Primitive neuroectodermal tumor of the pancreas

Perek, Sadık; Perek, Asiye; Şarman, Kemal; Tüzün, Hasan; Büyükünal, Evin

doi:10.1159/000071776

Cited by 26 publications

(2 citation statements)

References 3 publications

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“…A third patient effectively treated with vincristine, doxorubicin, and cyclophosphamide was disease-free after 43 months. Perek et al [ 19 ] reported the case of a man who underwent three surgical procedures associated with first, second, and third lines of chemotherapy for metastatic PNET, getting one of the largest known global survival, 50 months.…”

Section: Discussionmentioning

confidence: 99%

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

Teixeira

Goldoni

Unterleider

et al. 2015

Case Reports in Surgery

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Primitive neuroectodermal tumors (PNETs) are presented as rare malignant neoplasms. In unusual cases, those neoplasms may arise in solid organs containing neuroendocrine cells, such as the pancreas. Herein the case of a 28-year-old patient that underwent gastroduodenopancreatectomy after the diagnosis of a huge mass (PNET) located in both head and body of the pancreas is reported. This is the 19th case of pancreatic PNET reported in literature.

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Section: Discussionmentioning

confidence: 99%

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

Teixeira

Goldoni

Unterleider

et al. 2015

Case Reports in Surgery

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“…1 They are identified as part of the Ewing sarcoma fam-ily of tumors because they harbor the same cytogenetic abnormality involving the EWSR1 gene on 22q12, namely the chromosomal translocation t(11;22)(q24;q12) creating the EWS/FLI-1 fusion gene. 2 They usually occur in childhood and adolescence, and, in the majority of cases, they originate in the peripheral nervous system and soft tissue of the thorax, the pelvis, and the lower extremities; occasionally, they originate in the solid organs containing neuroendocrine cells (such as the pancreas, kidney, ovary, and uterus). 3 To our knowledge, except for the present case, there have been only 14 reports of pancreatic PNETs, accounting for approximately less than 0.5% of all primary pancreatic tumors.…”

mentioning

confidence: 99%

Image of the Month—Diagnosis

2012

Arch Surg

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Extra-osseous Ewing sarcoma of the pancreas: case report with radiologic, pathologic, and molecular correlation, and brief review of the literature

et al. 2018

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In 2002, due to extensive histomorphologic, immunohistochemical, and cytogenetic similarities, the World Health Organization unified undifferentiated small round blue cell neoplasms of soft tissue and bone (previously segregated as Ewing sarcoma or Primitive Neuroectodermal tumor) into one category: Ewing family of tumors (EFT). Osseous EFT are more common, and while extra-osseous EFT can occur anywhere in the body, those of the pancreas are rare and likely to be seen in the second decade of life in the head of the pancreas. We report the case of a 39-year-old Caucasian male with a large heterogeneously enhancing mass in the pancreatic body. Pathologic examination showed a malignant round blue cell tumor diffusely positive for CD99, chromogranin, and synaptophysin; Ki-67 proliferation index was greater than 80%. FISH showed EWSR1 gene rearrangement in 90% of cells and Archer FusionPlexTM-targeted RNA sequencing analysis identified the EWSR1-FLI1 fusion transcript. The diagnosis of EFT of the pancreas was rendered. Unfortunately, the patient had minimal improvement and was transitioned to oral pain medications to continue care at a different institution.

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Primitive neuroectodermal tumor of the pancreas

Cited by 26 publications

References 3 publications

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

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

Image of the Month—Diagnosis

Extra-osseous Ewing sarcoma of the pancreas: case report with radiologic, pathologic, and molecular correlation, and brief review of the literature

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