Primary spinal peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare entities that predominantly occur in children and young adults. Few studies have reported more than three cases. There are no current optimum treatment strategies due to the paucity of data. Here, we present 13 patients (nine females and four males) with primary intraspinal pPNETs who were surgically treated from April 2008 to February 2014. Histopathologic findings revealed the expression of CD99 in all cases. Limb weakness was the most common initial symptom (11/13, 85 %). The tumors were located mainly at the cervical level (6/13, 46 %) and in the epidural space (10/13, 77 %). The radiological diagnosis was neurinoma or meningioma in most cases (10/13, 77 %). Gross total resection was achieved in 77 % (10/13) of patients. During a mean follow-up of 25.5 months, local relapse occurred in 8 (61.5 %) patients and distant metastases occurred in 8 (61.5 %) patients. The overall 1-year survival rate was 77 % (10/13), and the overall 2-year survival rate was 54 % (7/13). The 2-year survival rate was 57.1 % in patients with adjuvant chemotherapy and 50 % in those without chemotherapy. Gross total resection and adjuvant radiotherapy with or without chemotherapy demonstrated a longer survival period (1-year survival rate: 100 %; 2-year survival rate: 86 %). Our data showed that primary spinal pPNETs are extremely rare and aggressive tumors with a poor prognosis. Radical resection is advocated. Gross total resection combined with adjuvant radiation may help to significantly improve patient survival period. Chemotherapy may also help to slightly prolong patient life.

This retrospective study aimed to identify the pattern of visual impairment in pituitary adenoma patients. Patients' eyes were categorized into four groups according to their visual acuity score. Trans-sphenoidal hypophysectomy was performed on all patients. Significant differences between groups were evaluated for visual field defects, visual symptoms duration, degree of suprasellar extension and tumour pathology. In the 201 patients (402 eyes) evaluated, 166 eyes had impaired visual acuity (45 eyes, < 4.0; 43 eyes, 4.0 - 4.4; and 78 eyes, 4.5 - 4.8), 236 eyes scored > 4.8 and were considered to be free of this disorder. A linear correlation was found between visual field defects and visual acuity score. Duration of visual symptoms between visual acuity groups was not significant. Suprasellar extension (measured by Hardy grades) was significant between visual acuity groups and may be the main cause of visual acuity loss in pituitary adenoma patients. Non-functional pituitary adenomas tended to affect visual acuity more than adenomas with other pathological diagnoses which are adrenocorticotropic hormone and prolactin. Further investigation is required to clarify visual loss cause in pituitary adenoma patients.

Object Intramedullary spinal cord cavernous malformations (CMs), once thought to be extremely rare, have been diagnosed more frequently since the advent of MR imaging. In the literature, however, only a few studies include more than 10 cases. The aim of this study was to discuss the clinical presentation of intramedullary spinal cord CMs and the outcome of microsurgery for these histologically benign but clinically progressive lesions. Methods The authors retrospectively reviewed the records of 20 patients who underwent microsurgery for intramedullary spinal cord CMs. All patients had undergone pre- and postoperative MR imaging, and they were all treated using microsurgical resection. The diagnosis of spinal cord CMs was based on pathological criteria. The pre- and postoperative neurological states of the patients were classified according to the McCormick scale and Frankel scale. The microsurgical outcomes are presented and discussed. Results In most cases, CMs can be diagnosed on the basis of MR imaging findings, since these lesions have certain characteristic imaging patterns. Patients with intramedullary spinal cord CMs may present with either a rapid, acute onset of symptoms or slow, progressive neurological decline. The CMs in 19 of 20 patients in this series were totally resected, and most patients neurologically improved postoperatively. As previously reported, the authors confirm that the treatment of choice for symptomatic intramedullary CMs is total removal of the lesion to avoid recurrence and the possibility of further hemorrhage. Conclusions This study has defined the clinical features of symptomatic intramedullary spinal cord CMs. Surgery is the mainstay treatment. Surgical outcome is associated with low mortality with a high probability of functional recovery, especially when symptoms are not severe and are of relatively recent onset.