Disseminated Cerebrospinal Embryonal Tumor in the Adult

Caporlingua, Alessandro; Armocida, Daniele; Caporlingua, Federico; Lapadula, Gennaro; Elefante, Grazia Maria; Antonelli, Manila; Salvati, Maurizio

doi:10.1155/2016/6785459

Cited by 5 publications

(8 citation statements)

References 9 publications

(11 reference statements)

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“…According to our results, pPNETs can metastasize to distant sites like the brain, bone, lungs, lymph nodes, and liver, 14 which is consistent with reported cases. 15,16 As such, preoperative whole-body CT and/or MRI is highly recommended.…”

Section: Discussionmentioning

confidence: 99%

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

Wang

Zhang

Song³

et al. 2021

J Neurol Surg A Cent Eur Neurosurg

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Background and Study Aims Primary intraspinal primitive neuroectodermal tumors (PNETs) account for ∼0.4% of all intraspinal tumors, but information about these tumors in the medical literature is limited to single case reports. We report four cases of primary intraspinal PNETs and present a systematic literature review of the reported cases. Materials and Methods We retrospectively reviewed and analyzed the clinical data of 4 patients with primary intraspinal PNETs who underwent neurosurgical treatment at our clinic between January 2013 and January 2020, and of 32 cases reported in the literature. Results The female-to-male ratio was 2.6:1. The mean patient age was 21.42 ± 15.76 years (range: 1–60 years), and patients <36 years of age accounted for 83.30% of the study cohort. Progressive limb weakness and numbness were the chief symptoms (accounting for ∼55.6%). The mean complaint duration was 0.89 ± 0.66 months for males and 2.72 ± 3.82 months for females (p = 0.028). Epidural (41.7%) was the most common site, and thoracic (47.3%) was the most frequent location. Most PNETs were peripheral, and magnetic resonance imaging (MRI) appearance was isointense or mildly hypointense on T1-weighted images and hyperintense on T2-weighted images. Homogeneous contrast enhancement was observed. The 1-year survival rate of patients who underwent chemoradiation after total or subtotal lesion resection was better compared with patients who did not undergo chemotherapy, radiotherapy, or total or subtotal resection. The modality of treatment was associated with survival time (p = 0.007). Conclusion Primary intraspinal PNETs mainly occur in young people with a female preponderance. In patients with a rapid loss of lower limb muscle strength and large intraspinal lesions on MRI, PNETs should be considered. Surgical resection and adjuvant radio chemotherapy are key prognostic factors.

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Section: Discussionmentioning

confidence: 99%

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

Wang

Zhang

Song³

et al. 2021

J Neurol Surg A Cent Eur Neurosurg

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“…50 Embryonal tumors without this genetic alteration are therefore classified as ETMR, C19MC-non-altered. 51,52 Identification of other putative diagnostic molecular assays in these rare tumors is still an object of research. At present, the category CNS embryonal tumor, NOS comprises tumors previously classified as CNS PNET for which molecular parameters have not been tested or tumors have been tested but without any evidence of diagnostic molecular alterations and classical histologic aspects to suggest either medulloepithelioma or neuroblastoma/ganglioneuroblastoma.…”

Section: Discussionmentioning

confidence: 99%

Hemorrhagic Suprasellar Central Nervous System Embryonal Tumor in an Adult: Uncommon Features of an Extremely Rare Neoplasm

Piloni

Gagliardi

Bailo

et al. 2021

J Neurol Surg A Cent Eur Neurosurg

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Background Occurrences of suprasellar central nervous system (CNS) embryonal tumors in adults are extremely rare. Hemorrhagic onset is further uncommon, with only anecdotic cases reported in the literature. The authors describe the case of a 57-year-old man affected by a suprasellar CNS embryonal tumor, with hemorrhagic onset and a unique diffusion pattern along the optic pathways. Material and Methods A 57-year-old man presenting with acute visual acuity worsening and left homonymous hemianopia was referred to our hospital. Neuroradiologic studies demonstrated an infiltrating, high-grade lesion involving the optic chiasm and right retrochiasmatic pathways with a hemorrhagic area in the ipsilateral pulvinar. Results The patient underwent microsurgical biopsy. Pathologic assessment confirmed the diagnosis of CNS embryonal tumor, not otherwise specified (NOS) according to the 2016 World Health Organization (WHO) classification of CNS tumors. The patient was referred to a multimodal adjuvant treatment; he eventually died 4 months after surgery. Competent literature has been systematically reviewed in the light of the relevant changes made in the last version of the WHO classification. Conclusion Embryonal tumors should be considered in the differential diagnosis for sellar and suprasellar space-occupying lesions, despite the rarity of the disease and the uncommon features at time of presentation. As per our knowledge, this is the first case ever described of hemorrhagic suprasellar embryonal tumor with a diffusion pattern along white matter fibers. Histogenesis, biomolecular and neuroradiologic features, and classification of embryonal tumors are an open field of research, with considerable implications for the definition of better diagnostic pitfalls and therapeutic regimens.

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“…Peripheral neuroectodermic tumors are derived outside the central and autonomic nervous system. [ 4 5 6 ]…”

Section: Discussionmentioning

confidence: 99%

“…In adults, supratentorial embryonic tumors may present with moderate to severe surrounding edema. [ 3 4 6 9 13 15 ] Angiography can show focal areas of prominent vascularization within the tumor and correlate with the areas of nodular contrast. [ 9 ]…”

Section: Discussionmentioning

confidence: 99%

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Supratentorial embryonal tumor in adult patient: Case report and literature review

et al. 2020

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Embryonal tumors are the new nomenclature of the primitive neuroectodermal tumors or PNET. Their supratentorial location and their manifestation in adult population are not very frequent. Embryonal tumors are conformed from undifferentiated neuroepithelial cells that have the ability to show differentiation to several cell lines. Next is presented a case from an adult male patient with a clinical headache pictures and convulsions. With imaging study that shows a meningeal enhancement with frontal lobe infiltration with histopathological study of embryonic tumor with areas of glial differentiation.

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Disseminated Cerebrospinal Embryonal Tumor in the Adult

Cited by 5 publications

References 9 publications

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

Hemorrhagic Suprasellar Central Nervous System Embryonal Tumor in an Adult: Uncommon Features of an Extremely Rare Neoplasm

Supratentorial embryonal tumor in adult patient: Case report and literature review

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