Pancreatoblastoma in Adults: A Review of the Literature

Cavallini, Alvise; Falconi, Massimo; Bortesi, Laura; Crippa, Stefano; Barugola, Giuliano; Butturini, Giovanni

doi:10.1159/000178877

Cited by 55 publications

(58 citation statements)

References 27 publications

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“…Symptoms are typically vague and non-specific (3,17,24,30). For example, the majority of adult patients present with abdominal pain or a palpable mass.…”

Section: Discussionmentioning

confidence: 99%

“…The liver has been found to be the most common site of distant metastasis and 25% of cases are diagnosed with secondary liver disease upon initial staging (19,24,30). However, bone, pulmonary, peritoneal, brain and mediastinal metastases have also been described (19,26).…”

Section: Discussionmentioning

confidence: 99%

“…In paediatric PB patients, elevated AFP and CEA levels are the most common abnormal serological markers, and elevated AFP expression has been reported in ≤68% of cases (12,24,25,32), By contrast, these PB tumour markers are typically within the normal range in adult patients (1,3). Serum AFP levels may be used to monitor clinical response to therapy or recurrence in those patients whose tumors produce it (32).…”

Section: Discussionmentioning

confidence: 99%

“…The majority of PB tumours are well-defined, large and heterogeneous on cross-sectional imaging, with mixed solid-cystic appearance (3,19,24,26,30). On magnetic resonance, the tumours are most commonly described as heterogeneous with low-intermediate T1 signal intensity and high T2 intensity.…”

Section: Discussionmentioning

confidence: 99%

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Adult pancreatoblastoma: A case report and review of the literature

et al. 2015

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Abstract. The present study describes the case of a 24-year-old patient who presented with obstructive jaundice and weight loss, and was diagnosed with pancreatoblastoma (PB). Abdominal imaging studies revealed a heterogenous lesion of the pancreatic head with dilatation of the common bile duct. The patient underwent pancreaticoduodenectomy, however, three months after surgery multiple liver and bone metastases were identified on follow-up computed tomography scans. Despite treatment with four cycles of systemic chemotherapy and five courses of radiofrequency ablation, the patient succumbed due to tumour dissemination 13 months after initial diagnosis. PB is a malignant tumour of the pancreas that typically occurs in the pediatric population. The aim of the present study was to highlight the aggressive behavior of this rare clinical entity, focusing on the pitfalls of pre-operative diagnosis and the lack of management strategy guidelines in adults. Preoperative diagnosis of PB based on radiographic features may be difficult, as the imaging characteristics are non-specific. Furthermore, cytology may also be misleading, as the neoplasm consists of multiple cell lines (acinar, ductal and neuroendocrine cells) and diagnosis depends largely on the identification of the distinctive histological characteristic of squamoid corpuscles, which present as nests of flattened cells with a squamous appearance. Despite the use of surgical resection and adjuvant chemoradiotherapy for the treatment of this malignancy, its aggressive nature means that PB is associated with a poor prognosis in adult patients.

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“…Symptoms are typically vague and non-specific (3,17,24,30). For example, the majority of adult patients present with abdominal pain or a palpable mass.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Adult pancreatoblastoma: A case report and review of the literature

et al. 2015

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“…No targeted therapies have been shown to be effective to date. Various adjuvant chemotherapy regimens have been applied, and ambiguous results have been published as case reports [151]. As for most pancreatic malignancies, surgical resection is still the gold standard.…”

Section: Pancreatoblastomamentioning

confidence: 99%

The genetic classification of pancreatic neoplasia

2015

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Cancer is caused by the accumulation of inherited and/or acquired alterations in specific genes. The recent decline in the cost of DNA sequencing has allowed tumor sequencing to be conducted on a large scale, which, in turn, has led to an unprecedented understanding of the genetic events that drive neoplasia. This understanding, when integrated with meticulous histologic analyses and with clinical findings, has direct clinical implications. The recent sequencing of all of the major types of cystic and noncystic neoplasms of the pancreas has revealed opportunities for molecular diagnoses and for personalized treatment. This review summarizes the results from these recent studies focusing on the clinical relevance of genomic data.

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Uncommon Cancers of the Pancreas

Diab

Philip

2017

Textbook of Uncommon Cancer

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Pancreatoblastoma in Adults: A Review of the Literature

Cited by 55 publications

References 27 publications

Adult pancreatoblastoma: A case report and review of the literature

Adult pancreatoblastoma: A case report and review of the literature

The genetic classification of pancreatic neoplasia

Uncommon Cancers of the Pancreas

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