Cervicomedullary primitive neuroectodermal tumor of the spine: Case report

Nery, Breno; Pereira, Leandro César Tângari; Costa, Rodrigo Antônio Fernandes; Queiroz, Rodolfo Mendes; Abud, Lucas Giansante; Quaggio, Eduardo; Coronatto, Lígia Henriques; Prado, Isadora Salviano Teixeira; Miyake, Cecília Hissae; Filho, Fred Bernardes

doi:10.4103/sni.sni_41_18

Cited by 2 publications

(1 citation statement)

References 21 publications

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“…It develops from the primitive nerve cells of the nervous system (1). It is a member of Ewing's sarcoma family, mainly occurring in adolescents and in children (2). According to the differential classification, it can be divided into peripheral PNET (pPNET) and central PNET (cPNET).…”

Section: Introductionmentioning

confidence: 99%

Primitive neuroectodermal tumors: a clinical and radiological analysis of six cases

Liu

Zhao

Shi

et al. 2019

Quant. Imaging Med. Surg

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Background: Primitive neuroectodermal tumor (PNET) is extremely rare and highly aggressive with poor prognosis. Few studies concerning PNET's the imaging features have been published.Methods: Six cases of PNET, all confirmed by pathology and immunohistochemical (IHC) examinations, were treated during January 2012 to December 2016. These cases' clinical course and imaging findings were retrospectively analyzed.Results: Among six PNET cases, one located in left superior abdomen, one case at posterior abdominal wall, one case in right orbit, one case in left frontal temporal lobe, one case in pelvic cavity, and one case located in left supraclavicular fossa. The tumor's density was uniform for small tumor and heterogeneous for large tumors on CT images, while the size of tumors differed during presentation depending on the location of the tumor. Marked enhancement was visualized after injection of contrast media. The demarcation between the lesion and adjacent tissues or organs tended to be unclear. On magnetic resonance imaging (MRI) images, the mass mainly showed heterogeneously long T1 and long T2 signal intensity, mixed high signal intensity on fluid-attenuated inversion recovery (FLAIR) image. In two cases maximum intensity projection image reconstruction demonstrates tortuous blood vessels within the tumor on enhanced CT images.Five cases were treated by surgical resection with two cases received adjuvant radiotherapy and three cases received adjuvant chemotherapy. Six patients were followed up with a mean period of 34.5 months (ranging from 6 to 55 months). Five patients survived and one died. Among the five patients undergoing surgeries, one patient presented pelvic and abdominal recurrence/metastasis 2 months after abdominal PNET resection. One patient had a recurrent lesion in the right orbit involving the right ethmoid sinus 6 months after right orbital PNET resection. One patient's pelvic tumor recurred 7 months after PNET operation, and this patient died after 1 year and 10 months of follow-up. During the follow-up period, the remaining three cases did not show obvious recurrence and/or metastasis.Conclusions: Overall, the imaging appearances of PNET lack characteristics. PNETs generally do not have clear boundary, or partially so, with its adjacent organs or tissues suggesting their invasive nature. Upon further validation, maximum intensity projection image reconstruction demonstrates tortuous blood vessels within the tumor on enhanced CT images may be valuable information for the diagnosis of PNET.

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Section: Introductionmentioning

confidence: 99%

Primitive neuroectodermal tumors: a clinical and radiological analysis of six cases

Liu

Zhao

Shi

et al. 2019

Quant. Imaging Med. Surg

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Primitive neuroectodermal tumor in the cervical spine of a dog - Case Report

Silva,

Sousa,

Néspoli

et al. 2024

Arq. Bras. Med. Vet. Zootec.

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A case of peripheral primitive neuroectodermal tumor in the cervical region of a canine is described. The patient was a canine, male, five years old, large size, with acute neurological clinical signs, severe neck pain and tetraplegia. On computed tomography, an oval-shaped mass located dorsally to cervical vertebrae C4 to C7 was observed, with infiltration over the spinal cord between vertebrae C4 and C5, discreetly widening the dorsal intervertebral foramina of the cervical vertebrae and compressing the dorsal portion of the spinal cord of this segment. Under microscopy, the neoplastic cells were small, irregular in shape and with scarce cytoplasm. Immunoreactivity for vimentin, which can only be expressed in PTNEs tumors, was decisive for diagnostic confirmation. Additionally, the positivity of the S-100 protein, which is expressed in neuroectodermal cells mainly of the neural crest cells, reinforced the classification as PTNE, and the negativity in the expression for glial fibrillary acidic protein (GFAP) ruled out the presence of small cell glioma of the central nervous system. Based on the clinical history, imaging exams, cellular morphology and mainly on immunohistochemical staining, the diagnosis of primitive neuroectodermal tumor (PTNE) with infiltration and compression of the spinal cord in the cervical region of the dog was confirmed, considered uncommon in this species.

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Cervicomedullary primitive neuroectodermal tumor of the spine: Case report

Cited by 2 publications

References 21 publications

Primitive neuroectodermal tumors: a clinical and radiological analysis of six cases

Primitive neuroectodermal tumors: a clinical and radiological analysis of six cases

Primitive neuroectodermal tumor in the cervical spine of a dog - Case Report

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