Cargando…

A rare case of pelvic primitive neuroectodermal tumor with misleading symptoms: A case report

Peripheral Primitive NeuroEctodermal Tumors (pPNETs) are rare highly malignant tumors; originating from the neuroectoderm. Although PNETs may arise in various locations (most commonly in the extremities), very few cases have been reported in the pelvis. There is still poor evidence about the managem...

Descripción completa

Detalles Bibliográficos
Autores principales: Ayoub, Kusay, Niazi, Ammar, Shebli, Baraa, Batal, Rand, Kozom, Hamed, Ghabreau, Lina, Mahli, Nihad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7251491/
https://www.ncbi.nlm.nih.gov/pubmed/32477503
http://dx.doi.org/10.1016/j.amsu.2020.05.010
Descripción
Sumario:Peripheral Primitive NeuroEctodermal Tumors (pPNETs) are rare highly malignant tumors; originating from the neuroectoderm. Although PNETs may arise in various locations (most commonly in the extremities), very few cases have been reported in the pelvis. There is still poor evidence about the management of these tumors in the literature. We present a rare case of pelvic PNET in a 20-year-old male. The patient presented with symptoms mimicking a lumbar disk hernia, which delayed the diagnosis. He was managed with a combination of a debulking procedure, adjuvant chemotherapy, radiotherapy; and has been in remission for 2 years upon follow-up. This case highlights the importance of diagnosing such aggressive tumors as early as possible (as prognosis may vary significantly), and the challenge in the management of PNETs due to poor evidence.