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Open access

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.

Open access

Supraorbital keyhole approaches in the first 3 years of practice: outcomes and lessons learned. Patient series

Adnan Hussain Shahid, Danner Butler, Garrett Dyess, Maxon Bassett, Luke Harris, Ursula Hummel, Danielle Chason, and Jai Deep Thakur

BACKGROUND

Supraorbital keyhole approaches (SKAs) have garnered criticism for a limited surgical exposure, restrictive surgical freedom, blind spots, and the learning curve. This retrospective study of patients who underwent SKA aims to explore the outcomes, technical nuances, and the learning curve reflected in a single surgeon’s experience in the initial 3 years of practice.

OBSERVATIONS

A total of 20 SKA operations were performed in 19 patients. Gross- or near-total resection was achieved in 14 of 17 tumor cases. The mean blood loss was 80.5 mL, the mean duration of surgery was 5 hours, and the median stay was 3 days. Endoscopic augmentation was used in 11 cases in which additional tumor removal occurred in 8 of the 11 cases. There were no cases of cerebrospinal fluid leakage or wound infection. A 30-day readmission and typical narcotics after discharge were seen in one patient each. When comparing two halves of a neurosurgery practice over 3 years, the duration of surgery was significantly longer in the later year, which is likely due to operating on a larger tumor size as the years progressed. No cases required static retractors or conversion to larger craniotomies.

LESSONS

Careful case selection and respecting the learning curve allows the safe incorporation of SKA in the early stages of neurosurgical practice.

Open access

Role of stereotactic radiosurgery for recurrent skull base acinic cell carcinoma: illustrative case

Tomohiro Yoshihira, Motoyuki Umekawa, Yuki Shinya, Hirotaka Hasegawa, Masahiro Shin, Yodai Kikuchi, Yuki Saito, Kenji Kondo, Atsuto Katano, Aya Shinozaki-Ushiku, and Nobuhito Saito

BACKGROUND

Acinic cell carcinomas (AcCCs), rare malignancies of the salivary glands, often recur and metastasize, particularly in the skull base. Conventional radical resection can be invasive for skull base AcCCs adjacent to cranial nerves and major vasculature, and the effectiveness of stereotactic radiosurgery (SRS) as an alternative is not well established.

OBSERVATIONS

This case report details the application of SRS for recurrent skull base AcCCs. A 71-year-old male with a history of resection for a right mandibular AcCC 23 years earlier experienced tumor recurrence involving the right cavernous sinus and nasal cavity. He underwent endoscopic transnasal surgery followed by SRS targeting different tumor locations—the cavernous sinus to the pterygopalatine fossa, maxillary sinus, and clivus—each with a prescribed dose of 20 Gy to the 40% to 50% isodose line. After the first skull base metastasis, additional sessions of localized SRS after endoscopic surgery led to a 12-year survival without sequela.

LESSONS

This is a report indicating that SRS for skull base AcCCs can achieve favorable local control, functional preservation, and long-term survival. SRS may be suitable for skull base AcCC given the lesion’s tendency toward multiple local recurrences. Further investigation is needed to validate the treatment’s efficacy.

Open access

Chronic lymphocytic leukemia/small lymphocytic lymphoma arising in the pituitary gland: illustrative case

Hang Zhou, Xiaowei Zhang, Xin Jia, Liang Jia, and Qingjiu Zhang

BACKGROUND

The authors describe a 60-year-old female who underwent a correlative examination for an accidental scalp injury, revealing a sellar mass, which was surgically excised and pathologically confirmed to be a non-Hodgkin’s small B-cell lymphoma. These findings in combination with the immunophenotype led to a final diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma. Previous studies have shown that hematological solid tumors occurring in the pituitary gland are extremely rare, and there are only approximately three other cases of living patients with similarities to this case, all of which had ambiguous expression of subsequent hematological treatment.

OBSERVATIONS

In this case, the authors used an endoscopic approach to completely excise the tumor. Follow-up of the patient was continued after surgery, and the patient is currently receiving standardized treatment with zanubrutinib.

LESSONS

This patient did not have any previous history of tumor, had a good postoperative recovery with a normal quality of life, and still receives hormone replacement and zanubrutinib on a standardized basis. This is a complete case that has not been previously reported and reveals the diagnostic and therapeutic process of rare diseases in the sellar area.

Open access

Acute management of ruptured cavernous malformation of the optic nerve: illustrative case

Philip Kawalec, Marc R Del Bigio, and Anthony M Kaufmann

BACKGROUND

A cavernous malformation of the optic nerve (CMON) is a rare condition that often presents with an abrupt decline in vision. Acute management of ruptured optic nerve cavernous malformations is generally surgical, although the timing of surgery is controversial.

OBSERVATIONS

A 47-year-old female experienced the sudden loss of vision in her left eye. Examination showed that this eye was nearly blind, and her right eye had a temporal field defect. Neuroimaging showed hemorrhage in her left optic nerve and optic chiasm. She was taken to the operating room on an emergent basis where the optic canal was decompressed, the hemorrhage was evacuated, and a vascular malformation with features of a cavernoma was removed from the optic nerve. Over the next 2 days, the vision in her right eye significantly recovered.

LESSONS

CMONs remain rare, and it is unlikely that enough cases can be gathered to form a larger trial to compare the role and timing of surgery. On the basis of our experience with this case, the authors recommend that acute CMON-related hematomas should be treated as a surgical emergency and managed with acute optic nerve decompression, hematoma evacuation, and cavernoma resection to improve chances of vision recovery and prevent further vision loss.

Open access

Delayed symptomatic cerebral vasospasm following vestibular schwannoma resection: illustrative case

Paurush Pasricha, Alay V Khandhar, and Basant K Misra

BACKGROUND

Symptomatic cerebral vasospasm following posterior fossa extraaxial tumor resection is a rare phenomenon, with only 13 cases previously reported in the literature. The condition appears similar to vasospasm following supratentorial tumor resection, intraaxial posterior fossa tumor resection, and aneurysmal subarachnoid hemorrhage (aSAH). The majority of patients were not evaluated for vasospasm prior to symptom onset, leading to a delay in diagnosis.

OBSERVATIONS

The authors present their experience in a 56-year-old female who developed delayed cerebral vasospasm after excision of a solid-cystic vestibular schwannoma. Routine postoperative brain computed tomography showed evidence of subarachnoid hemorrhage in the basal cisterns. She was discharged on the 9th postoperative day. On the 11th day after tumor excision, she developed left hemiparesis, dysarthria, and dysphagia and was readmitted. Angiography confirmed bilateral diffuse cerebral vasospasm. The patient responded to standard hyperdynamic therapy used for vasospasm secondary to aSAH.

LESSONS

Symptomatic distant cerebral vasospasm after posterior fossa extraaxial tumor excision is a rare but challenging complication with a very high morbidity rate in reported cases. A high index of suspicion is required for early diagnosis and prompt management for a favorable outcome.

Open access

“Open-window” craniectomy for the removal of frontal sinus mucosa to prevent a delayed mucocele: illustrative case

W. Caleb Rutledge, 1 2 MS, Ahmad Ozair, Javier E. Villanueva-Meyer, Brian Niehaus, and Michael W. McDermott

BACKGROUND

Frontal craniotomies for a medial subfrontal approach necessitate crossing the frontal sinus. Large superior extensions of the frontal sinus into frontal bone can result in mucosal retention in a free craniotomy bone flap, leading to a delayed mucocele with significant associated morbidity. The authors describe an “open-window” craniectomy technique that permits mucosal removal under direct vision and maintains the inner table on the bone flap’s inferior side, helping to seal off the sinus opening with a pericranial flap.

OBSERVATIONS

An illustrative case involving a medial right frontal craniotomy for a third ventricle mass in a patient with a large superior extension of the frontal sinus into frontal bone is presented. After creating a free frontal bone flap, the inner table was drilled out to the margins of the frontal sinus cavity and any remaining mucosa was cleared. A portion of the inner table above the bone flap’s inferior margin was left in place, resembling an open window when viewed from the inner table side. The remaining anterior and posterior wall of the flap inferiorly provided a matched surface for the opening into the remaining frontal sinus, which was covered by pericranium. Long-term follow-up indicated no major complications or delayed mucocele.

LESSONS

The open-window craniectomy technique can be considered for frontal sinus violations in patients with large superior frontal bone extension.

Open access

Brainstem anesthesia during awake craniotomy: illustrative case

Yun Chen, Mei Sun, Hongmin Bai, Ruixin Yang, and Huan He

BACKGROUND

Awake craniotomy (AC) is performed to remove the lesions near or in eloquent areas, during which the patients are alert and without any airway instrument. Apnea is a severe complication in AC. Here, the authors describe a case of sudden apnea induced by unexpected local anesthesia of the brainstem during AC.

OBSERVATIONS

A 42-year-old male underwent AC for a large, recurrent, bilateral frontal lobe mass and experienced transient apnea and loss of brainstem reflexes during the surgery. The patient recovered spontaneous breath rhythm just a few minutes after the removal of a lidocaine cotton pledget, which was found near the patient’s midbrain. Then the patient awoke and cooperated to finish the surgery.

LESSONS

The administration of a local anesthetic subdurally in AC is common but risky. The scouring action of cerebral spinal fluid can spread those agents and cause unexpected brainstem anesthesia. A lower concentration of the anesthetic and keeping away from the cistern can make it safer.

Open access

Management of frontoethmoidal osteoma causing pneumocephalus and cerebrospinal fluid leakage with minimally invasive techniques: illustrative cases

Nicholas G Candy, Kyle C Wu, Guilherme Finger, Kyle VanKoevering, and Daniel M Prevedello

BACKGROUND

Osteoid osteoma is a common benign bone tumor frequently seen in the frontoethmoid region. However, involvement of the skull base is rare, with few cases previously reported.

OBSERVATIONS

The authors report two cases of spontaneous, symptomatic frontoethmoidal osteoma: one presented with neurological deficit secondary to tension pneumocephalus and the other with cerebrospinal fluid leakage. The first case was managed with a transfrontal sinus craniotomy and pneumocephalus decompression with osteoma resection and skull base reconstruction. The second case was managed with a uninaral endoscopic endonasal approach to the anterior skull base with osteoma resection and reconstruction.

LESSONS

Given the paucity of cases with associated tension pneumocephalus described in the literature, it was relevant to describe the authors’ experience with surgical decision-making and the expected outcomes among patients with this pathology when using minimally invasive techniques.

Open access

BRAF-mutated suprasellar glioblastoma mimicking craniopharyngioma: illustrative case

Bryan Zheng, Belinda Shao, Jennifer Mingrino, Jonathan Poggi, Richard S Dowd, Douglas C Anthony, John E Donahue, and Curtis E Doberstein

BACKGROUND

Suprasellar masses commonly include craniopharyngiomas and pituitary adenomas. Suprasellar glioblastoma is exceedingly rare with only a few prior case reports in the literature. Suprasellar glioblastoma can mimic craniopharyngioma or other more common suprasellar etiologies preoperatively.

OBSERVATIONS

A 65-year-old male with no significant history presented to the emergency department with a subacute decline in mental status. Work-up revealed a large suprasellar mass with extension to the right inferior medial frontal lobe and right lateral ventricle, associated with significant vasogenic edema. The patient underwent an interhemispheric transcallosal approach subtotal resection of the interventricular portion of the mass. Pathological analysis revealed glioblastoma, MGMT partially methylated, with a BRAF V600E mutation.

LESSONS

Malignant glioblastomas can mimic benign suprasellar masses and should remain on the differential for a diverse set of brain masses with a broad range of radiological and clinical features. For complex cases accessible from the ventricle where the pituitary complex cannot be confidently preserved via a transsphenoidal approach, an interhemispheric approach is also a practical initial surgical option. In addition to providing diagnostic value, molecular profiling may also reveal therapeutically significant gene alterations such as BRAF mutations.