Concurrent occurrence of brain tumors in the same location is very unusual and has been noted in patients with neurofibromatosis. Two lesions, occurring in close contact but of different histology, are called contiguous tumors. Schwannoma and meningioma are the two common histologies reported to present as contiguous tumor. We present two patients with contiguous tumors in the cerebellopontine angle. The magnetic resonance imaging characteristics in both cases demonstrate important findings that should be identified to raise the possibility of contiguous tumors. We discuss the surgical implications in identifying the facial nerve in this complex tumor morphology and steps we took to preserve function. Variable displacement of the facial nerve in the presence of multiple tumors needs to be kept in mind during surgical management and can be significantly aided by intraoperative monitoring.
Background:The treatment and classification of atlantoaxial dislocations (AADs) remain controversial. Here, we utilized intraoperative X-ray to differentiate between reducible and irreducible AADs.Methods:Five patients were diagnosed as having irreducible AAD on dynamic and post-traction X-rays. Under general anesthesia, they were placed prone in a neutral position utilizing skeletal traction. The X-rays and motor evoked potential (MEP), were then monitored before, during, and after placing a thumb on the C2 spinous process and pushing it anteriorly to attain reduction.Results:The intraoperative X-ray confirmed reducibility of AAD in four patients; they subsequently underwent a C1–C2 posterior fusion, which maintained that reduction. For the one patient with an irreducible AAD (despite thumb maneuver), an anterior release was required first to attain reduction, followed by posterior C1–C2 fusion.Conclusion:Here, we divided irreducible AAD into two categories: a) reducible—utilizing a thumb maneuver to compress/push the C2 spinous process forward with the patient positioned prone and b) irreducible—those who cannot be reduced with this technique. A posterior only approach was sufficient for those with “reducible” AAD, whereas those who could not be reduced required an anterior release followed by posterior fusion.
Spinal clear cell meningiomas (CCMs) are rare and dural-based lesion usually affecting the younger population. We report the rare case of giant nondural-based spinal CCM mimicking schwannoma and review the literature. A literature search was performed at PubMed and Embase until January 1, 2020. A total of 19 cases of nondural-based spinal CCM was reported. The following relevant data were extracted: authors, publication year, patient and tumor characteristics, treatment, and outcome. The mean age of the presentation was 20.58 years. Twelve (63.16%) were female and seven patients (36.84%) were male. The most common location was lumbosacral region 15 (79%). Fifteen (79%) tumors had cranio-caudal dimension ≤2 vertebral level, and only four (21%) tumors had dimension ≥2 vertebral level. Gross total resection (GTR) was performed in 18 (95%) patients and subtotal resection (STR) in 1 patient. Recurrences were reported in five (26.14%) patients. Four of them showed recurrences within 6 months; earliest at 2.3 months in the patient had undergone STR. Our patient is 19-year-old male diagnosed with a lumbosacral intradural lesion. Craniocaudal dimension is ≥2 vertebral level shows the foraminal extension and vertebral scalloping. GTR is performed. Intraoperatively, the tumor has foraminal extension and shows attachment with right S1S2 nerve root. No dural attachment is found. Six-month follow-up magnetic resonance image shows no evidence of disease. Nondural-based spinal CCMs are extremely rare and should be kept as a differential diagnosis in young patients with giant intradural tumor, and whose radiological features suggesting of schwannoma. It affects young patients and usually involves more than one vertebral level. The chances of recurrences and metastasis are always high even after GTR; hence, close follow-up of the entire neuraxis is warranted.
Background: Although hemorrhages associated with cervical and thoracic intraspinal schwannomas are typically localized to the subarachnoid hemorrhages (SAH) or subdural hemorrhages (SDH) compartments, rare intratumoral bleeds may also occur. Methods: In the literature, we found and analyzed multiple factors for 13 cases (e.g., epidemiological, clinical, and pathological) of cervical schwannomas with intratumoral hemorrhages (ITH). We added the 14th case of a 35-year-old female with along segment cervical schwannoma with ITH who presented with acute quadriplegia and respiratory decompensation. Results: These 14 patients averaged 51.77 years of age, 60% were male, and the tumor involved 2.83 segments. The incidence of SAH and ITH was noted in five cases each, while SDH’s were very rare. The pathological characteristics were consistent with the diagnosis of cellular schwannomas with S-100 positivity. The clinical outcomes were good (100%) in all the cases, including the one presented (modified McCormick score III). Conclusion: Cervical schwannomas with ITH are rare, and the surgical outcomes in such patients are good-excellent (>90%). The histopathology is always of prime importance and decisive in establishing and confirming the etiology of such ITH.
Dear Editor, Chondrosarcomas are malignant neoplasms composed of cartilage-forming cells, and account for 6 % of all skull base lesions and 0.15 % of all intracranial tumors [1,5]. Chondrosarcomas usually arise as slow-growing indolent tumors within the skull base [1]. The predisposition of chondrosarcomas to involve the skull base is explained by the theory that intracranial chondrosarcomas develop from chondrocytes, within rests of endochondral cartilage present in the skull base [1,3]. In contrast to the skull base, the skull vault or the calvarium develops primarily by intramembranous ossification and hence chondrosarcomas primarily involving the calvarium are extremely rare [5]. We report a giant aggressive chondrosarcoma arising from the parieto-occipital skull vault.A 38-year-old male presented with progressively increasing swelling over the parieto-occipital region of 2-year duration with rapid increase in size in the last 4 months. On examination, a large, midline, firm-to-hard swelling was noted over the parieto-occipital region of the skull (Fig. 1a). X-ray of the Skull showed a partially calcified lesion arising from the parieto-occipital bone and exhibiting the characteristic 'sunburst' appearance ( Fig. 1b, c). Magnetic resonance imaging (MRI) of the brain showed an extradural parieto-occipital lesion, with heterogenous intensities on T1-and T2-weighted sequences and exhibiting heterogeneous contrast enhancement (Fig. 1d, e, f).Our differential diagnoses were osteoma, osseous hemangioma, monostotic fibrous dysplasia, intra-osseous meningioma, calvarial metastasis, and osteosarcoma [5]. On CT, osteomas are juxta-cortical, well-defined sclerotic lesions with preservation of diploe; while osseous hemangiomas are well-defined osteolytic lesions with the characteristic 'sun-burst' or 'honeycomb' appearance. Monostotic fibrous lesions usually occur in the second decade of life and have a characteristic CT appearance of an intra-diploic, expansile lesion with 'ground glass' density [5]. Intra-osseous meningiomas are usually slowgrowing tumors that demonstrate intense, homogenous contrast enhancement. Our patient had a rapid increase in size of the lesion and showed heterogenous contrast enhancement. Calvarial metastases are usually multiple and the patients harboring them are usually in the advanced stage of malignancy; which was not the case with our patient. The most likely differential was calvarial osteosarcoma; however, primary skull osteosarcomas are very rare and usually occur in children. Calvarial osteosarcomas may occur in the third or fourth decade of life, but are secondary to Paget's disease, fibrous dysplasia, chronic osteomyelitis, or radiotherapy.The patient underwent bilateral parieto-occipital craniotomy and gross total resection of tumor with wide (2 cm) bony margins on all sides. Calvarium was reconstructed with titanium mesh cranioplasty (Fig. 1j). No dural attachment was noted. Histopathology revealed a lobulated, partially encapsulated, variably cellular chondroid neoplasm composed o...
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