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Radiographics : a Review Publication of... 2021A wide spectrum of hereditary syndromes predispose patients to distinct pancreatic abnormalities, including cystic lesions, recurrent pancreatitis, ductal...
A wide spectrum of hereditary syndromes predispose patients to distinct pancreatic abnormalities, including cystic lesions, recurrent pancreatitis, ductal adenocarcinoma, nonductal neoplasms, and parenchymal iron deposition. While pancreatic exocrine insufficiency and recurrent pancreatitis are common manifestations in cystic fibrosis and hereditary pancreatitis, pancreatic cysts are seen in von Hippel-Lindau disease, cystic fibrosis, autosomal dominant polycystic kidney disease, and McCune-Albright syndrome. Ductal adenocarcinoma can be seen in many syndromes, including Peutz-Jeghers syndrome, familial atypical multiple mole melanoma syndrome, Lynch syndrome, hereditary breast and ovarian cancer syndrome, Li-Fraumeni syndrome, and familial pancreatic cancer syndrome. Neuroendocrine tumors are commonly seen in multiple endocrine neoplasia type 1 syndrome and von Hippel-Lindau disease. Pancreatoblastoma is an essential component of Beckwith-Wiedemann syndrome. Primary hemochromatosis is characterized by pancreatic iron deposition. Pancreatic pathologic conditions associated with genetic syndromes exhibit characteristic imaging findings. Imaging plays a pivotal role in early detection of these conditions and can positively affect the clinical outcomes of those at risk for pancreatic malignancies. Awareness of the characteristic imaging features, imaging-based screening protocols, and surveillance guidelines is crucial for radiologists to guide appropriate patient management. RSNA, 2021.
Topics: Genetic Predisposition to Disease; Humans; Multiple Endocrine Neoplasia Type 1; Neoplastic Syndromes, Hereditary; Pancreas; Pancreatic Neoplasms
PubMed: 34143711
DOI: 10.1148/rg.2021200164 -
The Gulf Journal of Oncology May 2023Pancreatoblastoma is a rare malignant epithelial neoplasm of the pancreas. It primarily occurs in the pediatric population and is extremely uncommon in adults. A...
Pancreatoblastoma is a rare malignant epithelial neoplasm of the pancreas. It primarily occurs in the pediatric population and is extremely uncommon in adults. A 64-year-old male patient with no known systemic disease presented to our clinic with abdominal pain and dyspeptic complaints. On physical examination, a tender epigastric mass was palpated. The patient was operated on with a preliminary diagnosis of gastrointestinal stromal tumor. Enbloc resection of the mass was performed. The transverse colon was segmentally resected with wedge resection of the gastric corpus. A stapled side-to-side anastomosis was performed. The macroscopic examination of the case revealed a tumoral lesion of approximately 16x13.5x10m, located in the submucosal area between the gastric corpus and the transverse colon. The microscopic examination showed acini, which have a highly cellular appearance, contain areas of necrosis, and form nested structures in places, stratification in places. The immunohistochemical examination demonstrated positive Trypsin expression, while focal positive expression of neuroendocrine markers such as Synaptophysin, Chromogranin, and Insulinomaassociated protein 1 (INSM-1) was observed. In betacatenin staining, aberrant nuclear and cytoplasmic positive expression was observed, and this staining pattern and morphology confirmed the diagnosis of pancreatoblastoma. Pathological Stage:pT3,N0,Mx the patient had an uneventful postoperative period and was referred to the oncology department for adjuvant chemotherapy. Pancreatoblastoma is an extremely rare type of pancreatic cancer and there are no established guidelines for the treatment of this aggressive disease. Surgical resection is recommended if anatomically possible. Pancreatoblastoma should be considered in the differential diagnosis of asymptomatic masses containing cystic-solid components and reaching very large sizes. Key words: Pancreas,Rare tumor, Pancreatoblastoma.
Topics: Male; Adult; Humans; Child; Middle Aged; Pancreatic Neoplasms; Chemotherapy, Adjuvant; Diagnosis, Differential
PubMed: 37283265
DOI: No ID Found -
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 -
Seminars in Diagnostic Pathology Sep 2016Our understanding about the family of acinar neoplasms of the pancreas has grown substantially over the past 25 years. The prototype is acinar cell carcinoma, an... (Review)
Review
Our understanding about the family of acinar neoplasms of the pancreas has grown substantially over the past 25 years. The prototype is acinar cell carcinoma, an uncommon variant of pancreatic carcinoma that demonstrates production of pancreatic exocrine enzymes, verifiable using immunohistochemistry, and exhibits characteristic histologic features. Related neoplasms include mixed acinar carcinomas such as mixed acinar neuroendocrine carcinoma and mixed acinar ductal carcinoma. In the pediatric age group, pancreatoblastoma is also closely related. Cystic and extrapancreatic forms have been described. These neoplasms share molecular alterations that are distinct from the more common ductal and neuroendocrine neoplasms of the pancreas. Although there is a broad range of genetic findings, a number of potential therapeutic targets have emerged. This review explores the clinical and pathologic features of pancreatic acinar neoplasms along with their more common molecular phenotypes. The differential diagnosis with other pancreatic neoplasms is explored as well.
Topics: Acinar Cells; Carcinoma, Acinar Cell; Humans; Pancreatic Neoplasms; Research
PubMed: 27320062
DOI: 10.1053/j.semdp.2016.05.009 -
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 -
Pediatric Reports May 2024Complete surgical resection in the context of a multimodal approach has been associated with excellent long-term survival in children diagnosed with pancreatoblastoma... (Review)
Review
Complete surgical resection in the context of a multimodal approach has been associated with excellent long-term survival in children diagnosed with pancreatoblastoma (PB). Traditionally, curative intent surgery for PB implies standard pancreatic resections such as pancreaticoduodenectomies and distal pancreatectomies with splenectomies, surgical procedures that may lead to significant long-term pancreatic functional deficiencies. Postoperative pancreatic functional deficiencies are particularly interesting to children because they may interfere with their development, considering their long life expectancy and the significant role of pancreatic functions in their nutritional status and growth. Thus, organ-sparing pancreatectomies, such as spleen-preserving distal pancreatectomies and central pancreatectomies, are emerging in specific tumoral pathologies in children. However, data about organ-sparing pancreatectomies' potential role in curative-intent PB surgery in children are scarce. Based on the literature data, the current review aims to present the early and late outcomes of pancreatectomies in children (including long-term deficiencies and their potential impact on the development and quality of life), particularly for PB, and further explore the potential role of organ-sparing pancreatectomies for PB. Organ-sparing pancreatectomies are associated with better long-term pancreatic functional outcomes, particularly central pancreatectomies, and have a reduced impact on children's development and quality of life without jeopardizing their oncological safety. The long-term preservation of pancreatic functions should not be disregarded when performing pancreatectomies for PB in children. A subset of patients with PB might benefit from organ-sparing pancreatectomies, particularly from central pancreatectomies, with the same oncological results as standard pancreatectomies but with significantly less impact on long-term functional outcomes.
PubMed: 38804376
DOI: 10.3390/pediatric16020033 -
Journal of Gastrointestinal Surgery :... Dec 2023
Topics: Adult; Humans; Pancreatic Neoplasms
PubMed: 37620658
DOI: 10.1007/s11605-023-05816-4 -
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 -
Journal of Gastrointestinal Cancer Sep 2018
Review
Topics: Adult; Humans; Male; Pancreatic Neoplasms
PubMed: 28194556
DOI: 10.1007/s12029-017-9925-x -
The Indian Journal of Radiology &... Jul 2021We describe the sonography and computed tomography (CT) findings in three children with pancreatoblastoma and highlight the tendency of these tumors to invade the...
We describe the sonography and computed tomography (CT) findings in three children with pancreatoblastoma and highlight the tendency of these tumors to invade the splenoportal axis. All three of our cases showed tumor thrombus within the portal vein which is a helpful diagnostic feature on imaging studies.
PubMed: 34790306
DOI: 10.1055/s-0041-1735914