Pancreatoblastoma Research Articles

Proteomic basis for pancreatic acinar cell carcinoma and pancreatoblastoma as similar yet distinct entities

Acinar cell carcinoma (ACC) and pancreatoblastoma (PBL) are rare pancreatic malignancies with acinar differentiation. Proteogenomic profiling of ACC and PBL revealed distinct protein expression patterns compared to pancreatic ductal adenocarcinoma (PDAC) and benign pancreas. ACC and PBL exhibited similarities, with enrichment in proteins related to RNA processing, chromosome organization, and the mitoribosome, while PDACs overexpressed proteins associated with actin-based processes, extracellular matrix, and immune-active stroma. Pathway activity differences in metabolic adaptation, epithelial-to-mesenchymal transition, and DNA repair were characterized between these diseases. PBL showed upregulation of Wnt-CTNNB1 and IGF2 pathways. Seventeen ACC-specific proteins suggested connections to metabolic diseases with mitochondrial dysfunction, while 34 PBL-specific proteins marked this pediatric cancer with an embryonic stem cell phenotype and alterations in chromosomal proteins and the cell cycle. This study provides novel insights into the proteomic landscapes of ACC and PBL, offering potential targets for diagnostic and therapeutic development.

Comparison between pancreatoblastoma (PB) and solid pseudopapillary neoplasm (SPN) in pediatric patients with enhanced CT

Rationale and objectivesTo investigate whether computed tomography features can differentiate pancreatoblastoma (PB) from solid pseudopapillary tumor (SPN) in children. Materials and methodsClinical and imaging data of 18 cases of PB and 61 cases of SPN confirmed by surgery or biopsy were retrospectively analyzed. All enrolled patients underwent 3 phases (non-contrast, arterial, and portal venous phases) of CT scanning. Qualitative CT analysis (location, margin, solid/cystic component proportion, calcification, hemorrhage, peritumoral vascularity, bile duct dilatation, pancreatic duct dilatation, pancreatic atrophy, vascular invasion, peripancreatic invasion, and distant metastases) and quantitative analysis (maximum tumor diameter, interface between tumor and parenchyma [delta], arterial enhancement ratio [AER], and portal enhancement ratio [PER]) were performed. The general CT morphologic features, age and tumor markers were compared also compared between the groups. Univariate analysis and the F test were conducted to identify features of PB. Then logistic Regression classifier was trained using the top five features with the highest F-value. Moreover, we used 5-fold cross-validation techniques for the validation of our model. ResultsPB exhibited a significantly higher frequency of location in the body/tail, larger tumor size, poorly defined margins, calcification, peritumoral vascularity, pancreatic atrophy, and less hemorrhage. In addition, PB had higher AER, PER and lower delta relative to SPN (p < 0.05). PB presented a younger age and higher levels of AFP. Results of the F test indicated that AFP, AER, Age, calcification and pancreatic atrophy were the top five features included in the model that could differentiate pediatric PB from SPN. The combined model of CT and clinical features performed well in differentiating PB from SPN, with an AUC of 0.981 in the training cohort and 0.953 in the validation cohort. ConclusionsAFP, AER, age, calcification and pancreatic atrophy are robust CT and clinical features for differentiating pediatric PB from SPN. A combination of qualitative and quantitative CT features may provide good diagnostic accuracy in differentiating PB from SPN in children.

Diagnosis, treatment, and prognosis of adult pancreatoblastoma.

Pancreatoblastoma (PB) is one of the rare malignant tumors that typically occurs in children. Cases of PB in adults are highly unusual. This disease often presents with subtle symptoms and lacks characteristic clinical manifestations, leading to diagnostic challenges. This study integrates the relevant literature on adult PB, conducting data analysis on clinical features, laboratory and imaging results, pathological characteristics, and treatments according to inclusion and exclusion criteria. Kaplan-Meier univariate analysis and Log-rank tests are employed to analyze survival data from adult PB follow-up, exploring factors influencing prognosis. A total of 65 articles were included, encompassing 103 cases of adult PB. The average age of PB patients was 41.78 years (range 19-81 years), and the male-to-female ratio was 1.06:1. Patients frequently presented with abdominal pain as the initial symptom. Laboratory results lacked specificity and imaging findings often presented as large, well-defined masses. PB exhibited distinctive pathological features, including squamous corpuscles (n = 76, 89.41%) and acinar differentiation (n = 34, 40%), with frequent positive expression of Trypsin, Chymotrypsin, and AACT (Alpha-1-Antichymotrypsin). APC (Adenomatous Polyposis Coli) gene mutation was the most common molecular alteration in adult PB. During the follow-up period, 43.59% of patients died (range 3 days to 348 months). The primary factors affecting prognosis were the presence of metastasis (χ2 = 3.996, p = 0.046) and incomplete surgical resection (χ2 = 5.586, p = 0.018), with mean survival times of 48 months and 27 months, respectively. PB in adults is an invasive tumor. The key to distinguishing PB from other pancreatic tumors lies in recognizing its unique pathological feature, the squamous corpuscles. Timely and complete surgical resection is the preferred treatment following diagnosis. Patients with incomplete resection or the presence of lymph nodes or (and) distant metastases have a poor prognosis.

Paediatric pancreatic acinar cell carcinoma with a novel SEC31A-BRAF fusion gene.

Paediatric pancreatic acinar cell carcinoma (PACC) presents a diagnostic challenge, often confused with pancreatoblastoma (PB) due to its rarity. It is crucial to differentiate between PB and PACC, given their distinct therapeutic strategies and prognoses. Histologically, the absence of squamoid nests and scarcity of tumor mesenchyme support PACC. Conversely, the identification of a BRAF alteration leans towards PACC. Here, we present the case of an 8-year-old girl with a well-defined mass in the pancreas. The tumor exhibited a SEC31A-BRAF fusion gene and amplification of 18p, showcasing unequivocal acinar differentiation and a minor degree of neuroendocrine differentiation. Additionally, the tumor displayed scant fibrous stroma, and an absence of squamoid nests, further supporting PACC. Notably, this is the first reported instance of a solid tumor featuring a SEC31A-BRAF gene fusion. The discovery of this novel fusion gene expands our understanding of BRAF fusion partner profiles, particularly in the context of paediatric PACC.

Preservation of Pancreatic Function Should Not Be Disregarded When Performing Pancreatectomies for Pancreatoblastoma in Children.

Complete surgical resection in the context of a multimodal approach has been associated with excellent long-term survival in children diagnosed with pancreatoblastoma (PB). Traditionally, curative intent surgery for PB implies standard pancreatic resections such as pancreaticoduodenectomies and distal pancreatectomies with splenectomies, surgical procedures that may lead to significant long-term pancreatic functional deficiencies. Postoperative pancreatic functional deficiencies are particularly interesting to children because they may interfere with their development, considering their long life expectancy and the significant role of pancreatic functions in their nutritional status and growth. Thus, organ-sparing pancreatectomies, such as spleen-preserving distal pancreatectomies and central pancreatectomies, are emerging in specific tumoral pathologies in children. However, data about organ-sparing pancreatectomies' potential role in curative-intent PB surgery in children are scarce. Based on the literature data, the current review aims to present the early and late outcomes of pancreatectomies in children (including long-term deficiencies and their potential impact on the development and quality of life), particularly for PB, and further explore the potential role of organ-sparing pancreatectomies for PB. Organ-sparing pancreatectomies are associated with better long-term pancreatic functional outcomes, particularly central pancreatectomies, and have a reduced impact on children's development and quality of life without jeopardizing their oncological safety. The long-term preservation of pancreatic functions should not be disregarded when performing pancreatectomies for PB in children. A subset of patients with PB might benefit from organ-sparing pancreatectomies, particularly from central pancreatectomies, with the same oncological results as standard pancreatectomies but with significantly less impact on long-term functional outcomes.

ABCD1 as a Novel Diagnostic Marker for Solid Pseudopapillary Neoplasm of the Pancreas.

The diagnosis of solid pseudopapillary neoplasm of the pancreas (SPN) can be challenging due to potential confusion with other pancreatic neoplasms, particularly pancreatic neuroendocrine tumors (NETs), using current pathological diagnostic markers. We conducted a comprehensive analysis of bulk RNA sequencing data from SPNs, NETs, and normal pancreas, followed by experimental validation. This analysis revealed an increased accumulation of peroxisomes in SPNs. Moreover, we observed significant upregulation of the peroxisome marker ABCD1 in both primary and metastatic SPN samples compared with normal pancreas and NETs. To further investigate the potential utility of ABCD1 as a diagnostic marker for SPN via immunohistochemistry staining, we conducted verification in a large-scale patient cohort with pancreatic tumors, including 127 SPN (111 primary, 16 metastatic samples), 108 NET (98 nonfunctional pancreatic neuroendocrine tumor, NF-NET, and 10 functional pancreatic neuroendocrine tumor, F-NET), 9 acinar cell carcinoma (ACC), 3 pancreatoblastoma (PB), 54 pancreatic ductal adenocarcinoma (PDAC), 20 pancreatic serous cystadenoma (SCA), 19 pancreatic mucinous cystadenoma (MCA), 12 pancreatic ductal intraepithelial neoplasia (PanIN) and 5 intraductal papillary mucinous neoplasm (IPMN) samples. Our results indicate that ABCD1 holds promise as an easily applicable diagnostic marker with exceptional efficacy (AUC=0.999, sensitivity=99.10%, specificity=100%) for differentiating SPN from NET and other pancreatic neoplasms through immunohistochemical staining.

Aggressive Adult Pancreatoblastoma with Early Recurrence: A Case Report

Background: Pancreatoblastoma (PB) is a rare malignant epithelial tumor of the pancreas, mainly occurring in pediatric population. Pancreatoblastoma of the adult is extremely rare and usually shows a more aggressive biological behavior and a worse prognosis. Case Presentation: The patient was a 54-year-old man admitted for abdominal pain and weight loss. A computed tomography (CT) scan showed a 12 cm heterogeneous hypoattenuating mass in the pancreatic head. An endoscopic ultrasound-guided biopsy suggested the diagnosis of pancreatoblastoma (PB). The patient underwent a pancreaticoduodenectomy (PD) combined with limited liver resections for intraoperative finding of multiple liver metastasis. Pathological findings confirmed the diagnosis of PB metastatic to the liver. The tumor recurred in the liver 5 months after surgery and the patient succumbed due to tumor dissemination 8 months after initial diagnosis. Conclusion: Adult pancreatoblastoma is a rare pancreatic neoplasm with malignant behavior. The aim of this manuscript is to report our experience with pancreatoblastoma in the adult and to contribute improving knowledge about the clinical behavior of this rare and aggressive malignancy.

Rare Non-Neuroendocrine Pancreatic Tumours

The most common tumour of the pancreas is ductal adenocarcinoma (PDAC). It remains one of the most lethal non-neuroendocrine solid tumours despite the use of a multi-approach strategy. Other, less-common neoplasms, which are responsible for 15% of pancreatic lesions, differ in treatment and prognosis. Due to the low incidence rate, there is a lack of information about the rarest pancreatic tumours. In this review, we described six rare pancreatic tumours: intraductal papillary mucinous neoplasm (IPMN), mucinous cystadenoma (MCN), serous cystic neoplasm (SCN), acinar cell carcinoma (ACC), solid pseudopapillary neoplasm (SPN) and pancreatoblastoma (PB). We distinguished their epidemiology, clinical and gross features, covered the newest reports about courses of treatment and systematised differential diagnoses. Although the most common pancreatic tumour, PDAC, has the highest malignant potential, it is still essential to properly classify and differentiate less-common lesions. It is vital to continue the search for new biomarkers, genetic mutations and the development of more specific biochemical tests for determining malignancy in rare pancreatic neoplasms.

CT, MRI, and 18F-FDG PET/CT imaging features of seven cases of adult pancreatoblastoma

ObjectiveThis study mainly analysed the imaging data for seven cases of adult pancreatoblastoma (PB) and summarized additional imaging features of this disease based on a literature review, aiming to improve the understanding and diagnosis rate of this disease.Materials and methodsThe imaging data for seven adult patients pathologically diagnosed with adult PB were retrospectively analysed. Among the seven patients, six underwent computed tomography (CT) scans, two patients underwent abdominal magnetic resonance imaging (MRI), and five patients underwent 18F-FDG PET/CT.ResultsThe tumours were located in the head of the pancreas in three cases, in the tail of the pancreas in two cases, and in the gastric antrum and neck of the pancreas in one case. Six tumours showed blurred edges, and an incomplete envelope was observed in only two cases when enhanced, which showed extruded growth and cyst-solid masses; one tumour was a solid mass with ossification. Showing mild or significant enhancement in the arterial phase (AP) for six cases. In the MRI sequence, isointensity was found on suppressed T1-weighted imaging, and hyperintensity was noted on suppressed T2-weighted imaging in two cases, with significant enhancement. Pancreatic duct dilatation was found in four cases. Tumour 18F-FDG PET/CT imaging exhibited high uptake in five cases.ConclusionAdult PB involves a single tumour and commonly manifests as cystic-solid masses with blurred edges. Capsules are rare, ossification is an important feature, tumours can also present in ectopic pancreatic tissues, with mild or strengthening in the AP, and 18F-FDG uptake is high. These features are relatively specific characteristics in adult PB.

MCL1 as putative target in pancreatoblastoma

Pancreatoblastoma (PB) is a rare tumor of the pancreas. In case of metastases, the treatment options are sparse and targeted approaches are not developed. We here evaluate MCL1 amplification as a putative target in PB.Thirteen samples from adult (10/13) and pediatric patients (3/13) were collected. Three of these samples had been previously subjected to whole-exome sequencing (2 cases) or whole-genome sequencing (1 case) within a precision oncology program (NCT/DKTK MASTER), and this analysis had shown copy number gains of MCL1 gene. We established a fluorescence in situ hybridization (FISH) test to assess the copy number alterations of MCL1 gene in 13 formalin-fixed paraffin-embedded PBs, including the 3 cases assessed by genome sequencing. FISH analysis showed the amplification of MCL1 in 2 cases (both were adult PB), one of which was a case with the highest copy number gain at genomic analysis. In both cases, the average gene copy number per cell was ≥ 5.7 and the MCL1/1p12 ratio was ≥ 2.4. Our data support MCL1 as a putative target in PB. Patients with MCL1-amplified PB might benefit from MCL1 inhibition. Sequencing data is useful to screen for amplification; however, the established FISH for MCL1 can help to determine the level and cellular heterogeneity of MCL1 amplification more accurately.

Pancreatoblastoma in children: Clinical management and literature review

PurposeThe aim of this study is to analyze the clinical and pathological features of pancreatoblastoma (PB) and to obtain better management for patients with relapsed or metastatic disease. MethodsFour cases treated in our institution and 59 cases reported previously in the literature from the PubMed biomedical database (2000–2020) were reviewed and analyzed. ResultsFour cases with PB presented with abdominal pain and palpable abdominal masses, with the tumor size ranging from 5.2 to 18 cm in diameter. The invasion of the splenic vein and superior mesenteric artery, duodenum, and lymph nodes were risk factors for PB. Three cases were treated with combination therapy and showed favorable outcomes, while one case was treated with chemotherapy alone due to tumor progression and died of the disease. Squamous corpuscles were revealed in the tumor samples and considered a defining component for histological diagnosis. ConclusionsMultidisciplinary diagnosis plays an important role in clinical management. The risk factors should be considered in the therapeutic stratification of PB before surgery.

Adult pancreatoblastoma: A rare malignant tumor of the pancreas

Pancreatoblastoma (PB) is a rare malignant tumor in adults. 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.

Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations.

Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm that affects typically young children. Signs related to advanced upper-abdominal tumor accompanied by elevated serum α-fetoprotein levels in a young child suggest PBL, however histopathological confirmation is mandatory. The mainstay of the treatment is a complete surgical resection. Unresectable and/or metastatic PBL may become amenable to complete delayed surgery after neoadjuvant chemotherapy. This manuscript presents the international consensus recommendations for the diagnosis and treatment of children with PBL, established by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the EU-funded PARTNER (Paediatric Rare Tumors Network - European Registry) project.

Treatment Strategies for Children With Relapsed Pancreatoblastoma: A Literature Review.

Pancreatoblastoma (PB) is a tumor typically seen in childhood. Despite its rarity, there are some internationally agreed recommendations for its first-line treatment, but very little is known about the management of relapse. We reviewed the literature on the treatment and outcome of children with progressing/recurrent PB, and the role of high-dose chemotherapy (HD-CT) or liver transplantation in difficult cases. A first analysis concerned 15 patients: liver metastases were the most frequent cause of first-line treatment failure. Eight patients underwent surgery, only 3 were irradiated. Various second-line chemotherapy regimens were adopted, with evidence of response in 8 children. The most often-used combinations included etoposide, cyclophosphamide/ifosfamide, and cisplatin/carboplatin. Overall, 7 patients are alive with a median follow-up of 24 months (range, 3 to 88 mo). In a separate analysis, considering patients in first-line or second-line treatment, we found 5 of 6 patients alive after HD-CT and 3 of 3 after liver transplantation. Our review shows that the outcome for patients with recurrent PB is not always dismal, especially when surgery is possible. Different chemotherapy combinations can be used, and HD-CT or liver transplantation may be considered in selected cases.

Differential diagnosis of pancreatoblastoma (PB) and solid pseudopapillary neoplasms (SPNs) in children by CT and MR imaging.

To determine whether features on computed tomographic and/or magnetic resonance imaging can differentiate pancreatoblastoma (PB) from solid pseudopapillary neoplasms (SPNs) of the pancreas in children. Clinical and imaging data for 20 cases of SPNs and 14 cases of PB confirmed by surgery or biopsy were retrospectively analysed. The size, border, calcification, haemorrhage, solid/cystic component proportion, intratumoural vessels, tumour capsulation, pancreatic duct dilatation, peripancreatic vessel invasion, distant metastasis status and apparent diffusion coefficient (ADC) values of the two groups were examined, and key diagnostic features were identified. Statistical analysis was performed using the chi-square test and Student's t test. Sensitivity and specificity values were calculated when a single criterion was used. Age ≤ 5 years, elevated serum α-fetoprotein (AFP), larger size, ill-defined border, calcification, absence of haemorrhage, intratumoural vessel, peripancreatic vessel invasion and distant metastasis differentiated PB from SPN (p < 0.05). ADC values of SPN were higher than those of PB (p = 0.001). There were no significant differences regarding tumour capsule (p = 0.435), pancreatic duct dilatation (p = 1.000) or cystic degeneration area over 50% of the tumour volume (p = 1.000) between the two groups. The following features are helpful for differentiating PB from SPN: age ≤ 5 years, elevated serum AFP, larger size, ill-defined border, calcification, haemorrhage absence, intratumoural vessel, peripancreatic vessel invasion, distant metastasis and lower ADC value. • CT and MRI are helpful to differentiate pancreatoblastoma (PB) from solid pseudopapillary neoplasms (SPNs) of the pancreas in children. • The following features are helpful to differentiate PB from SPN: age ≤ 5 years, elevated serum AFP, larger size, ill-defined border, calcification, absence of haemorrhage, intratumoural vessel, peripancreatic vessel invasion, distant metastasis and lower ADC value.

Pancreatoblastoma. Case report and review of literature

Although pancreatoblastoma (PB) is a rare tumor, it is the most common malignant pancreatic tumor in children. Clinic presentation is insidious, so early diagnostic suspicion allows timely therapy. We reported 3 cases of PB treated at our center. The first two cases achieved complete disease response after full tumor resection. The first one is in complete remission at 7 months after chemotherapy. The second patient is in second complete remission at 206 months after diagnosis and 128 months after metastatic relapse. The third case died from disease progression 61 months after the initial metastatic unresectable tumor. Histology, clinical features and treatment options are discussed along with presentation of the cases.

Pancreatoblastoma: Cytologic and histologic analysis of 12 adultcases reveals helpful criteria in their diagnosis and distinction from common mimics.

Pancreatoblastoma (PBL) is a rare malignant pancreatic tumor seen predominantly in childhood, and its cytologic diagnosis remains challenging. Twelve fine-needle-aspirations from 11 adults were analyzed. In total, 6 men and 5 women (median age, 45years; age range, 32-60years) had tumors measuring a median 5.6cm (range, 2.5-12cm) located in the pancreatic head (n=7) or tail (n=4), including 3 with familial adenomatous polyposis (FAP)/FAP-related syndromes and 4 with metastasis at diagnosis. The median follow-up was 39.8months (range, 0.8-348months), and 5 patients died of disease. The original cytology diagnoses were: PBL (n=2), neuroendocrine neoplasm (n=2), poorly differentiated neuroendocrine carcinoma (n=2), well differentiated neuroendocrine tumor (n=1), poorly differentiated carcinoma (n=2), "positive for malignancy" (n=1), acinar cell carcinoma (n=1), and epithelioid neoplasm with endocrine and acinar differentiation versus PBL (n=1). Universal cytopathologic findings included hypercellularity; 3-dimensional clusters; and single, monotonous, blast-like cells that were from 1.5 to 2.0 times the size of red blood cells with high nuclear-to-cytoplasmic ratio, fine chromatin, small, distinct nucleoli, and a resemblance to well differentiated neuroendocrine tumor and poorly differentiated neuroendocrine carcinoma. Branching pseudopapillae (n=7) and grooved nuclei (n=3) raised the differential diagnosis of solid-pseudopapillary neoplasm, but with more atypia. Uncommon features included pleomorphism (n=4) and numerous mitoses (n=1). Squamoid morules were seen on smears (n=5) or cell blocks (n=6) in 70% of patients and were characterized by epithelioid cells with elongated, streaming nuclei, fine chromatin, absent nucleoli, and positive nuclear β-catenin (n=6 of 8). The median Ki-67 index was 21% (range, 2%-70%), and neuroendocrine marker expression was common (100%), but acinar markers were variable (63%). A combination of cytologic findings in PBL, including a predominant population of primitive blast-like cells, subtle squamoid morules, frequent neuroendocrine and variable acinar phenotype, should facilitate accurate cytologic diagnosis and distinction from common mimics.

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

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.

Ultrasound and CT findings in children pancreatoblastoma

Objective To improve the imaging diagnostic performance by analyzing US and CT findings of pancreatoblastoma in children. Methods From January 2000 to December 2016, 9 patients with pancreatoblastoma proven pathologically were enrolled in the study. The clinical and imaging features in 9 cases were analyzed retrospectively, the tumor parameters compared with intraoperative and postoperative pathology were location, size, texture, capsule, calcification, adjacent organs invasion and lymph node metastasis. The parameter of tumor size was evaluated by intraclass correlation coefficient. Results The tumor diameter ranged from 30-95 mm (median diameter 40 mm). Six tumors located in the head of the pancreas, 3 tumors located in the body and tail of the pancreas. One tumor was pure cystic, 2 tumors were solid and cystic mixed, 5 tumors were solid. Calcification was seen in 5 cases. Capsule was seen in 5 cases. Blood vessels, biliary tract and bowel around tumors were infiltrated by tumors in 3 cases proven by the pathology. Lymphadenectasis was seen in 2 cases, in which 1 case was metastasis. There were no significant differences in location, nature, calcification on US and CT. The largest diameter measured by CT and US was consistent with the tumor's actual size, ICC=0.90. Capsule was shown by US in 5 cases and CT in 3 cases. CT showed the morphology of calcification in 5 cases, lymphadenectasis in 2 cases, abdominal effusion in 2 cases, cavernous transformation of the portal vein with the extensive collaterals in 1 case, which were not shown by US. Both US and CT dit not show the surrounding tissue infiltrated by tumors in 3 cases, but showed biliary system expansion. Conclusions The typical pancreatoblastoma manifested as encapsulated solid tumor originated in the pancreas with necrosis and calcification. US examination took a slight advantage on displaying capsule of tumor than CT. CT was more clear and intuitive in showing calcification and relationship between tumor with surrounding tissues and lymph node, and no operator dependent. Key words: Pancreatic neoplasms; Ultrasonography; Tomography, X-ray computed; Child

Pancreatoblastoma in children: 9 case reports and literature review

Objective To analyze the clinical features, treatment and prognosis of the pancreatoblastoma(PB). Methods A total of 9 patients including 4 boys and 5 girls diagnosed as pancreatoblastoma in Beijing Tongren Hospital Affiliated to Capital Medical University between July 2008 and February 2016 were enrolled.The clinical features, therapy and prognosis were analyzed. Results (1)Clinical manifestations: 6 cases had abdominal mass, 1 case had jaundice, 2 cases had abdominal pain at the time of diagnosis; 5 cases were found in caput pancreatis, 2 cases were found in corpora pancreatis and 2 cases were found in cauda pancreatis; 4 cases had local tumor, 3 cases had local invasion, and 2 cases had liver metastasis at diagnosis.(2)Enzymology and tumor marks: elevated alpha-fetoprotein(AFP) was found in 5 cases (48.5-52 971.0 μg/L), elevated neuron-specific enolase(NSE) was found in 5 cases(22.4-53.4 μg/L), elevated carbohydrate antigen 125(CA-125) was found in 5 cases [(40.3-122.8) ×103 U/ L], elevated carbohydrate antigen 19-9(CA19-9) was found in 2 cases[(65.4-362.5)×103 U/ L], elevated lactate dehydrogenase was found in 2 cases(380~838 U/L) and elevated hemodiastase was found in 2 cases (105.5-122.8 U/L). (3)Treatment and prognosis: in 9 patients, the tumors were resected at first in 6 patients, and 5 of them remitted until now, 1 of them relapsed after the comprehensive treatment for 9 months.Two of the patients underwent operation after the chemotherapy, in which 1 case received complete resection but without chemotherapy after the surgery, whom relapsed 1 year later; and the other one was resected partly.But after stopping the comprehensive treatment for 6 months, the disease relapsed.One patient could not receive operate because of huge tumor size, and the chemotherapy failed to control tumor progression; the patient died without operation.Three relapsed children were still in chemotherapy.The median follow-up period was 21 months(8-98 months). Conclusions AFP may be used as monitoring indicator for PB.Complete tumor resection is an important factor affecting the outcome.Adjuvant chemotherapy may reduce recurrence and prolong survival. Key words: Pancreatoblastoma; Treatment; Prognosis