Malignant meningeal melanoma

We report the case of an isolated pigmented lesion of the cerebellar tentorium which was initially interpreted as meningeal melanocytoma. The immunohistochemical study required as a result of a subsequent post-surgical recurrence prompted us to reconsider the early diagnosis in favour of malignant meningeal melanoma. On the basis of data in the literature, the possibility that the lesion may have been a dural melanoma is discussed.     

成人松果体区肿瘤的手术治疗

目的探讨成人松果体区肿瘤的临床特点和显微手术治疗方法及效果。方法对21例成人(20岁以上)松果体区肿瘤,包括胶质瘤11例,脑膜瘤4例,生殖细胞瘤3例,胆脂瘤2例,胶质增生1例,分别采用侧脑室入路(2例)、Poppen入路(12例)和Krause入路(7例)进行显微手术治疗。结果肿瘤全切除16例,次全切除4例,仅行肿瘤活检1例,无手术死亡病例。术后随访3月～8年,其中15例恢复良好;1例较术前加重,出现中度残疾;1例肿瘤复发后放弃治疗;4例死亡。结论成人松果体区肿瘤以胶质瘤和脑膜瘤常见,肿瘤全切除是其治疗的根本方法,合理的手术入路及血管保护是手术成功的关键。     

Glioblastoma Multiforme in the Pineal Region with Leptomeningeal Dissemination and Lumbar Metastasis

We report a case of a 31-year-old woman with glioblastoma multiforme (GBM) in the pineal region with associated leptomeningeal dissemination and lumbar metastasis. The patient presented with severe headache and vomiting. Magnetic resonance imaging (MRI) of the brain showed a heterogeneously enhanced tumor in the pineal region with obstructive hydrocephalus. After an urgent ventricular-peritoneal shunt, she was treated by subtotal resection and chemotherapy concomitant with radiotherapy. Two months after surgery, MRI showed no changes in the residual tumor but leptomeningeal dissemination surrounding the brainstem. One month later, she exhibited severe lumbago and bilateral leg pain. Thoracico-lumbar MRI showed drop like metastasis in the lumbar region. Finally she died five months after the initial diagnosis. Neurosurgeons should pay attention to GBM in the pineal region, not only as an important differential diagnosis among the pineal tumors, but due to the aggressive features of leptomeningeal dissemination and spinal metastasis.     

经枕幕上到松果体区锁孔入路的应用显微解剖

目的进行经枕幕上锁孔手术入路到达松果体区显微解剖学研究以指导开展松果体区锁孔手术。方法应用显微外科解剖学技术,对经甲醛固定,血管内灌注红、蓝乳胶的10具成人尸头标本,按手术入路逐层解剖,观察环池、四叠体池、胼胝体压部、第三脑室后部、大脑大静脉系统、松果体及四叠体的显露情况。结果经枕幕上锁孔手术入路对松果体区的显露与常规经枕幕上入路的显露范围一致,而无效脑暴露少,对脑组织的牵拉损伤小。结论经枕幕上锁孔手术入路行松果体区手术是可行的。     

26例松果体区肿瘤显微手术探讨

目的探讨显微手术切除松果体区肿瘤的方法和治疗经验。方法回顾性分析26例经显微手术切除的松果体区肿瘤患者的临床资料和随访结果。结果26例中19例采用Poppen入路,7例采用Krause入路。全切15例,次全切除5例,大部分切除6例。随访3个月~7年,肿瘤未见复发17例,复发2例（1例再手术）,植物状态生存1例,死亡2例（术后因瘤卒中死亡1例,术后2年死亡1例）,4例失随访。结论显微手术是松果体区肿瘤治疗的首选方法,Poppen入路是一种较理想的入路。     

Malignant melanoma in early Parkinson's disease: the DATATOP trial.

The available epidemiological data on the incidence of malignant melanoma in Parkinson's disease are contradictory. The role of levodopa therapy in this context has been debated. We identified all known cases of malignant melanoma (N = 5) in the DATATOP clinical trial cohort and compared that to published expected values (N = 1.5) for a standard healthy population. The standardized event ratio was 3.3 (95% confidence interval, 1.1-7.8), indicating that incidence of malignant melanoma was higher than expected. We found no association between levodopa therapy and the incidence of melanoma.     

Pineal gland in rats suffering from malignancy

Summary Studies of the pineal gland in rats suffering from fibrosarcomas induced by 9, 10-dimethyl-1, 2-benzanthracene have been carried out. The weight of the pineal gland in these animals did not differ from that in control animals. However, the cells and their nuclei in the pineal glands of tumour bearing animals were larger and contained more lipid than those in the pineal glands of control animals. The results indicate that there is increased metabolic activity in the pineal glands of tumour bearing animals.     

经穹窿间中间帆锁孔入路解剖与临床意义

目的研究经穹窿间中间帆锁孔入路的手术显露。方法导航辅助下在16具尸头标本上模拟经穹窿间中间帆锁孔入路手术。显微镜下观察分析此入路对第三脑室、松果体区的显露。结果导航辅助下经穹窿间中间帆锁孔入路手术能在尸头上顺利完成,可清晰显露第三脑室后部及松果体区。结论经穹窿间中间帆锁孔入路技术上可行,值得临床应用和完善。     

Upfront Stereotactic Radiosurgery for Pineal Parenchymal Tumors in Adults

ObjectivePineal parenchymal tumors (PPTs) in adults are rare, and knowledge regarding their optimal management and treatment outcome is limited. Herein, we present the clinical results of our series of PPTs other than pineoblastomas managed by stereotactic radiosurgery (SRS) at upfront setting.MethodsBetween 1997 and 2014, nine consecutive adult patients with the diagnosis of PPTs, either pineocytoma or pineal parenchymal tumor of intermediate differentiation, were treated with SRS. There were 6 men and 3 women. The median age was 39 years (range, 31-53 years). All of the patients presented with symptoms of hydrocephalus. Endoscopic third ventriculostomy and biopsy was done for initial management. After histologic diagnosis, patients were treated with Gamma Knife with the mean dose of 13.3 Gy (n=3) or fractionated Cyberknife with 32 Gy (n=6).ResultsAfter a mean follow-up of 78.6 months (range, 14-223 months), all patients were alive and all of their tumors were locally controlled except for one instance of cerebrospinal fluid seeding metastasis. On magnetic resonance images, tumor size decreased in all patients, resulting in complete response in 3 patients and partial response in 6. One patient had experienced temporary memory impairment after SRS, which improved spontaneously.ConclusionSRS is effective and safe for PPTs in adults and can be considered as a useful alternative to surgical resection at upfront setting.     

Neuroimaging in Pineal Tumors

BACKGROUND AND PURPOSE: The authors report radiological findings in 11 tumors in the pineal region, which were histologically diagnosed as germinomas, pineocytomas pineoblastomas, ependymomas, teratomas, and astrocytomas. METHODS: Computed tomography (CT) was performed in seven patients and magnetic resonance imaging (MRI) was performed in all patients. RESULTS: CT showed a solid or solid/cystic mass with variable contrast enhancement. MRI showed a heterogeneous mass, with hypointense signal on T1 and iso/hyperintense signal on T2-weighted images (WI) and gadolinium enhancement. Extension to adjacent structures occurred in five patients and spread through the cerebral spinal fluid (CSF) in two. CONCLUSIONS: Pineal region tumors have no pathognomonic imaging pattern. MRI and CT are complementary in diagnosis and are important to determine localization, extension, and meningeal spread.     

Malignant rhabdoid tumor: primary intracranial manifestation in an adult

Summary Clinical, histopathological, and ultrastructural features of an extended intracranial malignant neoplasm are presented. Following incomplete extirpation of a large epidural tumor at the temporal base of the skull, the 21-year-old male patient suffered from several local recurrences and died at the age of 27. The neoplasm was defined by its histopathological and ultrastructural characteristics and by the pattern of its antigenicity as a malignant rhabdoid tumor (MRT). Considering previous publications on MRT, this case may be the first of primary intracranial MRT appearing in an adult.     

Primary meningeal melanocytoma of the spinal cord: report of a paediatric case with benign course and review of the literature

A melanin producing encapsulated, intradural-extramedullar tumour in the thoracic spinal cord was surgically removed from a 12 year old girl. The rumour histologically proved to be a melanocytoma and the patient was treated with radiation therapy. During 18 years of follow up she has been free of symptoms. The clinical and pathological features of the case and the literature are reviewed. The importance of differentiating this benign lesion from meningeal malignant pigmented tumours is emphasized.     

Pineal region schwannoma arising from the trochlear nerve

Schwannomas arising from the trochlear nerve are very rare and to our knowledge, less than 35 histologically documented cases have been reported in the literature. There are no reports of a schwannoma in the pineal region. We report a 24-year-old woman who underwent a para-occipital trans-tentorial approach and gross total excision of a pineal region schwannoma arising from the trochlear nerve. This is the first such reported case.     

Primary Paraspinal Malignant Peripheral Nerve Sheath Tumor

Malignant peripheral nerve sheath tumors (MPNSTs) are very rare tumors. We experienced a case of MPNST in the cervical paraspinal space which was not associated with neurofibromatosis. The tumor located in left C6-7 foramen and compressed C7 root. The tumor was removed through the occipital triangle. We report a case of the primary cerivcal MPNST in a patient who did not have neurofibromatosis-1.     

Pineal melatonin and sensory symptoms in Parkinson disease

Sensory symptoms have been reported in 40–60% of patiens with Parkinson's disease, and in at least 10% of patients these symptoms precede the onset of the motor disorder. The pathophysiology of these symptoms remains unknown. Diminished brain serotonin concentration has been reported to be associated with sensory symptoms. Serotonin metabolism is regulated by pineal melatonin. The secretory activity of the pineal gland may be diminished in Parkinson's disease. In experimental animals pineal melatonin has been shown to exert analgesic effects by interacting with opiate receptors. In addition, since opioid peptides mediate the analgesic effects of melatonin, decreased opioid peptide functions in Parkinson's disease may be associated with disruption of the “fine-tuning” pain modulatory functions of melatonin and possibly indirectly facilitate the emergence of sensory symptoms.

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Sommario Nel 40–60% dei pazienti affetti da morbo di Parkinson sono stati descritti sintomi sensoriali e nel 10% di essi questi sintomi precedono l'inizio dei disturbi motori. La patofisiologia di questi sintomi è, per ora, sconosciuta. è stata però descritta una diminuzione della concentrazione della serotonina cerebrale associata ai sintomi sensoriali. Il metabolismo della serotonina è regolato dalla melatonina pineale: perciò l'attività secernente della ghiandola pineale può essere diminuita nel morbo di Parkinson. Nelle esperienze sugli animali la melatonina pineale risulta esercitare un'azione analgesica in quanto interagisce coi recettori oppioidi. Inoltre, piché i peptidi oppioidi mediano gli effetti analgesici della melatonina, la riduzione degli effetti dei peptidi oppioidi nel morbo di Parkison può essere associato colla scomparsa delle funzioni modulatorie della melatonina sul dolore e ciò può facilitare indirettamente la comparsa di sintomi sensoriali.

     

Pineal rhythms are synchronized to light-dark cycles in congenitally anophthalmic mutant rats

Genetically mutant anophthalmic rats lacking a complete visual system due to the absence of eyeballs and optic nerves up to the optic chiasma were used as a model to study photo-regulated physiological activities. The photoreception in these mutant rats was determined by measuring the neuroendocrine response of the pineal gland-melatonin levels in the serum, and synaptic ribbon complexes (SRCs) in the pinealocytes. These parameters were studied in both normal and anophthalmic rats maintained under light–dark (LD 12:12), continuous dark (DD) and light (LL) conditions. Both normal and mutant anophthalmic animals showed nocturnal increases in serum melatonin levels and in the number and diameter of SRC and their vesicles in the pinealocytes in LD. The daily rhythms persisted even upon transfer to DD both in normal and mutant rats, whereas in LL, the nocturnal elevation of both the parameters disappeared. These observations suggested that congenitally blind rats can perceive light. The studies of these parameters in both normal and mutant rats in reversed-LD conditions confirmed that pineal rhythms can be entrained by light–dark cycles in congenitally anophthalmic mutant rats through a nonvisual system for light perception.     

Pineal ganglioglioma with premature thelarche

We report a case of a 6-year-old girl with a ganglioglioma in the pineal region presenting with a rare clinical picture of premature thelarche. Intracranial gangliogliomas are rare, especially in the pineal region. Their character, origin, treatment, and prognosis remain controversial. The pathophysiology of precocious puberty associated with pineal ganglioglioma is discussed.