Simultaneous Occurrence of Pancreatoblastoma and Neuroblastoma in a Newborn With Beckwith-Wiedemann Syndrome

Sorrentino, Stefania; Conte, Massimo; Nozza, Paolo; Granata, Claudio; Capra, Valeria; Avanzini, Stefano; Garaventa, Alberto

doi:10.1097/mph.0b013e3181dccd1e

Cited by 17 publications

(6 citation statements)

References 20 publications

(18 reference statements)

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“…Literature review reveals a significant variation in the type of adrenal mass detected in BWS (Table ) . We subcategorized these masses into the three most commonly reported adrenal findings: adrenal hyperplasia and cysts, adrenal adenoma, and neuroendocrine tumors; we also report other masses and tumors such as adrenal calcification and adrenal carcinoma.…”

Section: Resultsmentioning

confidence: 99%

Management of adrenal masses in patients with Beckwith–Wiedemann syndrome

MacFarland

Mostoufi‐Moab

Zelley

et al. 2017

Pediatric Blood & Cancer

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Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth and cancer predisposition syndrome, associated with both benign and malignant adrenal findings. Literature review and an institutional case series elucidate the wide spectrum of adrenal findings in BWS patients. The altered expression of the 11p15 region is likely related to adrenal gland hyperplasia and growth dysregulation. Given the absence of guidelines for managing adrenal findings in BWS, we propose a systematic approach to adrenal findings in BWS patients, to allow for maximum detection of potentially malignant pathology without posing additional risk to patients.

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Section: Resultsmentioning

confidence: 99%

Management of adrenal masses in patients with Beckwith–Wiedemann syndrome

MacFarland

Mostoufi‐Moab

Zelley

et al. 2017

Pediatric Blood & Cancer

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“…10 In our patient, liver metastasis occurred. Beckwith–Wiedemann syndrome 11 and Cushing’s syndrome 12 can co-occur with this tumour in some patients, and the specific mechanisms need to be further studied.…”

Section: Discussionmentioning

confidence: 99%

Adult pancreatoblastoma: a case report

Peng

Fan

et al. 2021

J Int Med Res

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Pancreatoblastoma (PB) is a rare epithelial malignancy usually occurring in the paediatric population. Adult PB is rare, and its imaging findings are similar to those of other tumours, making preoperative diagnosis a considerable challenge. We report correlative ultrasound, contrast-enhanced ultrasonography, contrast-enhanced magnetic resonance imaging, and positron emission tomography-computed tomography findings in a 60-year-old woman with PB. PB often presents with uncommon imaging features and should be considered in the differential diagnosis of pancreatic masses. It is important for clinicians to be aware of these differences to provide effective treatment.

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“…Cases in adults have also been described [41], [42]. They are usually sporadic, but some arise in patients with the Beckwith-Weidemann syndrome, and pancreatoblastomas in these patients can be congenital and are often cystic [14], [43], [44], [45], [46]. A case of FAP-associated pancreatoblastoma has been reported as well [23].…”

Section: Pancreatoblastomamentioning

confidence: 95%