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World Journal of Gastroenterology Jul 2021Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas, which may pose diagnostic... (Review)
Review
Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas, which may pose diagnostic difficulties. Because of its rarity, little is known about its clinical and pathologic features. This article reviews the clinical and pathologic features of pancreatoblastoma in adults including differential diagnosis, treatment, and follow-up. Although pancreatoblastoma commonly occurs in childhood, there have now been more than 70 adult pancreatoblastomas described in the literature. There is a slight male predominance. There are no symptoms unique to pancreatoblastomas and adult patients are frequently symptomatic. The most common presenting symptom is abdominal pain. Grossly, the tumours are often large and well-circumscribed. Microscopically, pancreatoblastomas are composed of neoplastic cells with predominantly acinar differentiation and characteristic squamoid nests. These tumours are positive for trypsin, chymotrypsin, lipase, and BCL10. Loss of heterozygosity on chromosome 11p is the most common molecular alteration in pancreatoblastomas. Adult pancreatoblastomas are aggressive tumours with frequent local invasion, recurrence, and distant metastasis. Treatment consists of surgical resection. Chemotherapy and radiotherapy may have a role in the treatment of recurrent, residual, unresectable, and metastatic disease. It is important to distinguish pancreatoblastomas from morphological mimics such as acinar cell carcinomas, solid pseudopapillary neoplasms, and pancreatic neuroendocrine neoplasms.
Topics: Adult; Carcinoma, Acinar Cell; Humans; Male; Neoplasm Recurrence, Local; Pancreas; Pancreatic Neoplasms
PubMed: 34326617
DOI: 10.3748/wjg.v27.i26.4172 -
Journal of Gastrointestinal Cancer Sep 2018
Review
Topics: Adult; Humans; Male; Pancreatic Neoplasms
PubMed: 28194556
DOI: 10.1007/s12029-017-9925-x -
Pancreatology : Official Journal of the... 2009Pancreatoblastoma is a very uncommon neoplasm in adults and its management represents a great challenge with regards to different treatment options. Given the rarity of... (Review)
Review
BACKGROUND
Pancreatoblastoma is a very uncommon neoplasm in adults and its management represents a great challenge with regards to different treatment options. Given the rarity of the disease, the aim of this study was to review our personal experience with adult pancreatoblastoma as well as the cases reported in the literature in order to support clinicians observing this entity.
METHODS
Adult patients with histologically proven pancreatoblastoma were identified from our prospective database of pancreatic resections. After a search on the Medline database, a review of all cases was performed as well, focusing on clinical, radiological and hystopathological features and treatment options.
RESULTS
At our Institution, 2 adult males, 26 and 69 years old, underwent successful pancreatic resection for pancreatoblastoma. The diagnosis of pancreatoblastoma mainly depends on the pathological findings characterized by squamoid corpuscles at histopathology. Only 21 cases of adult pancreatoblastoma have been identified in the literature. In general, despite aggressive treatment, pancreatoblastoma in adults is associated with poorer outcome than in children, with a median survival time of 18.5 months. Both our patients are disease free after 15 months (case 2) and 51 months (case 1). The latter represents the most successful result in long-term disease-free survival.
CONCLUSION
Pancreatoblastoma is a rare neoplasm in adults. The differential diagnosis includes nonfunctional pancreatic endocrine tumor, acinar cell carcinoma, solid pseudopapillary tumor and adenocarcinoma. Surgical resection is the only treatment associated with long-term survival. Chemotherapy may play a role as palliative treatment in advanced disease.
Topics: Adult; Aged; Carcinoma, Neuroendocrine; Child; Child, Preschool; Diagnosis, Differential; Humans; Male; Pancreatic Neoplasms; Pancreaticoduodenectomy
PubMed: 19077457
DOI: 10.1159/000178877 -
Gastroenterologie Clinique Et Biologique 1997Pancreatoblastoma is a rare pancreatic tumor, which usually occurs during childhood. We describe here the ninth adult case in a 21-year-old woman, who died 7 months... (Review)
Review
Pancreatoblastoma is a rare pancreatic tumor, which usually occurs during childhood. We describe here the ninth adult case in a 21-year-old woman, who died 7 months after diagnosis despite complete surgical resection and adjuvant chemotherapy. Pancreatoblastoma in adults has a poor prognosis, at variance with what may be observed in childhood.
Topics: Adult; Age Factors; Carcinoma, Acinar Cell; Carcinoma, Squamous Cell; Female; Humans; Microscopy, Electron; Pancreatic Neoplasms
PubMed: 9587540
DOI: No ID Found -
Clinical Imaging 2018Our purpose was to report a case of adult pancreatoblastoma, and review the literature in order to assist clinicians in the management of the disease. (Review)
Review
PURPOSE
Our purpose was to report a case of adult pancreatoblastoma, and review the literature in order to assist clinicians in the management of the disease.
MATERIALS AND METHODS
The demographic, clinical, and imaging findings of 41 patients with pathologically proven pancreatoblastoma from 1986 to 2017 identified in PubMed were reviewed. The key words used for searching PubMed were: "pancreatoblastoma", "pancreatic tumor", and "adult pancreatoblastoma." We also reported the details of a case of adult pancreatoblastoma treated at our institution.
RESULTS
We identified 41 cases of adult pancreatoblastomas, and the mean age at diagnosis was 41.4 ± 17.4 years. Pancreatoblastomas occurred in the pancreatic head in 48.4% of patients, and in 39.0% of cases the tumor was >8 cm in diameter at diagnosis. Patient age and tumor size were similar between males and females (P = 0.59; P = 0.32, respectively). Metastases was present in 17 of the 41 adult patients (41.5%). No significant difference in age, sex, tumor size, and tumor location was found between patients with and without metastases (P = 0.57, 0.58, 0.64, 0.39, respectively).
CONCLUSION
Preoperative diagnosis of adult pancreatoblastoma is difficult because of the heterogeneous, variable cellular differentiation and atypical clinical and imaging features. A pancreatoblastoma should be considered when tumors in the pancreas are solid and cystic.
Topics: Adult; Fatal Outcome; Female; Humans; Male; Middle Aged; Pancreas; Pancreatic Neoplasms; Young Adult
PubMed: 29753278
DOI: 10.1016/j.clinimag.2018.05.001 -
JOP : Journal of the Pancreas Jan 2007Pancreatoblastoma (PB), or infantile pancreatic carcinoma, is an extremely rare pancreatic tumor in childhood, comprising 0.5% of pancreatic non-endocrine tumors....
Pancreatoblastoma (PB), or infantile pancreatic carcinoma, is an extremely rare pancreatic tumor in childhood, comprising 0.5% of pancreatic non-endocrine tumors. Although PB mainly presents during childhood but can also occur in adults. PB tend to be less aggressive in infants and children compared to adults. Children with PB usually present late with upper abdominal pain and many have a palpable mass in the epigastrium. Mechanical obstruction of the upper duodenum and gastric outlet by tumor in the head of the pancreas may be associated with vomiting, jaundice and gastrointestinal bleeding. Histologically, PB is characterized with distinct acinar and squamoid cell differentiation. PB has been associated with alterations in the Wnt signaling pathway and chromosome 11p loss of heterozygosity (LOH), Beckwith-Wiedemann syndrome and familial adenomatous polyposis. The majority of these tumors arise in the head of the pancreas. Alpha-fetoprotein may be elevated in up to 68% of patients with PB. Ultrasound and CT scan may be useful but preoperative diagnosis is often quite difficult. The treatment of choice is complete resection, that may often be curative. The role of adjuvant chemotherapy or radiotherapy is still under discussion due to small number of patients treated as yet. Chemotherapy regimens consisting of cyclophosphamide, etoposide, doxorubicin, and cisplatin have been used in neoadjuvant setting with anecdotal benefit. Prognosis of this rare tumor is good, when resected completely. Prognosis is poorer, when there is metastasis or when it is inoperable. On the whole, PB is regarded to be a curable tumor; hence the clinical diagnosis should be made early. Awareness of this rare tumor of pancreas is essential for early detection and proper management. The author review the clinical presentation, etiology, diagnosis, treatment and prognosis of PB in this presentation.
Topics: Adolescent; Adult; Aged; Carcinoma; Child; Child, Preschool; Female; Humans; Infant; Male; Middle Aged; Pancreas; Pancreatic Neoplasms
PubMed: 17228135
DOI: No ID Found -
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 -
Gland Surgery Aug 2015Pancreatoblastoma (PB) is a rare malignant neoplasm of the pancreas with unknown etiology. It occurs mostly in the pediatric population with very rare documented cases... (Review)
Review
Pancreatoblastoma (PB) is a rare malignant neoplasm of the pancreas with unknown etiology. It occurs mostly in the pediatric population with very rare documented cases in adults. This is a review of the case reports of the adult pancreatoblastoma in the literature. A total of 35 cases were identified and reviewed with the mean age of 41 years (range, 18-78 years) and the male sex accounted for 51.4% of the cases. Adult Pancreatoblastoma seem to have a predilection for the head of the pancreas which accounted for approximately 49% of the cases reviewed with an average size of 8 cm (range, 1.8-20 cm). The median follow up for patients was 15 months (range, 1-108 months) Metastatic disease and local infiltration of surrounding tissues is common with poor prognosis in adult patients. Preoperative diagnosis is difficult because of the unhelpful tumor markers in adults and the cellular heterogeneity of the tumor which makes fine needle aspiration cytology unreliable. Histopathological review of the tumor is essential for diagnosis. Pancreatoblastomas should be considered a differential diagnosis of solid and cystic pancreatic neoplasms. Surgical resection of the tumor is the treatment of choice with a variable combination with radiotherapy and chemotherapy.
PubMed: 26312218
DOI: 10.3978/j.issn.2227-684X.2015.04.05 -
Journal of Pediatric Surgery Nov 1997Pancreatoblastoma is a rare pancreatic tumor with distinct acinar and squamoid cell differentiation that generally affects infants and young children. Just over 50 cases...
BACKGROUND
Pancreatoblastoma is a rare pancreatic tumor with distinct acinar and squamoid cell differentiation that generally affects infants and young children. Just over 50 cases have been reported in the literature.
METHODS
Five cases of pathologically proven pancreatoblastoma treated at Seoul National University Hospital from 1984 to 1994 were reviewed. There were three girls and two boys who were 2 years to 5 years of age. All cases came to medical attention because of an abdominal mass.
RESULTS
Abdominal pain was observed in one case and anorexia, vomiting, and weight loss in one case. There was marked elevation of serum alpha-fetoprotein (27,000 ng/mL) in one case of liver metastases. Complete excision was performed in two cases in which the tumors were located in the tail of the pancreas. Partial excision was performed in two patients who had unresectable tumors of the head of the pancreas. One patient had an unresectable tumor at diagnosis and needle aspiration biopsy was carried out under ultrasound guidance. Electron microscopy was performed on pathological specimens of three cases and showed zymogen granules but not neuroendocrine granules. Immunocytochemical studies for alpha-fetoprotein, insulin, glucagon and somatostatin were performed in one patient, and results were all negative. Of two patients who underwent complete excision, one patient presented with liver metastases 4 months after operation and received chemotherapy, but died of tumor 6 months after operation. The other patient had local recurrence 1 year after operation. Reoperation and chemotherapy were performed, and the child is now alive without evidence of disease for 32 months. All three patients who had unresectable tumor died of tumor despite adjuvant radiotherapy and chemotherapy.
CONCLUSIONS
The authors emphasize that the diagnosis of pancreatoblastoma in childhood should be suspected with palpation of an abdominal mass, and the chance for cure may be determined by complete excision of the tumor.
Topics: Child, Preschool; Combined Modality Therapy; Fatal Outcome; Female; Humans; Male; Microscopy, Electron; Pancreatic Neoplasms; Tomography, X-Ray Computed
PubMed: 9396538
DOI: 10.1016/s0022-3468(97)90465-6 -
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