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Annals of Surgery Dec 2023To present comprehensive information on the clinicopathological, molecular, survival characteristics, and quality of life (QOL) after surgery for solid pseudopapillary...
OBJECTIVE
To present comprehensive information on the clinicopathological, molecular, survival characteristics, and quality of life (QOL) after surgery for solid pseudopapillary neoplasm (SPN) of the pancreas in a large cohort after long-term follow-up.
BACKGROUND
SPN is a rare tumor with an uncertain malignant potential, and solid information on long-term prognosis and QOL remains limited.
METHODS
All hospitalized patients with SPNs who underwent surgery between 2001 and 2021 at the Peking Union Medical College Hospital were retrospectively reviewed. The clinicopathological characteristics of the patients were retrieved. A cross-sectional telephone questionnaire was administered to inquire about the QOL. Molecular analyses were performed using whole-exome sequencing.
RESULTS
Exactly 454 patients with SPN were enrolled, of whom 18.5% were males and 81.5% were females. The mean patient age was 31 ± 12 years. In total, 61.3% of the patients had no symptoms. The size of the tumors was 5.38 ± 3.70 cm; 83.4% were solid cystic tumors, and 40.1% had calcifications. The proportions of local resection, distal pancreatectomy with or without splenectomy, and pancreaticoduodenectomy with or without pylorus preservation were 29.7%, 28.9% or 22.9%, and 11% or 6.8%, respectively. Over the years, there has been a significant shift from open to minimally invasive surgery. Among all surgical procedures, pylorus-preserving pancreaticoduodenectomy (PPPD) had the highest incidence of grade 2 to 4 complications (up to 32.3%), compared with 6.7% in distal pancreatectomy ( P < 0.001). Regarding histopathology, tissue invasion, perineural invasion, cancerous microvascular emboli, lymph node metastasis, and distant metastasis were present in 16.5%, 2.2%, 0.7%, 2.0%, and 3.1% of patients, respectively. Sixty patients were lost to follow-up. Sixteen of the 390 patients who underwent resection (4.1%) experienced local recurrence or distant metastasis after surgery. In total, 361 patients responded to the telephone survey. Nearly 80% of patients claimed their QOL was not significantly affected after surgery; however, the remaining 20% complained of lower QOL during 3 to 6 years of follow-up after surgery. No clinicopathological factor could reliably predict clinical recurrence or metastasis after resection. A total of 28 driver genes were detected with mutations in at least 2 tumor samples and the top 3 frequently mutated genes were CTNNB1 , ATRNL1 , and MUC16 .
CONCLUSIONS
This study presented the largest cohort of patients with SPN after surgery from a single center and reported the QOL of these patients. SPN is associated with extremely favorable long-term survival, even in patients with metastasis, and most patients have a good QOL after surgery.
Topics: Male; Female; Humans; Young Adult; Adult; Retrospective Studies; Quality of Life; Treatment Outcome; Cross-Sectional Studies; Pancreatic Neoplasms; Pancreas; Pancreatectomy; Neoplasm Recurrence, Local
PubMed: 37036095
DOI: 10.1097/SLA.0000000000005842 -
Der Pathologe Sep 2021Beyond pancreatic ductal adenocarcinoma, which is by far the most frequent pancreatic neoplasm, a great variety of tumors occur in the pancreas. They include solid and... (Review)
Review
Beyond pancreatic ductal adenocarcinoma, which is by far the most frequent pancreatic neoplasm, a great variety of tumors occur in the pancreas. They include solid and cystic masses and epithelial and nonepithelial neoplasms, and they show a great diversity in their biological behavior, ranging from benign tumors to highly aggressive neoplasms. As examples of rare pancreatic tumors, clinical, morphological, and molecular aspects of acinar cell carcinoma, pancreatoblastoma, solid pseudopapillary neoplasm, and serous cystic neoplasms are presented and discussed.
Topics: Carcinoma, Acinar Cell; Carcinoma, Pancreatic Ductal; Humans; Pancreas; Pancreatic Neoplasms
PubMed: 34402979
DOI: 10.1007/s00292-021-00967-0 -
Monographs in Clinical Cytology 2020Non-ductal tumors of the pancreas are relatively rare tumors and include pancreatic neuroendocrine tumors (PanNETs), poorly differentiated neuroendocrine carcinomas,... (Review)
Review
Non-ductal tumors of the pancreas are relatively rare tumors and include pancreatic neuroendocrine tumors (PanNETs), poorly differentiated neuroendocrine carcinomas, acinar cell carcinoma, solid pseudopapillary neoplasm, and pancreatoblastoma. These tumors have a morphology and biology that is distinct from that of ductal neoplasms of the pancreas. PanNETs are the most common tumors among this group. A brief summary of each tumor is described here with an emphasis on the clinical presentation, cytological features, tumor histology, and immunohistochemical profile. Differential diagnoses for each entity are also discussed.
Topics: Carcinoma, Acinar Cell; Carcinoma, Neuroendocrine; Diagnosis, Differential; Humans; Neuroendocrine Tumors; Pancreas; Pancreatic Neoplasms
PubMed: 32987393
DOI: 10.1159/000455737 -
BMJ Case Reports Dec 2022A man aged above 70 years old with a medical history of ulcerative colitis presented with unintentional weight loss. A pancreatic mass associated with pancreatic duct...
A man aged above 70 years old with a medical history of ulcerative colitis presented with unintentional weight loss. A pancreatic mass associated with pancreatic duct dilatation was detected on imaging procedures. Initial investigations including fine needle aspiration and cytology examination were inconclusive. A diagnosis of intraductal tubulopapillary neoplasm (ITPN) was made with histopathology and immunohistochemistry examination on a surgically resected specimen. Two years after surgery, the patient remained well with no radiological evidence of recurrence.ITPN is a rare pancreatic duct tumour with limited case reports in medical literature. Risk factors are not well established. We report the first case of ITPN occurring in a patient with ulcerative colitis. A typical presentation of this rare tumour is reported to encourage clinicians to consider ITPN in the differential diagnoses of a pancreatic mass.
Topics: Male; Humans; Aged; Carcinoma, Pancreatic Ductal; Colitis, Ulcerative; Neoplasm Recurrence, Local; Pancreatic Neoplasms; Pancreas
PubMed: 36549752
DOI: 10.1136/bcr-2021-246398 -
Der Chirurg; Zeitschrift Fur Alle... Aug 2020
Topics: Carcinoma, Pancreatic Ductal; Humans; Neoplasm Recurrence, Local; Pancreas; Pancreatectomy; Pancreatic Neoplasms
PubMed: 32749507
DOI: 10.1007/s00104-020-01208-5 -
The Surgical Clinics of North America Jun 2020This article outlines the principles behind the management of pancreatic cystic lesions. We outline what the general surgeon needs to know in managing and triaging these... (Review)
Review
This article outlines the principles behind the management of pancreatic cystic lesions. We outline what the general surgeon needs to know in managing and triaging these patients. It is our feeling that the general surgeon is often the first line of evaluation of these complex patients and a working knowledge of the different types of cysts is critical to safe care of the patient.
Topics: Adult; Aged; Cell Transformation, Neoplastic; Diagnosis, Differential; Early Diagnosis; Endosonography; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Pancreas; Pancreatectomy; Pancreatic Cyst; Pancreatic Ducts; Pancreatic Intraductal Neoplasms; Pancreatic Neoplasms; Tomography, X-Ray Computed
PubMed: 32402302
DOI: 10.1016/j.suc.2020.02.006 -
Monographs in Clinical Cytology 2020Inflammatory, developmental, and neoplastic lesions may all present as cystic masses on imaging. Pseudocyst is the most common of these and presents in association with... (Review)
Review
Inflammatory, developmental, and neoplastic lesions may all present as cystic masses on imaging. Pseudocyst is the most common of these and presents in association with a history of pancreatitis. Pancreatic cystic neoplasms are uncommon compared to solid neoplasms. They often present incidentally; therefore, an incidentally discovered cyst in the pancreas should be assessed with a high index of suspicion for neoplasm. The most common and frequently encountered cystic neoplasms include serous cystadenoma, mucinous cystic neoplasm, and intraductal papillary mucinous neoplasm. Less common epithelial cystic neoplasms include acinar cell cystadenoma and cystadenocarcinoma. Any solid neoplasm occurring in the pancreas or vicinity of the pancreas that has undergone cystic degeneration may present as a cystic mass. Non-epithelial lesions, such as lymphangioma, are also included in the differential diagnosis. The work-up needs to begin with a review of the clinical and imaging findings to establish a differential diagnosis. The primary focus of the pathologist will be first on differentiating mucinous from non-mucinous entities, since this will determine if the mass is an intraductal papillary mucinous neoplasm or a mucinous cystic neoplasm. If it is mucinous, the next step is to determine if the cystic neoplasm contains cells with high-grade cytological features. If it is non-mucinous, the pathologist needs to assess for neoplastic cells that would indicate a different neoplastic process. The cytological features need to be integrated with cyst fluid carcinoembryonic antigen and amylase measurements. Currently, molecular pathology is being integrated into the analysis of pancreatic cyst fluids. Here we will cover the cytological features and ancillary findings in cystic masses of the pancreas.
Topics: Cyst Fluid; Cystadenocarcinoma; Diagnosis, Differential; Endosonography; Humans; Pancreas; Pancreatic Cyst; Pancreatic Neoplasms
PubMed: 32987387
DOI: 10.1159/000455735 -
Gastrointestinal Endoscopy Clinics of... Jul 2023The overall prevalence of pancreatic cysts (PCs) is high in the general population. In clinical practice PCs are often incidentally discovered and are classified into... (Review)
Review
The overall prevalence of pancreatic cysts (PCs) is high in the general population. In clinical practice PCs are often incidentally discovered and are classified into benign, premalignant, and malignant lesions according to the World Health Organization. For this reason, in the absence of reliable biomarkers, to date clinical decision-making relies mostly on risk models based on morphological features. The aim of this narrative review is to present the current knowledge regarding PC's morphologic features with related estimated risk of malignancy and discuss available diagnostic tools to minimize clinically relevant diagnostic errors.
Topics: Humans; Pancreatic Neoplasms; Pancreatic Cyst; Biomarkers; Precancerous Conditions
PubMed: 37245940
DOI: 10.1016/j.giec.2023.03.011 -
APMIS : Acta Pathologica,... Dec 2021Aquaporins are water channel proteins facilitating passive transport of water across cellular membranes. Aquaporins are over- or ectopically expressed in a multitude of... (Review)
Review
Aquaporins are water channel proteins facilitating passive transport of water across cellular membranes. Aquaporins are over- or ectopically expressed in a multitude of cancers, including pancreatic ductal adenocarcinoma, which is a highly aggressive cancer with low survival rate. Evidence suggests that aquaporins can affect multiple cellular processes involved in cancer development and progression including epithelial-mesenchymal transition, cellular migration, cell proliferation, invasion, and cellular adhesions. In pancreatic ductal adenocarcinoma, aquaporin-1, aquaporin-3, and aquaporin-5 are overexpressed and have been associated with metastatic processes and poor survival. Thus, aquaporin expression has been suggested as diagnostic markers and therapeutic targets in pancreatic ductal adenocarcinoma.
Topics: Animals; Aquaporins; Biomarkers, Tumor; Carcinoma, Pancreatic Ductal; Cell Movement; Cell Proliferation; Epithelial-Mesenchymal Transition; Humans; Neoplasm Invasiveness; Pancreatic Neoplasms
PubMed: 34582595
DOI: 10.1111/apm.13184 -
Pancreas Oct 2019Vasoactive intestinal peptide-secreting tumors (VIPomas) are a group of rare neuroendocrine tumors, which cause a typical syndrome of watery diarrhea. Most of these... (Review)
Review
Vasoactive intestinal peptide-secreting tumors (VIPomas) are a group of rare neuroendocrine tumors, which cause a typical syndrome of watery diarrhea. Most of these tumors are found in the pancreas and are usually detected at a later stage. Although curative resection is not possible in most of these tumors, both symptom and tumor control can be achieved by a multidimensional approach, to enable a long survival of most patients. There are no clear-cut guidelines for the management of VIPomas because of the rarity of this neoplasm and lack of prospective data. In this review, we discuss the available evidence on the clinical features and management of these rare tumors.
Topics: Diarrhea; Humans; Magnetic Resonance Imaging; Neuroendocrine Tumors; Pancreas; Pancreatic Neoplasms; Survival Analysis; Tomography, X-Ray Computed; Vasoactive Intestinal Peptide; Vipoma
PubMed: 31609932
DOI: 10.1097/MPA.0000000000001402