The "Duct-Ectatic" Variant of Mucinous Cystic Neoplasm of the Pancreas: Clinical and Radiologic Studies of Seven Cases

Seven cases of the "duct-ectatic" variant of mucinous cystic neoplasm of the pancreas are presented. This new entity, the duct-ectatic variant of mucinous cystic neoplasm of the pancreas, probably masquerades as pancreatic pseudocyst clinically and radiologically. However, it has the characteristic pathologic findings of the classic "megacystic" type of mucinous cystic neoplasm. If there are cysts visualized on pancreaticogram which are in a diffusely duct-ectatic configuration and are in communication with the main pancreatic duct, the duct-ectatic variant of mucinous cystic neoplasm should be strongly suspected rather than the pancreatic pseudocyst.     

Serous cystic neoplasm of the pancreas-indications for surgery

BACKGROUND/AIMS: Serous cystic neoplasm is a rare pancreatic tumor. Almost all of these tumors are benign and only 9 cases of serous cystadenocarcinoma have been reported. Although serous cystic neoplasm is typically a microcystic lesion, there is a wide range of cyst sizes from micro to macro and even unilocular cysts have been reported. Thus, the diagnosis is difficult and indications for surgery are controversial. We aimed to elucidate the clinicopathological and imaging features of serous cystic neoplasm of the pancreas. METHODOLOGY: We investigated 15 cases of resected and 6 cases of nonresected cases of serous cystic neoplasm, evaluating the symptoms, imaging findings, preoperative diagnosis, macroscopic morphology, microscopic findings, and results of follow-up. RESULTS: Imaging diagnosis of serous cystic neoplasm was not easy, because not so many tumors had the typical microcystic pattern. Most of the resected serous cystic neoplasms were non-microcystic or were small tumors, which could not be precisely evaluated. CONCLUSIONS: Small serous cystic neoplasms, which can be diagnosed by imaging, do not need to be resected because serous cystadenocarcinoma is rare. Tumors of the pancreas that cannot be confirmed to be serous cystic neoplasm should be resected because of the possibility of pancreatic cancer, mucinous cystadenocarcinoma, or mucinous cystadenoma with malignant potential.     

Primary retroperitoneal mucinous cystadenoma:A case report

Primary retroperitoneal mucinous cystic tumors are extremely rare.These tumors can be classified as a primary retroperitoneal mucinous cystadenoma with or without borderline malignancy or primary retroperitoneal mucinous cystadenocarcinoma.The most common of these is primary retroperitoneal mucinous cystadenoma,which almost always occurs in female patients;only ten cases have been reported in males.The most common clinical findings for this tumor type include nonspecific abdominal pain and a palpable abdominal mass.A definitive diagnosis is usually obtained from histopathology after surgical excision.Here,we report the case of a 60-year-old female patient who complained of abdominal pain that had been present for 3 mo and presented with a palpable abdominal mass.Multidetector computed tomography scanning revealed a large,unilocular cystic mass in the left retroperitoneal space.Surgical intervention was performed and the tumor was completely removed.Histopathologic examination confirmed that the tumor was a primary retroperitoneal mucinous cystadenoma.Two years after surgery,the patient remains disease free.     

Benign inflammatory pancreatic mucinous cystadenomas mimicking locally advanced cystadenocarcinomas: Presentation of 3 cases

We report 3 cases of benign mucinous cystadenoma of the pancreas mimicking, both clinically and on imaging findings, locally advanced cystadenocarcinoma spreading to neighbouring organs (stomach, splenic and mesenteric vessels, and diaphragm). Surgical resection was performed in light of the suspicion of invasive carcinoma in all 3 cases. Histological examination of the resected specimens showed entirely benign mucinous cystadenomas associated with marked peri-tumoural inflammation that accounted for the pre-operative misdiagnoses. All 3 patients are alive 40, 47 and 54 months after surgery without evidence of tumour relapse. These cases indicate that surgery must be considered in patients with cystic tumour of the pancreas suggesting locally advanced cystadenocarcinoma, even when pre-operative imaging findings suggest tumour extension into neighbouring organs.     

Huge mucinous cystadenoma of the pancreas developing during pregnancy: a case report

A 33-year-old woman, gravida 2, para 1, was found to have a huge intra-abdominal cyst (2619 ml) in the 15th week. The cyst increased in volume to 3660 ml during the next 31 days. A tentative diagnosis of ovarian or pancreatic mucinous cystadenoma was made. Since the cyst was considered to cause fetal intra-uterine growth restriction, the patient underwent surgery in the 23rd week of pregnancy. The cyst was found to originate from the pancreas, and distal pancreatectomy with splenectomy was performed. The specimen weighed 5500 gm, which is the largest so far reported. Her postoperative course was uneventful, and she had a healthy full-term infant. Histological diagnosis was a benign mucinous cystadenoma. Up to the present, there have been three reported cases of pancreatic mucinous cystadenoma, including our case, and two cases of pancreatic mucinous cystadenocarcinoma, in association with pregnancy. Our case is the third reported of successful resection of the tumor during pregnancy resulting in a healthy infant.     

Mucinous cystadenocarcinoma of the pancreas during pregnancy

The occurrence of a mucinous cystadenocarcinoma of the pancreas during pregnancy is extremely rare, reported in only twice in the English literature. We describe a 34-year-old patient with this rare condition who was successfully managed by resection of the tumor with distal pancreatectomy and splenectomy. The operation was performed at 15 weeks of gestation, and the patient went on to deliver a healthy infant at 39 weeks. To our knowledge, this is the first report of successful resection of a cystadenocarcinoma of the pancreas during pregnancy. The specimen weighed 2500 g and measured 20 cm across, making it the largest reported mucinous cystadenocarcinoma of the pancreas encountered during pregnancy to date.     

Risk of malignancy in serous cystic neoplasms of the pancreas

BACKGROUND: In contrast to mucinous cystic neoplasms of the pancreas, which are known to have considerable malignant potential, the serous variant is generally thought to be benign. There are, however, several reports of malignancy in serous cystic neoplasms of the pancreas. AIMS: To assess the risk of malignancy of serous cystic tumors of the pancreas and to investigate specific clinical and histological features. METHODS: Clinical and pathological characteristics of benign and malignant serous cystic neoplasms of the pancreas were investigated by a review of the literature and documented by a case of a serous cystadenocarcinoma and immunohistochemical analysis of a series of serous cystadenomas. Reviewing the literature prevalence, age and sex distribution of serous cystic neoplasms were analyzed. RESULTS: The prevalence of cancer among serous cystic neoplasms reported since 1989 was 3%. Serous cystadenoma of the pancreas present at an earlier age (61 years) than serous cystadenocarcinoma (66 years; p = 0.056) and are symptomatic in the majority of patients.Pathological examination of the primary tumor was not able to distinguish cystadenoma from cystadenocarcinoma in 38% of cases. In 25% the diagnosis of cancer was established only after growth of metachronous metastases. In the present case, nuclear atypia, papillary structures, proliferation marker Ki-67 and p53 protein were increased in the primary tumor and/or metachronous metastasis. CONCLUSION: Serous cystic neoplasms of the pancreas do have malignant potential with a risk of malignancy of 3% and should be surgically treated if the operative risk is acceptable. Routine analysis of genetic and proliferation markers may improve diagnosis of malignancy in these tumors.     

Immunohistochemical study of pancreatic cystadenocarcinoma.

Seven cases of pancreatic cystadenocarcinoma were studied clinicopathologically and immunohistochemically. Four patients died of the disease or recurrence with metastasis to the liver and peritoneum within 2 yr after surgery. The remaining three patients are well after surgery. To distinguish the cystoadenocarcinoma with excellent prognosis from that with poor prognosis, we performed immunohistochemical staining with monoclonal antibodies directed against tumor markers. Five specimens were strongly and diffusely reactive with tumor marker antibodies, whereas two were reactive with the apical portion of lining epithelial cells. With the findings of the immunohistochemical examination, the diagnosis of two patients could be revised from cystadenocarcinoma to premalignancy. Thus, immunohistochemical examination with tumor markers could correlate with the clinical outcome of the patients and is useful in distinguishing two distinct populations of mucinous cystic tumor.     

Mucinous cystic neoplasm of the pancreas associated with pregnancy: report of two cases

Pancreatic neoplasms during pregnancy are rare. We describe two cases of mucinous cystic neoplasm (MCN) associated with pregnancy. A 34-year-old woman in the 26th week of pregnancy was given a diagnosis of MCN measuring 19 cm in diameter. We decided to resect it post partum in consideration of the risk of abortion. She had a healthy infant in the 40th week of pregnancy and three months later, distal pancreatectomy was performed. Pathological analysis revealed a mucinous cystadenoma of the pancreas. The second case was a 36-year-old woman. She was given a diagnosis of MCN, measuring 16 cm in diameter, post partum and distal pancreatectomy was performed. Pathologically, the tumor was an invasive mucinous cystadenocarcinoma of the pancreas. In this report, we did an extensive literature review and discussed the management of MCN during pregnancy with special reference to the timing of the operation and the relationship between tumor growth and sex hormones.     

Simultaneous mucinous cystadenoma of ovary and mucinous cystadenocarcinoma of pancreas

Mucinous cystic tumors were discovered synchronously in the tail of the pancreas and in the right ovary of an adult female. Both tumors were amenable to surgical resection. The pancreatic tumor was a noninvasive mucinous cystadenocarcinoma and the ovarian tumor was a mucinous cystadenoma. We feel these tumors represent two primaries, an uncommon occurrence, and not a single primary tumor with metastasis.     

Simultaneous mucinous cystadenoma of ovary and mucinous cystadenocarcinoma of pancreas.

Mucinous cystic tumors were discovered synchronously in the tail of the pancreas and in the right ovary of an adult female. Both tumors were amenable to surgical resection. The pancreatic tumor was a noninvasive mucinous cystadenocarcinoma and the ovarian tumor was a mucinous cystadenoma. We feel these tumors represent two primaries, an uncommon occurrence, and not a single primary tumor with metastasis.     

Mucin-Producing Tumor of the Pancreas: A Unique Clinical Entity

We report our experience with nine patients with "mucin-producing tumor of the pancreas," in which abundant mucin secreted by the tumor cells played a major role in the characteristic alterations of the pancreatic duct system. Four of nine patients presented with pancreatitis. Ultrasound and computed tomography demonstrated a well-defined cystic mass and dilated main pancreatic duct. Endoscopic retrograde pancreatography showed ductectatic character, i.e., diffuse dilatation of main duct and/or cystic dilatation of the branch ducts with filling defects of mucin. Ultrasound proved to be a good screening test. However, the diagnosis was confirmed on endoscopic retrograde pancreatography. Nine of our cases had no peripancreatic invasion or metastasis, resulting in a good prognosis after pancreatectomy. Mucin-producing tumor of the pancreas is a unique clinical entity that should be distinguished from "common" pancreatic carcinomas, and a favorable prognosis can be expected after surgical operation.     

Obstructive Jaundice Caused by Mucinous Cystic Tumor of Gallbladder: A Case Report and Literature Review

Mucinous cystic tumor of the gallbladder is an extremely rare benign tumor, with potential for malignant degeneration. Mucinous cystic tumors of the cystic duct are divided into mucinous cystadenoma and mucinous cystadenocarcinoma. Currently, cystadenoma is generally considered to be a precancerous lesion of cystadenocarcinoma. At present, there are few cases reported worldwide, and there are no relevant guidelines for diagnosis and treatment of this disease. This article presents the collected clinical data of a patient with mucinous cystic tumor of the gallbladder who was admitted to the First Affiliated Hospital of Hunan Normal University, with the characteristics of the disease summarized in combination with a focused literature review.     

Hemolymphangioma: A rare differential diagnosis of cystic-solid or cystic tumors of the pancreas

We report a case of pancreatic hemolymphangioma. Hemolymphangioma is a malformation of both lymphatic vessels and blood vessels. The incidence of this disease in the pancreas is extremely rare. To the best of our knowledge, only seven cases have been reported worldwide (PubMed). A 39-year-old woman with a oneday history of abdominal pain was admitted to our hospital. There was no obvious precipitating factor. The preoperative examination, including ultrasonography and computed tomography, showed a cystic-solid tumor in the pancreas, and it was considered to be a mucinous cystadenoma or cystadenocarcinoma. Pancreaticbody-tail resection combined with splenectomy was performed. After the operation, the tumor was pathologically demonstrated to be a pancreatic hemolymphangioma. Although pancreatic hemolymphangioma is rare, we believe that it should be considered in the differential diagnosis of cystic-solid tumors of the pancreas, particularly when there is no sufficient evidence for diagnosing cystadenoma, cystadenocarcinoma or some other relatively common disease of the pancreas.     

Clinicopathological study on cystadenocarcinoma of the pancreas

Although rare, mucinous cystadenocarcinoma of the pancreas is an established pathological entity. However, its characteristic features on diagnostic imaging have not yet been defined. Based upon clinicopathological studies on 5 cases, two subgroups of mucinous cystadenocarcinoma of the pancreas are proposed: the endophytic and the exophytic types. The former consist of large well-demarcated cystic tumors with numerous inward-growing cauliflower-like excrescences in mucinous material, which were characteristically demonstrated either by CT or ultrasonography. Tumors of this subtype were visualized as hypervascular masses in the capillary phase of angiography. Areas of malignant tissue were well-demarcated without capsular invasion, and tumors were free from lymph node or distant metastasis. Though cystic, the exophytic subtype had negligible inward-growing papillary projections. Corresponding to their macroscopic features, these tumors were demonstrated as cystic lesions lacking papillary projections on ultrasonography or CT. No tumor stains could be demonstrated by angiography. Despite of their smaller size than the former subtype, cancerous growth penetrated through the cyst wall and metastasized to lymph nodes, causing poorer prognosis than in the former.     

Usefulness of selective endoscopic retrograde pancreatography (selective ERP) and endoscopic pancreatic biopsy (EPB) in the diagnosis of mucin-producing tumor of the pancreas]

Selective ERP and EPB were employed in the diagnosis of mucin-producing tumor (MPT) of the pancreas and their usefulness was evaluated. Thirty five cases of MPTs were subdivided into three subtypes, i.e., 1) main duct type, 2) branch duct type and 3) peripheral type (mucinous cystadenoma/cystadenocarcinoma). Selective ERP was proved to be useful in demonstrating precise and whole pancreatograms, especially in revealing multiple lesions, when compared with standard ERP. Selective ERP could demonstrate communication between cystic tumors of the peripheral type and the pancreatic duct in seven (88%) of eight cases of the peripheral type, which suggests high frequency of the communication in the peripheral type. For preoperative tissue diagnosis, EPB was shown to be a useful method to yield sufficient tissue materials for histopathological evaluation and may be used as an adjunction method to diagnose the intraductal extent of the tumorous lesion.     

Mixed serous cystadenoma with mucinous cystadenoma of the pancreas

We report a case of serous cystadenoma of the pancreas mixed with mucinous cystadenoma. A 65-year-old woman was admitted to our hospital for evaluation of a palpable, elastic, hard mass measuring 6 cm in diameter in the right upper quadrant of the abdomen. A diagnosis of mucinous cystadenocarcinoma of the pancreas was made, and pancreatoduodenectomy was performed. The tumor was composed of a dominant compartment of macroscopic cyst, and its thick wall was filled with numerous microscopic cysts. The light microscopy findings with hematoxylin and eosin staining, and by the periodic acid-Schiff reaction, were almost perfectly consistent with the characteristics of microcystic or glycogen-rich cystadenoma, but the apical portion of the cytoplasm of the neoplastic cells was stained with Alcian blue at pH 2.4 and by the mucicarmine method. Neoplastic cells containing epithelial acidic mucin are usually found in mucinous cystadenomas. No K-ras point mutations were detected at the sites where neoplastic cells were present, whether or not they contained epithelial acidic mucin. Pancreatic serous cystadenomas that include a mucinous-cystadenoma component are extremely rare, and the difference between serous and mucinous cystadenomas is not always distinct.     

Mucin-Hypersecreting Tumor of the Pancreas with Mucin Extrusion through an Enlarged Papilla

Patient records, imaging films, macroscopic and microscopic features, and clinical follow-up data of seven Japanese patients with a mucin-hypersecreting tumor of the pancreas were reviewed. The mucin-hypersecreting tumor was defined as an enlarged major or minor papilla with a dilated orifice and visible mucin oozing on endoscopy. The series consisted of six men and one woman ranging from 47 to 79 yr old. The excretion of mucin through a patulous orifice of the enlarged ampulla of Vater was seen in six patients and of the enlarged minor papilla in the other patient with pancreas divisum. The main pancreatic duct was dilated in all patients. Amorphous mucin was seen in the dilated duct of five patients, and papillary nodules were present in two patients. Computed tomography and/or ultrasonography showed dilatation of the main pancreatic duct with multilocular cysts in six patients and with a solid tumor in the other patient. The tumor was located diffusely in the main pancreatic duct in one patient, whereas it was confined to branches in the head (four patients), body (one patient), or tail (one patient) of the pancreas in the six other patients. Histopathologic diagnosis was a cyst lined by hyperplastic mucus-secreting epithelium in one patient, mucinous cystadenoma in two, and mucinous cystadenocarcinoma in two. Five patients underwent resection and have survived for 1-46 months after the operation. The authors would like to emphasize this endoscopic syndrome because about half of mucin-hypersecreting tumors are malignant with a favorable prognosis.     

Endoscopic ultrasound characteristics of mucinous cystic neoplasms of the pancreas

OBJECTIVE: Mucinous cystic neoplasms of the pancreas have a more favorable prognosis than ductal adenocarcinoma. Management of a subgroup, intraductal papillary-mucinous neoplasms, is controversial. Endoscopic ultrasound (EUS) with fine-needle aspiration biopsy may emerge as the imaging modality of choice. There are few studies describing the EUS features of these tumors. METHODS: A total of 35 consecutive cases of cystic tumors of the pancreas with an established pathological diagnosis were analyzed for characteristic EUS features. RESULTS: Mucinous cystadenocarcinomas (n = 14) were more likely to be characterized by hypoechoic cystic/solid mass or complex cyst and were frequently associated with a dilated main pancreatic duct. Benign mucinous duct ectasia (n = 6) were characterized by a dilated main pancreatic duct in conjunction with hyperechoic thickening of the duct wall. The two cases of intraductal mucinous hyperplasia additionally showed a hypoechoic mass. Intraductal papillary carcinoma (n = 11) had features in common with mucinous cystadenocarcinoma but also had echogenic foci in the mass and intraductal hyperechoic lesions. The two cases of microcystic cystadenoma showed either a mixed hypoechoic solid/cystic mass or a complex cyst without the additional features seen in mucinous cystadenocarcinoma. CONCLUSIONS: EUS features seem to exist that may help to differentiate cystic neoplasms from adenocarcinoma of the pancreas and, thus, to establish the preoperative diagnosis of cystic tumors of the pancreas.     

Clinicopathologic review of 41 cases of pancreatic mucinous cystic neoplasms]

BACKGROUND/AIMS: Intraductal papillary mucinous neoplasms (IPMN) and mucinous cystic neoplasms are included in mucin-producing pancreatic tumors. The reports about IPMN are not uncommon but those about the mucinous cystic neoplasms are relatively few. The aims of this study were to define the natural history of resected mucinous cystic neoplasms of the pancreas and to identify the findings which suggest malignancy. METHODS: The authors retrospectively evaluated the clinical outcomes of 41 patients with mucinous cystic neoplasms who were surgically resected at Asan Medical Center between 1995 and 2004. RESULTS: Women (n=33) were more frequently affected than men (n=8). Thirty three patients (80.6%) had adenoma, 1 (2.4%) borderline malignancy, 1 (2.4%) carcinoma in situ, and 6 (14.6%) invasive mucinous cystadenocarcinoma. The most frequent symptom was abdominal pain (39%). About half of the enrolled patients were asymptomatic. Unilocular type (79%) was more frequent than the multilocular type (21%) on gross morphology. The tumor size of invasive mucinous cystic neopolasms was larger than that of non-invasive mucinous cystic neoplalsms (p=0.01). Abdominal pain was more frequent in invasive mucinous cystic neoplasms (p=0.026). On gross morphology, mural nodules were detected in 4 of 6 patients with invasive mucinous cystic neoplasms. However, they were not detected in any patients with non-invasive mucinous cystic neoplasms. Recurrence developed in none of the 35 patients with non-invasive mucinous cystic neoplasms, however 2 of the 6 patients with invasive mucinous cystic neoplasms died within 5 years. CONCLUSIONS: Clinical predictors of invasive mucinous cystic neoplasms are suggested to be tumor size and abdominal pain. The prognosis of the non-invasive mucinous cystic neoplasms is excellent when curative resection is performed.