Adult pancreatoblastoma: A case report and review of the literature

Zouros, Efstratios; Manatakis, Dimitrios K.; Delis, Spiros; Agalianos, Christos; Triantopoulou, C.; Dervenis, Christos

doi:10.3892/ol.2015.3001

Cited by 23 publications

(29 citation statements)

References 26 publications

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“…Because of the rarity of this tumor, only individual cases of PB have been reported in the clinical or imaging literature, and most of our understanding of the imaging characteristics of adult PB is based on these case reports 5,[9][10][11][12][13][14][15][16] . To the best of our knowledge, we believe that our series of seven patients is the largest of its kind in the radiology literature.…”

Section: Discussionmentioning

confidence: 99%

Adult pancreatoblastoma: clinical features and Imaging findings

Zhang

Wang

et al. 2020

Sci Rep

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The objective of this study was to illustrate the clinical, CT, MRI, and 18 F-FDG PET/CT features of adult pancreatoblastoma, an extremely rare disease. In this study, the clinical and imaging features of seven adult patients with pathologically confirmed pancreatoblastoma were retrospectively analyzed. The following parameters were evaluated: size, location, shape, margination, solid-cystic ratio, CT attenuation values or signal intensity and contrast enhancement pattern. We also analyzed whether abnormal FDG uptake occurred during 18 F-FDG PET/CT imaging. All seven patients were male (mean age 45 years; range 22-65 years). Six tumors were irregular in shape, exogenous, and grew outward from the pancreatic parenchyma, similar to branches growing from a tree trunk (85.7%). The tumor margins were clear in five patients (71.4%), and three tumors (42.9%) were encapsulated. Six tumors (71.4%) were solid, with homogeneous enhancement observed on contrast-enhanced CT and MRI. Dynamic-enhanced CT and MRI showed progressive enhancement for all tumors. On 18 F-FDG PET/CT, one tumor exhibited abnormal FDG uptake, and two tumors exhibited no abnormal uptake (66.7%). In conclusion, adult pancreatoblastoma most commonly occurs in male patients, and it usually appears as an exophytic, irregular, and hypovascular mass with well-defined margins and progressive enhancement on CT and MRI. This type of tumor always grows out of the parenchyma of the pancreas, similar to branches growing outward from a tree trunk. Abbreviations PB Pancreatoblastoma ROI Region of interest TAC Time-density curve SUVmax Maximum standardized uptake value SPN Solid pseudopapillary neoplasm ACC Acinar cell carcinoma Pancreatoblastoma (PB) is a very rare malignant epithelial neoplasm of the pancreas with multiple lines of differentiation. The annual incidence of PB is approximately 0.004 per 100,000 people, and it afflicts individuals of all ages 1. The vast majority of PB has been detected in children, with a median age of 5 years at presentation 2. This tumor type is more common in Asians, and two-thirds of the tumors in the medical literature have presented in patients of Asian descent 3-5. More rarely, PB can occur in adults, and the prognosis is worse for adults than for children 5-9. Therefore, it has been suggested that this tumor type shows biological differences between these two age groups 9. Currently, fewer than 50 adult PB cases have been reported in the literature 10 , and the information on these patients is mainly limited to clinical and pathological characteristics. To the best of our knowledge, a comprehensive analysis of their radiological features has not yet been reported. In this article, we present the cases of seven adult patients with pathology-proven PB who were examined at our institution and characterize the imaging appearance of these extremely rare tumors.

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Section: Discussionmentioning

confidence: 99%

Adult pancreatoblastoma: clinical features and Imaging findings

Zhang

Wang

et al. 2020

Sci Rep

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“…Pancreatoblastoma typically mainly occurs in the pediatric population, with approximately only 40 cases described in adults. 96,97 Prognosis in this small group of patients is dismal, with an mOS of 5 months in the advanced stages. 98 In children, PLADO regimen represents the criterion standard in the firstline setting.…”

Section: Pancreatoblastomamentioning

confidence: 92%

Systemic Chemotherapy for Advanced Rare Pancreatic Histotype Tumors

Brunetti

Aprile²,

Marchetti

et al. 2018

Pancreas

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“…16,17 Immunohistochemical staining may identify tumor cells with acinar differentiation, which are highlighted with trypsin, chymotrypsin, lipase, and esterase. 3,4,11 The ductal components stain with CK7 and CK19, and neuroendocrine differentiation with expression of chromogranin and synaptophysin is also a crucial element of a PB diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…Adult PB typically presents with nonspecific symptoms mostly related to tumor mass effects, which are predominantly abdominal pain, palpable mass, weight loss, obstructive jaundice, and abdominal distention. 3 Compared with pediatric patients, adult patients with PB have a poor prognosis, and it usually exhibits a malignant behavior with high rates of local invasion, recurrence, and occasionally distant metastasis. 4 Surgical resection is considered the chief treatment strategy and the only potentially curative therapy.…”

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confidence: 99%

Complete Surgical Resection and Aggressive Treatment for Liver Metastasis May Be Beneficial to Adult Patients With Pancreatoblastoma: A Case Report and Review of Literature

Yin

Liu

Chou

et al. 2019

International Surgery

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Introduction: Pancreatoblastoma (PB) is a rare pancreatic neoplasm that occurs most in pediatric patients. Here, we report a rare case of adult PB with liver metastasis and review the literature in order to assist clinicians in the management of the disease. Case Presentation: A 27-year-old female patient suffered from postprandial fullness, anorexia, and weight loss in the past 3 months. An abdominal ultrasound and contrast-enhanced computed tomography scans confirmed right abdominal mass with compression of major liver vessels, as well as the P-duct and biliary ducts and causing mild dilatation. Pancreatoduodenectomy was performed and pathologic findings showed typical squamoid corpuscles, which confirmed the diagnosis of PB. The patient was alive and disease-free for 1 year and 10 months until a new metastatic lesion was found. Radiofrequency ablation was arranged as a curative treatment, and no viable tumor or sign of recurrence was found until this paper was submitted. Based on a review of previous case reports, we found adult PB patients with only liver metastasis presented with a smaller tumor size ( P = 0.031), more frequent pancreatic head origin ( P = 0.043), and decreased 1-year mortality ( P = 0.009) compared with patients with other distal metastases. Therefore, we assumed that PB with liver metastasis might present favorable outcome by complete surgical resection or other curative treatment. Conclusion: PB patients with liver metastasis are more likely to show a pancreatic head origin, smaller tumor size, and more favorable outcomes compared with other sites of metastasis. PB should be treated aggressively with surgical resection or other curative treatment as opposed to chemotherapy alone.

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Adult pancreatoblastoma: A case report and review of the literature

Cited by 23 publications

References 26 publications

Adult pancreatoblastoma: clinical features and Imaging findings

Adult pancreatoblastoma: clinical features and Imaging findings

Systemic Chemotherapy for Advanced Rare Pancreatic Histotype Tumors

Complete Surgical Resection and Aggressive Treatment for Liver Metastasis May Be Beneficial to Adult Patients With Pancreatoblastoma: A Case Report and Review of Literature

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