Primitive Neuroectodermal Tumor of the Pancreas: A Case Report and Review of the Literature

Teixeira, Uirá Fernandes; Goldoni, Marcos Bertozzi; Unterleider, Michelle; Diedrich, João; Balbinot, D.R.; Rodrigues, Pablo Duarte; Monteiro, Rodolfo; Gomes, Daniel; Sampaio, José Artur; Fontes, Paulo Roberto Ott; Waechter, Fábio Luiz

doi:10.1155/2015/276869

Cited by 9 publications

(19 citation statements)

References 20 publications

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“…Primitive neuroectodermal tumors usually originate in the bone and soft tissue, and there have been cases where they originated from solid organs containing neuroendocrine cells, like the pancreas, which accounts for 0.3% of primary pancreatic neoplasms at all ages[ 33 ].…”

Section: Pancreatic Malignant Tumors In Childrenmentioning

confidence: 99%

Could the burden of pancreatic cancer originate in childhood?

Diaconescu¹,

Gîlcă-Blanariu²,

Poamaneagra³

et al. 2021

WJG

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The presence of pancreatic cancer during childhood is extremely rare, and physicians may be tempted to overlook this diagnosis based on age criteria. However, there are primary malignant pancreatic tumors encountered in pediatric patients, such as pancreatoblastoma, and tumors considered benign in general but may present a malignant potential, such as the solid pseudo-papillary tumor, insulinoma, gastrinoma, and vasoactive intestinal peptide secreting tumor. Their early diagnosis and management are of paramount importance since the survival rates tend to differ for various types of these conditions. Many pediatric cancers may present pancreatic metastases, such as renal cell carcinoma, which may evolve with pancreatic metastatic disease even after two or more decades. Several childhood diseases may create a predisposition for the development of pancreatic cancer during adulthood; hence, there is a need for extensive screening strategies and complex programs to facilitate the transition from pediatric to adult healthcare. Nevertheless, genetic studies highlight the fact the specific gene mutations and family aggregations may be correlated with a special predisposition towards pancreatic cancer. This review aims to report the main pancreatic cancers diagnosed during childhood, the most important childhood diseases predisposing to the development of pancreatic malignancies, and the gene mutations associates with pancreatic malignant tumors.

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Section: Pancreatic Malignant Tumors In Childrenmentioning

confidence: 99%

Could the burden of pancreatic cancer originate in childhood?

Diaconescu¹,

Gîlcă-Blanariu²,

Poamaneagra³

et al. 2021

WJG

View full text Add to dashboard Cite

show abstract

“…Advancing age, advanced stage tumors, non-functioning tumors and those with rapid growth generally have poor outcomes. In patients with advanced PNETs with metastasis, the development of new therapeutic options that arrest tumor growth and progression have shown to be promising [7,29,30,31,32,33,34]. From our review, 4 out of 25 cases died with the disease within the range of 6 months to 50 months from the time of diagnosis while one died with postoperative complications.…”

Section: Reviewmentioning

confidence: 98%

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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“…Ewing sarcoma and PNET are part of a spectrum of bone and soft tissue tumours known as the Ewing Sarcoma Family of Tumours (ESFT). At one end, PNET shows neural differentiation while Ewing sarcomas are undifferentiated . More than 50% of primary adult cases are extra‐skeletal and can occur in any soft tissue with pain and compression to surrounding structures .…”

mentioning

confidence: 99%

“…Obtaining a tissue biopsy for histological and molecular confirmation is therefore crucial given the broad spectrum of differentials to consider. Both Ewing sarcoma and PNET share the common cytogenetic change of t(11; 22) (q24; 12) chromosomal translocation . Differentials include rhabdomyosarcomas, which stain positively for actin, desmin and myoglobin, hemangiopericytomas which stain for factor VIII and small‐cell metastatic carcinomas and melanomas which stain for cytokeratin …”

mentioning

confidence: 99%

“…Case reports have suggested that in the absence of unresectable metastatic disease, alternating vincristine‐based chemotherapy with IE followed by radical en bloc surgery with completion of chemoradiotherapy offered the best outcomes . ESFTs are aggressive tumours and recurrences necessitating repeat surgery can be considered . Ultimately, a multi‐disciplinary approach towards the management of ESFTs is paramount.…”

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confidence: 99%

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