Presentation
On univariate analysis, factors associated with a worse prognosis were synchronous (p = 0.05) or metachronous metastases (p < 0.001), non-resectable disease at presentation (p < 0.001) and age > 16 years at time of presentation (p = 0.02). [ncbi.nlm.nih.gov]
Symptoms[edit] Children with pancreatoblastoma rarely present with early-stage disease, instead, most present with locally advanced or metastatic disease. Common presenting symptoms include abdominal pain, emesis, and jaundice. [en.wikipedia.org]
Immune System
- Abdominal Lymphadenopathy
The patient is a 24-yr-old African American woman, who presented with a pancreatic mass, hepatic masses, and abdominal lymphadenopathy. [ncbi.nlm.nih.gov]
Entire Body System
- Fatigue
A 3-year-old boy with nausea and general fatigue was referred to our center. Imaging studies showed an approximately 7.5-cm, left-sided abdominal mass and multiple metastases in the lung. [ncbi.nlm.nih.gov]
Symptoms - Pancreatoblastoma * Asymptomatic in early stages * Upper abdominal pain * Abdominal pain above the bellybutton * Gastrointestinal obstruction * Vomiting * Jaundice * Weight loss * Enlarged abdomen * Abdominal mass * Loss of appetite * Fatigue [checkorphan.org]
They include growth problems (in children), obesity (in adults), hair loss, slow heart rate, low blood sugar, low blood pressure, fatigue, and problems with reproduction. [cancer.gov]
Symptoms include fatigue, weight loss, decrease in sex drive, infertility, sensitivity to cold, decreased facial or body hair in men, and puffiness of the face. Acquired panhypopituitarism can occur as a result of tumors in the pituitary gland. [medicinenet.com]
Symptoms can include one or more of the following: Stomach pain, decreased appetite, nausea and vomiting, and constipation Excessive thirst and urination Fatigue and/or weakness Anemia, meaning weakness from not having enough red blood cells Headache [hormone.org]
- Hunting
[PMID 16185976] Savides TJ, Donohue M, Hunt G, Al-Haddad M, Aslanian H, Ben-Menachem T, et al. EUS-guided FNA diagnostic yield of malignancy in solid pancreatic masses: a benchmark for quality performance measurement. [pancreas.imedpub.com]
- Cushingoid
Figure 1: Clinical image of the child at relapse showing typical Cushingoid features Click here to view Figure 2: Axial (a) and sagittal (b) view of computed tomography images showing the lesion in the uncinate process of pancreas displacing the superior [cancerjournal.net]
Gastrointestinal
- Abdominal Pain
One was a 3.5-year-old boy who presented with abdominal pain. Physical examination demonstrated abdominal masses. Multiple pancreatic and hepatic masses were noted by magnetic resonance imaging. [ncbi.nlm.nih.gov]
Symptoms - Pancreatoblastoma * Asymptomatic in early stages * Upper abdominal pain * Abdominal pain above the bellybutton * Gastrointestinal obstruction * Vomiting * Jaundice * Weight loss * Enlarged abdomen * Abdominal mass * Loss of appetite * Fatigue [checkorphan.org]
- Recurrent Vomiting
Case report: A 7 year old boy presented with recurrent vomiting, weight loss and an abdominal lump. Work up revealed a large tumor arising from the pancreas encasing major vessels and the biopsy was suggestive of a pancreatoblastoma. [hpbonline.org]
Liver, Gall & Pancreas
- Liver Mass
The aspiration smears of the liver mass showed a biphasic tumor composed of bland-appearing primitive spindled stromal fragments with "spider-web"-like long fibrils interconnecting with sharply angulated islands of cohesive epithelium. [ncbi.nlm.nih.gov]
An initial fine needle aspiration of the pancreatic mass was nondiagnostic, and a subsequent fine needle aspiration of a liver mass was read as metastatic acinar cell carcinoma. [springerlink.com]
We report a case of pancreatoblastoma occurring in an adult patient, presenting as a pancreatic head mass with liver metastasis and jaundice. [archivesofpathology.org]
Skin
- Keratosis
Large epithelioid cells islands or whorled nests of spindled cells, as well as keratosis, may be visible. In contrast to the surrounding cells, cells in the squamoid corpuscle has large oval nuclei, with inconspicuous nucleoli. [ijpmonline.org]
Musculoskeletal
- Shoulder Pain
CASE REPORT A 27-year-old woman with a past medical history of iron deficiency anemia from dysfunctional uterine bleeding presented with a 2-month history of pressure like chest pain, shortness of breath, and right shoulder pain. [pancreas.imedpub.com]
Workup
Neonatal workup confirmed the presence of an abdominal mass in the region of the pancreas, but precise anatomic localization was not possible. [ncbi.nlm.nih.gov]
Provides all the necessary tools to make a comprehensive diagnostic workup including data from ancillary techniques and molecular findings whenever appropriate. [books.google.com]
Based on initial findings on contrast-enhanced computed tomography (CT, Figure 1A), additional workup included ultrasound (US, Figure 1B) and MRI (MRI, Figure 1C) through the abdomen. [jaocr.org]
To review the current knowledge on nomenclature, etiology, pathophysiology, clinical presentation, diagnostic workup and management of adult patients w... Meso-Rex bypass for extrahepatic portal vein obstruction in children. [allmedx.com]
The best example clearly is the SPN, in which virtually all cases have the same underlying CTNNB1 mutation and immunohistochemistry for β-catenin is routinely used in the diagnostic workup. [diagnosticpathology.biomedcentral.com]
Biopsy
- Liver Biopsy
The initial liver biopsy diagnosis was metastatic neuroendocrine carcinoma based on morphology and synaptophysin positivity. At pancreatic resection, the diagnostic features of pancreatoblastoma were recognized. [ncbi.nlm.nih.gov]
Therefore, a needle biopsy of the liver mass was obtained and was initially diagnosed as a neuroendocrine carcinoma, consistent with metastasis from a pancreatic primary. [archivesofpathology.org]
Imaging studies showed the presence of an abnormal pancreatic tumour and multiple nodular lesions in the liver, the biopsies from which led to a diagnosis of pancreatoblastoma. [ecancer.org]
Treatment
Surgical resection is the only treatment associated with long-term survival. Chemotherapy may play a role as palliative treatment in advanced disease. [ncbi.nlm.nih.gov]
Conclusion: When disease is localized, the treatment of choice is a complete surgical resection. [mdanderson.elsevierpure.com]
Prognosis
Prognosis The prognosis for pediatric cases is usually good if the tumor is resectable, but recurrences still occur. If unresectable and in the presence of metastasis, pancreatoblastoma has an aggressive course and prognosis is poor. [orpha.net]
The biology may help define prognosis and therapy for this kind of tumor. [ncbi.nlm.nih.gov]
Prognosis: Prognosis of this rare well encapsulated tumour is good in paediatric patients, when resected completely. Prognosis is poorer, when there is metastasis (usually in adult patients) or when the tumour is inoperable. [surgical-pathology.com]
Etiology
Etiology The etiology is unknown but pancreatoblastoma can occur in any part of the pancreas. [orpha.net]
The author review the clinical presentation, etiology, diagnosis, treatment and prognosis of PB in this presentation. [ncbi.nlm.nih.gov]
Epidemiology
Summary Epidemiology The incidence is less than 1/1,000,000 in Europe. Pancreatoblastoma accounts for 0.5% of all pancreatic exocrine tumors and occurs equally in females and males. [orpha.net]
Epidemiology Congenital forms affect both sexes equally and are diagnosed early in childhood. The epidemiology of acquired or secondary forms depends on the underlying cause. [unboundmedicine.com]
Pathophysiology
Pathophysiology Pathology is based on specific deficiency or deficiencies. [unboundmedicine.com]
Trousseau's syndrome and other manifestations of chronic disseminated coagulopathy in patients with neoplasms: Clinical, pathophysiologic, and therapeutic features. Medicine (Baltimore) 1977; 56:1. Shaib W, Deng Y, Zilterman D, Lundberg B, Saif MW. [serena.unina.it]
Trousseau's syndrome and othermanifestations of chronic disseminated coagulopathy in patients withneoplasms: Clinical, pathophysiologic, and therapeutic features.Medicine (Baltimore) 1977; 56:1. [pancreas.imedpub.com]
Pathophysiology The effects of hypopituitarism in children depend on the affected hormones. GH deficiency can result in hypoglycemia and short stature. [emedicine.medscape.com]
Prevention
Prevention - Pancreatoblastoma Not supplied. [checkorphan.org]
Personal hygiene can go a long way towards preventing paraphimosis. If treated quickly, the condition is not serious. [medicalnewstoday.com]
Returning the foreskin to its normal position after pulling it back may help prevent this condition. Circumcision, when done correctly, prevents this condition. Elder JS. Anomalies of the penis and urethra. In: Kliegman RM, Stanton BF, St. [medlineplus.gov]