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Open access

Cerebellar high-grade glioma with H3 K27M mutation: illustrative case

Mikiko Takahashi, Yusuke Tomita, Shoko Merrit Yamada, Kazuto Yamazaki, Hideaki Yokoo, Tatsuya Aso, and Masashi Kawamoto

BACKGROUND

Diffuse midline glioma H3 K27M-mutant is a distinct subtype of glial tumors newly introduced in the revised fourth edition of the World Health Organization Classification of Tumours of the Central Nervous System. They are aggressive pediatric tumors with a poor prognosis but have also been reported in adults.

OBSERVATIONS

The authors present the case of a man in his 60s who presented with rotatory vertigo, taste disorder, and right facial paralysis. Magnetic resonance imaging showed a tumor expanding from the cerebellum to the pons, which was histologically identified as glioblastoma, grade IV, IDH wild type. After tumor resection, the patient received chemoradiotherapy but showed only a partial response. His condition gradually worsened, and he died of progressive disease 12 months postoperation, after which an autopsy was performed. Tumor cells with a high nuclear-to-cytoplasm ratio were immunohistochemically analyzed and found to test positive for H3 K27M and negative for H3 K27me3. Furthermore, mutational analysis revealed HIST1H3B K27M mutation, and the tumor was finally identified as a high-grade glioma H3 K27M-mutant. The tumor invaded widely along the cerebral ventricle and disseminated to the spinal cord.

LESSONS

When a glioblastoma shows localization or dissemination patterns different from those of typical glioblastoma, an H3 K27M-mutant glioma should be suspected.

Open access

Intraoperative contrast-enhanced ultrasound for intramedullary spinal neoplasms: patient series

Ariana Barkley, Lynn B. McGrath Jr., and Christoph P. Hofstetter

BACKGROUND

Primary intramedullary spinal tumors cause significant morbidity and death. Intraoperative ultrasound as an adjunct for localization and monitoring the extent of resection has not been systematically evaluated in these patients; the effectiveness of intraoperative contrast-enhanced ultrasound (CEUS) remains almost completely unexplored.

OBSERVATIONS

A retrospective case series of patients at a single institution who had consented to the off-label use of intraoperative CEUS was identified. Seven patients with a mean age of 52.8 ± 15.8 years underwent resection of intramedullary tumors assisted by CEUS performed by a single attending neurosurgeon. Histopathological evaluation revealed 3 cases of hemangioblastoma, 1 case of pilocytic astrocytoma, 2 cases of ependymoma, and 1 case of subependymoma. Contrast enhancement correlated with gadolinium enhancement on preoperative magnetic resonance imaging. Intraoperative CEUS facilitated precise lesion localization and myelotomy planning. Dynamic CEUS studies were useful in demonstrating the blood supply to lesions with a dominant vascular pedicle. Regardless of contrast uptake, the differential enhancement between spinal cord tissue and neoplasm assisted in determining interface boundaries.

LESSONS

Intraoperative CEUS constitutes a useful adjunct for the intraoperative delineation of contrast-enhancing intramedullary tumors and in vivo confirmation of gross-total resection. Systematic investigation is needed to establish the role of CEUS for resection of intramedullary spinal tumors of various pathologies.

Open access

Intraoperative MRI for the microsurgical resection of meningiomas close to eloquent areas or dural sinuses: patient series

Constantin Tuleasca, Rabih Aboukais, Quentin Vannod-Michel, Xavier Leclerc, Nicolas Reyns, and Jean-Paul Lejeune

BACKGROUND

Meningiomas are the most commonly encountered nonglial primary intracranial tumors. The authors report on the usefulness of intraoperative magnetic resonance imaging (iMRI) during microsurgical resection of meningiomas located close to eloquent areas or dural sinuses and on the feasibility of further radiation therapy.

OBSERVATIONS

Six patients benefited from this approach. The mean follow-up period after surgery was 3.3 (median 3.2, range 2.1–4.6) years. Five patients had no postoperative neurological deficit, of whom two with preoperative motor deficit completely recovered. One patient with preoperative left inferior limb deficit partially recovered. The mean interval between surgery and radiation therapy was 15.8 (median 16.9, range 1.4–40.5) months. Additional radiation therapy was required in five cases after surgery. The mean preoperative tumor volume was 38.7 (median 27.5, range 8.6–75.6) mL. The mean postoperative tumor volume was 1.2 (median 0.8, range 0–4.3) mL. At the last follow-up, all tumors were controlled.

LESSONS

The use of iMRI was particularly helpful to (1) decide on additional tumor resection according to iMRI findings during the surgical procedure; (2) evaluate the residual tumor volume at the end of the surgery; and (3) judge the need for further radiation and, in particular, the feasibility of single-fraction radiosurgery.

Open access

Treatment of postoperative vasospasm with intraarterial verapamil after removal of intracranial tumor: patient series

Ketevan Mikeladze, Anton Konovalov, Andrey Bykanov, Evgeniy Vinogradov, and Sergey Yakovlev

BACKGROUND

The authors report on four clinical cases with intraarterial verapamil administration to resolve vasospasm in patients who underwent surgery for intracranial tumors. Iatrogenic subarachnoid hemorrhage after tumor resection and subsequent vasospasm (an increase in the systolic linear velocity of blood flow through the M1 segment of the middle cerebral artery of more than 250 cm/sec; Lindegaard index: 4.1) were observed in four patients during the early postoperative period after the removal of intracerebral tumors. Each vasospasm case was confirmed by angiography data, was clinically significant, and manifested as the development of a neurological deficit.

OBSERVATIONS

Resolution of vasospasm with the intraarterial administration of verapamil was achieved in all four cases as confirmed by angiographic data in all four cases and complete regression of neurological symptoms in two cases. In all four presented cases, vasospasm was resolved; unfortunately, the resolution did not always lead to significant clinical improvement. However, lethal outcomes were avoided in two cases, and almost full recoveries were achieved in the other two.

LESSONS

The authors believe that the removal of intracranial tumors can cause expected and potential complications, such as cerebral vasospasm, which must be diagnosed and treated in a timely manner.

Open access

Mixed germ cell tumor infiltrating the pineal gland without elevated tumor markers: illustrative case

Koji Shiomi, Yoshiki Arakawa, Sachiko Minamiguchi, Haruki Yamashita, Yukinori Terada, Masahiro Tanji, Yohei Mineharu, Katsutsugu Umeda, Megumi Uto, Junko Takita, Hironori Haga, Takashi Mizowaki, and Susumu Miyamoto

BACKGROUND

Tumors in the pineal region consist of various histological types, and correct diagnosis from biopsy specimens is sometimes difficult. The authors report the case of a patient with a mixed germ cell tumor infiltrating into the pineal gland despite showing no elevation of tumor markers.

OBSERVATIONS

An 18-year-old man complained of headache and nausea and showed disturbance of consciousness. Magnetic resonance imaging showed hydrocephalus associated with a cystic pineal tumor. The patient underwent tumor biopsy followed by endoscopic third ventriculostomy for hydrocephalus in a local hospital. A pineocytoma was diagnosed, and the patient was referred to the authors’ hospital for treatment. Concentrations of placental alkaline phosphatase, alpha-fetoprotein (AFP), and beta-human chorionic gonadotropin in cerebrospinal fluid were not elevated. However, the authors’ review of the tumor specimen revealed some immature cells infiltrating the pineal gland. These cells were positive for AFP, Sal-like protein 4, and octamer-binding transcription factor 3/4; and the diagnosis was changed to mixed germ cell tumor. Chemoradiotherapy was initiated, followed by surgical removal of the residual tumor.

LESSONS

Careful examination of all tumor specimens and immunohistochemical analyses are important for accurate diagnosis of pineal tumors.

Open access

Asymptomatic superficial siderosis after posterior fossa tumor resection: illustrative case

Anshit Goyal, Cody L. Nesvick, Joshua A. Spear, and David J. Daniels

BACKGROUND

Superficial siderosis of the central nervous system is a rare syndrome notable for the presence of hemosiderin deposition due to chronic, repetitive hemorrhages into the subarachnoid space.

OBSERVATIONS

The authors presented a case of superficial siderosis in a 14-year-old girl. It arose as a late postoperative complication after resection of a medulloblastoma. Despite the patient being asymptomatic, surveillance imaging demonstrated diffuse hemosiderin deposition within the cerebellar folia and cisternal segments of cranial nerves VII and VIII on gradient echo (GRE) sequences. Formal audiometric testing demonstrated bilateral loss of high-frequency tone recognition consistent with early sensorineural hearing loss. A pseudomeningocele due to multiple dural defects was identified as the likely cause, and definitive surgical repair was performed. Intraoperatively, the presence of blood-tinged cerebrospinal fluid confirmed a diagnosis of superficial siderosis.

LESSONS

This case highlighted the potential need to routinely include GRE or susceptibility-weighted sequences in postoperative surveillance imaging after resection of pediatric posterior fossa tumors.

Open access

Spontaneous closure of an incidental high-flow paravertebral arteriovenous fistula caused by vertebral giant cell tumor curettage: illustrative case

Yen-Heng Lin, Yu-Cheng Huang, and Fon-Yih Tsuang

BACKGROUND

Paravertebral arteriovenous fistula (AVF) after spinal surgery is rarely reported in the literature. Its natural course is largely unknown.

OBSERVATIONS

The authors report a 31-year-old woman with a high-flow AVF after T12 vertebral giant cell tumor curettage. Eight months after the initial surgery, revision en bloc surgery was planned. Preoperative computed tomography angiography was performed for vascularity assessment, which incidentally revealed a large paravertebral early-enhanced venous sac. High-flow AVF was confirmed through subsequent spinal angiography. Endovascular embolization was scheduled before the surgery to avoid massive blood loss. However, the AVF closed spontaneously 1 month after the spinal angiography. The plan was changed to preoperative embolization; subsequently, three-level en bloc spondylectomy was performed smoothly.

LESSONS

Iatrogenic AVF is possible, prompting investigation by vascular imaging when suspected. Embolization is a preferred treatment method when feasible. However, for iatrogenic etiology, the prothrombotic property of the contrast medium may induce the resolution. Multidisciplinary discussion can be very helpful before aggressive spinal surgery.

Open access

Adamantinomatous craniopharyngioma associated with a compromised blood–brain barrier: patient series

Eric W. Prince, Lindsey M. Hoffman, Trinka Vijmasi, Kathleen Dorris, Jennifer A. McWilliams, Kimberly R. Jordan, David M. Mirsky, and Todd C. Hankinson

BACKGROUND

Adamantinomatous craniopharyngioma (ACP) is a highly morbid adult and pediatric brain tumor derived from epithelial remnants of the craniopharyngeal canal (Rathke’s pouch), which gives rise to the anterior pituitary gland. Standard therapy includes maximal safe resection with or without radiation therapy. Systemic antitumor therapy remains elusive. Immune-related paracrine signaling involving the interleukin-6 receptor (IL-6R) may contribute to ACP pathogenesis. Tocilizumab, a recombinant humanized monoclonal antibody against IL-6R, is approved by the US Food and Drug Administration but does not cross an intact blood–brain barrier.

OBSERVATIONS

In a phase 0 trial design, a single dose of tocilizumab was delivered intravenously before clinically indicated surgical intervention in 3 children with ACP. The presence of tocilizumab was assayed in plasma, tumor tissue, tumor cyst fluid, and cerebrospinal fluid (n = 1) using a novel enzyme-linked immunosorbent assay. Tocilizumab reached ACP tumor tissue and/or cyst fluid after one systemic dose in every patient.

LESSONS

This finding helps explain extant data that indicate tocilizumab may contribute to ACP therapy. It further indicates that ACP does not reside behind an intact blood–brain barrier, dramatically broadening the range of potential antitumor therapies against this tumor. This has substantial implications for the design of future clinical trials for novel therapies against ACP in both children and adults.

Open access

Long-term survival in patients with long-segment complex meningiomas occluding the dural venous sinuses: illustrative cases

Zhishuo Wei, Arka N. Mallela, Andrew Faramand, Ajay Niranjan, and L. Dade Lunsford

BACKGROUND

Invasive sagittal sinus meningiomas are difficult tumors to cure by resection alone. Stereotactic radiosurgery (SRS) can be used as an adjuvant management strategy to improve tumor control after incomplete resection.

OBSERVATIONS

The authors reported the long-term retrospective follow-up of two patients whose recurrent parasagittal meningiomas eventually occluded their superior sagittal sinus. Both patients underwent staged radiosurgery and fractionated radiation therapy to achieve tumor control that extended to 20 years after their initial surgery. After initial subtotal resection of meningiomas that had invaded major cerebral venous sinuses, adjuvant radiosurgery was performed to enhance local tumor control. Over time, adjacent tumor progression required repeat SRS and fractionated radiation therapy to boost long-term tumor response. Staged multimodality intervention led to extended survival in these patients with otherwise unresectable meningiomas.

LESSONS

Multimodality management with radiosurgery and fractionated radiation therapy was associated with long-term survival of two patients with otherwise surgically incurable and invasive meningiomas of the dural venous sinuses.

Open access

The underestimated incidence of an orbital angioleiomyoma is possibly associated with an orbital cavernous hemangioma: illustrative case

Seonah Choi, JaeSang Ko, Se Hoon Kim, and Eui Hyun Kim

BACKGROUND

Orbital angioleiomyoma is generally considered a rare tumor; approximately 40 cases have been reported. However, after their experience with 6 consecutive cases in their single institution during 3 years, the authors speculate that the incidence of orbital angioleiomyomas is possibly underestimated.

OBSERVATIONS

A 34-year-old female presented with progressive exophthalmos of 2 years’ duration. Orbital computed tomography and magnetic resonance imaging revealed a well-circumscribed orbital tumor with partial and heterogeneous gadolinium enhancement. Technetium-99m red blood cell single-photon emission computed tomography showed positive perfusion in the late blood-pool phase, which was exactly consistent with the finding of a cavernous hemangioma. Under the impression of a cavernous hemangioma, the authors accessed the mass with an endoscopic endonasal approach and completely removed it without neurological deficit. Pathological examination revealed that the final diagnosis was an angioleiomyoma with positive immunostaining results for smooth muscle actin (SMA).

LESSONS

The incidence of orbital angioleiomyomas may not be very low, as these lesions have possibly been misdiagnosed as orbital cavernous hemangiomas because of their histological similarity. Preoperative presumption and differentiation from cavernous hemangiomas are very challenging because of the rarity of orbital angioleiomyoma and similar radiological findings. SMA immunostaining may be critical to differentiate orbital angioleiomyomas from cavernous hemangiomas.