Pancreatoblastoma an Unusual Occurrence of a Tumour in Paediatric Age Group: A Case Report

Halder, Pankaj; Mukhopadhyay, Bedabrata; Das, Chhanda; Mukhopadhyay, Madhumita

doi:10.7860/jcdr/2016/15336.7413

Cited by 1 publication

(1 citation statement)

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“…Montemarano et al suggested that the majority of PBs are large, well-defined, heterogeneous masses that are at least partially circumscribed 17 . Other studies have reported that PB can present with obvious necrosis or even complete cystic degeneration [27][28][29] . However, in our study, six tumors (85.7%) were purely solid and homogeneous, which may be related to the small mean diameter of the included tumors.…”

Section: Discussionmentioning

confidence: 97%

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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