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Pancreatoblastoma


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]
  • Most patients presented with incidental abdominal masses, although pain, weight loss, and obstructive jaundice were present, but rarely.[ncbi.nlm.nih.gov]
  • We report a case of pancreatoblastoma occurring in an adult patient, presenting as a pancreatic head mass with liver metastasis and jaundice.[ncbi.nlm.nih.gov]
  • We describe the 9 infantile (aged 3 mo and younger) and 4 congenital cases previously reported and summarize their clinical presentation and outcome.[ncbi.nlm.nih.gov]
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]
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]
  • The following signs and symptoms may be noted: Failure to thrive (infants) Distended abdomen Abdominal pain, back pain Loss of appetite Weight loss Indigestion Yellowing of skin (jaundice) Nausea and vomiting Dark-colored urine Fatigue (getting tired[dovemed.com]
  • They include yellowing of the skin and eyes, pain in the abdomen and back, weight loss and fatigue. Also, because the pancreas is hidden behind other organs, health care providers cannot see or feel the tumors during routine exams.[icd10data.com]
Swelling
  • On abdominal examination, an 8 cm x 10 cm cystic, non-tender, retroperitoneal swelling was palpable in the left hypochondrium and lumbar region.[tropicalgastro.com]
  • ‘Her stomach swelled up and we took her to Doncaster Royal Infirmary where they told us it was definitely cancer but they weren't sure what kind.[dailymail.co.uk]
  • She complained of a painful swelling in the upper abdomen and had diarrhoea during the month prior to admission. There was no fever, or vomiting followed.[hkjpaed.org]
  • […] attention to common symptoms such as: abdominal or back pain weight loss poor appetite tiredness irritability digestive problems gallbladder enlargement blood clots, deep venous thrombosis ( DVT ), or pulmonary embolism fatty tissue abnormalities diabetes swelling[medicalnewstoday.com]
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]
  • Metastatic lesions in liver and lymphadenopathy have been reported in 35% of cases 3.[radiopaedia.org]
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]
  • A 37-year-old man with a history of abdominal pain, diarrhea, and weight loss was found to have an 8-cm-diameter tumor in the head of the pancreas, a biopsy specimen of which revealed histologically and ultrastructurally typical pancreatoblastoma.[ncbi.nlm.nih.gov]
  • A 11-year-old boy, who had excision of dilated common bile duct with hepaticoduodenostomy when 9 years old was referred to our institute for further management of abdominal pain and steatorrhea.[ncbi.nlm.nih.gov]
  • We report a case of a 3-yr-old boy who presented to with abdominal pain our hospital and a progressive bulge in his right abdomen. Biochemical evaluation and serum levels of tumoral markers were within reference limits.[ncbi.nlm.nih.gov]
  • She received postoperative chemotherapy and went into remission for a few months before presenting with jaundice and abdominal pain due to recurrent, metastatic disease in the liver and porta hepatitis.[ncbi.nlm.nih.gov]
Hepatic Mass
  • Multiple pancreatic and hepatic masses were noted by magnetic resonance imaging. He underwent surgical resection but tumor could not be removed completely, which was confirmed by the pathology.[ncbi.nlm.nih.gov]
  • 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]
  • In spite of adjuvant chemotherapy with Adriamycin and gemcitabine, the patient returned 11 months later with several large hepatic masses, invading the pancreatic head and enlarged tracheobronchial lymph nodes.[ncbi.nlm.nih.gov]
Hepatosplenomegaly
  • Physical examination revealed pale conjunctiva, yellowish skin color and hepatosplenomegaly. Abdominal sonography and CT scan showed dilated common bile duct and a tumor mass about 2 x 2 cm in dimension located a pancreatic head.[ncbi.nlm.nih.gov]
Suggestibility
  • Among the various retroperitoneal organs displaced by the tumor, only the pancreatic tail was inseparable from the mass, suggesting that the pancreatic tail was the origin of the tumor.[ncbi.nlm.nih.gov]
  • These results suggest that this tumour arose from primitive multipotential stem cells, showing exocrine and neuroendocrine differentiation.[ncbi.nlm.nih.gov]
  • Immunocytochemical results suggested epithelial differentiation with positivity to alpha-1-antitrypsin (AAT), keratin, CA19-9, and AFP. No endocrine elements were recognized.[ncbi.nlm.nih.gov]
  • Therefore, these two cases suggest that chemotherapy may be proposed before any attempt at surgical excision. Nevertheless, early consolidation by radical resection or irradiation must be considered.[ncbi.nlm.nih.gov]
  • It is suggested that although there was no change in overall size of the tumour, as assessed by various imaging techniques, the changes in serological markers may indicate that the treatment did in fact cause considerable tumour necrosis, AFP and its[ncbi.nlm.nih.gov]

Workup

  • Provides all the necessary tools to make a comprehensive diagnostic workup including data from ancillary techniques and molecular findings whenever appropriate.[books.google.com]
  • 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]
  • 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]
  • 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]
  • 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]
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]
  • The neuroendocrine-appearing component was positive for synaptophysin, which was also seen in the original liver biopsy and which helped lead to the initial diagnosis of neuroendocrine carcinoma.[archivesofpathology.org]
  • (d) Histological image showing the surgical resection of the pancreatic tumour, the characteristics of which were similar to those observed in the previous liver biopsy (H & E, 200 ).[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]
  • All data about pancreatoblastoma have to be collected in order to choose the treatment to elucidate the molecular pathogenesis of the tumor, to diagnose it early and to develop target-specific treatments.[ncbi.nlm.nih.gov]
  • The combined therapy including the intensive chemotherapy with SCT and the radiation followed by surgical treatment is thought to be effective for the treatment of advanced PB.[ncbi.nlm.nih.gov]
  • We began treatment with chemotherapy (cisplatin, 5FU, vincristine, and doxorubicin), which significantly reduced the tumor burden in both the pancreas and liver.[ncbi.nlm.nih.gov]
  • The treatment of choice is radical resection. Adjuvant chemotherapy or radiotherapy should be considered because of the metastatic potential of the tumour.[ncbi.nlm.nih.gov]

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]
  • At present, the prognosis of such cases is rather poor.[ncbi.nlm.nih.gov]
  • Pancreatoblastoma is one of the pancreatic tumours with a relatively good prognosis. The treatment of choice is complete resection with long-term follow-up aiming to treat any early local recurrence or metastasis.[ncbi.nlm.nih.gov]
  • The biology may help define prognosis and therapy for this kind of tumor.[ncbi.nlm.nih.gov]
  • Unresectable or metastatic tumors have a poor prognosis despite adjuvant chemotherapy or radiotherapy.[ncbi.nlm.nih.gov]

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]
  • Clinical and epidemiological analysis of bone marrow transplantation in a pediatric oncology unit. J Pediatr (Rio J). 2003;79(5):413-22. Portuguese.[scielo.br]
  • Jul 31st, 2019 - To elucidate current epidemiological, clinical profiles, and treatment of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome.[allmedx.com]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • Trousseau's syndrome and other manifestations of chronic disseminated coagulopathy in patients with neoplasms: Clinical, pathophysiologic, and therapeutic features. Medicine (Baltimore) 1977; 56:1. 9.[143.225.131.3]
  • 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 evidence is stronger for a link with heavy drinking, of at least six drinks per day. [3] [43] Pathophysiology [ edit ] Micrograph of pancreatic ductal adenocarcinoma (the most common type of pancreatic cancer).[en.wikipedia.org]

Prevention

  • This has been linked to the displacement of E-cadherin and beta-catenin from their normal membrane location, which prevents adherens junctions to form.[ncbi.nlm.nih.gov]
  • Prevention - Pancreatoblastoma Not supplied.[checkorphan.org]
  • Currently, there are no specific methods or guidelines to prevent Pancreatoblastoma Children with a family history of Beckwith-Wiedemann syndrome or familial polyposis syndrome may benefit from increased monitoring for pancreatic tumors What is the Prognosis[dovemed.com]
  • ., had used oral ketoconazole preoperatively, which prevented postoperative complications in their patient with ectopic ACTH secretion. [13] The stormy postoperative course in our patient could have been avoided if medical therapy was initiated.[cancerjournal.net]

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