Intraventricular Undifferentiated Pleomorphic Sarcoma: A Case Report

Sieg, Emily; Stepanyan, H.; Payne, Russell; Frauenhoffer, Elizabeth E.; Specht, Charles S.; Langan, Sara; Rizk, Elias

doi:10.7759/cureus.876

Cited by 2 publications

(2 citation statements)

References 9 publications

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“…Diagnosing intraventricular tumors using imaging alone is difficult, as the differential diagnosis includes ependymoma, meningioma, astrocytoma, choroid plexus carcinoma, choroid plexus papilloma, germ cell tumor, metastasis, neurocytoma, and subependymoma [32-36]. Of 18 pre-surgical diagnoses proposed for the intraventricular ganglioglioma cases, 4 (22%) were astrocytomas, 4 (22%) were neurocytomas, 2 (11%) were plexus papillomas, 2 (11%) were subependymomas, and 1 (6%) of each were diagnosed as craniopharyngioma, ependymoma, germ cell tumor, ganglioglioma, malignant glioma, and meningioma.…”

Section: Discussionmentioning

confidence: 99%

Ganglioglioma Arising from the Septum Pellucidum: Case Report and Review of the Literature

et al. 2019

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Background: Gangliogliomas are low-grade neoplasms that typically affect patients under the age of 30 and present with epilepsy and symptoms of mass effect. Here, we report a case of an intraventricular ganglioglioma involving the septum pellucidum in a pediatric patient with history of optic glioma. Only one other pediatric intraventricular ganglioglioma arising from the septum pellucidum has been reported previously. Case Report: The patient initially presented at 9 months of age with a pilocytic astrocytoma centered on the optic chiasm, treated with chemotherapy and radiation at 3 years of age. Routine follow-up imaging at 13 years of age revealed the development of a mass in the septum pellucidum, which was subtotally resected endoscopically because of its proximity to the fornices. Pathology confirmed a ganglioglioma positive for the BRAF V600E mutation. The tumor residual progressed and was treated with stereotactic radiosurgery. The patient was asymptomatic at her 6-month follow-up visit and the size of the nodule remained stable. Literature Review: Our review of the 25 previously reported intraventricular gangliogliomas found that their pre-surgical diagnoses were often incorrect, reflecting the difficulty of making the diagnosis with signs, symptoms, and imaging alone. Patients can be reassured that the prognosis is generally favorable following uncomplicated neurosurgical resection.

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Section: Discussionmentioning

confidence: 99%

Ganglioglioma Arising from the Septum Pellucidum: Case Report and Review of the Literature

et al. 2019

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“…Undifferentiated pleomorphic sarcoma (UPS) is among the most frequent STS in adults (10-20%), 1,2 osteosarcoma (OS) is the most common primary bone tumor occurring predominantly in adolescents and young adults. 3 …”

Section: Introductionmentioning

confidence: 99%

Cytokine Induced Killer cells are effective against sarcoma cancer stem cells spared by chemotherapy and target therapy.

et al. 2018

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Metastatic bone and soft tissue sarcomas often relapse after chemotherapy (CHT) and molecular targeted therapy (mTT), maintaining a severe prognosis. A subset of sarcoma cancer stem cells (sCSC) is hypothesized to resist conventional drugs and sustain disease relapses. We investigated the immunotherapy activity of cytokine induced killer cells (CIK) against autologous sCSC that survived CHT and mTT. The experimental platform included two aggressive bone and soft tissue sarcoma models: osteosarcoma (OS) and undifferentiated-pleomorphic sarcoma (UPS).To visualize putative sCSC we engineered patient-derived sarcoma cultures (2 OS and 3 UPS) with a lentiviral sCSC-detector wherein the promoter of stem-gene Oct4 controls the expression of eGFP.We visualized a fraction of sCSC (mean 24.2 ± 5.2%) and confirmed their tumorigenicity in vivo. sCSC resulted relatively resistant to both CHT and mTT in vitro. Therapeutic doses of doxorubicin significantly enriched viable eGFP+sCSC in both OS (2.6 fold, n = 16) and UPS (2.3 fold, n = 29) compared to untreated controls. Treatment with sorafenib (for OS) and pazopanib (for UPS) also determined enrichment (1.3 fold) of viable eGFP+sCSC, even if less intense than what observed after CHT.Sarcoma cells surviving CHT and mTT were efficiently killed in vitro by autologous CIK even at minimal effector/target ratios (40:1 = 82%, 1:4 = 29%, n = 13). CIK immunotherapy did not spare sCSC that were killed as efficiently as whole sarcoma cell population. The relative chemo-resistance of sCSC and sensitivity to CIK immunotherapy was confirmed in vivo. Our findings support CIK as an innovative, clinically explorable, approach to eradicate chemo-resistant sCSC implicated in tumor relapse.

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Intraventricular Undifferentiated Pleomorphic Sarcoma: A Case Report

Cited by 2 publications

References 9 publications

Ganglioglioma Arising from the Septum Pellucidum: Case Report and Review of the Literature

Ganglioglioma Arising from the Septum Pellucidum: Case Report and Review of the Literature

Cytokine Induced Killer cells are effective against sarcoma cancer stem cells spared by chemotherapy and target therapy.

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