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MRCP showing a large, lobulated pancreas replaced by conglomerate cysts of varying sizes. The pancreatic duct was not present.
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Diffuse serous cystadenomas of the pancreas are extremely rare, with only 8 cases reported previously, and have been associated with neuroendocrine tumors in only two patients. Some have been seen in von Hippel-Lindau disease. The management of these tumors poses a challenge due to their rarity and uncertain malignant potential. We report a case of...
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Introduction:
Pancreatic manifestations in Von Hippel-Lindau (VHL) disease can present as a multitude of forms, and their management can be challenging.
Presentation of the case:
A 66-year-old woman presented with increasing abdominal girth without other associated symptoms of nausea, vomiting, abdominal pain, weight-loss, and jaundice. Her medi...
Cystic lesions of pancreas include a myriad of different conditions ranging from the common pseudocyst to unusual cystic neoplasm. With the development of better imaging modalities, cystic neoplasms are diagnosed with greater frequency and accuracy leading on to better understanding of the natural course of these lesions. Serous cystadenoma is one...
Citations
... In this area, multiple SCNs with a multifocal appearance occur with a complete transformation of the pancreatic parenchyma [34]. In addition, there is an association with the occurrence of neuroendocrine tumors [35]. Asymptomatic patients with proven SCN should be followed up with once per year, after which, symptom-based follow-up is recommended (grade 2C recommendation) [7]. ...
Due to the increasing use of cross-sectional imaging techniques and new technical possibilities, the number of incidentally detected cystic lesions of the pancreas is rapidly increasing in everyday radiological routines. Precise and rapid classification, including targeted therapeutic considerations, is of essential importance. The new European guideline should also support this. This review article provides information on the spectrum of cystic pancreatic lesions, their appearance, and a comparison of morphologic and histologic characteristics. This is done in the context of current literature and clinical value. The recommendations of the European guidelines include statements on conservative management as well as relative and absolute indications for surgery in cystic lesions of the pancreas. The guidelines suggest surgical resection for mucinous cystic neoplasm (MCN) > 40 mm; furthermore, for symptomatic MCN or imaging signs of malignancy, this is recommended independent of its size (grade IB recommendation). For main duct IPMNs (intraductal papillary mucinous neoplasms), surgical therapy is always recommended; for branch duct IPMNs, a number of different risk criteria are applicable to evaluate absolute or relative indications for surgery. Based on imaging characteristics of the most common cystic pancreatic lesions, a precise diagnostic classification of the tumor, as well as guidance for further treatment, is possible through radiology.
... 16 Macroscopically and microscopically, VHL-associated SCNs share the same morphology as sporadic SCN cases, but they sometimes involve the whole pancreas in a patchy or diffuse pattern (in the latter case also called diffuse serous cystadenoma). 17 In addition, they are sometimes associated with other lesions, such as NETs and simple cysts. 18 The preoperative diagnosis of SCN is challenging owing to the scarcity of tumor cells usually present in endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) material. ...
Context.—
Pancreatic cystic lesions are increasingly diagnosed. Among other criteria, they are often distinguished in mucinous versus nonmucinous cysts. Mucinous pancreatic cystic lesions have received increasing attention, especially those known as precursors of pancreatic ductal adenocarcinoma. However, the group of nonmucinous cystic lesions of the pancreas includes numerous entities that may pose a diagnostic challenge. Their accurate diagnosis and classification are crucial for adequate patient management.
Objective.—
To review the spectrum of nonmucinous cystic lesions of the pancreas, taking into consideration their epidemiology and typical clinical context, their characteristic gross morphology and histomorphology, as well as their immunohistochemical and molecular profile.
Data Sources.—
Literature was searched and reviewed with MEDLINE via PubMed. Macroscopic and microscopic images were obtained from the archives of the Institute of Pathology, Heinrich Heine University and University Hospital of Duesseldorf, Germany.
Conclusions.—
Nonmucinous cysts of the pancreas comprise numerous, mostly rare entities displaying different biological behaviors. The most frequent are serous cystic neoplasms, solid-pseudopapillary neoplasms, cystic neuroendocrine tumors, and pancreatitis-associated pseudocysts. Accurate diagnosis can be achieved if characteristic clinical context, histomorphology, and immunoprofile are taken into account.
... Enhanced CT and MRI are commonly used during the diagnosis of pancreatic diseases. Using these image examinations, the tumor size and location can be easily verified as well as the anatomical relationship with surrounding organizations (12). EUS can effectively avoid the interference of intestinal gas, bone, and subcutaneous fat. ...
Background Pancreatic cystadenoma coexistent with neuroendocrine tumor is a rare disease which is reported sporadically.
Case Presentation We herein present one such interesting case in a 67-year old man. There was only one mass detected preoperatively by different examinations. Endoscopic ultrasound-guided biopsy indicated a pancreatic neuroendocrine tumor. Therefore, a distal pancreatectomy was performed and the final histopathology revealed two separate masses in the pancreas, one was serious cystadenoma and the other neuroendocrine tumor.
Conclusions SCA coexistent with PNET is rarely found during clinical practice. The component of PNET may not be confirmed until the operation. Based on the recorded cases, the malignant potential, clinical characteristics, treatment, and prognosis are remaining further researches.
... MSNNs are termed mixed tumors containing two components with different pathologies, namely, PSCN and pancreatic neuroendocrine tumor (PanNET). For MSNNs, PSCN diffuse involvement of the whole pancreas is extremely rare, with only eight cases reported in the literature [3][4][5][6][7][8][9][10] . Here, we report a novel case of diffuse PSCN admixture with an isolated PanNET in a Chinese woman and review the previous literature. ...
... Nevertheless, PanNETs are considered low-grade malignancies [2] , so the prognosis of MSNN is dependent on the PanNET component. As demonstrated by Agarwal et al [7] , lymph node metastasis occurred even when the diameter of the PanNET component was less than 0.5 cm. Therefore, the pathologist must perform a careful gross examination and sampling to exclude the presence of a concomitant PanNET in PSCN specimens. ...
... until June 2019; however, only 22 cases, including ours, contain detailed clinicopathological data[3][4][5][6][7][8][9][10][13][14][15][16][17][18][19][20][21][22] to allow analysis. In the remaining specimens, the patients range in age from 25 to 78 years (mean, 50.1 years). ...
Background:
Pancreatic mixed serous-neuroendocrine neoplasms (MSNNs) are mixed tumors containing two components with different pathologies, namely, pancreatic serous cystic neoplasm (PSCN) and pancreatic neuroendocrine tumor (PanNET). For MSNNs, diffuse PSCN involving the whole pancreas is extremely rare, with only eight previous case reports.
Case summary:
A 45-year-old Chinese woman, with a free previous medical history and no obvious symptoms, was found to have a pancreatic neoplasm and admitted to our hospital for further diagnosis in March 2018. Abdominal palpation revealed a painless, mobile mass in the epigastrium, and no abnormalities were observed in an examination of the nervous system and ocular system. A computed tomography scan showed multiple cystic lesions involving the whole pancreas ranging in diameter from 0.4 to 2 cm and also revealed an enhanced mass, 2.2 cm in diameter, in the head of the pancreas. Moreover, multiple cysts were found in the kidneys bilaterally, and the right lobe of the liver contained a small cyst. A Whipple operation with total pancreatectomy and splenectomy was performed. A diagnosis of pancreatic MSNN was established, consisting of diffuse serous microcystic cystadenoma with a concomitant grade 2 PanNET. Of note, the patient had no personal or family history of Von Hippel-Lindau syndrome or other disease.
Conclusion:
We report the first case of MSNN with a diffuse PSCN component involving the entire pancreas in a Chinese woman. It is important to be aware of its relationship with VHL syndrome, and close clinical follow-up is recommended.
... 10,13,[15][16][17][18][19][20][21][22][23] We found 33 published cases of pancreatic SCN coexisting with pancreatic (n = 30) or duodenal (n = 3) NEN in patients without VHL disease (Table 1). 10,13,16,[24][25][26][27][28][29][30][31][32][33][34][35][36][37] These NENs were located in the head, body, or tail of the pancreas. Of the reviewed literature, most publications were case reports with 1 or 2 cases. ...
Simultaneous presence of pancreatic serous cystic neoplasms and neuroendocrine neoplasms is rare. We present a case with the incidental finding of a duodenal neuroendocrine tumor (NET) with 2 lymph node metastases in a Whipple resection specimen performed to remove a pancreatic cystic neoplasm that postoperatively turned out to represent a serous cystic neoplasm (SCN). The patient was a 75-year-old female. She presented with loss of appetite and weight. Preoperative contrast-enhanced computed tomography scan of the abdomen showed a multicystic lesion in the head of pancreas. On histologic examination of the resection specimen, a pancreatic SCN was found, and in addition in 2 peripancreatic lymph nodes, metastases from a NET. Further examination of the resected specimen revealed a duodenal NET. Review of the literature revealed only one prior study with 4 cases of pancreatic SCN associated with a duodenal NET. In 1 of the 4 cases, the patient had von Hippel-Lindau disease. Our report emphasizes the importance of careful examination of pancreatic resection specimens including the peripancreatic lymph nodes also when dealing with SCNs, as coexisting and more malignant tumors may otherwise be missed.
... Only three cases of PNET with complete cystic replacement of the pancreas have been described in the literature (Table 1) [16][17][18][19]. Baek et al. and Jung et al. reported on the same patient. ...
Background:
von Hippel-Lindau disease is a dominantly inherited multi-system syndrome with neoplastic hallmarks. Pancreatic lesions associated with von Hippel-Lindau include serous cystic neoplasms, simple cysts, and neuroendocrine tumors. The combination of pancreatic neuroendocrine tumors and serous cystic neoplasms is relatively rare, and the surgical treatment of these lesions must consider both preservation of pancreatic function and oncological clearance. We report a patient with von Hippel-Lindau disease successfully treated with pancreas-sparing resection of a pancreatic neuroendocrine tumor where the pancreas had been completely replaced by serous cystic neoplasms, in which pancreatic function was preserved.
Case presentation:
A 39-year-old female with von Hippel-Lindau disease was referred to our institution for treatment of a pancreatic neuroendocrine tumor. Abdominal computed tomography demonstrated a well-enhanced mass, 4 cm in diameter in the tail of the pancreas, and two multilocular tumors with several calcifications, 5 cm in diameter, in the head of the pancreas. There was complete replacement of the pancreas by multiple cystic lesions with diameters ranging from 1 to 3 cm. Magnetic resonance cholangiopancreatography showed innumerable cystic lesions on the whole pancreas and no detectable main pancreatic duct. Endoscopic ultrasound-guided fine-needle aspiration of the mass in the pancreatic tail showed characteristic features of a neuroendocrine tumor. A diagnosis of pancreatic neuroendocrine tumor in the tail of the pancreas and mixed-type serous cystic neoplasms replacing the whole pancreas was made and she underwent distal pancreatectomy while avoiding total pancreatectomy. The stump of the pancreas was sutured as firm as possible using a fish-mouth closure. The patient made a good recovery and was discharged on postoperative day 9. She is currently alive and well with no symptoms of endocrine or exocrine pancreatic insufficiency 8 months after surgery.
Conclusions:
A pancreas-sparing resection should be considered for patients with pancreatic neuroendocrine tumors and complete cystic replacement of the pancreas to preserve quality of life after surgery.
... The systematic search yielded a total of 14 studies comprising 17 patients. [6][7][8][9][10][11][12][13][14][15][16][17][18][19] Hough et al [7] reported a case of diffuse cystic changes in the pancreas associated with PanNETs, but detailed data was not available. Baek et al [8] and Jung et al [12] reported the same patient in Korea, while Heresbach et al [6] reported a case of cystadenocarcinoma with MSNN in the pancreatic head. ...
... As a result, once a diagnosis of MSNN is made, we are more inclined to surgical treatment; in the cases that have been followed up, this lesion seemed to have a better prognosis after curative surgery. [9,11,15,16,18,19] However, the robustness of our treatment recommendation is diminished by the small number of cases. Evaluation of the malignant potential and prognostic outcomes of mixed tumors requires a long-term follow-up and data from additional reports of such cases. ...
Introduction
The aim of this study was to report a new case of mixed serous neuroendocrine neoplasm (MSNN) and review the literature concerning this type of lesion, which was added to the World Health Organization classification of pancreatic tumors in 2010.
Results
A 73-year-old woman presented with a pancreatic mass. The lesion was an intriguing combination of serous cystic neoplasm (SCN) and pancreatic neuroendocrine tumor (PanNET), in which the PanNET component grew into the wall of the serous oligocystic adenoma. We searched different databases for studies that had investigated MSNN. A total of 15 patients (age, 28–78), including the patient in the present study, were evaluated. We discuss these cases in detail especially regarding morphology and pathology; our case was the only one involving a collision type combination.
Conclusion
Although MSNN is recognized as a variant of SCN, it is quite different from SCN or PanNET. A new morphological analysis of MSNN may help in elucidating its histogenesis and prognosis.
... The simultaneous presence of pancreatic exocrine and endocrine tumors, although rare, has been already reported (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14). We report a case of simultaneous presence in the pancreas of a solid pseudopapillary tumor (SPT), a serous cystadenoma (SCA) and a neuroendocrine tumor (PanNET). ...
... In conclusion, the association between pancreatic tumors originating from different cell lineages is already known. The most common reported combinations involve SCAs with PanNETs or ductal adenocarcinoma, and PanNETs with ductal adenocarcinoma (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14). Yan et al. documented a 2 cm SPT of the tail of the pancreas engulfing a 0.7 cm well-differentiated PanNET (22). ...
Solid pseudopapillary tumors (SPT) of the pancreas are rare neoplasms mainly affecting young women. Pancreatic serous cystadenomas (SCAs) and pancreatic neuroendocrine tumors (PanNETs) account for about 2% of all pancreatic neoplasms. The combination of these three lesions, to our knowledge, has never been described in literature. Here we report a case of combined SPT, SCA and PanNET affecting a 33-year-old woman.
... Serous neoplasms should be differentiated from pancreatic pseudo cysts, lymphangiomas, solid pseudo papillary tumours, renal cell carcinoma with clear cells, and clear cell endocrine tumours [7,8]. Pancreatic pseudo cyst is common cystic tumour of pancreas in [ that, the wall show inflammatory cells without epithelial cell lining. ...
Cystic tumours of the pancreas are less common, representing 5% to 10% of all pancreatic neoplasms. They constitute an important
subset because many cystic tumour are either benign or low-grade (indolent) malignant neoplasm. Cystic lesions are also detected more
commonly owing to the increased use of sensitive imaging techniques. A female patient 48-year-old, presented with upper abdominal
symptoms. CT abdomen reveals a large multicystic mass with central scar shows no communication with pancreatic duct. Resected
specimen, show a large lobulated mass with central scar and many small cysts. Microscopic examination confirms the diagnosis of
serous adenoma. PAS positivity is used to demonstrate the glycogen content of the lining epithelium.
... A 10 year retrospective study done on 49 Southeast Indian patients with apparently sporadic HB, found clinical VHL syndrome in ten patients (20 %), with no relevant family history [11]. In case reports, such as that of bilateral multicentric RCC [12], sporadic pancreatic serous cystadenoma associated with neuroendocrine pancreatic carcinoma [13], multi-focal PCC [14], VHL was suspected, if not diagnosed, because of the specific cluster of manifestations occurred. A recent high throughput gene expression profiling study on three Asian Indian subjects with type-2 diabetes mellitus incidentally identified more than three-fold change in the coexpression of VHL compared to controls [15]. ...
The general prevalence of the familial multi-organ tumor disorder, von Hippel-Lindau syndrome (VHL), was estimated to be 1 in 25-40,000 in western studies two decades back. Few studies were done in Indian sub-continent, amidst a surge in clinical reports on VHL specific manifestations. The syndrome is correlated with mutations of the gene VHL (located in Chr 3p25.3). We aimed to conduct a prospective case series describing phenotypic and genotypic characteristics in Indian population. The VHL-specific clinical and radiological features were collected from patients and family members. Genotypic changes such as deletion/duplication or point mutation in the VHL locus were identified using sequencing and MLPA. Thirty-one subjects, from fifteen families with diagnosed VHL, were included in the study. Multicystic pancreas was found in 71 % (22/31), CNS hemangioblastoma in 68 % (21/31), renal cell carcinoma and retinal angiomas in 23 % (7/31) each, pheochromocytoma in 9.7 % (3/31) of the population and endolymphatic sac tumor in one subject. Four families (9 subjects) had full length deletion of VHL, three families (4 subjects) had a deletion of exon 3, eight families (18 subjects) had different exonic, splice-site and intronic point mutations and one subject had a de novo in-frame indel in exon 1. Multicystic pancreas and CNS hemangioblastomas were the most common manifestations in our population. The phenotypic expression patterns in terms of tumorigenesis, tissue tropism and penetrance in comparison to the genotypic features were found to be different from previous correlative studies.
