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]

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]

The characteristic squamoid nest shown in the image is a defining feature of pancreatoblastoma, present in every case. Comment Here Reference: Pancreatoblastoma Back to top [pathologyoutlines.com]

• 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]

  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]

• 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]

• Abdominal Mass

  Imaging studies showed an approximately 7.5-cm, left-sided abdominal mass and multiple metastases in the lung. [ncbi.nlm.nih.gov]

  Children's Memorial Hospital Abstract 8 year old female with palpable abdominal mass Keywords pancreatoblastoma, pancreaticoblastoma, pancreatic blastoma, CT, 812 Publication Date: 2005-06-21 History 8 year old female with palpable abdominal mass Discussion [mirc.luriechildrens.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 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]

• 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]

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]

[…] complete blood count, urine pregnancy test followed with quantitative blood beta-hCG Imaging: transvaginal ultrasound If patient is unstable: IV fluid resuscitation, urgent obstetrics and gynecology consultation If patient is stable: continue diagnostic workup [en.wikipedia.org]

• 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]

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]

(March 2018) Treatment[edit] If the tumor is operable, the first line of therapy should be surgical resection. Then, after surgical resection, adjuvant chemotherapy should be given, even in stage I disease. [en.wikipedia.org]

Conclusion: When disease is localized, the treatment of choice is a complete surgical resection. [mdanderson.elsevierpure.com]

Has a better prognosis than adult pancreatic adenocarcinoma. [radiopaedia.org]

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]

Complete resection of the tumor offers the best prognosis. [primescholars.com]

The biology may help define prognosis and therapy for this kind of tumor. [ncbi.nlm.nih.gov]

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]

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 Pathology is based on specific deficiency or deficiencies. [unboundmedicine.com]

Only about 200 cases reported in the literature Sites About 50% occur in pancreatic head, with the rest relatively equally split between tail and body (Gland Surg 2015;4:322) 1 reported case found in ampulla of Vater (Arch Pathol Lab Med 2003;127:1501) Pathophysiology [pathologyoutlines.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]

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]