Adult pancreatoblastoma - Case report and review of literature

Vilaverde, Filipa; Reis, Ana Mafalda; Rodrigues, Pedro; Carvalho, A.; Scigliano, Horácio

doi:10.3941/jrcr.v10i8.2737

Cited by 15 publications

(13 citation statements)

References 23 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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“…Diagnosis with FNA is difficult due to multiple differentiation lines that resemble cytologic features seen in other tumors such as the pancreas' acinar cell carcinoma. Thus, the definitive diagnosis of PB depends on the pathological examination characterized by the presence of squamous cell nests with prominent acinar differentiation and foci of ductal, squamous, and endocrine cells [8,9].…”

Section: Discussionmentioning

confidence: 99%

Adult pancreatoblastoma: A rare malignant tumor of the pancreas

Sánchez-Morales

Payan

Valladares

et al. 2021

Ann Hepatobiliary Pancreat Surg

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It has an overall incidence of 0.004 per 100,000 inhabitants. Its diagnosis with fine-needle aspiration (FNA) is difficult due to multiple differentiation lines present on PB that overlap with other tumors. A 76-year-old male patient presented with jaundice, weight loss of 10 kg in 6 months, and appetite loss. Abdominal computed tomography scan showed a tumor in the pancreas head. Transendoscopic ultrasound with FNA biopsy revealed a malignant epithelial neoplasia compatible with PB with immunohistochemistry CK19 (+), P63(+), synaptophysin (-), and Ki67 50%. The patient underwent surgical resection and adjuvant chemotherapy. Currently, the patient is in follow-up with the latest imaging showing no evidence of disease at 32 months after his initial diagnosis. PB is an uncommon malignant neoplasia with an aggressive behavior. Its diagnostic and therapeutic protocols are unclear. Its preoperative diagnosis may be difficult since its imaging features and serological markers are non-specific. However, FNA may be useful in some situations. Surgical resection is currently the recommended treatment that is associated with the best long-term survival.

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“…15 The presence of squamoid corpuscles, which is characterized by the squamous appearance of plump epithelioid cells and variably sized foci of staining, flattened cells with a whorled nested pattern, is the most distinguishing feature of PB. 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%

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 - Case report and review of literature

Cited by 15 publications

References 23 publications

Adult pancreatoblastoma: clinical features and Imaging findings

Adult pancreatoblastoma: clinical features and Imaging findings

Adult pancreatoblastoma: A rare malignant tumor of the pancreas

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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