Treatment Strategy for Patients with Cystic Lesions Mimicking a Liver Tumor a Recent 10-Year Surgical Experience in Japan

ORIGINAL ARTICLE Treatment Strategy for Patients With Cystic Lesions Mimicking a Liver Tumor A Recent 10-Year Surgical Experience in Japan

Mitsuo Shimada, MD, PhD; Kenji Takenaka, MD, PhD; Tomonobu Gion, MD; Yuh Fujiwara, MD; Kenichi Taguchi, MD; Kiyoshi Kajiyama, MD; Ken Shirabe, MD; Keizo Sugimachi, MD, PhD

Objective: To clarify some of the difficulties in deter- rhage in 7 patients, localized cystic dilation of the bile duct mining the appropriate surgical indications for cystic le- due to hepatolithiasis in 1, cystadenoma in 1, and mucin- sions mimicking a neoplasm in the liver. producing cholangiocarcinoma in 1. In only one case was postoperative diagnosis identical to the preoperative diag- Design: A retrospective review of hepatic resections for nosis. In one case, an intraoperative pathological exami- cystic lesions mimicking a neoplasm in the liver be- nation showed the tumor to be a mucin-producing cho- tween August 1, 1986, and July 31, 1996. langiocarcinoma instead of a cystadenocarcinoma. A tumor- marker analysis of the fluid in the cystic lesions also did Setting: A university hospital with a long history of he- not contribute to a definite diagnosis. Furthermore, cyto- patic resection for cystic lesions mimicking a neoplasm logical examination of the fluid could not completely ex- in the liver. clude malignancy. Neither mortality nor morbidity occurred in any of the patients, and their mean length of Patients: Ten patients with such cystic lesions in the hospitalization after hepatectomy was only 13.7 days. liver, who underwent a hepatectomy during a recent 10- year period, were included in this study. Conclusions: The accurate diagnosis of cystic lesions mimicking a tumor remains problematic; however, the Main Outcome Measures: Detailed clinicopatho- results of hepatectomy for such cases are normally sat- logic data were analyzed, and comparisons were made isfactory. Such cystic lesions of the liver should be indi- between the preoperative and postoperative diagnoses. cated for hepatectomy whenever the possibility of a cancerous neoplasm cannot be completely ruled out. Results: The postoperative diagnoses consisted of cysts, including cysts complicated by an infection or hemor- Arch Surg. 1998;133:643-646

HERE HAS been tremen- modalities. Both surgeons and physi- dous progress in refine- cians have hesitated to some extent to se- ment of the diagnostic mo- lect hepatic resection for treating cystic le- dalities for hepatic tumors, sions, even when malignancy could not including ultrasonogra- always be ruled out. phy,T computed tomography, magnetic However, thanks to great improve- resonance imaging, and angiography com- ments in operative technique, periopera- bined with digital subtraction angiogra- tive patient management, and patient phy. However, obtaining an accurate pre- selection criteria, hepatic resection has operative diagnosis of cystic lesions been established as one of the most effec- mimicking a neoplasm in the liver still re- tive treatment modalities even for pa- mains problematic. tients with cirrhosis and hepatocellular Until recently, a hepatic resection had carcinoma.1,2 been considered one of the most danger- The aim of this study is to clarify From the Departments of ous operative procedures in general sur- the problems of surgical indications Surgery II (Drs Shimada, gery. Furthermore, for patients who have for cystic lesions, based on a recent Takenaka, Gion, Fujiwara, Shirabe, and Sugimachi) and nonneoplastic, nonparasitic, or nonhyda- 10-year experience of hepatic resection Pathology II (Drs Taguchi and tid cysts, but not those who have a symp- for cystic lesions mimicking a tumor. Kajiyama), Faculty of tomatic polycystic liver, either fenestra- The treatment strategies for such Medicine, Kyushu University, tion or ethanol injection is considered one special-entity disorders in the liver are Fukuoka, Japan. of the safest and most effective treatment also discussed.

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 PATIENTS AND METHODS however, has only been routinely available since 1990. All customary laboratory examinations including serological tests for parasites were also performed. In addi- PATIENTS tion, cytological and bacteriological evaluations were performed if needed. Our treatment strategy was as fol- Ten out of 613 patients with cystic lesions mimicking a tu- lows: (1) an asymptomatic benign cyst was merely fol- mor who underwent a hepatic resection at Kyushu Uni- lowed up, (2) a symptomatic simple cyst was indicated versity Hospital during the 10-year period between Au- for ethanol injection,3 or (3) either suspected malignant gust 1, 1986, and July 31, 1996, were retrospectively studied. or definitely malignant cystic lesions were indicated for Their ages ranged from 42 to 75 years (mean age, 56.6 years). a hepatectomy. Six patients (60%) were men and 4 (40%) were women. In principle, the final decisions were made by our sur- The operative procedures were as follows: right trisegmen- geons. However, during the process of determining the fi- tectomy, 1; right lobectomy, 2; segmentectomy, 2; bisub- nal indications, a preoperative conference was held among segmentectomy, 2; and subsegmentectomy or other mi- our surgeons and radiologists expert in liver tumors on each nor procedure, 3 (Table 1). patient’s liver cyst. All 10 patients evaluated in this study finally underwent a hepatectomy either because malig- DETERMINATION OF SURGICAL INDICATION nancy could not be ruled out (9 patients) or a malignant transformation was strongly suspected (1 patient). In- The diagnoses were made using ultrasonography, dy- formed consent for surgical treatment was obtained from namic computed tomography, magnetic resonance imag- each patient. Typical preoperative images, in which ma- ing, and angiography. Magnetic resonance imaging, lignancy could not be ruled out, are shown in the Figure.

Table 1. Preoperative and Postoperative Diagnoses, With Operative Variables, for Hepatic Cystic Lesions Mimicking Liver Tumor in 10 Patients

Diagnosis Hospital Patient Operation Blood Stay After No. Preoperative Postoperative Procedure Time, min Loss, mL Operation, d* 1 Cystadenoma Complicated cyst Subsegmentectomy 225 1000 13 2 Hepatoma with necrosis Cyst Partial resection 210 270 13 3 Cystadenoma Cyst Partial resection 350 1100 17 4 Atypical hemagioma† Localized cystic dilatation Right lobectomy 225 850 16 of bile duct due to hepatolithiasis 5 Metastatic tumor Cyst Left lateral segmentectomy 166 350 11 6 Cystadenocarcinoma Cystadenoma Right trisegmentectomy 295 800 13 7 Cystadenocarcinoma Complicated cyst Right lobectomy 290 1800 14 8 Cystadenoma Cyst Posterior segmentectomy 310 1300 14 9 Cystadenoma Cystadenoma (suspected)‡ Bisubsegmentectomy 247 1200 13 10 Cystadenocarcinoma Cholangiocarcinoma Bisubsegmentectomy with 353 2000 13 extended lymph node dissection

*No postoperative complications occurred in any of the patients. †The final diagnosis of this tumor was not obtained before operation. Furthermore malignancy such as cholangiocarcinoma could not be ruled out before operation. ‡The tumor encapsulated by fibrous tissue was completely composed of necrotic tissue with cystic space, calcification, cholesterin clefts, and hemorrhage. The cystic wall was partially lined by a cuboidal epithelium.

RESULTS onic antigen and ␣-fetoprotein were normal. The concentrations of such tumor markers as carbohydrate antigen The postoperative diagnoses consisted of cysts includ- and carcinoembryonic antigen in 3 patients, from whom ing the following: those complicated by an infection or cystic fluid was obtained, were analyzed. Table 2 gives hemorrhage,7; a localized cystic dilation of the bile duct the results of the cystic fluid analysis. The 3 patients in due to hepatolithiasis, 1; cystadenoma, 1; and mucin- this study with elevated tumor-marker levels in the cys- producing cholangiocarcinoma, 1. No definite diagno- tic fluid had cysts that were complicated by either an old sis could be made preoperatively, and in only 1 case was infection or a hemorrhage, and the tumor marker analy- the postoperative diagnosis identical to the preopera- sis of their cystic fluid did not confirm a definite preop- tive diagnosis. erative diagnosis. Cytological examination of the cystic Three patients had abnormal laboratory findings in- fluid was performed in 3 patients (2 with complicated cluding elevated ␥-glutamyl transpeptidase (1 patient), cysts and 1 with cystadenoma), and all 3 patients’ re- alkaline phosphatase (2 patients), and carbohydrate an- sults were negative. However, a negative cytological re- tigen 19-9 (1 patient). However, both the carcinoembry- sult could not completely rule out malignancy.

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B D

Preoperative images of cystic lesions mimicking a tumor. A, Case 9. A magnetic resonance imaging sagittal view shows a slightly enhanced thick wall of the cyst (arrows). However, no enhancement was recognized in the renal cyst (arrowheads). A cystadenoma was strongly suspected; however, it was found to be a cyst complicated by an old infection. B, Case 8. A magnetic resonance imaging sagittal view shows a slightly enhanced thick wall of the cyst (arrowheads) and a solid portion (arrow). A cystadenoma was strongly suspected; however, the contents of the cyst were completely necrotic and the solid portion consisted of fibrous tissue. No definite evidence of a neoplasm was observed. C, Case 6. A computed tomographic scan shows a cystic mass with a solid projection (arrow) and calcific foci (arrowheads). The internal solid portion had grown rapidly, thus a cancerous transformation of the cystadenoma was strongly suspected. D, Case 10. An enhanced magnetic resonance image shows a cystic mass with a mural mass projected in a papillary fashion (arrow) and an enhanced cyst wall (arrowheads). The fluid content can also be seen.

The single patient with cholangiocarcinoma under- for a cure.9-12 Surgical resection, therefore, should be indi- went an extensive lymph node dissection and a hepatec- cated even for cystadenoma, which can usually be differ- tomy, since an intraoperative pathological examination entiated from cystadenocarcinoma. The presence of mu- revealed mucin-producing cholangiocarcinoma instead ral or septal nodules, a discrete soft tissue mass, and a of a cystadenocarcinoma. This patient’s case exempli- possible thick and coarse calcification increase the likeli- fies how an intraoperative pathological examination is hood that a suspected cystadenoma is really a cystadeno- valuable in accurately diagnosing and determining the carcinoma.13 However, no characteristic imaging features treatment strategy. have yet been identified that differentiate cystadenocarci- Operation time ranged from 166 to 353 minutes noma from cystadenoma. If a cystadenoma is suspected, (mean time, 267 minutes) and estimated blood loss ranged then surgery must be performed immediately because bili- from 270 to 2000 mL (mean blood loss, 1067 mL). No ary cystadenoma and cystadenocarcinoma cannot be reli- mortality or morbidity was observed, and the length of ably differentiated based on the findings in the macro- hospitalization after operation ranged from 13 days to scopic specimen alone. 17 days (mean hospitalization, 13.7 days) (Table 1). Another problem that should be considered in the management of cystic lesions in the liver is that cysts com- COMMENT plicated by hemorrhage or infection often tend to be mis- diagnosed as either a cystadenoma or a cystadenocarci- Definite preoperative differentiation between cystad- noma. The differential diagnosis of cystic hepatic lesions enoma and cystadenocarcinoma is difficult using medical includes a complicated cyst, abscess, hematoma, echi- imaging,4 regardless of the advances in radiographic im- nococcal cyst, mesenchymal hamartoma, undifferenti- aging modalities. Moreover, cystadenoma can also be po- ated embryonal sarcoma, and cystic metastasis. Simple tentially malignant.5-8 For both biliary cystadenoma or cyst- hepatic cysts usually do not contain internal septations adenocarcinoma, a complete excision offers the best chance or thick irregular walls.14 Hepatic abscesses usually have

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 results of hepatectomy for such diseases, however, have Table 2. Concentrations of Tumor Markers been satisfactory. Hepatectomy should be indicated for in the Cystic Fluid of 3 Patients* cystic lesions mimicking a tumor of the liver whenever a potential malignancy cannot be completely ruled out. Patient No. CEA, µg/L CA 19-9, U/mL Histological Diagnosis 6 250.7 926 950 Cystadenoma Reprints: Mitsuo Shimada, MD, The Department of Sur- 7 1.2 120 000 Cystadenoma (suspected) gery II, Faculty of Medicine, Kyushu University Hospital, 8 7.3 61 000 Complicated cyst 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-82, Japan. *CEA indicates carcinoembryonic antigen (reference range, Ͻ2.5 µg/L); CA19-9, carbohydrate antigen 19-9 (reference range, Ͻ37 U/mL). REFERENCES

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