Mucinous cystic pancreatic neoplasms (MCPN) are rare tumors of the pancreas, which mostly occur in middle-aged females. The survival rate for this disease is far better than pancreatic ductal adenocarcinomas. The tumors frequently are confused with intraductal papillary mucinous neoplasms (IPMN). According to the World Health Organization (WHO), intraductal papillary mucinous neoplasms differ from mucinous cystic pancreatic neoplasms because ovarian stroma is observed in the latter. 
The etiology of mucinous cystic pancreatic neoplasms is unclear. Professionals hypothesize that these tumors are related to female hormones due to the preponderance of these tumors in women. This is believed to be the case because ovarian mucinous cystic neoplasms share the same histology and characteristics as mucinous cystic pancreatic neoplasms. These shared characteristics include the mucinous secreting columnar epithelium and the surrounding ovarian stroma that are seen in both ovarian and pancreatic mucinous cystic lesions. Also, the presence of estrogen receptors in mucinous cystic pancreatic neoplasms is another feature alluding to a hormonal cause. Finally, another common feature is the benign nature of the ovarian mucinous cystic tumors, which rarely metastasize to other organs or involve lymph nodes akin to mucinous cystic pancreatic neoplasms. However, due to the rarity of these tumors, other etiologies are unknown. A benign mucinous cystic pancreatic neoplasm can potentially evolve into aggressive cancer, and hence, surgical resection is recommended in younger patients.
Few studies have investigated the true prevalence and incidence of mucinous cystic pancreatic neoplasms. Mucinous cystic pancreatic neoplasms mostly affect females, particularly middle-aged women [48 +/- 15 years (84% < 60 y)], as shown in a retrospective study that identified the disease in 95% of women. A recently published study indicated an incidence rate of 2.4% and a prevalence rate of 2.6%, although other retrospective trials reported a prevalence rate as high as 17.5%. Mucinous cystic pancreatic neoplasms are mostly (93%) located in the body or tail of the pancreas. Median size was 5 cm (61% > or = 5 cm). These tumors are identified in 10% to 20% of resected pancreatic tumors. Those tumors are usually benign (72%), borderline (10.5%), in situ carcinoma (5.5%), and invasive carcinoma (12%). Invasive mucinous cystic pancreatic neoplasm usually presents in elderly patients, and the difference in average age between benign and malignant disease of 11 years alludes to the fact that benign lesions can be precancerous. The five-year disease-specific survival for benign and malignant mucinous cystic pancreatic neoplasms is 100% and 57%, respectively. Patients who develop recurrence of their disease have a guarded prognosis.
Mucinous cystic pancreatic neoplasms have two distinct histologic components, which are the inner epithelial layer composed of tall mucin-secreting cells and dense cellular ovarian stroma. The latter was described in 1978 by Compagno and Oertel, and the disease was distinguished from other serous cystic tumors and intraductal papillary mucinous neoplasms. Mucinous cystic pancreatic neoplasms are usually benign tumors and rarely metastasize. The high prevalence of these tumors in females indicates hormonal factors as a potential instigator, as mentioned above. A study found high expression of estrogen receptors in these tumors (ovarian stroma). Mucinous cystic pancreatic neoplasms with invasive carcinoma typically have malignant neoplastic cells beyond the epithelial lining of the cyst. These tumors are divided into two distinct types, which are intracapsular, if neoplastic invasion did not penetrate through the wall of the capsule, and extracapsular if extension through the capsular wall is evident and tumor extends into the surrounding pancreatic or extrapancreatic parenchyma. These cystic lesions, as opposed to intraductal papillary mucinous neoplasms, do not connect with pancreatic ducts, and this is often a distinguishing characteristic.
Conversely, most mucinous cystic pancreatic neoplasms lesions are benign and rarely metastasizes to lymph nodes or other organs. It is believed that benign lesions can transform into malignant mucinous cystic pancreatic neoplasms due to the older age of presentation in patients with invasive cancer. Tumors that are larger than 4 cm and have nodules are most likely malignant. WHO nomenclature uses the term mucinous cystic pancreatic neoplasms in conjunction with a histologic grade (low-grade dysplasia, intermediate-grade [moderate] dysplasia, or high-grade dysplasia).
Patients with mucinous cystic pancreatic neoplasms present with abdominal pain (62.2%), weight loss (11%), abdominal mass (11%), acute pancreatitis (9.6%), fatigue (8.9%), and 16% were asymptomatic. Large mucinous cystic pancreatic neoplasms can compress surrounding organs and structures, leading to abdominal pain and a sensation of fullness. Conversely, many patients are asymptomatic, and lesions are found incidentally on imaging. Patients occasionally (16.5%) have elevated levels of CA 19-9 (> 37 U/L). On physical examination, patients can have abdominal tenderness and distention, and occasionally an epigastric mass can be palpated if it is significantly large. These lesions are often located in the body and tail of the pancreas and, thus, rarely cause obstructive jaundice. If patients present with a pancreatic head mucinous cystic pancreatic neoplasm, then jaundice may be evident.
Providers should follow an essential physical examination and laboratory studies, including a complete blood count (CBC), comprehensive metabolic panel (CMP), and a CA 19-9. They should also order a computed tomography (CT) or magnetic resonance (MR) scans. An MRI can be quintessential to assess major vessel involvement and resectability and help distinguish intraductal papillary mucinous neoplasms from mucinous cystic pancreatic neoplasms. Rarely, if cytology or a biopsy is needed, an endoscopic ultrasound (EUS) with fine-needle aspiration (FNA) should be performed.
Treatment of mucinous cystic pancreatic neoplasms mainly involves surgical resection. The surgery involves distal pancreatic resection with or without splenectomy. Other surgical procedures include pancreaticoduodenectomy, enucleation, middle pancreatectomy, or total pancreatectomy. In published trials, there is no significant mortality associated with surgery. However, postoperative morbidity can be as high as 49%. It is still recommended practitioners pursue non-operative management for tumors that are smaller than 3 cm and without mural nodules, specifically in elderly patients with comorbidities. This is because mucinous cystic pancreatic neoplasms that are smaller than 4 cm and with no nodules are usually adenomas or borderline neoplasms.
Hence, the patients mentioned above can be followed with serial imaging. Conversely, middle-aged women with a long life expectancy presenting with mucinous cystic pancreatic neoplasms, surgical resection is recommended as many of the benign lesions can progress to invasive carcinoma. Since we still cannot distinguish lesions that progress from those that can remain benign, it is recommended to resect all mucinous cystic pancreatic neoplasms regardless of size in patients who are a fit candidate for surgery. No patient with noninvasive disease had a recurrence after resection. Moreover, since small mucinous cystic pancreatic neoplasms without nodules are most likely benign, lymphadenectomy can be avoided, and parenchyma-sparing surgery such middle-pancreatectomy and enucleation can be performed as these less invasive surgeries decrease the rate of postoperative pancreatic insufficiency and have been proven to be safe and well tolerated. This decreases the development of postoperative pancreatic endocrine and exocrine insufficiency. In lesions that are less than 5 cm, minimally invasive procedures should be considered. In large surgical centers, laparoscopic resection of neoplasms was safe and well-tolerated and decreased the length of postoperative hospitalization, surgical wound healing, and led to spleen preservation in distal pancreatectomy surgeries. Considering the rarity of this tumor, the need to distinguish it from intraductal papillary mucinous neoplasm, and the improved outcome in high-volume surgical centers, it is recommended to manage patients with mucinous cystic pancreatic neoplasms in specialized centers. Extrapolating from data in pancreatic adenocarcinoma, after surgical resection of the pathologic assessment, shows invasive carcinoma then adjuvant chemotherapy with gemcitabine or gemcitabine in conjunction with capecitabine is recommended.
It is imperative to distinguish intraductal papillary mucinous neoplasm from mucinous cystic pancreatic neoplasms. Surgical resection of mucinous cystic pancreatic neoplasms is recommended in fit patients, given the potential for malignant transformation.
Mucinous cystic pancreatic neoplasms (MCPN) are very difficult to diagnose and successfully treat rare tumors of the pancreas which mostly occur in middle-aged females. The tumors frequently are confused with intraductal papillary mucinous neoplasms (IPMN). The interprofessional healthcare team must work together to make the diagnose and successfully treat patients with MCPN. Care should be coordinated by nurses and physicians and pharmacists should provide assistance to the team in managing chemotherapeutic agents. Operative and perianesthesia nurses are involved in patient care and monitoring. Oncology nurses administer treatment, provide patient education, and keep the team updated on patient status. Oncologic pharmacists evaluate prescribed chemotherapy, check for drug-drug interactions, and participate in patient education. Outcomes can be improved by team coordination. [Level V]
|||Pancreatic Cysts and Guidelines., Farrell JJ,, Digestive diseases and sciences, 2017 Jul [PubMed PMID: 28528374]|
|||Insights into the Pathogenesis of Pancreatic Cystic Neoplasms., Sethi V,Giri B,Saluja A,Dudeja V,, Digestive diseases and sciences, 2017 Jul [PubMed PMID: 28500587]|
|||Pancreatic mucinous cystic neoplasm defined by ovarian stroma: demographics, clinical features, and prevalence of cancer., Reddy RP,Smyrk TC,Zapiach M,Levy MJ,Pearson RK,Clain JE,Farnell MB,Sarr MG,Chari ST,, Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association, 2004 Nov [PubMed PMID: 15551256]|
|||Cystic tumors of the pancreas. New clinical, radiologic, and pathologic observations in 67 patients., Warshaw AL,Compton CC,Lewandrowski K,Cardenosa G,Mueller PR,, Annals of surgery, 1990 Oct [PubMed PMID: 2171441]|
|||Natural History of Patients Followed Radiographically with Mucinous Cysts of the Pancreas., Pak LM,D'Angelica MI,DeMatteo RP,Kingham TP,Balachandran VP,Jarnagin WR,Allen PJ,, Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract, 2017 May 17 [PubMed PMID: 28516310]|
|||New criteria to differentiate between mucinous cystic neoplasm and serous cystic neoplasm in pancreas by endoscopic ultrasound: A preliminarily confirmed outcome of 41 patients., Zhang W,Linghu E,Chai N,Li H,, Endoscopic ultrasound, 2017 Mar-Apr [PubMed PMID: 28440237]|
|||Patients with a resected pancreatic mucinous cystic neoplasm have a better prognosis than patients with an intraductal papillary mucinous neoplasm: A large single institution series., Griffin JF,Page AJ,Samaha GJ,Christopher A,Bhaijee F,Pezhouh MK,Peters NA,Hruban RH,He J,Makary MA,Lennon AM,Cameron JL,Wolfgang CL,Weiss MJ,, Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.], 2017 May - Jun [PubMed PMID: 28416122]|
|||A Serous Cystic Neoplasm of the Pancreas Coexisting with High-Grade Pancreatic Intraepithelial Neoplasia Mimicking an Intraepithelial Papillary Mucinous Neoplasm: A Case Report., Kawanishi A,Hirabayashi K,Kono H,Takanashi Y,Hadano A,Kawashima Y,Ogawa M,Kawaguchi Y,Yamada M,Nakagohri T,Nakamura N,Mine T,, Case reports in oncology, 2017 Jan-Apr [PubMed PMID: 28413390]|
|||Mucinous cystic neoplasms of the pancreas: update on the surgical pathology and molecular genetics., Fukushima N,Zamboni G,, Seminars in diagnostic pathology, 2014 Nov [PubMed PMID: 25441310]|
|||Epidemiology, diagnosis, and management of cystic lesions of the pancreas., de Jong K,Bruno MJ,Fockens P,, Gastroenterology research and practice, 2012 [PubMed PMID: 22007199]|
|||The diagnosis of pancreatic mucinous cystic neoplasm and associated adenocarcinoma in males: An eight-institution study of 349 patients over 15 years., Ethun CG,Postlewait LM,McInnis MR,Merchant N,Parikh A,Idrees K,Isom CA,Hawkins W,Fields RC,Strand M,Weber SM,Cho CS,Salem A,Martin RCG,Scoggins CR,Bentrem D,Kim HJ,Carr J,Ahmad SA,Abbott DE,Wilson G,Kooby DA,Maithel SK,, Journal of surgical oncology, 2017 Jun [PubMed PMID: 28211072]|
|||Pancreatic pseudocyst or mucinous cystadenocarcinoma of pancreas? A diagnostic dilemma., Joshi U,Poudel P,Ghimire RK,Basnet B,, Clinical case reports, 2017 Apr [PubMed PMID: 28396777]|
|||Pancreatic cystic neoplasm: the role of cyst morphology, cyst fluid analysis, and expectant management., Leung KK,Ross WA,Evans D,Fleming J,Lin E,Tamm EP,Lee JH,, Annals of surgical oncology, 2009 Oct [PubMed PMID: 19536601]|
|||Surgical Management of Pancreatic Cysts: A Shifting Paradigm Toward Selective Resection., Gerry JM,Poultsides GA,, Digestive diseases and sciences, 2017 Jul [PubMed PMID: 28421458]|
|||Cystic pancreatic neoplasms: observe or operate., Spinelli KS,Fromwiller TE,Daniel RA,Kiely JM,Nakeeb A,Komorowski RA,Wilson SD,Pitt HA,, Annals of surgery, 2004 May [PubMed PMID: 15082969]|
|||Management of the rare entity of primary pancreatic cystic neoplasms., Stamatakos M,Sargedi C,Angelousi A,Kontzoglou K,Safioleas P,Petropoulou C,Safioleas M,, Journal of gastroenterology and hepatology, 2009 Jul [PubMed PMID: 19467142]|
|||Association of Preoperative Risk Factors With Malignancy in Pancreatic Mucinous Cystic Neoplasms: A Multicenter Study., Postlewait LM,Ethun CG,McInnis MR,Merchant N,Parikh A,Idrees K,Isom CA,Hawkins W,Fields RC,Strand M,Weber SM,Cho CS,Salem A,Martin RC,Scoggins C,Bentrem D,Kim HJ,Carr J,Ahmad S,Abbott DE,Wilson GC,Kooby DA,Maithel SK,, JAMA surgery, 2017 Jan 1 [PubMed PMID: 27760255]|
|||Pancreatic Cystic Lesions: Diagnostic, Management and Indications for Operation. Part I., Bauer F,, Chirurgia (Bucharest, Romania : 1990), 2017 Mar-Apr [PubMed PMID: 28463669]|
|||Risk of malignancy in resected cystic tumors of the pancreas < or =3 cm in size: is it safe to observe asymptomatic patients? A multi-institutional report., Lee CJ,Scheiman J,Anderson MA,Hines OJ,Reber HA,Farrell J,Kochman ML,Foley PJ,Drebin J,Oh YS,Ginsberg G,Ahmad N,Merchant NB,Isbell J,Parikh AA,Stokes JB,Bauer T,Adams RB,Simeone DM,, Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract, 2008 Feb [PubMed PMID: 18040749]|
|||Long-term outcomes after endoscopic ultrasound-guided ablation of pancreatic cysts., Choi JH,Seo DW,Song TJ,Park DH,Lee SS,Lee SK,Kim MH,, Endoscopy, 2017 May 16 [PubMed PMID: 28511236]|
|||Management of mucin-producing cystic neoplasms of the pancreas., Fritz S,Warshaw AL,Thayer SP,, The oncologist, 2009 Feb [PubMed PMID: 19211618]|
|||Enucleation in pancreatic surgery: indications, technique, and outcome compared to standard pancreatic resections., Hackert T,Hinz U,Fritz S,Strobel O,Schneider L,Hartwig W,Büchler MW,Werner J,, Langenbeck's archives of surgery, 2011 Dec [PubMed PMID: 21553230]|
|||Nature and management of pancreatic mucinous cystic neoplasm (MCN): A systematic review of the literature., Nilsson LN,Keane MG,Shamali A,Millastre Bocos J,Marijinissen van Zanten M,Antila A,Verdejo Gil C,Del Chiaro M,Laukkarinen J,, Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.], 2016 Nov - Dec [PubMed PMID: 27681503]|