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Acute brainstem compression by intratumoral hemorrhages in an intracranial hypoglossal schwannoma
Abstract
A 77-year-old female in the hospital was found tachycardic and hypothermic by a nurse, and the patient's respiration subsequently ceased. Forensic autopsy revealed an intracranial cystic tumor that would have compressed the brainstem. On microscopic examination, the tumor was diagnosed as an Antoni A schwannoma growth, and recent multiple intratumoral hemorrhages in the intracranial schwannoma were observed, suggesting the sudden enlargement of the intracranial schwannoma due to intratumoral hemorrhaging. Accordingly, we diagnosed the cause of death as brainstem compression induced by the intratumoral hemorrhaging in the intracranial schwannoma. Meanwhile, a rhinopharyngeal tumor was also detected by the autopsy, which was compatible with an antemortem diagnosis of a dumbbell-shaped hypoglossal schwannoma.
... In almost all cases, there are varying degrees of paresis of the HyN, which appear as ipsilateral deviation, hemiatrophy and fasciculations of the tongue. These symptoms may be accompanied by hoarseness, intermittent-to-persistent headaches, dysarthria, slurred speech, swallowing difficulties and throat cramps (2,4,(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28). Whereas the effects on the specific functions of the nerve may be attributable to the local damage of the tumour on the nerve, these types of lesions, particularly when they increase in volume, may determine mass effects on surrounding or adjacent structures; for example, Mariniello et al (19) reported on the case of a patient who exhibited signs of cerebellar ataxia, right-sided hypotonia and increased intracranial pressure. ...
... Independently from the nerve that is affected, surgical treatment consists of systematic exeresis of the lesions. This has been furthermore confirmed by the experience of the present authors and by case reports (2,(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)30,31). In fact, all except one of the cases were treated surgically. ...
Schwannomas are benign tumours that originate
from the myelin sheath of peripheral nerves. They are
characterised by a slow growth tendency. Benign schwannomas
represent 35% of the head and neck district tumours.
Hypoglossal schwannomas account for 5% of non‑vestibular
schwannomas, and malignant schwannomas occur very
rarely. In the present case report, the case of a 49‑year‑old
man who presented with paraesthesias in the left parotid and
submandibular region, associated with sensation of foreign
bodies and dysphagia for solids, is described. A clinical
examination revealed the presence of an ovoid palpable mass
in the lateral‑cervical region of the neck. The patient subsequently
underwent excisional surgery, and neuropathological
evaluation of the specimen confirmed the diagnosis of benign
schwannoma with Antoni areas A and B. Despite the rarity
of schwannomas, this condition should be considered in
differential diagnoses for masses localised in the neck, as in
cases where they reach considerable sizes (>3 cm in diameter).
Surgery therefore represents the first‑choice treatment.
... schwannoma j attenuated bacterial vaccines j Salmonella typhimurium j checkpoint blockade Schwannomas are slow-growing benign neoplasms derived from Schwann-lineage cells that typically first appear in childhood and adolescence (1). Depending on location and size, these tumors can cause a variety of gain-and loss-of-function neurological deficits including hearing loss, imbalance, tinnitus, motor loss, severe pain (2), and in some cases, fatal brainstem compression (3). Schwannomas may arise sporadically ("sporadic schwannoma") or from the debilitating genetic syndromes neurofibromatosis type 2 (NF2) and schwannomatosis (4). ...
Schwannomas are slow-growing benign neoplasms that develop throughout the body causing pain, sensory/motor dysfunction, and death. Because bacterial immunotherapy has been used in the treatment of some malignant neoplasms, we evaluated attenuated Salmonella typhimurium strains as immunotherapies for benign murine schwannomas. Several bacterial strains were tested, including VNP20009, a highly attenuated strain that was previously shown to be safe in human subjects with advanced malignant neoplasms, and a VNP20009 mutant that was altered in motility and other properties that included adherence and invasion of cultured mammalian cells. VNP20009 controlled tumor growth in two murine schwannoma models and induced changes in cytokine and immune effector cell profiles that were consistent with induction of enhanced innate and adaptive host immune responses compared with controls. Intratumoral (i.t.) injection of S. typhimurium led to tumor cell apoptosis, decreased tumor angiogenesis, and lower growth of the injected schwannoma tumors. Invasive VNP20009 was significantly more efficacious than was a noninvasive derivative in controlling the growth of injected tumors. Bacterial treatment apparently induced systemic antitumor immunity in that the growth of rechallenge schwannomas implanted following primary bacterial treatment was also reduced. Checkpoint programmed death-1 (PD-1) blockade induced by systemic administration of anti-PD-1 antibodies controlled tumor growth to the same degree as i.t. injection of S. typhimurium, and together, these two therapies had an additive effect on suppressing schwannoma growth. These experiments represent validation of a bacterial therapy for a benign neoplasm and support development of S. typhimurium VNP20009, potentially in combination with PD-1 inhibition, as a schwannoma immunotherapy.
... In addition, tumour metabolites and constriction of the spinal cord can induce increased permeability of the blood vessels to the extent that the protein components increase, which blocks arachnoid granulation and further progresses into reactive basicranial arachnoiditis, resulting in a barrier to cerebrospinal fluid circulation [16,17,20,21]. Another report in 2013 described a patient who died of brain stem compression induced by intratumoural haemorrhaging within an intracranial schwannoma, showing that brain stem compression can result in respiratory disturbances [22]. The definite diagnosis and location make it goal-directed to find the cause, while the herein-reported cases, for which this information was unavailable, prove the importance of spinal cord examination in forensic practice. ...
Intramedullary schwannoma of the upper cervical spinal cord is rarely reported in forensic medicine. We herein report a case involving a patient who died of compression from an intramedullary schwannoma in the upper cervical spinal cord. A 30-year-old man initially presented with a five-day history of pain in the left chest that progressed to weakening in the left arm. Although the patient was treated with analgesic poultices, he developed inspiratory dyspnoea and died while working the next day without having undergone any medical imaging examination or surgical treatment. Anatomical and histopathological examinations revealed an intramedullary schwannoma in the left cervical spinal cord (C3–C5) underneath the spinal nerve root. The cause of death might have been asphyxia secondary to the tumour, which interfered with the nerve function in the respiratory muscles. This finding suggests that an autopsy is essential for pathologists and medicolegists to comprehensively undertake their due obligation to obtain “the first evidence”, especially when there is a lack of directly related evidence. As part of the central nervous system, the spinal cord could be systematically included in a routine pathological autopsy in some cases.
... Magnetic resonance imaging (MRI) is considered superior to computed tomography for the diagnosis of skull base tumors, as MRI accurately demonstrates the relationship between tumor location and the surrounding soft tissues (4). Although 39 cases of dumbbell-shaped, hypoglossal schwannoma have been described in the literature to date (3,(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15), only a small proportion of these cases (<30%) achieved a complete tumor resection (12), and no recurrent cases were reported. At present, the standard surgical technique used for the treatment of hypoglossal schwannomas is the far lateral approach with partial resection of the condyle, which exposes the hypoglossal canal (3,16). ...
Dumbbell-shaped hypoglossal Schwannomas of the 12th cranial nerve are extremely rare, and complete removal of these tumors is difficult, particularly in elderly patients with recurrent tumors. The present study reports the case of a 61-year-old male with a giant recurrent dumbbell-shaped hypoglossal schwannoma that arose extracranially. The recurrent tumor was completely removed in a one-stage surgical procedure via the far lateral suboccipital approach in combination with the transcervical approach. To the best of our knowledge, such a lesion has not been reported previously. The life expectancy and natural course of the disease are important factors to take into account when considering the individual end-point of surgery in patients. More studies on hypoglossal schwannomas are required, particularly cases in which the hypoglossal schwannoma was not totally resected, not only in order to develop more definitive and secure surgical treatments, but also to reduce the resultant unnecessary suffering of patients.
Extensive large dumbbell-shaped hypoglossal schwannoma is extremely rare, and total resection is nearly impossible. We present a case of a 61-year-old male with a giant-size hypoglossal schwannoma with moderate tongue atrophy. The tumor extended from the enlarged hypoglossal canal to the brainstem intradurally and the high cervical region extradurally. Through the extreme lateral infrajugular transcondylar (ELITE) skull base approach, the tumor was totally removed in a single-stage operation. Single-stage total resection is feasible by an experienced skull base team utilizing transcondylar skull base techniques and high cervical dissection.
Central neural circuits orchestrate a homeostatic repertoire to maintain body temperature during environmental temperature challenges and to alter body temperature during the inflammatory response. This review summarizes the functional organization of the neural pathways through which cutaneous thermal receptors alter thermoregulatory effectors: the cutaneous circulation for heat loss, the brown adipose tissue, skeletal muscle and heart for thermogenesis and species-dependent mechanisms (sweating, panting and saliva spreading) for evaporative heat loss. These effectors are regulated by parallel but distinct, effector-specific neural pathways that share a common peripheral thermal sensory input. The thermal afferent circuits include cutaneous thermal receptors, spinal dorsal horn neurons and lateral parabrachial nucleus neurons projecting to the preoptic area to influence warm-sensitive, inhibitory output neurons which control thermogenesis-promoting neurons in the dorsomedial hypothalamus that project to premotor neurons in the rostral ventromedial medulla, including the raphe pallidus, that descend to provide the excitation necessary to drive thermogenic thermal effectors. A distinct population of warm-sensitive preoptic neurons controls heat loss through an inhibitory input to raphe pallidus neurons controlling cutaneous vasoconstriction.
Intracranial schwannoma involving the XIIth cranial nerve is rare. We report an unusual clinical presentation and pathological verification of a schwannoma, which had become haemorrhagic and necrotic, simulating acute purulent meningitis.
A literature review of intracranial tumors presenting as acute purulent meningitis, with emphasis on schwannomas, was undertaken.
Few cases of hypoglossal schwannoma have been reported; the association with purulent meningitis has not been previously documented.
In patients presenting with sterile purulent meningitis, a necrotic tumor should be considered as a possible etiology.
A patient is described with a vertebrobasilar aneurysm who was erroneously thought to have myasthenia gravis on the basis of the clinical presentation and investigations, which were interpreted as supportive of a disorder of the neuromuscular junction. Despite the correct diagnosis being made at a late stage the patient made a full recovery after radiological intervention.
Dumbbell-shaped schwannomas of the hypoglossal nerve are very rare. This report concerns a case with an extradural, dumbbell-shaped hypoglossal schwannoma extending both intra- and extracranially.
A 25-year-old woman presented with a right hypoglossal palsy. Imaging revealed a dumbbell-shaped tumor with considerable compression and medial displacement of the medulla oblongata, diagnosed as a hypoglossal schwannoma. The tumor mass extended extracranially to the parapharyngeal space through the enlarged hypoglossal canal. The tumor was partially excised by a right far-lateral suboccipital approach and the tumor was found to be predominantly extradural with minimal intradural extension. A histopathologic diagnosis of schwannoma was made.
This case emphasizes the importance of recognizing this extradural variant of schwannoma. Staged extracranial and intracranial approaches to these tumors may be necessary.
Fifty-six cases of sudden death due to brain tumor, autopsied in Tokyo Medical Examiner's Office during the past 21 years (1958-1978), were subjected to statistical analysis. The rate incidence to total deaths by 'natural causes' was 0.02%. Regarding sex and age incidence, it is of interest that 17.6% of cases were distributed in females of 20-29 years. Thirty-nine cases (69.6%) had visited a doctor or a hospital for some complaints prior to death; 15 cases were diagnosed as brain tumor or brain tumor suspected (12 cases were postoperative), 18 cases had clinical diagnosis other than brain tumor, such as epilepsy, common cold, syphilitic locomotor ataxia, psychopathy, and 4 cases 'physically not remarkable'. There was no particular past medical history in 17 cases. Chief premonitory symptoms given were frequently vomiting (43.3%), headache (36.4%), convulsion (16.1%) and unconsciousness (13.2%). The relationship between site, size and type of the tumors and the factors which may be responsible for sudden death are briefly discussed.
This chapter deals with primary intracranial neoplasms as a cause of sudden, unexpected death, giving an overview of our current
understanding of both clinical and pathological features of these tumors. A review of the literature and this author’s autopsy
files shows that the incidence of sudden death as a result of primary intracranial neoplasms has declined over the last decades.
Additionally, a proposal for a guideline for cases of sudden, unexpected death that are presumed to have an intracranial cause
is presented.
Dumbbell-shaped hypoglossal schwannomas with intradural and extradural extension are extremely rare, and complete removal of these tumors is very difficult. This report describes such lesions in three patients that were completely removed via a purely endoscopic transoral approach.
Three patients with intradural and extradural growth hypoglossal schwannomas (three women, aged 16, 42 and 43 years) were treated by direct surgery via a purely endoscopic transoral approach to the posterior fossa.
In this series, radical resections of the dumbbell-shaped hypoglossal schwannomas were achieved in all three patients via a purely endoscopic transoral approach without creating additional cranial nerve deficits but temporary left vagus palsy in one case and a temporary left hypoglossal palsy in one case. The postoperative vagus and hypoglossal palsy had recovered in 3 months after surgery. No patient experienced complications such as postoperative cerebrospinal fluid leak, meningitis and cerebrovascular evidence. At the time of this review, the preoperative lingual motor function and muscular bulk had recovered but hemiatrophy of the tongue was still detectable. The preoperative vagus palsy had recovered by the 10th day after surgery. The hearing loss and facial palsy before surgery had completely recovered in 3 months postoperatively. No patient in our series has experienced a recurrence for the follow-up period (3-11 months).
Dumbbell-shaped hypoglossal schwannomas tend to cause lower cranial nerve deficits, facial paralysis and hearing loss. With appropriate preoperative evaluation and careful planning of the perioperative period, complete tumor resection can be achieved via the purely endoscopic transoral approach. The endoscopic transoral approach is an effectice choice for management of dumbbell-shaped hypoglossal schwannomas.
Intratumoral hemorrhage of hypoglossal schwannoma is very rare. A 37-year-old man was admitted to our hospital with severe headache and gait disturbance. Radiologic examination revealed subarachnoid hemorrhage and a well-circumscribed enhanced lesion compressing the medulla oblongata. Gross total removal of the intracranial tumor was performed. Histopathological examination revealed the characteristic features of a schwannoma. The tumor had several hemorrhagic areas and numerous hyalinized blood vessels. Although the mechanism of hemorrhage from schwannomas is unclear, it is hypothesized that tumor vessels showing focal sinusoidal dilatation and hyaline thickening of the walls lead to spontaneous thrombosis with consequent necrosis, and often hemorrhage.
A very rare case of continuous hypertension and tachycardia after excision of a cerebellar hemangioblastoma at the dorsal medulla oblongata is presented. This 21-year-old man was admitted to the authors' hospital with a headache and dizziness. Radiological examination revealed a tumor located behind the dorsal medulla oblongata and compressing it substantially. The tumor was completely resected, but after the surgery the patient experienced prolonged hypertension and tachycardia. Postoperative MR imaging showed a small injury at the dorsocaudal medulla that was located at the caudal site of the nucleus of the tractus solitarius (NTS). Because the NTS has been reported to play a central role in cardiovascular regulation along with the rostral ventrolateral medulla, the authors considered it possible that the NTS injury was the cause of the prolonged elevation of sympathetic tone.
Massive intratumoral or subarachnoid hemorrhage from neurinomas is very rare. The authors report on six patients, four men and two women, with neurinomas that presented as spontaneous intratumoral hemorrhage. The average age of the patients was 56.8 years (range, 31-74 years). Neurinomas originated from the acoustic nerve in four patients and from the trigeminal nerve in two. Four cases were accompanied by the sudden occurrence of clinical symptoms such as headache, double vision, and hemisensory or motor disturbance. The maximum diameter of the tumors ranged from 2.8 to 3.8 cm (average, 3.1 cm). Histological examinations showed massive hemorrhage and increased vascularity, with dilated, thin-walled vessels, in all cases. The size of the tumor and the increase in vascularity with dilated, thin-walled vessels within neurinomas are important pathogenetic factors of hemorrhage. When sudden onset of symptoms or rapid worsening of chronic symptoms occurs in neurinomas, intratumoral or subarachnoid hemorrhage should be considered as a possible cause, and magnetic resonance imaging can be an important tool in obtaining correct diagnosis.
The case is reported of a patient with progressive left hemiparesis due to vascular compression of the medulla oblongata. Metrizamide computerized tomography cisternography revealed that the left vertebral artery was compressing and distorting the left lateral surface of the medulla. Compression was surgically relieved and symptoms improved postoperatively. Neurological and symptomatic considerations are discussed in relation to the topographical anatomy of the lateral corticospinal tract.
In 10,995 consecutive medicolegal autopsies, there were 19 deaths due to an unsuspected primary intracranial neoplasm. Nine (47.4%) of the tumors were in the astrocytoma-glioblastoma category. The remainder included four cases of oligodendroglioma and one case each of medulloblastoma, microglioma, meningioma, teratoma, colloid cyst and pituitary chromophobe adenoma. In six cases, death occurred following abrupt loss of consciousness, or else the patient was found dead. In five of these six cases, there were no known preceding symptoms. The remaining 13 patients exhibited the characteristic symptoms produced by intracranial neoplasms, including symptoms of increased intracranial pressure, epilepsy, and psychiatric manifestations. Only one patient presented with a focal neurologic deficit which resolved in 24 hours. A comparison of the duration and type of symptomatology exhibited by these patients with a hospital patient population in which death was caused by a previously diagnosed primary intracranial neoplasm and an autopsy was performed underscored 1) the shorter duration of acute symptomatology, 2) the nonlocalizing nature of the symptoms manifested, 3) the lack of progression or change in symptoms in those patients in whom epilepsy was the primary manifestation of their underlying disease, and 4) the lower incidence of focal neurologic deficits as the presenting symptoms in our series.
A case of a 30-year-old man who showed progressive pyramidal tract signs caused by compression of the left vertebral artery is presented. Initial decompression of the vertebral artery by placing a piece of sponge between the artery and medulla had no long-term effect. The left vertebral artery distal to the origin of the posterior inferior cerebellar artery was then sectioned, decompressing the medulla oblongata. The patient's symptoms improved postoperatively. This is the first reported case of brain-stem compression by an elongated vertebral artery treated by sectioning of the artery.
A 65-year-old male with a giant aneurysm of the vertebral artery, which had caused minimal neurological deficits for a few years, suffered rapid onset of respiratory disturbance followed by coma. Serial neuroimaging studies showed the thrombosed aneurysm continued to enlarge, resulting in increasing compression of the brainstem. Intraoperative observation and histological examination revealed that the aneurysm was composed of totally organized thrombus. Total extirpation of the giant aneurysm resulted in the patient regaining spontaneous respiration and clear consciousness, suggesting that growth of this aneurysm is due to increases in the size and number of the intrathrombotic capillary channels.
Compression of the medulla oblongata by a tortuous vertebral artery is rare. We report two patients with this lesion who were treated with vascular decompression of the vertebral artery.
A 36-year-old man developed right hemiparesis with lower cranial nerve deficits, and a 47-year-old man developed left lower cranial nerve deficits and left cerebellar dysfunction. In both patients, magnetic resonance imaging revealed a tortuous vertebral artery compressing the medulla oblongata.
In both patients, the compressed medulla oblongata was treated by detaching the vertebral artery from the medulla oblongata, shifting it, and anchoring it to the nearby dura mater. Postoperatively, both patients are asymptomatic and have returned to their previous jobs.
Although compression of the medulla oblongata by a tortuous vertebral artery is rare, it can cause brainstem dysfunction. Magnetic resonance imaging clearly revealed the vascular compression in these patients. Surgical treatment was effective. The symptoms related to a tortuous vertebral artery and some techniques for surgical treatment are discussed. Awareness of this rare lesion is necessary to ensure appropriate treatment.
A case of progressive brainstem syndrome secondary to vertebral artery (VA) dolichoectasia is reported. The patient presented with partial bilateral abduction paralysis, which progressed to quadriparesis, ataxia, and areflexia. The initial diagnosis was stroke, but because of the patient's deterioration, a diagnosis of Miller-Fisher syndrome was made. Neuroimages obtained at that time revealed an ectatic left VA with minimal cervicomedullary compression. The patient continued to deteriorate despite medical management. Follow-up imaging demonstrated worsened cervicomedullary compression. An emergency posterior fossa neurovascular decompression was performed using a Gore-Tex sling and resulted in mild neurological improvement. This case emphasizes that early recognition and surgical intervention to prevent progressive neurological sequelae are crucial in symptomatic VA dolichoectasia.
Focal or microscopic hemorrhage in a neurinoma is common, but tumor origin from the hypoglossal nerve and extensive symptomatic intratumoral hemorrhage are both rare. A 59-year-old male presented with severe neck pain, nausea and vomiting of 1-day duration, accompanied by right hypoglossal nerve palsy. Neuroimaging disclosed a tumor located in the right cerebellomedullary fissure and containing a hematoma. The right hypoglossal canal was slightly dilated. The intracranial tumor was resected via a suboccipital approach. Histological examination demonstrated spindle-shaped tumor cells with nuclear palisading and also relative hypervascularity with hyaline degeneration of the vessels. Extensive hemorrhage was present, as was necrosis. Thickening and hyalinization of arterial walls, a common occurrence in neurinomas, may have contributed to symptomatic intratumoral hemorrhage.
Bulbar compression by vertebral artery (VA) aneurysm is extremely rare and only reported in a few cases. We report two cases with thrombosed VA aneurysm compressing medulla oblongata; one presented with bulbar apoplexy hemorrhage and the other with subarachnoid hemorrhage (SAH). A 61-year-old male (case 1) presented with consciousness disturbances, left hemiplegia, and subsequent respiratory arrest. He was subjected to controlled ventilation, and computed tomography (CT) scan showed bulbar hemorrhage. Magnetic resonance imaging (MRI) and digital subtraction angiogram (DSA) revealed fusiform right VA aneurysm with partial thrombosis and bulbar compression. Intra-aneurysmal embolization with Guglielmi detachable coils (GDCs) relieved his clinical symptom including respiratory disturbance. Three months after the onset, he remained moderately disabled. A 76-year-old male (case 2) presented with severe headache and subsequent respiratory disturbance. CT scan on admission showed subarachnoid hemorrhage with acute hydrocephalus. Ventricular drainage rapidly improved consciousness while respiratory disturbance persisted over several days. MRI and DSA suggested spontaneous thrombosis of the right VA dissection with bulbar compression. He showed gradual recovery of his respiration over a week. After ventriculo-peritoneal shunting, he was transferred with moderate disability. These results suggest that the elimination of the pulsatile effect of VA aneurysm adjacent to medulla oblongata can improve symptoms caused by aneurysm-related compression. Early diagnosis and appropriate treatment such as intra-aneurysmal embolization for ameliorating the blood flow inside the aneurysm can relieve mass effect and clinical symptoms.
We describe an autopsy case of sudden unexpected death due to severe brainstem compression by an unruptured giant vertebral aneurysm. A 71-year-old male was found dead in his bedroom. The forensic autopsy revealed no severe trauma leading to his death. On internal examination, a giant intracranial aneurysm (3.4 x 2.6 x 2.7 cm) was observed on the trunk of the right vertebral artery. The aneurysm compressed the right side of the lower one-third of the pons and adjacent medulla oblongata. On sectioning, almost all of the aneurysm lumen was filled with a firm, clearly laminated organized thrombus. There was no evidence of subarachnoid hemorrhage. Histopathological analyses revealed congestion and hypoxic tissue changes in all organs examined. In microscopic sections of the giant vertebral aneurysm, thick fibrotic walls, intimal hyperplasia and organized thrombi in the lumen were found. Lots of intrathrombotic clefts with fresh erythrocytes were also observed. Moreover, Elastica van Gieson staining revealed fragmentation and disruption of the intimal elastic lamina in the aneurysmal wall. Collectively, we considered that some triggers in his daily life, including head rotation, might have caused the rapid onset of respiratory disturbance due to severe brainstem compression by a giant vertebral aneurysm.