Surgical treatment of lymph nodes with metastatic melanoma from unknown primary site

To determine the prognosis of patients with lymph node metastases from an unknown primary melanoma, we retrospectively reviewed the clinicopathologic features of 188 such patients treated from 1971 through 1986 and compared their records with those of patients with clinical stage II melanoma with known primary lesions. Patients with lymph node metastases from an unknown primary melanoma represented 4.6% of all patients with melanoma treated during that period. The five- and ten-year survival rates were 42% and 40%, respectively (median, 37 months). When stratified by number of tumor-containing lymph nodes, there was no significant difference in survival between patients with an unknown primary melanoma and lymph node metastases and those with clinical stage II melanoma and known primary sites. The prognosis of the former patients is no worse than that of patients with lymph node metastases from a known primary site and should be treated in a comparable manner.     

阴茎恶性黑色素瘤1例报告并文献复习

目的探讨阴茎原发恶性黑色素瘤的临床诊治及预后。方法对本院1例阴茎原发恶性黑色素瘤的诊疗情况进行回顾性分析。结果患者在本院行阴茎全切加双侧腹股沟淋巴结清扫术。病理证实阴茎浸润性恶性黑色素瘤伴双侧腹股沟多个淋巴结转移。患者术后3月复查时发现有远处转移。结论阴茎原发恶性黑色素瘤临床罕见,确诊依靠病理及免疫组化,手术为主要的治疗手段,但总体预后不佳。     

Metastatic melanoma with an unknown primary.

An infrequent initial presentation for malignant melanoma is the diagnosis of metastatic disease without a history of an obvious primary lesion. Confusion exists in the literature concerning the workup, treatment, and prognosis of the unknown primary melanoma. A retrospective, computer-aided chart review of 580 consecutively registered patients with melanoma at the University Treatment Center (Tampa, FL), identified 18 patients with an unknown primary presentation. There were 10 males and 8 females with a mean age of 38.4 years. Ninety-four percent of the patients were diagnosed with metastatic disease in a nodal basin, whereas 1 patient had a resected isolated lung mass as the initial presentation. In the patients who presented after having a biopsy of a single positive node for diagnosis, more disease was recovered in the nodal basin with a formal node dissection in 59% of the patients. Actuarial survival curves were constructed for the group with unknown primary melanoma. As a control population, survival curves were constructed of the subpopulation of patients with melanoma who had a known primary and had stage III (regional nodal disease) at diagnosis. There was no difference in survival between those with known and unknown primary melanoma (p = 0.96).     

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.     

Metastatic malignant melanoma of unknown primary site to the brain: A case report

Introduction and importanceThe natural history of metastatic melanoma in the absence of a known primary site has been poorly defined. The disease usually presents a significant cause of morbidity and mortality. Around 90% of melanomas have cutaneous origin, but still there are melanomas that could be found in visceral organs or lymph nodes with unknown primary site. Spontaneous regression of the primary site could be an explanation. The disease is frequently diagnosed after treatment for known extracranial metastases and has a poor outcome despite various local and systemic therapeutic approaches.Case presentationHerein, we present a case of a 43-year old female presented with history of headaches and enlarged a left inguinal lymph node. Notably, no cutaneous lesions could be identified by history or on physical examination. CT-scan of the brain revealed a space occupying lesion and the inguinal lymph node biopsy confirmed the diagnosis of metastatic malignant melanoma. The patient succumbed shortly after establishment of diagnosis.Clinical discussionMost patients with brain metastases from malignant melanoma are diagnosed after treatment for known extracranial metastases and have a poor outcome despite various local and systemic therapeutic approaches.ConclusionMetastatic melanomas of brain with unknown primary present a significant morbidity and mortality and confer a poor prognosis. Delay in diagnosis and treatment is of serious concern when it comes to improve the prognosis of patients with this disease. The optimal treatment depends on the objective situation, often surgery, radiosurgery, whole brain radiotherapy and chemotherapy can be used in combination to obtain longer remissions and optimal symptom relieve.     

Clinical Aspects of Unknown Primary Melanoma

Of 980 patients with malignant melanoma treated during the past seven years, 55 (5.6%) were found to have metastatic disease and no detectable primary tumor. Thirty-six of these patients with “unknown primary melanoma” had disease limited to lymph nodes (Stage II), whereas 19 had disseminated melanoma (Stage III). The sex and age distribution of these 55 patients were similar to those of a control group of 86 patients with palpable lymph node metastases from a known primary. The site of lymph node metastases for Stage II patients in each group was similar although unknown primary patients seemed to have slightly more involved lymph nodes. By studying patients with the same stage and similar extent of disease, the prognosis of unknown primary melanoma could be determined and compared to known primary melanoma. The overall recurrence rate of patients with unknown primary was no higher than that of patients with known primary. This observation appeared to be true even when patients were compared with respect to adjuvant immunotherapy. The use of adjuvant immunotherapy appeared to favorably affect recurrence rates among the unknown primary patients. Since the recurrence rate for patients with unknown primary melanoma was no higher than that of patients with known primary melanoma, we advocate aggressive surgical management. The occasional long-term survivor with Stage III unknown primary suggests that judicious surgical intervention may benefit these patients as well.     

Two cases of primary malignant melanoma of the lacrimal sac

BACKGROUND: Malignancy of the lacrimal sac is rare, and primary malignant melanoma in this region is extremely rare. METHODS: We report two cases of malignant melanoma of the lacrimal sac presented with epiphora and a palpable mass in the medial canthal area. We performed radical surgery and radiation therapy. RESULTS: The light microscopic, immunohistochemical, and electron microscopic studies confirmed the diagnosis. One of the patients, a 65-year-old-woman, has no evidence of recurrence or distant metastasis 3 years after surgery and radiotherapy, whereas another patient, a 56-year-old man, died of distant metastasis 6 months after surgery and radiotherapy. CONCLUSIONS: We present two cases of malignant melanoma of the lacrimal sac that masqueraded as chronic dacryocystitis. Head and neck surgeons should be aware of this disease entity when encountered with patients with epiphora and mass in the medial canthal area.     

阴茎原发恶性黑色素瘤4例

目的 探讨阴茎原发恶性黑色素瘤的临床症状、病理特点及诊断治疗.方法 对4例阴茎原发恶性黑色素瘤的病理和临床资料进行回顾性分析.结果 4例均行阴茎部分切除,双侧腹股沟淋巴结清扫术.术后4例均行化疗,其中2例化疗联合生物学治疗.结论 阴茎原发恶性黑色素瘤临床罕见,确诊依靠病理,手术为主要治疗手段,术后行化疗联合生物学治疗可提高生存期,但因其恶性程度高,预后不佳.     

Malignant melanoma: the patient with an unknown site of primary origin.

Four percent of 2,446 patients with malignant melanoma did not have a known site of primary origin. More than half were admitted with nodal disease only, and were treated with regional node dissections. Thirty-three percent of this group lived five years, and 22 percent lived ten years following treatment. One third were admitted with visceral metastases, many of which were amenable to surgery, and this group experienced a 5 percent five-year survival rate. Cutaneous dissemination carried a lethal prognosis. Recurrences following treatment tended toward the same region of the body as the original metastasis, and 50 percent of these recurrences occurred within six months of therapy. The sex ratio, age incidence, family history, and survival rates in these patients with unknown primary tumors are consistent with an unnoticed cutaneous lesion as the site of origin for the metastatic disease. It must be supposed that this lesion had undergone spontaneous regression.     

Primary vaginal melanoma: A critical analysis of therapy

Background Primary vaginal melanoma is a rare and highly malignant disease. The impact of therapy on outcomes is poorly understood. Methods Records of all patients treated for primary vaginal melanoma at Memorial Sloan-Kettering Cancer Center from 1977 to 2001 were reviewed. Survival analysis was performed based on appropriate patient, tumor, and treatment variables. Pathologic materials were reviewed to confirm the original diagnosis and examine appropriate clinicopathologic features. Results Thirty-five women were treated for vaginal melanoma; the primary treatment selected was surgical for 69% (24) and radiotherapy for 31% (11) of the patients. Surgical removal of the tumor was achieved in 92% (22) of the 24 patients selected for surgical therapy. At operation, radical excision with en bloc removal of involved pelvic organs was performed in 50% (12) of the 24 patients, a wide excision was performed in 42% (10), and a total vaginectomy was performed in 8% (2). Elective pelvic lymph node dissection was performed in 74% (26) of the 35 cases. Lymph node metastasis was found in only 8% (2) of these 26 patients. The overall median survival was 20 months. Primary surgical therapy was associated with longer overall survival (25 vs. 13 months;P=.039). Recurrence-free survival was not associated with the extent of surgery. None of the examined clinicopathologic features were associated with survival differences. Conclusions The prognosis is poor for patients with primary vaginal melanoma. Improved clinical outcomes were associated with surgical removal of gross disease whenever possible. Because of the low rate of lymph node metastasis, elective pelvic lymph node dissection is not obligatory. In cases of surgically unresectable disease, primary radiation therapy is indicated.     

Eleven-year survival from an intra-dermal melanoma.

Stage IV metastatic malignant melanoma of unknown primary (TxNxM1a) is known to have a poor prognosis. However, some patients suffering from cutaneous disease originally thought to represent metastasis have fared much better than expected. We report a patient who has survived 11 years following such a diagnosis. Due to the prolonged survival and absence of an identified primary, it is unlikely that the lesion was metastatic but may represent one of a number of other possibilities. A small number of similar cases in the literature suggest a need for awareness of this unusual group of patients.     

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、临床病理特点、诊断治疗方法及其预后。方法报告1例阴茎原发恶性黑色素瘤的诊断治疗、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,行阴茎全切尿道会阴造口＋双侧腹股沟淋巴结清扫术,术后行化疗并结合免疫治疗,于术后18个月出现远处转移,术后30个月因全身多发转移、多器官功能衰竭死亡。结论阴茎原发恶性黑色素瘤临床罕见,恶性程度高。其确诊依靠病理,外科手术为主要治疗手段,对于Ⅰ期、Ⅱ期的患者,阴茎全切术式较之部分切除术后生存期明显延长,对于Ⅲ期患者手术范围则对于术后生存期并无明显影响。术后结合化疗以及免疫治疗可提高生存期,但总体预后差。     

Peritoneal serous micropapillomatosis of low malignant potential (serous borderline tumors of the peritoneum). A clinicopathologic study of 17 cases.

Primary peritoneal serous micropapillomatosis of low malignant potential, or serous borderline tumor of the peritoneum, is a relatively rare lesion that is histologically indistinguishable from peritoneal "implants" associated with ovarian papillary serous tumors of low malignant potential. We analyzed 17 cases to further define the pathologic features and prognosis of this entity. The ages of the patients ranged from 16 to 67 years (mean, 33 years). Eight patients were symptomatic with chronic pelvic or abdominal pain (five patients), adnexal mass (one patient), small-bowel obstruction (one patient), and possible endometriosis (one patient). In nine cases (53%), peritoneal serous micropapillomatosis of low malignant potential was an incidental finding discovered during evaluation or treatment of other conditions. Grossly, the peritoneal lesions were focal or diffuse. They commonly appeared as miliary granules and often were believed to be peritoneal carcinomatosis. Microscopically, peritoneal serous micropapillomatosis of low malignant potential had all of the patterns seen in superficial ("noninvasive") peritoneal implants of ovarian serous borderline tumors. Psammoma bodies were a prominent feature of all cases. Twelve patients also had typical endosalpingiosis. Most patients were treated by hysterectomy and bilateral salpingo-oophorectomy. Surgical treatment in seven patients consisted only of biopsy. Ten patients had residual unresected disease at the time of their initial operation. Several patients received adjuvant chemotherapy. Follow-up was available for 14 of the 17 patients. One patient died of metastatic breast carcinoma at 3.8 years; another patient died 7 weeks after operation, possibly as a complication of therapy. The other 12 patients were alive at last known contact after follow-up intervals of 8 months to 16.2 years (mean, 7.5 years). Two of these 12 patients developed multiple episodes of small-bowel obstruction due to persistent peritoneal serous micropapillomatosis of low malignant potential; neither received adjuvant chemo- or radiotherapy. Both were alive without progressive disease 10.9 and 16.2 years after initial diagnosis, respectively. This excellent prognosis supports a regimen of conservative therapy for these patients.     

Subungual melanoma of the hand

Thirteen patients with subungual melanoma on the fingers had a mean delay before diagnosis of 1.2 years. Four patients presented with local recurrence after inadequate initial treatment and two presented with systemic metastases. Mean primary subungual melanoma thickness was 6.1 mm. and nine patients had Clark level IV or V disease. All patients underwent digital amputation. Two of seven patients who had localised disease initially are alive at 29 and 44 months. One of four patients who had locally recurrent melanoma is alive at 36 months. Both patients with systemic disease at presentation died. Advanced disease and delayed presentation contributed to the poor prognosis of subungual melanoma in our patients.     

泌尿男生殖系多原发恶性肿瘤(附22例报告)

目的　探讨泌尿男生殖系多原发恶性肿瘤的发病率及预后。　方法　回顾性分析近 8年收治的 2 2例多原发恶性肿瘤的发病率、第 1及第 2恶性肿瘤间隔时间及预后。　结果　泌尿男生殖系多原发恶性肿瘤占同期收治的泌尿男生殖系恶性肿瘤的 3 .2 % ,7例同时发病者存活时间平均 15个月 ;异时发生 15例中 3例存活 7个月～ 5年 ,12例在第 2癌治疗后 8个月至 15年仍存活。　结论　泌尿男生殖系多原发恶性肿瘤近年有增加趋势 ,第 1癌治疗后应密切随访 ,及早发现和正确治疗第 2原发癌是延长生存期的关键     

Primary genitourinary melanoma presenting as voiding dysfunction

A case of primary malignant melanoma of the urethra in a 67-year-old female is presented. Cystourethroscopy performed during a workup for pelvic organ prolapse revealed a bladder and urethral mass. Initial histologic examination was interpreted as undifferentiated sarcoma; however, after immunohistochemical staining by two separate institutions, malignant melanoma was diagnosed. Being rare, urethral melanoma is often misdiagnosed, and treatment can be delayed. Given its poor prognosis, early diagnosis is essential, and clinicians need to include it in their differential when working up a patient with genitourinary complaint.     

Primary mucosal malignant melanoma of the oral cavity

Mucosal malignant melanoma arising from the head and neck region is a rare entity, and it is more aggressive than cutaneous melanoma. Furthermore, the complex anatomy of the oral cavity makes complete surgical excision difficult. Thus, early diagnosis and treatment of a mucosal lesion are important. In this study, three mucosal malignant melanoma cases and the literature have been reviewed. Three cases who presented with a primary malignant melanoma of the oral cavity have been retrospectively analyzed. All three patients were female with a mean age of 31.3 years, and the median follow-up period was 18.6 (6–36) months. The tumor was located on the maxillary gingiva in case 1 and in the hard palate–maxillary gingiva in cases 2 and 3. Case 2 had a distant metastasis during first admission. The tumor was excised with a 2-cm surgical margin in all cases. Case 2 received adjuvant chemotherapy. During the follow-up period, case 1 had a cervical lymphadenopathy 8 months after the first operation, so she underwent cervical lymph node dissection then received chemotherapy. Melanoma of the oral cavity is very rare with an extremely poor prognosis. As some melanomas may be amelanotic, a high index of suspicion is necessary. A biopsy should be taken from any suspicious lesion in the oral cavity. Surgical excision combined with adjuvant therapy is the main treatment approach for these cases. Prognosis of the disease depends on early diagnosis and treatment. A multicenter prospective study is required to introduce staging of the disease and the optimal treatment regimen.     

Primary malignant melanoma of the oral cavity. A review of 20 cases

Twenty patients with primary malignant melanoma of the oral cavity have been described. They formed 3.9 percent of the total number of patients with malignant neoplasms of the oral cavity. The upper gingiva was most commonly affected. In this series, there were 14 male patients and 6 female patients who ranged in age from 26 to 80 years (average 58 years). The first symptom of melanoma was hyperpigmentation of the mucosa in 10 patients, tumor in 7, and pain in 3 edentulous patients with prostheses. Radical surgery was performed in 13 patients, followed by chemotherapy and radiotherapy in 9 cases. Only 1 patient survived 9 years. The remaining 12 died 11 to 18 months after radical treatment. Palliative therapy was applied in three patients, two patients were treated symptomatically, and two patients refused treatment. Early detection of melanoma is an indication for radical treatment and may increase the survival rate of patients with this disease, which is still very low.     

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.     

Thoracic metastasis of malignant melanoma of unknown primary: A case report and literature review

IntroductionMetastatic melanoma of unknown primary (MUP) is an unusual entity found in distant sites without evident skin lesion. We report a case of 45-year-old woman who underwent monobloc resection of a metastatic thoracic malignant melanoma of unknown primary, and who is currently under immunotherapy without local or distant recurrence during a follow-up of 18 months. We demonstrate through this case that R0 resection of an MUP associated with immunotherapy improves the prognosis and survival in these patients.Case reportThis is a 45-year-old woman who underwent monobloc resection of a mass carrying the anterior arch of the second left rib associated with a wedge resection of a nodule at the left upper lobe. Histology confirmed that it was a malignant melanoma. Her history was negative for melanocytic lesions, physical examination and imaging had failed to identify a primary lesion. The patient is currently under nivolumab for Stage IV melanoma and does not present any complications or recurrence during the long term follow up.DiscussionMetastatic melanoma of unknown primary (MUP) is a melanocytic lesion in distant sites in the absence of apparent skin involvement and is rare, accounting for 3, 2% of all incident melanomas as well as being yet poorly understood in terms of pathogenesis (Bae et al., 2015) [1]. MUP is clinically understudied, investigators to date have reported largely on the use of localized treatment for MUP (surgery or radiotherapy), while the efficacy of systemic therapy in MUP patients remains unexplored. Clinical trials of immunotherapy and targeted therapy in patients with advanced cutaneous melanoma have not explicitly reported response rates specific to MUP patient subgroups due to its low incidence and lack of annotation. MUP's response to these now FDA-approved therapies could add to the discussion of MUP's elusive biological characteristics, as well as aid in making clinical recommendations (Utter et al., 2017).ConclusionMetastatic MUP is an extremely rare entity which is still poorly understood, few cases are described in the literature, its treatment remains controversial and there are no specific treatment recommendations for patients with MUP. Several authors recommend local treatment when possible and tend to apply similar strategies for patients with paired stage primary known melanoma (PKM).