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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 -
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 -
World Journal of Gastroenterology Dec 2017Pancreatic carcinomas with acinar differentiation are rare, accounting for 1%-2% of adult pancreatic tumors; they include pancreatic acinar cell carcinoma (PACC),... (Review)
Review
Pancreatic carcinomas with acinar differentiation are rare, accounting for 1%-2% of adult pancreatic tumors; they include pancreatic acinar cell carcinoma (PACC), pancreatoblastoma, and carcinomas of mixed differentiation. Patients with PACC have a prognosis better than pancreatic ductal adenocarcinomas but worse than pancreatic neuroendocrine tumors. Reports of overall survival range from 18 to 47 mo. A literature review on PACCs included comprehensive genomic profiling and whole exome sequencing on a series of more than 70 patients as well as other diagnostic studies including immunohistochemistry. Surgical resection of PACC is the preferred treatment for localized and resectable tumors. The efficacy of adjuvant treatment is unclear. Metastatic PACCs are generally not curable and treated with systemic chemotherapy. They are moderately responsive to chemotherapy with different regimens showing various degrees of response in case reports/series. Most of these regimens were developed to treat patients with pancreatic ductal adenocarcinomas or colorectal adenocarcinomas. Review of PACC's molecular profiling showed a number of gene alterations such as: , , , , , , , , , and DNA mismatch repair abnormalities. PACCs had multiple somatic mutations with some targetable with available drugs. Therefore, molecular profiling of PACC should be an option for patients with refractory PACC.
Topics: Antineoplastic Combined Chemotherapy Protocols; Biomarkers, Tumor; Carcinoma, Acinar Cell; Gene Expression Profiling; Humans; Immunohistochemistry; Mutation; Pancreas; Pancreatectomy; Pancreatic Neoplasms; Prognosis; Signal Transduction; Survival Rate; Exome Sequencing
PubMed: 29259370
DOI: 10.3748/wjg.v23.i45.7945 -
The Journal of International Medical... Aug 2021Pancreatoblastoma (PB) is a rare epithelial malignancy usually occurring in the paediatric population. Adult PB is rare, and its imaging findings are similar to those of...
Pancreatoblastoma (PB) is a rare epithelial malignancy usually occurring in the paediatric population. Adult PB is rare, and its imaging findings are similar to those of other tumours, making preoperative diagnosis a considerable challenge. We report correlative ultrasound, contrast-enhanced ultrasonography, contrast-enhanced magnetic resonance imaging, and positron emission tomography-computed tomography findings in a 60-year-old woman with PB. PB often presents with uncommon imaging features and should be considered in the differential diagnosis of pancreatic masses. It is important for clinicians to be aware of these differences to provide effective treatment.
Topics: Adult; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Pancreatic Neoplasms; Ultrasonography
PubMed: 34461770
DOI: 10.1177/03000605211039565 -
Cureus Oct 2023In this report we present a rare case of pancreatoblastoma in an adult patient. Whilst they are amongst the most common malignant pancreatic tumours in children,...
In this report we present a rare case of pancreatoblastoma in an adult patient. Whilst they are amongst the most common malignant pancreatic tumours in children, presentations in adults are exceedingly rare, with a small number of reported cases. Its presentation is often non-specific in terms of clinical examination, and subsequent imaging can show similar findings to those seen in benign neoplasms. This report highlights the difficulty of achieving a diagnosis and subsequent treatment of such an uncommon disease. Biopsy and resultant histology are essential in diagnosis and surgical resection remains the preferred modality of treatment. However, the use of chemotherapy and its efficacy in adults remains unclear, and the prognosis documented in existing literature for adults is worse when compared to paediatric presentations. This case emphasises the need to consider pancreatoblastoma as a differential diagnosis when suspecting pancreatic or abdominal malignancies to achieve early detection and diagnosis, in order to provide optimal treatment and improve patient outcomes.
PubMed: 38046486
DOI: 10.7759/cureus.48063 -
The Turkish Journal of Gastroenterology... Sep 2004In this report we present four cases with solid and cystic papillary neoplasms (SCPN) of the pancreas, and discuss the histopathological and immunohistochemical findings... (Review)
Review
BACKGROUND/AIMS
In this report we present four cases with solid and cystic papillary neoplasms (SCPN) of the pancreas, and discuss the histopathological and immunohistochemical findings with a review of the literature.
METHODS
The four cases reported here consisted of three women (ages 20-48, mean: 32) and one man (age: 58). The clinical diagnoses were confirmed with ultrasound (US) and computerized tomography (CT). The surgical management of the tumors included enucleation (1 patient), distal pancreatectomy with splenectomy (1) and distal pancreatectomy (2).
RESULTS
The tumors were large (mean diameter of the resected tumor was 15 cm), had cystic degenerations between solid areas, and were distributed in the body and the tail of the pancreas. The cystic spaces contained hemorrhagic, necrotic and thrombotic material. The immunohistochemical studies revealed that the four tumors were all positive for a1 antitrypsin and neuron specific enolase, and were all negative for chromogranin. Vimentin and synaptophysin were positive in three different cases. The follow-up of the patients has been uneventful for 2, 1, 7 and 12 years, respectively.
CONCLUSIONS
SCPN of the pancreas is an uncommon clinicopathologic entity with a relatively low grade malignant potential. The majority of the cases are young women. Fifty percent of the cases are asymptomatic, and the patients with symptoms generally suffer from an abdominal mass or abdominal pain. In spite of the characteristic macroscopic and microscopic aspects, the immunophenotypical view is nonspecific. Prognosis is excellent after complete surgical resection and recurrence is rarely seen. Metastatic spread is not expected and the tumor usually has a manner of local invasion. Acinar cell carcinoma, pancreatoblastoma and pancreatic endocrine tumor must be considered in the differential diagnosis.
Topics: Adult; Carcinoma, Papillary; Female; Humans; Male; Middle Aged; Pancreatectomy; Pancreatic Neoplasms; Splenectomy; Tomography, X-Ray Computed; Ultrasonography
PubMed: 15492918
DOI: No ID Found -
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 -
Cancer Cytopathology Nov 2019Pancreatoblastoma (PBL) is a rare malignant pancreatic tumor seen predominantly in childhood, and its cytologic diagnosis remains challenging.
BACKGROUND
Pancreatoblastoma (PBL) is a rare malignant pancreatic tumor seen predominantly in childhood, and its cytologic diagnosis remains challenging.
METHODS
Twelve fine-needle-aspirations from 11 adults were analyzed.
RESULTS
In total, 6 men and 5 women (median age, 45 years; age range, 32-60 years) had tumors measuring a median 5.6 cm (range, 2.5-12 cm) located in the pancreatic head (n = 7) or tail (n = 4), including 3 with familial adenomatous polyposis (FAP)/FAP-related syndromes and 4 with metastasis at diagnosis. The median follow-up was 39.8 months (range, 0.8-348 months), and 5 patients died of disease. The original cytology diagnoses were: PBL (n = 2), neuroendocrine neoplasm (n = 2), poorly differentiated neuroendocrine carcinoma (n = 2), well differentiated neuroendocrine tumor (n = 1), poorly differentiated carcinoma (n = 2), "positive for malignancy" (n = 1), acinar cell carcinoma (n = 1), and epithelioid neoplasm with endocrine and acinar differentiation versus PBL (n = 1). Universal cytopathologic findings included hypercellularity; 3-dimensional clusters; and single, monotonous, blast-like cells that were from 1.5 to 2.0 times the size of red blood cells with high nuclear-to-cytoplasmic ratio, fine chromatin, small, distinct nucleoli, and a resemblance to well differentiated neuroendocrine tumor and poorly differentiated neuroendocrine carcinoma. Branching pseudopapillae (n = 7) and grooved nuclei (n = 3) raised the differential diagnosis of solid-pseudopapillary neoplasm, but with more atypia. Uncommon features included pleomorphism (n = 4) and numerous mitoses (n = 1). Squamoid morules were seen on smears (n = 5) or cell blocks (n = 6) in 70% of patients and were characterized by epithelioid cells with elongated, streaming nuclei, fine chromatin, absent nucleoli, and positive nuclear β-catenin (n = 6 of 8). The median Ki-67 index was 21% (range, 2%-70%), and neuroendocrine marker expression was common (100%), but acinar markers were variable (63%).
CONCLUSIONS
A combination of cytologic findings in PBL, including a predominant population of primitive blast-like cells, subtle squamoid morules, frequent neuroendocrine and variable acinar phenotype, should facilitate accurate cytologic diagnosis and distinction from common mimics.
Topics: Adenomatous Polyps; Adult; Biopsy, Fine-Needle; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Male; Middle Aged; Pancreas; Pancreatic Neoplasms; Tumor Burden
PubMed: 31581358
DOI: 10.1002/cncy.22187 -
JOP : Journal of the Pancreas May 2012Pancreatoblastoma is a rare neoplasm in adults with a total of only 24 cases that have been reported in the literature. Adult pancreatoblastomas are large tumors and... (Review)
Review
CONTEXT
Pancreatoblastoma is a rare neoplasm in adults with a total of only 24 cases that have been reported in the literature. Adult pancreatoblastomas are large tumors and majority are larger than 8 cm at the time of diagnosis. Metastasis is seen in 26% of adults and usually involves the liver and then the lymph nodes. Metastasis is usually observed in cases where the primary tumor measures more than 10 cm. Pancreatoblastoma is named after its resemblance to fetal pancreatic tissue in the seventh week of life. The presence of squamoid corpuscles with a morular appearance is the most characteristic feature of the tumor. Pancreatoblastomas can have mixed features of both endocrine and exocrine cells; however, acinar differentiation is the most prevalent feature.
CASE REPORT
We present a case of a 27-year-old female with a 3.6 cm pancreatoblastoma with metastasis to the liver and lungs as well as to the breast. This case has several distinguishing features from previously reported cases. Such widespread metastasis is unusual given the small size of the primary tumor. Also, metastasis to the breast from a pancreatoblastoma has been previously undescribed in literature. The histological features in our case of pancreatoblastoma were atypical, characterized by the absence of acinar component, supported by the lack of staining for both trypsin and lipase in the tumor, which has not been described in literature. Additionally, the nests of squamous cells in this tumor had a pilomatricoma like morphology as opposed to the morular appearance of the squamoid corpuscles seen in classical cases.
CONCLUSION
Pancreatoblastoma can have an atypical clinical picture and a small primary with extensive metastasis to unusual sites may present a diagnostic challenge. Given its rarity, a high index of suspicion is required to correctly diagnose this condition. The histology reported on this case is unique and has not been reported in the literature.
Topics: Adult; Biomarkers; Fatal Outcome; Female; Humans; Keratins; Neoplasm Metastasis; Pancreatic Neoplasms
PubMed: 22572137
DOI: No ID Found -
Genes Dec 2023Adult pancreatoblastoma (PBL) is a rare pancreatic malignancy, with recent evidence suggesting a possible link to familial adenomatous polyposis (FAP). This study aims... (Review)
Review
BACKGROUND
Adult pancreatoblastoma (PBL) is a rare pancreatic malignancy, with recent evidence suggesting a possible link to familial adenomatous polyposis (FAP). This study aims to review the latest evidence and explore a possible association between adult PBL and FAP.
METHODS
Two independent literature reviews were conducted: (1) on PBL and FAP, and (2) on PBL in the adult population not diagnosed with FAP.
RESULTS
Out of 26 articles on PBL and FAP screened, 5 were selected for systematic review, including 1 additional case. We identified eight FAP-related PBL cases, with a median age of 40 (IQR: 34-50). Of these, seven (87%) occurred in adults. We found 65 cases of adult PBL not FAP-related; thus, 7 out of 65 cases (10.7%) of adult PBL reported in the literature are associated with a clinical diagnosis of FAP or were carriers of germline pathogenic variants (GPVs).
CONCLUSION
Data suggest a non-random association between adult PBL and FAP. Further research is essential to optimise surveillance protocols and develop more effective treatment strategies.
Topics: Adult; Humans; Adenomatous Polyposis Coli; Germ-Line Mutation; Pancreatic Neoplasms
PubMed: 38254934
DOI: 10.3390/genes15010044