Mixed Acinar-endocrine Carcinoma Research Articles

Carboxypeptidase A1 (CPA1) immunohistochemistry is highly sensitive and specific for acinar cell carcinoma (ACC) of the pancreas

Abstract Introduction/Objective Introduction: Carboxypeptidase A1 (CPA1) is a zinc metalloprotease which is produced in pancreatic acinar cells and plays a role in cleaving C-terminal branched-chain and aromatic amino acids from dietary proteins. This study assessed the utility of immunohistochemical CPA1 staining for diagnosing pancreatic acinar cell carcinoma. Methods/Case Report Methods: A total of 15,680 tumor samples from 132 different tumor types and subtypes as well as 8 samples each of 76 different normal tissue types were analyzed by immunohistochemistry in a tissue microarray format. Results (if a Case Study enter NA) Results: CPA1 was strongly expressed in acinar cells of all normal pancreas samples but not in any other normal tissues. CPA1 immunostaining was detected in 100% of 11 pancreatic acinar cell carcinomas and one mixed acinar endocrine carcinoma (MAEC), but absent in 449 pancreatic ductal adenocarcinomas, 75 adenocarcinomas of the ampulla of Vater, and 11,739 other evaluable cancers from 128 different tumor entities. A weak to moderate diffuse staining of epithelial and stromal cells of cancer tissues immediately adjacent to non-neoplastic pancreatic acinar cells often occurred and was considered to be caused by diffusion of the highly abundant CPA1 from normal acinar cells that may have suffered some autolytic cell damage. Conclusion Our data show that CPA1 is a highly sensitive and largely specific marker for normal and neoplastic pancreatic acinar cells. CPA1 immunohistochemistry greatly facilitates the otherwise often difficult diagnosis of pancreatic acinar cell carcinoma.

Carboxypeptidase A1 (CPA1) Immunohistochemistry Is Highly Sensitive and Specific for Acinar Cell Carcinoma (ACC) of the Pancreas.

Carboxypeptidase A1 (CPA1) is a zinc metalloprotease that is produced in pancreatic acinar cells and plays a role in cleaving C-terminal branched-chain and aromatic amino acids from dietary proteins. This study assessed the utility of immunohistochemical CPA1 staining for diagnosing pancreatic acinar cell carcinoma (ACC). A total of 12,274 tumor samples from 132 different tumor types and subtypes as well as 8 samples each of 76 different normal tissue types were interpretable by immunohistochemistry in a tissue microarray format. CPA1 was strongly expressed in acinar cells of all normal pancreas samples but not in any other normal tissues. CPA1 immunostaining was detected in 100% of 11 pancreatic ACCs and 1 mixed acinar endocrine carcinoma, but absent in 449 pancreatic ductal adenocarcinomas, 75 adenocarcinomas of the ampulla Vateri, and 11,739 other evaluable cancers from 128 different tumor entities. A weak to moderate diffuse staining of epithelial and stromal cells of cancer tissues immediately adjacent to non-neoplastic pancreatic acinar cells often occurred and was considered to be caused by the diffusion of the highly abundant CPA1 from normal acinar cells that may have suffered some autolytic cell damage. In conclusion, our data show that CPA1 is a highly sensitive and largely specific marker for normal and neoplastic pancreatic acinar cells. CPA1 immunohistochemistry greatly facilitates the otherwise often difficult diagnosis of pancreatic ACC.

Carboxypeptidase A1 (CPA1) immunohistochemistry is highly sensitive and specific for acinar cell carcinoma (ACC) of the pancreas

Carboxypeptidase A1 (CPA1) is a zinc metalloprotease which is produced in pancreatic acinar cells and plays a role in cleaving C-terminal branched-chain and aromatic amino acids from dietary proteins. This study assessed the utility of immunohistochemical CPA1 staining for diagnosing pancreatic acinar cell carcinoma. A total of 15,680 tumour samples from 132 different tumour types and subtypes as well as 8 samples each of 76 different normal tissue types were analyzed by immunohistochemistry in a tissue microarray format. CPA1 was strongly expressed in acinar cells of all normal pancreas samples but not in any other normal tissues. CPA1 immunostaining was detected in 100% of 11 pancreatic acinar cell carcinomas and one mixed acinar endocrine carcinoma (MAEC), but absent in 449 pancreatic ductal adenocarcinomas, 75 adenocarcinomas of the ampulla Vateri, and 11,739 other evaluable cancers from 128 different tumour entities. In conclusion, our data show that CPA1 is a highly sensitive and largely specific marker for normal and neoplastic pancreatic acinar cells. CPA1 immunohistochemistry greatly facilitates the otherwise often difficult diagnosis of pancreatic acinar cell carcinoma.

The Cytologic Features of Pancreatic Mixed Acinar-endocrine Carcinoma

The Cytologic Features of Pancreatic Mixed Acinar-endocrine Carcinoma

Treatment of a mixed acinar-endocrine carcinoma with uptake on 68Gallium-DOTATOC positron emission tomography-computed tomography: A case report

The case of a 35-year old female patient with a diagnosis of metastatic mixed acinar-endocrine carcinoma (MAEC) is investigated in the present study. The patient was believed to have a well-differentiated neuroendocrine tumor (NET) with a high Ki-67 index and uptake on 68Gallium-DOTATOC positron emission tomography-computed tomography for 9 years, and was treated accordingly. The patient had long lasting disease control by treatment with sunitinib, and a response was observed in numerous lesions with peptide receptor radionuclide therapy (PRRT). Following treatment for metastatic disease for >4 years, liver transplantation was performed, as an exception to normal recommendations, at the time of progression of a centrally located liver lesion inducing obstructive jaundice. Following transplantation, the diagnosis of a Grade 3 NET, as defined by the WHO 2010 classification, was challenged and changed to MAEC. MAEC is a rare type of tumor of the pancreas, exhibiting endocrine and acinar differentiation. It is difficult to diagnose, often being misidentified as acinar cell carcinoma or predominantly as neuroendocrine neoplasms. Immunohistochemical labelling provides the only evidence for the dual differentiation of neuroendocrine (synaptophysin and chromogranin) and acinar (lipase, trypsin and chymotrypsin) cell markers. Studies investigating MAECs with a clear histopathological diagnosis are scarce, in addition to evidence of disease behaviour and treatment options. It is generally agreed that surgery is the primary treatment in patients with resectable tumors. The responses to sunitinib and PRRT suggested that treatments considered or developed for NETs may be beneficial in MAEC cases.

Metastatic mixed acinar-endocrine carcinoma of the pancreas treated with a multidisciplinary approach: a case report

BackgroundPancreatic neoplasms are usually characterized by ductal, acinar, or endocrine differentiation. Mixed exocrine and endocrine pancreatic tumours are extremely rare. Here, we report a case of pancreatic mixed acinar-endocrine carcinoma (MAEC) with multiple synchronous liver metastases that were treated with surgery and transcatheter arterial chemoembolization (TACE) that later recurred in the stomach.Case presentationA 45-year-old female with severe anaemia was referred to our hospital. Computed tomography (CT) demonstrated a hypervascular tumour, 17 cm in diameter, that was in the tail of the pancreas. In addition, there were multiple hypervascular tumours in the liver. She underwent a distal pancreatectomy with splenectomy after the liver metastases were treated with TACE. Pathology confirmed that the pancreatic tumour was MAEC. After 4.5 years, a follow-up CT showed a hypervascular tumour at the upper part of the stomach. Gastric endoscopy showed a big tumefactive lesion with surface irregularities, gastric erosion, and multiple dilated vessels in the fornix and greater curvature of the stomach. She underwent a proximal gastrectomy and survived 7 years and 2 months after the start of the treatment.ConclusionsThis is the first report of a metastatic stomach tumour from pancreatic MAEC, which was successfully treated with a multidisciplinary approach. Additionally, we review the literature and discuss the treatment of MAEC.

Mixed acinar-endocrine carcinoma of the pancreas

Mixed acinar-endocrine carcinoma of the pancreas

術前にEUS-FNAを施行した膵尾部原発mixed acinar-endocrine carcinomaの1例

術前にEUS-FNAを施行した膵尾部原発mixed acinar-endocrine carcinomaの1例

Mixed acinar-endocrine carcinoma of the pancreas treated with S-1.

The case of a 63-year-old male with a large mass in the pancreatic tail and multiple liver metastases, diagnosed as acinar cell carcinoma of the pancreas with a few scattered endocrine cells by liver biopsy is presented. The S-1 chemotherapy was effective, and partial response was obtained with decreased levels of serum CA19.9 and NSE. Ten months after starting chemotherapy, the tumor began to grow accompanied by marked elevation of serum NSE levels (266ng/ml). The patient died of liver failure due to multiple liver metastasis 18months after the initiation of the S-1 chemotherapy. Histological findings at autopsy were acinar cell carcinoma with an endocrine component of more than 30%; the final diagnosis was mixed acinar-endocrine carcinoma of the pancreas. This pathological change and clinical course may imply that S-1 was effective against the acinar component but less effective against the neuroendocrine component caused by tumor differentiation.

Mixed Acinar-Endocrine Carcinoma of the Pancreas

The objective of this study was to characterize the novel clinical and pathological features of mixed acinar-endocrine carcinoma of the pancreas. This was a retrospective review of medical records and surgical pathology specimens of patients with a diagnosis of mixed acinar-endocrine carcinoma of the pancreas at Cedars-Sinai Medical Center between 2005 and 2011. Additional immunohistochemistry was performed on the specimens of some patients. Five patients were identified. The median age at presentation was 74 years (range, 59-89 years), and all patients were male. The presenting symptoms were all related to tumor mass effects. The median size of the tumor was 10 cm (range, 3.9-16 cm). Preoperative clinical diagnosis aided by fine-needle aspiration biopsy was incorrect in all 5 cases. Most tumors (3/5) exhibited predominantly endocrine differentiation without hormonal production. Only 10% to 30% of cells were truly amphicrine, whereas most were differentiated into either endocrine or acinar phenotype. The clinical behavior ranged from moderate to aggressive with postoperative survival from 2.5 months to more than 3 years. Four patients received neoadjuvant or adjuvant chemotherapy with variable responses. Mixed acinar-endocrine carcinoma of the pancreas appears to be not uncommon in men, may harbor predominantly endocrine component, is often misdiagnosed by cytology, and exhibits variable clinical behavior. Mixed acinar-endocrine carcinoma of the pancreas should be considered in older patients with sizable pancreatic mass and may warrant aggressive surgical resection and chemotherapy.

多発肝転移・脾静脈腫瘍栓を伴ったmixed acinar-endocrine carcinomaの1例

多発肝転移・脾静脈腫瘍栓を伴ったmixed acinar-endocrine carcinomaの1例

Mixed acinar-endocrine carcinoma of the pancreas with intraductal growth into the main pancreatic duct: Report of a case

The patient was a 75-year-old asymptomatic man, in whom a tumor mass in the pancreatic tail had been found 6 months earlier. Computed tomography revealed a mass 7 cm in diameter, and an enhancement with contrast medium was observed at the periphery and partially inside the mass, but not in most parts of the tumor. Endoscopic retrograde cholangiopancreatography showed a filling defect in the main pancreatic duct. A distal pancreatectomy was performed because of the possibility of a malignant tumor. The tumor consisted of a lobular invasive growth component and a component with intraductal growth into the main pancreatic duct, and histologically the tumor cells had solid acinar to partially trabecular/tubular patterns. Trypsin (an acinic cell marker) expression was widely observed, followed by the expression of chromogranin A (an endocrine cell marker) in about 30% of the tumor cells. The tumor was diagnosed as mixed acinar-endocrine carcinoma according to the WHO classification.

Anti-Cytokeratin CAM 5.2 (Becton Dickinson) is not synonymous with CK8/18 monoclonal antibody. Comment on “Pancreatic-type mixed acinar-endocrine carcinoma with alpha-fetoprotein production arising from the stomach: a report of an extremely rare case. Med Mol Morphol (2009) 42:167–174”

Anti-Cytokeratin CAM 5.2 (Becton Dickinson) is not synonymous with CK8/18 monoclonal antibody. Comment on “Pancreatic-type mixed acinar-endocrine carcinoma with alpha-fetoprotein production arising from the stomach: a report of an extremely rare case. Med Mol Morphol (2009) 42:167–174”

Imaging Findings in a Case of Mixed Acinar-Endocrine Carcinoma of the Pancreas

Mixed acinar-endocrine carcinoma (MAEC) of the pancreas is extremely uncommon. We report here a rare case of MAEC of the pancreas presenting as watery diarrhea. This is the first report in the English-language literature that describes the imaging findings of MAEC of the pancreas, including computed tomography (CT), magnetic resonance (MR) imaging, and MR cholangiopancreatography features.

Pancreatic-type mixed acinar-endocrine carcinoma with alpha-fetoprotein production arising from the stomach: a report of an extremely rare case

An extremely rare case of mixed acinar-endocrine carcinoma (MAEC) arising from the stomach in a 56-year-old Japanese woman is herein presented. An endoscopic examination and computed tomography showed a protruding gastric tumor and a large extragastric mass, respectively. Macroscopic observation on the surgical specimen revealed the extragastric cystic mass was continued to the intragastric tumor. Histologically, the intragastric tumor consisted of large or small solid nests with acinar appearance. The cancer cells had an ovoid nuclei and polygonal cytoplasm, which was frequently amphophilic. Immunohistochemical examination showed that the cancer cells were positive for chromogranin-A, synaptophysin, alpha-amylase, lipase, and alpha-fetoprotein (AFP) but were negative for CD56, insulin, and other hormones. Ultrastructurally, the cancer cells contained 500-nm electron-lucent zymogen granules and 230-nm electron-dense neuroendocrine granules. This tumor was finally diagnosed to be MAEC with AFP production of the stomach. Although no ectopic pancreas was found in the stomach, this tumor may originate from ectopic pancreas. As another theory, it is possible for this tumor to originate from the pluripotent stem cells in the stomach. A gastric MAEC with AFP production has not been reported previously.

Mixed acinar-endocrine carcinoma of the pancreas: a case report and review of the literature

Pancreatic tumors usually display either a ductal, an acinar or an endocrine differentiation. Mixed exocrine and endocrine pancreatic tumors are extremely rare. There have been a few reports of the rare entity of mixed acinar-endocrine carcinoma of the pancreas, where the endocrine cells represent more than 30% of the tumor. We herein describe a case of such a pancreatic tumor in an asymptomatic patient.A 74-year-old male patient with no evident clinical symptoms was referred for surgical resection of a large mass located on the pancreatic head, which was confirmed by an abdominal U/S, CT and MRI. FNA of the mass under endoscopic ultrasound guidance showed the cytology specimen to comprise of cells with morphological and immunohistochemical characteristics of endocrine pancreatic neoplasms. The patient underwent a modified Whipple's procedure and his post-operative course was uneventful. Pathological examination of the tumor revealed a mixed acinar-endocrine carcinoma of the pancreas.Mixed tumors of the pancreas are extremely rare and their clinical features and pathogenesis remain unclear. The endocrine component seems to influence their prognosis favorably.Therefore, aggressive surgical therapy remains the only well established line of treatment for these tumors. Further accumulation of clinical cases will help clarify the clinical course and the optimal therapy for these unusual tumors.

Mixed acinar-endocrine carcinoma of the pancreas: a case report and review of the literature.

Pancreatic tumors usually display either a ductal, an acinar or an endocrine differentiation. Mixed exocrine and endocrine pancreatic tumors are extremely rare. There have been a few reports of the rare entity of mixed acinar-endocrine carcinoma of the pancreas, where the endocrine cells represent more than 30% of the tumor. We herein describe a case of such a pancreatic tumor in an asymptomatic patient. A 74-year-old male patient with no evident clinical symptoms was referred for surgical resection of a large mass located on the pancreatic head, which was confirmed by an abdominal U/S, CT and MRI. FNA of the mass under endoscopic ultrasound guidance showed the cytology specimen to comprise of cells with morphological and immunohistochemical characteristics of endocrine pancreatic neoplasms. The patient underwent a modified Whipple's procedure and his post-operative course was uneventful. Pathological examination of the tumor revealed a mixed acinar-endocrine carcinoma of the pancreas. Mixed tumors of the pancreas are extremely rare and their clinical features and pathogenesis remain unclear. The endocrine component seems to influence their prognosis favorably.Therefore, aggressive surgical therapy remains the only well established line of treatment for these tumors. Further accumulation of clinical cases will help clarify the clinical course and the optimal therapy for these unusual tumors.

Mixed acinar-endocrine carcinoma of the pancreas: A case report and review of the literature

Mixed acinar-endocrine carcinoma of the pancreas: A case report and review of the literature

Endoscopic Ultrasound-Guided Fine-Needle Aspiration of a Mixed Acinar-Endocrine Carcinoma: A Case Report

Endoscopic Ultrasound-Guided Fine-Needle Aspiration of a Mixed Acinar–Endocrine Carcinoma: A Case Report

Pancreatic mixed acinar‐endocrine carcinoma in a dog

A 10.5-year-old crossbreed dog was presented with a history of hypoglycaemic episodes and elevated serum insulin concentration. A pancreatic mass was removed at surgery along with an enlarged draining lymph node. An unresectable hepatic nodule was also present. Immunohistochemistry confirmed the pancreatic and lymph node masses as functional mixed acinar-endocrine carcinoma, previously unreported in domestic species. Persistent hypoglycaemia and hyperinsulinaemia post-operatively was highly suggestive of the hepatic mass being a functional metastasis. The dog was managed on prednisolone and remained asymptomatic 9 months post-operatively. This tumour type has only been rarely reported in human patients and may highlight the need for more rigorous immunohistochemical staining of pancreatic masses in veterinary species to identify the prevalence of this tumour type.