛门症状，术前误诊7例。全组均行腹会阴联合切除术，术后平均生存18个月，最长存活39个月。结论 肛管直肠恶性黑色素瘤恶性程度极高，死亡率高，较早发生淋巴和血行转移，宜 采用根治手术，辅以化疗及生物治疗的综合性治疗。     

原发性结直肠上皮样肉瘤的临床特征分析

目的了解原发性结直肠上皮样肉瘤的临床特点。方法回顾性分析我院收治及文献个案报道的共32例原发性结直肠上皮样肉瘤的临床资料。结果男女发病比例均等,平均发病年龄64岁;首发症状以下腹疼痛为最常见,其次为便血和大便性状改变,偶见便秘、体重下降等。肿瘤好发于结肠;肿瘤直径2~24cm;同时性和异时性转移,均以肝转移最常见,腹腔淋巴结转移其次;手术治疗以根治术为主;中位生存时间11.4个月。结论原发性结直肠上皮样肉瘤无特征性表现,恶性程度较高,首选手术治疗,预后效果不佳。     

肛管直肠恶性黑色素瘤26例临床分析

目的总结肛管直肠恶性黑色素瘤的临床特征、诊断及治疗经验。方法回顾性分析1977-2005年江苏省肿瘤医院收治的26例肛管直肠恶性黑色素瘤病人的临床资料。结果临床表现为便血23例(88.5%),肛门疼痛或不适14例(53.8%),肛门肿物7例(26.9%)。肿瘤距肛缘5cm以内26例(100%)。首次就诊误诊率84.6%(22/26),术前病理确诊率44.0%(11/25)。免疫组化示HMB45、S100、Vimentin阳性率分别为85.7%(6/7)、100%(7/7)、83.3%(5/6)。累积生存1、3、5年分别为12、4、3例。结论肛管直肠恶性黑色素瘤少见,易误诊,预后差,便血是最常见的症状,直肠指诊对该病的诊断十分重要,免疫组化有助确诊。     

肛管直肠恶性黑色素瘤的诊断和治疗（附9例分析）

目的 探讨肛管直肠恶性黑色素瘤的临床特点。方法 回顾性分析1996～2003年我科收治的9例肛管直肠恶性黑色素瘤的临床特点、诊治方法和预后。结果 肛管直肠恶性黑色素瘤最常见的临床表现是便血(78％)。本组行腹会阴联合切除术5例，局部广泛切除术2例，随访发现7例均在术后2年内发生局部复发或远处转移。最长生存32个月，2例晚期患者存活不到3个月。结论 肛管直肠恶性黑色素瘤临床少见，容易误诊漏诊，外科手术是首选的治疗方法，但是无论Miles’术还是局部广泛切除术，术后生活质量和生存期均不理想，提高治疗水平的唯一途径是早期诊断和早期规范治疗。     

肛管直肠恶性黑色素瘤6例临床分析

目的探讨肛管直肠恶性黑色素瘤的临床特点、诊治方法和预后。方法对我科2003～2009年收治的6例肛管直肠恶性黑色素瘤的相关资料进行回顾性分析。结果本组经腹直肠肛门切除术5例,1例生存至今48个月,4例分别于术后22、23、31、34个月发生局部复发或远处转移死亡;放弃手术1例,6个月后死亡。结论肛管直肠恶性黑色素瘤临床少见,容易误诊漏诊,外科手术是首选的治疗方法,术后生活质量和生存期均不理想,提高治疗水平的唯一途径是早期诊断和早期规范治疗。     

原发性肛管直肠恶性黑色素瘤病例1例并文献复习

目的:探讨原发性肛管直肠恶性黑色素瘤(PAMM)的临床及病理特征、诊断和治疗方法。方法:回顾性分析1例原发性肛管直肠恶性黑色素瘤患者的病历资料,结合国内外文献,总结PAMM的临床和病理特点、诊断和治疗方法。结果:该患者术前肠镜提示距肛缘4cm处肿块,肠镜下活检病理提示恶性黑色素瘤,行腹部会阴切除术,术后病理再次证实肛管直肠恶性黑色素瘤。术后患者顺利出院。结论:PAMM是非常罕见的恶性肿瘤,临床表现缺乏特异性,确诊需要病理诊断。预后差,手术切术是治疗原发性肛管直肠黑色素瘤的最佳方案。     

肛管直肠恶性黑色素瘤临床病理误诊分析

目的分析肛管直肠恶性黑色素瘤临床及病理误诊的原因 ,探讨避免误诊的办法 .方法对 15例肛管直肠恶性黑色素瘤进行回顾性分析和免疫组化观察 .结果临床误诊率为 86 7% (13/15).其中误诊为良性病变占 66 7% (10/15),误诊为痔或痔伴有肛周脓肿 7例、息肉 2例及慢性炎症 1例; 3例误诊为肛管直肠癌 .延误诊治时间 3个月至 1年 .活检病理误诊率为 53 3% (8/15),其中误诊为低分化腺癌 6例 ,平滑肌肉瘤、类癌各 1例 . 5例无色素性恶性黑色素瘤全部误诊 .结论肛管直肠恶性黑色素瘤临床误诊率高 ,病理活检难以准确分型 .临床和病理医生应密切配合 ,作直肠指诊 ,及时活检;对病理标本多作切片 ,仔细寻找黑色素颗粒并对可疑标本进行免疫组织化学检查 ,有助明确诊断 .     

肛管直肠恶性黑色素瘤

目的　总结肛管直肠恶性黑色素瘤的诊断与治疗经验。　方法　回顾性分析 10例肛管直肠恶性黑色素瘤的临床病例资料。　结果　出现症状至确诊时间平均 8( 2～ 12 )个月 ,首诊确诊 4例 ,误诊 6例。手术 9例 ,8例肿瘤直径 >3cm。其中 8例行腹会阴联合根治术 ,1例以直肠息肉、肛乳头肥大行局部切除 ,1例放弃手术仅行化疗 ,3月后死于肝、肺腹股沟等广泛转移。术后 8例行化疗 ,2例辅助放疗 ,化放疗联合应用 1例。术后 2年内死亡 5例 ,余 4例存活 ,存活最长者已超过 6年。　结论　肛管直肠恶性黑色素瘤恶性程度高 ,早期行腹会阴联合根治术是最优选择。长期生存有赖于早期治疗以及治疗方法的改进     

肛管直肠恶性黑色素瘤临床病理误诊分析

目的 分析肛管直肠恶性黑色素瘤临床及病理误诊的原因，探讨避免误诊的方法。方法 对１５例肛管直肠恶性黑色素瘤进行回顾性分析和免疫组化观察。结果 临床误诊率为８６．７％（１３／１５）。其中误诊为良性病变占６６．７％（１０／１５），误诊为痔或痔伴有肛周脓肿７例、息肉２例及慢性炎症１例；３例误诊为肛管直肠癌。延误诊治时间３个月至１年。活检病理误诊率为５３．３％（８／１５），其中误诊为低分化腺癌６例，平滑肌     

直肠肛管恶性黑色素瘤的诊治

目的 探讨直肠肛管恶性黑色素瘤的临床表现、诊断、治疗及预后。方法 回顾性分析了1981－1996年我科诊治的直肠肛管恶性黑色素瘤6例，并进行随访。结果 6例患者肿块位于齿状线附近，肉眼观为紫黑色或褐色，均行手术治疗，其中2例行Mile′s术，2例行后盆腔清扫术，1例行经肛门肿块局部扩大切除术，1例行剖腹探查术，所有患者手术时均已有淋巴结或肝脏转移。6例患者于确诊后5－23个月死亡，平均存活14．7个月。结论 直肠肛管恶性黑色素瘤恶性度极高，死亡率高，较早发生淋巴和血行转移，宜采用根治手术辅以化疗及生物治疗的综合性治疗。     

The Role of Surgical Methods in the Treatment of Anorectal Malignant Melanoma (AMM)

Purpose : Anorectal malignant melanoma (AMM) is a rare tumor with a poor prognosis. The aim of this study was to investigate the clinicopathological characteristics and treatment outcomes in patients with AMM. Methods : The study included 21 patients diagnosed with AMM between 2000 and 2010 that were evaluated with regard to age, sex, disease stage, treatment modality, and survival. Stage I, II, and III were defined as localized primary malignant melanoma, regional lymph node metastasis, and distant metastasis, respectively.

Results : In all, 12 (57%) patients were female and 9 (43%) were male; median age was 61 years (range: 30–84 years). Among the 21 patients, 7 (47%) underwent abdominoperineal resection and 8 (53%) were treated using wide local excision. Four (19%) patients were classified as stage I, 10 (48%) as stage II, and 7 (33%) patients as stage III. In total, 10 patients received adjuvant therapy. Median overall and progression-free survival was 12 and 9 months, respectively. The 1-year and 5-year overall survival estimates were 59% and 42%, and progression free survival were 49% and 7%, respectively. Patients aged > 60 years (P = 0.145), female patients (P = 0.076), patients with localized disease (P = 0.045), patients that underwent wide local excision (P = 0.619), and patients that received adjuvant therapy (P = 0.962) had longer survival.

Conclusions : The prognosis of AMM remains very poor and disease stage is the only predictor of survival. Abdomino-perineal resection does not confer an advantage, in terms of survival, in patients with AMM.     

肛管直肠恶性黑色素瘤的外科治疗

目的 探讨肛管直肠恶性黑色素瘤的临床特征、诊断及治疗经验.方法 总结中国医科大学附属第四医院及附属第一医院33例肛管直肠恶性黑色素瘤患者的临床资料.依据手术方式分组,应用Fisher确切概率法,Kaplan-Meier方法和Log-rank检验进行统计学分析.结果 肛管直肠恶性黑色素瘤以女性多见,发病年龄22～77(54.5 ±7.6)岁.便血、肛门疼痛为最常见的临床表现.首次就诊误诊率为67%(22/33).肿瘤平均直径(3.5 ± 1.7) cm,31例(94%,31/33)的肿瘤距肛缘不足5 cm.术后平均生存期(14.0 ± 6.5)个月,1、3、5年总生存率分别为48%、22%、10%.腹会阴联合切除组与局部切除组术后局部复发率比较,差异有统计学意义(P=0.049),3年特异性生存率差异无统计学意义(x2=0.268,P=0.582).结论 肛管直肠恶性黑色素瘤极易误诊,扩大切除并不能有效延长患者的生存期.

Abstract：

Objective To evaluate clinical features, diagnosis and treatment of anorectal malignant melanoma (ARMM).Methods The clinical data of 15 patients of ARMM in our hospital and 18 patients in the First Affiliated Hospital of China Medical University from 1990 to 2010 were reviewed.Twenty-five patients underwent curative surgical resection, 14 patients underwent abdominoperineal excision of the rectum (APR), and 11 patients underwent local excision (LE).Survival analysis was carried out.Fisher's exact test and Log-rank test was used to compare the effects of these two different surgical procedures.Results ARMM had a female predominance, the mean age was 22 -77(54.5 ± 7.6) years.The major clinical signs included hematochezia, anus pain.The misdiagnosis rate was 67% (22/33).The average tumor size was (3.5 ±1.7) cm.Thirty-one petients(94% ,31/33) had ARMM within 5 cm from anus margin.Mean survival time was (14.0 ± 6.5)months.The overall 1-,3-,and 5-year survival rates were 48% ,22% , and 10% , respectively.Local recurrence after curative LE was higher than APR (LE,64% vs APR, 21% , P = 0.049) , The overall 3-year disease-specific survival rates after curative LE was not significantly different from that of APR (LE, 28% vs APR ,31%, x2 = 0.268, P = 0.582).Conclusions Anorectal malignant melanoma has a high rate of misdiagnosis.Radical resection could not prolong the survival time significantly in anorectal malignent melanoma patients.     

A first reported case of metastatic anorectal amelanotic melanoma with a marked response to anti-PD-1 antibody nivolumab: A case report

IntroductionAnorectal amelanotic melanoma (AAMM) is a rare disease with poor prognosis. A standard treatment strategy for AAMM has not been established.Presentation of caseWe report a case of successful treatment of AAMM with nivolumab. A 67-year-old man was referred for colonoscopy which revealed type I tumor in the rectum. AAMM was diagnosed with immunostaining histopathological biopsy findings. Enhanced computed tomography (ECT) revealed the rectal tumor without distant organ metastasis. We performed laparoscopy-assisted abdominoperineal resection. ECT at three months after surgery revealed liver metastases and right ischial bone metastasis. Although we had started dacarbazine monotherapy, black spots that were suspicious of skin metastases had appeared on systemic skin. Therefore, we started nivolumab therapy. ECT at 3 months after initiation of nivolumab showed shrinkage of liver metastasis. We have continued strict follow-up every 2 months and checked no oncologic progression at 17 months after initiation of nivolumab.DiscussionThe anti-PD-1 antibody have improved prognosis of malignant melanoma. However, there are no reports of nivolumab for treatment of AAMM.ConclusionsOur patient is the first reported case of AAMM treated with nivolumab. We consider that nivolumab will be effective for non-cutaneous malignant melanoma.     

Primary ano-rectal melanoma: considerations on a clinical case and review of the literature

Anorectal melanoma is a rare disease (1% of all anorectal malignancies). It is characterised by aspecific symptoms and the differential diagnosis versus other lesions of the rectum and anus is often difficult. The prognosis is very poor: mean survival is about 24 months, and at diagnosis most patients present distant metastases. Surgery is suggested as being the best treatment for this disease, since radio- and chemotherapy are generally only used for palliative purposes. Long-term survival depends on the stage of the melanoma at diagnosis. The possible surgical treatments available consist in local resection, which is considered the first therapeutic choice, and abdominoperineal amputation when local resection cannot be performed, or as a palliative operation. Inguinal lymphadenectomy is indicated when the inguinal lymph nodes are involved. In this report we describe a case of anorectal melanoma in a 73-year-old woman who underwent abdominoperineal amputation as surgical palliative treatment, because of infiltration of the puborectal muscle. The case report is followed by a review of the literature.     

双侧肾上腺继发性肿瘤的临床分析

目的 了解双侧肾上腺继发性肿瘤的临床特点和发生发展规律，提高对本病的诊治效果。方法 回顾性分析1990年1月至2001年12月收治50例双侧肾上腺继发性肿瘤患者资料。男45例，女5例。中位年龄56岁（23～75）。肾上腺肿瘤直径：左侧2．0～11．0cm，中位3．7cm；右侧1．5～14．0cm，中位4．0cm。结果 50例患者原发肿瘤主要为肺癌、淋巴瘤、黑色素瘤、肾癌等，分别占64．0％、12．0％、6．0％、6．0％。肺癌中以小细胞未分化癌常见（46．9％）。肿瘤发生时间：与原发瘤同时诊断26例（52．0％），原发肿瘤治疗后发现双侧肾上腺转移24例，其中1年内18例（75．0％）。44例在诊断时即有肾上腺外转移（88．0％），单纯性肾上腺转移6例（12．0％）。50例患者总生存时间1～70个月，中位生存期6个月。3例行同期或分期切除后联合放化疗，其中1例食管黑色素瘤患者生存19个月，1例肺腺癌患者15个月，1例肾癌患者生存26个月健在。结论 双侧肾上腺继发性肿瘤的原发肿瘤以肺癌和淋巴瘤常见，大多在原发瘤发现同时或术后1年内诊断，多采用姑息性治疗，预后较差，手术切除联合化疗或化放疗可能延长患者的生存期。     

Anorectal malignant melanoma: treatment with surgery or radiation therapy, or both.

INTRODUCTION: Anorectal malignant tumours are increasing in frequency for unknown reasons. Surgery is the principal treatment, and the role of adjuvant therapy has not been defined. We therefore decided to review the experience of the Princess Margaret Hospital in Toronto, a large tertiary care cancer hospital, with respect to the surgical management of anorectal melanoma. METHODS: We reviewed the charts of all registered patients with anorectal malignant melanoma (AMM) treated with surgery or radiotherapy, or both, at the hospital between 1980 and 1999, paying particular attention to survival, and local and distant recurrences. RESULTS: There were 14 patients, all of whom were followed up to the time of death or for a minimum of 28 months for surviving patients. The mean ages at diagnosis were 56 years for men and 68 years for women. Clinical staging was as follows: local, 10 patients; locoregional, 3 patients and metastatic disease, 1 patient. Local therapy included local resection alone in 7 cases and abdominoperineal resection in 7. Seven patients received pelvic irradiation at some time during their disease, using different doses and fractionation schemes. Three of them had concomitant chemotherapy and radiotherapy with no tumour regression. In all 3 patients the lesions was reclassified as AMM and the patient underwent surgery. The other 4 patients had a short course of radiotherapy for palliation after the original lesion recurred. The overall median survival was 12 (range from 3-51) months. Two patients remained alive at last follow-up. Patients managed by local excision had a median survival of 12 (range from 3-51) months, and those managed by abdominoperineal resection had a median survival of 7 (range 5-51) months. Of the 10 patients treated initially with local excision, 6 required reoperation. Three underwent salvage abdominoperineal resection. Six patients were alive 1 year after treatment (median survival 32.5 mo [range from 21-51 mo]). Eight patients had a rapid evolution of their disease with a median survival of 5.5 (range from 3-12) months. Eleven of the 12 patients who died had metastatic disease. CONCLUSIONS: Systemic dissemination is almost universal in patients with AMM. The overall survival was poor regardless of local treatment. There was a 60% failure rate of local excision, which necessitated further surgery. Improving local control is important since some patients will survive up to 3 years.     

影响肛管直肠恶性黑色素瘤预后的多因素分析

目的:探讨肛管直肠恶性黑色素瘤(anorectal malignant melanoma,AMM)的生存状况及其影响因素。方法:回顾性随访1994至2004年我院治疗的8例AMM;检索CNKI全文数据库,收集国内同期有随访资料的个案报道共48篇136例,随访时间1~144个月,分析18项与生存期可能相关的因素,单因素分析采用Kaplan-Meier模型的Log-rank检验,多因素分析采用COX比例风险模型(SPSS10.0for windows)。结果:AMM的1年、2年、3年、5年和10年生存率分别为47.25%、34.17%、27.28%、17.78%和8.89%,中位生存时间为16.78月。单因素分析显示肿瘤大小、肿瘤占据肠腔周径、误诊疾病类型、治疗方式、手术方式共5项因素影响AMM的预后(P<0.05),多因素分析显示肿瘤大小、治疗方式、手术方式影响AMM的预后(P<0.05)。结论:AMM预后极差,影响其预后的因素可分为两个方面:肿瘤大小和治疗方案,说明早期诊断和恰当治疗的重要性。     

Melanoma associated with blue nevus and melanoma mimicking cellular blue nevus: a clinicopathologic study of 10 cases on the spectrum of so-called 'malignant blue nevus'

The term "malignant blue nevus" refers to a rare and heterogeneous group of melanomas that arise in several clinical settings. This includes melanomas arising in association with a common or cellular blue nevus and those arising de novo and resembling cellular blue nevi. We reviewed the clinicopathologic features of 10 cases of malignant blue nevi. Six cases proved to be de novo melanoma mimicking cellular blue nevus, but lacking a clear-cut benign component. Two melanomas arose in association with a common blue nevus, and two with a cellular blue nevus. The patients' (5 males, 5 females) ages ranged from 11 to 77 years (average age, 48.1 years). The head and neck was the most common location (6 of 10 patients), with five scalp tumors. Four tumors were located on the trunk; none was located on the extremities. Tumor size ranged from 0.5 to 2.2 cm (average size, 1.1cm). Most lesions had been present for many years before surgical removal. Pigmented dendritic cells were observed in 9 of 10 cases. The malignant and benign components were easily distinguished in the four cases that arose in association with a common or cellular blue nevus. Abrupt transition between a benign blue nevus and melanoma was readily recognized at scanning magnification as distinctive nodules of epithelioid to spindled cells with a sheet-like growth pattern. In all cases, malignancy was evidenced by increased mitotic rate, necrosis, nuclear atypia, pleomorphism, hyperchromasia, and prominent nucleoli. All 7 patients with follow-up information experienced recurrence (3 patients) or metastasis (4 patients). Three patients died of disease. Malignant blue nevus is a heterogeneous group of melanomas that are highly aggressive and often lethal, with a propensity for metastasis to the lymph nodes and lungs.     

Prophylactic isolation-perfusion as the primary therapy for invasive malignant melanoma of the limbs.

The most common causes of treatment failure in patients with malignant melanoma treated by surgical therapy alone are local or regional recurrences. These are presumed to be due to occult metastasis present at the time of the initial treatment. In an effort to control this occult regional disease, 202 patients with Stage I malignant melanoma underwent isolation-perfusion with 1-phenylalanine mustard between the years 1960 and 1970. The 2-5 and 10-year determinate survival rates were 98%, 86% and 83%, respectively. In these patients, 2% developed local recurrences, 3% developed intransit metastasis, 18% developed positive regional lymph nodes and 6% developed disseminated disease, as their first evidence of recurrence. Over 40% of these patients were benefitted by further therapy. When regional perfusion is used, the question of prophylactic lymph node dissection need not arise. There was one surgical death in this series and only a few patients had symptomatology referable to their limbs beyond 3 months.