Pancreatic primitive neuroectodermal tumour associated with precocious puberty

Menon, Bina; Juraida, Eni; Mohamed, Mahfuzah; Manaf, Zaleha Abdul; Zahari, Zakaria; Yusuf, Salim; Ting, Tzer Hwu; Muda, Zulaiha; Ibrahim, Hishamshah Mohd

doi:10.1002/pbc.22099

Cited by 10 publications

(3 citation statements)

References 2 publications

(4 reference statements)

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“…After conducting a comprehensive search and review of the literature, we discovered that, to date, there have been 44 reported cases of pancreatic Ewing’s sarcoma ( 1 , 2 , 5 – 7 , 12 – 41 ), as shown in Table 1 . This count also includes the case from the current study.…”

Section: Discussionmentioning

confidence: 99%

“…Ewing sarcoma is characterized by a unique, small, round-cell sarcoma that exhibits gene fusions, typically involving one element from the FET family of genes, commonly Ewing Sarcoma Breakpoint Region 1 (EWSR1), and an element from the E-twenty-six (ETS) protein family of transcription factors. The most common Ewing sarcoma translocation, which is present in approximately 85% of cases, is t ( 1 , 2 )(q24;q12), leading to the EWSR1-FLI1 (Ewing Sarcoma Breakpoint Region 1–Friend Leukemia Integration 1) fusion transcript ( 3 ). The use of terms such as “Askin tumor” and “primitive neuroectodermal tumor (PNET) “ is not recommended according to the 2020 World Health Organization’s (WHO) classification of undifferentiated small round cell sarcomas of bone and soft tissue to prevent potential confusion or misunderstanding ( 3 ).…”

Section: Introductionmentioning

confidence: 99%

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Ewing sarcoma of the pancreas: a pediatric case report and narrative literature review

Liu,

Bian,

Yang

et al. 2024

Front. Oncol.

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Ewing’s Sarcoma (ES) is an rare, small round-cell sarcoma that predominantly occurs in children and young adults, with both skeletal and extraskeletal manifestations. However, pancreatic ES, due to its rarity, is infrequently featured in scholarly literature, with only a scant 43 reported instances. Our study describes a case of pancreatic ES in an 8-year-old boy who was found to have an abdominal mass. Following an exhaustive examination, the boy was diagnosed with a neoplasm in the pancreatic head and underwent a complex surgical procedure encompassing pancreatoduodenectomy and partial transverse colectomy. Immunohistochemical assays confirmed the neoplastic cells’ positivity for Cluster of Differentiation 99(CD99), Vimentin, and NK2 Homeobox 2(NKX2.2), while genomic testing identified an EWSR1-FLI1(Ewing Sarcoma Breakpoint Region 1–Friend Leukemia Integration 1) gene fusion. This led to a conclusive diagnosis of pancreatic Ewing’s Sarcoma. The patient underwent seven cycles of adjuvant chemotherapy, alternating between VDC (Vincristine, Doxorubicin, Cyclophosphamide) and IE (Ifosfamide, Etoposide) tri-weekly, but did not undergo radiotherapy. At present, the patient remains neoplasm-free. Through our case analysis and comprehensive review of the existing literature, we aim to underscore th rarity of pancreatic Ewing’s sarcoma and to highlight the efficacy of our individualized therapeutic approach.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Ewing sarcoma of the pancreas: a pediatric case report and narrative literature review

Liu,

Bian,

Yang

et al. 2024

Front. Oncol.

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“…The tumor often stains positive for neuroendocrine markers such as synaptophysin, CD56, and chromogranin. There have been five reported cases of pancreatic PNETs with local recurrence, three cases with lung metastasis and one case with bone metastasis [27][28][29][30].…”

Section: Reviewmentioning

confidence: 99%

Extraosseus Ewing's Sarcoma in Pancreas: A Review

Patel¹,

Nandu

Reddy

2020

Cureus

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Primitive neuroectodermal tumors (PNET, previously referred to as peripheral neuroepithelioma) are rare malignant tumors with various degrees of differentiation belonging to the Ewing's family of sarcomas. They are classified as round cell tumors arising from soft tissues. In rare instances, PNETs may arise from solid organs containing neuroendocrine cells of kidney, bladder, heart, lungs, parotid glands and pancreas. Most cases occur in the second decade of life with a slight preponderance in males. PNET of the pancreas is an aggressive tumor with multiple recurrences and a relatively poor prognosis. These tumors should be considered in the differential diagnosis, especially in a diagnosed pancreatic tumor in individuals less than 35 years of age. Due to the nature of the tumor, surgery with subsequent chemoradiation are widely accepted modalities despite the poor prognosis. In this article, we review 25 cases of extraosseous Ewing's sarcoma (ES) of the pancreas which to the best of our knowledge, enlists most cases reported in the literature thus far.

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Pancreatic Neoplasms in Children

Adzick

Laje

2016

The Surgery of Childhood Tumors

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Pancreatic primitive neuroectodermal tumour associated with precocious puberty

Cited by 10 publications

References 2 publications

Ewing sarcoma of the pancreas: a pediatric case report and narrative literature review

Ewing sarcoma of the pancreas: a pediatric case report and narrative literature review

Extraosseus Ewing's Sarcoma in Pancreas: A Review

Pancreatic Neoplasms in Children

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