Imaging features of peripheral primitive neuroectodermal tumours

Abstract

Primitive neuroectodermal tumours (PNETs) are a rare group of extremely aggressive small round cell tumours. Most commonly, they occur within the central nervous system. However, they also sporadically involve extracranial sites (peripheral PNETs). The chest wall is the most common of these sites, the imaging features of which have been well described in earlier studies. The present pictorial essay illustrates the imaging features of PNETs at various extracranial locations.

Peripheral primitive neuroectodermal tumour – a rare cause of a popliteal fossa mass: A case report and review of the literature

“…Belonging to a family of 'small round-cell tumours', they are most frequently observed in children and young adults, but can occur at any age (12,13). Due to their aggressive nature, distant micrometastasis must always be assumed to be present and, hence, all patients should receive adjuvant chemotherapy for systemic control (13,14).…”

“…pPNETs are commonly classed with ES and Askin's tumour (pPNETs limited to the thoracopulmonary region) as members of the Ewing family of tumours (8,12,13,(15)(16)(17). They have almost identical pathological, immunohistochemical and genetic features, with the main distinct difference being that tumours demonstrating neural differentiation are labelled pPNET while those that are undifferentiated are diagnosed as ES (3,12,15,18,19).…”

Primarni ekstraskeletalni Ewing-ov sarkom torakalnog zida – prikaz bolesnika

“…ESFT se neznatno češće javlja kod osoba muškog pola. Većina pacijenata su bijele rase, izuzetno se rijetko javlja kod Afro-Amerikanaca [1,4,7,[10][11][12]. Simptomatologija zavisi od mjesta gdje se tumor razvije.…”

“…Kalcifikacije su atipične i javljaju se u oko 10% slučajeva. Nodalne metastaze su rijetke [3,10,11,13,16]. Magnetna rezonanca (MR) i CT ne mogu uvijek pouzdano diferencirati kompresiju pluća od infiltracije.…”

“…Magnetna rezonanca (MR) i CT ne mogu uvijek pouzdano diferencirati kompresiju pluća od infiltracije. MR je pouzdaniji u analizi invazije torakalnog zida, dok je CT senzitivniji za male plućne metastaze [7,[10][11][12]. MR, CT i PET/ CT imaju dopunsku ulogu u procjenjivanju lokalne tumorske proširenosti i resektabilnosti te u procjeni diseminacije ovog oboljenja [7,8,10,16].…”