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Open access

Metastatic intradural primary spinal osteosarcoma: illustrative case

Shreyas Thiruvengadam, Mark Lam, and Stephen Honeybul

BACKGROUND

Osteosarcomas are a common primary bone neoplasm among adolescents but represent 0.2% of all malignancies with an incidence of two to four cases per million persons annually worldwide. Although known to have significant metastatic potential, its rare incidence, treatment resistance, and poor prognosis have rendered it a poorly understood and infrequently documented pathology.

OBSERVATIONS

Herein the authors present the first documented case of lumbosacral intradural metastasis of a primary osteosarcoma in a young patient, possibly via intradural dissemination following pinhole durotomy in a prior thoracic surgery.

LESSONS

Osteosarcomas remain a difficult pathology to treat, particularly upon metastatic dissemination. The utility of adjuvant radiotherapy after resection of an osteosarcoma is increasingly evident in the reduction of local recurrence. In the context of intraoperative pinhole durotomies in resections of high-grade lesions, due consideration should be given to whole-spine radiation, although this remains an evidence-free zone.

Open access

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

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

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

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

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

Salvage pemetrexed for brain metastases from ALK-positive lung cancer after Gamma Knife radiosurgery: illustrative case

Ryuichi Noda, Atsuya Akabane, Mariko Kawashima, Masafumi Segawa, Sho Tsunoda, and Tomohiro Inoue

BACKGROUND

Systemic therapy for cancer treatment has improved, and therapeutic options for intracranial lesions are increasing. Combinations of treatment modalities are required in certain difficult cases. Gamma Knife radiosurgery (GKS) is effective for the treatment of brain metastases, especially for lesions that are inoperable because of their anatomical or functional location.

OBSERVATIONS

The authors report a case of brain metastases in anaplastic lymphoma kinase (ALK)-positive lung adenocarcinoma initially treated with GKS followed by the combination of repeat GKS and ALK tyrosine kinase inhibitors (ALK-TKIs) for tumor recurrence. During the clinical course, acquired resistance to ALK-TKIs due to the long exposure period was suspected. After a great deal of thought and discussion with the oncologist responsible for the treatment of the pulmonary lesions, the authors successfully controlled the lesion for the next 17 months by salvage pemetrexed administration.

LESSONS

This is the first report on the effectiveness of pemetrexed for recurrent brain metastasis from ALK-positive lung adenocarcinoma resistant to both radiosurgery and ALK inhibitors. Salvage pemetrexed showed a favorable therapeutic effect in this specific case.

Open access

Sentinel report of uniquely paired collision tumors: glioblastoma multiforme and coexistent intraventricular subependymoma. Illustrative case

Luke Antonio Silveira, Benjamin Abraham, Elizabeth Wicks, Raj Thakrar, Elnur Delahmetovic, Katherine Callahan, John DeWitt, Bruce Tranmer, and Brandon Liebelt

BACKGROUND

The presence of intracranial collision tumors, histologically distinct tumors occurring in anatomical proximity, is quite rare. Herein, the authors describe the sentinel case of a contiguous collision tumor combination consisting of glioblastoma multiforme and intraventricular subependymoma.

OBSERVATIONS

A 67-year-old male presented with several months of progressive fatigue superimposed on more recently noted word-finding difficulty, slight left-sided weakness, and episodic confusion. He was found to have a large right frontal mass abutting the right lateral ventricle with an additional nodular focus of enhancement within the right frontal horn. The patient underwent an awake right frontal craniotomy for gross-total resection of the tumor, noted to be of two distinct histological identities.

LESSONS

Although exceptionally rare, primary glial neoplasms of various histologies can be encountered simultaneously during resection, as in this case of co-occurring glioblastoma of the right frontal lobe and right frontal horn intraventricular subependymoma. Close attention to tumoral locations and the gross appearance of specimens during resection can prime the operative neurosurgeon for success in contributing to accurate diagnoses through sending separate pathological specimens for histological analysis when qualitatively different tissue is suspected.