Primary anorectal malignant melanoma

A case of primary anorectal malignant melanoma seen in a 46 year old woman is presented herein. Her most marked symptoms were bloody stools and anal pain. Endoscopic examination indicated a tumor with ulceration but without pigmentation in the anorectal region. Histologic examination of the biopsied specimens showed spindle-shaped cells with atypia proliferating in a bandlike arrangement, as in leiomyosarcoma. An abdominoperineal resection was done and detailed histological examination of the tumor comfirmed the nature of the tumor to be malignant melanoma. The postoperative immunochemotherapy consisted of Dimethyl-Triazeno-Imidasole-Carboxamide (DTIC), Amino-Methyl-Pyrimidinyl-Methyl-Chlorethyl-Nitrosourea-Hydrochloride (ACNU), Vincristine (VCR) and OK-432. The patient has been well without recurrence for fifteen months following her operation through the continuous administration of these agents.     

肛管直肠恶性黑色素瘤的诊治进展

目的：探讨肛管直肠恶性黑色素瘤的诊治进展，方法：复习相关文献并对本病病因，临床特点，早期诊断，治疗及预后作一综述报告。结果：肛管直肠恶性黑色素瘤是一种发病率很低的恶性肿瘤，发病原因可能与良性黑痔史，HIV感染，太阳光照射有关，主要症有便血，局部肿块，大便习惯改变等，早期诊断主要依赖对45－80岁的高危发病人群进行常规直肠检查，CEA多克隆抗体标记阳性在病理诊断中有一定帮助，治疗方法现存在争议，多主张在手术治疗（腹会阴联合切除或局部广泛切除）的基础上辅以化疗，放疗或免疫治疗。结论：肛管直肠恶性黑色素瘤早期诊断困难，预后差，应提高警惕，最佳治疗方法还有待进一步探讨。     

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.     

The Diagnostic Value of Endoscopic Ultrasonography and Magnetic Resonance Imaging for Anorectal Malignant Melanoma: Report of a Case

A 74-year-old Japanese woman presented with a 3-month history of anal bleeding. Proctoscopy revealed an unusual polypoid lesion with focal pigmentation at the dentate line, which was histologically diagnosed as a malignant melanoma. Whole-body clinical and radiographic evaluations revealed no alternative primary source. Endoscopic ultrasonography (EUS) showed well-delineated hypoechoic tumors invading the muscularis propria, and magnetic resonance imaging (MRI) revealed regional lymphadenopathy. Following this evaluation, an abdominoperineal resection with regional lymphadenectomy was performed. The excised tumor was histologically confirmed to be malignant melanoma, and its depth and metastatic lymph nodes proved to have been accurately and precisely evaluated by the preoperative examinations. Thus, EUS and MRI are useful preoperative diagnostic tools for the tumor staging of primary anorectal malignant melanomas, as for other rectal tumors. Received: February 1, 2002 / Accepted: September 3, 2002 Reprint requests to: H. Sashiyama     

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。     

足底内侧皮瓣在足跟部恶性黑色素瘤的应用

目的探讨足跟部恶性黑色素瘤的手术切除和修复重建的方法。方法5例老年足跟部恶性黑色素瘤患者，手术行局部扩大切除，切缘距病损2cm，深度达跟骨骨膜，足跟部软组织缺损Ф5～9cm，根据缺损大小，用带足底内侧血管神经束的皮瓣移位修复，皮瓣面积8cm×6cm-10cm×8cm。结果5例皮瓣全部成活。随访2年6个月～5年，患者均健在，肿瘤未见局部复发，皮瓣感觉功能良好，患足功能与对侧相比无明显差异。结论足跟部恶性黑色素瘤应行局部扩大切除术，切除范围应距肿瘤边缘不小于2cm为宜，在切缘阴性的基础上进行修复重建，应用足底内侧皮瓣移位修复，临床疗效满意。     

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.     

Long-term survivor of primary anorectal malignant melanoma by a multi-disciplinary approach

Anorectal malignant melanoma tends to show an aggressive biological behaviour. Therefore, the 5-year survival rate is limited. We herein present a successful case of a super-long-term survivor (20 years) who underwent multi-disciplinary treatment. The present case suggests that a multi-disciplinary approach may be beneficial for patients with thick and extensively sized lesions after radical resection for primary anorectal malignant melanoma.     

Primary Malignant Melanoma of the Small Intestine: Report of a Case

The small intestine is the most common site of gastrointestinal (GI) metastases from cutaneous malignant melanoma; however, primary malignant melanoma originating in the small intestine is extremely rare. We report the case of a 72-year-old man found to have a primary malignant melanoma in the ileum. The patient presented with anorexia, weight loss, diffuse colicky abdominal pain, and episodic rectal bleeding. A preoperative diagnosis of a small intestinal tumor was based on the findings of enteroclysis and computed tomography scanning. This diagnosis was confirmed at laparotomy and an enterectomy was performed. Histopathological examination of the resected specimen clarified the exact nature of the lesion, confirming the diagnosis of melanoma. A thorough postoperative investigation did not reveal a primary lesion in the skin, anus, oculus, or any other location. Thus, we diagnosed this tumor as a primary lesion. One year after his operation, the patient remains well without any evidence of recurrence. Primary malignant melanoma of the small intestine is an extremely rare lesion, which must be differentiated from other intestinal tumors. Received: August 6, 2001 / Accepted: March 5, 2002     

胃肠道恶性黑色素瘤70例临床分析

目的 探讨胃肠道恶性黑色素瘤的诊治方法,提高诊治水平.方法 总结1965年7月至2007年6月收治的70例胃肠道恶性黑色素瘤患者的临床资料.结果 70例患者中男性27例,女性43例,年龄25～75岁,中位年龄53岁,其中50例发生于直肠,10例发生于肛管,10例发生于食管.1、3、5年生存率分别为48.3%、14.6%、6.5%,中位生存时间为379 d.63例接受手术治疗,单纯手术的25例患者和术后辅助化疗、放疗、生物治疗等综合治疗的38例患者的总生存率无统计学差异,Ⅲ期患者综合治疗组较单纯手术组生存率显著升高.风险因素分析提示病变深度为胃肠道恶性黑色素瘤的危险因素.结论 手术加综合治疗能提高Ⅲ期胃肠道恶性黑色素瘤的生存率;病变深度是影响生存率的风险因素.     

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.     

A case report of anorectal malignant melanoma with mucosal skipped lesion

IntroductionWe report our experience involving a case of relatively rare anorectal malignant melanoma with skipped lesion.Presentation of caseThe patient was a 72-year-old man who had visited a local clinic complaining of a mass in the anal region, whereupon he was referred to our hospital on suspicion of a malignant melanoma. Close examination revealed a 25-mm black type 1 tumor one-third the size of the circumference of the anal canal and located externally to it. We performed transanal resection of the tumor and confirmed a diagnosis of malignant melanoma. Notably, multiple macular black lesions spaced away from the main lesion were observed during surgery in half of the circumference of the anal canal, from the tumor to the pectinate line. A biopsy of the area also revealed malignant melanoma; therefore, we performed abdominoperineal resection. Pathological diagnosis indicated a submucosal depth; the patient was thus diagnosed with T4 N2c M0 stage IIIb malignant melanoma and was followed on an outpatient basis.DiscussionPatients with anorectal malignant melanoma have very poor prognoses owing to early lymph node metastasis and hematogenous metastasis. Our case illustrates that small anorectal malignant melanoma lesions can spread from the main lesion and invade the mucosa; examinations may sometimes miss such skipped lesions.ConclusionSkipped lesions can occur in anorectal melanomas; thus, careful scrutiny of such lesions is required. Moreover, lesion resection is critical for anorectal malignant melanomas.     

直肠肛管恶性黑色素瘤的临床特征分析

目的了解原发性直肠肛管恶性黑色素瘤的临床特点。方法回顾性分析9例原发性直肠肛管恶性黑色素瘤的临床资料,并复习文献。结果直肠肛管恶性黑色素瘤以女性多见,平均发病年龄56岁,病程5.8个月;首发症状以血便为最常见,其次为肛门肿物突出。94.7%的直肠肛管恶性黑色素瘤在距离肛缘5.0cm范围内;肿瘤最大径(3.3±2.1)cm;其中54.5%可活动;有19.1%的肿瘤表面光滑;6.6%的肿瘤质地软;14.0%同期发现转移,肝转移最常见,腹股沟淋巴结转移其次;的病例出现误诊,超过者被误诊为良性疾病;手术治疗中以Miles术为主,经肛门局部切除术其次。结论直肠肛管恶性黑色素瘤极易误诊。手术治疗为主。     

皮肤恶性黑色素瘤手术结合大剂量罗扰素治疗的疗效分析

目的探讨皮肤恶性黑色素瘤手术结合大剂量罗扰素治疗的疗效。方法1998年1月～2005年12月，收治皮肤恶性黑色素瘤患者33例，男20例，女13例；年龄17～79岁。病程2个月～7年，中位病程3．5年。9例Ⅰ期患者行单纯病灶扩大切除结合中厚植皮（或邻位皮瓣）修复创面，23例Ⅱ期患者行病灶扩大切除及区域淋巴结清扫术（或截肢），1例Ⅲ期患者姑息性病灶切除。所有患者术后均辅以大剂量罗扰素生物治疗。结果9例Ⅰ期患者术后切口愈合好，获随访7个月～8年，无复发。Ⅱ期患者中，2例切口愈合延迟，经换药愈合；1例失访，余22例获随访5个月～7年，其中1例复发，部位于大腿原发灶旁，再次予病灶广泛切除后，大剂量罗扰素治疗。1例Ⅲ期患者治疗1年6个月后因肺部转移并发呼吸衰竭死亡。结论手术结合术后大剂量罗扰素综合治疗恶性黑色素瘤疗效满意。     

Small Bowell Malignant Melanoma-Report of Three Cases and Review of Literature

Malignant melanomas have a predilection to metastasize to the small bowel. Three patients with malignant melanoma involving the small bowel are reported. Two patients were operated on for small bowel obstruction and the third for gastrointestinal bleeding with anemia. Two patients remained well 6 month and 2 years, respectively, after surgery. One patient died of metastatic cerebral melanoma 6 months postoperatively.

One should suspect small bowel metastasis in every patient with malignant melanoma in his past medical history, who presents with recent changes in bowel habits, intestinal obstruction or gastrointestinal bleeding. Preoperative assessment can only raise the suspicion, even with advanced imaging methods: capsule endoscopy, enteroscopy, CT or PET-CT. The only therapeutic procedure is surgical resection, offering both short term survival as well as an improvement in the quality of life. Although prognosis is dismal there are factors associated with prolonged survival: complete surgical resection with no residual primary or metastatic tumor, so-called primary small bowel tumors in patients aged more then 60 years, LDH < 200 U/L, lack of tumor spread in mesenteric lymph nodes.     

Malignant melanoma of the anorectal area. Report of two cases

BACKGROUND/AIMS: Primary anorectal melanoma is a very rare malignant tumor with no more than 300 cases reported in the literature. METHODS: Two cases of anorectal melanoma are reported herein. RESULTS: Both patients, aged 44 and 74 years, presented at the outpatient department with anal bleeding, one after being treated for 3 months with antihemorrhoidal drugs. The diagnosis was established with proctoscopy and biopsy, and a palliative abdominoperineal resection in the presence of lymph node metastases was performed followed by chemotherapy with vindesine. Although the procedures were not curative, both patients had an uneventful postoperative recovery, and lived 4 years and 21 months, respectively, without bleeding problems albeit with the inconvenience of a colostomy. CONCLUSIONS: For the time being there is no convincing proof of the value of either types of proposed surgical management. We agree with those who believe that abdominal perineal resection has an advantage regarding the prognosis and quality of life.     

手指恶性黑色素瘤的临床特征与疗效分析

目的 探讨手指恶性黑色素瘤的临床特征及治疗效果.方法 回顾性分析1995年2月-2007年10月收治并经病理检查证实的22例手指恶性黑色素瘤的临床资料,其中拇指12例,示、中指各3例,环、小指各2例.手指黑斑及疼痛为共同的首发症状,15例有甲下病变,12例有外伤史,2例X线片显示指骨有溶骨性改变.主要采用手术、全身化疗及免疫治疗.所有患者均采用截指术,其中13例行同侧腋窝淋巴结清扫术.结果 22例患者获得随访,其中3例2年后失访.随访时间为1～10年,平均4.5年.1年生存率为86.4%(19/22),3年生存率为63.2%(12/19),5年生存率为31.6%(6/19).结论 手指恶性黑色素瘤临床少见,治疗应以手术、化疗、免疫治疗等综合方法为主.其预后与肿瘤大小、浸润深度及临床分期有关.     

肛管直肠恶性黑色素瘤91例外科治疗及预后分析

目的 探索肛管直肠恶性黑色素瘤（ARMM）的外科治疗方式及影响病人总生存的预后因素。方法 回顾分析2003年1月至2018年1月北京大学肿瘤医院胃肠肿瘤中心诊断为ARMM并接受手术治疗的91例病人的临床病理资料。比较不同临床病理因素、手术方式［扩大的局部切除（WLE）与腹会阴联合切除（APR）］对病人总生存的影响。结果 在91例ARMM病人中,男性35例（38.5%）, 女性56例（61.5%）,男女比例为1.0∶1.6。接受WLE 12例（13.2%）,APR 79例（86.8%）。;术前存在远处转移19例（20.9%）,无远处转移72例（79.1%）。病人1、3和5年存活率分别为74%、23%和9%,中位生存期为18.7个月。单因素分析示术前远处转移、肿瘤直径＞2 cm、肿瘤侵犯深度＞2 mm和淋巴结转移为总生存的影响因素;多因素分析显示术前存在同时性远处转移和淋巴结转移为总生存的独立预后因素。12例接受WLE病人中位生存期为21个月,79例接受APR病人中位生存期为21个月,接受WLE和接受APR病人两组生存期差异无统计学意义（P=0.94）。结论 术前存在远处转移、淋巴结转移是ARMM病人总生存的独立预后因素。APR与WLE相比,并不能带来生存的获益。     

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。     

Discriminant analysis of prognostic factors for malignant fibrous histiocytoma in soft tissue

We prospectively followed 32 patients with soft-tissue malignant fibrous histiocytoma (MFH). Parameters were age; sex; tumor size, location, and depth; operative method; chemotherapy; radiotherapy; and histology. Patients with recurrence or metastases due to MFH within 6 months after the initial operation were separated from those without these characteristics by discriminant analysis with statistically significant difference. The order of influential functions was histology, depth of tumor, operative method, and sex. Male patients with deep-seated storiform-pleomorphic type MFH, receiving less comprehensive surgery, had the greatest risk of local recurrence or early metastases. We have to pay particular attention to patients with these factors and perform adequate surgery, because local recurrence and metastases were found to be closely related, and to have a great influence on the prognosis of this disease. Discriminant analysis to separate patients with MFH recurrence or metastases within 6 months after the initial operation from those without these characteristics is worthwhile for prognostic assessment. Received: October 16, 2000 / Accepted: February 4, 2001