Diagnosis and treatment of pancreatoblastoma in children: a retrospective study in a single pediatric center

Huang, Yijin; Yang, Wei; Hu, Jiajian; Zhu, Zhiyun; Qin, Hong; Han, Wei; Wang, HuanMin

doi:10.1007/s00383-019-04524-y

Cited by 25 publications

(36 citation statements)

References 28 publications

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“…In our study, of the three patients, two are alive with no evidence of recurrence after surgery and one died because of hepatic recurrence at 46 months after surgery. Bien, et al reported 20 pediatric and adolescent patients with pancreatoblastoma in a multicenter study [25]. The 5-year OS was 79.4% and the 5-year event-free survival was 58.8%.…”

Section: Discussionmentioning

confidence: 99%

Surgery of pancreas tumors in pediatric and adolescent patients: a single institution experience in South America

et al. 2021

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Pancreas tumors are extremely rare in pediatric and adolescent patients. Surgical resection is the mainstay of treatment; however, the data is 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 18 years old 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 stula as the most frequent. Under a median follow-up period of 33.8 (0.5-138) months, 4 (11.8%) patients died. Of the 29 patients with SPN, the 3-and-5-year OS was 100% and 83.1%, respectively. Conclusions SPN was the most frequent cause of surgical treatment for pediatric and adolescent patients in the highvolume cancer center in Peru and was associated with favorable survival. Pancreaticoduodenectomy was safely performed in this patient group with acceptable morbidity and zero mortality.

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Section: Discussionmentioning

confidence: 99%

Surgery of pancreas tumors in pediatric and adolescent patients: a single institution experience in South America

et al. 2021

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“…Although it is of no diagnostic value, it has utility in terms of marker of response to chemotherapy and of recurrence in postoperative period. 8 The treatment is complete resection of the lesion. 13 The resection may include excision, pancreaticoduodenectomy, and distal pancreatectomy depending on the site of the lesion.…”

Section: Discussionmentioning

confidence: 99%

“…For unresectable lesions neoadjuvant chemotherapy in the form of PLADO is generally recommended as these tumours have shown sensitivity to this regimen. 8,9 Histopathologically they exhibit dense cellularity with characteristic "squamoid corpuscles". 2 The 15 year survival rate in children is reported to be 61%.…”

Section: Discussionmentioning

confidence: 99%

“…6,7 The child might also have to be subjected to neoadjuvant chemotherapy in order to ensure complete surgical removal. 8 The chemo sensitivity and radio sensitivity is a debatable issue but lack of any standardized protocol makes it necessary to individualize the management. 9 We describe here a child diagnosed as pancreatoblastoma, who had unresectable disease but responded well to chemotherapy and complete resection was possible.…”

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confidence: 99%

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Acinar cell tumour of pancreas in a 4 year old child

Bendre

Munghate

Bodhanwala

2020

Preprint

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Paediatric pancreatic tumours by virtue of their rarity pose a significant challenge for the management.1 Although pancreatoblastoma and acinar cell carcinoma are distinct tumour entities, there is considerable overlap of histopathological characteristics which makes the diagnosis difficult pathologically.2 In infancy and childhood pancreatoblastoma is the most common tumour.3 Acinar cell carcinoma usually presents in older children.4,5 We describe a four year old child with acinar cell carcinoma which is possibly only the fourth case with this condition at such a young age.

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“…Elevated serum levels of CA19-9[ 14 , 15 ] as well as corticotropin releasing hormone secretion[ 20 ] rarely occurs in adult pancreatoblastomas. Serum alpha fetoprotein (AFP) is elevated in some pancreatoblastomas[ 4 , 7 , 13 , 21 , 22 ]. In addition, AFP may be detected immunohistochemically in tumours associated with elevated serum levels of AFP[ 3 , 4 , 22 ].…”

Section: Clinical Presentationmentioning

confidence: 99%

Adult pancreatoblastoma: Current concepts in pathology

Omiyale¹

2021

WJG

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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.

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Diagnosis and treatment of pancreatoblastoma in children: a retrospective study in a single pediatric center

Cited by 25 publications

References 28 publications

Surgery of pancreas tumors in pediatric and adolescent patients: a single institution experience in South America

Surgery of pancreas tumors in pediatric and adolescent patients: a single institution experience in South America

Acinar cell tumour of pancreas in a 4 year old child

Adult pancreatoblastoma: Current concepts in pathology

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