Analysis of clinicopathological features and NAB2-STAT6 fusion variants of meningeal solitary fibrous tumor with ectopic salivary gland components in the cerebellopontine angle.

Solitary fibrous tumors (SFTs) are rare mesenchymal tumors that can occur at any location. Since the identification of specific NAB2-STAT6 fusion in SFTs, the fusion gene variants, NAB2 exon 4-STAT6 exon 2/3 and NAB2 exon 5/6/7-STAT6 exon 16/17/18, have been reported to be associated with clinicopathological features, and the latter variant is predominant in meningeal SFTs. SFTs developing in the salivary glands are rare, and more rarely, those involving ectopic salivary glands (ESGs) have been reported in the cerebellopontine angle (CPA); however, their characteristics remain not well understood. In this study, we performed a clinicopathological and molecular analysis of 3 cases of meningeal SFT with ESGs. All cases presented with an extra-axial mass in the CPA, which is a rarer location for intracranial ESGs compared to the sellar region. Histologically, except for the presence of ESGs, there was no significant difference between current cases and ordinary SFTs. The ESGs demonstrated no cellular atypia, and although the spindle tumor cells were immunopositive for STAT6, the ESGs were negative in all cases, supporting that the ESGs are non-neoplastic components. In 1 case, ESGs were found only in the primary tumor and disappeared in recurrence/dissemination. Of note, molecular analysis identified NAB2 exon 4-STAT6 exon 2 in all cases. In conclusion, our results suggest that ESGs particularly in the CPA may be associated with SFTs and that meningeal SFTs with ESGs may be associated with the minor fusion variant of NAB2-STAT6 in the intracranial lesions.

[Surgical treatment of ossified cephalhematoma: a case report].

We report a surgical case of ossified cephalhematoma which caused deformity of the skull. A boy was delivered with the aid of vacuum extractor at 40 gestational weeks. He presented with a big cephalhematoma in the left parietal region, and it remained and calcificated after 3 months. He was admitted after being diagnosed with ossified cephalhematoma. Plain skull radiograph showed a marginated radiolucent lesion with a protrusive outer table and a slightly invaginated inner table in the left parietal region. Plain CT scan showed a low density lesion between bony layers and the depressed inner table with irregular thickening. 3D-CT shows bony protrusion of the left parietal bone with thinning in the center. Magnetic resonance imaging revealed an old hematoma in the protruding bone and compression of the parietal lobe by the inner table. For cosmetic reasons and relief of compression of the brain, cranioplasty was performed at 7 months. The protruding bone was removed totally and was arranged to fit in the convex after being cut in pieces. Using bioabsorbable mini plates and screws, the bone was fixed firmly. For cranioplasty of a child, absorbable plates and screws are useful because of their non-interference with growth of the skull.

Capillary hemangioma of the central nervous system.

OBJECT: Capillary hemangiomas are benign tumors or tumorlike lesions that originate from blood vessels and have rarely been reported to develop in the brain or spinal cord. The authors summarize the clinical and histological features of capillary hemangiomas of the central nervous system (CNS). METHODS: The clinical features, imaging characteristics, and outcomes in 10 patients with CNS capillary hemangiomas were reviewed. Histological studies included immunostaining with CD31, alpha-smooth muscle actin, vascular endothelial growth factor, and Ki-67 antigen. Three patients with lesions in the brain presented with symptoms of increased intracranial pressure or seizures. Seven patients with lesions in the spinal cord presented with progressive sensorimotor disturbances of the lower limbs. Computerized tomography and magnetic resonance imaging demonstrated well-defined, enhancing lesions associated with marked perifocal edema. Angiography demonstrated hypervascular lesions, which have not recurred after resection. In two cases, multiple satellite lesions resolved after the systemic administration of steroid drugs or interferon-alpha. Histologically, all lesions were consistent with findings of capillary hemangioma of the skin or soft tissues. The CNS lesions differed significantly from other vascular neoplasms, such as hemangioendotheliomas, hemangiopericytomas, and hemangioblastomas. CONCLUSIONS: Capillary hemangiomas of the CNS are benign lesions that can be surgically removed and cured without adjuvant therapy.