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.     

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.     

Surgical therapy for anorectal melanoma

BACKGROUND: Anorectal melanoma is a rare but highly lethal malignancy. Historically, radical resection was considered the "gold standard" for treatment of potentially curable anorectal melanoma. The dismal prognosis of this disease has prompted us to recommend wide local excision as the initial therapeutic approach. The purpose of this study was to review our results in patients who underwent wide local excision or radical surgery (abdominoperineal resection [APR]) for localized anorectal melanoma. STUDY DESIGN: We reviewed the charts of all patients referred for resection of anorectal melanoma between 1988 and 2002. Endpoints included overall survival, disease-free survival, and local, regional, or systemic recurrence. RESULTS: Fifteen patients underwent curative-intent surgery; four underwent APR and 11 underwent wide local excision. Eight patients (53%) are alive; 7 (47%) are disease-free (followup 6 months to 13 years). Of 12 patients who have been followed for more than 2 years, 4 are alive (33%) and 3 are disease-free (25%). Seven patients have been followed for more than 5 years and two are alive and disease-free (29%). All of the longterm survivors underwent local excision as the initial operation. There were no differences in local recurrence, systemic recurrence, disease-free survival, or overall survival between the APR group and the local excision group. Local recurrence occurred in 50% of the APR group and 18% of the local excision group; regional recurrence occurred in 25% versus 27%. Distant metastases were common (75% versus 36%). CONCLUSION: In patients who have undergone resection with curative intent for anorectal melanoma, most recurrences occur systemically regardless of the initial surgical procedure. Local resection does not increase the risk of local or regional recurrence. APR offers no survival advantage over local excision. We advocate wide local excision as primary therapy for anorectal melanoma when technically feasible.     

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     

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。     

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。     

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.     

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.     

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.     

Laparoscopic Surgery for Anorectal Malignancies Other than Carcinoma

Objective:

Numerous concerns have been raised relative to the appropriateness of laparoscopic surgery for cure of rectal adenocarcinomas. However, because of their rarity, little information exists about the role of laparoscopy for other anorectal malignancies. We report the outcome of five patients who underwent laparoscopic surgery for other anorectal malignancies.

Methods:

All patients who underwent laparoscopic surgery for treatment of non-carcinomatous anorectal malignancy were assessed by means of endoscopic, radiological and histopathologic diagnostic tests.

Results:

Two patients with anorectal melanoma and one with anal leiomyosarcoma underwent laparoscopic abdominoperineal resection. A laparoscopic loop ileostomy was performed for an HIV-positive patient with rectal Kaposi''s sarcoma. Another patient with anorectal melanoma had intraoperative identification of distant liver metastasis and therefore underwent diagnostic laparoscopy instead of an intended abdominoperineal resection. There were no intraoperative laparoscopic complications. During the follow-up period three patients who underwent abdominoperineal resection were alive, one of whom had rectal melanoma and developed liver metastasis without local recurrence. The two patients with distant liver metastasis and rectal Kaposi''s sarcoma died 46 days and five months after surgery, respectively. There were no port-site or local recurrences.

Conclusion:

Laparoscopic abdominoperineal resection for non-carcinomatous anorectal malignancies is technically feasible and avoids many of the concerns associated with attempted curative laparoscopic resection of carcinoma.     

Anorectal melanoma: clinical characteristics and the role of abdominoperineal resection.

Twenty-four patients with primary anorectal melanoma diagnosed since 1974 have been retrospectively studied. The most common presenting symptom was rectal bleeding, typically misdiagnosed as hemorrhoids. Progressive disease most commonly presented as a large pelvic mass, diffuse bilateral pulmonary nodules, or diffuse liver metastases. Twenty-one patients (88%) died of their disease; none survived more than 6 years. Among the patients who have died of their disease, mean survival was 2.2 years. Among assessable stage I patients initially managed with abdominoperineal resection (APR), 50% developed recurrent local regional disease (mean disease-free interval = 23 months), compared with 100% of those managed with more limited surgery (mean disease-free interval = 16 months). Even after APR, however, distant metastases were common, and there was no prolongation of survival for patients treated with APR. Primary melanoma of the anorectum has a high metastatic potential and carries a grave prognosis. APR appears to have some effect in controlling local and regional disease, but prolongation of survival will depend both on earlier diagnosis and on development of more successful therapeutic approaches.     

Multiple Synchronous Anorectal Malignant Melanoma Coexisting with Adenocarcinoma of the Sigmoid Colon

Primary anorectal malignant melanoma is an exceptionally rare neoplasm associated with poor prognosis. Anorectal malignant melanoma has been very rarely described with coexisting primary tumors of the colorectum. A 56-year-old female patient was admitted with a history of rectal bleeding. She had experienced increasing constipation and a sense of obstruction in the rectum for 6 months. Flexible rectosigmoidoscopy showed a large, pedinculated polypoid lesion extending from the anal canal to the rectum. She underwent a transanal local excision and was diagnosed with a melanoma of the anorectum with positive margins. Therefore, a formal abdominoperineal resection was performed. In addition to multiple synchronous anorectal malignant melanoma, we incidentally found another primary tumor in the proximal surgical margin of the resected specimen. Histopathologically, the lesion was an intramucosal adenocarcinoma of the sigmoid colon. Postoperatively, the patient received adjuvant chemotherapy of six cycles duration. At present, the patient has completed 18 months of follow-up.     

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.     

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.     

Melanoma anorrectal. Revisión de conjunto

Anorectal melanoma is an uncommon and aggressive disease. Because the patients often present with non specific complaints, a high clinical suspicion is important to avoid a delayed diagnosis. Patients undergoing radical surgery have no significant survival difference compared to those undergoing wide local excision. Abdominoperineal resection should be reserved for selected patients in whom local excision is not technically possible or cannot obtain a clear margin. The indiscriminate use of groin dissection is not advisable in anorectal melanoma and should be use in selected cases. Systemic chemotherapy is generally a non effective treatment and continues be studied. Radiation therapy can be used as hypofractionated radiation therapy combined with local excision or in a palliative setting. The oncological outcomes in anorectal melanoma are very poor. The aim of the present study is to review clinicopathology features and management of anorectal melanoma.     

Primary malignant melanoma of the bladder

Primary malignant melanomma of bladder is extremely rare: 18 cases are reported to date. An 82 year-old man underwent trans-urethral resection of bladder for a bleeding tumor of the posterior wall. Histological diagnosis was melanoma of the bladder. There was no history of previous or regressed cutaneous malignant melanoma. Margins of the bladder lesion contained atypical melanocytes similar to those commonly seen in the periphery of primary mucous membrane lesions. Clinical studies and radiological examinations were negative for other primary site of melanoma. The patient had a bladder recurrence that was consistent with primary tumor and died of widespread disease 9 months after diagnosis.     

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。     

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.     

Melanoma of the anorectum

The therapy of choice in cases with the prognostically poor (mean 5-year-survival rate maximal 12%) and rare anorectal melanoma is the surgical removal. Primary local excision should be done if technically feasible. On the basis of an exact pathohistological examination with special respect to tumor thickness a radical exstirpation or resection should be performed in cases with lesions more than 2 mm thick. In patients with tumors less than 2 mm a clinically and endoscopically follow-up in short intervals (3 months) after local excision can be recommended. At present, no effective radiotherapy nor systemic treatment exist.