Incidental cystadenoma after laparoscopic treatment of hepatic cysts: which strategy?

Biliary cystadenoma is a very rare hepatic neoplasm, accounting for fewer than 5% of cystic neoplasms of the liver; regardless of the various diagnostic modalities, such a lesion may be difficult to distinguish preoperatively from a cystadenocarcinoma. Although a diagnosis of cystadenoma during open hepatic surgery demands a complete surgical resection, there are few reports describing the correct approach to such lesions after a laparoscopic approach. This article presents the first case series of incidental cystadenoma after laparoscopic surgery for hepatic cystic lesions. One patient with a polycystic liver disease treated with a laparoscopic enucleation of the larger cyst declined the reintervention after the diagnosis of cystadenoma; she had no recurrence at follow-up. One patient with a large simple hepatic cyst laparoscopically enucleated had no recurrence at the 18-month follow-up. In one patient, there was a high suspicion of recurrence of cystadenoma after the laparoscopic fenestration of a large cyst, but a histopathological specimen obtained after the open surgical resection could not confirm any signs of cystadenoma. The incidental finding of biliary cystadenoma after laparoscopic fenestration of a cystic hepatic lesion requires an open hepatic resection. When a complete laparoscopic enucleation of the cyst may be assured, a strict clinical, biochemical, and radiologic follow-up could be considered as the definitive treatment, demanding the surgical intervention only in case of recurrence or high suspicion for malignancy.     

Biliary cystadenoma: short- and long-term outcome after radical hepatic resection

The intrahepatic biliary cystadenoma is a rare benign tumor of the liver, originating from an intrahepatic bile duct: it becomes symptomatic only when it causes obstruction of the bile duct itself. Regardless of the various diagnostic modalities available, it is difficult to distinguish preoperatively the cystadenoma both from a simple liver cyst, and from a cystic carcinoma of the bile duct. An incomplete surgical removal of the cyst often results in a higher risk of size increase and recurrence, even considering that the lesion may degenerate into a cystadenocarcinoma. Between January 2004 and May 2011, 1,173 liver resections were carried out at the Hepatobiliary Surgery Unit of San Raffaele Hospital: 12 of these were performed for cystadenoma. Forty-six patients underwent laparoscopic liver cysts deroofing: definitive histological examination in six of these patients revealed instead the diagnosis of cystadenoma. In 50% of cases, the diagnosis of cystadenoma was therefore acquired as a result of an incidental finding. The patients were all female, median age 45 years. The liver resection included six cases of left hepatectomy, three left lobectomies, and three of the right hepatectomy. The operations were performed by laparotomy, with the exception of two left lobectomies completed laparoscopically. In all cases, the postoperative course was without major complications. The resection was radical in all cases and the median hospital stay was 5 days. At a median follow-up of 16 months (range 7-30), all patients are alive and disease free. Biliary cystadenomas can easily be misunderstood and interpreted as simple hepatic cysts. Radical surgical resection is necessary and provides good short- and long-term outcomes.     

Management and long-term follow-up of hepatic cysts.

PURPOSE: To provide an algorithm for the management of hepatic cysts through an analysis of our series over 16 years. METHOD: We reviewed the surgical management and outcome of patients with hepatic cysts between 1984 and 2000 at a single institution. Data were collected by chart review, telephone interview, and follow-up hepatic ultrasonography. RESULTS: Forty-four patients (36 females, 8 males) underwent a total of 46 operations for hepatic cysts (mean size 12.0 +/- 5.2 cm) with a mean follow-up of 5.1 +/- 4.0 years. We treated 28 simple cysts, 4 polycystic liver disease (PCLD), 7 cystadenomas, 2 hydatid cysts, 1 cystadenocarcinoma, 1 endometrioma, and 1 hepatic foregut cyst. Operations included simple drainage, wide unroofing (open and laparoscopic), and hepatic resection. Four patients experienced a symptomatic recurrence after definitive treatment; 3 of these patients had PCLD. Four of the 7 patients with cystadenomas had undergone previous operations that required subsequent definitive resection without a recurrence. CONCLUSIONS: The preoperative distinction between simple cysts and cystadenomas/cystadenocarcinomas can be difficult, yet the management is different. Unroofing is a safe and effective operation for patients with simple cysts. Patients with PCLD frequently have recurrences. Cystadenomas should be completely resected owing to the likelihood of recurrence after partial excision and the risk of eventual cystadenocarcinoma. We present a treatment algorithm for the preoperative evaluation and management of hepatic cysts based on the largest number of patients with the longest follow-up reported to date.     

Cystadenoma and cystadenocarcinoma of the liver: a single center experience

BACKGROUND: Biliary cystadenomas and cystadenocarcinomas comprise 5% of cystic lesions in the liver. Cystadenomas are often incorrectly diagnosed as simple cysts, which results in inadequate therapy. Recurrence and possible malignant transformation are consequences of incomplete excision. Cystadenocarcinomas are very rare tumors that are felt to be biologically indolent. STUDY DESIGN: A retrospective review of 18 cystadenomas and 4 cystadenocarcinomas treated at the Cleveland Clinic from July 1985 to November 2002. RESULTS: All 18 patients with cystadenomas were women; mean age was 48 years. The majority (16 of 18) were symptomatic. Preoperative CT scans demonstrated cyst(s) with septations in all patients. Fifty-five percent had undergone prior intervention(s) to treat the cyst. Thirteen patients had complete excision of the cystadenoma, either by enucleation or liver resection. None of the patients developed recurrent cystadenomas (mean followup 37 months). Of 4 patients with cystadenocarcinoma, 3 were women; mean age was 60 years. All were symptomatic. Preoperative CT scans demonstrated masses with both cystic and solid components. No patient had undergone prior intervention. All had a liver resection. Two patients died of metastatic disease at 6 and 12 months, respectively. One patient is alive and disease-free at 16 years; 1 is alive with metastatic disease 10 years after the liver resection. CONCLUSIONS: Cystadenomas are uncommon tumors that are often incorrectly diagnosed as simple cysts. Preoperative imaging that demonstrates the presence of internal septations highly suggests the diagnosis of cystadenoma. Intraoperative biopsy and frozen section(s) are essential, although they are not 100% accurate. Cystadenomas require complete excision to prevent recurrence and the possibility of malignant transformation. Cystadenocarcinomas are very rare. Despite complete resection, cystadenocarcinomas can recur in a short period of time. The biologic behavior of these tumors can vary widely.     

肝内胆管囊腺瘤与囊腺癌的诊断及外科治疗

目的 探讨肝内胆管囊腺瘤和囊腺癌的诊断和治疗方法.方法 对中国医学科学院肿瘤医院1996年1月至2007年10月收治的8例肝内胆管囊腺瘤和囊腺癌的临床资料进行回顾性分析.结果 肝内胆管囊腺瘤和囊腺癌无特征性临床表现.术前AFP均为阴性;B超和CT的诊断正确率分别为75%(6/8)、87.5%(7/8).8例病人均行手术切除,7例行肝肿瘤不规则切除术,1例行左半肝切除术.术后病人恢复良好.8例均获得随访,除1例囊腺癌术后3年死于肿瘤复发转移外,其余均生存良好.结论 肝内胆管囊腺瘤与囊腺癌主要依靠影像学和病理诊断,手术切除可以获得满意的疗效.     

Cystadenoma and laparoscopic surgery for hepatic cystic disease: a need for laparotomy?

Background This study aimed to evaluate the incidence of cystadenoma diagnosis in a series of laparoscopic treatments for nonparasitic liver cysts, as well as its management. Methods From 1996 to 2004, 26 patients with a nonparasitic cyst of the liver were selected for laparoscopic liver surgery. Solitary nonparasitic liver cysts were, whenever feasible, completely enucleated. Results In four patients, the histopathologic examination showed a cystadenoma. Three patients with 13, 9, and 12-cm cysts, respectively, had undergone complete enucleation of the lesion, with no evidence of recurrence in the follow-up visit. One patient with multicystic liver experienced a recurrence and required an open hepatic resection. Conclusions When a complete laparoscopic enucleation of the cyst can be ensured, a strict follow-up assessment should be considered as the definitive treatment, with surgical intervention demanded only in the case of recurrence or high suspicion for malignancy.     

肝内胆管囊腺瘤的诊治分析

目的：提高对肝内胆管囊腺瘤的认识。 方法：回顾性分析8年来收治的18例肝内胆管囊腺瘤患者的临床表现、影像特点及外科治疗的方式。 结果：4例为体检发现,其他14例均有不同症状,主要表现为右上腹不适或疼痛11例,腹部肿块3例,伴有黄疸发热等胆管炎症状2例。B超和CT提示肝内囊性占位性病变,可呈多囊或单囊,内壁可见乳头状物。均获得手术切除,18例肝内胆管囊腺瘤中6例发生癌变。 癌变者术后平均生存时间为35个月(27-58个月)。结论：术前确诊肝内胆管囊腺瘤困难,肝内胆管囊腺瘤易恶变,发现后应早期手术切除,可有效防止复发或恶变。     

Spontaneous rupture of a hepatic cystadenoma and cystadenocarcinoma: report of two cases

Hepatobiliary cystadenomas and cystadenocarcinomas are rare tumors. Differentiating between these tumors and benign hepatic cysts may be difficult. Because of their rarity, diagnosis is often delayed and may result in inaccurate treatment, resulting in unnecessary morbidity and mortality. The purpose of this report is to draw attention to these entities and their complications. We report on two cases with spontaneous rupture of hepatobiliary cystadenoma and cystadenocarcinoma cysts, initially treated as simple hepatic cysts by aspiration, or by aspiration combined with sclerotherapy. The spontaneous rupture of the cysts appeared years after the initial treatment of the cysts, leading in one case to a prolonged stay in an intensive care unit. In both cases, a formal liver resection was carried out and microscopic investigations revealed a mucinous cystadenocarcinoma and cystadenoma. In conclusion, although hepatobiliary cystadenomas and cystadenocarcinomas are rare findings, they should not be forgotten in the diagnostic workshop when examining patients with hepatic cysts. If hepatobiliary cystadenomas and cystadenocarcinomas cannot be excluded following radiological imaging, surgery is recommended.     

术前精确定位指导肝内胆管囊腺瘤的治疗3例

目的 观察肝内胆管囊腺瘤的临床、影像、病理及外科治疗特点,探讨该病的临床诊疗方法.方法 对2005年1月～2009年1月收治的3例肝内胆管囊腺瘤病例的临床表现、影像特点、诊疗策略及效果进行回顾性分析.结果 3例患者中2例男性、1例女性,临床上均表现为上腹痛,黄疸等症状;通过B超、MRCP及ERCP等手段予以肿瘤的精确定...     

Hepatobiliary cystadenoma combined with multiple liver cysts: report of a case

Hepatobiliary cystadenomas are rare benign tumors with malignant potential. They are almost always solitary lesions accompanied by multilocular cysts in the liver, and are difficult to differentiate from cystadenocarcinoma, despite the diagnostic modalities available. This report describes a case of hepatobiliary cystadenoma with multiple cysts in the left hepatic lobe, diagnosed by magnetic resonance imaging in a 48-year-old woman. Abdominal computed tomography revealed only multiple cystic lesions in the left lobe, but cholangiography via a nasogastric biliary drainage tube combined with percutaneous transhepatic cholangiography showed a stenotic region with fine irregularity in the left lateral posterior segmental bile duct and left lateral anterior segmental bile duct. Hepatobiliary cystadenocarcinoma with multiple liver cysts was suspected. We performed left hepatectomy, and microscopic examination confirmed a diagnosis of hepatic cystadenoma with multiple liver cysts. There was no nuclear atypia or mitosis in the epithelium of the locus, which was constructed of simple columnar-to-cuboidal epithelium with basal nuclei. The patient is well without recurrence more than 4 years after surgery. Received: July 5, 2000 / Accepted: January 9, 2001     

38例胰腺囊性肿瘤的临床诊断及外科治疗

目的探讨胰腺囊腺瘤、囊腺癌的诊断和治疗方法.方法对1980年1月～2005年4月中国医科大学附属第一医院收治的38例胰腺囊腺瘤和囊腺癌患者的临床表现、检查、手术方式及预后进行了分析.结果胰腺囊腺瘤、囊腺癌主要发生在胰体尾部（79%）,女性多见（84%）,肿瘤无特异性临床表现.超声和CT是主要的影像学检查方法,本组病例超声的诊断率为66%,CT为78%,血清肿瘤标志物检查对诊断意义不大.38例胰腺囊腺瘤、囊腺癌中行胰十二指肠切除2例,保留十二指肠的胰头切除1例,胰体部分切除1例,胰体尾切除21例,肿瘤摘除8例.手术切除的28例胰腺囊腺瘤中25例获得随访,2例因其他疾病死亡,23例健在.8例胰腺囊腺癌手术切除5例,4例获得随访,3例存活.手术后胰瘘的发生率为28%.结论胰腺囊腺瘤手术切除后可以达到治愈,囊腺癌切除后也有较高的生存率.术中病理检查是术式选择的关键,对于诊断明确的良性肿瘤可行胰腺局部切除术,或行肿瘤摘除术.     

Large cystic lesions of the liver in adults: a 15-year experience in a tertiary center.

BACKGROUND: Cystic lesions of the liver consist of a heterogeneous group of disorders and may present a diagnostic and therapeutic challenge. Large hepatic cysts tend to be symptomatic and can cause complications more often than smaller ones. STUDY DESIGN: We performed a retrospective review of adults diagnosed with large (> or = 4 cm) hepatic cystic lesions at our center, over a period of 15 years. Polycystic disease and abscesses were not included. RESULTS: Seventy-eight patients were identified. In 57 the lesions were simple cysts, in 8 echinococcal cysts, in 8 hepatobiliary cystadenomas, and in 1 hepatobiliary cystadenocarcinoma. In four patients, the precise diagnosis could not be ascertained. Mean size was 12.1 cm (range, 4 to 30 cm). Most simple cysts were found in women (F:M, 49:8). Bleeding into a cyst (two patients) and infection (one patient) were rare manifestations. Percutaneous aspiration of 28 simple cysts resulted in recurrence in 100% of the cases within 3 weeks to 9 months (mean 4(1/2) months). Forty-eight patients were treated surgically by wide unroofing or resection (laparoscopically in 18), which resulted in low recurrence rates (11% for laparoscopy and 13% for open unroofing). Four of the eight patients with echinococcal cysts were symptomatic. All were treated by open resection after irrigation of the cavity with hypertonic saline. There was no recurrence during a followup period of 2 to 14 years. Hepatobiliary cystadenomas occurred more commonly in women (F:M, 7:1) and in the left hepatic lobe (left:right, 8:0). Seven were multiloculated. All were treated by open resection, with no recurrence, and none had malignant changes. Cystadenocarcinoma was diagnosed in a 77-year-old man, and was treated by left hepatic lobectomy. CONCLUSIONS: Large symptomatic simple cysts invariably recur after percutaneous aspiration. Laparoscopic unroofing can be successfully undertaken, with a low recurrence rate. Open resection after irrigation with hypertonic saline is a safe and effective treatment for echinococcal cysts. Hepatobiliary cystadenomas have predilection for women and for the left hepatic lobe. Malignant transformation is an uncommon but real risk. Open resection is a safe and effective treatment for hepatobiliary cystadenoma, and is associated with a low recurrence rate.     

Cystic neoplasms of the pancreas

From 1963 to 1983, 26 patients with cystic neoplasms of the pancreas were treated at the Lahey Clinic, Burlington, Mass. Cystadenoma (15 patients) was more common than cystadenocarcinoma (11 patients). Preoperative symptoms, such as abdominal pain, were present for as long as 18 years before diagnosis. The mean size of cysts was 7 cm. Distal pancreatectomy, the most common operation, was performed in ten patients. Eight of the 11 patients with cystadenocarcinoma had metastatic disease at the time of surgical exploration. There was one postoperative death (3.8%). Patients with cystadenocarcinoma had an adjusted median survival time after operation of 6.0 months. The long prodrome in many of the cancer patients suggests that benign cystadenomas, particularly of the mucinous type, may undergo malignant degeneration. Benign cystadenoma seems unlikely to recur after adequate resection. Whenever possible, complete excision of cystadenoma and cystadenocarcinoma is the procedure of choice.     

Hepatic resection for cystic lesions of the liver.

OBJECTIVE. The purpose of this study was to report the authors' experience with hepatic resection for cystic lesions of the liver. SUMMARY BACKGROUND DATA. Past experience with aspiration, sclerosing therapy, internal drainage, fenestration, and marsupialization are of limited value. Hepatic resection has evolved into a safe operation over the last two decades. METHODS. A retrospective study of 44 patients with various cystic lesions of the liver (polycystic disease, 2; solitary or multiple congenital cysts, 19; biliary cystadenoma, 6; cystadenocarcinoma, 3; squamous cell carcinoma, 3; Caroli's disease, 5; and hydatid cyst, 6) was performed. RESULTS. After 7 trisegmentectomies, 24 lobectomies, 6 left lateral segmentectomies, and 7 nonanatomical hepatic resections, only 1 operative death occurred in a Jehovah's Witness. Symptomatic relief was complete and permanent in all of the patients with benign congenital or parasitic hepatic cysts, except for the two patients with polycystic disease of the liver. One of the 3 patients with adenocarcinoma and 3 patients with squamous cell carcinoma of the cyst wall died of tumor recurrence between 3 and 14 months after hepatic resection. CONCLUSIONS. Hepatic resection is safe and effective for cystic lesions of the liver. Symptomatic relief is complete and permanent after hepatic resection, except in cases of diffuse polycystic disease of the liver. Liver transplantation should be considered for diffuse polycystic disease of the liver when the symptoms are extremely severe.     

Giant biliary cystadenoma: a case report

Biliary cystadenoma is a very rare benign cystic tumour of the liver. Fewer than 150 cases have been described in the literature so far. The authors present a case of a 29-year-old female with a giant intrahepatic biliary cystadenoma who presented with abdominal pain and obstructive jaundice. The patient was treated with left hepatectomy and now 12 months after the surgery she is in good condition with no signs of recurrence of the disease. Clinical presentation of biliary cystadenoma is not specific. Diagnosis is based on imaging methods, mainly ultrasound and CT scan. However, it is often misdiagnosed. For treatment, radical resection is advocated because a biliary cystadenoma is considered to be a premalignant lesion. The prognosis of biliary cystadenoma after complete resection is excellent. Nevertheless, there is a risk of recurrence or malignant transformation after incomplete resection.     

Hepatic cysts: diagnosis and management

Cystic lesions of the liver are common and usually benign. The most frequent lesion is simple hepatic cyst: typical imaging findings make their diagnosis easy and they require no treatment. Complicated hepatic cysts (i.e., compression, hemorrhage, infection) have more variable imaging findings making diagnosis more difficult; they may evoke other diagnoses. Asymptomatic hepatic cysts do not require any treatment or follow-up. Symptomatic cysts may benefit from simple follow-up or may be treated by laparoscopic fenestration or alcohol sclerotherapy. The differential diagnosis of complicated hepatic cysts includes cystadenoma, cystadenocarcinoma, hydatid cyst, or cystic metastasis; surgical resection is usually indicated for these lesions.     

Therapeutic dilemmas in patients with symptomatic polycystic liver disease

Most reports of the operative treatment of symptomatic polycystic liver disease (PCLD) are anecdotal or consist of only a small subset of patients in an institution's overall experience treating hepatic cysts. We have reviewed our experience with nine consecutive patients with symptomatic PCLD undergoing operative treatment from 1981 to 1987. Indications for operation include chronic abdominal pain (4 patients), cyst infection (2 patients), biliary obstruction (2 patients), inferior vena cava obstruction (2 patients), and symptomatic abdominal distention (2 patients). The average duration of symptoms leading to operation was 7.8 months. Three types of cystic disease were identified based on gross morphology: dominant cystic disease (3 patients), diffuse cystic disease (4 patients), and mixed cystic disease (2 patients). Operations to treat symptomatic PCLD included unroofing and external drainage of infected cysts (2 operations), simple unroofing (1 operation), cyst fenestration alone (4 operations) and fenestration combined with resection (3 operations). Treatment directed at principally dominant cysts (five patients) was associated with resolution of symptoms and low morbidity and mortality. Treatment directed at diffusely cystic disease (four patients) resulted in significant morbidity and mortality including three deaths. Successful surgical treatment of symptomatic patients with PCLD depends on accurate preoperative identification of patients with symptoms related to one or more dominant cysts. In this setting fenestration or simple unroofing of the dominant cyst is safe and effective treatment. By comparison, extensive fenestration with or without hepatic resection in patients with symptoms attributed to a diffusely cystic liver may be associated with unacceptable morbidity and mortality.(ABSTRACT TRUNCATED AT 250 WORDS)     

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??Diagnosis and treatment of intrahepatic biliary cystadenoma: an analysis of 17 cases LIU Yuan, ZHOU Yong, GENG Dong-hua, et al. Department of General Surgery, Shengjing Hospital of China Medical University, Shenyang 110004, China
Corresponding author: LIU Jin-gang, E-mail:liujg@sj-hospital.org
Abstract Objective To study the diagnosis and treatment of intrahepatic biliary cystadenoma. Methods The clinical data of 17 cases of intrahepatic biliary cystadenoma admitted between January 2003 and December 2009 in Shengjing Hospital of China Medical University were analyzed retrospectively. Results The majority of 17 cases presented as upper abdominal discomfort or pain. Tow cases had undergone previous unroofing of the cyst for misdiagnosis as simple liver cyst. Abnormal serous carbohydrate antigen 19-9 (CA19-9) was observed in 6 cases. Serous alpha-fetoprotein (AFP) level was slightly elevated in one case, and serous carcinoembryonic antigen (CEA) was normal in all cases. Seven cases were diagnosed correctly according to imaging examination. All the cases underwent total resection of the cyst. Pathological diagnosis was intrahepatic biliary cystadenoma in all the 17 cases, and 3 of them showed malignant change. No recurrence was found after followed up for 8 to 60 months. Conclusion Preoperative diagnosis of intrahepatic biliary cystadenoma is difficult. Intrahepatic biliary cystadenoma is easy to develop to cystadenocarcinoma. The prognosis of radical resection is satisfied.     

Major hepatic resection. A 25-year experience.

Major hepatic resections were performed on 138 patients for a variety of conditions. There was one intraoperative death. Including this patient, there were 15 deaths within 30 days of the operation (operative mortality 10.9%). Important postoperative complications were intra-abdominal sepsis (17%), biliary leak (11%), hepatic failure (8%), and hemorrhage (6%). The results of 30 resections for the benign lesions, liver cell adenoma, focal nodular hyperplasia, hemangioma, and cystadenoma showed no operative mortality and low morbidity. Of 26 patients with hepatocellular carcinoma, seven died within a month of operation. The cumulative survival of the 26 at five years was 38%, and of the 19 who survived the procedure, 51%. Poor survival followed resections for cholangiocarcinoma and "mixed tumors." The five-year cumulative survival of 22 patients who had colorectal metastases excised was 31%. Apart from a patient with carcinoid, prolonged survival was rare after resection of other secondaries and after en bloc resections for tumors directly invading the liver. Hepatic resection was of value in the management of some patients with hepatic trauma, Caroli's disease, liver cysts, and intrahepatic stones.