Microcystic Adenoma Research Articles

Propranolol reduces viability and induces apoptosis in hemangioblastoma cells from von Hippel-Lindau patients.

BackgroundVon Hippel-Lindau (VHL) disease is a rare oncological disease with an incidence of 1:36,000, and is characterized by the growth of different types of tumors: hemangioblastomas in the central nervous system (CNS) and retina, renal carcinoma, pheochromocytomas, pancreatic serous cystadenoma, and endolymphatic sac tumors. These tumors do not express VHL protein (pVHL). pVHL ubiquitinates hypoxia inducible factor (HIF) protein for degradation by the proteasome; in the absence of VHL, HIF translocates to the nucleus to activate the expression of its target genes. Targeting VHL-derived tumors with drugs that have reduced side effects is urgent to avoid repeat CNS surgeries. Recent reports have shown that propranolol, a β-blocker used for the treatment of hypertension and other cardiac and neurological diseases, is the best option for infantile hemangioma (IH). Propranolol could be an efficient treatment to control hemangioblastoma growth in VHL disease because of its antiangiogenic effects demonstrated in IH and the hypothetical impact on HIF levels.MethodsHeLa 9X (HRE) hypoxia responsive element cell line and primary hemangioblastoma-derived cells were subjected to propranolol treatment and cell viability and apoptosis were evaluated. HIF1-α and Hif-2α expression after propranolol treatment was analyzed by western blotting. Quantitative PCR was performed to study the mRNA expression of HIF target genes. Vascular endothelial growth factor (VEGF) was measured in culture supernatants by immunoassay.ResultsPropranolol downregulated HIF-dependent transcription in HeLa 9XHRE cells. Under hypoxic conditions, propranolol decreased the expression of HIF target genes in hemangioblastoma cells, which stopped proliferating and died following long-term treatment. These results suggests that propranolol treatment promoted reduced HIF protein expression and corresponding downregulation of HIF target genes, and inhibited cell proliferation in parallel with induction of cell death by apoptosis.ConclusionsOur results suggest that propranolol could reduce the growth of HIF-dependent tumors and may thus be a promising treatment to delay surgery in VHL patients.

Hepatoid carcinoma of the pancreas combined with serous cystadenoma: a case report and review of the literature.

Pancreatic hepatoid carcinoma (HC) is an extremely uncommon neoplasm of pancreas that resembles hepatocellular carcinoma (HCC). We report a case of incidentally detected pancreatic HC combined with a serous microcystic cystadenoma, in a 47-year-old man, while he was being evaluated for renal calculi. Contrast enhanced computed tomography (CECT) of abdomen revealed a lesion with mild heterogeneous enhancement in the tail of pancreas and another proximal lesion having moderate enhancement, and a calculus in the neck of gallbladder. Serum chromogranin, carcinoembryonic antigen (CEA) and CA 19-9 levels were within normal limits. He underwent laparoscopic distal pancreatectomy with splenectomy and cholecystectomy. Pathologically the distal tumor was encapsulated and characterized by eosinophilic cytoplasm, vesicular nucleus with prominent nucleolus and intranuclear eosinophilic inclusions. The cells were arranged in trabecular pattern separated by sinusoids. Canalicular and intercellular bile plugs were seen. On immunohistochemistry tumor cells were positive for hepatocyte specific antigen and weakly positive for alpha fetoprotein (AFP). The proximal tumor showed features of serous microcystic adenoma. Based on these findings, the case was diagnosed as hepatoid tumor of pancreas combined with serous microcystic cystadenoma. Post operative AFP was 1.75 IU/mL. The patient is on follow up for the last eight months and there is no evidence of recurrence.

Combination of Right Nephrectomy and Total Pancreaticoduodenectomy for von Hippel-Lindau Disease

Von Hippel-Lindau disease is an inherited syndrome of multiorgan neoplasia caused by a germline mutation in the von Hippel-Lindau gene and can include central nervous system tumors, renal cell carcinomas and benign pancreatic cystic tumors. We report the case of a 56-year-old patient who had a past history of cerebellar hemangioblastoma and presented with abdominal pain. Imaging revealed renal tumors and multiple pancreatic tumors which caused duodenal and pancreatic duct compression. The patient was treated with a combination of radical right nephrectomy, total pancreaticoduodenectomy and splenectomy. Pathology identified a multifocal unilateral clear cell renal carcinoma which interestingly coexisted with multiple large pancreatic serous microcystic adenomas with infiltration of the fibrous capsule. In past cases of von Hippel-Lindau disease, pancreatic adenomas with malignant transformation have not been reported. In our case, the infiltration of the fibrous capsule by parenchymal cells may indicate malignant transformation.

Liver and pancreas

Various inflammatory processes and atypically appearing benign masses can have an imaging appearance very similar to or in some cases indistinguishable from cancer. In the liver such processes include inflammatory pseudotumor, sclerosed hemangioma and focal hepatic steatosis. In the pancreas heterotopic splenule, focal autoimmune pancreatitis and solid serous cystadenoma may be difficult to distinguish from pancreatic adenocarcinoma or neuroendocrine tumor. Inflammatory Pseudotumor is a localized benign hepatic mass that consists of fibrous stroma and chronic inflammatory infiltrates including plasma cells, histiocytes and lymphocytes. Three subtypes are recognized: xanthogranuloma type (histiocyte predominance), plasma cell granuloma type and hyalinized sclerosing type. The pathogenesis is unclear, but it often is associated with chronic recurrent biliary infections. The most common CT/MR appearance of inflammatory pseudotumor is that of a peripherally enhancing mass with a central area of poor enhancement. Histopathologically, the peripheral enhancing portion of the mass corresponds to fibroblastic proliferation, whereas the central poorly enhancing area corresponds to chronic inflammatory infiltrates. Other appearances include a solid mass with progressive homogeneous enhancement (hyalinized sclerosing type) and a necrotic appearing mass with internal septations. Differential diagnosis includes abscess, metastasis, cholangiocarcinoma and hepatocellular carcinoma. Hemangioma, the most common noncystic hepatic mass, has well recognized imaging characteristics; however, a hemangioma that has undergone degeneration and fibrous replacement (sclerosed hemangioma) may have atypical imaging features that can mimic a malignant hepatic neoplasm, in particular cholangiocarcinoma or metastasis. The imaging appearance varies depending on the degree of sclerosis. The mass often has an irregular contour and may cause capsular retraction if located peripherally. It may show rim enhancement or nodular peripheral enhancement; however, in contradistinction to most hemangiomas the enhancement is static and does show the usual centripetal progression. On MR imaging the mass may lack the expected degree of T2 hyperintensity. If serial imaging is available, a sclerosed hemangioma may decrease in size over time, with loss of previous regions of enhancement. Intrapancreatic splenuleappears as a round enhancing mass within the tail of the pancreas. Because of its vascularity it can be mistaken for a pancreatic neuroendocrine tumor. One of the keys to diagnosis is recognition that the mass has enhancement, attenuation and/or signal intensity characteristics that parallel the spleen on all image acquisitions. The diagnosis can be confirmed with a technetium 99m heat-damaged red blood cell scan, which demonstrates radiotracer uptake within the mass. Mass-forming chronic pancreatitis, particularly autoimmune pancreatitis, frequently is misdiagnosed as pancreatic adenocarcinoma or a neuroendocrine tumor. Most commonly mass-forming chronic pancreatitis is isoattenuating/isointense during both the pancreatic and hepatic parenchymal phases of contrast enhancement. In contradistinction, pancreatic adenocarcinoma typically is hypoattenuating/hypointense during both enhancement phases; however, approximately 10% of pancreatic adenocarcinomas are isoattenuating/isointense during both phases. On MR cholangiopancreatography (MRCP) the pancreatic duct within the mass-forming pancreatitis may be visible but narrowed (duct-penetrating sign), whereas the duct within pancreatic carcinoma often is occluded. One study has shown the duct-penetrating sign to be 94% accurate in distinguishing the two entities. Elevation of serum IgG4 is the best serological marker for autoimmune pancreatitis (sensitivity 73-75%; specificity 93-95%); however, approximately 10% of patients with pancreatic cancer may have elevated IgG4. Pancreatic serous cystadenoma is a benign mass that consists of numerous tiny cysts separated by glandular tissue and fibrous stroma. On CT it appears as a well-circumscribed hypoattenuating mass with varying degrees of contrast enhancement, depending on the size of the cysts and the proportion of cystic to glandular tissue. In most cases it is not difficult to distinguish this multicystic lesion from a solid pancreatic neoplasm; however a small proportion of serous cystadenomas consist largely of glandular tissue and fibrous septa with only a small proportion of tiny cysts. Such lesions appear hypervascular and may mimic a pancreatic neuroendocrine tumor. Clues to the diagnosis include precontrast attenuation value within the range of fluid and the presence of small cystic areas with the enhancing mass. In addition, this diagnosis should be considered if the mass is found incidentally in an elderly individual or a patient with von Hippel Lindau disease.

Endoscopic ultrasound guided radiofrequency ablation, for pancreatic cystic neoplasms and neuroendocrine tumors.

To outline the feasibility, safety, adverse events and early results of endoscopic ultrasound (EUS)-radiofrequency ablation (RFA) in pancreatic neoplasms using a novel probe. This is a multi-center, pilot safety feasibility study. The intervention described was radiofrequency ablation (RF) which was applied with an innovative monopolar RF probe (1.2 mm Habib EUS-RFA catheter) placed through a 19 or 22 gauge fine needle aspiration (FNA) needle once FNA was performed in patients with a tumor in the head of the pancreas. The Habib™ EUS-RFA is a 1 Fr wire (0.33 mm, 0.013") with a working length of 190 cm, which can be inserted through the biopsy channel of an echoendoscope. RF power is applied to the electrode at the end of the wire to coagulate tissue in the liver and pancreas. Eight patients [median age of 65 (range 27-82) years; 7 female and 1 male] were recruited in a prospective multicenter trial. Six had a pancreatic cystic neoplasm (four a mucinous cyst, one had intraductal papillary mucinous neoplasm and one a microcystic adenoma) and two had a neuroendocrine tumors (NET) in the head of pancreas. The mean size of the cystic neoplasm and NET were 36.5 mm (SD ± 17.9 mm) and 27.5 mm (SD ± 17.7 mm) respectively. The EUS-RFA was successfully completed in all cases. Among the 6 patients with a cystic neoplasm, post procedure imaging in 3-6 mo showed complete resolution of the cysts in 2 cases, whilst in three more there was a 48.4% reduction [mean pre RF 38.8 mm (SD ± 21.7 mm) vs mean post RF 20 mm (SD ± 17.1 mm)] in size. In regards to the NET patients, there was a change in vascularity and central necrosis after EUS-RFA. No major complications were observed within 48 h of the procedure. Two patients had mild abdominal pain that resolved within 3 d. EUS-RFA of pancreatic neoplasms with a novel monopolar RF probe was well tolerated in all cases. Our preliminary data suggest that the procedure is straightforward and safe. The response ranged from complete resolution to a 50% reduction in size.

A novel approach to the diagnosis of pancreatic serous cystadenoma: needle-based confocal laser endomicroscopy

The differential diagnosis of solitary pancreatic cystic lesions is frequently difficult. Needle-based confocal laser endomicroscopy (nCLE) performed during endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) is a new technology enabling real-time imaging of the internal structure of such cysts. The aim of this pilot study was to identify and validate new diagnostic criteria on nCLE for pancreatic cystic lesions. A total of 31 patients with a solitary pancreatic cystic lesion of unknown diagnosis were prospectively included at three centers. EUS-FNA was combined with nCLE. The final diagnosis was based on either a stringent gold standard (surgical specimen and/or positive cytopathology) or a committee consensus. Six nonblinded investigators reviewed nCLE sequences from patients with the most stringent final diagnosis, and identified a single feature that was only present in serous cystadenoma (SCA). The findings were correlated with the pathology of archived specimens. After a training session, four blinded independent observers reviewed a separate independent video set, and the yield and interobserver agreement for the criterion were assessed. A superficial vascular network pattern visualized on nCLE was identified as the criterion. It corresponded on pathological specimen to a dense and subepithelial capillary vascularization only seen in SCA. The accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of this sign for the diagnosis of SCA were 87 %, 69 %, 100 %, 100 %, and 82 %, respectively. Interobserver agreement was substantial (κ = 0.77). This new nCLE criterion seems highly specific for the diagnosis of SCA. The visualization of this criterion could have a direct impact on the management of patients by avoiding unnecessary surgery or follow-up.Clinicaltrials.gov NCT01563133.

The limitations of endoscopic ultrasound-guided fine needle aspiration cytology in the diagnosis of pancreatic serous cystadenoma: a brief case report.

Endoscopic ultrasound (EUS) fine needle aspiration (FNA) biopsy is commonly used in the diagnosis of pancreatic tumors. Although adenocarcinoma is the most commonly diagnosed primary pancreatic neoplasm, other neoplasms can also arise in this organ. For accurate diagnosis, pathologists must be familiar with the cytomorphology associated with all types of pancreatic tumors. Serous cystadenoma (SCA) is a rare, benign neoplasm that originates in the centro-acinar epithelium of the pancreas. Excluding any symptomatic or diagnostic uncertainty, patients presenting with SCA need no further management. Here, we report a case of pancreatic SCA that was suspected to be adenocarcinoma in preoperative test using EUS-guided FNA.

A particular case of duodenal obstruction due to rare pancreatic serous cystadenoma

Objective: Pancreatic tumors are very rare in children, particularly serous cystadenoma. Although this tumor is not potentially malignant, all patients required accurate evaluation and attention should be paid to possible related complications.

Pancreatic serous cystadenoma – A case report

Microcystic serous cystadenoma is a benign tumor of pancreas, also termed as Clear cell or Glycogen rich adenoma. It occurs at any site in pancreas and composed of small cystic spaces lined by small cuboidal cells with clear cytoplasm, glycogen. There is an association with von Hippel Lindau syndrome in few cases. Clinical symptoms are variable. Excision is almost curative.We report a case of 70 year old male who presented to the OPD with vague complaints of local discomfort. Ultrasound showed a mass in the pancreas. Pancreatectomy was done and diagnosed as microcystic serous cystadenoma of pancreas on histopathology.

Pancreatic serous cystadenoma related mortality is almost nil

s / Pancreatolog S4 Aims: To evaluate the long-term outcome after surgical resection of sporadic, asymptomatic NF-pNET < 2 cm, stratifying the results by resection type and histopathologic features. Patients & methods: Patients resected between June 1997 and November 2008 at our institution (minimum follow-up of 60months) were retrieved from a prospectively maintained electronic database and analyzed retrospectively. Results: Study population consisted of 49 patients (27 females and 22 males), with amedian age of 59 years (range 38-61). 31 patients underwent parenchyma-sparing resections (15 enucleations, 15 middle pancreatectomies, 1 spleen-preserving distal pancreatectomies), while 18 patients underwent formal resections (8 pancreaticoduodenectomies, 10 distal pancreactomies with splenectomy). Mean lymph node yielding was 1.35 (0-9) in atypical resections and 19 (7-32) in formal resections (p1⁄40.0001). Lymph node metastases were found in only one patient undergoing pancreaticoduodenectomy. The median tumor size was 16 mm. 48 tumors were G1, one was G2, none was G3 (2010 WHO classification). The final pathologic report showed microscopic local invasion (perineural invasion, lymph vascular invasion or fat invasion) in 8 patients. At a median followup of 80 months (60-193), 4 patients died of other causes, 4 were lost to follow-up the remaining 37 patients did not develop recurrence and are alive and disease-free. Conclusion: Long-term outcome of surgically resected asymptomatic sporadic NF-pNET < 2 cm is excellent. Atypical resections with limited lymph node dissection may be oncologically adequate. Microscopic local invasion did not impact on the natural history of the disease.

794c Pancreatic Serous Cystadenoma Related Mortality Is Nil. Results of a Multinational Study Under the Auspices of the International Association of Pancreatology and the European Pancreatic Club

794c Pancreatic Serous Cystadenoma Related Mortality Is Nil. Results of a Multinational Study Under the Auspices of the International Association of Pancreatology and the European Pancreatic Club

Characterization of solid focal pancreatic lesions using endoscopic ultrasonography with real-time elastography

Objectives. The aim of this study was to evaluate the diagnostic potential of strain assessment in solid focal pancreatic lesions using real-time elastography in combination with endoscopic ultrasonography (EUS). Material and methods. Forty-eight solid focal pancreatic lesions in 39 patients were included prospectively over a 3-year period and studied by EUS with real-time elastography (EUS-RTE). Lesions previously described as cystic by CT were not included. Distribution patterns of tissue strain were assessed using strain ratio (SR) measurements, continuous visual analog scale (VAS), and a visual categorical score (VCS), based on color coding of relative strain. Final diagnosis was based on histopathology, fine-needle aspiration cytology, and/or follow-up for ≥6 months. Results. The 48 lesions included 11 adenocarcinomas, 7 malignant neuroendocrine tumors (NETs), 11 benign or indeterminate NETs, 8 focal pancreatic lesions, 2 microcystic adenomas, and 9 other benign lesions. Malignant lesions had significantly higher median SR (7.05 vs. 1.56) and VAS scores (93.0 vs. 63.5) than benign lesions. A receiver operation characteristic curve analysis showed sensitivity of 67% and specificity of 71%, when using SR = 4.4 as a cut-off for malignancy. The highest SR values were found in two benign microcystic adenomas. Conclusions. EUS-RTE with SR measurements and VAS evaluation demonstrated a significant strain difference between benign and malignant lesions. However, the variation within the entities was substantial and some benign lesions presented with low strain. Benign lesions were generally characterized by a strain similar to reference tissue, whereas malignant lesions were harder. The recorded strain pattern in individual lesions must be interpreted with caution.

A case of solid‐type serous cystadenoma mimicking neuroendocrine tumor of the pancreas

Pancreatic cystic neoplasms, including serous cystic neoplasm (SCN) and mucinous cystic neoplasm, constitute approximately only 1–2% of all pancreatic tumors. Nevertheless, a great proportion of pancreatic cystic neoplasms are now being detected due to the recent improvements in imaging modalities. SCN is currently categorized into five subtypes: serous microcystic adenomas, serous oligocystic ill-demarcated adenomas, solid-type serous cystadenomas or solid serous adenoma (SSCA), von Hippel–Lindau disease-associated cystic neoplasms and serous cystadenocarcinomas. Among them, SSCA is by far the rarest subtype. SCN is a basically benign entity, so watchful observation is recommended unless the patients appear to have symptoms or the tumor turns to have malignant potential. However, an accurate preoperative diagnosis of SSCA is difficult because of its rarity and its resemblance in image findings to neuroendocrine tumor (NET) of the pancreas. Here we report a case of SSCA which was preoperatively diagnosed as NET of the pancreas, with a review of the published literatures. CASE REPORT

Characterization of Pancreatic Serous Cystadenoma on Dual-Phase Multidetector Computed Tomography

Characterization of Pancreatic Serous Cystadenoma on Dual-Phase Multidetector Computed Tomography

Characterization of Pancreatic Serous Cystadenoma on Dual-Phase Multidetector Computed Tomography

The objective of the study was to characterize pancreatic serous cystadenomas on dual-phase multidetector computed tomography in a surgical series. This is a retrospective review of preoperative dual-phase multidetector computed tomographic scans from 68 patients with surgically resected and pathologically confirmed pancreatic serous cystadenomas. Pancreatic serous cystadenomas were most commonly found in the tail (39%). The mean (SD) axial dimension was 4.5 (2.7) cm. A total of 36% contained internal calcifications. Dilatation of the main pancreatic duct (14%) and pancreatic parenchymal atrophy (11%) were uncommon. The mean (SD) attenuation of components with the highest attenuation was 49.1 (35.0) Hounsfield units on the arterial phase and 48.5 (33.4) Hounsfield units on the portal venous phase. Only 20% of neoplasms demonstrated "classic" morphology, as defined by multiple thin nonenhancing septations, calcifications, as well as the absence of main pancreatic duct dilatation and vascular involvement. Only 20% of surgically resected serous cystadenomas fulfilled classic morphology. Attenuation was helpful in differentiating serous cystadenomas from insulinomas and other cystic pancreatic masses, but it was not helpful in differentiation from pancreatic adenocarcinomas. Morphologic features were more helpful in differentiating serous cystadenomas from malignant masses.

An IPMN Inside a Serous Cystadenoma: Is That Possible?

Abstract Objectives: Although rare, the pancreatic cystic neoplasms are detected with increased frequency lately. The treatment of these modalities however remains controversial. Methods: In this case reports a multifocal Intraductal Papillary Mucinous Neoplasm (IPMN) along with Pancreatic Serous Cystadenoma (PSC) were identified in the pancreatic specimen following a distal pancreatectomy. The results of the histopathology confirmed the existence of an IPMN inside a large PSC. Results: In our case, the patient with this extremely rare type of mixed cystic neoplasm was managed successfully with extensive distal pancreatectomy. Conclusions: The preoperative evaluation of the extent of the pancreatic involvement and the definition of the histologic type of the involved neoplasms before the pancreatic resection is challenging.

Unusual case of a giant serous oligocystic adenoma with communication to main pancreatic duct

Benign serous cystic tumors of the pancreas are rare and include microcystic serous adenoma, serous oligocystic adenoma (SOA), ill-demarcated adenoma, and macrocystic serous cystadenoma. Microcystic serous adenoma usually present are either unilocular cyst or single lesion containing fewer cysts of more than 2 cm in diameter. It is a rare tumor which usually varies from 210 cm in size and at times difficult to differentiate from mucinous cyst. With firm diagnosis small asymptomatic SOAs could be managed without surgery. We describe here a case of middle aged male with a giant serous oligocystic adenoma of 15 cm size arising from head of pancreas producing pressure symptoms. Endoscopic ultrasound and cyst fluid tumor markers were suggestive of serous cystadenoma, computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) scan were indicative of mucinous adenoma showing unilocular cyst with dilated common bile duct (CBD), main pancreatic duct (MPD) and communication of cyst with main pancreatic duct. A pancreaticoduodenectomy was done. Histopathology reported the lesion as serous oligocystic adenoma. We are presenting this case due to unusual features, a large unilocular cyst of more than 15 cm in size presented with waxing/waning of surgical obstructive jaundice and on MRCP there was a communication of cyst with MPD with cyst wall thickness of 1 cm (approx.).

Unique presentation of giant metastatic microcystic serous adenocarcinoma of the pancreas.

Tumors of the pancreas that contain substantial cystic components include mainly mucinous cystic neoplasm, intraductal papillary mucinous neoplasm, solid pseudopapillary tumor, and cystadenomas (which encompass microcystic, macrocystic/oligocystic, and rare solid serous adenomas). Microcystic adenoma of the pancreas is a tumor that is benign in nature. Malignant transformation in the tumor with metastases is rare and only about 26 cases have been reported so far. Here we present a giant microcystic adenoma of the pancreas, possibly the largest ever malignant type in this group ever reported in the literature with extensive metastases to the liver and causing extensive compression and encasement on surrounding structures.

Serous microcystic adenoma of the pancreas

A new case of serous microcystic adenoma in a 75-year-old man is presented. The tumor sized 55x45x55 mm was located in the body and the tail of the pancreas. It was well-circumscribed and spongy on cross-section, with eccentrically located fibrous scar. At microscopic examination, the lesion was composed of many small cystic spaces lined by one layer of flat or cuboidal cells occasionally with clear cytoplasm. A few papillary structures protruding to the lumina of cysts were also seen. Regardless benign nature of the tumor, the patient has died at early postoperative period due to unrelated conditions.

Magnetic resonance imaging of cystic pancreatic lesions in adults: an update in current diagnostic features and management

Magnetic resonance (MR) imaging has become a widespread diagnostic solving tool for the detection and characterization of a large range of pancreatic cystic lesions. Benign and malignant cystic lesions of the pancreas including serous microcystic adenoma, mucinous cystic tumor, intraductal papillary mucinous tumor, solid pseudopapillary tumor, and also the less common lesions such as cystic endocrine tumors, cystic metastases, and lymphangiomas have suggestive MR imaging presentation that allows them to be differentiated from each other. Knowledge of MR imaging findings of cystic pancreatic lesions is critical to help suggest the diagnosis and chose the best therapeutic approach. The purpose of this review is to discuss and illustrate MR imaging features that are helpful for pancreatic cystic lesion detection and characterization and to provide an update in current MR imaging diagnostic features and management.