Hepatic cyst arising from the left hepatic duct. (Pathologic Quiz Case).
A 32-year-old woman presented with epigastric epigastric adjective Referring to the body region between the costal margins and the subcostal plane pain and fullness in February 1998. Her past medical history was unremarkable. Physical examination revealed slight jaundice and an abdominal mass palpable in the epigastrium epigastrium /epi·gas·tri·um/ (ep?i-gas´tre-um) the upper and middle region of the abdomen, located within the sternal angle.epigas´tric
n. pl. . There was no evidence of ascites. Laboratory tests demonstrated elevated transaminases (alanine aminotransferase twice the normal level) and [gamma]-glutamyl transpeptidase (8 times the normal level). Hydatid hydatid /hy·da·tid/ (hi´dah-tid)
1. hydatid cyst.
2. any cystlike structure.
hydatid of Morgagni
1. serology was negative.
Abdominal ultrasound studies showed a lobulated lobulated /lob·u·lat·ed/ (lob´ul-at-id) made up of lobules.
made up of lobules. cystic mass in the left lobe of the liver and a filling defect in the left hepatic duct left hepatic duct
The duct that drains bile from the left half of the liver. , extending 4 cm along 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. . Computed tomographic scan demonstrated a large cystic mass involving the left lobe of the liver, measuring 14 x 9 cm; the tumor was homogeneous and had a few septa septa /sep·ta/ (sep´tah) [L.] plural of septum.
Septum (plural, septa)
The dividing partition in the nose that separates the two nostrils. It is composed of bone and cartilage. (Figure 1). There was compression of the porta hepatis with dilatation of the intrahepatic bile ducts.
[FIGURE 1 OMITTED]
Surgical treatment was undertaken and consisted of a left hepatic lobectomy lobectomy /lo·bec·to·my/ (lo-bek´tah-me) excision of a lobe, as of the lung, brain, or liver.
Excision of a lobe of an organ or a gland. with resection of the common hepatic duct common hepatic duct
The part of the biliary duct system that is formed by the confluence of the right and left hepatic ducts and is joined by the cystic duct to become the common bile duct. and common bile duct, followed by anastomosis to the right hepatic duct right hepatic duct
The duct that conveys bile to the common hepatic duct from the right half of the liver. . Cholecystectomy was also performed. The filling defect in the extrahepatic ducts was found to be a polypoid tumor measuring 5.5 cm with a gelatinous gelatinous /ge·lat·i·nous/ (je-lat´i-nus) like jelly or softened gelatin.
1. Of, relating to, or containing gelatin.
2. Resembling gelatin; viscous. appearance.
On gross examination, the left hepatectomy hep·a·tec·to·my
Excision of liver tissue.
surgical excision of liver tissue.
hepatectomy Surgery Segmental resection of the liver Indications Cancer, parasites, major trauma–eg, MVAs specimen included a globular, 15-cm, cystic tumor arising from the left hepatic duct. The cut surface demonstrated multiple loculi This article is about the commune in Sardinia. For the architectural term for burial niches, see Loculus. For Loculi, see Loculi (disambiguation).
Loculi is a comune ranging in size from 2 to 10 cm, a smooth white internal lining, and mucinous mucinous /mu·ci·nous/ (mu´si-nus) resembling, or marked by formation of, mucin.
relating to, resembling or containing mucin. content (Figure 2).
[FIGURE 2 OMITTED]
Microscopically, the cysts were lined by a single layer of cuboidal cuboidal /cu·boi·dal/ (ku-boi´d'l) resembling a cube.
adj See cuboid. or flattened mucinous epithelial cells with round regular nuclei that were basally located (Figure 3). There were occasional small papillary papillary /pap·il·lary/ (pap´i-lar?e) pertaining to or resembling a papilla, or nipple.
adj similar to a small, nipple-shaped elevation or projection. structures and cystic invaginations, but there was no evidence of malignancy or dysplasia. The underlying stroma was cellular and was composed of compact spindle-shaped cells with uniform ovoid nuclei and small blood vessels (Figure 4). Focal collections of foamy macrophages and cholesterol clefts associated with foreign-body giant cells were present beneath areas denuded of lining cells. The separate pedunculated lesion resected from the extrahepatic ducts had an identical microscopic appearance.
[FIGURE 3 & 4 OMITTED]
The postoperative recovery was uneventful, and after 2 years of follow-up the patient was well without any clinical or computed tomographic scan evidence of recurrence.
What is your diagnosis?
Pathologic Diagnosis: Hepatobiliary Cystadenoma With Mesenchymal Stroma
Hepatobiliary cystadenoma is a rare intrahepatic neoplasm representing approximately 5% of intrahepatic cystic lesions. (1) It was defined by Edmondson in 1958 as a multilocular multilocular /mul·ti·loc·u·lar/ (-lok´u-ler) having many cells or compartments.
Having or consisting of many small compartments or cavities. lesion lined by columnar epithelium with an accompanying densely cellular "ovarian-like" stroma. (2) In recent years, reports of this tumor have appeared more frequently in the radiological and surgical literature, largely due to wider use of and improved diagnostic imaging techniques, (3) but cases arising in the extrahepatic biliary tree remain rare. (4)
This tumor occurs more frequently in middle-aged women. It may be associated with preexisting lesions, such as polycystic disease of the liver, and abnormalities in hepatobiliary anatomy. (4) The most frequent symptoms and signs are pain, nausea, fullness, and palpable mass, and less frequently jaundice, rupture, or infection are present.(5)
In most instances, hematologic and biochemical tests are normal, but elevated levels of alkaline phosphatase, [gamma]-glutamyl transpeptidase, and bilirubin can be found when intrahepatic or extrahepatic biliary duct compression is present. (3) Elevated serum CA 19-9 levels have been demonstrated in a few cases. (6) Ultrasonography, computed tomographic scan, and magnetic resonance imaging magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures. are the most discriminating noninvasive diagnostic techniques, but a definite diagnosis is rarely made with imaging alone. Frequent complications include intracystic hemorrhage, bacterial infection, or rupture, which may complicate the diagnosis. (3)
Grossly, hepatobiliary cystadenoma is usually a multiloculated tumor, which may present as a cyst within the liver, as a pedunculated lesion arising from its surface, or as a polyp within a large bile duct. (5) The size ranges from 15 to 25 cm for all types noted, and it has a globular shape and a smooth external surface. The cut surface shows locules of various size containing yellow, brown, or hemorrhagic mucinous fluid. (5, 7)
Microscopically, the cysts are lined by a single layer of cuboidal, columnar, or flattened mucinous epithelial cells. The nuclei are basally located and regular. Occasional small papillary structures or cystic invaginations may be seen. Focal intestinal and Paneth cell metaplasia have been described, as well as oncocytic differentiation. (4)
A basement membrane separates the epithelium from the underlying ovarian-like stroma. This stroma is composed of spindle cells with round to elongated nuclei and a small amount of pink cytoplasm. (7) The septa between cysts often show secondary changes, such as foamy or pigmented macrophages, cholesterol clefts, multinucleated multinucleated
characterized by having more than one nucleus per cell.
multinucleated giant cell
see giant cell. giant cells, fibrosis, scarring, and calcification. (4, 5, 7)
On immunohistochemistry, the epithelial component stains positive for cytokeratin, epithelial membrane antigen, and carcinoembryonic antigen. Scattered lysozyme positivity with apical accentuation may be seen, as well as chromogranin, serotonin, gastrin, and somatostatin positivity in the endocrine component. (4, 8) Stromal cells are strongly immunoreactive immunoreactive
exhibiting immunoreactivity. for vimentin and smooth muscle actin; they show focal positivity with desmin, lysozyme, type IV collagen, progesterone, and estrogen receptors. (4)
The histogenesis histogenesis /his·to·gen·e·sis/ (-jen´e-sis) the formation or development of tissues from the undifferentiated cells of the germ layers of the embryo.histogenet´ic
n. of this tumor is still unknown. The positivity of epithelial lining for cytokeratin and carcinoembryonic antigen (4) and the appearance of the stroma, similar to mesenchymal tissue in the fetal gallbladder, support the hypothesis that remnants of primitive hepatobiliary cells constitute the progenitor of the hepatic cystadenoma. (3, 9) Meanwhile, the positivity of the stroma for hormone receptors and its apparent restriction to tumors arising in female patients suggest a sex-specific (perhaps hormonal) factor. (1, 4)
The only curative therapy is complete surgical resection. (5, 10) Partial excision and drainage procedures have usually been unsuccessful. (7, 10) Undetected synchronous malignancy corresponding to biliary cystadenocarcinoma occurs in 2% to 4% of cases, (10) Benign and malignant areas may coexist. Foci of cellular atypia, multilayering, or solid epithelial masses indicate borderline changes; capsular invasion signifies malignancy. (4, 7)
In summary, hepatobiliary cystadenoma with mesenchymal stroma is a rare multilocular tumor that arises in women and has potential for malignant transformation. Benign and malignant areas may coexist, emphasizing the need for thorough pathologic sampling. The only curative therapy is total excision, even if the lesion presents as a benign tumor.
The authors thank S. Caplin, FRCS, for reviewing the English text.
(1.) Pedram-Canihac M, Le Bail B, Rivel J, Blanc JF, Saric J, Bioulac-Sage P. Cystadenome hepatobiliaire a stroma mesenchymateux: une tumeur hormonodependante? A propos de cinq cas avec etude immunohistochimique des recepteurs hormonaux. Ann Pathol. 2000;20:14-18.
(2.) Edmondson HA. Tumors of the Liver and Intrahepatic Bile Ducts. Washington, DC: Armed Forces Institute of Pathology Armed Forces Institute of Pathology A section of the US military which provides consultations, reference atlases and educational programs for pathologists ; 1958: 24-28. Atlas of Tumor Pathology; 2nd series, fascicle fascicle /fas·ci·cle/ (fas´i-k'l)
1. a small bundle or cluster, especially of nerve, tendon, or muscle fibers.
2. a tract, bundle, or group of nerve fibers that are more or less associated functionally. 26.
(3.) Gadzijev EM, Pleskovic A, Stanisavljevic D, Ferlan-Marolt V, Trotovsek B. Hepatobiliary cystadenoma can protrude and grow into the bile ducts. Hepatogastroenterology. 1998;45:1446-1451.
(4.) Daveny K, Goodman ZD, Ishak KG. Hepatobiliary cystadenoma and cystadenocarcinoma: a light microscopic and immunohistochemical study of 70 patients. Am J Surg Pathol. 1994;18:1078-1091.
(5.) Paradinas FJ. Liver tumors and tumor-like conditions. In: Wight DG, ed. Liver, Biliary Tract and Exocrine Pancreas. New York, NY: Churchill Livingstone; 1994:469-541. Systemic Pathology; 3rd ed.
(6.) Thomas JA, Scriven MW, Puntis MCA, Jasani B, Williams GT. Elevated serum CA 19-9 levels in hepatobiliary cystadenoma with mesenchymal stroma: two case reports with immunohistochemical confirmation. Cancer. 1992;70:1841-1846.
(7.) Wheeler DA, Edmondson HA. Cystadenoma with mesenchymal stroma (CMS) in the liver and bile ducts: a clinicopathological study of 17 cases, 4 with malignant change. Cancer. 1985;56:1434-1445.
(8.) Gourley WK, Kumar D, Bouton MS, Fish JC, Nealon W. Cystadenoma and cystadenocarcinoma with mesenchymal stroma of the liver: immunohistochemical analysis. Arch Pathol Lab Med. 1992;116:1047-1050.
(9.) Subramony C, Herrera GA, Turbat-Herrera EA. Hepatobiliary cystadenoma: a study of five cases with reference to histogenesis. Arch Pathol Lab Med. 1993; 117:1036-1042.
(10.) Knott AW, Ampudia RJ, Evankovich CH, et al. Biliary cystadenoma: rare variant of intrahepatic cystic disease. South Med J. 2000;93:698-702.
Accepted for publication March 15, 2001.
From the Departments of Pathology (Dr Mourra) and Radiology (Dr Arrive), Hopital St-Antoine Paris, France; and the Department of Surgery (Dr Hannoun), Hopital Pitie-Salpetriere, Paris, France.
Reprints not available from the authors.