Melanoma of the small intestine

doi:10.1016/S1470-2045(09)70036-1

The Lancet Oncology

Volume 10, Issue 5, May 2009, Pages 516-521

https://doi.org/10.1016/S1470-2045(09)70036-1 Get rights and content

Summary

Intestinal melanomas can be primary tumours or metastases of cutaneous, ocular, or anal melanomas. Primary intestinal melanoma is extremely rare, whereas metastatic melanoma of the small bowel is common because of the tendency for cutaneous melanoma to metastasise to the gastrointestinal tract. Because distinguishing between primary and metastatic intestinal melanoma can be difficult, the main features of each are discussed, and the diagnostic images used to detect intestinal melanoma are assessed. Routine barium examinations and CT have limited sensitivity, but PET imaging can improve detection of melanoma metastases to the small bowel. Although various treatment strategies have been tried in patients with intestinal melanoma, surgical removal of intestinal metastases is the treatment of choice in patients with resectable tumours. No systemic therapy improves survival in patients with melanoma metastatic to the intestines; thus, the prognosis for these patients is poor. Patients with primary melanoma of the small intestine have a worse prognosis than do patients with metastases of cutaneous melanoma.

Introduction

Primary malignant tumours of the small intestine are rare; most gastrointestinal tumours are metastatic, and less than 2% originate in the small bowel.¹ Cutaneous melanoma is one of the most common types of tumour to metastasise to the gastrointestinal tract,² where melanoma accounts for 1–3% of all malignant disease.³ The small intestine is the most common site of melanoma metastases to the gastrointestinal tract.³ Most melanomas in the small intestine are metastases from primary cutaneous lesions (figure 1), but melanoma can also develop as a primary mucosal tumour in the gastrointestinal tract. Therefore, we need to be able to differentiate primary from metastatic melanoma of the small bowel.

Section snippets

Primary melanoma of the small intestine

Primary melanoma of the small intestine is an extremely rare neoplasm for which the cause is unknown. According to Mishima and colleagues,⁴ the disease might arise from schwannian neuroblast cells associated with the autonomic innervation of the gut. Amar and co-workers⁵ postulated an origin for intestinal primary melanoma in melanoblastic cells of the neural crest, which migrate to the distal ileum through the omphalomesenteric canal. The amine-precursor uptake and decarboxylation (APUD) cell

Metastatic intestinal melanoma

Melanoma of the small intestine can be metastatic in patients with a history of a cutaneous, anal, or ocular melanoma. Metastatic intestinal melanoma is very common, and among affected patients, the proportion with involvement of the small intestine ranges from 35% to 70%.2, 17, 21 Agrawal and co-workers²² noted small-bowel involvement in 91% of patients who had surgical exploration (laparotomy) for melanoma metastatic to the gastrointestinal tract.

Although around 60% of patients who die from

Clinical presentation

Clinical presentation of small-intestine melanoma is typically identical to that of other types of gastrointestinal tumours.⁷ The most common symptoms are abdominal pain, intestinal obstruction, constipation, haematemesis, melaena, anaemia, fatigue, weight loss, and presence of a palpable abdominal mass.¹⁴ Intestinal melanoma presented as single or multiple polypoid lesions can cause intestinal intussusception; bowel perforation is rare.²⁹ Substantial lymphadenopathy indicates spread to the

Diagnosis and diagnostic imaging

Clinical examination with endoscopic and radiological imaging is essential for diagnosis of small-bowel melanoma. Radiological studies have been used in the diagnosis of melanoma of the small intestine, although imaging of the small bowel is difficult because of its length and complex loops. Diagnosis of intestinal melanoma is typically made by abdominal ultrasonography, conventional barium contrast studies, endoscopy, CT, or PET (panel);³⁰ however, the rate of clinical detection is low (only

Treatment of intestinal melanoma

Surgery is the treatment of choice in patients with primary small-bowel melanoma and in those with intestinal metastatic melanoma.²² Wide surgical resection of the tumour with sufficient free margins proximally and distally from the lesion, together with a subtended wedge of mesentery to remove regional lymph nodes, is recommended.⁴⁵ Morbidity and mortality associated with this type of surgery are very low.28, 46

Surgical resection of melanoma metastases of the small bowel should be done in all

Conclusion

Melanoma typically develops where melanocytes are found (skin, eyes, meninges, and anal region) and can also develop as a primary tumour in the small intestine. Primary tumours of the small bowel are rare, however, and most cases of small-bowel melanoma are metastases from cutaneous melanoma. Metastases to the gastrointestinal tract are found at autopsy in 60% of patients who die with malignant melanoma. The clinical picture of small-bowel melanoma is similar to the clinical presentation of

Search strategy and selection criteria

Data for this Review were identified by searches of Medline (from January, 1966, to October, 2008) and Embase (from January, 1974, to October, 2008) by use of the search terms “melanoma”, “intestinal”, “small bowel”, and “metastases”. The reference lists of all relevant articles were checked for papers not found in the database searches. Abstracts and reports from relevant conference proceedings were hand searched. No language restriction was applied.

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Cited by (129)

Primary malignant melanoma of the small bowel: A case report
2024, Radiology Case Reports
Malignant melanoma comprises 1%-3% of all malignant tumors of the gastrointestinal tract. The small bowel melanoma is an extremely rare malignancy. Very few cases have been reported in the literature. The small intestine is mostly affected by the metastatic tumors of the primary lesions especially cutaneous. This malignancy is diagnosed at the late stages as the patient remains asymptomatic. In this report we present a case of malignant melanoma arising from the small bowel in a 58-year-old male. There was no primary lesion in the eye, skin, anus, rectum or elsewhere in the body. The patient was treated with surgery. Afterward the patient presented to the emergency room with respiratory distress for which he was on ventilator support, sadly the patient died after 10 days. Malignant melanoma is an aggressive tumor and does not respond well to chemotherapy or radiotherapy.
How the Radiologist Must Reason for a Correct Diagnosis in Patients With Small Bowel Mural Thickening Studied by CT or MRI: A Pictorial Review
2023, Current Problems in Diagnostic Radiology
Conditions that lead to small bowel mural thickening fall into a broad spectrum of diseases, including inflammatory, infectious, vascular or neoplastic. Computed tomography (CT) and Magnetic Resonance Imaging (MRI), especially CT-enterography and MR-enterography, permit evaluation of both entire small bowel and extraluminal structures. In CT/MR-enterography, the main prerequisite for the correct evaluation of small bowel is to obtain optimal intestinal distension. In fact, most errors are related to poor intestinal distension of the bowel which can lead to interpret as pathological a small bowel segment that is not very distended (false positive), or not to recognize presence of pathology in a collapsed segment (false negative). Once the examination has been performed, the images are analyzed in order to identify the presence of small bowel pathology. Pathology of the small bowel can manifest as endoluminal alteration and/or intestinal wall thickening. Once bowel wall thickening has been identified, the radiologist's first objective is trying to define benign or malignant nature of the alteration, using also patient's history and clinical features. Once the suspicion of benign or malignant pathology has been raised, the radiologist must try to formulate a diagnosis of nature. In this pictorial review we describe how the radiologist must reason for a correct diagnosis by answering a pattern of sequential questions in a patient with suspected small bowel disease studied by CT or MRI.
Case report of metastatic melanoma presenting as an unusual cause of gastrointestinal hemorrhage in an elderly gentleman
2022, Annals of Medicine and Surgery
Citation Excerpt :
This is understood to occur primarily through hematogenous spread, likely as a product of extensive vascular supply throughout the small intestines. More specifically, epidemiological data from multiple studies suggests small bowel involvement is observed in approximately 35%–70% of patients with known primary cutaneous metastatic melanoma [1,3]. Interestingly, despite such a strong preponderance for cutaneous melanoma to spread to the small bowel, less than 10% of metastatic lesions are detected pre-mortem [1].
and Importance: Melanomas are capable of metastasizing to both regional and distant sites and are notably known to metastasize to the skin, lungs, brain, liver, bone, and gastrointestinal tract. Metastatic melanoma is infrequently diagnosed in vivo, and usually found only on post-mortem evaluation at autopsy.
Here we present the case of a 64-year-old male who originally presented with melena, fatigue, exertional dyspnea and one episode of near-syncope. He was found to have a hemoglobin of 5.4 gm/dL on initial presentation with largely unremarkable abdominal examination. CTAP demonstrated an area of circumferential small bowel wall thickening, concerning for malignancy. The patient underwent an EGD that was noted for mild gastric fundal erosions, which failed to explain his presenting symptoms. VCE was later performed following discharge to visualize the small bowel, which revealed two bleeding lesions within the small bowel. This was complicated by the device becoming lodged on the more proximal mass, and he was admitted again for push-enteroscopy and device retrieval. At the time of this admission, he continued to be symptomatic and was profoundly anemic with a hemoglobin of 4.7 gm/dL.
EGD with push enteroscopy was performed, revealing two small masses in the mid-distal duodenum and jejunum, which were tattooed and biopsied. He underwent robotic-assisted laparoscopic small bowel resection of the affected portions of the small bowel, without complications. Surgical samples were consistent with melanoma, and further dermatologic examination revealed a suspicious lesion located on the patient's posterior right shoulder was biopsied and also consistent with melanoma, confirming the suspicion for metastatic process from primary cutaneous lesion.
We present this case as a rare diagnostic opportunity to observe metastatic melanoma of the small bowel, including a review of pertinent symptomatology and epidemiological data from previous literature. Our case serves as a reminder to consider metastatic melanoma as a rather uncommon cause of severe blood loss anemia, while also providing an overview of endoscopic modalities available for visualizing the small bowel in the management of suspected small bowel malignancy.
Presentation, Management, and Prognosis of Primary Gastrointestinal Melanoma: A Population-based Study
2021, Journal of Surgical Research
Primary 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.
Patients diagnosed with primary GI and cutaneous melanomas were identified from Surveillance, Epidemiology, and End Results 1973-2016 data.
A 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.
Despite 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.
Surgical treatment of melanoma metastases to the small bowel: A single cancer referral center real-life experience
2021, European Journal of Surgical Oncology
Treatment of metastatic melanoma has rapidly changed during the last years, and patients often require a multidisciplinary approach to achieve effective results. We aimed to assess the survival benefit achieved through surgical approach to patients with small bowel (SB) metastases from cutaneous melanoma, to emphasize the potential role of surgery in association with novel therapies.
Ninety consecutive patients with cutaneous melanoma diagnosed as having resectable SB metastases from 1995 to 2015 were retrospectively investigated.
Median age at surgery of melanoma metastases was 53.4 years. Among 30 patients who had a curative-intent resection, the 5- and 10-year survival rates were 61% and 54%, respectively, while among 60 patients treated with a palliative surgery the corresponding rates were both 4%. Among 29 patients, for whom the interval time between the occurrence of SB metastases and the previous surgical event on GI tract was ≥36 months, the 5-year overall survival rate was 42%; for 56 patients who had an interval time <36 months the corresponding survival rate was 14%. Within the whole series, an absence of any residual disease after surgery (R0) was a factor affecting better survival, regardless of the evidence of metastases in other organs.
Our observational data showed that surgical treatment for patients with SB metastases from melanoma might increase survival, but further studies are needed to confirm this finding. In the age of novel available therapies, the increase in survival time given by surgery may offer important chances for patients to benefit from systemic therapies.
Duodenal malignant melanoma: Primary and metastatic case series and literature review
2024, Medicine (United States)

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ReviewMelanoma of the small intestine

Summary

Introduction

Section snippets

Primary melanoma of the small intestine

Metastatic intestinal melanoma

Clinical presentation

Diagnosis and diagnostic imaging

Treatment of intestinal melanoma

Conclusion

Search strategy and selection criteria

Am J Gastroenterol

J Invest Dermatol

J Am Acad Dermatol

Pathology

Mayo Clin Proc

Am J Surg

Am J Surg

Am J Gastroenterol

Eur J Surg Oncol

Gastrointest Endosc Clin N Am

Small intestinal neoplasms

J Clin Gastroenterol

Secondary tumors of the gastrointestinal tract: surgical pathologic findings and comparison with autopsy survey

Mod Pathol

Melanocytic and nevocytic malignant melanomas. Cellular and subcellular differentiation

Cancer

Primary malignant melanoma of the small intestine

Gastroenterol Clin Biol

Primary malignant melanoma of the small intestine and the APUD cell concept

J Surg Oncol

Primary and metastatic diseases in malignant melanoma of the gastrointestinal tract

Curr Opin Oncol

Gastrointestinal malignant melanoma

Int J Dermatol

Malignant gastrointestinal melanomas of unknown origin: should it be considered primary?

World J Gastroenterol

Malignant melanomas in the small intestine: a study of 103 patients

Am J Gastroeneterol

Spontaneous regression of malignant melanoma

Colonic melanoma, primary or regressed primary

J Clin Gastroenterol

Melanoma metastatic to the gallbladder and small bowel: report of a case and review of the literature

Melanoma Res

Jejuno–jejunal invagination due to intestinal melanoma

World J Gastroenterol

Mucosal melanomas in cutaneous melanoma

Review
Melanoma of the small intestine