Mucinous Cystic Neoplasm of the Pancreas Demonstrated by Ultrasound and Endoscopic Retrograde Pancreatography

Mucinous cystic neoplasms of the pancreas are rare lesions. They may undergo subclinical malignant transformation; hence, establishment of the diagnosis prior to surgical exploration is of great importance. The case herein reported is of interest because the use of endoscopic retrograde pancreatography and ultrasound strongly supported a clinical suspicion of a true cyst of the pancreas. It also represents the second case reported studied by these diagnostic modalities.     

Multiple-line Chemotherapy for a Patient with Unresectable Mucinous Cystic Neoplasm of the Pancreas

A 74-year-old woman with a cyst in her pancreatic tail was referred to our hospital. Computed tomography confirmed a large cystic lesion with irregular wall thickening, abdominal lymph node swelling, and ascites. We diagnosed her with an unresectable mucinous cystic neoplasm, since ascites cytology revealed adenocarcinoma. The patient received chemotherapy up to the fifth line for 55.2 months. Gemcitabine plus nab-paclitaxel and modified FOLFIRINOX achieved a partial response with a progression-free survival time of 12.1 and 20.4 months, respectively. The overall survival time from the beginning of first-line chemotherapy was 69.4 months.     

Clinical Features and Natural History of Serous Cystic Neoplasm of the Pancreas

Aims: To clarify the clinical features and the natural history of serous cystic neoplasm (SCN) of the pancreas. Methods: We retrospectively analyzed data from 30 patients affected by SCN. SCNs were classified as (1) microcystic type, (2) micro- and macrocystic type, and (3) macrocystic type according to the modified WHO classification. Eighteen patients who underwent serial radiographic imaging were identified, and tumor growth rate in these patients was evaluated. Results: The median age was 62 years, and the female:male ratio was 2:1. Twenty-five patients (83%) were asymptomatic and 5 (17%) were symptomatic. The median tumor size was 2.6 cm. Fifteen cases (50%) had the microcystic type, 7 (23%) the micro- and macrocystic type, and 8 (27%) the macrocystic type. Age, gender, symptoms, location or tumor size did not differ significantly among the three subtypes. Eighteen patients were followed up for a median of 58 months. Morphological changes were observed in 3 patients (17%) and enlargement of tumor size in 9 patients (50%) during the follow-up. The growth rate was 0.29 cm per year and doubling time was 3.5 years; these rates did not differ among morphological subtypes or size of tumors. Conclusions: In asymptomatic patients with a clear imaging diagnosis of SCN, non-operative management with a careful follow-up should be recommended. Surgery should be suggested in only symptomatic patients, those with giant tumors (110 cm), rapid growing or when the presence of a potentially malignant tumor cannot be excluded.     

胰腺实性假乳头状瘤293例临床荟萃分析

背景：胰腺实性假乳头状瘤（SPNP）是一种低度恶性的胰腺肿瘤,近年报道的例数明显增多。目的：总结SPNP的临床特征。方法：对以“囊实性肿瘤／胰腺”、“实性假乳头状瘤／胰腺”为关键词,在中国医院知识仓库医学专题全文数据库（CHKD）检索得到的1997年10月～2008年8月发表的32篇文献中293例SPNP患者的临床特征进行分析。结果：人组SPNP患者的男女比例为1：8．2,平均年龄27．3岁,临床表现无特异性。SPNP影像学表现为由不同比例的囊实性成分组成,术前影像学检查的诊断率为41．0％。所有患者均为单发性肿块,平均最大径7．6cm。218例患者的包膜完整,75例不完整。共196例患者接受治疗,根治性手术切除率94．9％。220例患者平均随访33_3个月,6例复发．3例死亡。结论：SPNP是一种并非罕见的低度恶性肿瘤,好发于年轻女性。手术切除对SPNP的疗效较好．患者预后佳。     

Frequency of Intraductal Papillary Mucinous Neoplasm in Patients with and without Pancreas Cancer

Purpose: To determine the frequency of intraductal papillary mucinous neoplasm (IPMN) in patients with and without invasive ductal adenocarcinoma (IDAC). Methods: 82 patients underwent pancreatectomy for pancreas adenocarcinoma. 68/82 subjects underwent at least one preoperative imaging study including CT (n = 43), MRI (n = 25), or both (n = 12). Imaging studies were retrospectively evaluated to determine if IPMN was present in the gland at a location distant from IDAC. In 183 different adult patients undergoing MRI for renal mass, images were evaluated to determine the frequency of IPMN. Fisher's exact test was used to test whether the prevalence of IPMN was greater among patients with pancreas cancer than those without. Results: Five of 68 (7.3%) patients who underwent pancreatic resection for IDAC had IPMN at a site distant from the cancer. Two of 182 (1.1%) patients undergoing MRI for renal cancer had imaging evidence of IPMN. There was a significant difference (p = 0.017) in the prevalence of IPMN between patients with and without IDAC. The odds ratio for IPMN as a predictor of pancreas cancer was estimated as 7.18. Conclusion: IPMN occurs with increased frequency in patients with pancreas cancer as opposed to those without pancreas cancer.     

Possible Oncogenesis of Mucinous Cystic Tumors of the Pancreas Lacking Ovarian-Like Stroma

Background/Aims: Clinicopathological features and postoperative results from mucinous cystic tumors of the pancreas (MCTs) were reviewed. MCTs with ovarianlike stroma (MCTs-OLS+; n = 6) and those lacking ovarianlike stroma (MCTs-OLS–; n = 4) were compared to elucidate the oncogenesis of MCT without OLS. Patients and Methods: Ten patients with MCT were studied. Results: The 6 MCTs-OLS+ cases occurred in females and were located in the body and tail of the pancreas. The mean tumor size was 6.5 cm (range 2–11 cm). The majority (5/6) of MCTs-OLS+ were multilocular and exhibited tiny loculi on the cyst wall and septum characteristic of MCTs-OLS+. Pathological classifications were adenoma in 4 patients and noninvasive adenocarcinoma in 2 patients. All 6 patients were alive without tumor recurrence 6–124 months after tumor resection. Of the 4 MCTs-OLS– cases, 2 were males and 2 females; MCTsOLS– were located in the tail of the pancreas. The mean tumor size was 6.9 cm (range 4–8.4 cm). Invasive cancer in the pancreatic parenchyma or extrapancreatic tissue was recognized in all 4 patients, and the pathological classification of epithelia of the cyst wall were adenocarcinomas. These findings were also compatible with common invasive ductal carcinomas of the pancreas with secondary retention cyst on pseudocyst. All patients died of the disease (15, 27, 31 and 80 months after resection, respectively). Whether or not OLS is specific for MCTs of the pancreas should be clarified in future studies. Conclusion: The results of our study led to three hypotheses regarding the oncogenesis of MCTs-OLS–: (1) MCTs in which OLS disappears during the development of invasive carcinoma; (2) advanced cancer derived from intraductal papillary mucinous tumor of the pancreas, and (3) invasive ductal carcinoma of the pancreas with secondary cyst.     

Pancreas and Cystic Fibrosis: The Implications of Increased Survival in Cystic Fibrosis

Pancreatitis affects 0.5% people with cystic fibrosis (CF) in the UK and 0.01% of the normal population. Why do some with CF get pancreatitis and some not? And does pancreatitis in neonates result in pancreatic failure with no further inflammation or risk of pancreatic cancer? Review of the literature would suggest that 85% of those with CF have pancreatic destruction as children with minimal risk of further inflammatory or neoplastic changes. Those with a functioning pancreas are at risk of developing pancreatitis. There are several case series of pancreatic cancer reported in CF patients, but overall the risk is unknown. As patients with CF and pancreatic sufficiency are living longer, further studies to assess the riskof developing pancreatic cancer in this subgroup should be considered.     

Cystic Lesions of the Pancreas

Opinion statement Pancreatic cystadenomas are a group of benign, premalignant, and malignant cystic tumors of the pancreas. Serous cystadenomas are benign lesions that often do not require surgical excision unless they are complicated by bleeding, obstruction, or pain. Mucinous cystadenomas are premalignant lesions that may be surgically excised if there is a concern regarding malignant degeneration. However, it may be difficult to predict the timing and risk of malignant change. Also, it is controversial whether all mucinous cystadenomas should be resected. Cystadenocarcinomas should be surgically managed if they are resectable, that is, there is no evidence of metastatic disease. Intraductal papillary mucinous tumors share many features with mucinous cystadenomas. However, intraductal papillary mucinous tumors arise from the pancreatic duct and are often associated with a dilated pancreatic duct. These lesions are often managed with surgical resection, including total pancreatectomy for diffuse lesions with evidence of localized malignancy.