Detection of Recurrent Pancreatic Primitive Neuroectodermal Tumor by Tc-99m Hydrazinonicotinyl-Tyr3-Octreotide Scan

Jing, Hongli; Li, Fang; Chen, Libo; Zhang, Taiping; Zhao, Yupei

doi:10.1097/rlu.0b013e3181feeffa

Cited by 14 publications

(1 citation statement)

References 14 publications

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“…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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Section: Reviewmentioning

confidence: 98%

Extraosseus Ewing's Sarcoma in Pancreas: A Review

Patel¹,

Nandu

Reddy

2020

Cureus

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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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Extraosseous Ewing’s sarcoma of the pancreas

et al. 2011

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The Ewing's family of tumors (EFT) comprises a molecularly defined group of "small round blue cell tumors", consisting of Ewing's sarcoma of bone (ESB), extraosseous Ewing's sarcoma (EES), peripheral primitive neuroectodermal tumor (pPNET), and Askin's tumor. Characteristic translocations that disrupt the EWSR1 gene located at 22q12 create novel fusion genes that are central to the pathogenesis. The EFT also shares certain clinical characteristics, such as a peak incidence during the teenage years, a tendency to spread rapidly, and responsiveness to the same chemotherapeutic regimens and radiation therapy. Nearly all patients have occult disseminated disease at diagnosis; hence, chemotherapy is routinely used. Improvements in multimodality treatment have had a dramatic impact on outcomes. EES/pPNET has been reported in a variety of sites, including the pancreas, though this is extremely rare. We describe a case of pancreatic EES/pPNET in a 35-year-old woman and provide a brief review of the relevant literature.

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Detection of Recurrent Pancreatic Primitive Neuroectodermal Tumor by Tc-99m Hydrazinonicotinyl-Tyr3-Octreotide Scan

Cited by 14 publications

References 14 publications

Extraosseus Ewing's Sarcoma in Pancreas: A Review

Extraosseus Ewing's Sarcoma in Pancreas: A Review

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

Extraosseous Ewing’s sarcoma of the pancreas

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