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Pediatric Blood & Cancer Jun 2021Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm that affects typically young children. Signs related to advanced upper-abdominal tumor accompanied by...
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.
Topics: Chemotherapy, Adjuvant; Child; Child, Preschool; Humans; Neoadjuvant Therapy; Pancreatectomy; Pancreatic Neoplasms; Rare Diseases
PubMed: 34174157
DOI: 10.1002/pbc.29112 -
Radiographics : a Review Publication of... 2021A wide spectrum of hereditary syndromes predispose patients to distinct pancreatic abnormalities, including cystic lesions, recurrent pancreatitis, ductal...
A wide spectrum of hereditary syndromes predispose patients to distinct pancreatic abnormalities, including cystic lesions, recurrent pancreatitis, ductal adenocarcinoma, nonductal neoplasms, and parenchymal iron deposition. While pancreatic exocrine insufficiency and recurrent pancreatitis are common manifestations in cystic fibrosis and hereditary pancreatitis, pancreatic cysts are seen in von Hippel-Lindau disease, cystic fibrosis, autosomal dominant polycystic kidney disease, and McCune-Albright syndrome. Ductal adenocarcinoma can be seen in many syndromes, including Peutz-Jeghers syndrome, familial atypical multiple mole melanoma syndrome, Lynch syndrome, hereditary breast and ovarian cancer syndrome, Li-Fraumeni syndrome, and familial pancreatic cancer syndrome. Neuroendocrine tumors are commonly seen in multiple endocrine neoplasia type 1 syndrome and von Hippel-Lindau disease. Pancreatoblastoma is an essential component of Beckwith-Wiedemann syndrome. Primary hemochromatosis is characterized by pancreatic iron deposition. Pancreatic pathologic conditions associated with genetic syndromes exhibit characteristic imaging findings. Imaging plays a pivotal role in early detection of these conditions and can positively affect the clinical outcomes of those at risk for pancreatic malignancies. Awareness of the characteristic imaging features, imaging-based screening protocols, and surveillance guidelines is crucial for radiologists to guide appropriate patient management. RSNA, 2021.
Topics: Genetic Predisposition to Disease; Humans; Multiple Endocrine Neoplasia Type 1; Neoplastic Syndromes, Hereditary; Pancreas; Pancreatic Neoplasms
PubMed: 34143711
DOI: 10.1148/rg.2021200164 -
Frontiers in Oncology 2021Pancreatoblastomas are unfrequent tumors usually found in children. We report two cases of metastatic pancreatoblastomas observed in young women. A systemic chemotherapy...
Pancreatoblastomas are unfrequent tumors usually found in children. We report two cases of metastatic pancreatoblastomas observed in young women. A systemic chemotherapy (FOLFIRINOX regimen) was associated with a disease control in one case and a partial response in the second with an improvement of general status for both. A high-throughput sequencing of the tumor described in both cases alteration in the Wnt/β-catenin pathway: a mutation in (exon 3, c.110C>G, p.S37C, reported as a hotspot in COSMIC) in one case and a homozygous loss associated with breakage targeting (5q22.2) in the second.
PubMed: 33796447
DOI: 10.3389/fonc.2021.564506 -
Pediatric Surgery International Aug 2021Pancreas tumors are extremely rare in pediatric and adolescent patients. Surgical resection is the mainstay of treatment; however, the data are limited with respect to...
PURPOSE
Pancreas tumors are extremely rare in pediatric and adolescent patients. Surgical resection is the mainstay of treatment; however, the data are limited with respect to morbidity and mortality. We aimed to evaluate short- and long-term outcomes of pediatric and adolescent patients who underwent surgical resection of pancreatic tumors.
METHODS
Patients [Formula: see text] 18-year-olds who underwent resection of pancreas tumor at the National Institute of Neoplastic Diseases INEN during 2000-2020 were included.
RESULTS
Thirty-four patients were diagnosed; 28 patients were female and 6 were male. The median age was 13.4-years-old. Histological diagnosis was solid pseudopapillary neoplasm (SPN) (n = 29, 85.3%), pancreatoblastoma (n = 3), neuroendocrine carcinoma (n = 1), and insulinoma (n = 1). No patient experienced postoperative mortality and 15 (44.1%) patients developed postoperative complications including pancreatic fistula as the most frequent. Under a median follow-up period of 33.8 (0.5-138) months, four (11.8%) patients died. Of the 29 patients with SPN, the 3- and-5-year OS rates were 100% and 83.1%, respectively.
CONCLUSIONS
SPN was the most frequent cause of surgical treatment for pediatric and adolescent patients in the high-volume cancer center in Peru and was associated with favorable survival. Pancreaticoduodenectomy was safely performed in this patient group with acceptable morbidity and zero mortality.
Topics: Adolescent; Carcinoma, Papillary; Child; Female; Humans; Male; Pancreatic Neoplasms; Pancreaticoduodenectomy; Peru; Postoperative Complications; Retrospective Studies
PubMed: 33742268
DOI: 10.1007/s00383-021-04877-3 -
Clinical Nuclear Medicine Aug 2021A 36-year-old asymptomatic woman was incidentally found to have a huge mass in the pancreas by ultrasound during routine health screening. The mass was suspected of...
A 36-year-old asymptomatic woman was incidentally found to have a huge mass in the pancreas by ultrasound during routine health screening. The mass was suspected of neuroendocrine tumor or solid pseudopapillary tumor by subsequent abdominal CT. 18F-FDG and 68Ga-DOTATATE PET/MR were acquired for presurgical assessment of the tumor invasion and malignant potential, which revealed intense FDG uptake and mild DOTATATE uptake. The tumor was completely resected, and postsurgical pathology demonstrated pancreatoblastoma with neuroendocrine manifestations. This case showed the metabolic and biological features of pancreatoblastoma on the 18F-FDG and 68Ga-DOTATATE PET/MR.
Topics: Adult; Female; Fluorodeoxyglucose F18; Humans; Incidental Findings; Magnetic Resonance Imaging; Multimodal Imaging; Organometallic Compounds; Pancreatic Neoplasms; Positron-Emission Tomography
PubMed: 33661203
DOI: 10.1097/RLU.0000000000003568 -
Pediatric Blood & Cancer Apr 2021Mutations of the APC (adenomatous polyposis coli) gene correlate mainly with familial adenomatous polyposis (FAP), but can occasionally be pathogenic for medulloblastoma...
INTRODUCTION
Mutations of the APC (adenomatous polyposis coli) gene correlate mainly with familial adenomatous polyposis (FAP), but can occasionally be pathogenic for medulloblastoma (MBL) wingless-related integration site (WNT) subtype, the course of which has only recently been described.
METHODS
We retrieved all patients with documented germline APC mutations and a diagnosis of MBL to examine their outcome, late effects of treatment, and further oncological events.
RESULTS
Between 2007 and 2016, we treated six patients, all with a pathogenic APC variant mutation and all with MBL, classic histotype. None had metastatic disease. All patients were in complete remission a median 65 months after treatment with craniospinal irradiation at 23.4 Gy, plus a boost on the posterior fossa/tumor bed up to 54 Gy, followed by cisplatin/carboplatin, lomustine, and vincristine for a maximum of eight courses. Five of six diagnostic revised MRI were suggestive of the WNT molecular subgroup typical aspects. Methylation profile score (in two cases) and copy number variation analysis (chromosome 6 deletion in two cases) performed on four of six retrieved samples confirmed WNT molecular subgroup. Four out of six patients had a positive family history of FAP, while gastrointestinal symptoms prompted its identification in the other two cases. Four patients developed other tumors (desmoid, MELTUMP, melanoma, pancreatoblastoma, thyroid Tir3) from 5 to 7 years after MBL.
DISCUSSION
Our data confirm a good prognosis for patients with MBL associated with FAP. Patients' secondary tumors may or may not be related to their syndrome or treatment, but warrant adequate attention when planning shared guidelines for these patients.
Topics: Adenomatous Polyposis Coli; Adolescent; Adult; Cerebellar Neoplasms; Child; Disease Management; Female; Humans; Male; Medulloblastoma; Pedigree; Prognosis; Quality of Life; Young Adult
PubMed: 33459525
DOI: 10.1002/pbc.28912 -
The Gulf Journal of Oncology Sep 2020Pancreatoblastoma (PB), also known as infantile pancreatic carcinoma, is an exceedingly rare pancreatic tumor in childhood, which is considered a malignant exocrine...
Pancreatoblastoma (PB), also known as infantile pancreatic carcinoma, is an exceedingly rare pancreatic tumor in childhood, which is considered a malignant exocrine pancreatic tumor. Some cases have been reported in Saudi Arabia. Although PB primarily presents during childhood, it may occur in adults too. PB tends to be less hostile in infants and children when compared to adults. Histologically, PB is characterized by distinguished acinar and squamoid cell differentiation. Most of these tumors develop in the head of the pancreas and increase alpha-fetoprotein in up to 68% of patients. Ultrasound and CT scan play a significant role in preoperative diagnosis, which is often quite difficult. The best treatment is surgical removal of the pancreas. The role of adjuvant chemotherapy or radiotherapy is still under consideration due to a minor number of patients treated. Chemotherapy regimens consisting of cyclophosphamide, etoposide, doxorubicin, and cisplatin have been used in the neoadjuvant setting with anecdotal benefit. It was found that a poorer prognosis was associated with patients who had metastasis, and patients who could not be operated on surgically.
Topics: Child; Female; Humans; Male; Pancreatic Neoplasms
PubMed: 33431369
DOI: No ID Found -
Journal of Pediatric Hematology/oncology Nov 2021Pancreatoblastoma (PB) is a tumor typically seen in childhood. Despite its rarity, there are some internationally agreed recommendations for its first-line treatment,... (Review)
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.
Topics: Antineoplastic Combined Chemotherapy Protocols; Child; Combined Modality Therapy; Humans; Liver Transplantation; Neoplasm Recurrence, Local; Pancreatic Neoplasms; Prognosis
PubMed: 33323880
DOI: 10.1097/MPH.0000000000002033 -
Pancreatology : Official Journal of the... Jan 2021Pancreatoblastoma is a rare pediatric malignant neoplasm characterized by its histological resemblance to fetal pancreatic tissue and poor clinical outcomes....
BACKGROUND
Pancreatoblastoma is a rare pediatric malignant neoplasm characterized by its histological resemblance to fetal pancreatic tissue and poor clinical outcomes. Preoperative diagnosis of the neoplasm is difficult due to its rarity, variable clinical presentation, and its lack of distinct laboratory markers. Current mainstay of treatment is surgical resection of the tumor, although a standard of care has not yet been established.
METHODS
Data were collected on one patient admitted to the University of Virginia Hospital System. Radiology, hematopoietic cell transplant, and biopsy data were collected according to the best clinical practice.
RESULTS
Herein, we describe the case of an adult patient with pancreatoblastoma treated with high-dose chemotherapy and autologous peripheral blood hematopoietic cell transplantation. To the authors' knowledge, this is the first documented successful treatment of pancreatoblastoma using autologous hematopoietic cell transplantation in the United States, and the first successful treatment in an adult patient worldwide.
CONCLUSIONS
While it is difficult to draw conclusions based on a single case, we would like to highlight the success of this treatment modality in the management of our patient with a 51-month remission and open further discussion into exploring the use of autologous hematopoietic cell transplantation for pancreatoblastoma. Our patient is currently living 57 months after diagnosis despite the average survival rate being less than 18 months.
Topics: Antineoplastic Agents; Combined Modality Therapy; Female; Hematopoietic Stem Cell Transplantation; Humans; Neoplasm Metastasis; Neoplasm Recurrence, Local; Pancreatic Neoplasms; Remission Induction; Treatment Outcome; Young Adult
PubMed: 33199137
DOI: 10.1016/j.pan.2020.10.049