Tumor primitivo ectodérmico pancreático: relato de caso

Dias, André Roncon; Arantes, Thatyana; Sampaio, Renato Catojo; Jureidini, Ricardo; Cunha, José Eduardo Monteiro da; Cecconello, Ivan

doi:10.1590/s0102-67202013000200021

Cited by 5 publications

(3 citation statements)

References 11 publications

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“…What determines the familiarity between PNETs and ESs is the presentation, in both, of the same cytogenetic change. This change is seen in most of their malignant cells and consists of the characteristic chromosomal translocation t(11; 22) (q24; 12) [ 2 , 3 , 5 , 11 – 13 ]. More than 90% of the ES/PNET shows this specific and reproducible translocation that evolves with variable degrees of differentiation.…”

Section: Discussionmentioning

confidence: 99%

“…Because of this metastatic power, the association of chemotherapy with adequate local control by surgery or radiotherapy is prioritized. There is a trend of surgical treatment in most centers, even though it is not clear in the literature which method would give a better control of the primary tumor [ 2 , 6 , 12 , 13 ]. The prognostic factors are, however, controversial.…”

Section: Discussionmentioning

confidence: 99%

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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%

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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“…In reviewing the literature, 25 cases of pPNET of the pancreas including the present case have been reported to date (Table 1 ) [ 2 , 8 – 24 ]. The mean age at diagnosis was 18.2 ± 9.6 (2–37 years) and the median was 20 years old, which was 12 years older than that in the retrospective study of 975 Ewing tumors of bones in Europe [ 25 ].…”

Section: Case Presentationmentioning

confidence: 99%

A peripheral primitive neuroectodermal tumor originating from the pancreas: a case report and review of the literature

et al. 2015

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A peripheral primitive neuroectodermal tumor (pPNET) is a small round cell tumor occurring mostly in children or young adults and categorized into the Ewing sarcoma family of tumors. pPNETs originating from the pancreas are especially rare, and only 25 cases have been reported in the literature. We report a case of a 22-year-old man who had a giant expansive tumor located in the uncinate process of the pancreas, 80 mm in diameter resulting in obstruction in the duodenum. The patient underwent a pancreaticoduodenectomy. The histological examination showed that the pancreatic tumor was composed of atypical small round cells. Immunohistochemical findings were positive for CD99. An Ewing sarcoma breakpoint region 1 gene 22q12 rearrangement was proven by a two-color fluorescence in situ hybridization assay. We diagnosed the tumor as a pPNET of the pancreas, which, according to the literature, is highly aggressive with poor prognosis. A multidisciplinary approach to treat these neoplasms should improve the prognoses.

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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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Tumor primitivo ectodérmico pancreático: relato de caso

Cited by 5 publications

References 11 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

A peripheral primitive neuroectodermal tumor originating from the pancreas: a case report and review of the literature

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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