Acinar cell carcinoma of the pancreas

Acinar cell carcinoma of the pancreas is a rare tumor which is defined as a carcinoma that exhibits pancreatic enzyme production by neoplastic cells. This review includes re-cent developments in our understanding of the epidemiology and pathogenesis of ACC, imaging and pathological diagnosis and ap-proaches to treatment with reference to the literature.     

Acinar cell carcinoma of the pancreas

Acinar cell carcinoma of the pancreas is a rare tumor for which the best chemotherapy regimen has not been clearly established. Here, we report on a female patient with an unusually long survival and a remarkable response to weekly paclitaxel. To our knowledge, this is the first time that paclitaxel has been associated with an objective response in this disease. The patient recurred after initial resection and failed multiple prior chemotherapeutic regimens. She received weekly paclitaxel at 80 mg/m² and responded after eight doses, maintaining controlled disease for a total of 4 mo. We present her case in detail and review the available literature regarding this rare type of tumor.     

Acinar cell cystadenocarcinoma of the pancreas

An unusual tumor of the pancreas occurred in a 42-year-old man who presented with a gradually enlarging abdominal mass and weight loss. The mass was a 2 kg/25 cm diameter encapsulated multicystic tumor closely attached to the body and tail of the pancreas. On light microscopy it showed a predominantly tubular architecture and ultrastructurally numerous zymogen granules could be demonstrated in the tumor cells. It is therefore presumed to be of acinar cell origin. This tumor has features similar to the so-called acinar cell cystadenocarcinoma described in 1981 by Cantrell et al. Sixteen months after resection of the primary tumor a solitary liver metastasis was removed at a second operation.     

Acinar cell carcinoma of the pancreas: new genetic and treatment insights into a rare malignancy

Background.

Acinar cell carcinoma (ACC) of the pancreas is a rare neoplasm, accounting for 1% of all pancreatic neoplasms. There remains a lack of data regarding the use of systemic therapy in this disease. We present a series of 40 consecutive cases of ACC of the pancreas treated at Memorial Sloan-Kettering Cancer Center, with an emphasis on evaluation of activity of new therapeutic agents.

Methods.

Patients reviewed at our institution from January 2000 through January 2011 were identified from an institutional database with prior institutional review board approval. Pathology was confirmed in all cases as ACC or a closely related entity.

Results.

Forty patients were identified; 29 were male (73%). The median age at diagnosis was 65 years (range, 16–87 years). The median overall survival (OS) time for patients with localized, resectable disease was 56.9 months and the OS time for patients with metastatic ACC (n = 18) was 19.6 months. Six patients with metastatic or recurrent ACC had a partial response to chemotherapy and five patients had stable disease for ≥6 months on systemic chemotherapy. Clinical observation was made of a patient with ACC and hereditary nonpolyposis colorectal cancer and a patient with ACC and a BRCA1 germline mutation.

Conclusions.

ACC is moderately chemoresponsive to agents that have activity in pancreatic adenocarcinoma and colorectal carcinoma. A potential association between germline mutations in DNA mismatch repair genes and ACC warrants further evaluation.     

Squamous cell carcinoma of the pancreas

Epidermoid cancer of the panceras is a rare variety of this malignancy. A case is presented. It has a characteristic angiographic appearance. Though in the past it has had a prognosis similar to the more common adenocarcinoma of the pancreas, a chemotherapeutic agent effective against epidermoid tumors at other sites has been effective in a case reported in the recent cancer treatment literature.     

Squamous cell carcinoma of the pancreas

Pancreatic malignancies can be subdivided into endocrine and non-endocrine processes. Of the non-endocrine tumours, ductal carcinoma is the most common, and the ductal carcinomas can be further subdivided into adenocarcinomas and squamous cell carcinomas. The adenocarcinomas constitute most of the non-endocrine pancreatic malignancies, and the treatment options for these, although limited in efficacy, are relatively well established. The squamous cell carcinoma pathology is a rare entity, and few reports of it are found in the literature. As a result, treatment options for squamous cell carcinoma of the pancreas are poorly understood. Here, we report the presentation of a 48-year-old woman with metastatic squamous cell carcinoma of the pancreas. The subsequent investigations, treatment, and outcome are described.     

Alterations in glycoproteins and lipids in azaserine-induced acinar cell carcinoma of rat pancreas

Glycoproteins and lipids of rat pancreatic acinar cell carcinomas maintained in nude mice and in cell culture, were analyzed. The tumor contained significantly elevated levels of glycoproteins when compared with their normal counterparts. SDS-PAGE of tumor glycoproteins revealed that there were increased amounts of small molecular weight glycoproteins and the tumor also contained a 51,000 dalton glycoprotein which was not detected in the pancreas, liver or the sera of the control animals. The tumor in nude mice and cancer cells in culture had decreased lecithins and triglycerides, and increased amounts of free fatty acids, and both free and esterified cholesterols. The results indicate that altered glycoprotein and lipid compositions represent some of the characteristic features of the acinar cell carcinoma.     

Nonfunctioning islet cell carcinoma of the pancreas

Opinion statement Islet cell cancers of the pancreas may follow an indolent course. Unlike adenocarcinoma of the pancreas, which is considered inoperable for cure in the presence of metastatic disease, islet cell carcinoma of the pancreas in selected cases warrants an aggressive approach toward resection. Significant palliation may be obtained from resection of primary tumors despite the presence of liver metastases. Furthermore, resection or ablation of limited, metastatic disease in the liver may be indicated. Therapeutic decisions must consider the patient’s symptoms, coexisting morbidities, and the extent of the disease.     

胰腺腺泡细胞癌的CT表现

背景与目的：胰腺腺泡细胞癌(acinar cell carcinoma of the pancreas,ACCP)是一种罕见的胰腺恶性肿瘤,相关的影像学报道较少。该研究旨在探讨ACCP的CT表现。方法：收集自2011年1月-2014年1月经手术病理证实为ACCP的9例患者资料,回顾性分析其CT征象。结果：在9例ACCP患者中,肿块最大径均值为52 mm,肿瘤边缘不清的6例(66.7%),外生型生长者有6例(66.7%),强化程度低于正常胰腺组织者8例(88.9%),强化不均者6例(66.7%),累及血管者7例(77.8%),淋巴结转移者5例(55.6%),无出现肝转移病例,仅1例出现胰管扩张。结论：较大体积的乏血供胰腺肿块,内部异质性明显,呈外生型生长而无显著胰管扩张时,提示ACCP的可能。     

Islet cell carcinoma: neuroendocrine tumors of the pancreas and periampullary region

Most patients who have islet cell tumors, except those who have insulinomas, present with locally advanced or metastatic disease. In contrast with patients who have adenocarcinoma of the pancreas, those who have islet cell carcinomas can achieve long-term survival even if their disease is advanced. Liver-directed therapies, somatostatin analogs, and interferon are not curative but can be used to relieve tumor-associated symptoms. Similarly, palliative chemotherapy has been used with limited success. Advances in our understanding of the molecular mechanisms underlying tumor progression have translated into intense interest in biologically based strategies to treat this disease.     

Small cell carcinoma of the pancreas and biliary tract

Four cases of anaplastic carcinoma of the pancreas or biliary tract were studied clinicopathologically and immunohistochemically. All four cases were intermediate cell type and contained a minimum amount of microscopic foci of differentiated glandular adenocarcinoma. Argyrophilic tumor cells were not seen in any of the four tumors. Immunohistochemically, no tumor was positive for hormonal products, but all tumors were positive for epithelial markers. These findings suggest that the anaplastic carcinoma are not derived from argyrophilic cells, but rather from adenocarcinomas which have the potential for anaplastic metaplasia. The long-term survival of one patient emphasized the importance of chemotherapy in the treatment of small cell carcinoma of the pancreas and biliary tract.     

Disrupting cholesterol esterification by bitter melon suppresses triple‐negative breast cancer cell growth

Triple negative breast cancer (TNBC) is aggressive with a worse prognosis. We have recently shown that bitter melon extract (BME) treatment was more effective in inhibition of TNBC tumor growth in mouse models as compared to ER positive breast tumor growth. Aberrant dysregulation of lipid metabolism is associated with breast cancer progression, however, anti‐cancer mechanism of BME linking lipid metabolism in breast cancer growth remains unexplored. Here, we observed that accumulation of esterified cholesterol was reduced in BME treated TNBC cell lines as compared to control cells. We next evaluated expression levels of acyl‐CoA: cholesterol acyltransferase 1 (ACAT‐1) in TNBC cells treated with BME. Our results demonstrated that BME treatment inhibited ACAT‐1 expression in TNBC cells. Subsequently, we found that sterol regulatory element‐binding proteins‐1 and ‐2, and FASN was significantly reduced in BME treated TNBC cell lines. Low‐density lipoprotein receptor was also downregulated in BME treated TNBC cells as compared to control cells. We further demonstrated that BME feeding reduced tumor growth in TNBC mammospheres implanted into NSG mice, and inhibits ACAT‐1 expression. To our knowledge, this is the first report demonstrating BME suppresses TNBC cell growth through ACAT‐1 inhibition, and have potential for additional therapeutic regimen against human breast cancer.     

Undifferentiated small cell carcinoma of the pancreas: a report of five cases

Of 485 pancreatic malignancies diagnosed at Hines Veterans Administration Hospital from 1951 to 1979, five cases were undifferentiated carcinoma proved on postmortem examination. The tumors are histologically akin to the more frequently encountered oat cell carcinoma of the lung. Their clinical manifestation and rapid, fatal course closely resemble that of the ductal adenocarcinoma of the pancreas. A possible nonislet cell derivation of the neoplasm is postulated.     

Pleomorphic giant cell carcinoma of the pancreas: A review of nine cases

Nine cases of pleomorphic giant cell carcinoma of the pancreas collected over a 26-year period are analyzed. The tumor presents a distinctive histology. The head of the pancreas appears to be the predominant site of the lesions in this study, and the cell origin is considered to be the ductal epithelium. Its clinical manifestation and rapid, fatal course of usually two months are almost indistinguishable from the more conventional pancreatic adenocarcinoma.     

Dimethyltriazenoimidazole carboxamide therapy of islet cell carcinoma of the pancreas

Four patients with metastatic glucagonoma and one patient with metastatic diarrheogenic islet cell carcinoma of the pancreas were treated with dimethyltriazenoimidazole carboxamide (DTIC), 250 mg/M2 daily for five days repeated every four weeks. All patients responded clinically and chemically in one or more ways by a reduction in plasma glucagon levels, improved glucose tolerance, decreased measureable tumor, weight gain, and resolution of necrolytic migratory erythema and diarrhea. This experience and other cases from the literature call for the investigation of DTIC as the initial therapy in metastatic islet cell carcinoma of the pancreas and as being of possible benefit in other tumors of neuroendocrine origin.     

Plasma carcinoembryonic antigen and acinar cell carcinoma of the pancreas

Seventeen patients with histologically proven pancreatic cancers were studied in order to clarify the relationship of histologic types to plasma carcinoembryonic antigen (CEA) values. Two cases with marked elevation of plasma CEA values having 6100 ng/ml and 2500 ng/ml, respectively, disclosed histologically acinar cell carcinoma and mixed acinar and ductal cell carcinoma, respectively. Despite of massive hepatic metastases, the other 15 cases with ductal cell carcinoma, including 3 cases with cystadenocarcinoma, adenoacanthoma, and undifferentiated pancreatic cancer, respectively, showed normal or very modest elevation of plasma CEA values. No correlation was obtained between plasma CEA values and several biochemical tests. Two patients with marked elevation of plasma CEA value revealed strong staining in the cancerous areas of the pancreas by using a peroxidase-antiperoxidase staining technique. These findings suggest that acinar cell carcinoma of the pancreas may contribute to increase the circulating plasma CEA value.     

Differentiation of muscarinic cholinergic receptors in acinar carcinoma of rat pancreas

Analysis of muscarinic cholinergic receptors in pancreatic acinar carcinoma of rat by measurement of N-[methyl-3H]scopolamine binding has revealed a single homogenous population of muscarinic receptors in the tumor. The plasmalemma density (approximately 25 receptors/micron 2 of cell membrane surface) and dissociation constant (approximately 0.4 nM) of muscarinic receptors in acinar carcinoma cells are identical to the density and affinity of muscarinic receptors in normal acinar cells of rat pancreas. Muscarinic receptors in the carcinoma are functionally linked to cholinergic stimulation of cellular Ca2+ release. An equivalent number of functional muscarinic receptors is present in poorly differentiated carcinoma cells which are deficient in zymogen granules and protein secretion, as compared to well-differentiated carcinoma cells which contain granules and secrete protein in response to cholinergic stimulation. These observations indicate that muscarinic cholinergic receptors are displayed in normal fashion on tumor membranes and are fully expressed in carcinoma cells regardless of their degree of secretory development. Expression of muscarinic cholinergic receptors is thus a stable phenotypic property of pancreatic acinar carcinoma cells. This suggests that muscarinic receptor maturation is an early event in the differentiation of pancreatic exocrine cells, preceding acquisition of secretory responsiveness to cholinergic stimulation.