Although the expression of major matrix components was markedly enhanced, matrix synthesis was enhanced only modestly, and the changes of matrix in human OA menisci were rather modest in the non-degenerated areas.
Plasma cell myeloma (PCM) of the skull base is rarely encountered in neurosurgical practice. PCM has a wide spectrum of pathology, including a quite benign, solitary plasmacytoma (SPC), and an extremely malignant, multiple myeloma (MM) at the two ends of the spectrum. We have described two patients with PCM of the skull base, of which one harbored SPC, and the other progressed to MM. In case 1, a 46-year-old man presented with left multiple cranial nerve impairments and had a large skull base tumor. Subtotal removal was performed. The specimen and general examination, including bone marrow aspiration, revealed SPC. Postoperatively 50Gy of external radiotherapy was administered. The patient has no manifestation of MM 24 months after the initial presentation. In case 2, a 53-year-old woman presented with left abducens palsy and had a left petroclival osteolytic mass. Gross total resection was performed. The specimen revealed a plasmablastic tumor, i.e., myeloma. General examination established the diagnosis of MM. She was administrated adjuvant chemotherapy and autologous bone marrow transplantation. She is alive without local recurrence 30 months after the initial presentation.
Up to now diffuse white matter demyelination of the cerebrum has been reported in only a few cases of mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS). Here we document an autopsy case with this rare neuropathology. Most MELAS cases are diagnosed antemortem by A3243G transition of mitochondrial DNA. While cerebral damage including necrotic foci in the cerebral cortex are common findings in MELAS, prominent white matter involvement best characterizes this MELAS case. There were numerous necrotic foci, varying in size and chronological stage, in the cerebral white matter. In the areas of the white matter without necrotic foci, there was diffuse fibrillary gliosis with the loss of axons and oligodendrocytes. The gliosis was dominant in the deep white matter, sparing the U-fiber. The cerebral cortex showed diffuse cortical atrophy with few scattered necrotic foci. Distribution of the cerebral lesions does not coincide with the territory of blood supply. The vascular wall presented only slight to mild hyalinosis. We assumed a common pathogenesis to the cortical lesions and the white matter change. The pathogenesis of the present diffuse cerebral lesions may not be just secondary to circulatory disturbance but partly due to metabolic abnormality.
Objective Falcotentorial meningioma occurs close to the falcotentorial edges and the confluence of the vein of Galen. The posterosuperior approach conventionally used to reach this site does not allow direct visualization of the tumor matrix, making detachment difficult. Meningiomas at this location are therefore among those that are not well amenable to radical resection. We devised an alternative anterolateral approach that, when used in addition to the posterosuperior approach, provides an operating field which allows to overview large, bilaterally extending tumors. We report this parieto-occipital interhemispheric transfalcine, trans-bitentorial approach, together with associated procedural modifications. Methods We used the approach in four patients with falcotentorial meningioma between February 2008 and July 2017. We began by extending a parieto-occipital craniotomy slightly beyond the midline, to pass across the most caudal bridging vein on the rostral side. We then created a fan-shaped fenestration as large as possible in the falx, between the superior sagittal sinus and the inferior sagittal and straight sinuses (window 1). We further performed wedge-shaped resections of both tentorial edges to the left and right of window 1 (windows 2 and 3). Tumor debulking was then carried out via these three windows (the triple-window method). Finally, we detached the tumor in the area of the falcotentorial edges and the confluence of the vein of Galen. To obtain a superorostral operating field as wide as possible from laterally, thereby exposing the potential blind spots, the operating surgeon used both hands while retracting the precuneus, and the assistant surgeon used both hands to turn over the falcotentorial edges (twosome four-hand retractorless microsurgery). Results The wide operating field provided by this parieto-occipital interhemispheric transfalcine, trans-bitentorial approach and twosome four-hand retractorless microsurgery provides a direct view of delicate structures at the falcotentorial edges and the confluence of the vein of Galen, a site that is most likely to be a blind spot in conventional approaches. Retraction of the precuneus on the nondominant side enabled radical resection with no neurologic deficit in any of the patients. Conclusions The parieto-occipital interhemispheric transfalcine, trans-bitentorial approach with the triple-window method opens an anterolateral operating field in addition to a posterosuperior operating field in large tumors located in the falcotentorial and pineal region, extending anteroposteriorly and bilaterally. The twosome four-hand retractorless technique via this approach enables visualization of the tumor matrix at sites, which are barely visible with the conventional approach. Thus, the tumor can be removed more radically and safely.
Classic medulloblastoma is a relatively common and distinct clinicopathologic entity consisting of primitive multipotential cells with differentiating capacity to neuroblastic and/or glial cell lines. Desmoplastic medulloblastoma has some features in common with cerebellar neuroblastoma, in which ultrastructural evidence of significant neuroblastic differentiation is extremely rare. We studied three cerebellar tumors with evidence of neuronal differentiation as compared to four classical desmoplastic medulloblastomas. Two of three tumors contained the regions of different degrees resembling desmoplastic medulloblastoma and one consisted of neuroblastic cells exclusively. This spectrum of differentiation suggests a relationship between cerebellar neuroblastoma and medulloblastoma, especially of the desmoplastic type. The nature of cerebellar neuroblastoma and its nosology are briefly discussed.
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