Langerhans Cell Histiocytosis Involving Second Cervical Vertebra and the Hypothalamus and Pituitary in an Adult.

BACKGROUND: Adult-onset Langerhans cell histiocytosis (LCH) with simultaneous involvement of the high cervical spine and the hypothalamus is rare. CASE DESCRIPTION: We have reported a case of adult-onset LCH in the second cervical vertebra with bony destruction and subsequent diabetes insipidus due to simultaneous involvement of the hypothalamus and pituitary stalk. Magnetic resonance imaging of the hypothalamus and pituitary lesion and immunohistochemistry of the cervical lesion revealed LCH. Posterior fusion of the cervical spine (first, third, and fourth cervical vertebrae) was performed, followed by systemic chemotherapy. The cervical fusion was well maintained, and the patient achieved clinical remission. No new LCH lesion was found during the follow-up of >2 years. CONCLUSIONS: Patients with known LCH of the spine showing new symptoms of diabetes insipidus should be examined for infiltrating lesions of the pituitary stalk or hypothalamus. In cases of severe instability of the spine, surgical treatment should be performed. If multiple and systemic LCH lesions are found, systemic chemotherapy should be administered.

Staged, Combined Management of Ruptured Vertebral Artery Dissecting Aneurysms Involving the Posterior Inferior Cerebellar Artery: Report of 4 Cases and Review of the Literature.

BACKGROUND: Ruptured vertebral artery dissecting aneurysms (VADAs) involving the posterior inferior cerebellar artery (PICA) are the most difficult to treat among variations of VADAs but require prompt treatment. The major challenge is to preserve the PICA while occluding the aneurysm. Despite advances in the management of ruptured VADAs involving the PICA, each treatment, whether it is combined or not, is associated with a significant degree of risk. CASE DESCRIPTION: This study presents 4 cases of ruptured VADAs involving the PICA that were successfully treated using a staged, combined method. Embolization of the rupture point in the acute stage was followed 3-4 weeks later by occlusion of the proximal vertebral artery (VA) and PICA origin after occipital artery-PICA bypass in the chronic stage. CONCLUSIONS: Although it is sometimes very difficult to determine the exact rupture point of VADAs, ruptures tend to occur at distal segments of a dissecting aneurysm presenting as bleb. Also, when planning a trapping of the VA, careful examination of angiography is needed to assess the contralateral VA and rupture point. Our staged and combined strategy may provide another valuable treatment option for treating VADAs involving the PICA with special emphasis on the safety and efficacy in our method.

Bifrontal Interhemispheric Approach Involving Cutting the Superior Sagittal Sinus for Distal Anterior Cerebral Artery Aneurysms.

BACKGROUND: The unilateral interhemispheric approach for distal anterior cerebral artery aneurysms presents several risks, such as postoperative venous infarction due to occasional sacrifice of parasagittal bridging vein and postoperative frontal lobe damage due to retraction force. To overcome these risks, we used a bifrontal craniotomy with straight dural incision and cutting of the superior sagittal sinus. METHODS: We retrospectively reviewed 61 patients (42 unruptured and 19 ruptured A2 and A3 aneurysms) who under aneurysm clipping through bifrontal interhemispheric approach between March 2007 and December 2017. There were 35 A2 aneurysms and 27 A3 aneurysms, and mean size of aneurysms was 5.45 mm. The modified bifrontal interhemispheric approach involved 3 steps: bifrontal craniotomy of centrobasal portion of the frontal bone, ligation and division of anterior one third of the superior sagittal sinus, and approaching the aneurysm via the interhemispheric space. All patients underwent computed tomography on postoperative days 3 and 7 for evaluation of brain retraction damage or venous infarction. RESULTS: Among patients with ruptured aneurysms, 79% had a favorable outcome (Glasgow Outcome Scale score 4 or 5) 6 months after primary subarachnoid hemorrhage; all patients with unruptured aneurysms had favorable outcomes. Surgical outcome was strongly related to preoperative neurologic Hunt and Hess grade. Three patients had poor outcomes due to poor Hunt and Hess grade on admission (grade 3 in 2 patients, grade 4 in 1 patient). Follow-up CT showed that venous infarction did not occur in any of the patients. CONCLUSIONS: Modified bifrontal interhemispheric approach may be a safe and effective method for treating A2 and A3 aneurysms with relatively good clinical outcome and no surgery-related complications.

Concurrent p16 methylation pattern as an adverse prognostic factor in multiple myeloma: a methylation-specific polymerase chain reaction study using two different primer sets.

Disruption of cell cycle control genes, including p16, is known to contribute to the cancerogenesis of multiple myeloma (MM). We investigated the methylation status of p16 and its association with common cytogenetic changes, clinicolaboratory findings, and survival in MM. Methylation-specific polymerase chain reaction was performed in 99 newly diagnosed MM patients using two different sets of primers (p16M1 and p16M2). Four patterns of p16 promoter methylation were observed: (1) concurrent methylation of p16M1 and p16M2 (P1P2), 27.3%; (2) methylation of p16M1 alone (P1N2), 7.1%; (3) methylation of p16M2 alone (N1P2), 26.3%; and (4) no methylation (N1N2), 39.4%. Patients with p16P1P1 showed shorter survivals than those with the other methylation patterns (P1N2, N1P2, or N1N2; median survival, 12 vs. 43 months; P < 0.001), regardless of the treatment protocol. In a multivariate analysis, p16P1P2 was an independent prognostic factor of adverse outcome in MM. According to International Staging System (ISS), the study population could be divided into 21.2% (20/94) for stage I, 22.3% (21/94) for stage II, and 56.4% (53/94) for stage III (P = 0.003). ISS can divide patients into prognostic groups. Of note, in patients older than 60 years, ISS was not reflective of disease stage (P = 0.114). If p16P1P2 sets up as stage 4 of ISS, modified ISS could be a more reliable staging system irrespective of age in Korean MM patients (P = 0.003 and P = 0.004 in patients younger than 60 years and in patients older than 60 years, respectively). Our study suggests the potential use of p16 methylation status in predicting the outcome of MM patients and the applicability of demethylating agents in MM.