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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.

Open access

Brain metastasis and intracranial leptomeningeal metastasis from malignant peripheral nerve sheath tumors: illustrative cases

Masasuke Ohno, Shoichi Haimoto, Satoshi Tsukushi, Waki Hosoda, Fumiharu Ohka, and Ryuta Saito

BACKGROUND

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft-tissue tumors. Intracranial metastasis from MPNSTs is quite rare.

OBSERVATIONS

The authors report on a 73-year-old male whose MPNST metastasized to the brain and a 32-year-old male with leptomeningeal metastasis from MPNST and review 41 cases of MPNST that developed intracranial metastasis, as reported in the literature.

LESSONS

Brain metastasis and leptomeningeal metastasis of MPNSTs show different clinical courses and require pathology-specific treatment.

Open access

Rapidly progressive diffuse leptomeningeal glioneuronal tumor in an adult female: illustrative case

Jonathan Bao, Jared F. Sweeney, Yang Liu, Frank L. Genovese, Matthew A. Adamo, and Robert S. Heller

BACKGROUND

Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare brain tumor only recently classified by the World Health Organization in 2016 and has few reports on its incidence in adults.

OBSERVATIONS

The authors describe a case of DLGNT presenting in a 47-year-old female with seizures, cranial neuropathies, and communicating hydrocephalus with rapid clinical progression. Workup demonstrated progressive leptomeningeal enhancement of the skull base, cranial nerves, and spine, and communicating hydrocephalus. Elevated serum rheumatological markers and early response to systemic corticosteroids and immunosuppressant therapy complicated the diagnosis. Multiple biopsy attempts were required to obtain diagnostic tissue. Pathology demonstrated hypercellularity surrounding leptomeningeal vessels with nuclear atypia, staining positive for GFAP, Olig2, S100, and synaptophysin. Molecular pathology demonstrated loss of chromosome 1p, BRAF overexpression but no rearrangement, and H3K27 mutation. Repeat cerebrospinal fluid (CSF) diversion procedures were required for hydrocephalus management due to high CSF protein content.

LESSONS

This report describes a rare, aggressive, adult presentation of DLGNT. Leptomeningeal enhancement and communicating hydrocephalus should raise suspicion for this disease process. Biopsy at early stages of disease progression is essential for early diagnosis and prompt treatment. Further study into the variable clinical presentation, histological and molecular pathology, and optimal means of diagnosis and management is needed.

Open access

Malignant transformation of a cerebral dermoid cyst into a squamous cell carcinoma with malignant intraperitoneal spreading along a ventriculoperitoneal shunt: illustrative case

Robert C. Nickl, Vera Nickl, Magnus Schindehütte, Camelia-Maria Monoranu, Ralf-Ingo Ernestus, and Mario Löhr

BACKGROUND

Malignant progression of intracranial dermoid cysts into squamous cell carcinoma is extremely rare with only three reports published so far. Intracranial dermoid cysts are uncommon benign tumors lined by stratified squamous epithelium of embryonic ectodermal origin.

OBSERVATIONS

Here, the authors present the case of a 64-year-old female with a recurrent temporal dermoid cyst. After surgery for the recurrent dermoid cyst, once in the early 1990s and another 16 years later, the patient presented with headache and nausea due to hydrocephalus. After implantation of a ventriculoperitoneal shunt, she deteriorated rapidly and died only 60 days after admission. Autopsy revealed malignant transformation of the epithelial lining of the dermoid cyst into a squamous cell carcinoma resulting in neoplastic meningiosis and intraperitoneal tumor spread along a previously implanted ventriculoperitoneal shunt.

LESSONS

Malignant transformation should be considered in patients with dermoid cyst who show new leptomeningeal contrast enhancement. In the case of hydrocephalus, alternatives to peritoneal shunting should be considered.

Open access

Embolization of a third ventricular choroid plexus papilloma: illustrative case

Jordan C. Xu, Shuichi Suzuki, Jennifer Chrislip, Ali Nael, and William G. Loudon

BACKGROUND

Choroid plexus tumors are rare neoplasms that are typically found in the lateral ventricles. They have infrequently been reported in the third ventricle, and treatment strategies have varied.

OBSERVATIONS

The authors described a 6-month-old patient who presented with irritability and hypotonia. The patient was found to have a large tumor in the third ventricle with obstructive hydrocephalus. Preoperative angiography revealed a feeding artery from the right medial posterior choroidal artery (MPChA). The feeding artery was effectively embolized, and the patient subsequently received open resection of the tumor. Pathology revealed features consistent with atypical choroid plexus papilloma (CPP).

LESSONS

Choroid plexus tumors are often hypervascular, which can often make gross total resection (GTR) difficult. Preoperative angiography of tumors identifies feeding vessels, and embolization has the potential to decrease perioperative blood loss and allow for a higher GTR rate. Third ventricular CPPs appear to invariably receive vascular supply from the MPChA, arising from the right posterior cerebral artery. Embolization followed by resection of a choroid plexus tumor in the third ventricle is an effective treatment strategy.

Open access

Multifocal metastases to choroid plexus from papillary thyroid carcinoma: illustrative case

Isidora R. Beach, Adam M. Olszewski, Alissa A. Thomas, John C. DeWitt, and Brandon D. Liebelt

BACKGROUND

Choroid plexus metastases are extremely rare from all types of malignancy, with only 42 cases reported in the literature thus far. Most of these originate from renal cell carcinoma and present as a solitary choroid plexus lesion; only two cases of multifocal choroid plexus metastases have been reported to date.

OBSERVATIONS

The authors report the third case of multifocal metastases to the choroid plexus, that of a 75-year-old man who developed three measurable choroid plexus lesions approximately 3.5 years after undergoing total thyroidectomy and chemotherapy for papillary thyroid carcinoma. He underwent intraventricular biopsy of the largest lesion and subsequently died of hydrocephalus after opting for comfort care only.

LESSONS

This is the third case of multifocal choroid plexus metastasis in the literature and the second case of multifocal metastasis from thyroid carcinoma. As such, the natural disease course is not well characterized. This case is compared with the previous eight reports of choroid plexus metastases from thyroid carcinoma, seven of which involved solitary lesions. The eight prior cases are evaluated with attention to treatment modalities used and factors potentially influencing prognosis, specifically those that might contribute to hydrocephalus, a reported complication for this pathology.

Open access

Rare large colloid cyst obstructing the posterior third ventricle: illustrative case

Jakob V. E. Gerstl, Kristian Aquilina, and Jeffrey E. Florman

BACKGROUND

Colloid cysts of the posterior third ventricle are exceedingly rare. This location is a high-risk zone for colloid cysts because of potential obstruction of the cerebral aqueduct.

OBSERVATIONS

The authors report a case of a 57-year-old man who presented with a 6-month history of progressive headache, short-term memory loss, visual blurring, and an episode of double vision. Magnetic resonance imaging (MRI) revealed a colloid cyst, 22 mm in diameter, attached to the roof of the posterior third ventricle. The posterior third ventricle was obstructed, but both foramina of Monro were patent. The entirety of the cyst was resected via a right frontal parasagittal, interhemispheric, transventricular approach through the foramen of Monro. It was not contained within the velum interpositum. The patient did not experience any postoperative deficits.

LESSONS

This case supports the theoretical evidence that colloid cysts in the posterior zone of the third ventricle pose a risk for obstructive hydrocephalus. This report of a large cyst in a rare location describes a unique lesion and provides the first published MRI description.

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

Subcutaneous emphysema of the neck after shunt surgery for hydrocephalus in a case of metastatic ovarian cancer: illustrative case

Smrithi Sathish, M. Manoranjitha Kumari, Shyama S. Prem, and Gopalakrishnan M. Sasidharan

BACKGROUND

A 46-year-old female, a patient with a relapsed carcinoma in her ovary, had undergone ventriculoperitoneal (VP) shunt surgery for obstructive hydrocephalus due to vermian metastasis. Two weeks after the shunt surgery, she complained of discomfort in the neck. There was subcutaneous emphysema along the shunt track without tenderness or signs of inflammation. She was afebrile, and her vital parameters were stable.

OBSERVATIONS

The authors ruled out pneumothorax and airway trauma as potential sources of emphysema. They tapped the shunt chamber and detected gram-negative bacilli. Ascitic fluid culture grew gas-forming Escherichia coli.

LESSONS

Although some amount of air can get trapped in the subcutaneous plane during the tunneling procedure of a VP shunt tube insertion, the reappearance of a new, large column of air along the shunt track can be an ominous sign of shunt infection. The shunt became contaminated by bacteria of gut origin, which seeded the ascitic fluid, and a florid bacterial growth ascended up the shunt track, producing gas along the subcutaneous plane. Physicians should consider this rare etiology in their differential diagnoses of subcutaneous emphysema following VP shunt surgery.