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Open access

Pituitary germinoma after resection of a mature third ventricular teratoma: illustrative case

Tim J Hallenberger, Emma von Seth, Michel Roethlisberger, Raphael Guzman, and Jehuda Soleman

BACKGROUND

Metachronous intracranial germ cell tumors (iGCTs)—unrelated, histologically different iGCTs occurring at different time points—occurring within the same patient remain a rarity. Herein, the authors report such a case and discuss the literature and potential pathophysiological mechanisms leading to this phenomenon.

OBSERVATIONS

A 9-year-old boy presented with new-onset impaired balance, headaches, nausea, visual disturbances, and left facial paresis. Magnetic resonance imaging (MRI) scans revealed a suspected pineal region teratoma originating from the pineal gland with consecutive obstructive hydrocephalus. A mature teratoma was diagnosed and resected. Postoperative recovery was good, and the patient could return to his normal daily activities. However, a new, slowly progressive lesion in the sellar region with an enlarged infundibular stalk was detected on follow-up MRI 3.5 years after initial pineal region teratoma resection. Biopsy revealed a newly developed pure germinoma. The patient was treated with radiotherapy plus chemotherapy and remained relapse free at the last follow-up. Sixteen other cases have reported a surgically resected primary mature teratoma, wherein patients developed metachronous germinomas during follow-up. Different theories try to elaborate this phenomenon, yet none can completely account for it.

LESSONS

Although rare, metachronous iGCT is a phenomenon neurosurgeons should be aware of. In patients treated for iGCT, close long-term clinical, imaging, and laboratory follow-up is recommended.

https://thejns.org/doi/10.3171/CASE2443

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

The infundibulochiasmatic angle and the favorability of an endoscopic endonasal approach in type IV craniopharyngioma: illustrative case

Guilherme Finger, Maria Jose C Ruiz, Eman H Salem, Matthew D Marquardt, Kyle C Wu, Lucas P Carlstrom, Ricardo L Carrau, Luciano M Prevedello, and Daniel M Prevedello

BACKGROUND

Lesions located in the floor of the third ventricle are among the most difficult to access in neurosurgery. The neurovascular structures can limit transcranial exposure, whereas tumor extension into the third ventricle can limit visualization and access. The midline transsphenoidal route is an alternative approach to tumor invading the third ventricle if the tumor is localized at its anterior half and a working space between the optic apparatus and the pituitary infundibulum exists. The authors introduce the “infundibulochiasmatic angle,” a valuable measurement supporting the feasibility of the translamina terminalis endoscopic endonasal approach (EEA) for resection of type IV craniopharyngiomas.

OBSERVATIONS

Due to a favorable infundibulochiasmatic angle measurement on preoperative magnetic resonance imaging (MRI), an endoscopic endonasal transsellar transtubercular approach was performed to resect a type IV craniopharyngioma. At 2-month follow-up, the patient’s neurological exam was unremarkable, with improvement in bitemporal hemianopsia. Postoperative MRI confirmed gross-total tumor resection.

LESSONS

The infundibulochiasmatic angle is a radiological tool for evaluating the feasibility of EEA when resecting tumors in the anterior half of the third ventricle. Advantages include reduced brain retraction and excellent rates of resection, with minimal postoperative risks of cerebrospinal fluid leakage and permanent pituitary dysfunction.

Open access

A sellar-suprasellar malignant optic pathway glioblastoma in the absence of prior radiation therapy: illustrative case

Mestet Yibeltal Shiferaw, Tsegazeab Laeke Teklemariam, Abenezer Tirsit Aklilu, Dejen Teke Gebrewahd, Bereket Hailu Mekuria, Ermias Fikru Yesuf, and Taye Jemberu Robele

BACKGROUND

To date, only a few cases of sellar and suprasellar glioblastomas have been reported even though high-grade glioma constitutes the most common adult brain tumor, commonly arising in the cerebral hemispheres. It arises de novo from astrocytes within the optic nerve, optic chiasm, or optic tracts and is quite challenging to diagnose and treat. To the authors’ knowledge, there are 72 cases (including this one) of optic glioma malignancies in the medical literature, 30 corresponding to glioblastomas.

OBSERVATIONS

The authors present the diagnostic considerations and challenges, management strategies, and clinical course of a very large sellar-suprasellar glioblastoma in a 19-year-female who had never received radiation therapy or prior surgery.

LESSONS

Sellar-suprasellar glioblastomas, although extremely rare, are known to occur and pose challenges in their diagnosis and preoperative treatment planning. The presence of diffusion restriction on diffusion-weighted magnetic resonance imaging in a mass lesion that has ring and nodular postcontrast enhancement in addition to absent calcification on computed tomography should be alert to the possibility of a high-grade mass. This is extremely important for preoperative patient counseling and planning for the multimodal treatments, because sellar-suprasellar glioblastomas carry a poorer prognosis than the common benign mass lesions in the region.

Open access

Introducing next-generation transcranial surgery with the head-mounted 3D View Vision display in extracorporeal microsurgery: illustrative cases

Young Ju Kim, Hidehito Kimura, Hiroto Kajimoto, Tatsuya Mori, Masahiro Maeyama, Kazuhiro Tanaka, and Takashi Sasayama

BACKGROUND

Exoscopy in neurosurgery offers various advantages, including increased freedom of the viewing axis while the surgeon maintains a comfortable upright position. However, the optimal monitor positioning to avoid interference with surgical manipulation remains unresolved. Herein, the authors describe two cases in which a three-dimensional head-mounted display (3D-HMD) was introduced into a transcranial neurosurgical procedure using an exoscope.

OBSERVATIONS

Case 1 was a 50-year-old man who presented with recurrent epistaxis and was diagnosed with an olfactory neuroblastoma that extended from the nasal cavity to the anterior cranial base and infiltrated the right anterior cranial fossa. Case 2 was a 65-year-old man who presented with epistaxis and was diagnosed with a left-sided olfactory neuroblastoma. In both cases, en bloc tumor resection was successfully performed via a simultaneous exoscopic transcranial approach using a 3D-HMD and an endoscopic endonasal approach, eliminating the need to watch a large monitor beside the patient.

LESSONS

This is the first report of using a 3D-HMD in transcranial surgery. The 3D-HMD effectively addressed issues with the field of vision and concentration while preserving the effectiveness of traditional microscopic and exoscopic procedures when observed on a 3D monitor. Combining the 3D-HMD with an exoscope holds the potential to become a next-generation surgical approach.

Open access

Radiological features of internal carotid artery occlusion caused by pituitary apoplexy: illustrative case

Keigo Aramaki, Masanori Aihara, Yu Kanazawa, Takahiro Kawashima, Rei Yamaguchi, Masahiro Matsumoto, Masahiko Tosaka, and Yuhei Yoshimoto

BACKGROUND

Pituitary apoplexy rarely causes internal carotid artery (ICA) occlusion and acute ischemic stroke. Some cases have been reported, but the neuroimaging findings, including cerebral angiography, have not been discussed.

OBSERVATIONS

A 55-year-old male suffered the sudden onset of right cervical pain and left mild hemiparesis. Computed tomography indicated a pituitary mass, and magnetic resonance angiography showed a right ICA occlusion. The initial diagnosis was ICA occlusion caused by ICA dissection. His symptoms worsened and the region of cerebral infarction expanded, so the patient was transferred to our hospital. Magnetic resonance imaging and cerebral angiography showed the sudden stoppage of right ICA blood flow caused by local compression of the tumor near the distal dural ring. The diagnosis was acute ischemic stroke resulting from ICA pseudo-occlusion caused by pituitary apoplexy, and emergent endoscopic transsphenoidal resection was performed. Postoperatively, the right ICA was completely patent, and hemiparesis was improved with rehabilitation.

LESSONS

ICA occlusion caused by pituitary apoplexy is very rare, but emergent treatment is necessary. However, the pathology is difficult to diagnose quickly. Neuroimaging findings showing that the ICA is easily stenosed or occluded if rapidly compressed by the tumor near the distal dural ring may be useful to rapidly diagnose and treat.

Open access

Consecutive resections of double pituitary adenoma for resolution of Cushing disease: illustrative case

Stephanie A Armstrong, Samon Tavakoli, Ipsit Shah, Brandon R Laing, Dylan Coss, Adriana G Ioachimescu, James Findling, and Nathan T Zwagerman

BACKGROUND

Double pituitary adenomas are rare presentations of two distinct adenohypophyseal lesions seen in <1% of surgical cases. Increased rates of recurrence or persistence are reported in the resection of Cushing microadenomas and are attributed to the small tumor size and localization difficulties. The authors report a case of surgical treatment failure of Cushing disease because of the presence of a secondary pituitary adenoma.

OBSERVATIONS

A 32-year-old woman with a history of prolactin excess and pituitary lesion presented with oligomenorrhea, weight gain, facial fullness, and hirsutism. Urinary and nighttime salivary cortisol elevation were elevated. Magnetic resonance imaging confirmed a 4-mm3 pituitary lesion. Inferior petrosal sinus sampling was diagnostic for Cushing disease. Primary endoscopic endonasal transsphenoidal resection was performed to remove what was determined to be a lactotroph-secreting tumor on immunohistochemistry with persistent hypercortisolism. Repeat resection yielded a corticotroph-secreting tumor and postoperative hypoadrenalism followed by long-term normalization of the hypothalamic-pituitary-adrenal axis.

LESSONS

This case demonstrates the importance of multidisciplinary management and postoperative hormonal follow-up in patients with Cushing disease. Improved strategies for localization of the active tumor in double pituitary adenomas are essential for primary surgical success and resolution of endocrinopathies.

Open access

Occult neurohypophyseal germinoma discovered during the course of long-term diabetes insipidus: illustrative case

Hironori Yamada, Ryokichi Yagi, Akihiro Kambara, Yoshihide Katayama, Yuichiro Tsuji, Ryo Hiramatsu, Naokado Ikeda, Masahiro Kameda, Naosuke Nonoguchi, Motomasa Furuse, Shinji Kawabata, Toshihiro Takami, and Masahiko Wanibuchi

BACKGROUND

The authors report a case of occult neurohypophyseal germinoma detected in a patient with long-term diabetes insipidus. Central diabetes insipidus is the initial symptom in 95% of cases of neurohypophyseal germinoma. In occult neurohypophyseal germinomas, no abnormalities are seen on magnetic resonance imaging (MRI) at the onset of symptoms. It can take several months or even years for these changes to be detected on MRI.

OBSERVATIONS

A 20-year-old male was diagnosed with central diabetes insipidus at the age of 17 years, and gonadal and adrenal corticosteroid insufficiency was noted at the age of 19 years. Head MRI showed an enlarged and enhanced pituitary stalk. He was referred to our department for a suspected neoplastic lesion. Endoscopic transsphenoidal biopsy indicated a pure germinoma. He was treated with chemotherapy and radiotherapy and then was discharged.

LESSONS

In this case, new imaging findings appeared 19 months after the onset of diabetes insipidus, and the pathological diagnosis was made after almost 24 months. Because the patient had a history of growth hormone deficiency and had a positive test result for diabetes insipidus, occult neurohypophyseal germinoma was suspected, and periodic contrast-enhanced MRI monitoring was deemed essential.

Open access

Rathke’s cleft cyst presenting with recurrent aseptic meningitis and inflammatory apoplexy: illustrative case

Roger Murayi, Megh M. Trivedi, Joao Paulo Almeida, Gabrielle Yeaney, Carlos Isada, and Varun R. Kshettry

BACKGROUND

Rathke’s cleft cyst (RCC) is a benign sellar/suprasellar lesion often discovered incidentally. Rarely, symptomatic cases can present with headache and may exhibit concomitant aseptic meningitis or apoplexy. The authors describe a patient with an RCC presenting with recurring episodes of aseptic meningitis and ultimately inflammatory-type apoplexy.

OBSERVATIONS

A 30-year-old female presented with three episodes of intractable headaches over 2 months. Each episode’s clinical picture was consistent with meningitis though cerebrospinal fluid cultures, and viral tests remained negative. Imaging demonstrated a sellar lesion, initially thought to be coincidental. On the third presentation, there was rapid interval growth of the lesion, adjacent cerebritis, and new endocrinopathy. Resection was then performed via an endoscopic endonasal approach. Pathology showed an RCC with acute and chronic inflammation and no evidence of hemorrhage. Cultures were negative for organisms. The patient received several weeks of antibiotic treatment with the resolution of all symptoms and no recurrence.

LESSONS

Recurrent aseptic meningitis with apoplexy-like symptoms is a rare presentation of RCC. The authors propose the term inflammatory apoplexy to describe such a presentation without evidence of abscess, necrosis, or hemorrhage. The mechanism is unclear although may be due to intermittent microleakage of cyst contents into the subarachnoid space.

Open access

Missed pituitary microadenoma during endoscopic transsphenoidal surgery for short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing with symptom relief: illustrative case

Juan Silvestre G. Pascual, Madeleine de Lotbiniere-Bassett, Katrina Hannah D. Ignacio, David Ben-Israel, Jessica M. Clark, and Yves P. Starreveld

BACKGROUND

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is a rare headache disorder that has been associated with pituitary adenomas. Resection has been posited to be curative.

OBSERVATIONS

A 60-year-old female presented with a 10-year history of SUNCT, which had been medically refractory. Sellar magnetic resonance imaging (MRI) showed a 2 × 2 mm nodule in the right anterolateral aspect of the pituitary. Endoscopic endonasal transsphenoidal resection of the pituitary microadenoma with neuronavigation was performed. The patient felt immediate relief from the headaches. Postoperative MRI showed persistence of the pituitary microadenoma and the resection tract to be inferomedial to the lesion. The right middle and partial superior turbinectomy site was close to the sphenopalatine foramen (SPF). The patient was discharged on postoperative day 1 and remained headache-free without any medications at the 4-month follow-up.

LESSONS

Resection of pituitary lesions associated with SUNCT may not necessarily be the cause of SUNCT resolution. Manipulation of the middle and superior turbinate close to the SPF may lead to a pterygopalatine ganglion block. This may be the mechanism of cure for SUNCT in patients with related pituitary lesions who undergo endonasal resection.