A Rare Manifestation of a Presumed Non-Osteophilic Brain Neoplasm: Extensive Axial Skeletal Metastases From Glioblastoma With Primitive Neuronal Components

BackgroundGlioblastoma multiforme (GBM) is the most common malignant tumor of the central nervous system. GBM with primitive neuronal component (GBM-PNC) is an aggressive variant identified in 0.5% of GBMs. Extracranial metastasis from GBM-PNC is a rare and challenging situation.MethodsA special case of early-onset GBM with systemic bone metastasis was enrolled. Clinical data, including patient characteristics, disease course, and serial radiological images were retrieved and analyzed. Tumor tissues were obtained by surgical resections and were made into formalin-fixed paraffin-embedded sections. Histopathological examinations and genetic testing were performed for both the primary and metastatic tumor specimens.ResultsA 20-year-old man suffered from GBM with acute intratumoral hemorrhage of the left temporal lobe. He was treated by gross total resection and chemoradiotherapy following the Stupp protocol. Seven months later, he returned with a five-week history of progressive neck pain and unsteady gait. The radiographic examinations identified vertebral collapse at C4 and C6. Similar osteolytic lesions were also observed at the thoracolumbar spine, pelvic, and left femur. Anterior spondylectomy of C4 and C6 was performed. The resected vertebral bodies were infiltrated with greyish, soft, and ill-defined tumor tissue. One month later, he developed mechanical low-back pain and paraplegia caused by thoracolumbar metastases. Another spine surgery was performed, including T10 total en-bloc spondylectomy, T7-9, L2-3, and L5-S1 laminectomy. After the operation, the patient’s neurological function and spinal stability remained stable. However, he finally succumbed to the rapidly increased tumor burden and died 15 months from onset because of cachexia and multiple organ failure. In addition to typical GBM morphology, the histological examinations identified monomorphic small-round cells with positive immunohistochemical staining of synaptophysin and CD99, indicating the coexistence of PNC. The next-generation sequencing detected pathogenic mutations in TP53 and DNMT3A. Based on above findings, a confirmed diagnosis of systemic metastases from GBM-PNC (IDH-wild type, WHO grade IV) was made.ConclusionsThe present case highlights the occurrence and severity of extensive axial skeletal metastases from GBM-PNC. This rare variant of GBM requires aggressive multimodal treatment including surgery and chemoradiotherapy targeting PNC. The pathological screening of PNC is recommended in patients with early-onset GBM and intratumoral hemorrhage. Surgery for spinal metastasis is appropriate in patients with chemoradioresistance and relatively good general status, with the objectives of restoring spinal stability and relieving spinal cord compression.

The Value of MRI Findings Combined With Texture Analysis in the Differential Diagnosis of Primary Ovarian Granulosa Cell Tumors and Ovarian Thecoma–Fibrothecoma

ObjectiveThis study aims to explore the value of magnetic resonance imaging (MRI) and texture analysis (TA) in the differential diagnosis of ovarian granulosa cell tumors (OGCTs) and thecoma-fibrothecoma (OTCA–FTCA).MethodsThe preoperative MRI data of 32 patients with OTCA–FTCA and 14 patients with OGCTs, confirmed by pathological examination between June 2013 and August 2020, were retrospectively analyzed. The texture data of three-dimensional MRI scans based on T2-weighted imaging and clinical and conventional MRI features were analyzed and compared between tumor types. The Mann–Whitney U-test, χ2 test/Fisher exact test, and multivariate logistic regression analysis were used to identify differences between the OTCA–FTCA and OGCTs groups. A regression model was established by using binary logistic regression analysis, and receiver operating characteristic curve analysis was carried out to evaluate diagnostic efficiency.ResultsA multivariate analysis of the imaging-based features combined with TA revealed that intratumoral hemorrhage (OR = 0.037), log-sigma-20mm-3D_glszm_SmallAreaEmphasis (OR = 4.40), and log-sigma-2-0mm-3D_glszm_SmallAreaHighGrayLevelEmphasis (OR = 1.034) were independent features for discriminating between OGCTs and OTCA–FTCA (P < 0.05). An imaging-based diagnosis model, TA-based model, and combination model were established. The areas under the curve of the three models in predicting OGCTs and OTCA–FTCA were 0.935, 0.944, and 0.969, respectively; the sensitivities were 93.75, 93.75, and 96.87%, respectively; and the specificities were 85.71, 92.86, and 92.86%, respectively. The DeLong test indicated that the combination model had the highest predictive efficiency (P < 0.05), with no significant difference among the three models in differentiating between OGCTs and OTCA–FTCA (P > 0.05).ConclusionsCompared with OTCA–FTCA, intratumoral hemorrhage may be characteristic MR imaging features with OGCTs. Texture features can reflect the microheterogeneity of OGCTs and OTCA–FTCA. MRI signs and texture features can help differentiate between OGCTs and OTCA–FTCA and provide a more comprehensive and accurate basis for clinical treatment.

Intratumoral hemorrhage in jugular foramen schwannoma after stereotactic radiosurgery: A case report

Background: Clinically significant intratumoral hemorrhage is a rare complication of stereotactic radiosurgery (SRS) for benign tumors. Case Description: Here, we present the case of a 64-year-old man who underwent SRS for a relatively large dumbbell-shaped left jugular foramen schwannoma (JFS) and thereafter developed intratumoral hemorrhage. On post-SRS day 3, he developed lower cranial nerve palsies with radiographically evident tumor expansion. His neurological conditions had gradually improved thereafter; however, he suddenly developed headache, dizziness, and mild hearing deterioration at 7 months due to intratumoral hemorrhage. We managed the patient conservatively, and eventually, his symptoms improved except for slight ataxia and hearing deterioration. Follow-up images at 4 years from SRS demonstrated significant tumor shrinkage. This is the first report describing intratumoral hemorrhage after SRS for JFS. Conclusion: Transient expansion of the tumor and subsequent venous stasis around the tumor may have played a role in the hemorrhage. Intratumoral hemorrhage should be considered as a rare, but potential complication of SRS for JFSs.

Cauda equina schwannoma presenting with subarachnoid and subdural hemorrhage: Its underlying mechanism

Background: A patient presented with a spinal subarachnoid hemorrhage (SAH) and subdural hematoma (SDH) attributed to a spinal schwannoma at the T12-L1 level. Case Description: A 67-year-old male acutely presented with severe back pain and L1 paraparesis/sensory loss, with urinary incontinence. CT/MR studies showed a spinal SAH and SDH within a likely T12-L1 schwannoma. At surgery, the hemorrhage within the tumor was continuous through the lower pole of the tumor into the subarachnoid and subdural spaces; tumor was dissected away from the surrounding tissues and totally removed. The postoperative course was uneventful, and the preoperative neurological deficits gradually resolved. Histopathologically, the lesion was a schwannoma with intratumoral hemorrhage. Conclusion: This case demonstrates the rare acute presentation of a T12-L1 schwannoma with an accompanying intratumoral hemorrhage resulting in both a SDH/SAH.

Cervical schwannoma with acute worsening and intratumoral hemorrhage

Background: Primary spinal tumors are rare and include schwannomas. In the cervical region, these lesions can cause pain, radiculopathy, and/or myelopathy. Case Description: A 53-year-old male presented with 9 months of chronic neck pain and left upper extremity radiculopathy/myelopathy. The MRI revealed an intradural extramedullary C6-C7 mass left-sided mass with foraminal extension. Following a C5-C7 laminectomy with C5-T2 instrumented fusion, the diagnosis of schwannoma with evidence of recent hemorrhage was confirmed by biopsy. Three weeks postoperatively, the patient was pain free, no longer taking opioids, and neurologically intact. Although the MR 6 months later showed no tumor, the MRI 15 months later documented a recurrent enhancing C6-C7 lesion. The patient elected to be treated with external beam radiotherapy and remained asymptomatic. Conclusion: A 53-year-old underwent resection of a cervical C6-C7 schwannoma with intratumoral hemorrhage. Fifteen months following C5-C7 laminectomy with C5-T2 fusion, the tumor recurred and required external beam radiation therapy.

ACOUSTIC SCHWANNOMA WITH INTRATUMORAL HEMORRHAGE –REPORT OF AN UNUSUAL COMPLICATION

Acoustic Schwannoma is a benign neoplasm of the 8th cranial nerve pair, and accounts for 8-10% of all intracranialtumors. Intratumoral haemorrhage is unusual in these tumors and can lead to hydrocephalus or aggravate a preexisting condition. This paper reports a case of acoustic Schwannoma in a child with complication with intratumoral hemorrhage that lead patient to death. Female patient, 9 years old, presented anacusis on the right, sudden, progressing with vomiting, intense headache and vertigo. Magnetic resonance imaging of the skull showed expansive lesion in the cistern of the right cerebellar angle, with content inside the internalauditory canal, with compression of the adjacent brain stem. The diagnostic hypothesis was acoustic Schwannoma. It progressed with worsening of headache, initially controlled with medication and later loss of consciousness. Computed tomography of the skull revealed an expansive lesion in the cistern of the right cerebellar angle with signs of bleeding with extension to the ventricular system. External ventricular shunt was performed, but the patient evolved with several complications, septic shock and death. Intratumoral hemorrhage in the acoustic Schwannoma is a rare event, but its occurrence worsens the clinical picture and may lead to death of the patients due to intracranial hypertension.

Intratumoral Hemorrhage After Endoscopic Third Ventriculostomy for Obstructive Hydrocephalus Caused by Brain Tumors

IntroductionEndoscopic third ventriculostomy (ETV) for obstructive hydrocephalus and endoscopic biopsy (EB) for intra- and paraventricular tumors are recognized as standard therapies because of their minimal invasiveness. Although EB-associated hemorrhagic risk has been well documented, reports on the ETV-associated hemorrhagic risk are only few. Therefore, we conducted this retrospective study to review the incidence of hemorrhage due to EB and/or ETV in a single institution.Material and MethodsWe retrospectively reviewed data, including patient characteristics, procedure, pathological findings, and complications, including hemorrhage, of 100 patients with intra- and paraventricular tumors who underwent EB and/or ETV at our institution from 2000 to 2020.ResultsEB and ETV combined surgery (combined group), EB-alone surgery (EB-alone group), and ETV-alone surgery (ETV-alone group) were performed in 44 (44%), 24 (24%), and 32 (32%) patients, respectively, and all procedures were successful. The rates of definitive and suggestive diagnoses in EB were 76.5% and 23.5%, respectively. Adverse events were observed in six patients. An acute obstruction of the ETV stoma and a transient double vision were observed in the combined group. Two transient aqueductal stenosis/obstructions were recognized in the EB-alone group. Hemorrhage was observed in two patients in the ETV-alone group; these patients developed intratumoral hemorrhage despite ETV-alone surgery. Subsequently, these two patients underwent tumor removal, and the histopathological diagnosis was atypical teratoid/rhabdoid tumor (AT/RT) in both.ConclusionsFor obstructive hydrocephalus with AT/RTs, physicians must be aware of the risk of postoperative intratumoral hemorrhage after performing ETV.Trial registration numberE-2385 (2021/3/11)