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Background Chordomas are rare primary tumors of the bone that arise from embryonic notochord. They are locally aggressive tumors with a high tendency for postsurgical recurrence. On the other hand, distant metastases are rare. When they occur, they involve lungs, liver, lymph nodes, and bones. Skin and subcutaneous tissue involvement is even rarer and usually occurs by direct extension of the primary tumor or by local recurrence. Distant cutaneous metastasis from chordoma is an exceptional finding, with fewer than 20 cases reported in the literature. All the cutaneous metastases described derive from sacral chordomas, except for 2 cases in which the source of metastasis is skull-base chordomas. Case Description We report the case of a 55-year-old man with skin metastasis from a cervical chordoma. Conclusions Metastasis has to be taken into account in the differential diagnosis when a new skin lesion appears in a patient with a past medical history of chordoma. To the best of our knowledge, this is the first case of cutaneous metastasis from spinal cervical chordoma. A systematic literature review was performed.

Osteoid osteoma is a benign osteoblastic tumor, quite uncommon in the spine. We report a case of an osteoid osteoma involving the atlas in a 6-year-old boy, who presented with suboccipital pain and torticollis. Initial radiological findings were ambiguous as magnetic resonance imaging showed mainly edema of upper cervical soft tissues. The subsequent computed tomography depicted a lesion of left lamina of C1. As conservative treatment failed, the lesion was surgically resected and the patient became pain free. To our knowledge, this is the first case of osteoid osteoma involving the atlas associated with abnormal soft tissue reaction reported in literature.

Polycystic kidney disease (PKD) is commonly associated to cerebral aneurysms. We describe a young man with PKD and a middle cerebral aneurysm associated also to two temporal cavernous malformations. This association has been never reported. We suggest that CMs may belong to the phenotype of PKD and that may should be screened in PKD patients with cerebral haemorrhage.

Introduction: In the past 15 years, the endoscopic endonasal technique has been expanding its role in the surgery of craniopharyngiomas (CRph), allowing to treat not only endo- and suprasellar CRph, but also purely suprasellar tumors. We revised our surgical experience to investigate what has been changing in the choice of the surgical treatment, reporting out results with the innovative surgical approach. Materials and Methods: We retrospectively revised all midline craniopharyngiomas, treated between 1998 and 2012 through a transcranial and endoscopic endonasal approach at our center. All pre- and postoperative clinical reports and neuroimaging studies have been revised. Results: A total of 95 patients were operated through 126 surgical procedures. Of these patients, 26 were pediatrics. Overall, 65 patients had been operated through an EEA and 37 through a TA (seven cases had undergone to both surgeries). In the considered time, the endoscopic endonasal approach has progressively been becoming the approach of choice for all midline craniopharyngiomas, with the exception of the intraventricular tumors. The endoscopic endonasal approach has allowed to obtain a radical tumor removal in 84% of cases. Visual symptoms were normalized at follow-up in 34%, improved in 43%, and worsened in 3% (two cases). At endocrinological follow-up, 81% of patients presented panhypopituitarism, 17% partial hypopituitarism, and DI was present in 86% of cases. Quality of life is normal and was preserved in 86%. The recurrence rate was 11%. Complications consisted in 11 CSF leaks (17%), 2 (3%) hydrocephalus (one resulted fatal), and 1 chronic subdural hematoma (1.5%). Conclusion: During the past 15 years, endoscopic endonasal approach had proved an increasing role, becoming the treatment of choice for every CRphs, except the intraventricular tumors. This was because of the good visual outcome, the reduced neurological sequelae and the greater preservation of quality of life, which we observed with this approach. The endonasal route favors the dissection of the tumor though an arachnoid plane, allowing the preservation of nerves and vessels. Unfortunately, the EEA is hampered by a high complication rate, mainly represented by CSF leaks.

Object In the last decade the indications for the endoscopic endonasal approach have been progressively expanded to include lesions that have not been traditionally considered amenable to resection through the transsphenoidal route. In this study, the authors analyze their experience with hypothalamic gliomas treated via the endoscopic endonasal approach. Methods Consecutive cases of hypothalamic gliomas treated since 2007 via an endoscopic endonasal approach were reviewed. Preoperative and postoperative neuroimaging as well as endocrinological, neurological, and visual symptoms were analyzed to assess the surgical outcome. Signs and symptoms of hypothalamic dysfunction including body mass index (BMI), memory, sleep-wake rhythm, and polyphagia were prospectively collected pre- and postoperatively to assess hypothalamic function. Quality of life was evaluated using the Katz scale. Results In the initial phase the endoscopic endonasal approach was adopted in 3 cases with a palliative intent, to obtain a biopsy sample or for debulking of the mass followed by radio- or chemotherapy. In 2 later cases it was successfully adopted to achieve gross-total tumor resection. Complications consisted of 2 postoperative CSF leaks, which required an endoscopic endonasal reintervention. Visual deficit improved in 3 cases and normalized in the other 2. Four patients developed diabetes insipidus, and 3 an anterior panhypopituitarism. All patients had a moderate increase in BMI. No patients presented with any other signs of hypothalamic damage, and their quality of life at follow-up is normal. Conclusions Despite the limitations of a short follow-up and small sample, the authors’ early experience with the endoscopic endonasal approach has revealed it to be a direct, straightforward, and safe approach to third ventricle astrocytomas. It allowed the authors to perform tumor resection with the same microsurgical technique: dissecting the tumor with 2 hands, performing a central debulking, and controlling the bleeding with bipolar coagulation. The main limitations were represented by some anatomical conditions, such as the position of the chiasm and the anterior communicating artery complex and, finally, by the challenge of watertight plastic repair. To definitively evaluate the role of this approach in hypothalamic gliomas, a comparison with transcranial series would be necessary, but due to the rarity of these cases such a study is still lacking. The authors observed that more aggressive surgery is associated with a worse endocrinological outcome; thus they consider it to be an open question (in particular in prepubertal patients) whether radical removal is an advisable goal for hypothalamic gliomas.