Characteristics and prognosis of primary malignant melanoma of the esophagus

Primary malignant melanoma of the esophagus is an extremely rare but highly aggressive tumor. The preoperative diagnosis is complicated for the lack of specificity. Unfortunately, the prognosis of primary malignant melanoma of the esophagus remains dismal from most literatures. To better understand this special condition, we reviewed the medical records of patients with primary malignant melanoma of the esophagus in our center, retrospectively. Seven cases were seen at Cancer Hospital (Institute) of Chinese Academy of Medical Sciences from 1975 through 2006. Six patients had complete clinical data. Of the six patients, two were females and four were males. The patients mean age was 51 years, ranging from 41 to 60 years. Similar to esophageal carcinoma, dysphagia was the most common symptom. Only one patient, however, was pathologically diagnosed as primary malignant melanoma of the esophagus preoperatively. Surgical procedures were performed to all patients. Among the six patients one accepted radiotherapy perioperatively. Four patients accepted biochemotherapy postoperatively. The most common reason for death was metastasis. Four of the six patients had metastasis to the liver, adrenal gland, heart and lymph nodes, respectively. The survival varied from 5 months to 17 years and the median survival was 8 months. Our data show that primary malignant melanoma of the esophagus is a highly aggressive disease with poor prognosis. Surgery remains the first selected therapy. The role of radiotherapy and chemotherapy in the treatment of primary malignant melanoma of the esophagus is still uncertain.     

Primary malignant melanoma of the esophagus: report of a case successfully treated with pre- and post-operative adjuvant hormone-chemotherapy

Primary malignant melanoma of the esophagus is a very rare and deadly disease, with a survival of 2.2% at 5 years and a median survival of about 10 months. The aggressive biological behavior of this disease and advanced stage at the time of diagnosis together with the lack of effective treatment have contributed to its poor prognosis. We present the case of a 45-year-old Japanese man diagnosed as having a primary melanoma of the esophagus by clinical evaluation and a histological examination of endoscopic biopsy specimens. A novel approach consisting of pre- and post-operative chemo-hormone therapy with dacarbazine, nimustine, cisplatin and tamoxifen in conjunction with radical esophagectomy accompanied by lymph node dissection was carried out. The tumor size was decreased to 70% by the pre-operative chemo-hormone therapy. During the post-operative 32 months of follow-up, no evidence of recurrence or metastatic disease has been found. Although this is only one case, the outcome observed suggests that the combination of pre- and post-operative chemo-hormone therapy and radical esophagectomy with lymph node dissection is a modality that can increase the possibility of curability or at least improve the survival of patients with primary melanoma of the esophagus.      

原发性食管恶性黑色素瘤一例报告

Primary malignant melanoma of the esophagus （PMME） is an extremely rare and aggressive tumor, comprising less than 0.2% of all primary esophageal neoplasms. There are fewer than 270 reported cases in worldwide literature, most of which are scattered cases reported. This tumor has usually been reported as a pedunculated, polypoid tasion in the middle and lower third of the esophagus. Hematogenic and lymphogenic metastases are commonly reported for PMME cases. Although surgical resection has been considered as the best possible option, the prognosis has been nonetheless poor, even combined with adjuvant chemotherapy postoperatively, according to the literatures. Here we reported such a case on its clinical presentations and treatment.     

Asymptomatic primary malignant melanoma of the esophagus

BACKGROUND: Primary malignant melanoma of the esophagus is an exceedingly rare disease. This tumor is typically aggressive and disseminates early via the lymphatics and the bloodstream with a mean survival time between 10 and 15 months after radical surgical resection. The role of chemotherapy and immunotherapy is unclear. No treatment plan for the disease has yet been established. CASE REPORT: A 78-year-old man came for a checkup with a medical history of reflux esophagitis and chronic gastritis. Esophagogastroscopy showed a bluishgray tumor of the esophagus, and histology revealed features consistent with malignant melanoma. The patient underwent total transhiatal esophagectomy with curative intention, and esophagogastric anastomosis was performed. Immunohistochemistry revealed tumor cells strongly positive for the melanoma-specific antigen HMB45 and protein S-100, and negative for cytokeratin. A proposed postoperative chemotherapy was declined by the patient. Nine months after surgery, the patient's condition deteriorated, and a mediastinal lymph node conglomerate was found. Two months later, he died of bleeding into the cervical soft tissue. CONCLUSION: Up to date, radical surgical resection is the main treatment. Very little is known about the benefits of chemotherapy and immunotherapy. However, these therapeutic modalities may play an important role in the future.     

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.     

阴茎原发性恶性黑色素瘤1例报告并文献复习

目的:探讨阴茎原发恶性黑色素瘤的生物学行为、临床病理特征、诊断、治疗方法及其预后。方法:报告1例阴茎原发性恶性黑色素瘤的诊断治疗及随访情况,并结合国内外文献进行分析。结果:本例患者肿瘤分期为Ⅰ期,行阴茎部分切除术及双侧腹股沟淋巴结清扫术,同时给予化疗及生物治疗。术后随访24 个月,体格检查正常,行盆腔 MRI 检查未见复发及远处转移。结论:阴茎原发恶性黑色素瘤临床罕见,恶性程度高,总体预后差,其确诊依靠病理,外科手术为主要治疗手段,术后结合化疗以及生物治疗可延长生存期。     

食管原发性恶性黑色素瘤的临床病理观察

目的：探讨食管原发性恶性黑色素瘤的诊断、鉴别诊断及生物学行为。方法：报道l例食管原发性恶性黑色素瘤，对其进行光镜观察、免疫组织化学及特殊染色检测，并复习文献。结果：食管原发性恶性黑色素瘤多见于中老年人，常发生于食管中、下段1／3处，大体呈息肉状；光镜下瘤细胞异型性明显，呈梭形、圆形或不规则型，以梭形细胞为主。肿瘤细胞胞核体积大，核仁清晰、染色质丰富，部分细胞胞浆内有棕黄色颗粒；免疫组化结果肿瘤细胞显示波形蛋白（Vimentin）、S-100蛋白、HMB45和Melanoma—pan阳性；不表达神经特异性烯醇化酶（neuron—speciiticenolase，NSE）、白细胞共同抗原（1eukocytecommonantigen，LCA）、细胞角蛋白（cytokeratin，CK）、嗜铬素蛋白-A（chromograninA，Cgh）、人绒毛膜促性腺激素（humanchorionicgonadotropin，HCG）、上皮膜抗原（epithelial membtane antigen，EMA）、高分子量CK、CK18、小细胞肺癌（smallcell lungcancer，SCLC）和突触素（synaptophysin）等。结论：食管原发性恶性黑色素瘤是一种罕见肿瘤，由于其症状与食管其它肿瘤相似，在活检或手术前很难确诊，对发生于中老年人的食管中、下段息肉样肿物应考虑原发性恶性黑色素瘤的可能。     

A case of malignant melanoma of the esophagus

A 56-year-old female with chest pain after a meal was found to have the black mucous membrane of the middle intrathoracic esophagus by esophagogastroduodenoscopy. The lesion was diagnosed as primary malignant esophageal melanoma without lymph nodes and other organ metastasis. We underwent a subtotal esophageal by right thoracotomy and laparotomy. She survives with relapse-free for 3 years after the surgery. There is no standard therapy because primary malignant esophageal melanoma is not common. However, we thought a surgical treatment should be performed for a curatively resectable case.     

肛管直肠恶性黑色素瘤：附15例报告

对17年来收治的15例肛管直肠恶性黑色素瘤进行了总结.男性8例,女性7例.最常见的症状是血便(11/15),其次为肛门反复脱出肿物(8/15).8例行腹会阴联合切除术,术后平均生存16个月,最长存活30个月.此外,对本瘤的病因、诊断和预后进行了讨论分析.     

Anorectal melanoma

Primary malignant melanoma of the anorectum is a rare and virulent malignancy associated with an extremely poor prognosis in spite of aggressive initial therapy. Fifty-one patients with this disease were treated at Memorial Sloan-Kettering Cancer Center during the last 50 years and only six (12%) survived five years. This report views in detail 36 of the patients treated since 1950. In this group there was a female predominance (21 females, 15 males), and median age was in the 6th decade (range 27-75). Common presenting symptoms were pain, bleeding, mass or "hemorrhoids" of 1-12 months duration. In two-thirds, of the cases, a radical surgical approach was attempted. Other therapy included local excision alone or combined with groin dissection, local excision followed by delayed rectal resection and local tumor destruction by cryosurgery or fulguration. Mean survival was 21.5 months. Three patients had palliative treatment only with radiation therapy. Histopathologic study of 30 lesions showed that two-thirds were bulky or polypoid lesions and two-thirds showed junctional changes. The virulent prognosis of primary anorectal melanoma appears directly related to tumor size and thickness. Although all four of the five-year survivors had radical surgery, the three whose tumors could be measured had superficial lesions. In general, curative efforts by radical surgery were no more effective than local treatment by excision or cryosurgery. One patient, however, did have a thin lesion but also had nodal metastases and survived over five years after radical surgery. Although these data suggest that radical resection may cure patients with lesions thinner than 3 mm, it does not exclude the possibility that local excision might also be curative. For larger lesions that have not been cured by radical surgery, more conservative local approaches by excision or cryotherapy might be the optimum way of achieving local palliation.     

前哨淋巴结活检在皮肤型和肢端型黑色素瘤临床诊疗中的价值

背景与目的：前哨淋巴结活检（sentinel lymph node biopsy,SLNB）是评估皮肤型和肢端型黑色素瘤区域淋巴结转移情况及病理学分期的重要手段。SLNB作为外科诊疗规范的重要环节,已在各大诊疗指南中被推荐,也已在中国临床应用近10年。自2017年靶向和免疫治疗应用于中国黑色素瘤治疗领域,患者预后得到显著改善。本研究旨在分析复旦大学附属肿瘤医院近5年恶性黑色素瘤患者的临床资料,评估在新药治疗时代前哨淋巴结（sentinel lymph node,SLN）状态在临床应用的价值和对预后的影响。方法：对2017—2021年在复旦大学附属肿瘤医院黑色素瘤诊治中心接受诊治的381例恶性黑色素瘤患者的临床资料进行回顾性分析。每例患者均接受原发灶扩大切除和相应的SLNB,手术后随访至少6个月。SLN定位使用美兰染色或同位素示踪。结果：本研究共入组381例恶性黑色素瘤患者,平均Breslow浸润深度为3.10 mm,69.8%为肢端型,溃疡率为57.1%,SLN阳性率为34.6%,中位无复发生存率（relapse-free survival,RFS）为17个月。SLN状态是显著影响患者预后的独立危险因素。在N_1a和T₄亚组,SLN活检数>2枚的患者具有更好的RFS。接受完整的区域淋巴结清扫（complete lymph node dissection,CLND）和未清扫患者的RFS差异无统计学意义,在SLN活检数>2枚和SLN微转移直径<1 mm的亚组未清扫者反而具有更优的RFS。结论：SLN状态仍是影响临床无显性转移的黑色素瘤预后的重要因素,对于恶性黑色素瘤患者应常规开展SLNB,在保证微创的前提下,提高SLN定位的准确性,保证SLNB充分。SLN阳性后行即刻CLND对于皮肤型和肢端型黑色素瘤患者未能带来进一步的预后改善。     

A case of recurrent malignant melanoma of esophagus responsive to combined chemotherapy, radiotherapy and cellular immunotherapy

Treatment of primary malignant melanoma of the esophagus remains challenging. We treated a 53-year-old man with pT4N2M0, Stage IVa malignant melanoma of the esophagus with esophagectomy followed by adjuvant chemotherapy. Six months later, computed tomography revealed a 12 cm disseminated tumor of the mesenterium, multiple peritoneal dissemination, and a large amount of ascites. We administered chemotherapy consisting of dacarbazine combined with cisplatin and nimustine, and radiotherapy(50 Gy)was applied to the disseminated mesenteric tumor. At another clinic, the patient was administered synchronous cellular immunotherapy consisting of dendritic cells pulsed with autologous tumor lysates and lymphokine-activated killer cells. The mesenteric tumor was extremely responsive to this trimodal treatment. Because recurrence occurred later within the left orbita muscle, we added 50 Gy of radiation to prevent blindness. The patient responded to this treatment and survived another 6 months with high quality of life. It is difficult to treat advanced malignant melanoma of the esophagus, and patient prognosis is extremely poor. In this patient, the recurrent tumors responded well to trimodal therapy consisting of chemotherapy, radiotherapy and cellular immunotherapy.     

原发性食管恶性黑色素瘤临床病理特征及组织发生

目的　讨论原发性食管恶性黑色素瘤的诊断、鉴别诊断、组织发生和生物学行为。方法　报道 2例原发性食管恶性黑色素瘤并进行临床表现、组织形态学和免疫组织化学的观察研究 ,结合文献对其诊断、鉴别诊断和组织来源进行探讨。结果　原发性食管恶性黑色素瘤好发于中老年 ,临床症状多为进行性吞咽困难 ,肿瘤多位于食管中下段 ,大体形态多呈息肉状 ,Fontana组织化学染色瘤细胞显示出嗜银性黑色素颗粒 ;免疫组化染色瘤细胞S 10 0和HMB45均为阳性。结论　食管中下段息肉样肿物在中老年患者应想到原发性食管恶性黑色素瘤的可能 ,最后确诊特别是无色素性恶黑需要靠免疫组织化学标记或电镜的支持。该肿瘤的组织发生可能来自食管粘膜基底层的黑色素细胞。     

Characteristics of primary malignant melanoma of the esophagus with report of a case]

The case of a 72 year old woman with primary malignant melanoma of the esophagus, subjected twice to surgical operation with a 17-month interval and still alive 35 months after the onset symptoms is reported. In order to outline the biologic behaviour of this rare neoplasm, 44 similar bibliographic cases are analysed and comparisons made between melanoma and carcinoma of the esophagus as well as between melanomas of the esophagus and of the skin.     

Irinotecan (topoisomerase-I inhibitor) for the treatment of recurrent primary intracranial malignant lymphoma

Primary intracranial malignant lymphoma is a fetal disease with poor prognosis, and there is no effective treatment against recurrent primary intracranial malignant lymphomas. We report 3 cases of malignant lymphoma treated with irinotecan (topoisomerase-I inhibitor, camptothecin derivatives), an aromatic drug extracted from camptotheca acuminata. After the initial diagnosis, surgical resection followed by radiation therapy was performed for one cerebral, and two cerebellar malignant lymphomas. The tumors recurred 1 month, 18 months, and 18 months after the initial treatment, respectively. The former two cases were treated with additional radiation therapy and/or radiosurgery for the recurrent tumors; however, the tumors recurred again. All cases were treated finally with a combination therapy of irinotecan and cis-platinum followed by a maintenance therapy with irinotecan only. All cases showed a sharp roentgenographical response to the chemotherapy even after cumulative recurrences. One patient died of systemic infection, and another died of intracranial tumor recurrence 11 and 29 months after the initial diagnosis, respectively. Autopsies revealed multiple tumor recurrences in both these cases. The other patient died 31 months after the initial diagnosis, also due to intracranial tumor recurrences. These results indicate the usefulness of irinotecan for the treatment of recurrent primary intracranial malignant lymphoma; however, further investigation is necessary to establish a better protocol for irinotecan treatment against primary intracranial malignant lymphoma.     

Amelanotic malignant melanoma of the esophagus: report of a patient with recurrence successfully treated with chemoendocrine therapy

We report a case of primary amelanotic malignant melanoma of the esophagus, an extremely rare disease. A 58-year-old man was diagnosed as having middle esophageal cancer with lymph node metastasis, which was classified as esophageal cancer, Stage III:T3N1M0, by International Union Against Cancer (UICC) criteria. Preoperative chemotherapy was performed, but the response assessment was no change (NC). The patient underwent a subtotal esophagectomy via right thoracotomy and laparotomy. Reconstruction was performed by pulling up the stomach via the retrosternal route; the site of anastomosis was the neck. Adjuvant chemotherapy consisted of five courses of dacarbazine (DITC), nimustine (ACNU), vincristine (VCR), and interferon-β. Eleven months after the surgery, computed tomography (CT) demonstrated recurrence in the upper mediastinum. The patient received chemoendocrine therapy, consisting of the first planned course of DITC, ACNU, and cisplatin (CDDP), given intravenously; and tamoxifen (TAM), given orally. Subsequently with a modified regimen of this therapy he attained a complete response (CR). In general, the prognosis of esophageal malignant melanoma is very poor. Although our patient had a recurrence, he is alive 4 years and 5 months after the surgery and 3 years and 6 months after the recurrence. The chemoendocrine therapy probably contributed to this outcome.     

手术治疗原发性心脏肿瘤79例临床分析

 目的　总结原发性心脏肿瘤的临床特征和外科治疗经验。方法　回顾性分析 2002 年6 月至2012年 9月间经超声心动图确诊的79 例原发性心脏肿瘤患者的临床资料,所有患者均在全身麻醉体外循环下行原发性心脏肿瘤摘除术。结果　随访6个月至5年,5例术后复发,其余术后恢复良好,未见肿瘤复发和远处转移。结论　左房黏液瘤一经明确诊断即应尽早手术,外科手术效果佳,但是存在复发可能。心脏恶性肿瘤进展较快,应早期手术并结合综合性治疗以改善预后。     

Pancreatic Resection for Metastatic Melanoma. Case Report and Review of the Literature

Introduction

Pancreatic metastasis from several malignancies are increasingly encountered in clinical practice, and the usefulness of surgical resection has been suggested for certain neoplasms. Isolated pancreatic metastasis from malignant melanoma is a rare occurrence, and the role of surgery as an adjunct to systemic therapy for melanoma metastatic to a solitary or multiple sites is still debated.

Case Report

We report a patient with melanoma of unknown primary site metastatic simultaneously to the lung and pancreas 3 years after axillary lymph node dissection. Distal pancreatectomy with splenectomy and video thoracoscopic assisted resection of pulmonary metastasis were performed. The postoperative course was uneventful, but 6 months after surgery, the patient experienced single pulmonary recurrence. During chemotherapy with different drugs, pulmonary lesion remained stable for 1 year, and no abdominal recurrence occurred. After then, the size of the lesion progressively increased and a second metastasis occurred in the lung. Five months later, brain metastases occurred, and the patients died 24 months after surgery. Sixteen pancreatic resections for metastatic malignant melanoma, reported with adequate clinical details, were also retrieved from the literature.

Conclusion

In spite of the very limited experience, it appears that surgical resection is only a palliative procedure, because long-term survival is a rare event. However, considering the lack of effective systemic therapy, surgery may be considered as a part of an aggressive multidisciplinary approach in selected cases with malignant melanoma metastatic to single or multiple visceral sites.     

原发性单纯乳腺鳞状细胞癌的临床病理分析

目的 探讨原发性单纯乳腺鳞状细胞癌的临床病理持点和治疗方法。方法 对12例原发性单纯乳腺鳞状细胞癌患者的临床病理资料进行回顾性分析。结果 全部患者均以乳腺单发肿块为首发症状,肿块最大直径为2．5～10.0cm。均行手术治疗,术后按照美国癌症联合委员会(AJCC)与国际抗癌联盟(U1CC)联合制定的TNM分期标准分期,Ⅱa期6例,Ⅱb期2例,Ⅲa期2例,Ⅲb期2例。10例患者有随访资料,平均随访时间54．2个月,6例存活,4例死亡。结论 原发性单纯乳腺鳞状细胞癌在大多数情况下诊断采用排除其他癌的方法,最初可从穿刺细胞学诊断中得到提示。目前尚无规范性的治疗方案,预后评价不一。     

A Primary Sacral Melanoma of Unknown Origin: A Case Report

Malignant melanoma caused by malignant transformation of melanocytes is associated with high mortality and is difficult to manage. Metastasis is not uncommon (up to 31% of all cases) and is closely associated with a poor prognosis. Although rare (4–5%), extracutaneous melanoma has been reported; however, primary malignant melanoma of the sacrum is extremely rare (only three case reports to date). Here, we present a 51-year-old patient who underwent surgical treatment for a lesion of the spinal canal and associated bony structures; extensive aggressive resection was required. She underwent partial sacrectomy and lumbo-iliac fixation (to maintain spinal stability). Pathology revealed malignant melanoma. We discuss the diagnosis, surgical intervention, and postoperative follow-up, which may assist clinicians. Although metastatic malignant melanoma is usually fatal, primary extracutaneous melanoma of the spine may respond well to surgery and adjuvant radiotherapy.