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BMC Medical Imaging Dec 2022This study mainly analysed the imaging data for seven cases of adult pancreatoblastoma (PB) and summarized additional imaging features of this disease based on a... (Review)
Review
OBJECTIVE
This study mainly analysed the imaging data for seven cases of adult pancreatoblastoma (PB) and summarized additional imaging features of this disease based on a literature review, aiming to improve the understanding and diagnosis rate of this disease.
MATERIALS AND METHODS
The imaging data for seven adult patients pathologically diagnosed with adult PB were retrospectively analysed. Among the seven patients, six underwent computed tomography (CT) scans, two patients underwent abdominal magnetic resonance imaging (MRI), and five patients underwent F-FDG PET/CT.
RESULTS
The tumours were located in the head of the pancreas in three cases, in the tail of the pancreas in two cases, and in the gastric antrum and neck of the pancreas in one case. Six tumours showed blurred edges, and an incomplete envelope was observed in only two cases when enhanced, which showed extruded growth and cyst-solid masses; one tumour was a solid mass with ossification. Showing mild or significant enhancement in the arterial phase (AP) for six cases. In the MRI sequence, isointensity was found on suppressed T1-weighted imaging, and hyperintensity was noted on suppressed T2-weighted imaging in two cases, with significant enhancement. Pancreatic duct dilatation was found in four cases. Tumour F-FDG PET/CT imaging exhibited high uptake in five cases.
CONCLUSION
Adult PB involves a single tumour and commonly manifests as cystic-solid masses with blurred edges. Capsules are rare, ossification is an important feature, tumours can also present in ectopic pancreatic tissues, with mild or strengthening in the AP, and F-FDG uptake is high. These features are relatively specific characteristics in adult PB.
Topics: Humans; Adult; Fluorodeoxyglucose F18; Positron Emission Tomography Computed Tomography; Retrospective Studies; Positron-Emission Tomography; Tomography, X-Ray Computed; Pancreatic Neoplasms; Magnetic Resonance Imaging; Radiopharmaceuticals
PubMed: 36581821
DOI: 10.1186/s12880-022-00958-4 -
Gland Surgery Aug 2015Pancreatoblastoma (PB) is a rare malignant neoplasm of the pancreas with unknown etiology. It occurs mostly in the pediatric population with very rare documented cases... (Review)
Review
Pancreatoblastoma (PB) is a rare malignant neoplasm of the pancreas with unknown etiology. It occurs mostly in the pediatric population with very rare documented cases in adults. This is a review of the case reports of the adult pancreatoblastoma in the literature. A total of 35 cases were identified and reviewed with the mean age of 41 years (range, 18-78 years) and the male sex accounted for 51.4% of the cases. Adult Pancreatoblastoma seem to have a predilection for the head of the pancreas which accounted for approximately 49% of the cases reviewed with an average size of 8 cm (range, 1.8-20 cm). The median follow up for patients was 15 months (range, 1-108 months) Metastatic disease and local infiltration of surrounding tissues is common with poor prognosis in adult patients. Preoperative diagnosis is difficult because of the unhelpful tumor markers in adults and the cellular heterogeneity of the tumor which makes fine needle aspiration cytology unreliable. Histopathological review of the tumor is essential for diagnosis. Pancreatoblastomas should be considered a differential diagnosis of solid and cystic pancreatic neoplasms. Surgical resection of the tumor is the treatment of choice with a variable combination with radiotherapy and chemotherapy.
PubMed: 26312218
DOI: 10.3978/j.issn.2227-684X.2015.04.05 -
Archives of Pathology & Laboratory... Jul 2020Pancreatic acinar lesions encompass a broad spectrum of malignant tumors and benign reactive processes affecting both adults and children, with clinical, pathologic, and... (Review)
Review
CONTEXT.—
Pancreatic acinar lesions encompass a broad spectrum of malignant tumors and benign reactive processes affecting both adults and children, with clinical, pathologic, and molecular features that are distinct from more common ductal neoplasms. Accurate morphologic diagnosis and molecular assessment of these uncommon neoplasms is critical for effective patient care.
OBJECTIVE.—
To review the clinical, pathologic, and molecular features of pancreatic neoplasms with acinar differentiation, the most common of which is acinar cell carcinoma but which also includes mixed carcinomas with acinar components, cystic acinar lesions, and pancreatoblastoma.
DATA SOURCES.—
We assessed the current primary literature, as well as recently updated diagnostic manuals.
CONCLUSIONS.—
Pancreatic acinar neoplasms are a morphologically and molecularly heterogeneous group of diseases that are characterized by acinar differentiation of at least a subset of the neoplastic cells, defined either morphologically (granular cytoplasm, single prominent nucleoli) or immunohistochemically. Squamoid nests are a key morphologic feature of pancreatoblastoma. Alterations in WNT signaling and chromosomal 11p loss are common molecular features of both acinar cell carcinoma and pancreatoblastoma. Targetable molecular alterations in acinar carcinoma include BRAF rearrangements and DNA repair defects, including mismatch repair deficiency and BRCA pathway defects. For practicing pathologists, morphologic recognition of such acinar neoplasms is critical, and in the future, molecular diagnostics to identify lesions susceptible to targeted therapy will likely form an important component of patient care.
Topics: Biomarkers, Tumor; Biopsy; Carcinoma, Acinar Cell; Cell Differentiation; Genetic Predisposition to Disease; Humans; Immunohistochemistry; Molecular Diagnostic Techniques; Neoplasms, Complex and Mixed; Pancreatic Neoplasms; Phenotype; Predictive Value of Tests
PubMed: 31869246
DOI: 10.5858/arpa.2019-0472-RA -
Surgical Oncology Clinics of North... Oct 2021Pancreatic ductal adenocarcinoma (PDAC) is the most common type of pancreatic cancer. However, it should be kept in mind that there are other pancreatic cancers that are... (Review)
Review
Pancreatic ductal adenocarcinoma (PDAC) is the most common type of pancreatic cancer. However, it should be kept in mind that there are other pancreatic cancers that are classified by their cellular lineage: acinar cell carcinomas (acinar differentiation), neuroendocrine neoplasms (arising from the islets), solid-pseudopapillary neoplasms (showing no discernible cell lineage), and pancreatoblastomas (characterized by multiphenotypic differentiation, including acinar endocrine and ductal). This article focuses on the molecular and pathology alterations in PDAC.
Topics: Carcinoma, Acinar Cell; Carcinoma, Pancreatic Ductal; Humans; Neuroendocrine Tumors; Pancreatic Neoplasms
PubMed: 34511185
DOI: 10.1016/j.soc.2021.06.003 -
Virchows Archiv : An International... Aug 2022Pancreatoblastoma (PB) is a rare tumor of the pancreas. In case of metastases, the treatment options are sparse and targeted approaches are not developed. We here...
Pancreatoblastoma (PB) is a rare tumor of the pancreas. In case of metastases, the treatment options are sparse and targeted approaches are not developed. We here evaluate MCL1 amplification as a putative target in PB.Thirteen samples from adult (10/13) and pediatric patients (3/13) were collected. Three of these samples had been previously subjected to whole-exome sequencing (2 cases) or whole-genome sequencing (1 case) within a precision oncology program (NCT/DKTK MASTER), and this analysis had shown copy number gains of MCL1 gene. We established a fluorescence in situ hybridization (FISH) test to assess the copy number alterations of MCL1 gene in 13 formalin-fixed paraffin-embedded PBs, including the 3 cases assessed by genome sequencing. FISH analysis showed the amplification of MCL1 in 2 cases (both were adult PB), one of which was a case with the highest copy number gain at genomic analysis. In both cases, the average gene copy number per cell was ≥ 5.7 and the MCL1/1p12 ratio was ≥ 2.4. Our data support MCL1 as a putative target in PB. Patients with MCL1-amplified PB might benefit from MCL1 inhibition. Sequencing data is useful to screen for amplification; however, the established FISH for MCL1 can help to determine the level and cellular heterogeneity of MCL1 amplification more accurately.
Topics: Adult; Child; Gene Amplification; Humans; In Situ Hybridization, Fluorescence; Myeloid Cell Leukemia Sequence 1 Protein; Pancreatic Neoplasms
PubMed: 35668118
DOI: 10.1007/s00428-022-03349-w -
Archives of Pathology & Laboratory... Jul 2006Endocrine tumors of the pancreas represent 1% to 2% of all pancreatic neoplasms. The tumors tend to have an indolent behavior, and long-term survival is common. There is... (Review)
Review
Endocrine tumors of the pancreas represent 1% to 2% of all pancreatic neoplasms. The tumors tend to have an indolent behavior, and long-term survival is common. There is no gender or age predilection. Patients can present with symptoms due to hormonal excess or a local mass effect or be asymptomatic. The tumors tend to be solid and well circumscribed. Typical microscopic findings include an organoid pattern of growth, with cells containing scant to moderate amounts of cytoplasm, and nuclei with dispersed chromatin and inconspicuous nucleoli. The morphologic spectrum of these tumors can be variable, and the differential diagnosis includes chronic pancreatitis with neuroendocrine hyperplasia, ductal adenocarcinoma, solid pseudopapillary tumor, acinar cell carcinoma, and pancreatoblastoma. The classification of these tumors remains controversial, and prognosis is difficult to predict, but important features include metastasis and invasion of adjacent structures. Resection remains the mainstay of surgical treatment. It is important to be aware that unusual morphologic variants of pancreatic endocrine tumors are common, and immunohistochemical stains can help avoid misdiagnosis.
Topics: Adenoma, Islet Cell; Carcinoma, Acinar Cell; Carcinoma, Islet Cell; Carcinoma, Pancreatic Ductal; Carcinoma, Papillary; Diagnosis, Differential; Humans; Islets of Langerhans; Neoplasms, Germ Cell and Embryonal; Pancreatic Neoplasms; Pancreatitis, Chronic; Prognosis
PubMed: 16831051
DOI: 10.5858/2006-130-963-UOPET -
World Journal of Pediatric Surgery 2022
PubMed: 36475046
DOI: 10.1136/wjps-2021-000398 -
Chinese Clinical Oncology Dec 2017As the most common and most important cancer of the pancreas, with rapid mortality and now also as the third leading cause of cancer-related deaths in the United States,... (Review)
Review
As the most common and most important cancer of the pancreas, with rapid mortality and now also as the third leading cause of cancer-related deaths in the United States, pancreatic ductal adenocarcinoma (PDAC) has become synonymous with "pancreas cancer". PDAC is also the prototype of the "pancreatobiliary-type" adenocarcinomas, along the biliary tract, ampullary and gallbladder cancers with the similar morphology and behavior. Recent molecular profiling studies have identified distinct subsets of PDAC, potentially with different behaviors and targetability. Moreover, while PDAC is by far the most common cancer of the pancreas, there are various other types that occur in this organ and are erroneously classified together with PDAC. Many of these have different molecular and biologic characteristics that warrant their management separately although they are also technically "pancreatic cancers". While some are closely related to PDAC and have as aggressive behavior (such as adenosquamous carcinomas which are recently recognized under "basal" like category in profiling studies, which are actually even worse prognostically than PDACs), in the meantime, others such as colloid carcinoma has a much better behavior than PDAC, and as a carcinoma with intestinal lineage (MUC2/CDX2) colloid carcinoma may require an entirely different treatment approach as well. Similarly, medullary carcinomas also appear to have different biology. Additionally, non-ductal cancers such as acinar, neuroendocrine, solid-pseudopapillary neoplasms and pancreatoblastoma have their respective clinicopathologic and molecular associations and warrant careful elimination in the management and study protocols. Another very problematic aspect in the classification of "pancreas cancer" is its delineation from the cancers of neighboring organs, in particular, ampullary/duodenal and common bile duct (CBD) cancers, for which recently more refined criteria have been provided. Additionally, the possibility of metastasis from another site and lymphomas also need to be considered. In summary, there is a whole host of cancers that occur in the pancreas that ought to be considered carefully before a case is classified as an ordinary "pancreas cancer" (PDAC).
Topics: Humans; Male; Pancreatic Neoplasms
PubMed: 29307199
DOI: 10.21037/cco.2017.12.01 -
Cancer Imaging : the Official... Feb 2009'Blastomas' are tumors virtually unique to childhood. Controversy surrounds their nomenclature and there is no globally accepted classification. They are thought to... (Review)
Review
'Blastomas' are tumors virtually unique to childhood. Controversy surrounds their nomenclature and there is no globally accepted classification. They are thought to arise from immature, primitive tissues that present persistent embryonal elements on histology, affect a younger pediatric population and are usually malignant. The 'commoner' blastomas (neuroblastoma, nephroblastoma, hepatoblastoma, medulloblastoma) account for approximately 25% of solid tumors in the pediatric age range. We present examples of the more unusual blastematous pediatric tumors (lipoblastoma, osteoblastoma, chondroblastoma, hemangioblastoma, gonadoblastoma, sialoblastoma, pleuropulmonary blastoma, pancreatoblastoma, pineoblastoma, and medullomyoblastoma) that were recorded in our institution. Although these rare types of blastomas individually account for <1% of pediatric malignancies, collectively they may be responsible for up to 5% of pediatric tumors in a given population of young children. Imaging is often non-specific but plays an important role in their identification, management and follow-up. Some characteristic imaging features at diagnosis, encountered in cases diagnosed and treated in our institution, are described and reviewed.
Topics: Adolescent; Bone Neoplasms; Brain Neoplasms; Child; Child, Preschool; Chondroblastoma; Female; Gonadoblastoma; Hemangioblastoma; Humans; Infant; Infant, Newborn; Lipoma; Male; Medulloblastoma; Osteoblastoma; Pancreatic Neoplasms; Pineal Gland; Pinealoma; Radiography; Salivary Gland Neoplasms; Soft Tissue Neoplasms; Thoracic Neoplasms; Ultrasonography; Young Adult
PubMed: 19237343
DOI: 10.1102/1470-7330.2009.0001 -
Pathologica Feb 2022Pediatric solid neoplasms are rare and very different from those observed in adults. The majority of them are referred to as embryonal because they arise as a result of... (Review)
Review
New insights in gastrointestinal "pediatric" neoplasms in adult patients: pancreatoblastoma, hepatoblastoma and embryonal sarcoma of the liver. A practical approach by GIPPI-GIPAD Groups.
Pediatric solid neoplasms are rare and very different from those observed in adults. The majority of them are referred to as embryonal because they arise as a result of alterations in the processes of organogenesis or normal growth and are characterized by proliferation of primitive cells, reproducing the corresponding tissue at various stages of embryonic development. This review will focus on embryonal gastrointestinal pediatric neoplasms in adult patients, including pancreatoblastoma, hepatoblastoma, and embryonal sarcoma of the liver. Although they are classically considered pediatric neoplasms, they may (rarely) occur in adult patients. Hepatoblastoma represents the most frequent liver neoplasm in the pediatric population, followed by hepatocellular carcinoma and embryonal sarcoma of the liver; while pancreatoblastoma is the most common malignant pancreatic tumor in childhood. Both in children and adults, the mainstay of treatment is complete surgical resection, either up front or following neoadjuvant chemotherapy. Unresectable and/or metastatic neoplasms may be amenable to complete delayed surgery after neoadjuvant chemotherapy. However, these neoplasms display a more aggressive behavior and overall poorer prognosis in adults than in children, probably because they are diagnosed in later stages of diseases.
Topics: Child; Female; Hepatoblastoma; Humans; Liver Neoplasms; Pancreatic Neoplasms; Pregnancy; Sarcoma
PubMed: 35212317
DOI: 10.32074/1591-951X-559