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Cholangiocarcinoma

Cholangiocarcinoma is an adenocarcinoma of the biliary duct system. It is usually associated with environmental exposures such as polyvinyl chloride or Thorotrast (thorium dioxide). It is also associated with the parasite opisthorchis viverrini.

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  • Cholangiocarcinoma Support Group Forums
  • cholangiocarcinoma.org :: a resource for patients, friends, caregivers and loved ones.

Other related items

  • Bilirubin
  • Liver function tests
  • Primary sclerosing cholangitis
  • Klatskin tumor
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Cholangiocarcinoma metastatic to the neck: first report of a case
From Ear, Nose & Throat Journal, 11/1/02 by Scott H. Hardeman

Abstract

We describe a unique case of a cholangiocarcinoma that metastasized to a cervical lymph node--to our knowledge the only such case ever reported. The diagnosis was based on fine-needle aspiration cytology and confirmed by excision biopsy. This case illustrates the importance of keeping all possible options in mind when diagnosing head and neck masses.

Introduction

Extrahepatic bile duct carcinoma is a relatively rare disease. Nearly all bile duct tumors are malignant. Cholangiocarcinomas account for only 30% of these malignancies. Histologically, most of these tumors are adenocarcinomas. (1) Cholangiocarcinomas are more common in men than in women, and they typically occur in the fifth to sixth decades of life. (2) Their common clinical features are obstructive jaundice, cholangitis, weight loss, and right upper quadrant pain. (2) Most patients have locally advanced disease and regional lymphatic metastasis. Distant metastases to the skin, breast, and bone have also been reported. (2) In rare but clearly documented cases, some metastatic lesions were cutaneously implanted following percutaneous transhepatic biliary drainage. (3) In this article, we describe a unique case of cholangiocarcinoma metastasis to a cervical lymph node.

Case report

A 50-year-old man came to our otolaryngology office for evaluation of a left neck mass. He had been treated by a gastroenterologist for several months for a diagnosis of cholangitis. When he reported that his digestive symptoms had worsened, the gastroenterologist obtained abdominal computed tomography (CT), which detected the presence of a biliary tract mass (figure 1). Follow-up positron-emission tomography (PET) demonstrated two hypermetabolic areas--one in the right upper quadrant and one in left neck zone 4 (figure 2).

Findings on our head and neck examination, which included flexible fiberoptic nasopharyngoscopy, were normal with the exception of a 1 x 2-cm lymph node in left neck zone 4. Because we believed that this finding might represent a metastatic neck mass, we performed fine-needle aspiration of the node. Analysis of the aspiration smears revealed the presence of malignant cells (figure 3). We then performed an excisional biopsy of the node, and histologic examination of the specimen confirmed the malignancy (figure 4).

The patient elected to undergo chemotherapy and irradiation to the abdomen and neck. This treatment was administered at another institution, and the patient was subsequently lost to follow-up.

Discussion

Our patient's needle aspiration specimen contained an abundance of loosely clustered malignant cells. Cell groups were arranged in syncytial and three-dimensional patterns and featured abortive gland-like formations. Individual cells were characterized by pleomorphic nuclei with vesicular chromatin and a few prominent nucleoli, which constitute the cytomorphologic features of adenocarcinoma.

Our diagnosis of metastatic disease was rendered in the otolaryngology office and based on the results of the aspiration biopsy of the neck lesion. Because such a diagnosis is so important in the staging of a neoplasm, we excised the lesion and analyzed it histologically, which confirmed the presence of metastatic adenocarcinoma. The morphologic features seen on both the fine-needle aspiration and the surgical excision biopsies were compatible with those of a primary cholangiocarcinoma.

To our knowledge, this is the only documented case reported in the literature of a cholangiocarcinoma metastatic to a cervical lymph node. This case represents a rare but important histologic diagnosis that head and neck surgeons should add to our catalog of uncommon lesions that can metastasize to the head and neck.

References

(1.) Schwartz SI, ed. Principles of Surgery. New York: McGraw-Hill, 1994:1389-91.

(2.) Yiannakis PH, Patrikeos A, Ford HT, Davis CL. Metastatic cholangiocarcinoma with an occult primary. Clin Oncol (R Coll Radiol) 1995;7:394.

(3.) Loew R, Dueber C, Schwarting A, Thelen M. Subcutaneous implantation metastasis of a cholangiocarcinoma of the bile duct after percutaneous transhepatic biliary drainage. Eur Radiol 1997; 7:259-61.

RELATED ARTICLE: Editorial Comment

The lesson learned from this unique case

Since this might be the only case of neck metastasis from a carcinoma of this specific type ever encountered--one so rare that an ENT surgeon can expect to never see one--one might well ask, Of what value is this report to the average otolaryngologist? Actually, the report does have some value because it reminds us to sometimes consider rare possibilities because there are some "zebras" around.

When faced with a patient with an adenocarcinoma, pathologists are not always very good at predicting the most likely site of origin based on histologic examination alone. If such a tumor should show up in the head or neck area (particularly in an extranodal site), both the pathologist and the clinician might jump to the conclusion that it is a primary tumor, depending on the clinical and histologic parameters. If major or even radical therapy is directed at a presumed primary tumor and later it is discovered that the lesion was metastatic from some distal primary origin, a lawyer might easily persuade a jury that something was seriously wrong with the way the patient was managed.

Therefore, it is important that both clinicians and pathologists keep this type of problem in the back of their minds. It is prudent to maintain a high index of suspicion for a possible metastasis from an unusual site if the discovered adenocarcinoma seems to be in some way a bit peculiar, either clinically or pathologically. This report serves to remind us of this possibility.

Dennis K. Heffner, MD

Chairman, Department of Otolaryngologic and Endocrine Pathology

Armed Forces Institute of Pathology Washington, D.C.

From the Department of Otolaryngology--Head and Neck Surgery (Dr. Hardeman and Dr. Stack), the Department of Pathology (Dr. Collins), the Division of Nuclear Medicine (Dr. Lowe), and the Division of Transplant Surgery (Dr. Solomon), St. Louis (Mo.) University School of Medicine.

Reprint requests: Brendan C. Stack, Jr., MD, Division of Otolaryngology--Head and Neck Surgery, Milton S. Hershey Medical Center, Pennsylvania State University College of Medicine, PO Box 850, Hershey, PA 17033. Phone: (717) 531-8945; fax: (717) 531-6160; e-mail: bstack@psu.edu

COPYRIGHT 2002 Medquest Communications, LLC
COPYRIGHT 2002 Gale Group

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