Exacerbation of restless legs syndrome following amygdalohippocampectomy: A case report.

Background: Restless legs syndrome (RLS) is a neurological sensorimotor disorder characterized by an uncontrollable urge to move the legs. In the perioperative period, patients with RLS may experience an acute exacerbation of symptoms. Although studies on the exacerbation of RLS after brain surgery are limited, we present a case wherein symptoms worsened following left amygdalohippocampectomy. Case Presentation: A 58-year-old woman diagnosed with mesiotemporal lobe epilepsy accompanied by left hippocampal sclerosis underwent a left amygdalohippocampectomy. The patient reported uncomfortable sensations in the lower limbs preoperatively. However, the urge to move her legs was manageable and not distinctly diagnosed with RLS. The symptoms began to deteriorate on the fifth postoperative day primarily affecting the legs and back, with a notable emphasis on the right side. Pramipexole treatment effectively ameliorated these symptoms. Conclusion: No reports are available highlighting the exacerbation of RLS after amygdalohippocampectomy. Perioperative factors, such as anesthesia and iron deficiency due to hemorrhage, have been proposed as aggravating factors for RLS; however, the asymmetry of RLS, particularly the atypical right-sided exacerbation in this case, makes it unlikely that this was the primary cause. A negative correlation between opioid receptor availability in the amygdala and RLS severity has been reported, suggesting that amygdalohippocampectomy contributes to the exacerbation of RLS symptoms. This case provides valuable insights into the possible involvement of the amygdala in the pathophysiology of RLS and practical considerations for the clinical management of the condition.

Rare vermian pilocytic astrocytoma with recurrent spontaneous hemorrhage in the elderly: A case report and review of literature.

Background: Pilocytic astrocytoma (PA) is a benign glial tumor predominately seen in pediatrics and early adolescence with associated overall good outcomes. Very few cases of elderly PA have been reported in the literature, and they are known to display unique anatomic, histologic, and genetic peculiarities distinct from pediatric disease. We report a rare case of vermian PA in an octogenarian with recurrent spontaneous intratumoral hemorrhage as a presenting symptom. Furthermore, a review of the literature on the peculiarities of PA in the elderly will be discussed. Case Description: An 81-year-old woman presented with features suggestive of repeated posterior fossa hemorrhages characterized by headaches, diplopia, and alteration in sensorium occurring about 5 months apart. Brain neuroimaging showed a cerebellar vermian tumor with features suggestive of repeated intratumoral bleeding. She had an initial ventriculoperitoneal shunting for acute hydrocephalus and subsequently had a suboccipital craniotomy and subtotal tumor excision due to morbid adherence to the brainstem. The histologic diagnosis was PA with Ki-67 <1% and negative for isocitrate dehydrogenase-1. There was a slow but progressive clinical improvement, and she has remained symptom-free for 4 years on follow-up. Conclusion: PA in the elderly is a rare disease with distinct histologic and genetic peculiarities. This case review showed one of the oldest cases of cerebellar vermian PA presenting with recurrent spontaneous intratumoral hemorrhage, an extremely rare occurrence in benign glioma. Although complete surgical excision is recommended, partial resection is advocated for morbidly adherent tumors. Overall prognosis is worse in elderly PA.

The Importance of Intratumoral Venous Drainage Preservation in Two-Stage Surgery of Large Hypervascular Choroid Plexus Papilloma: A Case Report.

Two-stage surgery may be necessary when total tumor removal cannot be accomplished in the first surgery; however, the extent and condition in which the remaining tumor should be before the next surgery have not yet been established. There is a risk of postoperative hemorrhage in the residual tumor, especially in hypervascular tumors. We report a case of hypervascular choroid plexus papilloma (CPP) in a 22-year-old male patient where the preservation of intratumoral venous drainage was considered important to avoid hemorrhagic complications during a two-stage surgery. In the first surgery, it was difficult to control the bleeding from the debulked tumor, and the surgery was terminated due to severe blood loss. Large draining veins running in the tumor were preserved as it was suspected that these were important drainage routes of the bloodstream of the tumor. The preserved draining red veins changed to normal venous color in the second surgery performed after one week. The residual tumor was not vascularized during the second surgery and underwent gross total resection with less blood loss. The patient was discharged without sequelae. There was no recurrence of the tumor and no neurological deficit during the three-year follow-up. To prevent postoperative hemorrhage associated with a residual tumor, it may be important to preserve venous drainage of the tumor in hypervascular tumor resection.

Characteristics of an advanced epilepsy treatment gap in a region in Japan.

To investigate the quality of epilepsy care in a region in Japan that lacked specialised care, we retrospectively evaluated patients who visited our newly established epilepsy division between April 2018 and March 2021, and had been treated with anti-seizure medications (ASMs) for at least 1 year prior. Of the 231 patients included, 169 had ongoing seizure episodes at first visit (seizure-persist group) and 62 had no seizure episodes for more than a year (seizure-free group). Eighty-three patients in the seizure-persist group had not received specialised epilepsy care, 15 had been treated with unnecessary medications, and seven had experienced side effects from ASMs. Twelve patients in the seizure-free group had been treated with unnecessary ASMs, 10 had been treated with ASMs with teratogenic potential and four had experienced ASM side effects. These patients could be classified as having an advanced epilepsy treatment gap (ETG) because they had not previously received necessary specialised care. The progressive decline in the number of patients with advanced ETG suggests that our new epilepsy division has addressed this issue. This study highlights that a significant number of patients with advanced ETGs exist in Japan and that proper countermeasures are required to address this gap.

Postoperative Intratumoral or Peritumoral Hematomas After Vestibular Schwannoma Resection.

BACKGROUND: Surgical removal of a vestibular schwannoma is a complex and challenging procedure, which may be complicated by development of postoperative hematomas, particularly after incomplete resection of the tumor. OBJECTIVE: To investigate the occurrence of postoperative intra- or peritumoral hematomas after surgery for a vestibular schwannoma. METHODS: This retrospective study evaluated 49 patients (age range 17-78 years) with a vestibular schwannoma, who were treated surgically via the lateral suboccipital approach between 2011 and 2016. The tumors ranged in size from 0 mm (in a case of an intracanalicular lesion) to 56 mm. In 30 cases (61%), total or near-total resection was accomplished, and in 19 cases (39%), subtotal or partial resection was done. On the basis of their bleeding tendency during tumor removal, the patients were divided into a "less-bleeding" (38 cases; 78%) and a "more-bleeding" (11 cases; 22%) subgroups. RESULTS: A maximal vestibular schwannoma diameter >30 mm, patient age >60 years, and more bleeding during tumor removal were significantly associated with incomplete (subtotal or partial) resection. In six cases (12%), serial computed tomography after surgery demonstrated a postoperative hematoma, which was caused by insufficient irrigation of the surgical field (in two cases) or resulted from peritumoral hemorrhage (in two cases), intratumoral hemorrhage (in one case), or both intra- and peritumoral hemorrhage (in one case). The latter patient required urgent reoperation. In all cases, postoperative hematomas occurred after incomplete (subtotal or partial) resection of a vestibular schwannoma, and their development was significantly associated with more bleeding during tumor removal. CONCLUSION: For avoidance of postoperative hematomas, careful hemostasis is required after completion of vestibular schwannoma removal, especially in cases with incomplete resection and an excessive bleeding tendency of the tumor tissue.

Unexpected recovery from complete deafness to normal hearing post surgical excision of a cerebellopontine angle meningioma: A case report.

Background: Cerebellopontine angle (CPA) meningioma presents a significant management challenge due to its intricate relationship with the brainstem neurovascular bundles. The emphasis in the past has been on facial nerve preservation, but the current management standard is hearing preservation in patients with serviceable hearing; however, hearing restoration after complete loss is rare. We report an elderly man who had restoration of hearing in the right ear after complete loss following tumor resection through the retrosigmoid route. Case Description: A 73-year-old male patient presented with progressive hearing impairment in the right ear, culminating in hearing loss for about 2 months (the American Academy of Otolaryngology-Head and Neck Surgery [AAO-HNS] class D). He also had mild cerebellar symptoms, but other cranial nerves and long tracts were normal. Brain magnetic resonance imaging confirmed a right CPA meningioma, and he had tumor resection through the retrosigmoid route using meticulous microsurgical technique with vestibulocochlear nerve preservation, facial nerve monitoring, and intraoperative video angiography. He had restoration of hearing on follow-up (the American Academy of Otolaryngology-Head and Neck Surgery class A). Histology confirmed World Health Organization central nervous system grade 1 meningioma. Conclusion: This case illustrates that hearing restoration is possible after complete loss in patients with CPA meningioma. We advocate hearing preservation surgery even in patients with non-serviceable hearing, as the chance of hearing recovery is possible.

Feasibility and limitations of head MRI in patients with cochlear implants.

OBJECTIVE: Cochlear implants (CIs) were noncompatible with magnetic resonance imaging (MRI) initially; however, recently, implants have become available that are compatible with MRI without the need for magnet removal or bandage fixation. The images produced by MRI scans are sometimes deteriorated by artifacts and are not clinically useful. In this study, we discussed the size differences of such artifacts with respect to the imaging modality and sequences with their clinical validity. METHODS: We performed a head MRI, using a head bandage and without magnet removal in five patients who underwent cochlear implantation at our department and analyzed the MRI findings. RESULTS: Without magnet removal, diffusion-weighted images and T2 star-weighted images had larger artifacts and less useful images. T1-weighted images, T2-weighted images (T2WIs), T2-weighted fluid-attenuated inversion recovery (T2-FLAIR) images, and heavy T2WIs could evaluate the unimplanted side and middle of the head but had limited applicability on the CI side. CONCLUSION: The characteristic features of MRI scan images vary with the method used as well as with the sequence, suggesting that the choice of MRI is largely determined on the basis of clinical feasibility and the requirement. Accordingly, we need to judge well in advance of imaging whether the images would be clinically relevant.

Frontal Encephalocele Plus Epilepsy: A Case Report and Review of the Literature.

An encephalocele is a pathological brain herniation caused by osseous dural defects. Encephaloceles are known to be regions of epileptogenic foci. We describe the case of a 44-year-old woman with refractory epilepsy associated with a frontal skull base encephalocele. Epilepsy surgery for encephalocele resection was performed; however, the epilepsy was refractory. A second epilepsy surgery for frontal lobectomy using intraoperative electroencephalography was required to achieve adequate seizure control. Previous reports have shown that only encephalocele resection can result in good seizure control, and refractory epilepsy due to frontal lobe encephalocele has rarely been reported. To the best of our knowledge, this is the first report of frontal encephalocele plus epilepsy in which good seizure control using only encephalocele resection was difficult to achieve. Herein, we describe the possible mechanisms of encephalocele plus epilepsy and the surgical strategy for refractory epilepsy with encephalocele, including a literature review.

Postoperative hematoma can be a risk factor in delayed facial palsy after vestibular schwannoma resection via a retrosigmoid approach: a retrospective single-center cohort study.

PURPOSE: Delayed facial palsy (DFP) is a rare postoperative complication after vestibular schwannoma (VS) surgery. The exact mechanism of DFP remains uncertain and the risk factors for DFP are still controversial. The authors aimed to investigate the characteristics, risk factors, and etiology of DFP after VS resection retrospectively. METHODS: Ninety-one consecutive surgeries with VS were analyzed. Patients with neurofibromatosis 2 in eight surgeries and postoperative facial palsy House-Brackmann (HB) grade more than 3 in two surgeries were excluded. Eighty-one surgeries were included in this research. Facial nerve function was evaluated using the HB grade. Delayed facial palsy was defined as deterioration in the facial function of at least 1 HB grade more than 1 day after undergoing VS resection. The characteristics of patients with VS and risk factors for DFP were analyzed. RESULTS: All surgeries were performed via a retrosigmoid approach. DFP was observed in nine patients. There were no statistically significant differences between the DFP group and non-DFP group in terms of the following characteristics: sex, age, side, size, Koos grading system, postoperative facial palsy, or extent of resection. Postoperative hematoma in the cerebellopontine angle (CPA) cistern was significantly higher in the DFP group than in the non-DFP group (p = 0.0023), and was significantly associated with DFP after VS surgery (odds ratio 18.40, p < 0.001). DFP improved in seven patients, but two patients did not improve. CONCLUSION: DFP occurred in 11.1% of patients after VS surgery. This study revealed that postoperative hematoma in the CPA cistern was significantly associated with DFP after VS surgery.

[Brain Abscess].

Brain abscesses can be caused by bacteria, mycobacteria, fungi, or parasites. The incidence of brain abscess varies from 0.4 to 0.9 cases per 100,000 population. The rate of infection increases in immunosuppressed patients. Approximately 25% of brain abscesses occur in children. Outcomes for patients with brain abscesses have improved over the past 50 years. Despite the advantages in diagnostics and treatment leading to improved outcomes in patients with brain abscess, the overall outcome and quality of life issues in patients with brain abscess remain a challenge in neurosurgery. In this article, the etiology, pathogenesis, diagnostic imaging, and treatment of brain abscesses are discussed.

The Occurrence and Relationship of Postoperative Seizure and de novo Epilepsy after Craniotomy Surgery: A Retrospective Single-Center Cohort Study.

Objective: Postoperative seizures and epilepsy are common complications of craniotomy. In this study, we aimed to investigate the characteristics of seizures and epilepsy after craniotomy. Methods: A total of 293 consecutive craniotomy surgeries were analyzed. Infratentorial surgeries, epilepsy surgeries, surgeries using the same approach conducted for the same patients, and the cases with incomplete clinical data were excluded. A total of 211 surgeries were included in this study. We evaluated the following clinical characteristics in all patients: sex, age, preoperative epilepsy, use of preoperative antiseizure medication (ASM), indication for operation, early postoperative seizure (EPS), delayed postoperative seizure (DPS), and postoperative de novo epilepsy. The day of onset of EPSs was defined as within 7 days post-surgery, and the day of onset of DPSs was defined as later than 7 days and less than 60 days post-surgery. Results: Twenty-eight patients were previously diagnosed with epilepsy. Nine patients had EPSs (4.3%), and 10 patients had DPSs (4.7%). Seven cases of EPSs and six cases of DPSs were observed in 183 patients without previous epilepsy (3.8% and 3.3%, respectively). Three of the seven patients with EPSs (42.9%) and all six patients with DPSs (100%) developed de novo epilepsy. Postoperative de novo epilepsy was observed in 9 (4.9%) of the 183 patients without epilepsy. EPSs and DPSs were significant risk factors for epilepsy (p < 0.01). The odds ratios of EPSs and DPSs for the development of epilepsy were 12.71 (95% confidence interval [CI]: 3.94-112.80; p < 0.01) and 22.88 (95% CI: 5.38-55.72; p < 0.01), respectively. ASM was administered prophylactically to 51 patients. The prophylactic use of ASMs did not prevent EPSs or postoperative de novo epilepsy. Conclusion: EPSs and DPSs occurred in 4.3% and 4.7% of the patients, respectively, after craniotomy. Postoperative de novo epilepsy occurred in 4.9% of patients. This study revealed that EPSs and DPSs were risk factors for de novo epilepsy. Previous epilepsy was not a significant risk factor for EPSs. The prophylactic use of ASMs did not prevent EPSs or de novo epilepsy.

Necessity for craniospinal irradiation of germinoma with positive cytology without spinal lesion on MR imaging-A controversy.

BACKGROUND: Cerebrospinal fluid (CSF) cytology and spinal MR imaging are routinely performed for staging before treatment of intracranial germinoma. However, the interpretation of the results of CSF cytology poses 2 unresolved clinical questions: (1) Does positive CSF cytology correlate with the presence of spinal lesion before treatment? and (2) Is craniospinal irradiation (CSI) necessary for patients with positive CSF cytology in the absence of spinal lesion? METHODS: Multicenter retrospective analyses were performed based on a questionnaire on clinical features, spinal MR imaging finding, results of CSF cytology, treatments, and outcomes which was sent to 86 neurosurgical and 35 pediatrics departments in Japan. Pretreatment frequencies of spinal lesion on MR imaging were compared between the patients with positive and negative cytology. Progression-free survival (PFS) rates were compared between patients with positive CSF cytology without spinal lesion on MR imaging treated with CSI and with whole brain or whole ventricular irradiation (non-CSI). RESULTS: A total of 92 germinoma patients from 45 institutes were evaluated by both CSF cytology and spinal MR images, but 26 patients were excluded because of tumor markers, the timing of CSF sampling or incomplete estimation of spinal lesion. Of the remaining 66 germinoma patients, spinal lesions were equally identified in patients with negative CSF cytology and positive cytology (4.9% and 8.0%, respectively). Eleven patients treated with non-CSI had excellent PFS comparable to 11 patients treated with CSI. CONCLUSION: CSI is unnecessary for germinoma patients with positive CSF cytology without spinal lesions on MR imaging.

The Efficacy of Intraoperative Passive Language Mapping for Glioma Surgery: A Case Report.

Background: Recently, electrocorticographic (ECoG) studies have emphasized the importance of gamma band-based functional mapping in the presurgical localization of the eloquent cortex. Passive functional mapping using ECoG signals provides a reliable method for identifying receptive language areas without many of the risks and limitations associated with electrical cortical stimulation. We report a surgical case of left temporal malignant glioma with intraoperative passive language mapping. Case Description: A 78-year-old woman was diagnosed with left temporal glioma with inspection of her language difficulty. MRI showed a left temporal tumor measuring 74.6 × 50.0 × 51.5 mm in size. Real-time CortiQ-based mapping using high-gamma activity by word-listening and story-listening tasks was performed. Significant listening task-evoked high gamma activities were detected in 5 channels in the superior temporal gyrus and one channel in the middle temporal gyrus. The tumor was grossly removed except for the region corresponding to listening task-evoked high gamma activities. Postoperatively, the patient's symptoms of language comprehension difficulty improved, and no new neurological deficits were observed. Conclusion: Intraoperative passive language mapping was successfully performed, and the patient's language function was well-preserved. Intraoperative passive language mapping, which is applicable in a short time and under general anesthesia, can be an important tool for detecting language areas.

Microscopic Navigation-Guided Fence Post Technique for Maximal Tumor Resection During Glioma Surgery.

BACKGROUND: The fence post technique, which involves insertion of catheters as fence posts around a tumor, has been widely used to demarcate the tumor border for maximal resection of intraparenchymal tumors, such as gliomas. However, a standard procedure for fence post insertion has not been established, and there are some limitations. To overcome this problem, a simple microscopic navigation-guided fence post technique was developed. The feasibility and efficacy of this novel technique during glioma surgery were assessed. METHODS: The microscopic navigation-guided fence post technique was used in 46 glioma surgeries performed in 42 patients. Intraoperatively, the preplanned trajectory was overlaid on the microscopic surgical field, and the microscope angle was changed until the entry and target points of the trajectory overlapped. A fence post catheter was inserted as planned under microscopic view, and the tumor was resected with fence post guidance. Preoperative tumor characteristics and surgical outcomes were evaluated. RESULTS: Mean age of patients was 50 years (range, 16-78 years), and 19 (45%) of 42 patients were women. Maximal safe resection was successfully achieved in 45 surgeries (97.8%), which was planned preoperatively with identification of the tumor border with fence posts without adverse effects of brain shift. No surgical complications attributable to fence post insertion occurred. CONCLUSIONS: Clinical experience indicated that the microscopic navigation-guided fence post technique, in which fence posts can be placed without requiring the surgeon to take their eyes off the microscope, is safe and useful in glioma surgery.

Aggressive growing of the infantile cavernous hemangioma of the calvaria: a case report and review of literature.

INTRODUCTION: Primary intraosseous cavernous hemangiomas of the skull are very rare in the pediatric age group and usually slow-growing tumors. CASE REPORT: We present a case of 5-month-old girl with a left occipital cavernous hemangioma that is rapidly growing. The subcutaneous occipital tiny mass was first noted at birth, and the lesion became rapidly enlarged in size and became soft for 3 months. The left occipital subcutaneous lesion was 4.0 × 4.0 × 2.0 cm (AP × LR × HT) in size. There was no history of trauma or bone tumor in her family. She underwent resection of the lesion, and a pathologic diagnosis of calvarial cavernous hemangioma was made. No recurrence was seen 1 year after surgery. CONCLUSION: The rapid growth of the infant cavernous hemangioma might be related to not only bleeding and/or congestion of the lesion but the immature thin skull of the infant.

Intraoperative Lumbar Muscle Motor Evoked Potential Monitoring With Transcortical Stimulation.

BACKGROUND: Stimulating electrodes for lower extremity motor-evoked potential (LE-MEP) monitoring with transcortical stimulation are usually placed on the medial side of motor cortex convexity, which is not lower extremity but lumbar motor area. Lumbar MEP may be elicited with lower stimulation intensity than LE-MEP through this location, and it is useful to monitor lower extremity motor function intraoperatively. METHODS: Intraoperative lumbar and LE-MEP monitoring with transcortical stimulation during surgery of 12 patients with lesions involving the motor cortex from January 2012 to February 2019 at Shinshu University Hospital were reviewed retrospectively. Stimulations were delivered by a train of 5 pulses of anodal constant current stimulation. Stimulating electrode position was determined by motor cortex mapping. Recording needle electrodes were placed on bilateral lumbar muscles and contralateral leg muscles. The threshold-level stimulation method was used for MEP monitoring. The thresholds, monitoring result, and postoperative motor function of lumbar and lower extremities were compared. RESULTS: The mean baseline thresholds were 19.9 ± 8.9 mA for lumbar MEP and 26.5 ± 11.5 mA for LE-MEP (P = 0.02). Patterns of intraoperative monitoring changes were the same between lumbar and LE-MEP monitoring. CONCLUSIONS: Lumbar MEP was stimulated with lower stimulation intensity than the LE-MEP with the same intraoperative pattern of waveform changes in 12 patients. Lumbar MEP monitoring may be useful for preserving the corticospinal tract of lower extremities intraoperatively.

Supratentorial Intraparenchymal Neurenteric Cyst Treated by Neuroendoscopic Fenestration: A Case Report and Review of Literature.

Neurenteric cysts (NCs) are rare benign congenital neoplasms in the central nervous system that originate from endodermal elements. NCs are more commonly located in the spine than in the brain. Although almost all intracranial NCs are found in the posterior fossa, some have reported supratentorial NCs. The complete excision of the cyst wall is suggested as a curative treatment; however, endoscopic treatment is less discussed. We present a supratentorial intraparenchymal NC in the frontal lobe treated by neuroendoscopic fenestration and review the literature regarding supratentorial NCs. A 43-year-old woman presenting with right hemiparesis and gait disturbance who was found to have a huge cystic lesion with calcification in her left frontal lobe underwent endoscopic fenestration to the ipsilateral lateral ventricle and biopsy. The histopathological diagnosis was consistent with NC. Postoperatively, her right hemiparesis and gait disturbance disappeared. Postoperative MRI showed shrinkage of the cyst. She was discharged without neurological deficits and no recurrence was seen 1 year after surgery. To the best of our knowledge, there have been no reports of a supratentorial intraparenchymal NC treated by neuroendoscopic fenestration. Minimally invasive treatments, such as neuroendoscopic cyst fenestration, can be considered depending on the location of the cyst.

So-called bifocal tumors with diabetes insipidus and negative tumor markers: are they all germinoma?

BACKGROUND: The Delphi consensus statements on the management of germ cell tumors (GCTs) failed to reach agreements on the statement that the cases with (i) pineal and neurohypophyseal bifocal lesion, (ii) with diabetes insipidus, and (iii) with negative tumor markers can be diagnosed as germinoma without histological verification. To answer this, multicenter retrospective analysis was performed. METHODS: A questionnaire on clinical findings, histological diagnosis, and details of surgical procedures was sent to 86 neurosurgical and 35 pediatrics departments in Japan. RESULTS: Fifty-one institutes reported 132 cases that fulfilled the 3 criteria. Tissue sampling was performed in 91 cases from pineal (n = 44), neurohypophyseal (n = 32), both (n = 6), and distant (n = 9) lesions. Histological diagnosis was established in 89 cases: pure germinoma or germinoma with syncytiotrophoblastic giant cells in 82 (92.1%) cases, germinoma and mature teratoma in 2 cases, and granulomatous inflammation in 2 cases. Histological diagnosis was not established in 2 cases. Although no tumors other than GCTs were identified, 3 (3.4%) patients had non-germinomatous GCTs (NGGCTs). None of the patients developed permanent complications after endoscopic or stereotactic biopsy. Thirty-nine patients underwent simultaneous procedure for acute hydrocephalus without permanent complications, and hydrocephalus was controlled in 94.9% of them. CONCLUSION: All patients who fulfilled the 3 criteria had GCTs or granulomatous inflammation, but not other types of tumors. However, no fewer than 3.4% of the patients had NGGCTs. Considering the safety and the effects of simultaneous procedures for acute hydrocephalus, biopsy was recommended in such patients.

Primary myxoid temporal bone tumor: A rare neurosurgical manifestation of Carney complex?

BACKGROUND: Carney complex (CNC) is a rare autosomal dominant syndrome, manifesting mainly with cardiac, cutaneous, and mucosal myxomas. Osteochondromyxoma is known as an extremely rare bone lesion of CNC which usually appears early in life; however, there were no reports of primary bone myxoma of the skull in the patients with CNC. We present the first case of primary myxoid skull tumor in the patient with CNC. CASE DESCRIPTION: We report the left temporal bone tumor with significant intracranial mass effect in a 58-year- old woman already diagnosed with CNC. Complete resection of the tumor with skull bone reconstruction was carried out. Pathological diagnosis was labeled the lesion as an atypical myxoid spindle cell neoplasm. The features were different from atrial myxoma and osteochondromyxoma which has been described in CNC. There have been no signs of recurrence in 9 years follow-up. CONCLUSION: To the best of our knowledge, there have been no reports of the primary myxoid tumors in the skull in the patients with CNC. This paper highlighted a possible important association between CNC and primary intracranial myxoid tumors.