Imaging-documented repeated intratumoral hemorrhage in vestibular schwannoma: a case report.
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SUBMITTER: Mandl ES
PROVIDER: S-EPMC2760714 | biostudies-other | 2009 Oct
REPOSITORIES: biostudies-other
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Project description:Trochlear nerve schwannoma without neurofibromatosis is extremely rare. To our knowledge, only 31 surgical cases have been reported to date, and only 2 cases of trochlear nerve schwannoma with intratumoral hemorrhage have been reported. None of those cases presented with persistent hiccups. We report the case of a 44-year-old man with trochlear nerve schwannoma associated with intratumoral hemorrhage who presented with a 10-day history of persistent hiccups. Computed tomography and magnetic resonance imaging revealed a solid tumor with a 3-cm diameter and intratumoral hemorrhage in the left petroclival region that compressed the midbrain and pons. Subtotal removal of the tumor was performed via the zygomatic transpetrosal approach. Intraoperative findings revealed a tumor arising from the trochlear nerve. The histologic diagnosis was schwannoma of Antoni type A cells with intratumoral hemorrhage. Although the patient's left trochlear nerve palsy worsened temporarily, his postoperative course was uneventful. We present this rare case and discuss the mechanism underlying the patient's persistent hiccups.
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Project description:BackgroundSpinal schwannomas are slow growing, benign nerve sheath tumors. These may be asymptomatic or may present as backache with radicular pain, slowly progressive neurological deficits, but rarely with acute spastic quadriparesis attributed to intratumoral hemorrhage.Case descriptionA 38-year-old male presented with the chief complaint of neck pain radiating to the left upper extremity for the last 8 months. On admission, he exhibited diffuse hyper-reflexia but had no motor or sensory deficit. Magnetic resonance imaging showed a solid-cystic intradural extramedullary (IDEM) C2 to C4 mass severely compressing the spinal cord. The same day the patient acutely developed a spastic quadriparesis. Immediately, a partial C2, C3, and C4 laminectomy was performed for tumor excision; within 5 postoperative days, he fully regained neurological function. The final histopathology was consistent with a "schwannoma showing areas of congestion and hemorrhage."ConclusionSpinal schwannomas rarely present with intratumoral hemorrhage and acute spastic quadriparesis. Immediate operative decompression may lead to excellent postoperative neurological recovery.
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Project description:ObjectiveWe retrospectively evaluated the oncological and functional effectiveness of revision surgery for recurrent or remnant vestibular schwannoma (rVS).MethodsWe included 29 consecutive patients with unilateral hearing loss (16 women; mean age: 42.2 years) that underwent surgery for rVS. Previous surgeries included gross total resections (GTRs, n=11) or subtotal resections (n=18); mean times to recurrence were 9.45 and 4.15 years, respectively. House-Brackmann (HB) grading of facial nerve (FN) weakness (grades II-IV) indicated that 22 (75.9%) patients had deep, long-lasting FN paresis (HB grades: IV-VI). The mean recurrent tumor size was 23.3 mm (range: 6 to 51). Seven patients had neurofibromatosis type 2.ResultsAll patients received revision GTRs. Fourteen small- to medium-sized tumors located at the bottom of the internal acoustic canal required the translabyrinthine approach (TLA); 12 large and small tumors, predominantly in the cerebellopontine angle, required the retrosigmoid approach (RSA); and 2 required both TLA and RSA. One tumor that progressed to the petrous apex required the middle fossa approach. Fifteen patients underwent facial neurorrhaphy. Of these, 11 received hemihypoglossal-facial neurorrhaphies (HHFNs); nine with simultaneous revision surgery. In follow-up, 10 patients (34.48%) experienced persistent deep FN paresis (HB grades IV-VI). After HHFN, all patients improved from HB grade VI to III (n=10) or IV (n=1). No tumors recurred during follow-up (mean, 3.46 years).ConclusionsAggressive microsurgical rVS treatment combined with FN reconstruction provided durable oncological and neurological effects. Surgery was a reasonable alternative to radiosurgery, particularly in facial neurorrhaphy, where it provided a one-step treatment.
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Project description:BackgroundIntramedullary hemorrhages involving spinal hemangioblastomas are rare. They are frequently associated with devastating neurologic outcomes, despite with emergent surgical intervention. Here, we presented an example of an intramedullary hemorrhage occurring in a spinal hemangioblastoma, where the patient markedly improved with surgery. Additionally, the appropriate literature was reviewed (including intraoperative video).Case descriptionA 49-year-old female with a 4-year history of tingling in the left lower extremity presented with vomiting, stepwise worsening of bilateral scapular pain, new upper motor neuron signs, and severe sensory loss bilaterally below C4 on the left and T4 on the right. The magnetic resonance imaging demonstrated a well-circumscribed, uniformly enhancing intramedullary tumor at the C2 level with hyperintensity on the T2 study consistent with acute hemorrhage and cord edema. An urgent C2 laminectomy was performed for gross total tumor resection. Intraoperatively, intramedullary hemorrhage was identified anterior to the tumor mass and was confirmed histopathologically. Postoperatively, the patient had no new sensorimotor deficits and fully recovered within two postoperative months.ConclusionsPatients presenting with acute intramedullary hemorrhage within hemangioblastomas of the spinal cord may demonstrate significant postoperative neurological recovery.
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Project description:RATIONALE:Intracranial solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) are rare spindle cell tumors of mesenchymal origin that include benign and malignant neoplasms. PATIENT CONCERNS:We present a 66-year-old male with a 5-year history of headache and dizziness, with left progressive sensorineural hearing loss over 1 month. DIAGNOSES:WHO grade II SFT/HPC originating from the internal auditory canal in the left cerebellopontine angle. INTERVENTIONS:surgical resection. OUTCOMES:No local recurrence or metastases were observed in the follow-up 3 months after the surgery. LESSONS:Intracranial SFTs/HPCs are rare mesenchymal neoplasms that are challenging to manage. If the imaging characteristics of tumor are not typical, clinicians should depend on tissue biopsy and immunohistochemistry to make a definitive diagnosis.
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Project description:Objectives To describe a unique case report of a patient who had partial hearing preservation after translabyrinthine (TL) removal of a vestibular schwannoma (VS). Study Design Case report. Methods The patient's chart was reviewed for hearing levels before and after surgery. Preoperative magnetic resonance imaging (MRI) was compared with postoperative MRI for determination of completeness of tumor removal. The literature on hearing preservation after TL resection is reviewed. Results A 42-year-old woman underwent a TL removal of a VS. The patient's preoperative pure tone average (PTA) was 70 dB and word recognition score (WRS) was 40%. Postoperatively, the patient was able to hear ambient noise in the surgical ear. Her bone conduction PTA was 70 dB, but the WRS score dropped to 2%. One year later, she continues to hear ambient noise and sound in the operative ear. Discussion This is the fifth reported case of partial hearing preservation after TL VS resection. It suggests that by preserving the vestibule and the fluids within the vestibule when possible, there maybe enough residual auditory neural structures for a traditional cochlear implant to benefit such a patient. In addition, preserving the incus when possible may help maintain air conduction to help patients with sound localization.
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Project description:Spontaneous intratumoral hemorrhage associated with a meningioma is an extremely rare event and has a very high rate of morbidity and mortality. We report a patient with a history of subtotal resection of a petroclival meningioma followed by gamma knife radiosurgery who presented with sudden neurological deterioration following intratumoral hemorrhage after 4 asymptomatic years. The patient underwent early resection of this atypical meningioma and evacuation of hematoma via a retrosigmoid transpetrosal approach and had rapid neurological improvement.
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Project description:Introduction and importanceSchwannomas are benign tumors commonly found in the cranial vault at the cerebellopontine angle. Schwannomas could arise from any nerve that has Schwann sheath. Their most common extra-cranial localization is the parapharyngeal space. Schwannomas do not usually metastasize, and recurrence is uncommon.Case presentationWe present a case of a sympathetic trunk schwannoma in a patient that presented with dysphagia and a painful neck mass. Oro-pharyngeal examination revealed the presence of a mass filling-up the left tonsillar fossa, and pushing the uvula to the right side. The larynx was also deviated to the right due to the mass effect. The mass was resected en-bloc with the involved part of the sympathetic trunk. The patient developed Horner's syndrome postoperatively.Clinical discussionThe occurrence of Sympathetic trunk Schwannomas is very rare. The majority of patients presented with a cervical mass and non-specific symptoms. The most effective treatment is surgical resection. Our experience suggests sympathetic trunk Schwannomas as a differential diagnosis of slow-growing neck masses and asserts that the surgical resection is the main treatment.ConclusionThe occurrence of Schwannomas in the sympathetic trunk is rare. High clinical suspicion is required to achieve the preoperative diagnosis. The optimal management is the total surgical resection. Recurrence is uncommon when the mass is totally resected.
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Project description:Vestibular schwannomas are tumors arising from the vestibulocochlear nerve at the cerebellopontine angle. Their proximity to eloquent brainstem structures means that the pathology itself and the treatment thereof can be associated with significant morbidity. The vast majority of these tumors are sporadic, with the remainder arising as a result of the genetic syndrome Neurofibromatosis Type 2 or, more rarely, LZTR1-related schwannomatosis. The natural history of these tumors is extremely variable, with some tumors not displaying any evidence of growth, others demonstrating early, persistent growth and a small number growing following an extended period of indolence. Emerging evidence now suggests that far from representing Schwann cell proliferation only, the tumor microenvironment is complex, with inflammation proposed to play a key role in their growth. In this review, we provide an overview of this new evidence, including the role played by immune cell infiltration, the underlying molecular pathways involved, and biomarkers for detecting this inflammation in vivo. Given the limitations of current treatments, there is a pressing need for novel therapies to aid in the management of this condition, and we conclude by proposing areas for future research that could lead to the development of therapies targeted toward inflammation in vestibular schwannoma.
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Project description:BackgroundIntracranial intraparenchymal schwannomas (IS) are rare tumors that have mainly been described in case reports. Here, we report on a case of a brainstem IS and included a comprehensive literature review.Case descriptionA 74-year-old man presented with progressive gait disturbances. CT- and MRI-imaging revealed a contrast-enhancing mass accompanied by a cyst in the dorsolateral pons. Hemangioblastoma was suspected and surgery was advised. During surgery, gross total resection of a non-invasive tumor was performed. Postoperative recovery was uneventful. Based on histopathological examination, the intraparenchymal brainstem tumor was diagnosed as schwannoma.ConclusionOur extensive review illustrates that ISs are benign tumors that most often present in relatively young patients. Malignant cases have been described but form an extremely rare entity. Preoperative diagnosis based on radiological features is difficult but should be considered when peritumoral edema, calcifications, and cysts are noted. In benign cases, gross total resection of the lesion is curative. To adequately select this treatment and adjust the surgical strategy accordingly, it is important to include IS in the preoperative differential diagnosis when the abovementioned radiological features are present.
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