Serous Cystadenocarcinoma of the Pancreas: Clinical Features and Management of a Rare Tumor

Dyke, Timothy J Van; Johlin, Frederick C.; Bellizzi, Andrew M.; Howe, James R.

doi:10.1159/000444721

Cited by 15 publications

(8 citation statements)

References 30 publications

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“…There have been a limited number of reports of chemotherapy or radiotherapy for serous cystadenocarcinomas with uncertain results though could be considered in unresectable cases [14]. Yet, the unexpected result of the misjudged treatment that in long period received our patient raises the question of a possible therapeutic effect of the anti-VEGF multi-targeted receptor tyrosine kinase inhibitor (sunitinib) in this type of cancer.…”

Section: Discussionmentioning

confidence: 94%

“…Microscopically, the cells are uniform, round to polygonal, and have clear or pale eosinophilic cytoplasm with well-defined borders. Special stains highlight the abundant intracytoplasmic glycogen and absence of stainable mucin [3,14]. It is noteworthy to mention that in a recent review, Reid et al reported an association of SCNs in resection specimens with other neoplasms of the uninvolved pancreas in 13%.…”

Section: Discussionmentioning

confidence: 99%

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Serous Microcystic Cystadenocarcinoma of the Pancreas with Synchronous Liver Metastases: Clinical Characteristics and Management

Massaras

Pantiora

Koutalas

et al. 2020

Cureus

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Serous cystadenocarcinoma of the pancreas is a rare but well-established entity. The origin and evolution of this disorder remain unclear, but even metastatic cases have an excellent prognosis. These tumors are very similar to benign serous cystic neoplasms (SCNs) of the pancreas, except that they tend to be larger, are locally invasive, and present distant metastasis. The most frequent local invasion is adjacent vessels, spleen, stomach, and duodenum. The most common site of distant metastasis is the liver. Diagnosis via imaging as well as pathology examination may be misguided due to atypical characteristics of the tumor. In fact, in some, the diagnosis of malignancy was established only after metastases were detected. We present a 60year-old female patient with malignant serous microcystic cystadenocarcinoma of the pancreas and liver metastasis that was initially misdiagnosed as a metastatic renal cell carcinoma. The patient underwent tumor resection and liver metastasectomy and she is currently doing well after three years of follow-up, with no tumor recurrence or new metastatic liver nodules based on imaging findings.

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Serous Microcystic Cystadenocarcinoma of the Pancreas with Synchronous Liver Metastases: Clinical Characteristics and Management

Massaras

Pantiora

Koutalas

et al. 2020

Cureus

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“…SCNs have different biological characteristics than MCNs. SCNs are generally benign, with only 1 to 3% malignant potential [3], and can be followed up [5]. Approximately 10% of SCNs are manifested as unilocular without septa, which are easily misdiagnosed as MCNs [13].…”

Section: Discussionmentioning

confidence: 99%

“…SCNs and MCNs have different biological behaviors. Relevant studies have reported that only 1 to 3% of SCNs have been transformed into serous cystadenocarcinomas [3]. To date, only 25–30 serous cystadenocarcinomas have been reported worldwide [4].…”

Section: Introductionmentioning

confidence: 99%

A prospective study on endoscopic ultrasound for the differential diagnosis of serous cystic neoplasms and mucinous cystic neoplasms

et al. 2019

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Background To provide criteria for the differential diagnosis of serous cystic neoplasms (SCNs) and mucinous cystic neoplasms (MCNs) by analyzing the imaging features of these two neoplasms by endoscopic ultrasound (EUS). Methods From April 2015 to December 2017, a total of 69 patients were enrolled in this study. All patients were confirmed to have MCNs (31 patients) or SCNs (38 patients) by surgical pathology. All patients underwent EUS examination. The observation and recorded items were size, location, shape, cystic wall thickness, number of septa, and solid components. Results Head/neck location, lobulated shape, thin wall and > 2 septa were the specific imaging features for the diagnosis of SCNs. When any two imaging features were combined, we achieved the highest area under the curve (Az) (0.824), as well as the appropriate sensitivity (84.2%), specificity (80.6%), positive predictive value (PPV) (84.2%), and negative predictive value (NPV) (80.6%). Body/tail location, round shape, thick wall and 0–2 septa were the specific imaging features for the diagnosis of MCNs. When any three imaging features were combined, we obtained the highest Az value (0.808), as well as the appropriate sensitivity (77.4%), specificity (84.2%), PPV (80.0%) and NPV (82.1%). Conclusions Pancreatic cystadenomas that meet any two of the four imaging features of head/neck location, lobulated shape, thin wall and > 2 septa could be diagnosed as SCNs, and those that meet any three of the four imaging features of body/tail location, round shape, thick wall and 0–2 septa could be considered as MCNs. Trial registration The study was registered at the Chinese Clinical Trial Registry. The registration identification number is ChiCTR-OOC-15006118 . The date of registration is 2015-03-20.

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“…Nevertheless, serous cystadenoma (SCA) may have the potential to progress to serous cystadenocarcinoma (SCAC), a rare diagnosis first described in 1989 [ 2 ]. To date, 35 malignant pancreatic SCN cases (15 with distant metastases) have been reported worldwide [ 3 ]. A recent systematic review showed overall favorable outcome for SCAC with 77% (17/22) alive at a median follow-up of 2 years [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Targetable IDH1 mutation identified in a rare case of pancreatic serous cystadenocarcinoma but not a series of serous cystadenomas

Zhang

Hammonds

Tran-Harding

et al. 2022

Journal of Surgical Case Reports

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Serous cystadenocarcinoma (SCAC) of the pancreas is rare, with only 35 cases reported in the literature. We present a case of SCAC, comparing the clinical presentation, management and molecular features of this case to a series of serous cystadenoma (SCA), which may be a precursor. Compared with SCAs (n = 5), SCAC was larger (11.5 vs median 3.6 cm). The case of SCAC invaded the spleen and exhibited distant metastasis, a requirement for diagnosis since pathologic features are otherwise indistinguishable from SCA. VHL mutations have been reported in about half of SCA in the literature. Accordingly, we identified either somatic or germline VHL mutations in 3 of 5 SCAs (60%), yet no pathogenic mutation was identified in the SCAC. A somatic mutation in IDH1 was found in SCAC only. This has been associated with multiple malignancies, is targetable with the drug ivosidenib and should be studied as a progression factor in SCAC.

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Serous Cystadenocarcinoma of the Pancreas: Clinical Features and Management of a Rare Tumor

Cited by 15 publications

References 30 publications

Serous Microcystic Cystadenocarcinoma of the Pancreas with Synchronous Liver Metastases: Clinical Characteristics and Management

Serous Microcystic Cystadenocarcinoma of the Pancreas with Synchronous Liver Metastases: Clinical Characteristics and Management

A prospective study on endoscopic ultrasound for the differential diagnosis of serous cystic neoplasms and mucinous cystic neoplasms

Targetable IDH1 mutation identified in a rare case of pancreatic serous cystadenocarcinoma but not a series of serous cystadenomas

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