Endoscopic Endonasal Approach for Optic Canal Dural Metastasis in a Patient with Progressive Visual Dysfunction: A Case Report.

To improve optic nerve function in a patient with progressive visual dysfunction, performing early decompressive and debulking surgery for a metastatic tumor located in the optic canal is essential. The endoscopic endonasal approach could be a practical and effective alternative for lesions in the inferomedial part of the optic canal. A 66-year-old man with a right visual eye field deficit had multiple lesions in the pineal gland, occipital lobe, and right inferomedial optic canal. The optic nerve was distorted by a tumor compressing against the falciform ligament. Although a systemic examination suggested the presence of primary lung cancer, the patient only complained of progressive visual impairment in the right eye. We planned surgery with endoscopic transethmoidal and transsphenoidal approaches to restore visual function and make a pathological diagnosis. During the procedure, we drilled the sella floor, tuberculum sellae, and optic canal and successfully removed the tumor underneath the dura mater. The patient's visual function improved rapidly following surgery, and no complications were observed, such as cerebrospinal fluid leakage. After confirming the pathological diagnosis, the patient subsequently received whole-brain radiotherapy. The endoscopic endonasal skull base approach to the optic canal region could be a practical alternative for treating symptomatic metastatic tumors.

Bifocal germ cell tumor of pineal germinoma and neurohypophyseal embryonal carcinoma: illustrative case.

BACKGROUND: Bifocal germ cell tumors, with primarily identical tissue composition, occur concurrently in the neurohypophyseal and pineal regions. OBSERVATIONS: A 16-year-old male patient exhibited increased intracranial pressure symptoms, with concurrent tumors in the pineal and neurohypophyseal regions, causing obstructive hydrocephalus. His serum human chorionic gonadotropin level was elevated, measuring 506.6 mIU/mL. Upon gross endoscopic examination, the pineal tumor appeared white, whereas the neurohypophyseal tumor appeared red and hemorrhagic. Because of the limited sample size of the latter, a frozen section biopsy was feasible only for the pineal lesion, which indicated the presence of a germinoma. Subsequently, carboplatin and etoposide were administered, resulting in the reduction of the pineal tumor, but no effect was observed in the neurohypophyseal tumor. Histopathological analysis confirmed the pineal lesion as a germinoma, whereas the neurohypophyseal lesion was an embryonal carcinoma. Thus, the treatment was altered to ifosfamide, carboplatin, and etoposide (ICE), leading to a response in both tumors. The patient underwent three additional cycles of ICE therapy and high-dose chemotherapy, followed by whole craniospinal irradiation, achieving complete remission. LESSONS: Although most bifocal germ cell tumors share the same histological tissue, occasional differences may arise, necessitating separate biopsies for accurate assessment.

A Collision Tumor of Pit-1/SF-1-positive Double Pituitary Adenoma and a Craniopharyngioma Coexisting with Graves' Disease.

Double or multiple pituitary adenomas expressing different types of transcription factors and collision tumors of pituitary adenomas and craniopharyngiomas are rare. In this report, we present a case of pituitary adenoma of two different cell populations, Pit-1 and SF-1, and an adenoma and craniopharyngioma collision tumor with coexisting Graves' disease. The patient had a 16-mm pituitary tumor with pituitary stalk calcification and optic chiasm compression but no visual dysfunction. Based on hormonal profile results, the tumor in the sella was considered a nonfunctioning pituitary adenoma; nevertheless, the pituitary stalk was invaded by a different lesion, which was later confirmed to be a craniopharyngioma. Using an endoscopic endonasal approach, the pituitary adenoma was removed; however, a small remnant remained medial to the right cavernous sinus. Because the pituitary stalk lesion was isolated from the pituitary adenoma, it was preserved to maintain pituitary function. Three years after the initial surgery, the patient suffered from Graves' disease and was treated with antithyroid medications. However, the intrasellar residual and pituitary stalk lesions gradually increased in size. A second surgery was performed, and the residual intrasellar and stalk lesions were completely removed. As per the initial and second histopathologies, the pituitary adenoma comprised different cell groups positive for thyroid-(TSH) and follicle-stimulating hormones, and each cell group was positive for Pit-1 and SF-1. The pituitary stalk lesion was an adamantinomatous craniopharyngioma. We believe that TSH-producing adenoma was involved in the development of Graves' disease or that treatment for Graves' disease increased TSH-producing adenoma.

Parent Artery Complex Coil Protection for Side-Branched Wide-Neck Aneurysms.

This study aimed to validate the usefulness of parent artery complex coil protection for the treatment of wide-neck, side-branched, and ruptured aneurysms. A microcatheter was first introduced into the aneurysmal sac, and another microcatheter was introduced into the parent artery or near the orifice of the branch artery. A framing coil was deployed partially from the first microcatheter, and a protection coil was deployed from the second microcatheter to prevent protrusion of the first framing coil to the parent artery and side branches. After the first framing coil insertion, the protection coil was withdrawn to confirm the stability of the framing coil and blood flow. The procedures with this technique were successful for 3 patients. Parent artery complex coil protection can be an effective and safe coil embolization technique for the preservation of parent and side branch arteries and an alternative method for emergent ruptured cases.

Skull Base Reconstruction Using a Temporoparietal Galeal Flap in Simultaneous Transnasal and Transcranial Surgery for the Prevention of Carotid Blowout Syndrome: A Report of 3 Cases.

Background Carotid blowout syndrome (CBS) is a rare complication that usually occurs after removal of head and neck tumors. Since transnasal skull base surgery allows a wide exposure of the ventral skull base, neurosurgeons should pay attention to prevent this devastating complication. We present, three cases involving exposure of the internal carotid artery (ICA) at the skull base during the simultaneous transnasal and transcranial approach. Case Description The first patient was a 69-year-old man with a recurrent chordoma. The exposed ICA was covered by an abdominal fat graft and nasoseptal flap, but he experienced CBS 2 months later and died. The second patient was a 66-year-old man with an intraosseous cavernous angioma of the petrous bone. The exposed ICA was covered by a temporoparietal galeal flap (TPGF), abdominal fat graft, and nasoseptal flap. The third patient was a 73-year-old man with skull base radiation necrosis and intracerebral abscess after proton beam therapy for orbital adenoid cystic carcinoma. The exposed ICA was covered by TPGF. The second and third patients' postoperative courses were uneventful. Conclusion Based on our experience, a nasoseptal flap alone may be insufficient to protect ICA. TPGF is therefore another available reconstruction option that may help prevent CBS.

Validity of PETRA-MRA for Stent-Assisted Coil Embolization of Intracranial Aneurysms.

Objective: Pointwise encoding time reduction with radial acquisition (PETRA) using magnetic resonance angiography (MRA) is a non-enhanced MRA technique employing an ultrashort echo time, and is known to significantly reduce the magnetic susceptibility of coils and stents during post-embolization imaging. We evaluated the quality of PETRA-MRA images for use at the follow-up assessment of stent-assisted coil embolization procedures performed to treat aneurysms. Methods: A total of six aneurysm patients who were treated by stent-assisted coil embolization were included. All patients underwent PETRA-MRA, time-of-flight (TOF)-MRA performed with MAGNETOM Skyra (Siemens), and digital subtraction angiography (DSA) performed with Infinix Celeve-i INFX-8000V (Canon Medical Systems) and Allura Clarity FD20/15 (Philips). The PETRA-MRA images were compared with those from DSA and TOF-MRA to validate the aneurysm occlusion status and visually assess the blood flow within the stent. Four independent specialists graded occlusion status and flow visualization through the stent using a four-point scale, where 4 points represented excellent visualization of flow within the stent. Results: The aneurysm was located in the internal carotid artery in two patients, the middle cerebral artery in two patients, the top of the basilar artery in one patient, and the vertebral artery-posterior inferior cerebellar artery (VA-PICA) in one patient. Three patients were treated using a Neuroform Atlas Stent system, one using an Enterprise2 VRD, one using two Neuroform Atlas stents for Y-stenting, and the remaining patient using a Neuroform Atlas and an Enterprise2 VRD for Y-stenting. With DSA, the postoperative aneurysm occlusion status was neck remnant (NR) in five cases and complete obliteration (CO) in one case. DSA and PETRA-MRA evaluations demonstrated an equal occlusion status in five of six cases, whereas DSA and TOF-MRA were equal in two of six cases. The mean visualization score for PETRA-MRA was 3.33 ± 0.82, whereas that for TOF-MRA was 2.17 ± 1.33. On the PETRA-MRA images, blood flow through the stent was well-visualized and produced an aneurysm occlusion status score comparable to DSA, especially in the three cases using the Neuroform Atlas Stent System where the visualization was scored 4 points. In the case of the VA-PICA aneurysm, for which an Enterprise2 VRD was used, PETRA-MRA images were insufficient for postoperative assessment. Conclusion: PETRA-MRA can provide good visualization of the blood flow within a stent and displays a clear blood signal near the coils, barring small magnetic susceptibility artifacts. Therefore, PETRA-MRA may be an effective option for follow-up imaging after stent-assisted coil embolization.

A novel needle electrode for intraoperative fourth cranial nerve neurophysiological mapping.

OBJECTIVES: Trochlear nerve (CN-IV) mapping method has not been confirmed to date. The compound muscle action potential (CMAP) of CN-IV cannot be recorded because of the low mapping sensitivity and anatomical characteristics of the superior oblique muscle (SOM). The aim of this study was to evaluate the effectiveness of a novel needle electrode (NNE), for the intraoperative mapping of CN-IV. MATERIALS AND METHODS: The NNEs were inserted in the target extraocular muscles in 19 patients. We compared the CMAP amplitude of the NNE with that of the conventional needle electrode (CNE). Furthermore, we investigated the dissimilarity between the CMAP of the CN-IV and other extraocular cranial nerves (ECNs) and the correlation between the readings of the CN-IV mapping and its postoperative functional outcome. RESULTS: The CMAP of CN-IV has been measured in nine patients (47.4%). The CMAP of CN-IV was distinguishable from other ECNs. The CMAP of the NNE was found to be three times higher than that of the CNE. Although the NNE has shown the potential to record the CN-IV's CMAP, 4 cases ended up having a CN-IV postoperative dysfunction. CONCLUSIONS: For the first time, we confirmed the possibility of intraoperative mapping the CN-IV using an NNE inserted into the SOM. The NNE can also be useful for other neurophysiological monitoring methods.

[Coexisting Symptomatic Adrenocorticotropic Hormone- and Growth Hormone-producing Double Pituitary Adenomas with Difficulty in Tumor Localization:A Case Report].

Double functional pituitary adenomas are rare, and only a few cases of excessive clinical symptoms of both adrenocorticotropic hormone(ACTH)and growth hormone(GH)have been reported. We herein report a case of symptomatic ACTH-and GH-producing double pituitary adenomas, which were discretely located within the same pituitary gland. A 38-year-old woman presented with general malaise, facial and lower limb edema, unexplained weight gain, facial redness, acne, and nasal enlargement. Endocrinological findings matched with the diagnostic criteria for both acromegaly and Cushing's disease. Preoperative magnetic resonance imaging showed a 15-mm cyst-like lesion on the right side of the sellae surrounded by what was thought to be the normal contrast-enhancing pituitary gland. We assumed that the cyst-like lesion was an adenoma and performed endoscopic endonasal transsphenoidal surgery. However, the cyst-like lesion was a parenchymal tumor. Furthermore, the region we considered to be a normal pituitary gland was also found to be an adenoma. Both adenomas were completely resected. The postoperative blood analysis showed ACTH<1.0pg/dL, cortisol 1.8µg/dL, and insulin-like growth factor-1 60ng/mL, all of which were below reference levels. The histopathological examination confirmed the coexistence of two adenomas, a GH-producing adenoma and an ACTH-producing adenoma. We concluded that these adenomas were endocrinologically active within the pituitary gland. Thus, a diagnosis of double pituitary adenomas was made. When treating a patient with symptoms caused by hypersecretion of multiple anterior pituitary hormones, the possibility of coexisting multiple pituitary adenomas should be considered.

[Repair of Spontaneous Cerebrospinal Fluid Leakage due to Multiple Defects in the Skull Base:A Case Report].

Spontaneous cerebrospinal fluid(CSF)rhinorrhea may have multiple leakage sites, and occasionally, may become difficult to manage. A 46-year-old man was admitted to our hospital presenting with bacterial meningitis that occurred after CSF rhinorrhea. The CSF leak had stopped following conservative medical treatment;however, it recurred immediately. The leakage was assumed to be in the left lateral recess of the sphenoid sinus based on the location of fluid accumulation on the CT and MR images and the nasal endoscopic findings. First, we performed an endoscopic endonasal repair of the CSF leakage sites. There were multiple defects in the roof of the sphenoid sinus, including the left lateral sphenoid recess. These bone defects were repaired with abdominal fat;however, this did not stop the CSF leak. Therefore, he underwent a craniotomy and nasal endoscopy, which were performed simultaneously using a galeal flap. The galeal flap was inserted from the middle of the skull base to the sphenoid sinus to cover the bone defects, and the position was adjusted under the guidance of nasal endoscopy. Moreover, intraoperatively, another meningocele with CSF leak was discovered in the cribriform plate and was repaired using abdominal fat. The CSF leak stopped after the second surgery. It is important to be mindful of possible multiple leakage sites when treating cases of idiopathic CSF leakage;moreover, the determination of the leakage sites can be difficult. A repair surgery using a galeal flap is preferable in cases of multiple CSF leakage sites, such as in this case, because it allows for all defects to be covered and repaired simultaneously.

Acute paradoxical brain herniation after decompressive craniectomy for severe traumatic brain injury: A case report.

BACKGROUND: Sinking skin flap syndrome or paradoxical brain herniation is an uncommon neurosurgical complication, which usually occurs in the chronic phase after decompressive craniectomy. We report a unique case presenting with these complications immediately after decompressive craniectomy for severe traumatic brain injury. CASE DESCRIPTION: A 65-year-old man had a right acute subdural hematoma (SDH), contusion of the right temporal lobe, and diffuse traumatic subarachnoid hemorrhage with midline shift to the left side. He underwent an emergency evacuation of the right SDH with a right decompressive frontotemporal craniectomy. Immediately after the operation, his neurological and computed tomography (CT) findings had improved. However, within 1 h after the surgery, his neurological signs deteriorated. An additional follow-up CT showed a marked midline shift to the left, i.e., paradoxical brain herniation, and his skin flap overlying the decompressive site was markedly sunken. We immediately performed an urgent cranioplasty with the right temporal lobectomy. He responded well to the procedure. We suspected that a cerebrospinal fluid leak had caused this phenomenon. CONCLUSION: Decompressive craniectomy for severe traumatic brain injury can lead to sinking skin flap syndrome and/or paradoxical brain herniation even in the acute phase. We believe that immediate cranioplasty allows the reversal of such neurosurgical complications.

De Novo Pineal Region Germinoma in the Seventh Decade of Life: A Case Report.

Germ cell tumors typically occur in children and adolescents. We here report a rare case of de novo pineal region germinoma in the seventh decade of life. A 62-year-old man presented with double vision. Computerized tomography and magnetic resonance imaging (MRI) identified a heterogeneously enhanced tumor with calcification in the pineal region with ventricular dilatation due to aqueduct stenosis. The tumor had not been observed at all on MRI obtained 2 years previously. The patient underwent endoscopic biopsy and third ventriculostomy for the obstructive hydrocephalus. The tumor was histopathologically diagnosed as a pure germinoma. The patient underwent radiomonotherapy, resulting in his complete remission, which was confirmed by a series of follow-up MRI studies and hematological examinations. Intracranial germinoma should be considered in the differential diagnosis of pineal region tumors regardless of age, even though the tumor was undetectable on the previous neuroimaging.

Role of transcranial sphenoidotomy in skull base surgery: classification of surgical techniques based on the surgical anatomy of the sphenoid sinus.

OBJECTIVEAlthough sphenoidotomy is more commonly performed via the transnasal approach than via the transcranial approach, transcranial sphenoidotomy (TCS) remains indispensable for en bloc resection of locally advanced sinonasal malignant tumors (SNMTs) extending to the skull base. TCS also enables transsphenoidal transposition of the temporoparietal galeal flap (TPGF) to compensate for the lack of vascularized reconstructive tissue after endoscopic transnasal skull base surgery. The objective of this study was to review the authors' surgical experience using TCS with an emphasis on the surgical anatomy of the sphenoid sinus and on the purpose of TCS. Relevant anatomy is further illustrated through cadaveric dissection and photo documentation.METHODSThe authors reviewed the records of 50 patients who underwent TCS at the Nagoya University Hospital, Fukushima Medical University Hospital, or Aichi Medical University Hospital over the course of 7 years (between January 2011 and November 2017). The authors also performed cadaveric dissection in 2 adult cadaveric skull base specimens.RESULTSOf the 50 patients included in this study, 44 underwent craniofacial resection (CFR) for en bloc resection of SNMTs involving the anterior and/or lateral skull base, and 6 underwent transsphenoidal transposition of the TPGF flap. The authors categorized the TCS procedures according to the portion of the sphenoid sinus wall involved (i.e., superior, lateral, and superolateral). Superior sphenoidotomy was used in patients requiring anterior CFR. Lateral sphenoidotomy was further divided into 2 subtypes, with type 1 procedures performed for the transsphenoidal transpositioning of the TPGF, and type 2 procedures used in patients requiring lateral CFR. Superolateral sphenoidotomy was used in anterolateral CFR.CONCLUSIONSTCS still represents a useful tool in the armamentarium of neurosurgeons treating central skull base lesions. The newly proposed surgical classification facilitates a profound understanding of TCS and how to incorporate this technique into clinical practice.

Multiple Surgical Treatments for Repeated Recurrence of Skull Base Mesenchymal Chondrosarcoma.

We report a case of a young male who received multiple surgical treatments for repeated recurrence of skull base mesenchymal chondrosarcoma (MC). When the patient was 18 years old, we subtotally removed the skull base MC and he was treated with stereotactic radiosurgery for remnant tumors in the left cavernous sinus. After 30 months, we removed residual tumors that had regrown partially, via combined endonasal endoscopic and orbitozygomatic approaches. Over the next 65 months, the patient refused radical resection, and received six salvage surgeries, two stereotactic radiotherapies, and five stereotactic radiosurgeries for repeated recurrence. At 95 months after initial surgery, the tumors had extended to the skull base and nasal cavities. As a result, the left eye had been blinded and right visual acuity was deteriorated. We performed left anterior-middle cranial base resection, removal of nasal and intradural tumors, high flow bypass, en-bloc resection of the left cavernous sinus and clivus, and reconstruction using an abdominal flap. Even though the main tumors were removed with safety margins, tumors around the right optic nerve were removed by piecemeal to preserve right eye function. Six months after the radical resection, tumors in the right orbital apex recurred because we had been unable to remove the tumor with adequate safety margins. Skull base MC has a high tendency to recur locally, so these tumors should be radically removed with safety margins as early as possible to prevent recurrence.

Palliative shunt surgery for patients with leptomeningeal metastasis.

OBJECTIVES: Leptomeningeal metastasis (LM) is associated with poor prognosis and affects the quality of life (QOL) of end-stage cancer patients. Severe headache associated with hydrocephalus causes reduced QOL. We investigated the clinical value of surgical treatment for hydrocephalus in LM patients. PATIENTS AND METHODS: The medical records of 11 consecutive patients who underwent lumboperitoneal shunt (LPS) or ventriculoperitoneal shunt (VPS) at our institution between 2007 and 2016 were investigated. Primary brain tumor patients were excluded. We assessed the neurological status and therapeutic effects at 1 month after the shunt surgery. RESULTS: The patients were three males and eight females with a median age of 58 years (interquartile range [IR] 52-68 years). The median preoperative neutrophil-to-lymphocyte ratio was 6.4 (IR 4.8-9.2). Symptom improvement was observed in nine patients, and severe headache was relieved in seven (88%) out of eight patients. The median Karnofsky performance status scale increased from 40 to 60, and the median overall survival after primary malignancy diagnosis was 27.4 months (IR 19.6-63.1 months). The median survival after the diagnosis of brain parenchymal metastasis, LM, and shunt surgery were 7.2 months (IR 5.1-14.1 months), 3.9 months (IR 3.5-6.3 months), and 3.3 months (IR 2.9-5.7 months), respectively. CONCLUSION: Shunt surgery for hydrocephalus could offer an effective palliative surgical option for symptom relief especially relief of severe headache, contributing improvement of QOL in LM patients.

Has acromegaly been diagnosed earlier?

PURPOSE: We investigated whether acromegaly has been diagnosed earlier at the Niigata Medical and Dental University Hospital. METHODS: Patients with acromegaly (n = 81) who underwent their first transsphenoidal surgery from 2006 to 2015 were reviewed. Two groups were compared: those who underwent surgery between 2006 and 2010 (n = 35) and those who underwent surgery between 2011 and 2015 (n = 46). We compared clinical features and serum levels of the growth hormone (GH) and insulin-like growth factor-1 (IGF-1), hypertension (HT) and diabetes mellitus (DM) prevalence between the two groups. RESULTS: Compared with the early group, microadenomas (<10 mm) were more prevalent in the late group (0% vs. 15.2%, p < .05). Serum IGF-1 standard deviation score (SDS) was significantly lower in the late group (8.57 ±â€¯2.50 vs. 6.44 ±â€¯2.30, p < .001). In both groups, mean IGF-1 SDS was significantly lower in patients without DM than in those with DM (6.9 ±â€¯2.6 vs. 8.3 ±â€¯2.4, p = .02). Logistic regression analysis showed that serum GH and IGF-1 levels were significantly higher in patients with DM than in those without DM. CONCLUSION: Regarding operated cases of GH-producing pituitary adenoma, acromegaly clinical manifestations tended to be milder at diagnosis in later years of the decade, and acromegaly was diagnosed at lower IGF-1 levels and in smaller lesions. Further study is mandatory for the generalization of this trend.

Jugular Foramen Collision Tumor (Schwannoma and Plasma Cell Pseudotumor), a Probable IgG4-Related Disease.

BACKGROUND: Lower cranial nerve sheath tumors are relatively rare. Cases of schwannoma collision tumors have rarely been reported, with most of the reported cases describing schwannoma and meningioma collision tumors. CASE DESCRIPTION: We report a very rare case of a cerebellopontine angle collision tumor of the ninth cranial nerve schwannoma with an IgG4 plasma cell pseudotumor. IgG4 plasma cell pseudotumors comprise a group of diseases called IgG4-related diseases (IgG4-RDs). These diseases usually affect organs such as the pancreas and salivary gland. Few cases of nervous system IgG4-RDs have been reported. Under intraoperative microscopy, the tumor in our case did not appear different from usual cases of schwannoma, but histopathology showed significant infiltration of IgG4 plasma cells. CONCLUSION: IgG4-RDs have a distinctive histopathologic pattern; however, their pathophysiology remains unclear. Special attention must be paid to the diagnosis of such diseases because they mimic other diseases and can be missed.

[Primary Pituitary Malignant Lymphoma that was Difficult to Differentiate from Nonfunctioning Pituitary Adenoma:A Case Report].

We report a rare case of primary pituitary lymphoma in a 75-year-old immunocompetent woman. The patient was blind in the right eye and presented with visual disturbance in the left eye that started 2 months previously. She also exhibited right third and fifth cranial nerve palsy. Magnetic resonance imaging(MRI)revealed an intrasellar mass lesion with right cavernous sinus invasion and suprasellar extension with compression of the optic chiasm. The mass lesion was isointense on both T1WI and T2WI, and showed less enhancement than a normal pituitary gland on gadolinium-enhanced T1WI. We therefore suspected the tumor to be a nonfunctioning pituitary adenoma. The patient underwent endoscopic endonasal transsphenoidal surgery. The tumor was firm and grayish, and had an ill-defined border along the normal pituitary gland. Histological examination revealed a malignant CD5-positive diffuse large B-cell lymphoma. After surgery, the patient received both chemotherapy and radiotherapy. Although the visual acuity of the right eye did not improved, other symptoms improved. At the 34-month follow-up, no recurrence was detected on serial MRI. Patients with primary pituitary lymphoma often exhibit ophthalmoplegia and/or panhypopituitarism more frequently than expected from radiological findings. In cases of pituitary tumors with atypical symptoms, a biopsy and general physical examination should be performed immediately to determine the diagnosis and perform adjuvant therapy even when the tumor is assumed as nonfunctioning pituitary adenoma from the image findings.

Long-term outcomes in patients with pineal nongerminomatous malignant germ cell tumors treated by radical resection during initial treatment combined with adjuvant therapy.

BACKGROUND: For pineal nongerminomatous malignant germ cell tumors (NGMGCTs), we mainly performed radical tumor resection during initial treatment combined with adjuvant therapy. METHODS: We retrospectively analyzed 17 patients treated for pineal NGMGCTs between 1986 and 2007 at the University of Niigata. RESULTS: Twelve patients underwent total or subtotal resection of their tumor via the occipital transtentorial approach. Five patients underwent partial resection, and four of them later underwent total resection by salvage surgery. After surgery, eight patients were treated with combined radiochemotherapy including whole-brain irradiation, two received radiation monotherapy, one had chemotherapy with local irradiation, and six were treated with chemotherapy alone. The median follow-up period for surviving patients was 179 months. The 10-year overall survival and progression-free survival rates for the radiochemotherapy group were both 75.0 % (two patients had a recurrence and died); the rates for other adjuvant therapies were 77.8 % (two died) and 22.2 % (seven had a recurrence), respectively. Radiochemotherapy was significantly associated with an increased rate of progression-free survival compared with the other adjuvant therapies (p = 0.0396). CONCLUSIONS: For pineal NGMGCTs, initial treatment strategies including gross total resection of the tumor before or after whole-brain irradiation and chemotherapy provided good therapeutic outcomes. Obtaining complete remission of the primary tumor, irrespective of the timing of surgical resection (i.e., before or after adjuvant therapies), or complete response by neoadjuvant radiochemotherapy during an initial treatment appears to be essential for improving therapeutic outcomes of intracranial NGMGCTs.