|Year : 2023 | Volume
| Issue : 1 | Page : 4
Giant mucinous cystadenoma of the pancreas in a young female
Department of Radiology, St. John's Hospital, Bengaluru, Karnataka, India
|Date of Submission||18-Jun-2022|
|Date of Decision||31-Jul-2022|
|Date of Acceptance||23-Aug-2022|
|Date of Web Publication||09-Feb-2023|
Department of Radiology, St. John's Hospital, Bengaluru - 560 034, Karnataka
Source of Support: None, Conflict of Interest: None
Cystic tumors of the pancreas are rare and may be confused with hydatid cysts, especially in endemic areas. The incidence of cystic neoplasms of the pancreas is relatively rare in the pediatric population and young adults. We present a 21-year-old female with a well-defined giant multiloculated cystic lesion in the body and tail of the pancreas, which was diagnosed as a hydatid cyst elsewhere. The patient underwent laparoscopic spleen-preserving distal pancreatectomy. There was no visible communication between the mucinous cystic neoplasm and the pancreatic duct. Histopathology confirmed the diagnosis of benign mucinous cystadenoma of the pancreas. Ambiguity in the management of the lesion arises when mucinous cystadenoma is commonly misdiagnosed as pancreatic pseudocyst because the surgical management is entirely different.
Keywords: Giant mucinous cystadenoma of the pancreas, Laparoscopic spleen-preserving distal pancreatectomy, Pancreatic pseudocyst, Ultrasound
|How to cite this article:|
Reddy R. Giant mucinous cystadenoma of the pancreas in a young female. Digit Med 2023;9:4
| Introduction|| |
Mucinous cystadenoma of the pancreas is almost always attributed to postmenopausal females and represents between 10% and 45% of cystic lesions of the pancreas, which is often diagnostically challenging with relevant clinical implications. Pancreatic tumors are relatively rare in children and young adults, and mucinous cystadenomas are even rarer. Cystic neoplasms of the pancreas are classified based on epithelial differentiation as benign serous cystadenoma, mucinous cystadenoma, and malignant cystadenocarcinoma. Complications related to surgical management of mucinous cystadenoma include pancreatitis, portal vein thrombosis, biliary obstruction, gastrointestinal obstruction, abscess, and fistula formation. We report a case of mucinous cystic neoplasm of the pancreas in a 21-year-old female with emphasis laid on the diagnostic role of high-resolution ultrasound supplemented by histopathological findings.
| Case Report|| |
A 21-year-old female with rural background and a history of progressive distension of the upper abdomen causing mild discomfort for the 3 months was referred to the department of radiodiagnosis with a request for an ultrasound of the abdomen. Findings on high-resolution ultrasound revealed a well-defined giant multiloculated cystic lesion of size approximately 9 cm × 11 cm (anteroposterior × mediolateral) located in the body and tail of the pancreas extending up to the splenic hilum [Figure 1]a and [Figure 1]b. Morphology of the rest of the pancreas was unremarkable. The cystic lesion showed no solid component, septations had no vascularity, and the locules measured 2–4 cm in size demonstrated thick walls and low-level internal echoes within [Figure 1]c. There were associated foci of mural calcifications. However, there was the absence of a central scar with no evidence of a solid component protruding from the wall of the cystic lesion. Differential diagnosis being considered was a pancreatic hydatid cyst. The patient did not demonstrate complications such as biliary tract obstruction causing jaundice, intestinal obstruction, or new-onset diabetes due to islet cell dysfunction. Subsequently, the patient was referred to the department of gastrointestinal surgery where she underwent laparoscopic spleen-preserving distal pancreatectomy in view of its potential to turn malignant. Cystic fluid analysis demonstrated carcinoembryogenic antigen (CEA) levels of 398 ng/mL, CA 19–9 levels of 22 ng/mL, and low content of lipase, indicating no communication with the main pancreatic duct. Cytological findings were negative for malignancy. Histopathology of the resected specimen confirmed the diagnosis of benign mucinous cystadenoma of the pancreas. Postsurgery, the recovery period was uneventful and the patient was symptom-free. Follow-up at 6 months demonstrated no features of recurrence.
|Figure 1: Ultrasound image. (a) Transverse ultrasound image demonstrating a multiloculated cystic lesion in the pancreas. Locules (stars) and intervening septations (arrows) are demonstrated. (b) Color Doppler ultrasound image demonstrating a well-defined cystic lesion (arrows) in the pancreatic body and tail with left lateral extent up to the hilum of the spleen. (c) Color Doppler ultrasound image demonstrating no vascularity within the septations. PC: Portal confluence, RPSV: Retropancreatic splenic vein, SMA: Superior mesenteric artery, IVC: Inferior vena cava, A: Aorta|
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| Discussion|| |
Mucinous cystic neoplasms (MCNs) of the pancreas comprise mucinous cystadenoma, mucinous cystadenocarcinoma, and intraductal papillary mucinous neoplasm (IPMN). Pancreatic mucinous cystadenoma accounts for nearly half of cystic neoplasms of the pancreas and is almost exclusively encountered in females aged 40–60 years, often localized in the pancreatic body/tail demonstrating no communication with the main pancreatic duct. Complications include mass effect on the adjacent viscera, malignant transformation to mucinous cystadenocarcinoma, and rupture of the lesion with resultant peritonitis. Mucinous cystadenoma is a premalignant lesion, and surgical excision is often advocated. The lesion is rarely encountered in young females below 30 years and is extremely rare in males. Features indicative of the malignant transformation of mucinous cystadenoma of the pancreas include irregularity or increased wall thickness, obstruction or displacement of the main pancreatic duct, and eggshell calcification of the lesion.
MCNs are premalignant cystic neoplasms of the pancreas, and surgical excision is preferred due to its favorable prognosis. Imaging investigations include magnetic resonance imaging (MRI) or contrast-enhanced computed tomography (CECT) supplemented by endoscopic ultrasound (EUS) with fine needle aspiration (FNA). On ultrasound, MCN appears as a multilocular cystic lesion with internal septations as demonstrated in the index case. On nonenhanced CT (NECT), contents of MCN may be heterogenous in attenuation with multiple internal septations present. Furthermore, there may be associated calcifications that are more peripheral in MCN. MRI can be quintessential to assess major vessel involvement and resectability and help distinguish IPMNs from mucinous cystic pancreatic neoplasms. MCN is typically filled with mucin and appears hypointense on T1-weighted MR images and homogeneously hyperintense on T2-weighted MR images which may be altered. However, on T1 sequences, the signal intensity may vary depending on the proteinaceous content of the mucin. Pancreatic pseudocysts are sequelae of acute pancreatitis which resolve spontaneously, and usually, the treatment is conservative. Potential complications of larger pseudocysts include infection, hemorrhage, rupture, and disruptions of the main pancreatic duct. On ultrasound, pseudocysts appear as anechoic collections, with dependent low-level echoes representing debris. On CECT, pancreatic pseudocysts appear as well-circumscribed peripancreatic fluid collections of homogeneously low attenuation that are usually surrounded by a well-defined enhancing wall. On MRI, pancreatic pseudocysts appear hypointense on T1-weighted sequences and hyperintense on T2-weighted sequences with layering or dependent debris which is a highly specific sign for the diagnosis of pancreatic pseudocyst.
Solid pseudopapillary tumors (SPNs) are benign pancreatic neoplasms commonly encountered in young females and must be considered in the differential diagnosis. On NECT, SPN appears as a well-encapsulated lesion with varying solid and cystic components owing to hemorrhagic degeneration. On CECT, typically solid areas are noted peripherally and demonstrate enhancement, whereas cystic spaces do not enhance and are more centrally located. Calcifications and enhancing solid areas may be present at the periphery of the mass.
Imaging modalities such as CECT, EUS, and magnetic resonance cholangiopancreatography are useful in the differential diagnosis and treatment planning of cystic neoplasms of the pancreas. Nevertheless, positive findings on histopathology establish the diagnosis. Ambiguity in the management of the lesion arises when mucinous cystadenoma of the pancreas is commonly misdiagnosed as pancreatic pseudocyst because the surgical management is entirely different. MCNs of the pancreas are premalignant lesions, and complete surgical resection is the mainstay of treatment. Sometimes due to misdiagnosis of the entity, placement of a cystogastrostomy tube has been included as part of the management plan. The prognosis of mucinous cystadenoma of the pancreas is excellent with symptoms receding following surgical removal of the tumor. Follow-up at regular intervals is recommended to prevent recurrence of mucinous cystic neoplasm of the pancreas.
| Conclusion|| |
Although rare, cystic neoplasms of the pancreas should be considered in the differential diagnosis of cystic mass lesions involving the pancreas. In conclusion, this case report deserves a special mention as there are only handful cases of giant mucinous cystadenoma of the pancreas occurring in young adults reported in the literature. This case report also highlights the role of ultrasound in providing a differential diagnosis for cystic neoplasms of the pancreas and stresses the fact that the entity needs to be considered in young patients with incidentally detected cystic lesions of the pancreas and/or with borderline or raised CEA levels.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
I wish to thank Mani Sabbavarapu for her assistance in proofreading and native English editing of the manuscript.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Crippa S, Salvia R, Warshaw AL, Domínguez I, Bassi C, Falconi M, et al.
Mucinous cystic neoplasm of the pancreas is not an aggressive entity: Lessons from 163 resected patients. Ann Surg 2008;247:571-9.
Russell RT, Sharp KW. Mucinous cystadenoma of the pancreas associated with acute pancreatitis and concurrent pancreatic pseudocyst. Am Surg 2005;71:292-7.
Dorobanţu BM, Matei E, Herlea V, Boroş M, Tivadar B, Ciurea SH. Diagnosis, morphopathological profile and treatment of mucinous cystadenoma of the pancreas – A single center experience. Rom J Morphol Embryol 2018;59:1155-63.
Fujinaga A, Hirashita T, Iwashita Y, Nakanuma H, Tada K, Masuda T, et al.
A case of ruptured mucinous cystadenoma of the pancreas with recurrence-free survival for 8 years. Surg Case Rep 2020;6:52.
Yamashita Y, Ito K, Noda Y, Kobayashi G, Obana T, Horaguchi J, et al.
A case of mucinous cystadenoma of the pancreas resected after a 10-year follow-up. Nihon Shokakibyo Gakkai Zasshi 2011;108:1280-7.
Suzuki M, Fujita N, Onodera H, Kayaba Y, Suzuki S, Kagaya H, et al.
Mucinous cystic neoplasm in a young male patient. J Gastroenterol 2005;40:1070-4.
Sahani DV, Kadavigere R, Saokar A, Fernandez-del Castillo C, Brugge WR, Hahn PF. Cystic pancreatic lesions: A simple imaging-based classification system for guiding management. Radiographics 2005;25:1471-84.
Sarno A, Tedesco G, De Robertis R, Marchegiani G, Salvia R, D'Onofrio M. Pancreatic cystic neoplasm diagnosis: Role of imaging. Endosc Ultrasound 2018;7:297-300.
] [Full text]
Shuja A, Alkimawi KA. Solid pseudopapillary tumor: A rare neoplasm of the pancreas. Gastroenterol Rep (Oxf) 2014;2:145-9.
Fernández-del Castillo C. Mucinous cystic neoplasms. J Gastrointest Surg 2008;12:411-3.