Pancreatoblastoma in children:9 case reports and literature review / 中华实用儿科临床杂志

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 pg/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.

Pancreatoblastoma with multiple peritoneal metastases: a rare entity with atypical presentation and review of literature.

Pancreatoblastoma is a rare primary pancreatic neoplasm that predominantly effects young children and characterized by unique clinical features. Its biological behavior is aggressive with frequent local invasion and metastases. Only hundred cases have been reported in medical literature and its radiological features have been infrequently studied. We report the sonographic, contrast enhanced computed tomography findings of pancreatoblastoma in a 3 year old boy.

Pancreatoblastoma in an adult.

Pancreatoblastoma is a malignant pancreatic tumor that rarely occurs in adults. We report a case of an adult female with pancreatoblastoma. A mass was detected in the pancreatic head using computed tomography and ultrasonography. The clinical diagnosis was a solid- pseudopapillary neoplasm of the pancreas. However, after the operation, the fi nal diagnosis was pancreatoblastoma, which showed two lines of differentiation: Acinar differentiation and squamoid corpuscles. The patient is currently in good condition.

Pancreatoblastoma in an Adult / 대한췌담도학회지

A blastoma is a type of cancer, which is common in children; it is caused by malignancies derived from in the precursor cells, often called blasts. Examples are nephroblastomas, retinoblastomas, pleuropulmonary blastomas, and pancreatoblastomas. Pancreatoblastomas are extremely rarely in adults. It is difficult preoperatively to distinguish this tumor from other pancreatic tumors including solid and papillary epithelial neoplasm of the pancreas (SPEN), acinar cell carcinoma, islet cell tumor, and ductal adenocarcinoma with cystic degeneration. To our knowledge, this case may be the second report of a pancreatoblastoma occurring in an adult in Korea. We report a case of a pancreatoblastoma that was confirmed by pathology, despite the radiologic finding that assumed it was a SPEN.

Synchronous Ectopic Pancreatoblastoma in a Child: A Case Report

Pancreatoblastoma is a rare primary pancreatic neoplasm of children that may arise in any portion of the pancreas. We report a case of a 3-yr-old boy who presented to with abdominal pain our hospital and a progressive bulge in his right abdomen. Biochemical evaluation and serum levels of tumoral markers were within reference limits. On the computed tomography, two tumors were found. One located in the head of the pancreas; however, a laparotomy revealed that the head of pancreas was compressed but normal. The other was in the left abdomen near the spleen and the tail of the pancreas. The diagnosis of two synchronous pancreatoblastoma originating from the omentum was confirmed by pathology. Therefore, a pancreatoblastoma should be considered when a large well-defined, lobulated, and heterogeneous mass is identified in the pancreas of children. In addition, an ectopic pancreatoblastoma should be considered when identified within or near the ectopic pancreatic tissue.

Imaging manifestation of pancreatoblastoma in children / 中华消化外科杂志

Pancreatoblastoma is a rare kind of malignant tumor of pancreas,which is commonly seen among children.A female child aged 5 years was admitted to the Dapiag Hospital with the chief complaint of painless abdominal mass on June 18,2010.The results of B ultrasound showed acoustic shadow of tumor calcification and an absent of normal pancreatic echo.The results of computed tomography(CT)showed that the tumor was located at the body and tail of the pancreas with a low-density and isopycnic shadow of intermixed huge block.The parenchyma of tumor showed unequal enhancement,the periphery showed lobular-like reticular enhancement,the central necrotic area showed no enhancement,and multiple metastatic foci were observed in spleen and liver under enhanced CT scan.The patient underwent resection of the body and tail of the pancreas and the spleen.Chemotherapeutics with vincristine,cyclophosphamide and doxorubicin were adopted postoperatively.After a period of 6-month follow-up,the results of CT showed that the size of tumor was decreased.

Report of 2 cases of pancreatoblastoma / 中华内分泌外科杂志

Objective Todiscuss the diagnosis and therapy of the pancreatoblastoma(PB).Methods The data of 2 cases of PB were analyzed retrospectively and related literatures were reviewed.Results Both cases were males,11 years old and 8 years old respectively.The 2 cases both had solid mass located in the tail of the pancreas.Alpha-fetal protein(AFP) was normal in case 1 and 2 903 ng/ml in case 2.The 2 cases underwent resection of the pancreas tail,and the postoperative pathological examination confirmed the diagnosis of PB.Followup of 26 months in case 1 and 10 months in case 2 showed that the survival was good.Conclusions PB is an extremely rare tumor of exocrine pancreas and often occurs in male children.The solid mass located in the pancreas with elevated AFP can be considered as PB.Our experience showed a pancreatic mass with normal AFP can also be PB.Surgery is the best management of PB.

Pediatric Pancreatic Tumors-Clinical Experience

Pancreatic tumors in children are relatively rare, and their prognosis differs from that in adults. The purpose of this study is to examine the clinical characteristics, treatment, and prognosis for children with pancreatic tumors. We retrospectively reviewed the medical records of children under 15 years of age with pancreatic tumors who were treated surgically at Asan Medical Center between January 1992 and November 2009. There were 16 patients, fourteen of whom were pathologically diagnosed with solid pseudopapillary tumor. The other two patients were diagnosed with pancreatoblastoma and acinar cell carcinoma, respectively. Six patients of the 16 patients (38%) were male, and there was a male-to-female ratio of 1:1.6. The initial presentations were upper abdominal pain in eight patients (50%), palpable abdominal mass in three, and vomiting in one. Four patients were diagnosed incidentally. Six patients' tumors were located in the pancreatic head, six in the pancreatic body, and four in the pancreatic tail, respectively. The surgical procedures performed included distal pancreatectomy (n=7, 44%), median segmentectomy (n=3), enucleation (n=3), pancreaticoduodenectomy (n=2), and pylorus-preserving pancreaticoduodenectomy (n=1). Three patients underwent laparoscopic surgery. The median tumor size was 6.5cm (1.8~20 cm). Early surgical complications included pancreatic fistula (n=4), bile leakage (n=1), and delayed gastric emptying (n=1). A late complication in one patient was diabetes. The median follow-up period was five years and four months, and all patients survived without recurrence. While pancreatic tumors in adults have a poor prognosis, pancreatic tumors of childhood are usually curative with complete resection and thus have a favorable prognosis.

Pancreatoblastoma in Children

Pancreatoblastoma is a rare pediatric neoplasm with distinct acinar and squamoid differentiation that generally affects infants and young children. Although the prognosis is relatively favorable over typical pancreatic cancer, the optimal treatment of pancreatoblastoma has not been established. We experienced two cases of pancreatoblastoma in a 2-year-old girl and boy which was found incidentally by the parents due to the detection of abdominal masses.

Pancreatoblastoma 1 case / 한국간담췌외과학회지

Pancreatoblastoma is a rarely occurring malignant tumor in childhood, which has been reported less 60cases in the world. which is a kind of adenocarcinoma exhibiting solid encapsulating tumor. Its prognosis is better favorable than typical pancreatic cancer. We were experienced one case of pancreatoblastoma, the patient was a 8 years-old boy who was incidentally found by his parent because of left upper quadrant mass during having a bath without any previous medical history. His preoperative AFP level was elevated and his preoperative diagnosis was pancreatoblastoma by abdominal computed tomography. During laparotomy, the tumor was located between body and tail of pancreas, adhered to hilum of spleen and also surrounded by transverse mesocolon, displacing the arcade of mesocolic vessels. When dissecting the mesocolon from pancreatic tumor, superior mesenteric artery and gastroduodenal artery were embeded in the tumor. The patient underwent distal pancreatectomy and splenectomy after ligature and dividing of mid-colic vessel. After surgery, he was received six-cycle of combined chemotherapy. There is no evidence of recurrence and metastasis for nineteen months after surgery.

Two Cases of Pancreatoblastoma

Pancreatoblastoma is uncommon in children and is exceedingly rare in adults. Prognosis is known to be good if the tumor is discovered prior to metastasis. Complete tumor resection is the most important factor for long survival. We report two cases of pancreatoblastoma in a 4 year and 7 year- old females. Palpable abdominal mass was accidentally discovered by their parents. Abdominal CT scan showed huge retroperitoneal mass of unknown origin in one case and of the pancres in the other case. At laparotomy, well encapsulated tumor mass (10x10 cm in diameter) was noted in the body of pancreas in one case, and well encapsulated tumor mass ( 8x7 cm in diameter) in the tail of pancreas in the other case. We resected the tumor and saved spleen in both ases. Histologic examination revealed that the tumors were pancreatoblastoma. Patients received postoperative chemotherapy with 6 cycles of cisplatinum, adriamycin, ifosfamide and etoposide and are alive in good condition 19 months and 17 months after operation.

Pediatric Pancreatic Tumors / 대한암학회지

PURPOSE: Pancreatic tumors are relatively rare in children. Until now more than 150 cases have been reported in the English literature. In this paper, the authors report the tumors clinical characteristics and the results of surgery in eleven children. MATERIALS AND METHODS: Eleven cases of pancreatic tumor pathologically verified at Seoul National University Children's Hospital between 1984 to 1998 were retrospectively analyzed. Four were boys and seven were girls, and their mean age at diagnosis was 7.7 (range, 2 13) years. RESULTS: There were six solid and papillary epithelial neoplasms of the pancreas (SPENP) and five pancreatoblastomas. All children came to medical attention because of abdominal masses. Tumors ranged in size from 6.0X 5.0 cm to 10.5 x 8.0 cm. Eight tumors were located in head and three in tail. Complete excision was performed in eight cases (six in SPENP and two in pancreatoblastoma). Incomplete excision was performed in two cases of pancreatoblastoma. One patient with pancreatoblastoma had an unresectable tumor at the time of diagnosis and needle aspiration biopsy was done under the ultrasound guidance. No patient died during surgery. After a mean follow-up period of 4.1 years, all patients with SPENP were alive and there had been no recurrence. However, of two patients who received complete excision in pancreatoblastoma, one presented with liver metastasis at 4 months after operation and received chemotherapy, but died 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 montbs after the initial operation. All three patients with unresectable tumor died in spite of adjuvant radiotherapy and chemotherapy. CONCLUSION: Pediatric pancreatic tumors comprise rare heterogenous groups of malignancies with their prognosis dependent upon adequate resection and pathologic classification. Complete resection of pancreatic tumors arising anywhere in the pancreas was recommended.

A Case of Cystic Pancreatoblastoma Associated with Congenital Hemihypertrophy

We experenced a case of cystic pancreatoblastoma associated with congenital hemihypertrophy in a 4 months old male. The mass was located on the anterior side of pancreatic head without any connection to the pancreas. After exision of cystic pancreatoblastoma, chemotherapy(FAM regimen) was performed 15 times due to capsular tumor invasion. Until this time there was no drug side effect and metastasis. The patient's general condition is stable.

A Case of Pancreatoblastoma in an Adult / 대한소화기내시경학회지

Pancreatoblastoma is a rare tumor, which usually affects infants and young children. We report a case of pancreatoblastoma in a 51-year-old man. To our knowledge, this is the first case of pancreatoblastoma occurred in an adult in Korea. A 2,5 * 3.5 cm sized pancreatic mass was detected on routine examination. An enhanced computed, tamography scan showed a slightly low density solid mass in the body of the pancreas. An abrupt occlusion of the main pancreatic duct and filling defect at the body was noted in the endoscopic retrograde pancreatogram. On operation, a well circumscribed yellowish white mass with whitish capsule was found without adhesion. Histologic examination revealed that the tumor was made up predominantly of medium sized round to polygonal cells having finely granular cytoplaam arranged in solid sheet and acinar structure. Squamoid corpuscles with ovoid to elongated cells were also scattered. The patient is doing well postoperatively. In the review of the literature, the tumor may be biologically different in the older and younger age group.

Pancreatoblastoma: histopathological and ultrastructural analysis of two cases

Pancreatoblastoma has been described in children and characterized by unique histologic features and excellent clinical course. Ultrastructural and immunohistochemical studies of pancreatoblastoma reveal either exocrine alone or both endocrine and exocrine differentiation. We present two cases of pancreatoblastoma in children in which immunohistochemical and ultrastructural examination failed to demonstrate features of either enzyme or hormone production and which became worse in clinical course. We assume that pancreatoblastomas are tumors which differentiate more toward acinar or ductal elements than toward islet cell.