Intrahepatic biliary cystadenoma, a challenging diagnosis and management: A case report.

INTRODUCTION AND IMPORTANCE: Biliary cystadenoma (BC) is a benign hepatic cystic tumor with degenerative potential. Hepatic MRI can help guide the diagnosis. Surgical resection is recommended due to the malignant potential of biliary cystadenomas. Only anatomopathological examination of the surgical specimen can establish the definitive diagnosis of BC. The objective of this case report is to enhance our understanding of this disease and contribute to precise diagnosis for optimal management. CASE PRESENTATION: A 55-year-old woman with a history of hypertension and atrial fibrillation presented to the surgery department with paroxysmal right hypochondrial pain. Ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) revealed a large septated cystic lesion occupying segments II, III, and IV of the liver. The patient underwent left hepatectomy without incident. The postoperative course was marked by a deep collection opposite the sectional area, which was successfully treated with antibiotics and radiological drainage. The pathological diagnosis confirmed BC without signs of malignancy, and no recurrence was detected post-surgery. CLINICAL DISCUSSION: The rarity of BC, the absence of specific clinical signs and its potential for malignant transformation, underline the need for sophisticated imaging techniques. However, preoperative radiological diagnosis does not exceed 50 %. The operative decision requires a multidisciplinary discussion between radiologists and surgeons. This case highlights the unavailability of radical surgical treatment in cases of strong preoperative suspicion of BC. The cooperation of the pathologist in the histological diagnosis is crucial. CONCLUSION: The diagnosis of BC should be considered in cases of multilocular cystic lesions in the liver, particularly in instances of recurrent cysts. Imaging aids in both positive and differential diagnoses. Complete resection is the recommended treatment for any suspected BC.

Mucinous non neoplastic cyst of the pancreas: a case report.

The aim of this study was to introduce a patient with mucinous nonneoplastic cyst (MNNC) at an unusual age. MNNCs of the pancreas are uncommon primary tumors, which affect middle-aged women in their fifth decade of life and have significant malignant potential. Therefore, it is important to accurately diagnose and remove them. This case is a 28-year-old woman patient who presented with a pain in the right and upper abdomen from 3 months ago and worsened at night. A cystic lesion was observed near the upper bridge of the left kidney in abdominal ultrasonography. Contrast-enhanced abdominal computed tomography (CT) scan showed a cystic lesion in the trunk and umbilical cord of the pancreas. The patient underwent surgery and the mass was removed and the MNNC was diagnosed.

Mucinous cystic neoplasm of the liver with polypoid nodule prolapsing into the bile duct: a case report and review of literature.

BACKGROUND: Mucinous cystic neoplasm of the liver (MCN-L) is a rare cystic tumor as defined by the 2010 World Health Organization classification. MCN-L usually does not communicate with or grow into the bile duct. Herein, we present a rare case of MCN-L with a polypoid nodule protruding into the bile duct. CASE PRESENTATION: A 69-year-old woman was referred to our hospital for elevated serum liver enzyme levels and obstructive jaundice. The patient also complained of abdominal pain in the right hypochondriac region. Abdominal ultrasonography showed a cystic lesion in segment 4 (S4) of the liver. Computed tomography revealed a 4-cm multilocular cystic lesion with a thick wall and multiple septal formations, showing a cyst-in-cyst appearance in S4. Endoscopic retrograde cholangiography showed a contrast defect between the left hepatic duct and the common bile duct, which was suspected to be a nodular lesion in the bile duct. Bile cytology and biopsy of the nodular lesion showed no malignant findings. Based on these findings, the differential diagnosis in this patient included intraductal papillary neoplasm of the bile duct and MCN-L, which had malignant potential. The patient underwent left hemihepatectomy, including caudate lobe excision with bile duct resection and right hepatocholangiojejunostomy. Macroscopic findings showed a 40 × 29 mm multilocular cystic lesion with a polypoid nodule that protruded into the left intrahepatic bile duct. As an ovarian-like stroma was observed in both cystic and polypoid lesions microscopically, the histopathological diagnosis was MCN-L. The postoperative course was uneventful, and the patient was discharged 24 days after surgery. The patient is currently alive without recurrence 22 months after the surgery. CONCLUSION: Although MCN-L rarely communicates with the bile duct, it is necessary to consider that MCN-L could grow into the bile duct, occasionally causing obstructive jaundice.

Pedunculated mucinous cystic neoplasm of the liver: a case report.

In 2010, the World Health Organization classified mucin-producing bile duct tumors of the liver into two distinct entities; mucinous cystic neoplasm of the liver (MCN-L) and intraductal papillary mucinous neoplasm of the bile duct. We present the case of a patient with MCN-L having a uniquely pedunculated shape. A 32-year-old woman was referred to our institution with a diagnosis of biliary cystic neoplasm. She had undergone left salpingo-oophorectomy for ovarian cancer 15 years ago. Imaging studies showed an 8 cm-sized well defined, multiloculated cystic lesion suggesting a mucinous cystic neoplasm. The cystic mass was pedunculated at the liver capsule and pathologically diagnosed as MCN-L. The mass was resected with partial hepatectomy. The patient recovered uneventfully. She was discharged 7 days postoperatively. The patient has been doing well for 6 months after the operation. The patient will be followed up annually because of the favorable postresection prognosis of MCN-L.

A mucinous cystic neoplasm of the pancreas containing an undifferentiated carcinoma component and harboring the NRAS driver mutation.

A woman in her 20s visited our hospital with a chief complaint of abdominal distension and back pain. She was diagnosed with a cystic tumor (diameter 16 cm) in the tail of the pancreas and underwent a combined distal pancreatectomy, splenectomy, and left adrenalectomy. Histopathologically, the tumor presented as a mucinous cystic neoplasm with an undifferentiated carcinoma component of the pancreas. In addition, the cells demonstrated a partial rhabdoid-like morphology. These findings were considered relatively typical for a mucinous cystic neoplasm in the tail of the pancreas in a young woman. However, NRAS mutation, which is rare in pancreatic tumors, was detected.

Mesenteric and Retroperitoneal Mucinous Cystic Neoplasms: A Case Series.

Aims. Mucinous cystic neoplasms (MCNs) are cystic neoplasms with mucinous epithelium surrounded by ovarian-like stroma. Extraovarian MCN occurring in the liver and pancreas have been well characterized. However, only rare case reports of MCN arising outside of these locations have been reported. MCNs arising in unusual locations should enter the differential diagnosis of mucinous intra-abdominal tumors and must be distinguished from more common mimics. Therefore, we aimed to examine a series of MCNs of the retroperitoneum and mesentery to characterize the clinicopathologic features of this entity. Methods and results. Seven MCNs arising in the abdominal mesentery or retroperitoneum were retrospectively identified. A clinicopathologic, histologic, and immunohistochemical (keratin 7, keratin 19, keratin 20, calretinin, inhibin-α, steroidogenic factor-1 (SF-1), estrogen receptor (ER), progesterone receptor (PR), PAX8, CDX2, and CD10) analysis was performed. All 7 MCNs were from females with a median age of 41 years old and a median size of 8 cm. All cases demonstrated mucinous with or without concomitant non-mucinous epithelium overlying spindle cell ovarian-like stroma. Luteinized cells were noted. The epithelium was positive for keratin 7 and keratin 19 in all 7 cases, while the stroma expressed ER, PR, and SF-1 in all cases stained. Calretinin was focally positive in the stroma of 3 of 7 cases, while inhibin-α was focally expressed in 5 of 6 cases. Conclusions. These results highlight the clinicopathologic, histologic, and immunophenotypic similarities between MCNs of the mesentery, retroperitoneum, pancreas, and liver. Overlapping features suggest a common histogenesis for all MCNs, which could include periductal fetal mesenchyme, aberrant migration of primordial germ cells, or abnormal differentiation or metaplasia of the embryonic coelomic epithelium.

Molecular and Immunohistochemical Analysis of Mucinous Cystic Neoplasm of the Liver.

OBJECTIVES: Mucinous cystic neoplasm of the liver is characterized by neoplastic mucinous and/or biliary epithelium surrounded by ovarian-type stroma. Immunohistochemical studies have shown that the ovarian-type stroma expresses estrogen receptor, suggesting potential hormonal responsiveness. The molecular biology of mucinous cystic neoplasm of the liver remains poorly studied. METHODS: Transcriptome sequencing and immunohistochemistry were performed on a series of mucinous cystic neoplasms. RESULTS: Mucinous cystic neoplasm of the liver exhibited significantly increased RNA expression of ovarian stromal markers WT1, PR, and ER2 and sex cord stromal markers SF-1, inhibin-α, and calretinin compared with nonneoplastic liver. Immunohistochemistry confirmed the RNA-level data. Evidence for sex hormone biosynthesis was identified by significant overexpression of multiple estrogen biosynthetic enzymes. Expression of 17ß-hydroxysteroid dehydrogenase 1 was confirmed immunohistochemically. Pathway analysis also identified significant upregulation of the hedgehog and Wnt pathways and significant downregulation of T-helper 1 and T-helper 2 pathways. CONCLUSIONS: Mucinous cystic neoplasm of the liver recapitulates ovarian stroma at the morphologic, DNA, RNA, and protein levels. These data support the concept that this tumor likely arises from ectopic primitive gonadal tissue and/or stromal cells with capacity to transdifferentiate to ovarian cortical cells.

Mucinous cystic adenoma with inflammatory cell infiltration around the splenic artery mimicking pancreatic cancer: a case report.

A 45-year-old woman presented with upper abdominal and back pain. A cystic lesion in the pancreas and inflammation around the splenic artery were detected by computed tomography. Although imaging studies were difficult to exclude malignancy, pathological and cytological findings of a fine-needle aspiration showed no signs of malignancy. The patient was, therefore, followed-up for 3 months, during which time the cyst increased in size and developed a cyst-in-cyst structure. She was diagnosed with mucinous cyst neoplasm and underwent distal pancreatectomy. Histologically, the patient was diagnosed as low-grade mucinous cystic adenoma. Soft tissue shadows around the splenic artery were considered to indicate fibrosis and infiltration of inflammatory cells. After distal pancreatectomy, the patient has been uneventful with symptom resolution. This case highlights the potentially atypical presentation of mucinous cystic neoplasms with inflammatory cell infiltration around the splenic artery.

Mucinous Cystic Neoplasm of the Liver or Intraductal Papillary Mucinous Neoplasm of the Bile Duct? A Case Report and a Review of Literature.

Mucinous cystic neoplasm of the liver (MCN-L) and intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) are diagnoses that were classified by the World Health Organization in 2010 as mucin-producing bile duct tumors of the hepatobiliary system. The preoperative differential diagnosis between these two entities is difficult; the presence of a communication with the bile duct is usually considered as a typical sign of IPMN-B. However, the presence of an ovarian-like stroma (OLS) has been established to define the diagnosis of MCN-L. We present the case of a 33-year-old woman with a rapid progression of a cystic tumor of the liver. In 2 years, the lesion increased from 27 to 64 mm and a dilation of the left hepatic duct appeared. Percutaneous transhepatic drainage with a biopsy was performed. No malignant cells were found on biopsy. Because of the rapid progression of the cystic tumor and unclear malignant potential, left hemihepatectomy was performed. Even though tumor masses were present in the biliary duct, on the basis of the presence of OLS, histology finally confirmed MCN-L with intermediate-grade intraepithelial dysplasia to high-grade intraepithelial dysplasia. The patient is currently under oncologic follow-up with no signs of recurrence of the disease. We present a rare case where MCN-L caused a dilation of the left hepatic duct, a sign that is usually a characteristic of IPMN-B.

A case of mucinous cystic neoplasm of the gallbladder.

The patient was a 70-year-old woman in whom examination revealed a high level of carbohydrate antigen 19-9. Abdominal ultrasonography and computed tomography (CT) revealed a multilocular cystic lesion compressing the gallbladder. CT indicated the presence of a multilocular cystic tumor (67 × 68 × 72 mm) in contact with the right hepatic lobe. Intraoperative findings indicated that the cyst diameter was 8.5 × 6.0 cm, and the cyst was continuous with the gallbladder. The gallbladder was resected along with the cyst. The cyst was multilocular and originated from the cystic duct and gallbladder wall. The cyst wall contained cuboidal to columnar mucin-producing epithelial cells and ovarian-like stroma (OS). The final diagnosis was mucinous cystic neoplasm (MCN) of the gallbladder with low-grade dysplasia. In the 2010 WHO classification of tumors of the digestive system, MCN have been newly defined as a type of hepatobiliary tract epithelial neoplasms. MCN of the gallbladder with OS is extremely rare. Only three cases have been published in the literature. The presence of OS is necessary for diagnosis of MCN.

Laparoscopic resection of a hepatic mucinous cystic neoplasm: A case report.

INTRODUCTION: We aimed to present a case of hepatic mucinous cystic neoplasm (MCN-H) that was completely resected by laparoscopy. PRESENTATION OF CASE: A 47-year-old female exhibited mild elevation of serum liver enzyme levels. Abdominal computed tomography revealed a 45-mm multilocular cystic tumor in segment IV of the liver, along with intermittent border calcification and minimal wall thickness. Magnetic resonance imaging revealed fluid-to-fluid level in the cystic tumor, thereby increasing the suspicion of a mild hemorrhage. The patient underwent laparoscopic liver resection (LLR) with a diagnosis of suspected mucinous cystic neoplasm of the liver. The entire tumor was successfully resected with a laparoscopic approach. The resected specimen was a 4.2×3.3×2.2-cm cystic tumor. Histological findings revealed mucin-producing singular epithelium and ovarian-like stroma. The tumor was diagnosed as a MCN-H with no malignancy. DISCUSSION: This is the first report in which a MCN-H was completely resected by laparoscopy. MCN-H is rare and is observed in only<5% of liver cystic tumors. MCN-H has been reported to have the malignant potential. And complete resection might be a good treatment option. Along with technical development, LLR has been indicated for benign liver tumors to date. Benign liver tumors are commonly observed in young females. The smaller incisions of the laparoscopic approach might provide cosmetic advantages for patients. CONCLUSION: We presented the first case of a MCN-H completely resected by laparoscopy. Benign tumors and tumors with malignant potential might be good indications for a laparoscopic surgery.

Adult Multicystic Nephroma: Case Report and Review of the Literature.

Multicystic nephroma (MCN) is relatively a rare, nongenetic and benign lesion of the kidney which has a bimodal distribution. The congenital form is commonly seen under the age of 2. Adult-onset MCN is more often seen in women, especially in post menopausal females. The aetiopathogenesis of MCN is still unknown. Ovarian-like stroma of the tumour indicates the tumour originates from the mullerian tissue of the kidney. Hormonal theory is suggestive as the stroma of the tumour is positive for estrogen and progesteron. Preoperative diagnosis of MCN is difficult. Surgical approach is recommended both for the histological diagnosis and the treatment of the disease. We here present a 53-year-old female patient with MCN admitted to the urology clinic with left flank pain treated successfully with partial nephrectomy. No recurrence was noted after six months of clinical and radiological follow-up.

A case of mucinous cystic neoplasm of the liver: a case report.

A 71-year-old woman was referred to our institution for further investigation of epigastric pain. The patient had been detected to have a multilocular cyst in the medial segment of the liver measuring 69 mm in diameter at another hospital 2 years ago, and the diameter of the cyst had increased to 90 mm. Although the cyst had gradually increased in size, there was no evidence of mural nodules. As we were concerned about the malignant potential of the lesion, a left hepatic segmentectomy was performed. Pathologically, the cyst was lined by columnar and cuboidal epithelium with low-grade atypia. The epithelium covered an ovarian-like stroma, and the diagnosis was mucinous cystic neoplasm of the liver (MCN-L) with low-grade intraepithelial neoplasia. MCN-L is a rare disease and its characteristics are still poorly understood. MCN-L occurs at a lower frequency as compared to the counterpart of MCN of the pancreas, further investigations are necessary to clarify the biological malignancy of MCN-L.

Activated wnt signaling in stroma contributes to development of pancreatic mucinous cystic neoplasms.

BACKGROUND & AIMS: Pancreatic mucinous cystic neoplasm (MCN), a cystic tumor of the pancreas that develops most frequently in women, is a potential precursor to pancreatic ductal adenocarcinoma. MCNs develop primarily in the body and tail of the pancreas and are characterized by the presence of a mucinous epithelium and ovarian-like subepithelial stroma. We investigated the involvement of Wnt signaling in KRAS-mediated pancreatic tumorigenesis and development of MCN in mice, and Wnt activation in human MCN samples. METHODS: LSL-Kras(G12D), Ptf1a-cre mice were crossed with elastase-tva mice to allow for introduction of genes encoded by the replication-competent avian sarcoma-leukosis virus long-terminal repeat with splice acceptor viruses to pancreatic acinar cells and acinar cell progenitors, postnatally and sporadically. Repeat with splice acceptor viruses that expressed Wnt1 were delivered to the pancreatic epithelium of these mice; pancreatic lesions were analyzed by histopathology and immunohistochemical analyses. We analyzed levels of factors in Wnt signaling pathways in 19 MCN samples from patients. RESULTS: Expression of Wnt1 in the pancreatic acinar cells and acinar cell progenitors of female mice led to development of unilocular or multilocular epithelial cysts in the pancreas body and tail, similar to MCN. The cystic lesions resembled the estrogen receptor- and progesterone receptor-positive ovarian-like stroma of MCN, but lacked the typical mucinous epithelium. Activated Wnt signaling, based on nuclear localization of ß-catenin, was detected in the stroma but not cyst epithelium. Wnt signaling to ß-catenin was found to be activated in MCN samples from patients, within the ovarian-like stroma, consistent with the findings in mice. CONCLUSIONS: Based on studies of mice and pancreatic MCN samples from patients, the canonical Wnt signaling pathway becomes activated and promotes development of the ovarian-like stroma to contribute to formation of MCNs.

Clinicopathological features and prognosis of mucin-producing bile duct tumor and mucinous cystic tumor of the liver: a multi-institutional study by the Japan Biliary Association.

BACKGROUND: The aim of this study was to determine the clinicopathological features and surgical outcomes of mucinous cystic neoplasm of the liver (MCN) and mucin-producing intraductal papillary neoplasm of the intrahepatic bile duct (M-IPNB). METHODS: We performed a multi-institutional, retrospective study of patients with MCN or M-IPNB pathologically defined by the presence or absence of an ovarian-like stroma. RESULTS: The M-IPNB and MCN were diagnosed in 119 and nine patients, respectively. MCN was observed in female patients, while M-IPNB produced symptoms of cholangitis. M-IPNBs were classed as low or intermediate grade in 53 cases, high grade in 23 and invasive carcinoma in 43. Fifty-one of the M-IPNBs were the pancreatobiliary type (PT), 33 were the intestinal type (IT), 23 were the oncocytic type (OT), and 12 were the gastric type (GT). The 1-, 5- and 10-year survival rates for the 105 patients with M-IPNB were 96%, 84% and 81%, respectively, while the 5-year survival rate for patients with MCN was 100%. OT and GT M-IPNB had better 10-year survival rates than PT and IT M-IPNB. CONCLUSIONS: Although MCN has different features from M-IPNB, both diseases have a good prognosis after resection. The cellular type of M-IPNB appears to predict outcome.

Mucinous cystadenoma of the liver with ovarian-like stroma: the need for complete resection.

Cystadenoma of the liver is a rare neoplasm. Although many cystadenomas are asymptomatic, symptoms can include abdominal pain, postprandial epigastric discomfort, and nausea. Dramatic changes in hepatic imaging techniques have been helpful for diagnosing cystic lesions of the liver, such as simple cyst, hydatid cyst, cystadenoma, cystadenocarcinoma, and metastatic neuroendocrine tumors. However, it remains difficult to differentiate cystadenoma from cystadenocarcinoma for multiseptated cystic hepatic lesions with papillary projection on computed tomography (CT) and magnetic resonance imaging (MRI). Here we report the case of a 47-year-old woman with several months of postprandial discomfort and abdominal fullness. CT and MRI revealed multiseptated cystic lesions with papillary excrescences. A left hemihepatectomy was performed. Histology showed a benign mucinous cystic tumor with ovarian-like stroma.

Mucinous cystadenoma of the liver with ovarian-like stroma: the need for complete resection / 대한외과학회지

Cystadenoma of the liver is a rare neoplasm. Although many cystadenomas are asymptomatic, symptoms can include abdominal pain, postprandial epigastric discomfort, and nausea. Dramatic changes in hepatic imaging techniques have been helpful for diagnosing cystic lesions of the liver, such as simple cyst, hydatid cyst, cystadenoma, cystadenocarcinoma, and metastatic neuroendocrine tumors. However, it remains difficult to differentiate cystadenoma from cystadenocarcinoma for multiseptated cystic hepatic lesions with papillary projection on computed tomography (CT) and magnetic resonance imaging (MRI). Here we report the case of a 47-year-old woman with several months of postprandial discomfort and abdominal fullness. CT and MRI revealed multiseptated cystic lesions with papillary excrescences. A left hemihepatectomy was performed. Histology showed a benign mucinous cystic tumor with ovarian-like stroma.