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.     

Surgical management of primary anorectal melanoma

BACKGROUND: This aim of this study was to analyse outcome after surgery for primary anorectal melanoma and to determine factors predictive of survival. METHODS: Records of 40 patients treated between 1977 and 2002 were reviewed. RESULTS: Twelve men and 28 women of mean age 58.1 (range 37-83) years were included in the analysis. Overall and disease-free survival rates were 17 and 14 per cent at 5 years. Median overall survival was 17 months and disease-free survival was 10 months. The 5-year survival rate was 24 per cent for patients with stage I tumours, and zero for those with stage II or stage III disease. There was no significant difference in overall survival after wide local excision (49 and 16 per cent at 2 and 5 years respectively) and abdominoperineal resection (33 per cent at both time points). In patients with stage I and stage II disease, there was a significant association between poor survival and duration of symptoms (more than 3 months), inguinal lymph node involvement, tumour stage and presence of amelanotic melanoma. CONCLUSION: Anorectal melanoma is a rare disease with a poor prognosis. Wide local excision is recommended as primary therapy if negative resection margins can be achieved.     

Surgical therapy of anorectal abscesses and fistulas

Retrospective analyses were made of 171 of 200 patients on whom operations had been performed for anorectal abscesses and fistulas, between 1981 and 1985. For abscess exposure and deroofing, we tried to perform simultaneous fistulotomy, with no recurrence being recorded from 37 patients treated that way. The major courses of anorectal fistulas proved to be predictible in most cases. Surgical approach and postoperative management are described in some detail. Clinical records were evaluated together with a questionnaire, with particular reference being made to the type of fistula, susceptibility to recurrence, faecal continence as well as length of hospitalisation and certified absence from work.     

The role of abdominoperineal resection as surgical therapy for anorectal melanoma

OBJECTIVES: 1) Characterize changes in the surgical treatment of anorectal melanoma over time. 2) Determine if the extent of surgical resection is associated with outcome. 3) Identify prognostic factors correlating with survival. SUMMARY BACKGROUND DATA: Although early data suggested improved survival in patients undergoing abdominoperineal resection (APR) for primary anorectal melanoma, such an aggressive approach may be unwarranted as distant relapse rates are high. We have seen a trend toward less aggressive surgical treatment of the local disease over the past 20 years. METHODS: A retrospective review was performed of all patients with anorectal melanoma treated at our institution between 1984 and 2003. Extent of primary resection and pathologic factors were studied. RESULTS: Forty-six patients underwent a curative resection with a median follow-up of 29 months, and 5-year disease-specific survival (DSS) rate of 35%. While patient and tumor characteristics remained similar, there was a dramatic shift in surgical treatment toward less radical procedures. Prior to 1997, the majority of patients (15 of 21, 71%) underwent APR. After 1997, the majority of patients (21 of 25, 84%) underwent local excision (LE) (P < 0.0001). Local recurrence was noted in 11 of 46 (24%) patients: 4 of 19 (21%) who underwent APR and 7 of 27 (26%) who underwent LE (P = not significant). Five-year DSS was similar: 34% following APR and 35% following LE. Tumor perineural invasion (PNI) was the only factor identified as an independent predictor of worse outcome (P = 0.01). CONCLUSION: The extent of surgical treatment is not associated with outcome in primary anorectal melanoma. Therefore, LE of the primary tumor is recommended when technically feasible. The presence of PNI is an important prognostic factor and should be considered in future clinical trials.     

Surgical therapy of malignant melanoma

148 patients with malignant melanoma are presented. Localization, prognosis and clinical stadium are summarized. The surgical procedure is still three-dimensional excision. The latitude of defects asks for a specialists surgeon.     

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.     

Patterns of failure in anorectal melanoma. A guide to surgical therapy

Anorectal melanoma is an aggressive tumor with a reported 5-year survival rate of 6%. Recommendations for local surgical therapy vary from local excision to abdominoperineal resection. Therapy, patterns of failure, and survival were retrospectively examined in 32 patients with anorectal melanoma. Twenty-six patients were treated surgically, 14 with abdominoperineal resection and 12 with local excision. Local recurrence occurred less frequently in patients undergoing abdominoperineal resection (4 [29%] of 14) compared with patients undergoing local excision (7 [58%] of 12) but developed concomitantly with distant or regional metastasis in all but 2 of the 11 patients whose operations failed locally. Inguinal nodal disease developed in 15 patients (47%). Pelvic nodal disease became apparent in only 2 patients (7%). There was no difference in overall survival between the two surgically treated groups (median survival, 19.5 months for patients treated with abdominoperineal resection vs 18.9 months for patients treated with local excision). Therefore, local excision is recommended when technically feasible since these patients eventually succumb to metastasis regardless of surgical therapy.     

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 .     

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。     

Surgical treatment of anorectal malformations

The goal of surgery for anorectal malformations (ARM) is to achieve good bowel, urinary, and sexual functions, as well as the ability for children to become healthy adults. Various surgical procedures and surgical management protocols have been explored or devised by pediatric surgeons. These are described in this review. Making a correct type classification by invertography, fistelography and urethrography in the neonatal period allows pediatric surgeons to select an appropriate surgical strategy. Surgery for low-type malformations is principally neonatal perineoplasty, while that for intermediate- or high-type malformations is colostomy, followed by a pull-through operation during infancy. Posterior sagittal anorectoplasty or laparoscopy-assisted surgery has recently been accepted as alternative procedures. Fecal incontinence represents a devastating problem that often prevents a patient from becoming socially accepted and may cause serious psychological sequelae. One-third of adult patients with high- or intermediate-type malformations occasionally complain of fecal incontinence after surgery. Most patients with ARM have normal urinary function if they do not have urinary tract or sacral anomalies. These associated anomalies also influence the prognosis for sexual function, especially in males. Some female patients have experienced normal vaginal delivery and had children. In patients with cloacal malformation, however, fertility or sexual problems are also often present. Based on this information, it is clear that only well-planned and systemic treatments can provide a good functional prognosis after making a correct classification in the neonatal period.     

Surgical treatment of anorectal melanomas

Thirty cases of anorectal melanoma have been recorded in Israel from 1960 to 1981. The frequency of the disease doubled in the last decade (from 10 to 20 cases). A clear predominance has been noted among Jews of European descent (18 patients) as opposed to Sephardic Jews (9 patients) or Arabs (3 patients). From this series, the emerging conclusion is that in most cases radical surgery (abdominoperineal resection) is seldom indicated. It is doubtful whether this form of treatment increases the survival while affecting considerably the quality of life. Survival correlated well with the stage of disease at the time of diagnosis. No such correlation could be established with the type of treatment employed.     

Sentinel node mapping in anorectal melanoma

Anorectal melanoma is a rare condition and its surgical management is controversial. This article reports the case of a patient with anorectal melanoma who underwent abdominoperineal resection and Sentinel Lymph Node biopsy. Melanoma was classified pT4aN0. Fifty months after initial treatment, the patient is still alive disease free. SLN mapping allows better surgical excision of the presumed sites of the lymphatic dissemination in melanoma. SLN biopsy improve the accuracy of nodal staging. In case of sentinel node metastasis, it allows early therapeutic lymphadenectomy of the sentinel nodes's basin and could therefore reduce the high rate of regional recurrence in anorectal melanoma. Moreover, knowing the exact histological status of the regional nodes means that the relative merits of abdominoperineal resection and wild local excision could be compared in relation to tumor thickness.     

The surgical treatment of anorectal malignant melanoma

The clinical presentation and surgical treatment of 21 patients with anorectal melanoma over a 44 year period have been analysed. During the first 24 years of this period total rectal excision was regarded as the treatment of choice and since then wide local excision. Two groups of patients emerge for comparison of the results of surgical treatment. There were no long-term survivors in either group and little difference between survival rates after either procedure. It was concluded that the change from radical to conservative surgery for anorectal melanoma has made no difference in terms of survival and the possible advantages of this change have been discussed.     

The surgical management of anorectal malignant melanoma

The biological vagaries of anal malignant melanoma are illustrated by four cases in Chinese patients. All four died within five years. Their poor prognosis emphasizes the value of preoperative studies to detect clinically occult metastases and obviate futile radical surgery. Many patients already have disseminated disease at the time of diagnosis, and local excision of the tumour provides acceptable palliation. For localized disease, abdominoperineal resection prevents local recurrence and removes the mesenteric nodes which are frequently involved. Palpable inguinal nodes necessitate therapeutic groin dissection, but we perform elective resection only when affected nodes are found at laparotomy. Pelvic lymphadenectomy should be performed in conjunction with abdominoperineal resection. The efficacy of chemotherapy for anorectal melanoma remains uncertain.     

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。     

Surgical resection for gastrointestinal metastatic melanoma

AIM: Metastases of melanoma are frequent. On the gastro-intestinal tract, commonest localizations are small bowel, stomach and colon. Surgical treatment of digestive metastases from melanoma is not well known and its value is still debated. PATIENTS AND METHODS: Medical records of 10 patients (six female and four male) operated for metastatic melanoma to gastro-intestinal tract were reviewed to determine results of surgery. RESULTS: Gastro-intestinal metastases were symptomatic in eight patients (abdominal pain in three, bowel obstruction in three, abdominal mass and obstructive jaundice in one each). Two patients had anemia. Diagnosis has been suggested by imaging in seven patients and endoscopy in three. All patients were operated on by laparotomy for resection of metastases located on small bowel in four patients, gallbladder in two, stomach in two and colon in two. Complete resection suppressed symptoms in nine cases. In one patient, resection was incomplete but provided satisfying symptomatic relief. One patient died at day 3; in other patients, median survival was 18 months (range: 3-120). CONCLUSION: In a patient with previous history of melanoma, digestive symptoms indicate morphological explorations due to suspicion of metastases to gastro-intestinal tract. Surgical treatment of these metastases is usually palliative but, in some cases, allows long-term survival.     

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。