Primary Gastrointestinal Melanoma Research Articles

Prognostic models based on lymph node density for primary gastrointestinal melanoma: a SEER population-based analysis

ObjectiveThis study aimed to construct prognostic models to predict the overall survival (OS) and cancer-specific survival (CSS) of patients with primary gastrointestinal melanoma (PGIM).DesignAn observational and retrospective study.SettingData were obtained...

The Development and Validation of a Nomogram for Predicting Cancer-Specific Survival and a Risk Stratification System for Patients with Primary Gastrointestinal Melanoma.

The aim of our study was to develop and validate a nomogram to predict cancer-specific survival and make a risk stratification system for primary gastrointestinal melanoma. Patients with primary gastrointestinal melanoma in the Surveillance, Epidemiology, and End Results database between 2000 and 2018 were included and randomly divided into the training and validation cohort (8:2). A prediction nomogram of cancer-specific survival was constructed based on the risk factors identified in the multivariate Cox regression. Calibration curve, time-dependent receiver operating characteristic, and decision curve analysis were performed. Further, a risk stratification system was developed based on the nomogram. A total of 433 patients were included. The nomogram was constructed based on age, site, and tumor size, Surveillance, Epidemiology, and End Results (SEER) stage, and therapy. The area under the curves of the nomogram predicting 6-, 12-, and 18-month cancer-specific survival were 0.789, 0.757, and 0.726 for the internal validation and 0.796, 0.763, and 0.795 for the external validation. Calibration curves and decision curve analysis were performed. Further, patients were divided into 2 risk subgroups. The Kaplan-Meier analysis and the log-rank test showed that the risk stratification made well differentiation of patients with different risks of cancerspecific survival. We developed and validated a practical prediction model of cancer-specific survival and a risk stratification system for patients with primary gastrointestinal melanoma, which might be available in clinical practices.

Interaction between Age and Primary Site on Survival Outcomes in Primary GI Melanoma over the Past Decade.

Primary malignant melanomas of the Gastrointestinal mucosa are uncommon. Most cases of gastrointestinal (GI) melanomas are secondary, arising from metastasis at distant sites. The purpose of this study is to assess to what extent the interaction between independent prognostic factors (age and tumor site) of primary GI melanoma influence survival. Furthermore, we also aimed to investigate the clinical characteristics, survival outcomes, and independent prognostic factors of patients with primary GI melanoma in the past decade. A total of 399 patients diagnosed with primary GI melanoma, between 2008 and 2017, were enrolled in our study by retrieving data from the Surveillance, Epidemiology, and End Results (SEER) database. We analyzed demographics, clinical characteristics, and overall mortality (OM) as well as cancer-specific mortality (CSM) of primary GI melanoma. Variables with a p value < 0.1 in the univariate Cox regression were incorporated into the multivariate Cox model (model 1) to determine the independent prognostic factors, with a hazard ratio (HR) of greater than 1 representing adverse prognostic factors. Furthermore, we analyzed the effect of the interaction between age and primary location on mortality (model 2). Multivariate cox proportional hazard regression analyses revealed higher OM in age group 80+ (HR = 5.653, 95% CI 2.212-14.445, p = 0), stomach location of the tumor (HR = 2.821, 95% CI 1.265-6.292, p = 0.011), regional lymph node involvement only (HR = 1.664, 95% CI 1.051-2.635, p < 0.05), regional involvement by both direct extension and lymph node involvement (HR = 1.755, 95% CI 1.047-2.943, p < 0.05) and distant metastases (HR = 4.491, 95% CI 3.115-6.476, p = 0), whereas the lowest OM was observed in patients with small intestine melanoma (HR = 0.383, 95% CI 0.173-0.846, p < 0.05). Multivariate cox proportional hazard regression analyses of CSM also revealed higher mortality of the same groups and lower CSM in small intestine and colon melanoma excluding the rectum. For model 2, considering the interaction between age and primary site on mortality, higher OM was found in age group 80+, followed by age group 40-59 then age group 60-79, regional lymph node involvement only, regional involvement by both direct extension and lymph node involvement and distant metastases. The small intestine had a lower OM. The rectum as primary location and the age range 40-59 interacted to lower the OM (HR = 0.14, 95% CI 0.02-0.89, p = 0.038). Age and primary gastric location did not interact to affect the OM. For the CSM, taking into account the interaction between age and the primary location, higher mortality was found in the same groups and the colon location. The primary colon location also interacted with the age group 40-59 to increase the CSM (HR = 1.38 × 109, 95% CI 7.80 × 107-2.45 × 1010, p = 0). In this United States population-based retrospective cohort study using the SEER database, we found that only the age range 40-59 interacted with the rectum and colon to lower and increase mortality respectively. Primary gastric location, which was the single most important location to affect mortality, did not interact with any age range to influence mortality. With those results, we hope to shed some light on this rare pathology with a very dismal prognosis.

Incidence and survival of patients with primary gastrointestinal melanoma: a population-based study.

Primary gastrointestinal melanoma (PGIM) has received more attention because of its inferior prognosis. Less is known about the incidence and survival rate of PGIM. PGIM data were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. The incidence was estimated by age, sex, race, and primary site. Trends in incidence were described as annual percent change (APC). Cancer-specific survival (CSS) and overall survival (OS) rates were estimated and compared using log-rank tests. Cox regression analyses were performed to identify independent prognostic factors. The overall incidence of PGIM was 0.360/1,000,000 with a significant upward trend (APC = 1.77%; 95% CI 0.89%-2.67%, p < 0.001) from 1975 to 2016. Most PGIM occurred in the large intestine (0.127/1,000,000) and anorectum (0.182/1,000,000), and both incidences were almost 10 times higher than those of other sites, including the esophagus, stomach, and small intestine. The median survival time was 16months (IQR, 7-47months) for CSS and 15months (IQR, 6-37months) for OS, and the 3-year CSS and OS rates were 29.5% and 25.4%, respectively. Older age, advanced stage, absence of surgery, and melanoma in the stomach were the independent risk indicators of survival and associated with worse CSS and OS. The incidence of PGIM has been increasing over the past decades and the prognosis is poor. Thus, further studies are warranted to improve the survival, and more attention should be paid to the patients that are elderly, patients with advanced stage, and patients with melanoma in the stomach.

Primary Small Bowel Amelanotic Melanoma and Intestinal Intussusception: A Case Report

Primary gastrointestinal melanomas (PGM) are usually asymptomatic in the early stages, leading to misdiagnosis. We reported the case of a 75-year-old male patient admitted to our service due to hematochezia, and fatigue. Primary investigation showed small bowel intussusception and surgical treatment was performed. Histopathology and immunohistochemistry analysis identified primary small bowel amelanotic melanoma at an advanced tumor stage. Whereas the patient had a sudden impairment of performance status during hospitalization, no curative oncological approach could be performed. This report highlights the clinical and imaging findings that led to the primary gastrointestinal amelanotic melanoma diagnosis in an elderly patient with intestinal intussusception. The early recognition of prior intussusception signs in adults and elder patients may lead to an earlier oncological diagnosis and a better prognosis.

Gastrointestinal: Endoscopic ultrasound of rectal subepithelial lesion mimicking gastrointestinal stromal tumor.

A 60-year-old man presented with anorexia, weight loss, and constipation for 3 months. On peri-rectal examination, a bulge was palpated in the rectum. He underwent colonoscopy, which showed large subepithelial lesion (SEL) starting from anorectal junction extending proximally till rectosigmoid junction obliterating the lumen without complete obstruction (Fig. 1a). Contrast-enhanced computed tomography of the pelvis showed a large lobulated heterogeneous enhancing soft tissue lesion within the rectum (Fig. 1b). Endoscopic ultrasound (EUS) was performed, which showed a large well defined lobulated hetero-echoic lesion (70 × 63 mm) with multiple cystic spaces in peri-rectal area (Fig. 1c). Possibility of rectal malignant gastrointestinal stromal tumor (GIST) was considered looking at CT and EUS image. To confirm our diagnosis, EUS-guided fine needle aspiration (FNA) was performed. During FNA, dark brownish fluid was aspirated (Fig. 2a). Possibility of bleed within tumor was considered because of dark brownish fluid aspiration; however, black pigments were noticed on slide preparation (Fig. 2b). Histopathological examination showed pleomorphic cells with abundant brownish pigment with multinucleation suggestive of malignant melanoma (Fig. 2c). Patient was advised for surgery and referred to the surgeons. Malignant melanoma is an extremely rare and aggressive tumor of the rectum. It accounts for 0.5–4% of all anorectal malignancies and < 1% of all melanomas. It is most common site of primary gastrointestinal melanoma followed by stomach, small intestine, and colon. Patients usually present in the fifth or sixth decade, and it is more common in female patients. Anorectal melanomas usually appear as ulcerated polypoidal lesions rather than SEL. EUS is an important diagnostic tool in diagnosis and differentiation of SEL. There is lack of characterization of anorectal melanoma on EUS because of rarity of tumor. Yonal et al. described the EUS morphological features of esophageal melanoma as iso to hypoechoic solid tumor mass with variable areas of internal hyper-echogenicity and anechoic cystic spaces, similar to the findings from our case. Morphological features of some SELs are confusing; therefore, EUS FNA plays an important role in differentiation of SELs. Therapy in anorectal melanomas is dictated by the local staging of disease and also the presence of lymph node and distant metastases. Multidisciplinary approach is required depending on the stage of tumor. Surgery has been the mainstay of treatment for anorectal melanoma. Although these tumors are usually radio-resistant and chemo-resistant, combination of chemotherapy and immunotherapy remains a therapeutic option for advanced metastatic disease. Overall, 5-year survival is only 3–22%, and survival in recurrent or metastatic disease is less than 10 months. Although a rare entity, anorectal melanoma can mimic GIST and may be a differential during the evaluation of rectal SEL. Early diagnosis of tumor is of paramount importance for favorable prognosis.

METASTATIC MULTIPLE PRIMARY GASTROINTESTINAL MELANOMA: A CASE REPORT

Primary gastrointestinal melanoma is a rare tumor with a frequency of 1 new case per 5–7 years. Over the past 100 years, there have been no more than 25 reported cases of primary melanoma of the stomach and colon. The rarity of the disease and nonspecific clinical features pose diagnostic challenges. The tumor is characterized by an aggressive growth and early metastasis. Case description. We present a rare clinical case of primary gastrointestinal melanoma with metastatic involvement of the adrenal glands, liver and retroperitoneal lymph nodes. Conclusion. To date, treatment standards for primary gastrointestinal melanoma have not been developed and are the subject of debate. However, palliative chemotherapy used for the treatment of primary skin melanoma may lead to disease stabilization in patients with gastrointestinal melanoma.

Presentation, Management, and Prognosis of Primary Gastrointestinal Melanoma: A Population-Based Study

BackgroundPrimary gastrointestinal (GI) melanomas, compared with cutaneous melanomas, have a much lower incidence. As a result, there is a paucity of data regarding their presentation, treatment, and prognosis. The aim of this study was to analyze the incidence, patient characteristics, treatment patterns, and survival of primary GI melanomas in comparison with cutaneous melanomas using a population-based cohort. MethodsPatients diagnosed with primary GI and cutaneous melanomas were identified from Surveillance, Epidemiology, and End Results 1973-2016 data. ResultsA total of 872 primary GI melanomas and 319,327 cutaneous melanomas were identified. GI melanoma incidence increased by an annual percent change of 1.82 (P < 0.05) during the study period. The most common sites for GI melanoma were the anus (50%) and rectum (34%). Compared to cutaneous melanoma, patients diagnosed with GI melanomas were older, women (58% versus 45%), non-White (16% versus 6%), and presented with a higher stage (36% versus 4% distant stage, all P < 0.001). GI melanomas had significantly worse cancer-specific survival (CSS) than cutaneous melanoma. Despite the poor prognosis, the CSS has increased in recent years. Among patients with anorectal melanomas, local excision with chemotherapy and/or radiation had a similar CSS compared with those with major surgery only. ConclusionsDespite a steady increasing incidence since 1975, GI melanomas are rare, present with advanced stages, and have worse outcomes than cutaneous melanomas. The improved prognosis of these tumors in recent years might reflect the impact of novel targeted treatments and the more common use of local tumor excision over major resections.

Epidemiology and survival outcomes of primary gastrointestinal melanoma: a SEER-based population study.

Primary melanoma arising in the gastrointestinal (GI) tract is rare and poorly characterized. We sought to describe the epidemiology and survival outcomes of primary GI melanoma. GI melanoma cases were extracted from the Surveillance, Epidemiology, and End Results (SEER) database by tumor site and histology codes. We calculated age-adjusted incidence, and analyzed demographics, clinical characteristics, as well as overall survival (OS) and cancer-specific survival (CSS) of GI melanoma. A total of 1080 histologically confirmed cases of primary GI melanoma were identified, with a median age of 71years (IQR: 59-80). 49.9% of the cases originated from anus, 30.8% had distant disease at diagnosis, and 61.5% received cancer-directed surgery. The distribution of gender and age was varied in GI melanoma subtypes. The incidence of GI melanoma was 0.58 cases per million, and increased remarkably over age, especially in patients aged 60years or older. The median OS and CSS of the whole cohort was 14months (95% CI 12.7-15.3) and 22months (95% CI 18.8-25.2), respectively. Anal melanoma patients had prolonged survival, while those with gastric melanoma had the poorest OS (18 and 4months, respectively). Multivariate analysis showed that decreased survival was associated with age older than 80years, gastric and esophageal origin, advanced-stage disease, lymph node metastasis, and without surgery of primary site. Patients with primary GI melanoma trended topresent with advanced-stage disease. Overall, GI melanoma had a poor prognosis, but the outcome differed according to the primary sites.

A case report of primary gastric melanoma causing massive upper gastrointestinal bleeding in an adult

Background: Melanomas involving the gastrointestinal (GI) tract are usually metastatic from primary cutaneous sites. Primary GI melanoma is a rare entity and mostly presents with non-specific symptoms. The diagnosis in most of the cases is made retrospectively on the immunohistochemistry findings. Case Presentation: A 32-year-old male presented with massive upper GI bleeding necessitating a total gastrectomy, and to our surprise, on immunohistochemical staining, the diagnosis of primary gastric melanoma was confirmed. Conclusion: The possibilities of rare mesenchymal tumors of the stomach should be considered while treating such patients. A high index of suspicion in such atypical scenarios should warrant a positron emission tomography integrated with computed tomography to complete the staging workup. The natural course of the disease and its outcome is not known due to the paucity and rarity of this entity. Although the prognosis is dismal, it would help to prognosticate the patient and family before embarking onto a major surgical resection.

Primary versus Metastatic Gastrointestinal Melanoma: A Rare Case and Review of Current Literature.

Gastrointestinal (GI) melanomas are a rare diagnostic entity. Although there have been cases of melanomas solely in the GI tract, many debate their true origin: the gut versus a distant, undetected primary lesion that regressed known as melanoma of unknown primary. We present a case that involved diagnosing a GI melanoma and then backtracking to find a possible primary source. We review the most recent literature regarding possible etiologies of primary GI melanomas and how to differentiate whether it has a primary, metastatic, or unknown origin.

Anal Melanoma: A General Surgical Experience

Anal mucosal melanoma constitutes only 0.5 to 2% of all anorectal malignancies and less than 2% of all melanomas. Anal melanoma is the third most common melanoma after the skin and ocular varietiesand the most common site for primary gastrointestinal melanoma.

A primer melanoma malignum ritka gastrointestinalis lokalizációi

Primary gastrointestinal melanomas, part of the mucosal melanoma group, are uncommon. They constitute about five percent of all melanomas and most of them are located in the rectum (3 percent of all melanomas). The prognosis is poor, overall 5-year survival in rectal melanoma is 10-20 percent. We present three of our cases. The first case - a 68-year-old male patient - was operated on for histologically proved rectal melanoma. Three years after radical excision and oncological treatment a metastasis of the primary tumor was diagnosed in the stomach. Total gastrectomy was performed, followed by oncological treatment. In the second case of a 59-year-old male patient an appendectomy was performed for symptoms of appendicitis. The histopathological examination revealed melanoma of the appendix. Further investigations revealed the primary tumor in the stomach and metastases in the lungs as well. The third case - an 82-year-old female patient - was investigated for frequent defecations, mucus in stool and fecal incontinence. Primary melanoma was proved in the lower third of the rectum with multiple hepatic metastases. These three cases in our practice are remarkable for the rarity of the disease, and in two cases the presence of both the primary tumor and the metastasis were located in the gastrointestinal tract.

Primary Melanoma of the Rectum in an African-American Male

Purpose: Most melanomas of the gastrointestinal (GI) tract are metastases from cutaneous or ocular origin. There have been very few select reports of primary melanomas of the GI tract, and they have been known to have a poor prognosis. We report a case of a 56-year-old African-American male who presented with four kg of weight loss, four months of intermittent hematochezia, rectal discomfort, one month of epigastric pain, and one week of jaundice. Past medical history included a 40-pack-a-year smoking history. There was no personal or family history of cancer. His blood work showed hemoglobin of 12.5 gm/dl (13.5-17.5), MCV 95 (80-100), AST 236 IU/L (15-41),ALT 436 IU/L (17-63), total bilirubin 11.8 mg/dl (0.2-1.2), alkaline phosphatase 215 IU/L (32-91). Abdominal and pelvis computed tomography with contrast showed mild hepatomegaly with intrahepatic and extrahepatic ductal dilation, mildly prominent pancreatic head and uncinate process, non-specific rectal wall thickening up to 1.8 cm, and right adrenal thickening. Endoscopic retrograde cholangiopancreatogram (ERCP) showed multiple 5-20 mm bluish-black ulcers with thickened elevated borders in duodenum, normal ampulla, dilated 25-mm common bile duct (CBD) with 4-mm long apple core-shaped stricture at distal end and intrahepatic ducts dilation. Brush cytology specimen were taken and plastic stent was left in place. Colonoscopy showed a 6-cm long, elevated lesion with ulcerated necrotic center, involving 50% of the circumference, and occupying 80% of the lumen. The lesion was close, but not involving the dentate line. Biopsy of duodenal lesions and rectal mass was compatible with malignant melanoma, extensively involving mucosa and submucosa with tumor cells strongly positive for HMB45 and Melan-A, and weakly positive for S100 immunostain. BRAF and KIT gene mutations were negative. The brushing from the ERCP showed atypical cells compatible with same diagnosis. The biliary obstruction improved after plastic stent, and later, this was exchanged for a partially covered metallic stent with normalization of his total bilirubin. PET scan showed increased uptake in the posterior tongue, small bowel wall, retroperitoneal lymph nodes, adrenals, and rectal wall. ENT visualization of posterior tongue did not find a mass. Dermatology consult did not find any suspicious skin lesions for primary melanoma. Ophthalmology did not find any evidence of intraocular melanoma. The patient was enrolled in a trial with pazopanib and paclitaxel for unresectable melanoma. However, the patient died seven months after his diagnosis. Primary gastrointestinal melanomas are rare tumors with unknown risk factors, very aggressive, and with no proven established treatment.

Melanoma of Stomach

Melanoma of gastrointestinal tract can be either primary or secondary to metastases from other sites like skin, mucous membranes, and uvea. Primary gastrointestinal melanoma is rare and still rarer is stomach involvement. We report a case of primary malignant melanoma of stomach in a 50-year-old female who presented with an axillary lymphadenopathy. Melanoma of stomach is rare. It has characteristic endoscopic appearance. Differentiation of primary from secondary melanoma is possible on clinical behavior and histological characteristics.

Defining the Role of Surgery for Primary Gastrointestinal Tract Melanoma

ObjectiveThe objective of the study was to determine the outcomes for primary gastrointestinal melanomas (PGIM). Material and methodsThe Surveillance, Epidemiology, and End Results database (1973–2004) was queried. ResultsOverall, 659 cases of PGIM were identified. The annual incidence of PGIM was approximately 0.47 cases per million in 2000. Overall median survival time was 17 months. Tumors were identified in the oral–nasopharynx (32.8%), anal canal (31.4%), rectum (22.2%), esophagus (5.9%), stomach (2.7%), small bowel (2.3%), gallbladder (1.4%), and large bowel (0.9%). Univariate analysis demonstrated age, tumor location, stage, surgery, and lymph node status were significant predictors of improved survival. MST has not been reached for tumors located in the large bowel, while tumors located in the stomach demonstrated the shortest median survival (5 months). Improvement in MST was observed for those patients undergoing surgical resection. The presence of lymph node involvement conferred a poorer prognosis. Multivariate analysis of the cohort identified that location, advanced tumor stage, failure to undertake surgical resection, positive lymph node status, and age were all independent predictors of poorer outcome. ConclusionPGIM occurs most often in the oral–nasopharynx and anal canal. Surgical extirpation is the only identifiable treatment modality that significantly improves survival.