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Cover Journal of Neurosurgery: Case Lessons

Primary intraosseous xanthoma of the frontal bone in a child: illustrative case

Peter J Madsen, Adam J Kundishora, Benjamin C Reeves, Anne M Coyle, Daniel T Nagasawa, Judith M Wong, Isaac Yang, and Alexander M Tucker

BACKGROUND

Skull lesions are a common finding in children, with dermoid cysts and eosinophilic granulomas observed most frequently. However, primary intraosseous xanthomas of the calvaria, which are lytic, expansile lesions that develop without underlying hyperlipidemic disease, are rare in children, with only one prior case reported.

OBSERVATIONS

The authors describe the case of a healthy 6-year-old male who presented with a 2-month history of an enlarging midline skull mass that developed after a recent minor trauma. Imaging showed a full-thickness, lytic frontal bone lesion with an aggressive appearance and heterogeneous contrast enhancement. The patient underwent gross-total resection of the lesion with placement of a mesh cranioplasty. Histopathology revealed a primary intraosseous xanthoma. The patient was discharged on postoperative day 2 and required no further treatment at the 1-month follow-up.

LESSONS

This is the first reported case of a primary intraosseous xanthoma in the frontal bone of a pediatric patient. It emphasizes the need to include primary xanthomas in the differential diagnosis for pediatric skull lesions, particularly when the lesion has an aggressive radiographic appearance or the patient has a history of focal trauma. Furthermore, our findings indicate that resection, together with subsequent monitoring for lesion reccurrence, is an adequate first-line treatment.

Open access

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Cover Journal of Neurosurgery: Case Lessons

CyberKnife stereotactic radiosurgery for extramedullary plasmacytoma in the external auditory canal: illustrative case

Surya Patil, Elaheh Shaghaghian, Lorenzo Yuan, Aaryan Shah, Neelan J Marianayagam, David J Park, Scott G Soltys, Anand Veeravagu, Iris C Gibbs, Gordon Li, and Steven D Chang

BACKGROUND

Plasmacytoma, a rare plasma cell disorder, often presents as a solitary or multiple tumors within the bone marrow or soft tissues, typically associated with multiple myeloma. Extramedullary plasmacytomas (EMPs), particularly those located in the external auditory canal (EAC), are exceedingly rare and pose significant treatment challenges given their location, anatomical complexity, and high risk of recurrence.

OBSERVATIONS

The authors report the case of a 72-year-old male with a history of multiple myeloma, presenting with recurrent left EAC plasmacytoma. After initial conventional radiotherapy for the lesion, a recurrence was documented in 7 years. The patient subsequently underwent stereotactic radiosurgery, which proved successful, leading to complete resolution of the lesion without any long-term adverse effects or radiation-related complications over a 45-month period.

LESSONS

This case is a unique instance of utilizing stereotactic radiosurgery for recurrent EMP in the EAC, highlighting its potential as an effective approach in managing complex plasmacytomas.

Open access

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Cover Journal of Neurosurgery: Case Lessons

Phantom limb pain, traumatic neuroma, or nerve sheath tumor? Illustrative case

Patrick J Halloran, E. Antonio Chiocca, and Andres Santos

BACKGROUND

Phantom limb pain and traumatic neuromas are not commonly seen in neurosurgical practice. These conditions can present with similar symptoms; however, management of traumatic neuroma is often surgical, whereas phantom limb pain is treated with conservative measures.

OBSERVATIONS

A 77-year-old female patient with a long-standing history of an above-the-knee amputation experienced severe pain in her right posterior buttocks area for several years’ duration, attributed to phantom limb pain, which radiated down the stump of her leg and was treated with a variety of conservative measures. A recent exacerbation of her pain led to a prolonged hospitalization with magnetic resonance imaging of her leg stump, revealing a mass in the sciatic notch, at a relative distance from the stump. The anatomical location of the mass on the sciatic nerve in the notch led to a presumed radiological diagnosis of nerve sheath tumor, for which she underwent excision. At surgery, a neuroma of the proximal portion of the transected sciatic nerve that had retracted from the amputated stump to the notch was diagnosed.

LESSONS

Traumatic neuromas of transected major nerves after limb amputation should be considered in the differential diagnosis of phantom limb pain.

Open access

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Cover Journal of Neurosurgery: Case Lessons

Primary intracranial peripheral primitive neuroectodermal tumor: lessons from an exceptionally rare neoplasm. Illustrative case

Jhon E Bocanegra-Becerra, Luis Felipe Novoa-Ramírez, Alan Jesús Latorre-Zúñiga, Norka Tacas-Gil, and Rolando Rojas-Apaza

BACKGROUND

The primary intracranial peripheral primitive neuroectodermal tumor (pPNET) is a lesion subtype within the Ewing sarcoma family of tumors. pPNETs are extremely uncommon pathologies, accounting for 0.03% of intracranial tumors and 1% to 2% of Ewing sarcoma cases. Given its histological aspect similar to other highly proliferative malignant neuroectodermal neoplasms, pPNET merits extensive workup for accurate diagnosis and treatment.

OBSERVATIONS

A 36-year-old male presented to the emergency department with a 1-year history of headaches in the right frontoparietal area, generalized tonic-clonic seizures, and a history of the resection of a tumor labeled as a meningioma 5 years before admission. He was neurologically intact. Brain magnetic resonance imaging revealed a heterogeneous focal lesion of 25 × 35 × 23 mm with a necrotic center and neoformative appearance in the right frontal cortex. The patient underwent multimodal treatment with gross-total resection, radiotherapy, and chemotherapy. Histopathological examination results supported the diagnosis of pPNET. At the 2-year follow-up, the patient had no new-onset symptoms, and brain imaging revealed absent signs of tumor recurrence.

LESSONS

The present case describes an extraordinary pPNET case, initially confounded as a clear cell meningioma. Managing pPNET requires thorough investigation, careful differentiation from similar neuroectodermal lesions, and multimodal treatment to improve the patient’s prognosis.

Open access

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Cover Journal of Neurosurgery: Case Lessons

A rare case of atypical intradural extramedullary glioblastoma diagnosed utilizing next-generation sequencing and methylation profiling: illustrative case

William J Shelton, Andrew P Mathews, Karrar Aljiboori, J. Stephen Nix, Murat Gokden, and Analiz Rodriguez

BACKGROUND

Primary spinal cord tumors, especially primary spinal cord glioblastoma multiforme (PSC-GBM), are exceptionally rare, accounting for less than 1.5% of all spinal tumors. Their infrequency and aggressive yet atypical presentation make diagnosis challenging. In uncertain cases, a surgical approach for tissue diagnosis is often optimal.

OBSERVATIONS

A 76-year-old male presented with a rapidly progressing clinical history marked by worsening extremity weakness, urinary retention, and periodic fecal incontinence alongside diffuse changes on neuraxis imaging. The patient, in whom subacute polyneuropathy was initially diagnosed, received multiple rounds of steroids and intravenous immunoglobulin without clinical improvement. Histopathological review of the biopsy tissue yielded an initial diagnosis of spindle cell neoplasm. Next-generation sequencing (NGS) is done routinely on all neuropathology specimens at the authors’ institution, and methylation profiling is pursued in difficult cases. Ultimately, NGS and methylation profiling results were essential to an integrated final diagnosis of GBM.

LESSONS

PSC-GBM is a rare but highly aggressive occurrence of this tumor. Prolonged back pain, rapid neurological decline, and imaging changes warrant the consideration of lesional biopsy for precise disease characterization. In inconclusive cases, NGS has proved invaluable for clinical clarification and diagnosis, underscoring its importance for integrated diagnoses in guiding appropriate treatment strategies.

Open access

nlm-article

Cover Journal of Neurosurgery: Case Lessons

Migratory schwannoma of the cauda equina with a change in radicular pattern: illustrative case

Matthew S Parr, Sanford R Williams, James H Mooney, Travis J Atchley, and Mamerhi O Okor

BACKGROUND

Intradural spinal tumors are an uncommon entity with a variety of pathologies and symptom patterns. Few cases reports in the literature have described tumor migration within the spinal canal.

OBSERVATIONS

A 38-year-old male presented with bilateral upper lumbar radicular symptoms of anterior thigh pain, with an enhancing tumor of the cauda equina initially located at L1–2. He declined surgery initially, and at a follow-up 3 years later, his symptoms were unchanged but the tumor was now located at T12–L1. He again declined surgery, but 3 months later, he had a significant change in his pain distribution, which was now along his posterolateral right leg to his foot with associated dorsiflexion and extensor hallicus longus weakness. At this time, the tumor had migrated to L2–3. He underwent laminectomy and tumor resection with resolution of his radicular symptoms and improvement in his strength back to baseline by the 1-month follow-up. Pathology was consistent with a World Health Organization grade I schwannoma.

LESSONS

Migratory schwannoma is a rare entity but should be considered when radicular symptoms acutely change in the setting of a known intradural tumor. Repeat imaging should be performed to avoid wrong-level surgery. Intraoperative imaging can also be used for tumor localization.

Open access

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Cover Journal of Neurosurgery: Case Lessons

Successful resection of bilateral parafalcine meningioma with unilateral interhemispheric and contralateral transfalcine approach under nonintubated spontaneous breathing conditions: illustrative case

Seiichiro Hirono, Keisuke Kawano, Masato Ito, Kana Saito, Taiichiro Hayashida, and Yoshinori Higuchi

BACKGROUND

The best surgical approach for resecting bilateral parafalcine meningioma, as well as the optimal anesthesia and airway management for craniotomy in patients with interstitial pneumonia (IP) for preventing postsurgical exacerbation, remains unclear.

OBSERVATIONS

A 66-year-old female with a history of multiple relapses of IP underwent craniotomy for resection of a 4.5-cm bilateral parafalcine meningioma located just beneath the inferior sagittal sinus. To avoid mechanical ventilation or high-concentration oxygenation, the entire procedure was performed under nonintubated spontaneous breathing conditions with a supraglottic airway/laryngeal mask airway (SGA/LMA) device. Half of the tumor was resected using the ipsilateral interhemispheric approach, while the remaining half was resected using the contralateral transfalcine approach (CTA). No brain retractors were required. Preoperative embolization contributed toward reducing blood loss and surgery duration. During most of the operation, additional oxygen administration was not required. The postoperative course was uneventful, without exacerbation of the IP.

LESSONS

This case demonstrated the utility and feasibility of a unilateral interhemispheric approach combined with CTA for resection of a bilateral parafalcine meningioma. Additionally, this case provides an alternative method of airway and anesthesia management with an SGA/LMA device and nonintubated spontaneous breathing for the prevention of postoperative acute exacerbation of IP.

Open access

nlm-article

Cover Journal of Neurosurgery: Case Lessons

Time course of tumorigenesis of a newly developed sporadic hemangioblastoma in an elderly patient: illustrative case

Yu Sugiyama, Shunichiro Kuramitsu, Kaoru Eguchi, Masashi Ito, Ryo Ando, Hiroki Matsuno, Noriyuki Suzaki, and Satoshi Maesawa

BACKGROUND

von Hippel-Lindau disease–associated hemangioblastomas (HBs) account for 20%–30% of all HB cases, with the appearance of new lesions often observed in the natural course of the disease. By comparison, the development of new lesions is rare in patients with sporadic HB.

OBSERVATIONS

A 65-year-old man underwent clipping for an unruptured aneurysm of the anterior communicating artery. Fourteen years later, follow-up magnetic resonance imaging (MRI) revealed a strongly enhanced mass in the right cerebellar hemisphere, diagnosed as a sporadic HB. A retrospective review of MRI studies obtained over the follow-up period revealed the gradual development of peritumoral edema and vascularization before mass formation.

LESSONS

Newly appearing high-intensity T2 lesions in the cerebellum may represent a preliminary stage of tumorigenesis. Careful monitoring of these patients would be indicated, which could provide options for early treatment to improve patient outcomes.

Open access

nlm-article

Cover Journal of Neurosurgery: Case Lessons

Posterior-only 2-level vertebrectomy and fusion in a medically complex patient with lumbar metastasis: illustrative case

Ryan Johnson, Annabelle Shaffer, Ashley Tang, Kathryn Tsai, Gina Guglielmi, and Paul M Arnold

BACKGROUND

Spinal metastases are commonly seen in patients with cancer and often indicate a poor prognosis. Treatment can include curative or palliative surgery, chemotherapy, and radiation therapy. The surgical approach varies widely on the basis of the affected region of the spine, the location of the tumor (anterior versus posterior), the goal of surgery, the health of the patient, and surgeon preference.

OBSERVATIONS

The authors present a case of a 68-year-old male with intractable lower-back pain and substantially diminished ambulation. Diagnostic imaging revealed a lumbar metastasis from a cholangiocarcinoma primary at L2–3 (4.5 cm anteroposterior × 5.7 cm transverse × 7.0 cm craniocaudal). The patient underwent a 2-level vertebrectomy with expandable cage placement and T10 to S2 fusion via a posterior-only approach. The patient regained much of his mobility and quality of life after the surgery.

LESSONS

Although this was a high-risk surgery, the authors show that a posterior-only approach can be used for lumbar vertebrectomies and fusion when necessary. Palliative surgeries carrying a high risk, especially in the setting of a limited prognosis, should include multidisciplinary deliberations and a thorough discussion of the risks and outcome expectations with the patient.

Open access

nlm-article

Cover Journal of Neurosurgery: Case Lessons

Management of recurrent giant hemangiopericytoma: illustrative cases

Joshua Vignolles-Jeong, Guilherme Finger, Ben G McGahan, Thomas L Beaumont, Matthieu D Weber, Kyle C Wu, and Daniel M Prevedello

BACKGROUND

Hemangiopericytoma (HPC) is a rare malignancy accounting for 0.4% of intracranial tumors. HPCs are characterized by local aggressiveness, high rates of recurrence, and a tendency to metastasize to extracranial sites. These features make management of HPCs challenging, often requiring a combination of radical resection and radiation. Given their rarity, optimal treatment algorithms remain undefined.

OBSERVATIONS

The authors report a series of four patients who underwent resection of intracranial HPC. Mean age at presentation was 49.3 years. Three patients had reoperation for progression of residual tumor, and one patient was surgically retreated for recurrence. One patient received adjuvant radiotherapy following initial resection, and three patients received adjuvant radiotherapy following resection of recurrent or residual disease. There was one death in the series. Average progression-free survival and overall survival following the index procedure were 32.8 and 82 months, respectively. Progression occurred locally in all patients, with metastatic recurrence in one patient.

LESSONS

The current gold-standard treatment for intracranial HPC consists of gross-total resection followed by radiation therapy. This approach allows satisfactory local control; however, given the tendency for these tumors to recur either locally or distally within or outside of the central nervous system, there is a need for salvage therapies to improve long-term outcomes for patients.