Fronto-Orbital Advancement and Posterior Cranial Vault Expansion Using Distraction Osteogenesis in Patients With Multiple Craniosynostosis.

ABSTRACT: This study aimed to evaluate the treatment outcomes and the efficiency of techniques of fronto-orbital advancement (FOA) and posterior cranial vault expansion (PCVE) using distraction osteogenesis in patients with multiple craniosynostosis. We assessed the treatment results and outcomes of 8 patients with multiple craniosynostosis at the Kagoshima University Hospital between 2005 and 2019. Each 4 patients underwent FOA and PCVE, respectively, using distraction osteogenesis. The cranial volume and developmental quotient (DQ) were measured at the preoperative period and 1 year after surgery. The mean patient age at surgery was 22 months. The mean preoperative cranial volume was 1027 and 1071 cm3 in the FOA and PCVE groups, respectively. The mean preoperative DQ scores were 74 and 67, respectively. After 1-year of follow-up, the corresponding mean cranial volume became 1108 and 1243 cm3, respectively. The corresponding mean DQ scores also improved to 74 and 81, respectively. The postoperative follow-ups in all cases were uneventful, except for persistent epilepsy in 1 patient. Fronto-orbital advancement and PCVE using distraction osteogenesis might contribute to good outcomes in expanding cranial volume, cosmetic osteogenesis, and infantile development in patients with multiple craniosynostosis. Regarding the cranial volume expansion, especially, PCVE using distraction osteogenesis is more effective than FOA.

Solitary Fibrous Tumor or Hemangiopericytoma of the Sella in an Older Patient Treated with Partial Removal Followed by Fractionated Gamma Knife Radiosurgery.

Solitary fibrous tumor (SFT) or hemangiopericytoma (HPC) is a rare fibroblastic tumor of mesenchymal origin. SFT or HPC comprises <1% of all primary central nervous system tumors. SFT or HPC of the sellar or suprasellar region is even more unusual. We herein report a sellar SFT or HPC in an octogenarian who achieved favorable progress with partial removal followed by fractionated gamma knife radiosurgery. An 87-year-old woman presented with occasional headache and visual field defects. A rapidly growing tumor of the sella turcica was diagnosed. The patient underwent endoscopic transnasal transsphenoidal surgery; however, only partial resection of the tumor was possible, as it was fibrous and hard with increased vascularity. A histological examination confirmed the tumor to be grade II SFT or HPC. Two months after the resection, the residual tumor grew rapidly. Given the patient's advanced age, re-surgery was not the preferred option; thus, fractionated gamma knife radiosurgery (marginal dose, 30 Gy in five fractions) was performed. MRI and visual field examination performed 3 months after irradiation revealed tumor shrinkage and improvement in the visual field, respectively. One year and three months after irradiation, the tumor continued to shrink and her visual field had improved. Taking age into consideration, partial resection with fractionated gamma knife radiosurgery was the more appropriate choice for both local tumor control and the safety of the optic apparatus.

Histopathological variation in the demyelinating sentinel lesion of primary central nervous system lymphoma.

BACKGROUND: Primary central nervous system lymphoma (PCNSL) is one of the least common malignant brain tumors. It is usually diagnosed initially as diffuse large B cell lymphoma (DLBCL). In rare cases, however, a demyelinating lesion referred to as a "sentinel lesion" precedes the actual diagnosis, which usually depicts two distinct patterns of inflammatory cells during histological analysis. This case report describes a unique histological finding and describes the recognized variations in sentinel lesion histopathology. CASE DESCRIPTION: A 78-year-old female patient was found to have multiple white matter lesions of various degrees of enhancement on post-contrast T1-weighted magnetic resonance imaging. A stereotactic biopsy of a heterogeneous lesion in the left occipital lobe was performed, which revealed demyelination along with lymphocytic infiltration, reactive astrocytosis, abundant T cells, and foamy macrophages. There was no evidence of monoclonality, rapid regression of all lesions occurred, and the patient was thus treated for tumefactive demyelination. Three months later, all of the residual lesions had enlarged and were homogeneously enhancing. An endoscopic-guided biopsy of the right periventricular lesion showed diffuse atypical lymphoid cells. CONCLUSION: The sentinel lesion of PCNSL expresses a variable histological pattern of inflammatory cells. This case demonstrates a unique and rare picture of mixed perivascular and parenchymal infiltration of inflammatory cells, highlighting the importance of repeated biopsies and/or radiological examinations to obtain an accurate diagnosis.