An unusual case of primary liver cancer. Hepatocellular carcinoma in an accessory liver

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1332 An Unusual Case of Primary Liver Cancer Hepatocellular Carcinoma in an Accessory Liver Antonio Basile, M.D.,* Tiziano Croaffo, M.D.,* Alfred0 Gregoris, M.D.,* lrena TavCar, M.D.,* Carlo Zavaroni, M.D.,* Barfolomeo Costa, M.D.,* Pafrizia Li Volsi, M.D.,* Carlo Moreffi, M.D.,t and Sfefano Pizzoliffo,M.D.3 The occurrence of an accessory liver is extremely rare and usually has no clinical significance. This mal- formation may harbor the same pathologic processes present in the anatomically normal liver. Cancer 1994; 73:1332-4. Key words: accessory liver, hepatocellular carcinoma, congenital malformation, alpha-fetoprotein. Ectopic and accessory livers are infrequent developmen- tal abnormalities. In specialistic textbooks they are re- ported as extremely rare malformations of the liver, They have variable size and show a histologic pattern of typical hepatic tissue. They may occur as a separate ectopic organ’-3 or be attached to the liver by a bridge of hepatic tissue or a mesentery in which vascular connec- tions and biliary ducts are ~ontained.~ Usually, they have no clinical significance and are an incidental find- ing at autopsy or during laparotomy’ or radiologic in- vestigation. They may show the same pathologic fea- tures simultaneously present in the anatomically nor- mal li~er,’,~,~ but their isolated involvement is limited to complications such as torsion7 or compression of adja- cent structures.8 We report a case of hepatocellular carcinoma exclu- sively involving the accessory liver, whose removal looks promising as a cure. From the *Division of Internal Medicine and the tDepartment of Radiology, Ospedale di San Vito a1 Tagliamento, USL 9, Regione Friuli Ven. Giulia; and the $Institute of Pathologic Anatomy, Facoltl di Medicina, Universitl di Udine, Udine, Italy. The authors thank Professor G. Tasca, Divisione di Chirurgia- Ospedale San Vito a1 Tagliamento, for performing the operation. Address for reprints: Antonio Basile, M.D., Divisione di Medi- cina, USL 9, Ospedale San Maria dei Battuti, 33078 San Vito al Tag- liamento, Pordenone, Italy. Accepted for publication October 10, 1993. Case Report A-57-year-old man was admitted to the hospital because of hemorrhoids and anal canal polyps. Otherwise, the patient was asymptomatic and his physical examination was normal. Liver function and routine laboratory test results were nor- mal, except for unexpected high values of serum alpha-feto- protein (AFP) (2325 ng/ml; normal values < 15). Other tu- moral markers were normal. Investigations were undertaken to explain the finding. A computed tomography scan of the abdomen showed a round, clearly defined mass, 6 cm in diam- eter, which enhanced homogeneously with the same density of the liver after intravenous injection of contrast material. The mass was anterior to the gastric fundus and in contact with the diaphragm. It appeared to be separated from the liver by a hypodense fissure, except for its upper portion, which was attached to the posterior margin of the second liver segment by a thin parenchymatous bridge (Fig. 1). A percutaneous, computed tomography-guided biopsy of the mass disclosed a well-differentiated hepatocellular carci- noma. A digital celiac trunk angiography was performed as a preoperative study. No anomaly of the arterial vascular distri- bution was found. The mass was supplied by a vessel originat- ing from the left gastric artery and was clearly shown during the parenchymal phase as a pathologic round enhancement. During the operation, the mass was found to be adhering to the posterior free margin of the second liver segment and was easily removed together with a portion of the adjacent liver tissue. It measured 6.5 times 6 times 3.5 cm, weighed 110 mg, and was firm. A fibrous flap encircled its equatorial border. The cut surface appeared gray-yellow, micromacronodular, well demarcated, and encapsulated (Fig. 2). Light microscopic study revealed a well-differentiated hepatocellular carci- noma. It consisted entirely of epithelial tumor cells arranged in an alveolar-trabecular pattern (Fig. 3). There were scattered lymphocytic infiltrates, some of which had a follicular-like appearance. No normal liver parenchyma was found within the accessory liver. The peduncle. had the characteristic of a true mesentery and showed a fibrovascular structure that contained scattered islands of normal hepatic tissue only in its tract proximal to the border of the anatomical liver. At the

Transcript of An unusual case of primary liver cancer. Hepatocellular carcinoma in an accessory liver

Page 1: An unusual case of primary liver cancer. Hepatocellular carcinoma in an accessory liver

1332

An Unusual Case of Primary Liver Cancer

Hepatocellular Carcinoma in an Accessory Liver

Antonio Basile, M.D.,* Tiziano Croaffo, M.D.,* Alfred0 Gregoris, M.D.,* lrena TavCar, M.D.,* Carlo Zavaroni, M.D.,* Barfolomeo Costa, M.D.,* Pafrizia L i Volsi, M.D.,* Carlo Moreffi, M.D.,t and Sfefano Pizzoliffo, M.D.3

The occurrence of an accessory liver is extremely rare and usually has no clinical significance. This mal- formation may harbor the same pathologic processes present in the anatomically normal liver. Cancer 1994; 73:1332-4.

Key words: accessory liver, hepatocellular carcinoma, congenital malformation, alpha-fetoprotein.

Ectopic a n d accessory livers are infrequent developmen- tal abnormalities. In specialistic textbooks they a re re- ported as extremely rare malformations of the liver, They have variable size a n d show a histologic pattern of typical hepatic tissue. They may occur as a separate ectopic organ’-3 or be attached to the liver by a bridge of hepatic tissue or a mesentery in which vascular connec- tions a n d biliary ducts are ~ o n t a i n e d . ~ Usually, they have n o clinical significance a n d are an incidental find- ing at autopsy or during laparotomy’ or radiologic in- vestigation. They may show the same pathologic fea- tures simultaneously present in the anatomically nor- mal l i ~ e r , ’ , ~ , ~ but their isolated involvement is limited to complications such a s torsion7 or compression of adja- cent structures.8

We report a case of hepatocellular carcinoma exclu- sively involving the accessory liver, whose removal looks promising as a cure.

From the *Division of Internal Medicine and the tDepartment of Radiology, Ospedale di San Vito a1 Tagliamento, USL 9, Regione Friuli Ven. Giulia; and the $Institute of Pathologic Anatomy, Facoltl di Medicina, Universitl di Udine, Udine, Italy.

The authors thank Professor G. Tasca, Divisione di Chirurgia- Ospedale San Vito a1 Tagliamento, for performing the operation.

Address for reprints: Antonio Basile, M.D., Divisione di Medi- cina, USL 9, Ospedale San Maria dei Battuti, 33078 San Vito al Tag- liamento, Pordenone, Italy.

Accepted for publication October 10, 1993.

Case Report

A-57-year-old man was admitted to the hospital because of hemorrhoids and anal canal polyps. Otherwise, the patient was asymptomatic and his physical examination was normal. Liver function and routine laboratory test results were nor- mal, except for unexpected high values of serum alpha-feto- protein (AFP) (2325 ng/ml; normal values < 15). Other tu- moral markers were normal. Investigations were undertaken to explain the finding. A computed tomography scan of the abdomen showed a round, clearly defined mass, 6 cm in diam- eter, which enhanced homogeneously with the same density of the liver after intravenous injection of contrast material. The mass was anterior to the gastric fundus and in contact with the diaphragm. It appeared to be separated from the liver by a hypodense fissure, except for its upper portion, which was attached to the posterior margin of the second liver segment by a thin parenchymatous bridge (Fig. 1). A percutaneous, computed tomography-guided biopsy of the mass disclosed a well-differentiated hepatocellular carci- noma. A digital celiac trunk angiography was performed as a preoperative study. No anomaly of the arterial vascular distri- bution was found. The mass was supplied by a vessel originat- ing from the left gastric artery and was clearly shown during the parenchymal phase as a pathologic round enhancement. During the operation, the mass was found to be adhering to the posterior free margin of the second liver segment and was easily removed together with a portion of the adjacent liver tissue. It measured 6.5 times 6 times 3.5 cm, weighed 110 mg, and was firm. A fibrous flap encircled its equatorial border. The cut surface appeared gray-yellow, micromacronodular, well demarcated, and encapsulated (Fig. 2). Light microscopic study revealed a well-differentiated hepatocellular carci- noma. It consisted entirely of epithelial tumor cells arranged in an alveolar-trabecular pattern (Fig. 3). There were scattered lymphocytic infiltrates, some of which had a follicular-like appearance. No normal liver parenchyma was found within the accessory liver. The peduncle. had the characteristic of a true mesentery and showed a fibrovascular structure that contained scattered islands of normal hepatic tissue only in its tract proximal to the border of the anatomical liver. At the

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Hepatocellular Carcinoma of an Accessory Liver/Basile e t al . 1333

Figure 2. The cut surface of the mass is well demarcated and micromacronodular in appearance. The tumor entirely covers the accessory liver.

chymal tumors, were easily discarded by the high AFP values and the biopsy findings. The radiologic investi- gations, the evidence from the operation, and the micro- scopic study proved that the neoplasia was limited to the ectopic organ. The quick and complete normaliza- tion of AFP after the operation is further proof. Only the serendipitous discovery of high values of AFP led to the detection of the tumor, which, therefore, might

genital. Figure 1. Sequence of computed tomography sections of the upper have been present for a long time Or even been 'On-

abdomen after intravenous contrast material. (Top) The upper portion of the mass is connected with the second segment of the liver by a thin parenchymatous bridge. (Bottom) The mass is adjacent to the anterior wall of the gastric fundus.

In conclusion, the current case of hepatocellular car-

opposite side, the tumor appeared well demarcated, with few microscopic foci of neoplastic cells spreading outside the cap- sule.

Twenty days after the operation, AFP was reduced by approximately one tenth (218 ng/ml), and after 45 days its values were in the normal range (5 ng/ml). After 12 months the patient is asymptomatic and in good health. AFP remains normal. A computed tomography scan of the abdomen and liver biopsy results are normal.

Discussion

We reported a case of hepatocellular carcinoma devel- Figure 3. Histologic findings of the excised mass, high-power view. The tumor cells show moderate nuclear pleomorphism with striking nucleolar prominence and some multinucleated cells. (H & E,

'ping exclusively On an accessory liver' A different na- ture of the that 1st accessory spleen (usually, how- ever, smaller in size) or benign and malignant mesen- original magnification X400).

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1334 CANCER March 1, 1994, Volume 73, No. 5

cinoma in an accessory liver, although typical in its his- tology, showed an unusual clinical and biologic course (indolent and asymptomatic, with no evidence of liver disease or past viral infection or toxic agents) and makes us wonder whether the tumor could have remained qui- escent for the rest of the patient’s life.

References

1. Gaber M. Accessory liver containing metastatic tumor. Virchows Arch A Pnthol Anat Histopathol 1980; 385:361-4.

2. Vercelli Retta J. Fetal supradiaphragmatic accessory liver. Vir- chows Arch A Pathol Anat Histopathol 1978; 278259-63.

3. Davies JNP. An accessory liver in an African. Br Med 1946; 2736.

4. Iacconi P, Masoni T. Le foi accessoire: presentation de deux cas. Acta Chir Belg 1990; 90:228-30.

5. Castro Viera GA, Sanui ME, CBmara H. Higado accessorio en vescicula biliar. Rev Esp Enferiiz Dig 1990; 77(4):295-6.

6. Collan Y, Hakkiluoto A, Hastbacka J. Ectopic liver. Ann Chir Gynaecol 1978; 6727-9.

7. Cullen TS. Accessory lobes of the liver. Arch Surg 1925; 11:718. 8 . El Haddad MJ, Currie AB, Honeiman M. Pyloric obstruction by

ectopic liver. B r Surg 1985; 72:917.