Uncommon Presentation of Rare Disorder—Duodenal Ulcer Secondary to Invasive Pancreatic Primitive Neuroectodermal Tumor

Maxwell, Laura; Hederman, April; Jackson, Courtney; Sawaya, David; Giles, Henry; Nowicki, Michaeł

doi:10.1097/mph.0b013e31821041af

Cited by 11 publications

(4 citation statements)

References 34 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In pancreatic PNETs, specific characters of PNET like Homer-Wright (H-W) rosette or atypical rosette array of the cells are rarely present under light microscopy. The important criteria for the diagnosis of ES/PNETs is the cell cytoplasm containing neuronal secretory granules, neurofilaments, and pyknic nucleus granules [25,26]. From our present review, neural markers were positive for NSE (10/25 cases), synaptophysin (2/25 cases) and vimentin (6/25 cases).…”

Section: Reviewmentioning

confidence: 68%

Extraosseus Ewing's Sarcoma in Pancreas: A Review

Patel¹,

Nandu

Reddy

2020

Cureus

View full text Add to dashboard Cite

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.

show abstract

Section: Reviewmentioning

confidence: 68%

Extraosseus Ewing's Sarcoma in Pancreas: A Review

Patel¹,

Nandu

Reddy

2020

Cureus

View full text Add to dashboard Cite

show abstract

“…Sin embargo, el sarcoma de Ewing también puede afectar tejidos blandos. 2,7 La histología característica de estos tumores son células pequeñas, redondas y azules; es común en niños, adolescentes y adultos jóvenes. 1,5 Se considera que no hay diferencia histológica entre los tumores de esta familia.…”

Section: Discussionunclassified

“…1,5 Se considera que no hay diferencia histológica entre los tumores de esta familia. 2,8 Los tumores de páncreas son extremadamente raros en niños. 7,5 Los tumores neuroectodérmicos primitivos son histológicamente indistinguibles de los del tipo óseo y se han documentado en otros sitios como vagina, septo retrovaginal, intestino delgado, ovarios, próstata, esófago y riñón.…”

Section: Discussionunclassified

“…1 Los tumores neuroectodérmicos primarios son tumores de células pequeñas y redondas derivadas del tejido blando, pertenecen a la familia del sarcoma de Ewing. 2,3 Estas neoplasias exhiben un fenotipo neural, expresan la proteína MIC2-(CD99) y 85% de los casos muestra translocación cromosómica t(11:22) (q24; q12). 4,5 El tumor neuroectodérmico primario puede presentarse en órganos sólidos como riñón, pulmón, ovario, vejiga y páncreas, entre otros.…”

Section: Introductionunclassified

See 1 more Smart Citation

Tumor neuroectodérmico primitivo primario de páncreas en un paciente pediátrico. Reporte de caso y revisión de la literatura

et al. 2016

View full text Add to dashboard Cite

The primitive neuroectodermal tumor of the pancreas, are small round cell tumors arising from the soft tissue that belongs to the family of Ewing's sarcoma, The primary neuroectodermal (PNET) of the pancreas is very rare, have been reported only 17 cases in the literature. We report the case of a 9 year-old female patient, with PNET of the pancreas, was carried out a Whipple surgery, and began chemotherapy treatment with, vincristine, doxorubicin Pegylated, etoposide and ifosfamide and radiation therapy. Currently, the patient is in monitoring since March 2013, with a follow up of 23 months, overall in good condition and without evidence of tumor activity. Tumor neuroectodérmico primitivo primario de páncreas en un paciente pediátrico. Reporte de caso y revisión de la literatura RESUMEN Los tumores neuroectodérmicos primitivos son tumores de células pequeñas y redondas derivadas del tejido blando, pertenecen a la familia del sarcoma de Ewing. El tumor neuroectodérmico primario del páncreas es muy poco frecuente, en la literatura sólo se han reportado 17 casos. Reportamos el caso de una paciente de 9 años de edad, con diagnóstico de tumor neuroectodérmico primario del páncreas a la que se le realizó una cirugía de Whipple e inició tratamiento de quimioterapia con ciclofosfamida, vincristina, doxorrubicina pegilada, etopósido e ifosfamida y radioterapia. Desde marzo de 2013 la paciente se encuentra en vigilancia, con un periodo de seguimiento de 23 meses. Actualmente, se encuentra en buenas condiciones generales y sin evidencia de actividad tumoral.Palabras clave: tumor neuroectodérmico primitivo, tumor neuroectodérmico primitivo primario de páncreas, procedimiento de Whipple.

show abstract

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

et al. 2018

View full text Add to dashboard Cite

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.

show abstract

Uncommon Presentation of Rare Disorder—Duodenal Ulcer Secondary to Invasive Pancreatic Primitive Neuroectodermal Tumor

Abstract: The authors report an unusual presentation of a rare tumor in childhood, a pancreatic primitive neuroectodermal tumor invading the duodenum leading to anemia. A review of intra-abdominal primitive neuroectodermal tumors is discussed.

Cited by 11 publications

References 34 publications

Extraosseus Ewing's Sarcoma in Pancreas: A Review

Extraosseus Ewing's Sarcoma in Pancreas: A Review

Tumor neuroectodérmico primitivo primario de páncreas en un paciente pediátrico. Reporte de caso y revisión de la literatura

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

Contact Info

Product

Resources

About