Patient With Duodenal Strictures and a Mass at the Head of the Pancreas.
A 55-year-old woman had an 8-year history of duodenal ulcers. Two weeks prior to admission, she started having abdominal pain, including vomiting small amounts of blood. She denied fever, chills, or a change in bowel habits. Past medical history was significant for chronic obstructive pulmonary disease chronic obstructive pulmonary disease
n. Abbr. COPD
A chronic lung disease, such as asthma or emphysema, in which breathing becomes slowed or forced. . Past surgical history included a hysterectomy 13 years earlier. Current medications included omeprazole (Prilosec) and estriol estriol /es·tri·ol/ (es´tre-ol) a relatively weak human estrogen (q.v.), being a metabolic product of estradiol and estrone found in high concentrations in urine, especially during pregnancy. . The patient was admitted to the hospital and an esophagogastroduodenoscopy revealed severe gastritis and several duodenal strictures. A computed tomographic scan of the abdomen revealed a mass involving the duodenum and the head of the pancreas (Figure 1). A barium examination of the upper gastrointestinal tract showed the stricture as a filling defect. This defect was demonstrated on the radiograph, with very little barium passing into the third part of the duodenum (Figure 2). Laboratory studies showed a normal complete blood count and normal total bilirubin level at 0.4 mg/mL, but the patient's amylase level was elevated at 199 MU/mL, and her lipase was elevated at 64 U/L.
On hospital day 13, the patient underwent a radical pancreaticoduodenectomy (Whipple procedure) for the duodenal stricture. The specimen received in pathology consisted of a 7.2-cm segment of duodenum with attached pancreas, measuring 6.5 x 5.8 x 3.2 cm. The duodenum was opened to reveal a dilated ampulla of Vater Ampulla of Vater
The widened portion of the duct through which the bile and pancreatic juices enter the intestine. Ampulla is a Latin word for a bottle with a narrow neck that opens into a wide body.
Mentioned in: Jaundice , measuring 1.1 cm in diameter. The mucosa adjacent to the ampulla ampulla /am·pul·la/ (am-pul´ah) pl. ampul´lae [L.] a flask-like dilatation of a tubular structure, especially of the expanded ends of the semicircular canals of the ear. was friable, tan to pink, and focally hemorrhagic. The portion of pancreas had a dilated pancreatic duct but no discrete masses. Microscopic examination of the friable tissue surrounding the ampulla showed a nodular proliferation of benign ductular elements lined by low cuboidal cuboidal /cu·boi·dal/ (ku-boi´d'l) resembling a cube.
adj See cuboid. to columnar epithelium interspersed with hypertrophic smooth muscle fibers (Figure 3). The nuclei were basally oriented and no mitosis was noted (Figure 4).
What is your diagnosis?
Pathologic Diagnosis: Adenomyoma of the Ampulla and Distal Bile Duct
An adenomyoma is a benign lesion and a rare clinical finding. A "pseudotumor" of the extrahepatic biliary tract, the nodular lesion is characterized by tubular structures that are lined by columnar or cuboidal cells. The nuclei are basally located and monotonous, with no atypia or pleomorphism pleomorphism /pleo·mor·phism/ (-mor´fizm) the occurrence of various distinct forms by a single organism or within a species.pleomor´phicpleomor´phous
1. . Usually, no mitoses are seen. The connective tissue of the stroma stroma /stro·ma/ (stro´mah) pl. stro´mata [Gr.] the matrix or supporting tissue of an organ.stro´malstromat´ic
n. pl. stro·ma·ta
1. contains whorls of interlaced smooth muscle bundles.
Approximately 90% of adenomyomas are located in the stomach, duodenum, and jejunum jejunum: see intestine. . Occasionally, they are found in other sites of the gastrointestinal tract, the thorax, the gallbladder, and in the biliary tree. The first case in the hepatic bile duct was reported by Dowdy et al in 1962. Since that time, 9 additional cases in the common and hepatic bile ducts have been reported. Of these 10 cases, 7 were found in the common bile duct common bile duct
The duct that is formed by the union of the hepatic and cystic ducts and discharges into the duodenum. Also called gall duct. .
Adenomyomas have been described by other synonyms, largely depending on the anatomic site. The microscopic appearance is similar to a gallbladder lesion known as adenomyomatous hyperplasia and to an intestinal lesion known as adenoleiomyomatous hyperplasia. Digestive tract adenomyomas are often confused with ectopic ectopic /ec·top·ic/ (ek-top´ik)
1. pertaining to ectopia.
2. located away from normal position.
3. arising from an abnormal site or tissue.
adj. pancreatic tissue, myoepithelial hamartoma, fibroadenoma Fibroadenoma Definition
Fibroadenomas are benign breast tumors commonly found in young women. Fibroadenoma means "a tumor composed of glandular (related to gland) and fibrous (containing fibers) tissues. , and Brunner gland hyperplasia. Most adenomyomas do not contain pancreatic tissue, so they should not be confused with pancreatic heterotopia. Myoepithelial hamartoma is a misnomer, as hamartoma means an excessive overgrowth of mature normal indigenous cells within a tissue. A fibroadenoma does not describe the prominent smooth muscle component of the adenomyoma. Brunner gland hyperplasia usually denotes abundant Brunner glands with atrophic villi villi: see digestive system. and crypts in the adjacent mucosa. Therefore, the term adenomyoma should be reserved for a distinct nodular lesion with a particular histopathologic pattern.
The symptoms of the adenomyoma depend on the location and the size of the neoplasm neoplasm or tumor, tissue composed of cells that grow in an abnormal way. Normal tissue is growth-limited, i.e., cell reproduction is equal to cell death. . Pain and dyspepsia, as in this case, is a common presenting complaint. The pain, usually colicky in the right upper quadrant right upper quadrant Physical exam The abdominal region that contains the liver, duodenum and head of pancreas , sometimes radiates to the back. Other symptoms include weight loss, fever, dysphagia, malaise, and anemia. Occasionally, the lesions are an incidental finding at surgery or during an autopsy.
The surgical management of an adenomyoma depends on its location. If the lesion is growing in the proximal part of the hepatic duct, its removal may be difficult or impossible without major hepatic resection. Distal common bile duct lesions can be removed by a radical pancreaticoduodenectomy (Whipple procedure), as in this case. The Whipple procedure is not recommended in benign distal or intrahepatic bile duct lesions owing to its higher morbidity and mortality, while not conferring any advantage over wide local excision.
In summary, adenomyoma should be included in the differential diagnosis of a mass at the head of the pancreas. The term adenomyoma should be reserved for a distinctive nodular lesion with the characteristic cuboidal or low columnar glands, interspersed in bundles of smooth muscle fibers. A Whipple procedure may be necessary to remove an adenomyoma of the common bile duct.
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Accepted for publication September 28, 2000.
From the Department of Pathology, Emory University, Atlanta, Ga (Dr Chand), and the Medical College of Georgia In 1828, it was chartered by the state of Georgia as the Medical Academy of Georgia, with plans to offer a single course of lectures leading to a bachelor's degree. It opened the following year on October 1st at the Augusta hospital. , Augusta (Dr Caudell).
Reprints not available from the author.