Acinar Cell Carcinoma of the Pancreas in the United States: Prognostic Factors and Comparison to Ductal Adenocarcinoma

Introduction  Pancreatic acinar cell carcinoma (ACC) is a rare tumor with poorly defined prognosis. Objective  Our objective was to compare a large population of patients with ACC to pancreatic ductal cell adenocarcinoma (DCC) in order to determine distinguishing characteristics and to assess survival. Methods  Patients were identified from the National Cancer Database. Regression methods were used to identify differences between ACC and DCC and to identify predictors of survival for resected ACC. Eight hundred sixty-five patients with ACC were identified. Results  Median tumor size was 6.9 cm (vs. 4.6 cm DCC); 32.1% had nodal metastases (vs. 48.0% DCC); and 47% had high-grade tumors (vs. 37.3% DCC). Resection margins were R0 77.3%, R1 13.7%, and R2 9.0%. Patients with ACC were more likely to be male, white, and have larger tumor size, no nodal involvement, or pancreatic tail tumors. Stage-specific 5-year survival was significantly better for resected ACC vs. DCC Stage I: 52.4% vs. 28.4%, II: 40.2% vs. 9.8%, III: 22.8% vs. 6.8%, and IV: 17.2% vs. 2.8%. On multivariable analysis, age < 65, well-differentiated tumors, and negative resection margins were independent prognostic factors for ACC. Discussion  ACC carries a better prognosis than DCC. Aggressive surgical resection with negative margins is associated with long-term survival in these more favorable pancreatic cancers. This paper was presented at the Society for Surgery of the Alimentary Tract (SSAT) in San Diego, CA, USA, in May 2008. Grant Support: KYB is supported by the American College of Surgeons, Clinical Scholars in Residence program.     

Acinar Cell Carcinoma of the Pancreas: A Literature Review and Update

Pancreatic acinar cell carcinoma is a rare tumour, accounting for only about 1 % of all pancreatic tumours. The long-term survival for patients with acinar cell carcinoma is significantly better than the long-term survival of patients with pancreatic adenocarcinoma. As no large series of patients with acinar cell carcinomas exist, our understanding of this disease comes mainly from small case series and case reports. Aggressive surgical resection with negative margins is associated with long-term survival in these more favourable pancreatic cancers. There are no clear treatment guidelines for patients in whom complete surgical resection with curative intent is not possible. Acinar cell carcinomas are chemoresponsive to agents that have activity against pancreatic adenocarcinomas and colorectal carcinomas because of the shared genetic alterations between these cancers. The role of neoadjuvant or adjuvant chemoradiotherapy remains unproven. The aim of this article is to present current knowledge on acinar cell carcinoma and comprehensive review of available literature.     

Pancreatic Resection for Metastatic Renal Cell Carcinoma: Presentation,Treatment, and Outcome

Background: The diverse natural history of renal cell carcinoma (RCC) includes metastases to the pancreas, a very unusual site for distant spread of other cancers. Considering the relatively indolent behavior of some cases of metastatic RCC, pancreatic resection is offered to select patients.Methods: We reviewed the records of patients at three affiliated university hospital centers who had prior nephrectomy for RCC and subsequent pancreatic resection of metastases.Results: Fourteen patients—9 women and 5 men with a median age of 63.8 years—underwent a total of 15 pancreatic resections for metastatic RCC. Nine (64%) had solitary metastases. The median interval from nephrectomy to diagnosis of pancreatic metastases was 83 months. The median size of metastases was 4.6 cm. There was one perioperative death. Pancreatic recurrence occurred in five patients (36%), and one patient underwent repeat resection. At a median follow-up of 32 months, seven patients (50%) are alive without evidence of disease, and four patients (28%) are alive with recurrent disease.Conclusions: Resection of pancreatic metastases from RCC is associated with long-term survival and should be considered for patients in whom complete resection is possible.     

Acinar Cell Carcinoma of the Pancreas: An Institutional Series of Resected Patients and Review of the Current Literature

Introduction Acinar cell carcinoma (ACC) is a rare, malignant neoplasm with a generally poor prognosis. We report our institutional series of 14 patients with ACC to determine current guidelines for their evaluation and treatment. Materials and Methods The Johns Hopkins pathology prospective database was reviewed from 1988 to 2006 to identify patients with pancreatic neoplasms possessing features of acinar cell differentiation. Retrospective review and follow-up was performed for each patient. Results Fourteen patients with ACC were identified with a median age of 57 years. All patients presented with abdominal pain or discomfort with none showing evidence of lipase hypersecretion syndrome. Each patient underwent surgical resection, including nine pancreaticoduodenectomies and five distal pancreatectomies. Median tumor size was 3.9 cm with 12 patients found to have stage IIB disease or worse. Four patients underwent neoadjuvant chemoradiation. Eight of the fourteen patients developed recurrent disease. Overall median survival and disease-free survival were 33 and 25 months, respectively, as compared to a median survival of 18 months for pancreatic adenocarcinoma. Conclusion Acinar cell carcinomas are rare, aggressive neoplasms that are difficult to diagnose and treat. Operative resection represents the best first-line treatment. These lesions have a better prognosis than the more common pancreatic adenocarcinomas.     

Acinar Cell Carcinoma Arising from an Ectopic Pancreas

We herein report a rare case of ectopic pancreatic acinar cell carcinoma (ACC) which presented as a submucosal tumor of the pylorus. A 73-year-old man came to our hospital presenting with epigastralgia. Esophago-gastroduodenal endoscopy showed no mucosal lesions, but a submucosal tumor was observed around the pylorus. Abdominal computed tomography revealed two round masses. One was located in the pylorus, while the other was found between the portal vein and the inferior vena cava. An examination of a biopsy specimen was inconclusive. We diagnosed a gastrointestinal stromal tumor or malignant lymphoma preoperatively, and decided to perform an operation in order to confirm the diagnosis and select the optimal treatment. Intraoperatively, the mass in the pylorus invaded the pancreatic head, and the lymph node in the hepatoduodenal ligament was swollen. We performed a pancreaticoduodenectomy as a radical excision. The resected specimen showed the 7.6 × 4.9-cm size tumor to mainly originate from the pylorus. Histopathologically, the tumor was identified as pancreatic ACC with lymph node metastasis. The tumor cells were labeled by immunohistochemical staining for α1-antitrypsin. Because of the tumor location, we considered the tumor to have originated from the ectopic pancreatic tissue in the stomach. This is only the second case of ACC originating from an ectopic pancreas reported in the literature.     

Acinar cell cystadenoma of the pancreas in a 9-year-old boy

This report describes a rare benign cystic lesion of the pancreas known as acinar cell cystadenoma. There are 12 previously reported cases, the youngest patient from which was age 16. We report the youngest case to date, occurring in a 9-year-old boy. The pancreatic lesion was found incidentally and confirmed by laparoscopic biopsy. A pseudocyst formed, which was drained endoscopically. Given that the lesion was benign and the patient was asymptomatic, no further intervention was recommended. Complete resection would require a total pancreatectomy, with attendant morbidity, and review of the literature suggests a benign course. In asymptomatic cases, we recommend biopsy for histologic diagnosis, with close follow-up and without further surgical intervention.     

Resection of a Solitary Pancreatic Metastasis from Renal Cell Carcinoma with a Gallbladder Carcinoma: Report of a Case

Most metastatic pancreatic tumors are detected at an advanced stage and are not considered suitable for surgery; however, resection is sometimes indicated for a solitary pancreatic metastasis from renal cell carcinoma (RCC) and improves the prognosis. We report such a case, in which the hilar liver was resected with lymph node dissection and distal pancreatectomy. Histological examination revealed regional lymph node metastasis of gallbladder carcinoma (GBC), but all the surgical margins were free of cancer. Postoperative extra-beam radiation therapy was delivered to the hepatic portal lesion to prevent GBC recurrence. The patient remains disease-free 14 months after the completion of radiation therapy. Thus, if all affected areas can be resected, the prognosis associated with pancreatic metastasis from RCC may be favorable.     

Acinar cell carcinoma of the pancreas eroding the pylorus and duodenal bulb

A 74-year-old woman presented at the National Defense Medical College Hospital in April 2001 with a chief complaint of upper abdominal pain. She had been diagnosed as having adenocarcinoma on the basis of results of examination of a biopsy specimen taken from an ulcer of the duodenal bulb at a local hospital. On admission, she showed no jaundice, but a hard mass, about 10cm in diameter, was palpated in the right upper quadrant. Laboratory data showed high levels of serum carcinoembryonic antigen (CEA) and carbohydrate antigen (CA) 19-9. Abdominal computed tomography (CT) and angiography demonstrated a giant enhanced mass in a pattern of eccentric gradation extending to the pylorus, duodenal bulb, and pancreatic head. She underwent pancreatoduodenectomy with combined resection of the transverse colon. The histologic diagnosis was acinar cell carcinoma (ACC), originating in the pancreatic head and extending to the stomach, duodenum, and transverse colon, without any lymph node involvement. In most reported cases of ACC, the preoperative diagnosis was a pancreatic mass or endocrine tumor of the pancreas. The correct diagnosis in those cases was made by postoperative or postmortem pathological examination. If criteria for detecting the slight differences between ACC and endocrine tumors on some images were to be established, the diagnostic skill for ACC would improve dramatically.     

Acinar cell carcinomas with exophytic growth and intraductal pancreatic duct invasion: peculiar multislice computed tomographic picture

On an ultrasound and multislice computed tomography (MSCT), a 76-year-old woman was found to have a huge mass lesion in the pancreatic head. MSCT showed 8.4 cm well-enhancing exophytic tumor of the pancreatic head which also protruded into the duodenum. A tongue-like protrusion into the main pancreatic duct was depicted. Laboratory data showed elevated carbohydrate antigen 19-9. Whipple’s operation was performed. The pathological diagnosis was acinar cell carcinoma (ACC) originating in the pancreatic head and directly invading through the duodenal wall and the main pancreatic duct, without any lymph node involvement. A peculiar ACC with good enhancement, exophytic picture and tongue-like protrusion into the main pancreatic duct on MSCT is presented.     

Concomitant Duodenal and Pancreatic Metastases from Renal Cell Carcinoma: Report of a Case

The pancreas and duodenum are uncommon sites for metastasis from renal cell carcinoma. Pancreatic or small intestinal metastases mainly occur when there is widespread nodal and visceral involvement and evidence of metastatic disease elsewhere in the body. We describe herein the case of a 68-year-old man in whom metastases arising from renal cell carcinoma developed concomitantly in the duodenum and pancreas. The patient presented with duodenal bleeding; but as no other metastatic lesions were observed at the time of surgery, total pancreatectomy with duodenetomy was performed. We believe that metastases may easily develop in the duodenum and pancreas owing to the similar tissue characteristics. Received: November 22, 1999 / Accepted: September 26, 2000     

Solitary Pancreatic Metastasis from Renal Cell Carcinoma Concomitant with Early Gastric Cancer 17 Years After Nephrectomy: Report of a Case

The pancreas is an uncommon site for metastasis from renal cell carcinoma. We report the case of a 70-year-old man in whom a solitary pancreatic metastasis from renal cell carcinoma, found 17 years after nephrectomy, was successfully resected, combined with gastrectomy for early gastric cancer. We also discuss the relevant literature, including all the reports of pancreatic metastasis from renal cell carcinoma found in Medline. More than half the cases, like ours, were asymptomatic. A good prognosis can be expected once the pancreatic metastatic lesions are surgically excised, especially if it is a solitary metastasis. Therefore, surgical resection of pancreatic metastases is recommended to achieve the best chance of long-term survival. Special attention must be paid to the possibility of recurrence, even more than 10 years after nephrectomy for renal cell carcinoma, and imaging modalities should be part of the routine follow-up to detect metastases at an early stage.     

A Case of Prostate Carcinoma Metastasizing to Renal Cell Carcinoma

Metastasis of one cancer to another cancer is extremely rare. The most frequent metastisizing tumor is a king carcinoma, and the most common recipient tumor is a renal cell carcinoma. We report herein a case of prostate carcinoma metastasizing to a renal cell carcinoma, which has previously been reported only four times.     

Clinicopathologic Features and Treatment Trends of Pancreatic Neuroendocrine Tumors: Analysis of 9,821 Patients

The natural history of pancreatic neuroendocrine tumors (PNET) remains poorly defined. Our objectives were to examine the clinicopathologic features of PNETs, to assess treatment trends over time, and to identify factors associated with undergoing resection. From the National Cancer Data Base (1985–2004), 9,821 patients were identified with PNETs. Clinicopathologic features and treatment trends were examined. Multivariable logistic regression was used to assess factors associated with undergoing resection. Of 9,821 patients with PNETs, 85% were nonfunctional, 7.1% were functional, and 7.9% were carcinoid tumors. Of the 3,851 (39.0%) patients who underwent pancreatectomy, 449 (11.7%) received adjuvant chemotherapy, and 254 (6.6%) received adjuvant radiation. From 1985 to 2004, utilization of pancreatectomy increased from 39.4 to 44.3% (P < 0.0001). Patients were less likely to undergo resection if they were >55 years old, had tumors in the head of the pancreas, tumors ≥4 cm, or had distant metastases (P < 0.0001). Patients treated at NCCN/NCI, academic, or high-volume hospitals were more likely to undergo resection. There are disparities in the utilization of pancreatectomy for PNETs. As PNETs have a better prognosis than adenocarcinoma, concerns regarding the morbidity and mortality of pancreatic surgery and neoplasms should not preclude resection.     

Clear Cell Carcinoma and Clear Cell Odontogenic Carcinoma: a Comparative Clinicopathologic and Immunohistochemical Study

Clear cell carcinoma or hyalinizing clear cell carcinoma (CCC) and clear cell odontogenic carcinoma (CCOC) are rare, low-grade and typically indolent malignancies that can be diagnostically challenging. In this study the clinicopathologic, histologic, and immunohistochemical features of 17 CCCs and 12 CCOCs are examined. The differential diagnosis of clear cell malignancies in the head and neck is discussed. The relationship of CCCs and CCOCs to other clear cell tumors on the basis of their immunohistochemical staining patterns is postulated.     

TNF-α增强树突状细胞诱导的抗胰腺癌免疫应答的实验研究

目的探讨体外扩增树突状细胞（DC）的方法，体外研究DC介导的细胞毒性T淋巴细胞（CTL）对小鼠胰腺癌的特异性杀伤作用。方法联合应用重组鼠源性粒细胞-巨噬细胞集落刺激因子（GM—CSF）和IL-4体外诱导骨髓源性DC，与肿瘤细胞溶解物共培养制备DC疫苗；观察DC在负载抗原后体外诱导的主动特异性CTL对胰腺癌细胞的杀伤作用。结果用GM—CSF和IL-4成功地在体外扩增了骨髓源性DC。TNF—α可诱导骨髓源性DC成熟，使其表达的CD54、主要组织相溶性复合物-Ⅱ、CD86等表面分子明显升高，并增强自分泌IL-12和抗原的递呈能力，与对照组比较，差异有统计学意义（P〈0.05）。未负载肿瘤细胞抗原的DC所冲击的T淋巴细胞不能有效地识别特定的靶细胞并产生杀伤作用，DC通过捕获并递呈坏死肿瘤细胞抗原才能诱发显著的主动特异性CTL，体外对胰腺癌细胞的杀伤效果非常显著（P〈0.01）。结论TNF—α可诱导DC的成熟，使DC的抗原递呈和自分泌IL-12的能力显著提高，DC递呈抗原后能诱发显著的主动特异性CTL。     

Activated Akt and Erk Expression and Survival After Surgery in Pancreatic Carcinoma

Background Long-term survival of surgically resectable pancreatic cancer patients is uncommon. The epidermal growth factor receptor (EGFR) and the phosphoinositol-3-kinase pathways are often activated in pancreatic cancer, and an understanding of their role in resected cases may help refine adjuvant therapy. Methods We investigated the expression of EGFR, Erk, Akt, and their phosphoforms (p-) in pancreatectomy specimens and correlated these with survival. Thirty-nine consecutive surgically resected pancreatic adenocarcinoma cases were included. Immunohistochemical staining of paraffin-embedded blocks was performed by using monoclonal antibodies against EGFR, Erk, p-Erk, Akt, and p-Akt. A standard immunoperoxidase technique was used to detect the avidin-biotin peroxidase complex. Immunostaining was visually scored with the histoscore method by two surgical pathologists. Results Patient characteristics were as follows: 17 men and 22 women; median age, 66 years; and American Joint Committee on Cancer stage I, 5 patients; stage II, 4 patients; stage III, 27 patients; and stage IV, 3 patients. The tumor was World Health Organization grade 1 in 4, grade 2 in 17, and grade 3 in 18 cases. Adjuvant therapies were chemotherapy (n = 6), radiotherapy (n = 1), and chemoradiotherapy (n = 17). Immunohistochemistry revealed positive expression of EGFR in 30.8%, Erk in 92.3%, p-Erk in 45.9%, Akt in 71.8%, and p-Akt in 20.5% of cases. On univariate analyses, tumor grade (P = .0098), p-Akt (P = .0003), and p-Erk (P = .0052) expression correlated with survival. On multivariate analyses, age (P = .0002; hazard ratio [HR], 1.8), grade (P = .00318; HR, 3.0), Akt (P = .0433; HR, .4), p-Akt (P = .0002; HR, .2), and p-Erk (P = .0003; HR, 3.5) expression correlated significantly with survival. Conclusions p-Erk and p-Akt expression may have prognostic and therapeutic implications in pancreatic cancer.     

Variations in Surgical Treatment and Outcomes of Patients With Pancreatic Cancer: A Population-Based Study

Background There is ongoing debate on how variations in surgical technique affect outcomes in pancreatic cancer. This population-based study examines current surgical practice and outcomes for cancer of the pancreatic head. Methods All patients 18 to 85 years old diagnosed with nonmetastatic adenocarcinoma of the pancreatic head from 1998 through 2003 were identified from the Surveillance, Epidemiology and End Results (SEER) Program registry. Multivariable regression was used to elucidate factors associated with the type of pancreaticoduodenectomy performed, extent of lymph node (LN) assessment, early mortality, and late survival. Results Overall, 2111 patients were included in the study, with 83.7% treated with a standard Whipple pancreaticoduodenectomy (PD). However, there was marked regional variation in the use of pylorus-preserving pancreaticoduodenectomy (PPPD; range, .03%–32.0%; P < .0001) and total pancreatectomy (TP; range, .04%–19.5%; P < .0001). TP was associated with significantly higher early mortality (odds ratio, 2.6; 95% confidence interval, 1.6 to 4.1; P < .0001), but late survival did not differ significantly between TP, PPPD, and PD (P = .69). Significant variation was also seen in the number of LN assessed (range across SEER regions, 7.3–13.5; P < .0001). Decreased LN assessment reduced the odds of diagnosing a patient as node positive and was associated with worse late survival. Conclusions In this population-based study, we found marked clinically important variability in the surgical treatment of adenocarcinoma of the pancreatic head, with respect to the use of TP, PPPD, or PD, and the extent of LN assessment. Further research is warranted to elucidate the underlying reasons, and to clarify the role of adequate lymphadenectomy.     

胰腺腺泡细胞癌的临床诊治分析(附8例报告)

目的:探讨胰腺腺泡细胞癌的病理改变、临床特点、诊治及预后。方法:回顾2004年1月至2013年8月我院收治的8例胰腺腺泡细胞癌病例,分析其临床病理特征,结合随访资料进行总结。结果:8例胰腺腺泡细胞癌病人的平均年龄为(55.1±8.9)(37~67)岁,均为男性。术前主诉为腹胀(1例)、腹痛或不适(3例)、黄疸(2例)和体重下降(1例)。术前血清CA19-9平均值为(83.3±14.1)(3.7~285.1)U/mL。肿瘤位于胰头部3例,胰体尾部5例。术后病理学分期ⅠA期1例,ⅠB期3例,ⅡA期4例,无Ⅲ、Ⅳ期病例。术后死亡3例,死因分别为腹腔感染伴出血、全身多发转移伴恶病质和消化道出血。2例病人带瘤生存,分别为肝转移和肝、骨转移,其余3例情况良好。结论:胰腺腺泡细胞癌是一种少见的胰腺上皮源性恶性肿瘤,男性为主,预后较差,但明显好于胰腺导管腺癌。手术是胰腺腺泡细胞癌治疗首选。新辅助化疗和辅助化疗是否可改善病人预后,尚有待研究。     

Single Incision Laparoscopic Pancreas Resection for Pancreatic Metastasis of Renal Cell Carcinoma

Background:

Transumbilical single incision laparoscopic surgery (SILS) offers excellent cosmetic results and may be associated with decreased postoperative pain, reduced need for analgesia, and thus accelerated recovery. Herein, we report the first transumbilical single incision laparoscopic pancreatectomy case in a patient who had renal cell cancer metastasis on her pancreatic corpus and tail.

Methods:

A 59-year-old female who had metastatic lesions on her pancreas underwent laparoscopic subtotal pancreatectomy through a 2-cm umbilical incision.

Results:

Single incision pancreatectomy was performed with a special port (SILS port) and articulated equipment. The procedure lasted 330 minutes. Estimated blood loss was 100mL. No perioperative complications occurred. The patient was discharged on the seventh postoperative day with a low-volume (20mL/day) pancreatic fistula that ceased spontaneously. Pathology result of the specimen was renal cell cancer metastases.

Conclusion:

This is the first reported SILS pancreatectomy case, demonstrating that even advanced surgical procedures can be performed using the SILS technique in well-experienced centers. Transumbilical single incision laparoscopic pancreatectomy is feasible and can be performed safely in experienced centers. SILS may improve cosmetic results and allow accelerated recovery for patients even with malignancy requiring advanced laparoscopic interventions.