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。     

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。     

直肠肛管恶性黑色素瘤的外科治疗及预后

目的探讨直肠肛管恶性黑色素瘤的外科治疗及局部复发、预后的影响因素。方法回顾性分析50例直肠肛管恶性黑色素瘤患者的临床病理资料,并对预后进行单因素及多因素分析。结果本组47例患者行肿瘤切除术,其中31例行腹会阴联合根治术,16例行肿瘤局部切除术;术后局部复发率分别为16.1%（5/31）和68.8%（11/16）。χ^2检验显示,手术方式与局部复发相关（P=0.001）。47例患者5年生存率18.2%,单因素分析显示,病灶单发（P=0.0458）和肿瘤侵犯深度（P=0.0053）与预后相关。多因素分析显示,肿瘤侵犯深度（P=0.010）是影响预后最主要因素。结论直肠肛管恶性黑色素瘤预后差,影响预后最主要的因素是肿瘤侵犯深度,腹会阴联合根治术后复发率低。     

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

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诊治体会(附11例报告)

目的　探讨肛管直肠恶性黑色素瘤有效的治疗方法 ,以提高其生存率。方法　回顾性分析本院收治的 1 1例经病理证实的肛管直肠恶性黑色素瘤病人临床资料 ,结合有关文献 ,加以总结。结果　 1 1例病人中 ,7例行腹会阴联合切除术 (Mile’s)。其中 ,2例加行两侧腹股沟淋巴结清扫 ;3例仅行局部扩大切除 ;1例保留肛门经腹直肠切除吻合器吻合术 ,术后辅以化疗和生物治疗。 9例获得随访。根治性切除组的中位生存期为 2 7个月 ,而局部扩大切除组的中位生存期为 2 1个月。其中 1例因早期获得诊断 ,早期行根治性切除而存活近 4年。结论　早期诊断、早期发现是治疗的关键。早期行根治性切除 (Mile’s) ,术后辅以化疗及生物免疫治疗是处理肛管直肠恶性黑色素瘤的有效措施     

治疗方式对肛管直肠恶性黑色素瘤预后的影响

目的 探讨不同治疗方式对肛管直肠恶性黑色素瘤的预后影响.方法 回顾性分析1965-2007年收治的60例肛管直肠恶性黑色素瘤患者的临床资料,并对预后进行生存分析和COX风险因素分析.结果 60例患者中,肿瘤发生于直肠者50例,发生于肛管者10例.53例行手术切除治疗.对行单纯手术的23例患者和术后辅助化疗、放疗、生物治疗等综合治疗的30例患者的资料进行生存分析,总生存率差异无统计学意义(X2=0.078,P>0.05).53例手术病例中,37例行Miles术,16例行局部扩大切除术,两种术式生存分析差异无统计学意义(X2=1.464,P>0.05).风险因素分析结果提示,肿瘤浸润深度为危险因素,治疗方式为保护因素.结论 手术切除是肛管直肠恶性黑色素瘤的主要治疗手段,对肛管直肠恶性黑色素瘤病变局限者,应首选局部扩大切除术;病变深度和治疗方式是影响预后的风险因素.     

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

为了探讨肛管直肠恶性黑色素瘤的疗效，对１９６０～１９９６年收治的２７例肛管直肠恶性黑色素瘤（ＡＲＭＭ）进行了临床分析，其中１６例行经腹会阴联合根治术（Ｍｉｌｅ＇ｓ术），６例行局部切除术，５例行剖腹探查术。２２例获随访，随访时间为１～１２年。结果表明：中位生存时间Ｍｉｌｅ＇ｓ术组２１．５月、局部切除术组１３．５月、剖腹探查术组７．０月。Ｍｉｌｅ＇ｓ术效果优于局部切除术。提示：对ＡＲＭＭ应行Ｍｉｌｅ＇ｓ术，并对伴有腹股沟淋巴结转移或可疑者行腹股沟淋巴结清扫     

Primary amelanotic anorectal melanoma--a case report

BACKGROUND: The amelanotic melanoma of the rectal mucosa is very rare with an unfavourable prognosis. The surgical approach is still discussed controversially. Therefore transrectal ultrasound is of major importance in the preoperative staging and postoperative follow-up especially in diagnosis of local recurrence by using the ultrasound-guided, transrectal aspiration. METHODS: In literature 5 cases of amelanotic malignant melanoma were reported. The overall survival time is 10 months after diagnosis. All patients were female. RESULTS: We report about a 55-year-old female patient with an amelanotic melanoma of rectal mucosa. 7 months after a wide local excision of the tumour and interferon therapy in case of the absence of pararectal, inguinal metastases and other metastases the patient developed pararectal metastasis. An abdominoperineal resection and resection of inguinal lymph nodes was performed. Two months later paraaortal lymph nodes were detected. We started chemotherapy with Dacarbazin and with regard of the tumour progress the chemotherapy was changed to Vindesin 25 months after first operation supported by a radiotherapy with 40 Gray. The patient died 36 months after diagnosis. CONCLUSION: The prognosis of primary malignant anorectal melanoma is poor, irrespective of surgical treatment. Wide local resection is the first choice for primary anorectal melanoma. Abdominoperineal resection should be reserved for cases were complete tumour resection is impossible. Chemotherapy, radiotherapy and immunotherapy should be considered in the treatment of anorectal melanoma to influence the overall survival.     

Anorectal amelanotic melanoma

Objective  Anorectal melanoma is a rare, highly malignant tumour with a poor 5 year survival of 10%. Most anorectal melanomas have gross and/or histologic pigmentation, however about 30% of anorectal melanomas are amelanotic.
Method  We report three cases of amelanotic anorectal melanomas and integrate our data with six case reports of amelanotic malignant melanoma from the literature. Further we compare clinicopathological data and clinical outcome with large series of anorectal melanomas (both, amelanotic and pigmentated).
Results  There were seven females and two males, of median age 62 years (range: 45–75 years). Rectal bleeding was the leading symptom in all cases with a mean duration of 4 months before diagnosis. Eight of nine patients developed distant metastases. Median survival was 14 months (range: 3–60 months). A tumour thickness of < 4 mm was correlated with long-term disease-free survival, whereas tumour thickness of 4 mm or more was correlated with systemic recurrence.
Conclusion  Early diagnosis is key for efficient treatment and improved survival rate for patients with this unusual variant of melanoma. There is no difference in terms of age, time of diagnosis, stage and survival between pigmented and amelanotic anorectal melanoma.     

Malignant melanoma of the anorectum: report of four cases

Four cases of anorectal malignant melanoma are reported in this paper. All patients underwent an abdominoperineal resection with lymph node dissection for a curative operation and received postoperative chemotherapy with dacarbazine, ranimustine, and vincristine, either with or without interferon-β. One of these patients has been observed for more than 6 years postoperatively without any evidence of recurrence. The other three patients had advanced diseases at the time of diagnosis, and died within 3 years after operation. The prognosis of anorectal malignant melanoma is considered to be directly related to tumor size and depth. Therefore, a staging system and treatments based on the tumor size and depth (or thickness) are needed. Received: March 28, 2001 / Accepted: November 20, 2001     

Primary ano-rectal melanoma

Primary malignant anorectal melanoma is an uncommon disease that accounts for 1% of anorectal malignancies. Its virulent malignancy is associated with a poor prognosis and with difficult diagnostic and therapeutic problems. The operative management of these patients is controversial. Clinicopathologic features and surgical treatment of 6 patients with primary anorectal melanoma were studied retrospectively. There was a male preponderance (2:1) with a mean age of 62 years (range: 34-74). The site of origin of the melanoma was rectal in one patient and in the anorectal junction in five patients. Atypical intramucosal melanocyte proliferation was associated with rectal melanoma. The maximum tumor size from 2 to 5.5 cm. Common initial symptoms were rectal bleeding and/or tenesmus. CT was useful for tumor staging. Two patients had distant metastases at initial presentation. Four patients underwent "curative" treatments by abdominoperineal resection and 2 by local excision. The survival for the group as a whole was poor (mean: 12.6 months; range: 7-30 months). Surgery is the primary option. The prognosis, however, is poor, since metastatic disease is commonly established at presentation. Atypical intramucosal melanocyte proliferation may be a marker in association with tumor sited in the rectum.     

原发性消化道黑色素瘤的临床分析

目的 总结原发性消化道黑色素瘤的临床特点。方法 回顾性分析我院近10年住院治疗的8例原发性消化道黑色素瘤临床资料并复习献。结果 8例原发性消化道黑色素瘤中，原发灶位于直肠6例，小肠1例，食管1例，除1例直肠黑色素瘤术前得到确诊外，其余7例均被提诊，直肠黑色素瘤以便血，肛门疼痛和排便困难为主要症状；多表现为息肉样，均位于距齿状线以上5cm以内；少有色素沉着，小肠黑色素瘤以梗阻，腹痛，贫血为主要症状；造影和CT有助于发现病变，但确诊仍需病理学，食管黟 以素瘤多为黏膜下；胃镜可发现色素沉着；病理活检可协肋诊断。结论 原发性消化道黑色素瘤的临床表现与其他肿瘤类似，极易误诊，病理活检是确诊的主要手段。     

原发性肛管直肠恶性黑色素瘤29例诊治体会

目的了解肛管直肠恶性黑色素瘤的生物学特性和根治性外科手术在治疗该病中的临床意义。方法回顾性总结自１９６５年至１９９５年在我院接受治疗的肛管直肠恶性黑色素瘤患者２９例的临床和病理资料。结果本组女１９例,男１０例,平均年龄５０岁。２９例患者中,接受了根治性手术（Ｍｉｌｅｓ手术）２３例。术后复发１７例,复发率为７４％（１７／２３）,主要复发部位为远处转移。以性别,癌灶体积,色素产生,浸润深度和淋巴结转移为变量分析与预后的关系,仅淋巴结转移与术后复发的关系非常密切（Ｐ＜００５）。其余６例接受姑息性手术治疗。全组病例术后总５年生存率为２９％。根治性手术病例术后５年生存和无病生存率分别为３７％和２８％。未切除的６例全部死亡。中位生存期为１３５个月。结论肛管恶性直肠黑色素瘤是一种恶性程度极高的肿瘤。即使施行根治性外科手术治疗预后亦不佳。术中见到有淋巴结转移者预后更差。     

Anorectal Melanoma Misdiagnosed as Hemorrhoids: A Case Report and Review of the Literature

Anorectal melanoma is a rare and aggressive malignant neoplasm with an indolent course, manifesting with nonspecific symptoms and a poor prognosis. We present a case of anorectal melanoma that was initially treated as hemorrhoids and correctly diagnosed after lower gastrointestinal endoscopy. We also present the latest findings in the literature about anorectal melanomas and discuss updates about treatment options and management.     

Rectal malignant melanoma diagnosed by N-isopropyl- p-123I-iodoamphetamine single photon emission computed tomography and 5-S-cysteinyl dopa: report of a case

Malignant melanoma in the anorectal region is a rare disease associated with a very poor prognosis. Taking a biopsy of malignant melanoma is generally contraindicated because of the high risk of inducing metastasis. Although clinical examination and imaging findings are important for the preoperative diagnosis, conventional imaging techniques sometimes fail to provide information from which an accurate diagnosis can be made. We recently treated an 84-year-old woman with rectal malignant melanoma, in whom magnetic resonance imaging showed atypical findings. On the other hand, N-isopropyl-p-¹²³I-iodoamphetamine single photon emission computed tomography and 5-S-cysteinyl dopa in blood serum, as a tumor marker of malignant melanoma, proved very effective for establishing the preoperative diagnosis. Despite radical abdominoperineal resection, the patient died of multiple liver and lung metastases about 5 months after surgery. Received: April 8, 2002 / Accepted: November 19, 2002 RID="*" ID="*" Reprint requests to: S. Sato