Primitive neuroectodermal tumor arising in the abdominopelvic region: CT features and pathology characteristics

Li, Xinchun; Zhang, Weidong; Song, Ting; Sun, Chongpeng; Shen, Yuechun

doi:10.1007/s00261-010-9655-z

Cited by 19 publications

(30 citation statements)

References 15 publications

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“…6,7,[9][10][11][12][13][14][15][16]22 This may explain why these tumours grow rapidly and are easy to show cystic or necrotic changes; besides, these feeding vessels could help locate the origin of the lesions. Although calcifications are rare in pPNET, 9,10,22,23 calcifications were found in three lesions in our study showing linear or punctiform shapes, and the lesions were located in the site of the pleura, pancreas and adrenal gland. Hence, in regard to a huge soft-tissue mass with calcifications in children or adolescents, the diagnosis of pPNET should be considered, except when there is a diagnosis of childhood blastoma, such as neuroblastoma, nephroblastoma, pancreatoblastoma, hepatoblastoma or teratoma.…”

Section: Discussionmentioning

confidence: 93%

CT and clinical findings of peripheral primitive neuroectodermal tumour in children

Xiao

Bao

Tan

et al. 2016

BJR

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Section: Discussionmentioning

confidence: 93%

CT and clinical findings of peripheral primitive neuroectodermal tumour in children

Xiao

Bao

Tan

et al. 2016

BJR

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“…Primitive neuroectodermal tumors (PNETs) are a group of extremely rare malignant tumors that originate from the neuroectoderm and are composed of primitive undifferentiated small round cells [1,2]. Based upon the 2013 WHO classification of tumors of soft tissue and bone, PNETs belong to the Ewing’s sarcoma family of tumors, and more than 85% of PNET and Ewing sarcoma tumor cells exhibit the same chromosomal translocation (t(11;22)(q24;q12)) [3,4].…”

Section: Introductionmentioning

confidence: 99%

“…pPNETs are commonly located in the soft tissues of the thoracopulmonary region (Askin tumor), paraspinal region, and limbs. Tumors originating in the intra-abdominal and retroperitoneal regions, head and neck regions and bone are relatively rare [2,6–8]. …”

Section: Introductionmentioning

confidence: 99%

Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases

Yi¹,

Liu²,

Zhang³

et al. 2017

PLoS ONE

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ObjectivesTo characterize the imaging and clinicopathological features of primitive neuroectodermal tumors (PNETs) arising in intra-abdominal and retroperitoneal regions.MethodsEighteen patients with histopathologically proven intra-abdominal and retroperitoneal PNET were enrolled; computed tomography was performed for all cases, and magnetic resonance imaging was performed for a single case. Typical computed tomography and magnetic resonance imaging findings, including morphology, texture and enhancement features, as well as clinicopathological characteristics and prognosis data were retrospectively analyzed.ResultsOf eighteen PNET patients, fifteen were male and three were female, with a median age of 36 years (range, 2–65 years). The onset of symptoms was most often nonspecific and insidious. The mean tumor diameter was 7.2 cm (range, 3.0–12.1 cm), with necrosis in fifteen cases, cystic changes in eight, partition structure in five, calcification in five, hemorrhage in two, and mural nodules in one. Contrast enhanced computed tomography showed multiple tiny feeding arteries within the masses in six cases, resulting in a crab-like appearance, and mild ring enhancement pattern in five cases. Eleven cases showed surrounding invasion and metastasis. Of the eighteen PNET cases, nine cases showed smooth, well-defined margins, and nine cases had irregular, ill-defined margins. A median survival was 10.0±1.6 months. However, chemotherapy had efficacy on patients even those with advanced disease.ConclusionsPrimary intra-abdominal and retroperitoneal PNETs are rare, and imaging features documented here may help the diagnosis of this severe disease. Notably, two signs present in retroperitoneal PNET tumors, including a mild ring enhancement pattern and a crab-like appearance of the tiny feeding arteries, may have the potential to help us improve the ability to make a relatively reliable diagnosis.

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“…Pacijenti se najčešće žale na nejasnu bol, kao što je bio slučaj i kod našeg bolesnika, i palpabilnu, brzo rastuću masu. S obzirom da se radi o rijetkom tumoru kod odraslih, dijagnoza i tretman pacijenata sa ovim oboljenjem predstavlja veliki izazov [1,5,13,14]. Pacijenti sa metastatskom bolešću mogu imati povišenu temperaturu, malaksalost, biohumoralne znakove inflamacije te povišen LDH [1][2][3][4][5]15].…”

Section: Diskusijaunclassified

“…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.…”

Section: Diskusijaunclassified

Primarni ekstraskeletalni Ewing-ov sarkom torakalnog zida – prikaz bolesnika

Stajčić¹,

Vujnović²,

Radosavljević³

et al. 2014

Biomedicinska istraž.

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Kratak sadržajUvod. Ektraskeletalni Ewing-ov sarkom (EES) je neuroektodermalni tumor koštanog porijekla, koji se najčešće javlja u drugoj deceniji života. EES je izuzetno rijedak tumor. Cilj rada je prikazati RTG i CT nalaze kod rijetkog oblika torakalne lokalizacije EES kao što je slučaj kod našeg pacijenta.Prikaz bolesnika. U radu predstavljamo EES porijekla lijeve visceralne pleure, koji je dijagnostikovan kod dvadesetjednogodišnjeg muškarca, sa neodređenim bolom u ramenu. Inicijalno je načinjena radiografija grudnog koša, na kojoj je uočena pleuralna efuzija i jasno ograničena mekotkivna masa duž lijevog lateralnog torakalnog zida. Neposredno urađena je kompjuterizovana tomografija grudnog koša, koja jasnije prikazuje difuznu distribuciju multiplih nodularnih mekotkivnih zadebljanja pleure lijevog hemitoraksa. Histološki je dokazan EES visceralne pleure.Zaključak. EES -primitivni neuroektodermalni tumor je rijedak tumor koji treba razlikovati od drugih tumora malih okruglih ćelija na osnovu njegove morfologije i laboratorijskih nalaza. Iako je ekstremno rijedak , EES treba da bude uzet u obzir u diferencijalnoj dijagnozi primarnih tumora zida grudnog koša.Ključne riječi: ektraskeletalni Ewing-ov sarkom, tumori torakalnog zida

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Primitive neuroectodermal tumor arising in the abdominopelvic region: CT features and pathology characteristics

Cited by 19 publications

References 15 publications

CT and clinical findings of peripheral primitive neuroectodermal tumour in children

CT and clinical findings of peripheral primitive neuroectodermal tumour in children

Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases

Primarni ekstraskeletalni Ewing-ov sarkom torakalnog zida – prikaz bolesnika

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