Pancreatic blastomatous tumor in a child responding to therapy used for hepatoblastoma: Case report and review of the literature

Morgan, Elaine; Perryman, John H.; Reynolds, Marleta; González-Crussí, Frank

doi:10.1002/(sici)1096-911x(199604)26:4<284::aid-mpo13>3.0.co;2-f

Cited by 10 publications

(8 citation statements)

References 19 publications

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“…Pancreatoblastoma shares similar histologic and morphologic features with hepatoblastoma including secretion of a-fetoprotein [11]. In addition, it appears to affect a similar age group and it is generally responsive to the same chemotherapeutic agents (high risk Hepatoblastoma protocol) with a satisfactory clinical response [11]. Pancreatoblastoma can be associated with Beckwith-Wiedemann syndrome [12].…”

Section: Discussionmentioning

confidence: 99%

Pancreatic tumours in children: diagnosis, treatment and outcome

et al. 2015

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Paediatric pancreatic tumours are rare entities and are usually benign. Clinical symptoms are often non-specific and presentation may be late due to tumour inactivity in case of endocrine neoplasms. For most tumours, surgical resection is the optimal treatment which may be successfully performed laparoscopically when the lesion is in the body or tail of the pancreas. Long-term outcome is generally good.

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Section: Discussionmentioning

confidence: 99%

Pancreatic tumours in children: diagnosis, treatment and outcome

et al. 2015

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“…Pancreatic tumours are rarely encountered in paediatric practice. AFP is an important tumour marker in pancreatoblastoma, the commonest malignant paediatric pancreatic tumour [1][2][3]. Its association is also documented in acinar cell tumours of the pancreas [4,5].…”

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Don't assume an α‐fetoprotein secreting tumour is of germ cell or hepatic origin

Sharathkumar

Shaw

Cooke‐Yarborough

et al. 2002

Med. Pediatr. Oncol.

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Alpha-fetoprotein (AFP) is a valuable marker for the detection and follow-up of germ cell and hepatic neoplasms. Although the literature is replete with reports of AFP-secreting pancreatic tumours, this association is often forgotten [1][2][3][4][5]. This is especially likely when the clinical picture and laboratory data lead in another direction, as they did in the case of a 13-year old boy who came under our care. He presented in status epilepticus, requiring admission to the Paediatric Intensive Care Unit. His clinical examination revealed facial acne, a right eye ptosis, and a mass in the right lumbar and iliac region. He also had hypertension (systolic 140-160 and diastolic 110-120 mmHg) requiring therapy. His hypertension and ptosis improved spontaneously within 4 days of admission. The initial diagnosis was considered to be a primary malignant tumour in the abdomen, probably originating from an adrenal gland, with cerebral metastases. Phenytoin was started. Haematology and coagulation parameters were normal. The urinary cortisol level was significantly elevated (635 nmol/24h, normal value 5-90 nmol/24h) although his hormonal assays (LH, FSH, testosterone, ACTH, cortisol, DHEA-SO 4 , and T 4 ) were normal. He had a slightly elevated LDH of 740 U/l (normal 260-520 U/l) and a very high AFP (160,780 IU/ ml; normal level <16 IU/ml). Other biochemical parameters (renal and liver function, amylase) and tumour markers (NSE, CEA, and b-HCG) were normal. His MRI and CT scans confirmed a retroperitoneal lobulated tumour with cystic and solid areas, 10 Â 11 Â 13 cm, situated just below the liver and extending to the right iliac crest. On MRI the mass was separate from the right kidney and liver but the right adrenal could not be visualised. The mass was displacing the proximal body of the pancreas anteriorly. His cerebral MRI had multiple areas of high signal intensity within the subcortical region suggestive of infarcts, possibly secondary to tumour emboli. A PET scan failed to reveal any areas of glucose hypermetabolism within the brain, so favouring a vascular accident rather than tumour metastasis.The retroperitoneal mass was thallium avid but imaging did not reveal any evidence of dissemination. He underwent an open tumour biopsy. Intra-operatively, the mass was considered to be separate from the liver and kidney, though the organ of origin was not apparent.

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“…Она име-ет некоторые сходства, в том числе морфологи-ческие, с гепатобластомой и встречается в одной возрастной группе. Обе опухоли обычно наблю-даются в ассоциации с синдромом Беквита-Видемана и часто демонстрируют повышенный уровень альфа-фетопротеина (АФП) в плазме крови [7][8][9][10][11]. Имеются значительные сходства и на молекулярно-генетическом уровне, такие как потеря гетерозиготности 11p15.5, гиперэк-спрессия инсулиноподобного фактора роста-2 (Insulin-like growth factor 2, IGF2) и мутация гена бета-катенина [12].…”

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“…Общая выживаемость достига-ет 80% у детей с полностью резектабельными опу-холями на момент постановки диагноза [19]. При наличии отдаленных метастазов (обычно в печени или скелете) результаты неблагоприятны [11,15,20]. Опубликовано много работ, где химиотерапия, особенно цисплатинсодержащие схемы, приводи-ла к уменьшению опухолевой массы [11,[21][22][23][24][25].…”

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