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Concept: Craniopharyngioma

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Diabetes insipidus (DI) after endoscopic transsphenoidal surgery (ETSS) can lead to increased morbidity, longer hospital stays, and increased medication requirements. Predicting which patients are at high risk for developing DI can help direct services to ensure adequate care and follow-up. The objective of this study was to review our institution’s experience with ETSS and determine which clinical/laboratory variables are associated with DI in this patient population. The authors wanted to see if there was an easily determined single value that would help predict which patients develop DI. This represents the largest North American series of this type. We retrospectively reviewed the charts of patients who had undergone ETSS for resection of sellar and parasellar pathology between 2006 and 2011. We examined patient and tumor characteristics and their relationship to postoperative DI. Out of 172 endoscopic transsphenoidal surgeries, there were 15 cases of transient DI (8.7 %) and 14 cases of permanent DI (8.1 %). Statistically significant predictors of postoperative DI (p < 0.05) included tumor volume and histopathology (Rathke's cleft cyst and craniopharyngioma). Significant indicators of development of DI were postoperative serum sodium, preoperative to postoperative change in sodium level, and urine output prior to administration of 1-deamino-8-D-arginine vasopressin. An increase in serum sodium of ≥2.5 mmol/L is a positive marker of development of DI with 80 % specificity, and a postoperative serum sodium of ≥145 mmol/L is a positive indicator with 98 % specificity. Identifying perioperative risk factors and objective indicators of DI after ETSS will help physicians care for patients postoperatively. In this large series, we demonstrated that there were multiple perioperative risk factors for the development of DI. These findings, which are consistent with other reports from microscopic surgical series, will help identify patients at risk for diabetes insipidus, aid in planning treatment algorithms, and increase vigilance in high risk patients.

Concepts: Hospital, Developmental biology, Surgery, Prediction, Physician, Diabetes insipidus, Craniopharyngioma, Rathke's pouch

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Object Previous studies of systemic and intralesional administration of nonpegylated interferon have shown efficacy against craniopharyngioma. Pegylaion of interferon-α-2b (PI) prolongs the half-life, allowing sustained exposure of the drug over time, and enhances efficacy. The authors report the results of the use of PI in 5 children with recurrent craniopharyngiomas. Methods Five children, ranging in age from 9 to 15 years, with recurrent craniopharyngiomas were treated for up to 2 years with subcutaneous injections of PI at a dose of 1-3 μg/kg/week. Tumor response was assessed using MRI. Results All patients had stable disease or better in response to PI. One patient experienced a recurrence after gross-total resection (GTR). She initially showed an increase in the predominantly cystic tumor after 3 months of treatment, followed by a complete response. She required no further intervention and remains without evidence of disease 10 years after starting treatment. Another patient experienced recurrence 3.3 years after subtotal resection (STR) and radiation therapy. He had complete disappearance of the predominantly cystic component after 4 months of treatment, and a small residual calcified mass remains 5 years later. The third patient experienced recurrence after 3 GTRs. He had a complete response after 7 months of treatment and remains without evidence of disease 19 months after starting treatment. The fourth patient experienced recurrence after 2 STRs. He had a 30% decrease in tumor size after 4 months of treatment, which was maintained for 12 months at which point the cyst began to increase in size. The final patient experienced recurrence after GTR and has stable disease 6 months after starting treatment with PI. Conclusions The use of PI in children with recurrent craniopharyngiomas can result in significant and durable responses and potentially delay or avoid the need for radiation therapy.

Concepts: Electron, Medicine, Cancer, Radiation therapy, Cyst, Recurrence relation, Subcutaneous injection, Craniopharyngioma

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BACKGROUND: Rathke’s cleft cyst (RCC) with significant squamous and/or stratified epithelium including smooth transition from single cuboidal to squamous epithelium (tRCC) is rare and possibly represents an intermediate form to craniopharyngioma. METHODS: Twelve patients with histologically confirmed tRCC were retrospectively investigated from a series of 167 cases of RCC and 96 cases of craniopharyngiomas. Clinical data were reviewed, and immunohistochemistry findings for cytokeratins and β-catenin were examined. RESULTS: All lesions were located in the sella turcica with marked extension to suprasellar cistern. Six of the 12 patients had suffered postoperative re-enlargement, and three of these six patients required more than two additional operations and irradiation. CAM5.2 was positive in the glandular epithelium in all tRCCs and focally positive in the squamous epithelium of all these tRCCs. 34βE12 was positive in the squamous epithelium in all tRCCs and focally positive in the glandular epithelium in all but one tRCC. The findings of cytokeratin expression of tRCCs were very similar to those of craniopharyngioma. β-Catenin showed nuclear translocation in five cases. All patients with nuclear translocation of β-catenin suffered postoperative re-enlargement. CONCLUSIONS: tRCC carries an extremely high risk of re-enlargement. Cytokeratin expression resembles that in craniopharyngioma, which might indicate a very close origin of these pathologies. Nuclear translocation of β-catenin may be related to the aggressive clinical course.

Concepts: Anatomical pathology, Squamous epithelium, Immunohistochemistry, Sella turcica, Empty sella syndrome, Craniopharyngioma, Rathke's pouch, Cytokeratin

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Object An extensive craniopharyngioma is a tumor that extends into multiple compartments (subarachnoid spaces) and attains a size larger than 4 cm. A wide spectrum of approaches and strategies has been used for resection of such craniopharyngiomas. In this report the authors focused on the feasibility and efficacy of microsurgical resection of extensive craniopharyngiomas using a frontolateral approach. Methods A retrospective analysis was performed on 16 patients with extensive craniopharyngiomas who underwent operations using a frontolateral approach at one institution. The preoperative and postoperative clinical and radiological data, as well as the operative videos, were reviewed. The main focus of the review was the extent of radical tumor removal, early postoperative outcome, and approach-related complications. Results Gross-total resection of craniopharyngioma was achieved in 14 (87.5%) of 16 cases. Early after surgery (within 3 months), 1 patient showed improvement in hormonal status, while in the remaining 15 patients it worsened. No major neurological morbidity was observed. Two patients experienced temporary psychotic disorders. Visual function improved in 6 patients and remained unchanged in 9. One patient experienced a new bitemporal hemianopsia. Three patients with features of short-term memory disturbances at presentation did show improvement after surgery. There were no deaths or significant approach-related morbidity in this patient series. Only 1 patient required revision surgery for a CSF leak. Conclusions The safe and simple frontolateral approach provides adequate access even to extensive craniopharyngiomas and enables their complete removal with a reasonable morbidity and approach-related complication rate.

Concepts: Improve, Patient, Hospital, Physician, Neurology, Neurosurgery, Psychosis, Craniopharyngioma

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We present a pediatric case of a retrochiasmatic craniopharyngioma in the suprasellar region with third ventricular extension that was resected through a purely endoscopic endonasal approach (EEA) via the transplanum transtuberculum corridor. The patient is a 12-year-old boy who presented with progressive visual loss and panhypopituitarism. The EEA allows direct visualization of the undersurface of the optic chiasm and hypothalamus so that safe and meticulous tumor dissection can be performed to preserve these critical neurovascular structures. This video atlas demonstrates the operative technique and surgical nuances of the endoscopic skull base approach, microdissection of the tumor from the critical neurovascular structures, and multilayered reconstruction of the skull base defect with a nasoseptal flap. A gross total resection was achieved, and the patient was neurologically intact with improved visual acuity and visual fields. In summary, the EEA via the transplanum transtuberculum corridor is an important strategy in the armamentarium for surgical management of pediatric craniopharyngiomas. The link to the video can be found at: https://youtu.be/bmgO_PMRHPk .

Concepts: Brain, Physician, Skull, Visual system, Ophthalmology, The Link REIT, Optic nerve, Craniopharyngioma

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Craniopharyngiomas (CPs) are rare, benign tumors derived from Rathke’s pouch, known for their high recurrence rates and associated morbidity and mortality. Despite significant investigation on risk factors for recurrence, a lack of consensus persists. Recent research suggests that specific histopathological and molecular characteristics are prognostic for disease progression. In this systematic review, we analyzed and consolidated key features of CPs that contribute to increased recurrence rates. This systematic review was performed in accordance with PRISMA guidelines. A search string was created with the keywords “craniopharyngioma,” “histology,” “histopathology,” “molecular,” and “recurrence.” Literature was collected from 2006 to 2016 on the PubMed/Medline and Web of Science databases. The initial search resulted in 242 papers, examined with inclusion and exclusion criteria. The final review included a total of 37 studies, 36 primary studies covering a total of 1461 patients and 1 previous meta-analysis. Cystic lesions and whorl-like arrays were found to be associated with increased recurrence, while previously considered reactive gliosis and finger-shaped protrusions were not. The genetic elements found to be associated with increased risk of recurrence were Ki-67, Ep-CAM, PTTG-1, survivin, and certain RAR isotypes, as well as the glycoproteins osteonectin and chemokines CXCL12/CXCR4. The effects of VEGF, HIF-1α, and p53, despite extensive study, yielded conflicting results. Certain histopathological and molecular characteristics of CPs provide insight into their pathogenesis, likelihood of recurrence, and potential novel targets for therapy.

Concepts: Cancer, Pathology, Anatomical pathology, Histology, Histopathology, Benign tumor, Craniopharyngioma, Rathke's pouch

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Objectives  The current video presents the nuances of an endoscopic endonasal approach to a suprasellar craniopharyngioma.Design The video analyzes the presentation, preoperative workup and imaging, surgical steps and technical nuances of the surgery, the clinical outcome, and follow-up imaging.Setting The patient was treated by a skull base team consisting of a neurosurgeon and an ENT surgeon, at a teaching academic institution.Participants The case refers to a 67-year-old man who presented with vision loss and headaches, and was found to have a suprasellar mass, with imaging characteristics consistent with a craniopharyngioma.Main Outcome Measures The main outcome measures consistent of the reversal of the patient symptoms (vision loss and headaches), the recurrence-free survival based on imaging, as well as the absence of any complications.Results The patient’s vision improved after the surgery; at his last follow-up there was no evidence of recurrence on imaging.Conclusions The endoscopic endonasal approach is safe and effective in treating suprasellar craniopharyngiomas. The link to the video can be found at: https://youtu.be/p1VXbwnAWCo .

Concepts: Hospital, Surgery, Physician, Ophthalmology, Surgeon, Neurosurgery, The Link REIT, Craniopharyngioma

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Craniopharyngiomas are neoplasms of the sellar/parasellar region that are classified into adamantinomatous (ACP) and papillary (PCP) subtypes. Surgical resection of craniopharyngiomas is challenging, and recurrence is common, frequently leading to profound morbidity. BRAF V600E mutations render PCP susceptible to BRAF/MEK inhibitors, but effective targeted therapies are needed for ACP. We explored the feasibility of targeting the PD-1/PD-L1 immune checkpoint pathway in ACP and PCP.

Concepts: Antibody, Neurosurgery, Resection, Craniopharyngioma

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Xanthogranulomas are inflammatory masses most commonly found at peripheral sites such as the skin. Sellar and parasellar xanthogranulomas are rare and present a diagnostic challenge as they are difficult to differentiate from other sellar lesions such as craniopharyngiomas and Rathke’s cleft cysts pre-operatively. Their radiological imaging features are yet to be clearly defined, and clinical outcomes after surgery are also uncertain. This study reviews clinical presentation, radiological appearances, and clinical outcomes in a cohort of patients with pituitary xanthogranulomas.

Concepts: Medicine, Skin, Radiology, Neuroendocrinology, Craniopharyngioma, Rathke's pouch

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The increasing recognition of pituitary disorders and their impact on quality of life and longevity has made understanding of this small gland a subject of paramount importance. Pituitary pathology has seen many significant studies that indicate progress in identification and classification of pituitary lesions, as well as improved management strategies for patients. In this review, we outline six major areas of advances: (i) changes in terminology from ‘adenoma’ to ‘pituitary neuroendocrine tumour’; (ii) reclassification of hormone-negative tumours based on transcription factor expression that defines lineage; (iii) updates in new pathogenetic mechanisms, including those that underlie rare lesions such as X-LAG and pituitary blastoma; (iv) clarification of hypophysitis due to immunotherapy, xanthomatous hypophysitis due to rupture of a Rathke’s cleft cyst and IgG4 disease as the cause of inflammatory pseudotumour; (v) the consolidation of pituicytoma variants, including spindle cell oncocytoma and granular cell tumour based on thyroid transcription factor-1 (TTF-1) reactivity; and (vi) the pathogenetic mechanisms that distinguish papillary from adamantinomatous craniopharyngioma. The remaining challenge is clarification of the pathogenetic mechanisms underlying the development of many of these disorders.

Concepts: Gene expression, Cancer, Oncology, Developmental biology, Anatomical pathology, Neuroendocrinology, Craniopharyngioma, Rathke's pouch