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Pancreatology : Official Journal of the... Jan 2021Pancreatoblastoma is a rare pediatric malignant neoplasm characterized by its histological resemblance to fetal pancreatic tissue and poor clinical outcomes....
BACKGROUND
Pancreatoblastoma is a rare pediatric malignant neoplasm characterized by its histological resemblance to fetal pancreatic tissue and poor clinical outcomes. Preoperative diagnosis of the neoplasm is difficult due to its rarity, variable clinical presentation, and its lack of distinct laboratory markers. Current mainstay of treatment is surgical resection of the tumor, although a standard of care has not yet been established.
METHODS
Data were collected on one patient admitted to the University of Virginia Hospital System. Radiology, hematopoietic cell transplant, and biopsy data were collected according to the best clinical practice.
RESULTS
Herein, we describe the case of an adult patient with pancreatoblastoma treated with high-dose chemotherapy and autologous peripheral blood hematopoietic cell transplantation. To the authors' knowledge, this is the first documented successful treatment of pancreatoblastoma using autologous hematopoietic cell transplantation in the United States, and the first successful treatment in an adult patient worldwide.
CONCLUSIONS
While it is difficult to draw conclusions based on a single case, we would like to highlight the success of this treatment modality in the management of our patient with a 51-month remission and open further discussion into exploring the use of autologous hematopoietic cell transplantation for pancreatoblastoma. Our patient is currently living 57 months after diagnosis despite the average survival rate being less than 18 months.
Topics: Antineoplastic Agents; Combined Modality Therapy; Female; Hematopoietic Stem Cell Transplantation; Humans; Neoplasm Metastasis; Neoplasm Recurrence, Local; Pancreatic Neoplasms; Remission Induction; Treatment Outcome; Young Adult
PubMed: 33199137
DOI: 10.1016/j.pan.2020.10.049 -
Pathologica Sep 2020Pancreatic malignant exocrine tumors represent the most important cause of cancer-related death for pancreatic neoplasms. The most common tumor type in this category is... (Review)
Review
Pancreatic malignant exocrine tumors represent the most important cause of cancer-related death for pancreatic neoplasms. The most common tumor type in this category is represented by pancreatic ductal adenocarcinoma (PDAC), an ill defined, stroma-rich, scirrhous neoplasm with glandular differentiation. Here we present the relevant characteristics of the most important PDAC variants, namely adenosquamous carcinoma, colloid carcinoma, undifferentiated carcinoma, undifferentiated carcinoma with osteoclast-like giant cells, signet ring carcinoma, medullary carcinoma and hepatoid carcinoma. The other categories of malignant exocrine tumors, characterized by fleshy, stroma-poor, circumscribed neoplasms, include acinar cell carcinoma (pure and mixed), pancreatoblastoma, and solid pseudopapillary neoplasms. The most important macroscopic, histologic, immunohistochemical and molecular hallmarks of all these tumors, highlighting their key diagnostic/pathological features are presented. Lastly, standardized indications regarding gross sampling and how to compile a formal pathology report for pancreatic malignant exocrine tumors will be provided.
Topics: Adenocarcinoma; Carcinoma, Pancreatic Ductal; Humans; Pancreas; Pancreas, Exocrine; Pancreatic Neoplasms
PubMed: 33179623
DOI: 10.32074/1591-951X-167 -
European Radiology Apr 2021To determine whether features on computed tomographic and/or magnetic resonance imaging can differentiate pancreatoblastoma (PB) from solid pseudopapillary neoplasms...
OBJECTIVES
To determine whether features on computed tomographic and/or magnetic resonance imaging can differentiate pancreatoblastoma (PB) from solid pseudopapillary neoplasms (SPNs) of the pancreas in children.
METHODS
Clinical and imaging data for 20 cases of SPNs and 14 cases of PB confirmed by surgery or biopsy were retrospectively analysed. The size, border, calcification, haemorrhage, solid/cystic component proportion, intratumoural vessels, tumour capsulation, pancreatic duct dilatation, peripancreatic vessel invasion, distant metastasis status and apparent diffusion coefficient (ADC) values of the two groups were examined, and key diagnostic features were identified. Statistical analysis was performed using the chi-square test and Student's t test. Sensitivity and specificity values were calculated when a single criterion was used.
RESULTS
Age ≤ 5 years, elevated serum α-fetoprotein (AFP), larger size, ill-defined border, calcification, absence of haemorrhage, intratumoural vessel, peripancreatic vessel invasion and distant metastasis differentiated PB from SPN (p < 0.05). ADC values of SPN were higher than those of PB (p = 0.001). There were no significant differences regarding tumour capsule (p = 0.435), pancreatic duct dilatation (p = 1.000) or cystic degeneration area over 50% of the tumour volume (p = 1.000) between the two groups.
CONCLUSIONS
The following features are helpful for differentiating PB from SPN: age ≤ 5 years, elevated serum AFP, larger size, ill-defined border, calcification, haemorrhage absence, intratumoural vessel, peripancreatic vessel invasion, distant metastasis and lower ADC value.
KEY POINTS
• CT and MRI are helpful to differentiate pancreatoblastoma (PB) from solid pseudopapillary neoplasms (SPNs) of the pancreas in children. • The following features are helpful to differentiate PB from SPN: age ≤ 5 years, elevated serum AFP, larger size, ill-defined border, calcification, absence of haemorrhage, intratumoural vessel, peripancreatic vessel invasion, distant metastasis and lower ADC value.
Topics: Carcinoma, Papillary; Child; Child, Preschool; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Pancreatic Neoplasms; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 32997172
DOI: 10.1007/s00330-020-07309-3 -
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 -
World Journal of Gastrointestinal... Aug 2020Exocrine pancreatic neoplasms represent up to 95% of pancreatic cancers (PCs) and are widely recognized among the most lethal solid cancers, with a very poor 5-year... (Review)
Review
Exocrine pancreatic neoplasms represent up to 95% of pancreatic cancers (PCs) and are widely recognized among the most lethal solid cancers, with a very poor 5-year survival rate of 5%-10%. The remaining < 5% of PCs are neuroendocrine tumors that are usually characterized by a better prognosis, with a median overall survival of 3.6 years. The most common type of PC is pancreatic ductal adenocarcinoma (PDAC), which accounts for roughly 85% of all exocrine PCs. However up to 10% of exocrine PCs have rare histotypes, which are still poorly understood. These subtypes can be distinguished from PDAC in terms of pathology, imaging, clinical presentation and prognosis. Additionally, due to their rarity, any knowledge regarding these specific histotypes is mostly based on case reports and a small series of retrospective analyses. Therefore, treatment strategies are generally deduced from those used for PDAC, even if these patients are often excluded or not clearly represented in clinical trials for PDAC. For these reasons, it is essential to collect as much information as possible on the management of PC, as assimilating it with PDAC may lead to the potential mistreatment of these patients. Here, we report the most significant literature regarding the epidemiology, typical presentation, possible treatment strategies, and prognosis of the most relevant histotypes among rare PCs.
PubMed: 32879662
DOI: 10.4251/wjgo.v12.i8.833 -
Hepatobiliary & Pancreatic Diseases... Oct 2020
Topics: Antineoplastic Combined Chemotherapy Protocols; Child; Cisplatin; Doxorubicin; Female; Humans; Liver Neoplasms; Liver Transplantation; Neoadjuvant Therapy; Pancreatectomy; Pancreatic Neoplasms
PubMed: 32736920
DOI: 10.1016/j.hbpd.2020.07.003 -
Pediatric Blood & Cancer Sep 2020To better characterize short-term and long-term outcomes in children with pancreatic tumors treated with pancreaticoduodenectomy (PD). (Clinical Trial)
Clinical Trial
BACKGROUND
To better characterize short-term and long-term outcomes in children with pancreatic tumors treated with pancreaticoduodenectomy (PD).
METHODS
Patients 21 years of age or younger who underwent PD at Pediatric Surgical Oncology Collaborative (PSORC) hospitals between 1990 and 2017 were identified. Demographic, clinical information, and outcomes (operative complications, long-term pancreatic function, recurrence, and survival) were collected.
RESULTS
Sixty-five patients from 18 institutions with a median age of 13 years (4 months-22 years) and a median (IQR) follow-up of 2.8 (4.3) years were analyzed. Solid pseudopapillary tumor of the pancreas (SPN) was the most common histology. Postoperative complications included pancreatic leak in 14% (n = 9), delayed gastric emptying in 9% (n = 6), marginal ulcer in one patient, and perioperative (30-day) death due to hepatic failure in one patient. Pancreatic insufficiency was observed in 32% (n = 21) of patients, with 23%, 3%, and 6% with exocrine, or endocrine insufficiencies, or both, respectively. Children with SPN and benign neoplasms all survived. Overall, there were 14 (22%) recurrences and 11 deaths (17%). Univariate analysis revealed non-SPN malignant tumor diagnosis, preoperative vascular involvement, intraoperative transfusion requirement, pathologic vascular invasion, positive margins, and need for neoadjuvant chemotherapy as risk factors for recurrence and poor survival. Multivariate analysis only revealed pathologic vascular invasion as a risk factor for recurrence and poor survival.
CONCLUSION
This is the largest series of pediatric PD patients. PD is curative for SPN and benign neoplasms. Pancreatic insufficiency is the most common postoperative complication. Outcome is primarily associated with histology.
Topics: Adolescent; Adult; Child; Child, Preschool; Exocrine Pancreatic Insufficiency; Female; Humans; Infant; Male; Pancreatic Neoplasms; Pancreaticoduodenectomy; Postoperative Complications
PubMed: 32658372
DOI: 10.1002/pbc.28425 -
Scientific Reports Jul 2020The objective of this study was to illustrate the clinical, CT, MRI, and F-FDG PET/CT features of adult pancreatoblastoma, an extremely rare disease. In this study, the...
The objective of this study was to illustrate the clinical, CT, MRI, and F-FDG PET/CT features of adult pancreatoblastoma, an extremely rare disease. In this study, the clinical and imaging features of seven adult patients with pathologically confirmed pancreatoblastoma were retrospectively analyzed. The following parameters were evaluated: size, location, shape, margination, solid-cystic ratio, CT attenuation values or signal intensity and contrast enhancement pattern. We also analyzed whether abnormal FDG uptake occurred during F-FDG PET/CT imaging. All seven patients were male (mean age 45 years; range 22-65 years). Six tumors were irregular in shape, exogenous, and grew outward from the pancreatic parenchyma, similar to branches growing from a tree trunk (85.7%). The tumor margins were clear in five patients (71.4%), and three tumors (42.9%) were encapsulated. Six tumors (71.4%) were solid, with homogeneous enhancement observed on contrast-enhanced CT and MRI. Dynamic-enhanced CT and MRI showed progressive enhancement for all tumors. On F-FDG PET/CT, one tumor exhibited abnormal FDG uptake, and two tumors exhibited no abnormal uptake (66.7%). In conclusion, adult pancreatoblastoma most commonly occurs in male patients, and it usually appears as an exophytic, irregular, and hypovascular mass with well-defined margins and progressive enhancement on CT and MRI. This type of tumor always grows out of the parenchyma of the pancreas, similar to branches growing outward from a tree trunk.
Topics: Adult; Aged; Contrast Media; Fluorodeoxyglucose F18; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Male; Middle Aged; Pancreatic Neoplasms; Positron Emission Tomography Computed Tomography; Retrospective Studies; Tomography, X-Ray Computed; Young Adult
PubMed: 32647222
DOI: 10.1038/s41598-020-68083-2 -
World Neurosurgery Oct 2020Pancreatoblastoma is an extremely rare neoplasm that accounts for 0.5% of all pancreatic exocrine tumors. These rare entities typically manifest in the pediatric...
BACKGROUND
Pancreatoblastoma is an extremely rare neoplasm that accounts for 0.5% of all pancreatic exocrine tumors. These rare entities typically manifest in the pediatric population but can rarely occur in adults. Systemic seeding has been described before but intracranial metastasis in adults has yet to be described.
CASE DESCRIPTION
A 28-year-old woman with a history of pancreatoblastoma that had been in remission for 51 months after treatment with cisplatin, doxorubicin (Adriamycin), and etoposide had presented to the emergency room with chronic recurrent headaches. Conservative management of the headaches failed, which led to a diagnostic workup with magnetic resonance imaging of the brain. Magnetic resonance imaging demonstrated a well-circumscribed solitary cerebellar lesion. Metastatic disease was suspected, and the patient underwent suboccipital craniotomy for tumor resection with adjuvant gamma knife radiosurgery.
CONCLUSIONS
Central nervous system seeding of pancreatoblastoma is rare, and the available evidence suggests that the strategy we used could be adequate for treating such occurrences.
Topics: Adult; Cerebellar Neoplasms; Craniotomy; Female; Humans; Neoplasm Seeding; Pancreatic Neoplasms; Radiosurgery
PubMed: 32622062
DOI: 10.1016/j.wneu.2020.06.210 -
Modern Pathology : An Official Journal... Sep 2020There is now evidence that gene fusions activating the MAPK pathway are relatively common in pancreatic acinar cell carcinoma with potentially actionable BRAF or RET...
There is now evidence that gene fusions activating the MAPK pathway are relatively common in pancreatic acinar cell carcinoma with potentially actionable BRAF or RET fusions being found in ~30%. We sought to investigate the incidence of RAF1 fusions in pancreatic malignancies with acinar cell differentiation. FISH testing for RAF1 was undertaken on 30 tumors comprising 25 'pure' acinar cell carcinomas, 2 mixed pancreatic acinar-neuroendocrine carcinomas, 1 mixed acinar cell-low grade neuroendocrine tumor and 2 pancreatoblastomas. RAF1 rearrangements were identified in 5 cases and confirmed by DNA and RNA sequencing to represent oncogenic fusions (GATM-RAF1, GOLGA4-RAF1, PDZRN3-RAF1, HERPUD1-RAF1 and TRIM33-RAF1) and to be mutually exclusive with BRAF and RET fusions, as well as KRAS mutations. Large genome-wide copy number changes were common and included 1q gain and/or 1p loss in all five RAF1 FISH-positive acinar cell carcinomas. RAF1 expression by immunohistochemistry was found in 3 of 5 (60%) of fusion-positive cases and no FISH-negative cases. Phospho-ERK1/2 expression was found in 4 of 5 RAF1-fusion-positive cases. Expression of both RAF1 and phospho-ERK1/2 was heterogeneous and often only detected at the tumor-stroma interface, thus limiting their clinical utility. We conclude that RAF1 gene rearrangements are relatively common in pancreatic acinar cell carcinomas (14.3% to 18.5% of cases) and can be effectively identified by FISH with follow up molecular testing. The combined results of several studies now indicate that BRAF, RET or RAF1 fusions occur in between one third and one-half of these tumors but are extremely rare in other pancreatic malignancies. As these fusions are potentially actionable with currently available therapies, a strong argument can be made to perform FISH or molecular testing on all pancreatic acinar cell carcinomas.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Carcinoma, Acinar Cell; Databases, Factual; Female; Gene Fusion; Gene Rearrangement; Humans; Male; Middle Aged; Pancreatic Neoplasms; Proto-Oncogene Proteins c-raf; Young Adult
PubMed: 32358589
DOI: 10.1038/s41379-020-0545-9