A comparison of wide local excision with abdominoperineal resection in anorectal melanoma

Anorectal melanoma is a rare condition and its surgical management is controversial. Seventeen large case series from over the past 10 years were reviewed. The survival of patients treated by either abdominoperineal resection (APR) or wide local excision (WLE) was analysed according to the stage of the disease. Comparison of the survival of patients who underwent APR with those who underwent WLE showed no statistically significant advantage for either procedure in patients at all disease stages. APR should therefore only be performed when local excision is not possible or for palliative purposes.     

Primary anorectal malignant melanoma

Eleven patients with primary anorectal melanoma were analyzed retrospectively. Clinical symptoms and signs were rather unspecific; bleeding in nine, a palpable mass in five, and local pain in three patients. Two patients had the tumor diagnosed accidentally. In all patients the primary tumor was in an advanced stage. A summary of the histopathological characteristics is given in all patients. Six patients were subjected to abdominal perineal resection, four to local excision, and one to a colostomy followed by radiotherapy. All patients developed metastatic disease after a median time of 10 months. The median survival time was 14 months after both abdominal perineal resection and local excision. One patient is still alive after 11 years. Pertinent data form the literature are given.     

Malignant melanoma of the anorectal region

The charts of 15 patients with malignant melanoma of the anorectal region treated at Roswell Park Cancer Institute in the period 1975–1991 were reviewed. All the lesions except one developed at the pectinate line, in the area of transitional mucosa. Two of the patients at the time of initial presentation had distant metastases. Of the remaining 13, 8 were treated with abdominoperineal resection (APR) and 5 with local excision (LE). The incidence of local recurrence was 50% in the LE group and 22% in the APR group. Patients treated with APR had a 25%, 5-year survival rate compared with 0% for those treated with LE, although one of the latter group died 55 months following LE due to unrelated causes without recurrence. The median survival of those with LE was 15.7 months and of those with APR 13.7 months. © 1995 Wiley-Liss, Inc.     

Sphincter-sparing local excision and hypofractionated radiation therapy for anorectal melanoma: a 20-year experience

BACKGROUND:

Anorectal melanoma is a rare disease with a poor prognosis. Because survival is determined by distant failure, many centers have adopted sphincter‐sparing excision for primary tumor control. However, this approach is associated with high rates of local failure (～50%). In this study, the authors report their 20‐year experience with sphincter‐sparing excision combined with radiation therapy (RT) for the treatment of localized anorectal melanoma.

METHODS:

The authors reviewed the records of 54 patients with localized anorectal melanoma who were treated at the University of Texas MD Anderson Cancer Center from 1989 to 2008. All patients underwent definitive local excision with or without sentinel lymph node biopsy or lymph node dissection. RT (25‐36 grays in 5‐6 fractions) was delivered to extended fields that targeted the primary site and draining pelvic/inguinal lymphatics in 39 patients and to limited fields that targeted only the primary site in 15 patients.

RESULTS:

The 5‐year rates of local control (LC), lymph node control (NC), and sphincter preservation were 82%, 88%, and 96%, respectively. However, because of the high rate of distant metastasis, the overall survival (OS) rate at 5 years was only 30%. Although there were no significant differences in LC, NC, or OS based on RT field extent, patients who received extended‐field RT had higher rates of lymphedema than patients who received limited‐field RT.

CONCLUSIONS:

The current results indicated that combined sphincter‐sparing local excision and RT is a well tolerated approach that provides effective LC for patients with anorectal melanoma. Inclusion of the inguinal lymph node basins in the RT fields did not improve outcomes and was associated with an increased risk of lymphedema. Cancer 2011;. © 2011 American Cancer Society.     

肛管直肠恶性黑色素瘤的临床病理特征分析

目的探讨肛管直肠恶性黑色素瘤（anorectal malignant melanoma,AMM）的临床病理学特征。方法收集南京中医药大学附属南京中医院2008年7月至2019年8月收治的15例AMM患者的临床病理资料,分析其临床及病理组织学特征、免疫表型及BRAF基因突变情况,并复习相关文献进行分析。结果15例AMM患者年龄45～88岁（中位年龄66岁）,男5例,女10例。临床主要表现为便血、肛管直肠肿物、肛门坠胀疼痛。15例中仅有2例临床诊断为恶性黑色素瘤。镜下肿瘤细胞形态多样,异型性明显,核大,核仁明显,胞浆内可见黑色素颗粒。免疫组化结果显示肿瘤细胞表达S 100、Vimentin、HMB 45及Melan A,不表达CK、EMA、LCA,Ki 67表达10%～60%阳性不等,1例可见BRAF基因突变。结论AMM是一种临床少见、预后较差的恶性肿瘤,临床表现无特异性,临床误诊率高,明确诊断需结合免疫组化标记物检查。治疗上以手术为主,辅以放疗、化疗及免疫治疗等。     

Two cases of anorectal malignant melanoma

Although on anorectal malignant melanoma is relatively rare, we report two cases, that of a 88-year-old woman, and a 78-year-old man. Both patients were admitted to the hospital due to rectal bleeding and were diagnosed as having rectal carcinoma. Following an abdominoperineal rectal amputation, a pathological examination of the resected specimens revealed a malignant melanoma. Thus, it is necessary to carry out a careful examination of patients with tumors of the anorectal region when there is the least suspicion of a malignancy.     

Biochemotherapy in patients with metastatic anorectal mucosal melanoma

BACKGROUND: Patients with metastatic anorectal melanoma generally have an unfavorable prognosis, but no effective systemic therapy has been reported. METHODS: The authors retrospectively evaluated the medical records of all patients with metastatic anorectal melanoma treated with biochemotherapy between January 1991 and December 2001 at the University of Texas M. D. Anderson Cancer Center (Houston, TX). RESULTS: The search yielded 18 patients. Of these patients, 14 had undergone treatment with cisplatin (CDDP), vinblastine (VB), dacarbazine (DTIC), interferon alpha-2b (IFN), and interleukin 2 (IL-2); 2 had undergone treatment with CDDP, VB, DTIC, and IFN; 1 had undergone treatment with CDDP, IFN, and IL-2; and 1 had undergone treatment with CDDP, VB, temozolomide, IFN, and IL-2. All IL-2 treatments were administered intravenously. The median follow-up time was 12.2 months (range, 3.5-43.7 months). Eight patients (44%) had major responses, including two (11%) complete responses (CRs). Three patients were lost to follow-up evaluation after the completion of treatment. The median time to progression among the 15 remaining patients was 6.2 months. Four patients, including 1 with a CR, were alive at their last documented follow-up visits (survival: 14.0, 20.7, 31.3, and 43.7 months, respectively). The median overall survival was 12.2 months. Among 13 patients who received biochemotherapy as first-line systemic therapy, 6 patients (46%) had major responses, including two (15%) CRs. The median time to progression for this group was 6.2 months, and the median overall survival was 12.9 months. CONCLUSIONS: Biochemotherapy had substantial activity against metastatic anorectal melanoma and should be considered for use in the treatment of metastatic disease from primary anorectal melanoma.     

肛管直肠恶性黑色素瘤临床研究进展

肛管直肠恶性黑色素瘤临床罕见,临床症状不典型,误诊率高,结合指诊、内镜、超声、CT、MRI、PET-CT、电镜及免疫组织化学结果有助于提高诊断率.本病易复发转移,预后极差,尚无有效的治疗手段,应提高对肛管直肠恶性黑色素瘤的警惕,重点在于早期发现、正确诊断、多学科合理综合治疗,以提高患者生存率.     

直肠肛管恶性黑色素瘤诊治体会

目的探讨直肠肛管恶性黑色素瘤的临床特点及生存分析。方法回顾性分析22例直肠肛管恶性黑色素瘤患者的临床资料和生存率。结果本组22例,占同期收治结直肠癌的0.3%（22/7300）,首发症状为便血（77.3%）,误诊率68.2%,首诊转移率63.6%;腹会阴联合切除术16例,局部切除术4例;辅助化疗9例,辅助免疫治疗6例;辅助放疗1例。22例患者1、3、5年生存率分别为54.5%、13.6%、9.1%。中位生存时间12月（95%CI：7.48-16.52）。结论直肠肛管恶性黑色素瘤少见,易误诊,易转移,预后差。外科治疗是目前无转移直肠肛管恶性黑色素瘤的首选治疗方法。     

Malignant melanoma in the anorectal region--a report of two cases

Case 1. A 61-year-old male, suffering from an anorectal tumor with anal bleeding, underwent an abdominoperineal rectal amputation with an R3 lymphadenectomy. The tumor was a Stage I (H0, P0, N0, S0) malignant melanoma. Despite DAV (DTIC, ACNU, and VCR) therapy, liver metastasis was detected 1 year later and the patient died 1.5 years after the operation. Case 2. A 80-year-old female, complaining of bloody stool, was diagnosed to have an anorectal malignant melanoma. She was at Stage IV and underwent an abdominoperineal rectal amputation with an R3 lymphadenectomy. The tumor 4.5 X 2.7 X 1.2 cm in size, was diagnosed to be at Stage IV (pm, n2+). She is still alive 4 years later without having received sufficient chemotherapy.     

Survival following surgical treatment for anorectal melanoma seems similar for local excision and extensive resection regardless of nodal involvement

BackgroundAnorectal melanoma is a rare malignancy with a dismal prognosis. The purpose of this study was to investigate whether the survival per stage is influenced by the surgical approaches (local excision or extensive resection), to assess prognostic factors of survival, and to answer the question whether the practiced surgical approaches changed over time.MethodsDutch cancer registry organizations (IKNL and PALGA) were queried for all patients with a diagnosis of anorectal melanoma (1989–2019). Patients with disseminated disease at diagnosis were excluded. Survival outcomes were compared for the two surgical approaches stratified by stage (clinical node negative (cN0) and clinical node positive (cN+)) and date of diagnosis.ResultsA total of 103 patients were included in this study. In both cN0 and cN+ patients the surgical strategy did not significantly influence survival (cN0: 21.7% 5-year survival, median 25 months for local excision versus 13.7% 5-year survival, median 17 months for extensive resection (p = 0.228), cN+: 11.1% 5-year survival for local excision, median 17 months versus 8.7% 5-year survival, median 14 months for extensive resection (p = 0.741)). Stage and date of diagnosis showed to be prognostic factors of survival. The ratio between the two surgical approaches was unchanged over three decades.ConclusionsExtensive resection does not seem to improve survival in both cN0 and cN+ anorectal melanoma patients compared to local excision. However in the past three decades no shift towards local excision has been found. cN+ stage and an older date of diagnosis are predictors for worse survival.     

肛管直肠恶性黑色素瘤27例临床分析

目的：总结原发性肛管直肠恶性黑色素瘤的临床特征、诊断、外科治疗及预后。方法：回顾性分析我院1978-2005年收治的27例经外科治疗的肛管直肠恶性黑色素瘤病人的临床资料。结果：本组27例,占我院同期肛管直肠恶性肿瘤手术例数1.1%。主要临床特征：便血16例,肛门口肿物14例,排便习惯改变4例。手术方式：腹会阴联合切除术13例,局部广泛切除10例,局部切除2例,乙状结肠造瘘2例。全组病例3年生存率18.5%,5年生存率11.1%。结论：肛管直肠恶性黑色素瘤是一种恶性程度极高的疾病,早期诊断及治疗相当重要,局部广泛切除术创伤小,可提高患者生活质量,不降低生存时间。     

Primary anorectal malignant melanoma: report of two cases

Primary anorectal malignant melanoma is a fairly uncommon but highly malignant disease. It is sometimes mistaken for benign conditions such as hemorrhoids or rectal polyps. Here we describe two cases of primary malignant melanoma of the rectum: in one patient a wide local excision (WLE) was performed and in the other an abdominoperineal resection (APR), both with curative intent. Both patients developed systemic recurrences and died of their disease at 24 and 10 months, respectively. In conclusion, the prognosis of anorectal melanoma is poor, irrespective of surgical treatment. WLE is the first choice for primary anorectal melanoma, while APR should be reserved for those cases where complete transrectal tumor resection is technically impossible.     

肛管直肠恶性黑色素瘤38例临床分析

目的 通过对肛管直肠恶性黑色素瘤患者的临床病理特征、诊断治疗及生存预后进行分析,规范手术方式,探讨综合治疗模式.方法 回顾性分析38例经手术治疗的肛管直肠恶性黑色素瘤患者的临床病理资料,分析其与预后的相关性.结果 本组38例患者中,男10例,女28例,平均年龄58.7岁（28～75岁）,行腹会阴联合切除术28例,局部扩大切除术10例.1、3、5年无病生存率分别为64.9％、18.5％、5.7％,1、3、5年总生存率分别85.8％、24.1％、6.4％.肿瘤厚度≥1.51 mm、肿瘤直径≥3 cm与淋巴结转移相关（x2值分别为13.093、4.449;P值分别为0.011、0.020）,且肿瘤厚度亦与远处转移相关（χ^2=11.965,P=0.018）.单因素分析显示,术后辅助治疗与无病生存相关（χ^2=7.441,P=0.006）;肿瘤厚度、淋巴结转移、临床分期与总生存相关（χ^2值分别为16.741、16.474、16.775;P值分别为0.002、0.000、0.000）.多因素分析显示,术后辅助治疗为无病生存的独立危险因素（95％CI 1.420～17.621,P=0.012）;肿瘤厚度、淋巴结转移为总生存的独立危险因素（95％ CI 0.250～0.949,P=0.035;95％ CI 1.033～2.573,P=0.036）.结论 早期诊断、合理选择手术方式、重视免疫治疗的多学科协作诊疗是提高肛管直肠恶性黑色素瘤患者生存质量、延长生存期的关键.     

肛管直肠恶性黑色素瘤27例临床分析

目的:总结原发性肛管直肠恶性黑色素瘤的临床特征、诊断、外科治疗及预后。方法:回顾性分析我院1978-2005年收治的27例经外科治疗的肛管直肠恶性黑色素瘤病人的临床资料。结果:本组27例,占我院同期肛管直肠恶性肿瘤手术例数1.1%。主要临床特征:便血16例,肛门口肿物14例,排便习惯改变4例。手术方式:腹会阴联合切除术13例,局部广泛切除10例,局部切除2例,乙状结肠造瘘2例。全组病例3年生存率18.5%,5年生存率11.1%。结论:肛管直肠恶性黑色素瘤是一种恶性程度极高的疾病,早期诊断及治疗相当重要,局部广泛切除术创伤小,可提高患者生活质量,不降低生存时间。     

10例肛管直肠恶性黑色素瘤临床病理免疫组化研究

目的研究肛管直肠恶性黑色素瘤（简称恶黑）的临床病理特点及免疫组化染色在恶黑诊断中的作用。方法对肛管直肠恶黑的临床资料进行回顾性分析，用免疫组化s—P法作HMB45、S-100、Vimetin等染色。结果10例肛管直肠恶黑临床初步诊断恶黑1例，误诊其它疾病9例。病理形态：上皮样细胞为主7例，梭形细胞为主2例，小细胞似淋巴细胞样细胞为主1例。免疫组化染色：10例HMB-45、s-100均阳性，9例Vimetin阳性，其中1例CK阳性，LCA阴性。结论肛管直肠恶黑临床表现大便带鲜血，无明显黏液，肛门异物及息肉样突出肛门为特征，临床极易误诊。形态观察支持恶黑起源于表皮基底层黑色素细胞，免疫标记提示黑色素细胞起源于神经嵴。HMB45、S-100、Vimetin，三者联合应用能提高恶黑病理诊断的准确性。     

Maximizing local tumor control and survival after proton beam radiotherapy of uveal melanoma

PURPOSE: This study reports local tumor control and survival after proton beam radiotherapy (PBRT) of uveal melanoma. It identifies the risk factors for local tumor-control failure and for ocular tumor-related death. It presents the improvements implemented to increase the rate of local tumor control, and compares the survival rate of patients with locally controlled tumors to those of patients who had to receive a second treatment. PATIENTS AND METHODS: We have treated 2,435 uveal melanomas with PBRT between March 1984 and December 1998. Data were analyzed as of September 1999. Patients' age ranged from 9 to 89 years; there were 1,188 men and 1,247 women. The largest tumor diameter ranged from 4 to 26 mm, and tumor thickness from 0.9 to 15.6 mm. Median follow-up time was 40 months. RESULTS: Local tumor control probability at 5 years was improved from 90.6 +/- 1.7% for patients treated before 1988, to 96.3 +/- 0.6% for patients treated between 1989 and 1993, and became 98.9 +/- 0.6% for patients treated after 1993. Among 2,435 treated patients, 73 (3%) had to receive a second treatment because of tumor regrowth. Cause-specific survival at 10 years was calculated to 72.6 +/- 1.9% for patients with controlled tumors compared to 47.5 +/- 6.5% for those with recurrent tumors. CONCLUSION: Reduced safety margins, large ciliary body tumors, eyelids within the treatment field, inadequate positioning of tantalum clips, and male gender were identified to be the main factors impairing local tumor control. The improvement of local tumor control rate after 1993 is attributed to changes implemented in the treatment procedure. Our data strongly support that the rate of death by metastases is influenced by local tumor control failure: improvement of the local tumor control rate results in a better survival rate.     

Isolated limb infusion for local control of lower limb melanoma: Radiologic aspects

To describe the technical radiological aspects of isolated limb infusion (ILI) to assist those procedural radiologists who carry out ILI on an occasional only basis and to inform the Australian radiologist community about this deserving but relatively little known radiological procedure. Retrospective audit of radiological catheter placement for 23 lower limb ILI procedures carried out for 16 patients with locally recurrent melanoma over 2 years (January 2002 to December 2003). Arterial and venous catheter placement, although sometimes difficult, was successful in all but four occasions. Unfavourable vascular anatomy was the main reason for failure. If approached systematically and with the knowledge of the patient’s vascular anatomy, the outcome will be satisfactory in the hands of the generalist. Knowing the common technical pitfalls will certainly assist. Isolated limb infusion deserves to be more widely known in the radiological and surgical communities.     

Mechanisms of local immunosuppression in cutaneous melanoma

Cutaneous melanoma is highly immunogenic, yet primary melanomas and metastases develop successfully in otherwise immunocompetent patients. To investigate the local immunosuppressive microenvironment, we examined the presence of suppressor T lymphocytes and tolerising dendritic cells (DCs), the expression of immunosuppressive cytokines (IL-10, TGFbeta1 and TGFbeta2) and the enzyme indoleamine 2,3-dioxygenase (IDO) using qRT-PCR and immunohistochemistry in primary skin melanomas, negative and positive sentinel lymph nodes (SLN), and lymph nodes with advanced metastases. Our results indicate that tolerogenic DCs and suppressor T lymphocytes are present in melanoma at all stages of disease progression. They express transforming growth factor beta receptor 1 (TGFbetaR1), and are therefore susceptible to TGFbeta1 and TGFbeta2 specifically expressed by primary melanoma. We found that expression of IDO and interleukin 10 (IL-10) increased with melanoma progression, with the highest concentration in positive SLN. We suggest that negative SLN contain immunosuppressive cells and cytokines, due to preconditioning by tolerogenic DCs migrating from the primary melanoma site to the SLN. In primary melanoma, TGFbeta2 is likely to render peripheral DCs tolerogenic, while in lymph nodes IDO and TGFbeta1 may have a major effect. This mechanism of tumour-associated immunosuppression may inhibit the immune response to the tumour and may explain the discrepancy between the induction of systemic immunity by anti-melanoma vaccines and their poor performance in the clinic.