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Open access

Primary germinoma of the medulla oblongata: illustrative case

Pablo Albiña, Aracelly Solis, Jose Lorenzoni, Pablo Henny, and María Manriquez

BACKGROUND

Primary central nervous system germinomas of the medulla oblongata are extremely rare and usually have been found in young female Asian patients. The authors present an illustrative case of a patient who presented with severe medullary and posterior cord syndrome, the first South American case published to date, to the authors’ knowledge.

OBSERVATIONS

Initially, the radiological differential diagnosis did not include this entity. The lesion was located at the obex and exhibited a well-delineated contrast enhancement without hydrocephalus. An emergency decompressive partial resection following functional limits was performed. After histological confirmation, radiotherapy was indicated, with complete remission achieved at a 6-month follow-up. The patient, however, continued to have a severe proprioceptive disorder. The literature review identified 21 other such patients. The mean age for this location was 23 years, with a strong female and Asian origin predilection. All tumors exhibited contrast enhancement, and only one presented with hydrocephalus.

LESSONS

In the absence of elevated tumor markers, radiological clues such as a well-delineated, contrast-enhanced lesion arising from the obex, without hydrocephalus, associated with demographic features such as young age, female sex, and Asian heritage, should evoke a high level of suspicion for this diagnosis. Gross total resection must not be attempted, because this tumor is potentially curable with high-dose radiotherapy.

Open access

Primary isolated skull base eosinophilic granuloma confined to the anterior clinoid process: illustrative case

Ahmad Pour-Rashidi, Payam Asem, Kazem Abbasioun, and Abbas Amirjamshidi

BACKGROUND

Solitary eosinophilic granuloma (EG) occurs anecdotally in the skull base region, and it has been described in only three previous publications. The authors report the first case of EG of the anterior clinoid process (ACP), which was confined to the ACP and presented with decreased vision.

OBSERVATIONS

A 38-year-old woman presented with decreased vision of the left eye of 5 months’ duration. Her visual acuity was 3/10, other neurological examinations were intact, and there were no other osseous or soft tissue lesions. The lesion was excised using a left-sided craniotomy and transdural clinoidectomy, decompressing the optic nerve both intra- and extradurally. The lesion was characteristic for EG, and no recurrence was detected after 2 years.

LESSONS

EG can be confined to the ACP and impair vision. Imaging studies are sensitive but not specific, and surgical decompression is both diagnostic and treatment oriented. Close observation and even adjuvant therapy may be indicated in similar cases.

Open access

Metastatic primary brain rhabdomyosarcoma in a pediatric patient: illustrative case

Michel Gustavo Mondragón-Soto, Luis Del Valle, José Alfredo González-Soto, and Roberto Alfonso De Leo-Vargas

BACKGROUND

Primary intracranial rhabdomyosarcoma is an extraordinarily rare malignant tumor, with even fewer presenting with distant metastasis. To date, only five cases, including the one presented here, have been reported to present metastatic activity.

OBSERVATIONS

A 12-year-old boy presented with a few days of headache, nausea, vomiting, but no neurological deficit. Brain computed tomography and magnetic resonance imaging demonstrated hydrocephalus and a cystic lesion with left parieto-occipital extension. After resection, pathology reported primary rhabdomyosarcoma, with positive desmin and myogenin on immunohistochemistry. The patient presented with pulmonary metastasis. The patient had an overall survival of 21 months after diagnosis with optimal treatment.

LESSONS

Rhabdomyosarcoma is a malignant neoplasm arising from undifferentiated skeletal muscle cells, with morphological, immunohistochemical, ultrastructural, or molecular genetic evidence of primary skeletal muscle differentiation. It presents with a rapidly worsening clinical course and the final outcome is poor. Treatment is widely based on protocols that have been proven to be effective in extracranial versions of these tumors, although repeatedly ineffective. Primary brain rhabdomyosarcoma poses a diagnostic challenge because of its infrequent presentation, grade of undifferentiation and tumor heterogeneity. Immunohistochemical and genetic testing have proven to be useful tools for diagnosis.

Open access

Non-contrast–enhancing subdural empyema: illustrative case

Alexander D. Rebchuk, Stephano J. Chang, Donald E. G. Griesdale, and Christopher R. Honey

BACKGROUND

Subdural empyema (SDE) is a life-threatening intracranial infection that, without timely surgical intervention and appropriate antibiotic treatment, is inevitably fatal. SDE is classically recognized on brain imaging as a subdural collection surrounded by a contrast-enhancing ring.

OBSERVATIONS

The authors describe the case of a 41-year-old male with clinical features consistent with SDE but without any contrast enhancement on multiple computed tomography scans obtained more than 48 hours apart. Given the high clinical suspicion for SDE, a craniotomy was performed that demonstrated frank pus that eventually grew Streptococcus pyogenes.

LESSONS

This case demonstrates that SDE may present without ring enhancement on contrast-enhanced imaging. In critically ill patients with a high clinical suspicion for SDE despite lack of contrast enhancement, we demonstrate that exploratory burr holes or craniotomy can provide diagnostic confirmation and source control.

Open access

Central nervous system mature teratoma producing carbohydrate antigen 19-9: illustrative case

Shu Takeuchi, Yoshiki Arakawa, Yasuhide Takeuchi, Sachiko Minamiguchi, Masahiro Tanji, Yohei Mineharu, Hironori Haga, and Susumu Miyamoto

BACKGROUND

Central nervous system (CNS) mature teratoma is a rare disease with symptoms that can vary according to tumor location. Most lesions are benign; rarely, malignancy can develop in any of the somatic components. Elevated levels of tumor markers such as α-fetoprotein and β-human chorionic gonadotropin are not usually found in patients with CNS mature teratoma, and no reports have described an association with carbohydrate antigen 19-9 (CA19-9).

OBSERVATIONS

A 64-year-old woman with headache was found to have a mass lesion in the anterior cranial fossa. Magnetic resonance imaging of the brain suggested a mature teratoma. Serum and cerebrospinal fluid (CSF) tests showed significant CA19-9 elevations (2,770 U/mL and 4,387 U/mL, respectively). Other examinations, including whole-body 18F-fluorodeoxyglucose positron emission tomography, did not detect the origin of elevated CA19-9, suggesting that the high CA19-9 levels were caused by intracranial tumor. The patient underwent tumor removal. The histopathological diagnosis was mature teratoma with positive CA19-9 staining. CA19-9 levels in serum and CSF decreased significantly after tumor removal.

LESSONS

The histopathological findings and postoperative decreased CA19-9 levels established the diagnosis of CA19-9–producing CNS mature teratoma. CNS mature teratoma can cause elevations in CA19-9 in cases with absence of neoplasms in the trunk.

Open access

Seizure freedom after laser amygdalohippocampotomy guided by bilateral responsive neurostimulation in pediatric epilepsy: illustrative case

Belinda Shao, Bryan Zheng, David D. Liu, Matthew N. Anderson, Konstantina Svokos, Luca Bartolini, and Wael F. Asaad

BACKGROUND

For patients with difficult-to-lateralize temporal lobe epilepsy, the use of chronic recordings as a diagnostic tool to inform subsequent surgical therapy is an emerging paradigm that has been reported in adults but not in children.

OBSERVATIONS

The authors reported the case of a 15-year-old girl with pharmacoresistant temporal lobe epilepsy who was found to have bitemporal epilepsy during a stereoelectroencephalography (sEEG) admission. She underwent placement of a responsive neurostimulator system with bilateral hippocampal depth electrodes. However, over many months, her responsive neurostimulation (RNS) recordings revealed that her typical, chronic seizures were right-sided only. This finding led to a subsequent right-sided laser amygdalohippocampotomy, resulting in seizure freedom.

LESSONS

In this case, RNS chronic recording provided real-world data that enabled more precise seizure localization than inpatient sEEG data, informing surgical decision-making that led to seizure freedom. The use of RNS chronic recordings as a diagnostic adjunct to seizure localization procedures and laser ablation therapies in children is an area with potential for future study.

Open access

Solitary fibrous tumor with aneurysmal bone cyst–like change in the middle cranial fossa: illustrative case

Ako Matsuhashi, Taichiro Yoshimoto, and Gakushi Yoshikawa

BACKGROUND

Solitary fibrous tumor (SFT) is a rare mesenchymal tumor known for its propensity for recurrence and metastasis. Furthermore, aneurysmal bone cyst (ABC) is a benign osteolytic lesion. ABC-like areas can be seen in bone tumors that have undergone hemorrhagic cystic change. They are formed by disruptions in the osseous circulation caused by the associated lesion. The most common associated lesions are giant cell tumor, chondroblastoma, osteoblastoma, osteosarcoma, chondromyxoid fibroma, and fibrous dysplasia. There has been no reported case of SFT being the associated lesion.

OBSERVATIONS

A 42-year-old woman presented with a 6-month history of headache and impaired memory. Radiological examinations revealed a 50-mm cystic lesion with multiple fluid levels arising from the left temporal bone. Total resection of the tumor was conducted, and postoperative course was uneventful. Histopathological examination was consistent with SFT with ABC-like change.

LESSONS

This is the first documented case of SFT with ABC-like change in the cranial fossa. This should be considered a differential diagnosis when treating a lesion in the cranial fossa, such as in this case, to achieve complete resection of the tumor and have close follow-up postoperatively.

Open access

Bilateral papilledema with vision loss due to post–COVID-19–induced thiamine deficiency: illustrative case

Kern H. Guppy, Yekaterina K. Axelrod, and Han Kim

BACKGROUND

Bilateral papilledema with vision loss is considered a neurosurgical emergency due to high intracranial pressure. However, it may not be the only cause of papilledema. The authors reported an association among coronavirus disease 2019 (COVID-19), bilateral papilledema, blindness, and Wernicke’s encephalopathy (WE).

OBSERVATIONS

An 18-year-old woman presented to the neurosurgery service with rapid profound vision loss and bilateral papilledema. She had COVID-19 3 months earlier with subsequent loss of smell (anosmia) and taste (ageusia), which resulted in hyperemesis and a 43-lb weight loss. Examination revealed ataxia, horizontal nystagmus, and blindness. Magnetic resonance imaging and magnetic resonance venography of her brain were normal. Presumptive diagnosis of WE was made, and she was treated with intravenous thiamine with restoration of vision within 48 hours. Patient’s thiamine level was less than half the normal value.

LESSONS

Neurosurgeons should be aware of this unique correlation between papilledema and vision loss and its association with WE due to post–COVID-19 hyperemesis and weight loss from anosmia and ageusia.

Open access

Interactive spinal computed tomography angiography–guided spinal digital subtraction angiography and embolization for thoracolumbar epidural arteriovenous fistulas: illustrative case

Hirokuni Hashikata, Yoshinori Maki, Ryota Ishibashi, and Hiroki Toda

BACKGROUND

Spinal digital subtraction angiography (sDSA) is the gold standard for examining spinal arteriovenous fistulas; however, thorough sDSA evaluations of spinal arteriovenous fistulas require a long procedure, which may increase the radiation exposure time.

OBSERVATIONS

A 72-year-old man presented with progressive myelopathy due to a spinal epidural arteriovenous fistula. Spinal computed tomography angiography (sCTA) showed an epidural arteriovenous fistula fed by the left L3 segmental artery. To prepare for sDSA, the sCTA images were modified to mark the segmental artery bifurcations from T5 to L5 with multicolored markers. These modified sCTA images were loaded onto the multiwindow DSA display. The sCTA images were interactively modulated during sDSA. This sCTA-guided sDSA identified 18 segmental arteries within 47 minutes. The total radiation exposure was 1,292 mGy. Subsequently, transarterial embolization resolved the epidural arteriovenous fistula with clinical improvement.

LESSONS

Three-dimensional sCTA can provide detailed anatomical information before sDSA. Modified sCTA images with segmental artery bifurcation marking can provide interactive guidance on multipanel DSA displays. sCTA-guided sDSA is useful for accurate catheterization and reduction of procedure time.

Open access

Multiple-site neural tube defects complicated by multiple-site split cord malformations and thickened filum terminale: experience at a pediatric neurosurgical teaching hospital in Ethiopia. Illustrative case

Mestet Yibeltal Shiferaw, Yemisirach Bizuneh Akililu, Bethelehem Yesehak Worku, Tsegazeab Laeke T/Mariam, and Abenezer Tirsit Aklilu

BACKGROUND

Multiple-site open neural tube defects (MNTDs) and multiple-site split cord malformations (MSCMs) are extremely rare congenital anomalies that are defined by the simultaneous noncontiguous occurrence of more than one neural tube defect (NTD) and split cord malformation (SCM), respectively, in a single case with normal neural tissue in between. This work shows the cooccurrence of MNTDs and MSCMs, which has never been reported in the literature.

OBSERVATIONS

A single-stage repair for a 13-day-old female neonate with a preoperative diagnosis of MNTDs (thoracic meningocele and thoracolumbar myelomeningocele) plus an additional intraoperative diagnosis of MSCMs (type 3c) of thoracic and thoracolumbar spine, and thickened filum terminale was done with a favorable smooth postoperative course.

LESSONS

The use of intraoperative meticulous surgical technique along with preoperative skin stigmata helped for anticipation, detection, and treatment of associated complex spinal MNTDs, especially in resource-limited settings, where preoperative magnetic resonance imaging is not routinely used. Whether to repair the MNTDs as a single- versus multiple-stage procedure is mainly a function of the patient’s tolerance to the duration of anesthesia and the anticipated blood loss for the patient’s age. The overall developmental biology and long-term clinical outcome of MNTDs compared to single NTD/SCM is poorly understood and needs further study.