Author Affiliations: Department of Pathology, Forensic Medicine and Cytology (Bendic A, Glavina Durdov M and Karaman I) and Department of Abdominal Surgery (Stipic R), Clinical Hospital Center Split, University School of Medicine, Spinciceva 1, Split 21000, Croatia

 

Corresponding Author: Antonia Bendic, MD, Department of Pathology, Forensic medicine and Cytology, Clinical Hospital Center Split, University School of Medicine, Spinciceva 1, Split 21000, Croatia (Tel: 385-21-556- 486; Fax: 385-21-389-510; Email: antonia.bendic@gmail.com)

 

© 2013, Hepatobiliary Pancreat Dis Int. All rights reserved.

doi: 10.1016/S1499-3872(13)60016-8

 

Contributors: BA proposed the study. BA, GDM, SR and KI collected samples, analyzed the slides and wrote the first draft. All authors contributed to the design and interpretation of the study and to further drafts. BA is the guarantor.

Funding: None.

Ethical approval: Not needed.

Competing interest: No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.

 

 

BACKGROUND:The most common tumors in the ampulla of Vater are adenocarcinomas. Although malignant melanoma usually occurs on the skin, it can also arise in the gastrointestinal and biliary tract.

 

METHOD: We present a case of a 52-year-old, previously healthy man who presented with painless jaundice.

 

RESULTS: Clinical examination revealed dilated intrahepatic and extrahepatic bile ducts and pancreatic duct due to the obstructive mass in the ampulla of Vater. The patient underwent pancreaticoduodenectomy and histopathological diagnosis of the resected tumor mass was malignant melanoma. Thorough clinical examination was preformed, but no other primary or metastatic site of melanoma could be found. In the next few months, the patient developed multiple liver metastases and died.

 

CONCLUSION:An isolated amelanotic lesion in the ampulla of Vater can be a potential diagnostic pitfall, especially in patients who have had melanoma.

 

(Hepatobiliary Pancreat Dis Int 2013;12:106-108)

 

KEY WORDS: malignant melanoma; ampulla of Vater; biliary tract

Malignant melanoma is a highly aggressive neoplasm which usually occurs on the skin. It has the ability of unpredictable dissemination, mainly to the gastrointestinal (GI) and biliary tract. Visceral metastases are usually multiple, polypoid or ulcerated lesions, pigmented or amelanotic, rarely solitary tumors. Primary malignant melanomas can also occur on the GI and biliary tract mucosa. They are more aggressive, and have a much worse prognosis compared with cutaneous melanoma.^[1] We present a case of a 52-year-old man with an isolated obstructive mass occupying the ampulla of Vater, which was histologically identified as malignant melanoma. We discuss the criteria for diagnosis of primary malignant melanoma in the ampulla of Vater in the context of the published cases with review of the literature.

 

 

A 52-year-old, previously healthy man presented with a two-day history of weakness and painless jaundice. Laboratory evaluation showed elevated levels of bilirubin: total bilirubin 300 µmol/L, direct fraction 219 µmol/L, and indirect fraction 81 µmol/L and liver enzymes: alkaline phosphatase 1106 U/L, γ-glutamyltransferase 1576 U/L, aspartate aminotransferase 281 U/L, alanine aminotransferase 489 U/L, and lactate dehydrogenase 239 U/L.

 

Abdominal ultrasound and multislide computerized tomography revealed a 1.9×1.5 cm hypodense, obstructive mass in the ampulla of Vater. Three regional lymph nodes were enlarged, up to a maximum diameter of 1.5 cm. Intrahepatic and extrahepatic bile ducts as well as the common bile duct and pancreatic duct were dilated up to 1.9 cm in diameter, without intraluminal masses. Other abdominal organs showed no abnormalities. Endoscopic examination revealed a round, well limited, whitish mass occupying the ampulla of Vater. Percutaneous transhepatic cholangiopancreatography was done, and after drainage bilirubin levels were decreased significantly and symptoms were relieved. Pancreaticoduodenectomy by the Whipple procedure was done. A round, firm, white nodule, 1.4 cm in diameter, completely surrounded and narrowed the lumen of the ampulla of Vater. It was restricted by duodenal mucosa and pancreatic tissue (Fig. 1). Tumor tissue was composed of massive malignant cells with hyperchromatic nuclei and scant pale cytoplasm but without pigment (Fig. 2). Pancreatic tissue did not show signs of malignant infiltration. In one of the three regional enlarged lymph nodes, scattered malignant cells identical to those in the ampulla of Vater were found. The duodenal mucosa and stomach had nothing abnormal. Immunohistochemically, tumor cells were vimentin, S-100, melanoma antigen (Fig. 3) positive, but pancytokeratin (Fig. 4), cytokeratin 7, cytokeratin 8, cytokeratin 20, leukocyte common antigen, chromogranin, sinaptophizin and neurofilament negative. The proliferation activity measured by the expression of Ki-67 was high (>90%). Same immunohistochemical pattern was found in the infiltrated lymph node. Pathological examination of the resected tumor mass revealed a malignant melanoma. Complete clinical overview was done, but other primary or metastatic sites could not be found. Two months after the diagnosis the patient developed multiple liver metastases and died 4 months later.

 

 

Primary GI and biliary melanoma is rare. Distinguishing between primary mucosal and metastatic GI and biliary melanomas from an unknown or regressed primary site can be difficult. Clinically, a diagnosis of primary melanoma is likely in a patient with no obvious primary (or history) of melanoma, and an isolated GI or biliary lesion. Histologically, the diagnosis of primary mucosal melanoma is supported if there is a precursor lesion, such as junctional melanocytic proliferation or melanosis.^[1] We could not assess precursor lesions because the overlying mucosa was almost entirely ulcerated. In the surrounding duodenal mucosa there was no melanosis or junctional activity. In the literature, only a small number of primary biliary tract melanomas and ampullary melanomas are reported. Although in practice the term ampulla refers to the common bile duct (CBD), in the literature cases of primary biliary tract melanomas are separated from ampullary melanomas, probably because of the anatomical specificity of ampullary region. There are few cases of primary CBD melanomas, with histologically confirmed melanosis and junctional activity but all of them are located above the ampulla.^[2-4] Likewise, reported metastatic melanomas of the CBD in most cases are located above the ampulla, with either involvement of the gallbladder or extension into the ampulla.^[5-7] Most of the cases reported as ampullary melanomas are metastatic from cutaneous or vaginal primary melanoma.^[8-13] Almost all patients presented with painless jaundice and had proven primary cutaneous or vaginal melanoma at the time of diagnosis of GI metastasis or some time earlier (Table). Our patient did not have a history of melanoma, unlike the other had an isolated amelanotic lesion. All efforts to find a primary melanoma failed. Similar to ours one case was described with an isolated primary lesion in the ampulla of Vater.^[14] However, the overlying mucosa in this case was not ulcerated so it was easy to determine melanosis or junctional activity and the tumor was grossly pigmented unlike ours. In case of isolated, especially amelanotic lesion in the ampulla of Vater, melanoma is rarely considered at first in differential diagnosis. Adenocarcinomas are the first to be excluded. Also, endocrine tumors like carcinoids and duodenal gangliocytic paraganglioma, followed by a variety of benign or malignant soft tissue tumors (leiomyoma, GI stromal tumor, rhabdomyosarcoma) should be considered in addition to the most common metastatic ampullary tumor and renal cell carcinoma.

 

In conclusion, in patients with GI or biliary symptoms and a history of melanoma, it is necessary to bear in mind the possibility of metastasis. But since the place of origin for malignant melanoma may be the GI and biliary tract, all other potential primary sites should be excluded.

 

 

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