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Background: Malignant craniopharyngioma is a rare tumor with few published case reports. It can form de novo or transform from a benign variant and is associated with a dismal survival rate. We reviewed the literature for all published cases and studied the effect of radiation on the rate of malignant transformation. We analyzed the effect of chemotherapy on survival. Methods: We used various search engines to locate literature from 1980 onward and identified 31 case reports, one of which was excluded. Statistical analysis using the SAS software was conducted, and a significant value was identified if P < 0.05. Results: There was equal distribution among male and female patients. The average age at malignant diagnosis is 31.11 years (±15.16) and 12.19 years (±8.41) for the average interval of benign tumor progression to malignancy. The most common clinical presentation was visual loss and/or field deficits in 26/30 patients (86%). Almost 11/30 patients (37%) had endocrinological deficits, with panhypopituitarism as the most common in 8/11 patients (73%). Fifteen patients received radiation before malignant transformation (47%) and demonstrated no effect on malignant transformation (P = 0.379). Gross total resection was achieved in 2/30 patients. The average time to mortality postoperatively is 5.3 months ± 4.3. Ten patients received chemotherapy, and five were alive at last follow-up (P = 0.115). Conclusion: Malignant craniopharyngioma carries a dismal prognosis with no apparent benefits of radiation therapy and chemotherapy on survival.

Background: Rathke cleft cyst (RCC) apoplexy is an uncommon type of lesion that is challenging to diagnose without histopathological samples. Very few articles have been published describing the details of RCC apoplexy. We studied a good number of published articles to analyze its demographics, clinical and hormonal presentations, and outcomes. Methods: A literature review of English language publications about RCC apoplexy or pituitary apoplexy was conducted using Medline and EMBASE search engines. Thirty articles with histological diagnosis of RCC apoplexy were identified, the earliest of which was published in 1990 and the latest in 2019. We combined the findings of these articles with our own case report and then compared the demographics, clinical and hormonal presentations, and outcomes between RCC apoplexy and pituitary adenoma apoplexy. Results: Our data included 29 patients with RCC, with a mean age of 36.87 years (8–72) and a predominance of female patients (68%). The hemorrhagic type was most common, reported in 86%. Headache was the most common presenting symptom, being reported in 93% followed by hypogonadism (73%) and hormonal deficits (52%). All but three patients improved neurologically (90%); however, 45% of patients required long-term hormonal replacement, mostly thyroid hormone. No cases of worsening neurological or hormonal status were reported. Conclusion: RCC apoplexy presents with less severe neurological and hormonal abnormalities than pituitary adenoma apoplexy; it also has a better prognosis in endocrine functional recovery. We recommend applying current management guidelines of pituitary adenoma apoplexy to RCC apoplexy.

Surgical management of Skull –base pathologies remains one of the most challenging interventions for neurosurgeons. Advances in neuroimaging and surgical technologies coupled with promotion of tailored neurosurgical approaches promote precise clinical diagnosis, surgical planning and enhance neurosurgical outcome. The fronto-orbito-zygomatic (FOZ) approach with its variation welds multiple surgical avenues that facilitate better exposure and safe removal of the pathology. This approach is recommended for handling complex skull base tumors or vascular lesions located around the central skull base, cavernous sinus and upper clivus. The manuscript highlights detailed information about (FOZ) approach and how to master it.

Objective The hereditary syndrome of multiple congenital intraspinal cysts associated with distichiasis, lymphedema and other congenital deformities is extremely rare. Modern imaging techniques have promoted the non-invasive diagnosis of spinal pathology and paved the way for better surgical planning. We reviewed the clinical data, imaging studies and treatment outcomes of a 12-year-old boy with this syndrome. Clinical Presentation Progressive spastic paraparesis with signs of spinal cord compression leading to frequent falls. This was associated with bilateral double row of eyelashes and pretibial edema. The MRI of thoracic spine depicted two large elongated extradural lesions extending from D5-D10 with signal intensity compatible with cerebrospinal fluid leading to severe compression of the spinal cord dorsally. Treatment Laminotomy and complete microsurgical excision of the cysts resulted in a fast and full clinical recovery of his neurological deficit. Conclusion Type I congenital intraspinal cysts is a rare etiology of cord compression syndrome and may be associated with distichiasis, lymphedema and other congenital deformaties. It has several characteristics, which include the higher incidence in thoracic spine and younger age group, disproportional sever motor deficit as compared with sensory disturbances and the excellent clinical recovery following successful surgical treatment.