Craniopharyngioma in children: Report of three cases and review of the literature

Craniopharyngioma is a rare benign epithelial tumor, originating in the pituitary stem or pituitary gland and developing in the sellar and / or suprasellar region. Of embryonic origin, craniopharyngiomas are considered to develop from epithelial remains of Rathke's pouch. It affects children as well as adults. All ages combined, craniopharyngiomas represent between 3 to 4% of intracranial tumors worldwide, or 0.5 to 2 new cases per year and per million inhabitants. In children, they represent 10% of all intracranial tumors with a peak frequency between 7 and 13 years and a predominance of men. Although benign, this tumor remains a serious pathology because of the frequent visual, endocrine, neuro-intellectual sequelae and the risk of recurrence that it entails, involving the visual and vital functional.The management of this pathology is multidisciplinary and involves several modalities such as surgery, radiotherapy and medical treatment of hormonal deficits very often associated. We report in this article three cases of boys aged 9, 10 and 12; treated for symptomatic craniopharyngiomas with a favorable clinical and radiological outcome after partial surgical excision and adjuvant external radiotherapy. The aim of this article is to review the epidemiological, diagnostic, therapeutic and evolutionary aspects of these benign tumors of the sellar and / or suprasellar region, with an emphasis on the interest of radiotherapeutic treatment.

Spindle cell oncocytoma, a misdiagnosed rare entity of the pituitary – A case report with review of literature and special emphasis on the morphological differentials

Spindle cell oncocytoma (SCO) of the pituitary is a rare tumor of the adenohypophysis occurring in the sellar/suprasellar region. This tumor has been recognized as a distinct entity by the WHO Classification of CNS tumor in 2007. Spindle cell oncocytoma of the pituitary gland accounts for 0.1–0.4% of all sellar region tumors and is predominantly seen in the older adult population. This rare entity simulates clinical and radiological features of pituitary adenoma and is often misdiagnosed. Though WHO grade 1, the tumor can recur and have invasive properties. Herein, we report a 61-year-old woman with panhypopituitarism and temporal field cut, clinically and radiologically diagnosed as pituitary macroadenoma, while the histomorphological and immunohistochemistry features helped in arriving at a diagnosis of Spindle Cell Oncocytoma. The clinicopathological, histomorphological, immunohistochemical, and molecular properties of the tumor are further discussed.

A Single-institution Study of So-called “non-secreting” Intracranial Germinomas: Neurological Characteristics, Treatment Procedures, and Clinical Outcomes

Background and ObjectiveIntracranial germinomas are rare brain tumors. In the present study, we collected data of patients with histology-proven germinomas, and analyzed their neurological characteristics, treatment procedures, and clinical outcomes.MethodsWe reviewed the data of patients with “non-secreting” intracranial germinomas, and analyzed the neurological characteristics, treatment procedures, and clinical outcomes.ResultsThere were 162 (69.8%) male patients and 70 (30.2%) female patients. Pineal germinomas were mainly found in male patients (98.3%), and germinomas in sellar/suprasellar region were often observed in female patients (67.5%). The most common clinical presentations were polyuria/polydipsia (42.7%), headache (40.5%), visual disturbance (37.5%), and motor impairment (28.4%). Patients in the pure germinoma group had a higher serum beta-human chorionic gonadotropin (β-HCG) level than in the germinoma plus syncytiotrophoblastic giant cells (STGCs) group (P=0.001), and no significant difference was noted in serum alpha-fetoprotein (AFP) level between the two groups (P=0.540). Bifocal or multifocal tumors had a higher tendency to intracranial dissemination and spinal seeding (P<0.001 and P<0.001, respectively). The mean duration of open craniotomy was 268.8 min with an average volume of blood loss of 316.9 mL, compared with 27.0 min and 6.2 mL in cases who received stereotactic biopsy (P<0.001 and P<0.001). The most common complication in both groups was postoperative hemorrhage (7.0% and 8.5%). Patients who received biopsy had higher KPS scores after surgery and at hospital discharge than on admission in contrast to open craniotomy (P<0.001 and P=0.047, respectively). Deterioration of KPS at hospital discharge than on admission was observed in 4, 7, 18, and 1 cases who received partial resection, subtotal resection, gross total resection, and biopsy (P<0.001).ConclusionsBifocal and multifocal germinomas have a higher tendency of dissemination, and additional attention should be paid to the imaging findings. Patients may not necessarily benefit from the surgical resection because of the surgical trauma and the postoperative complications, and stereotactic biopsy is highly significant in clinical practice.

Combined endoscopic endonasal transtubercular and transclival approaches for large neurenteric cyst in posterior cranial fossa: A case report and literature review

Background: Intracranial neurenteric cysts (NCs) are extremely rare tumors that more commonly involve the posterior fossa than any other cranial part. While transcranial skull base surgery has been the mainstay of treatment, the utility of endoscopic transnasal surgery (ETS) remains to be established. Case Description: We report a case of a large posterior fossa NC extensively involving the suprasellar region, cerebellopontine angle, and prepontine cistern, which we successfully resected with ETS through a combination of transtubercular and transclival routes. Before surgery, the patient presented with abducens nerve and pseudobulbar palsies, which resolved within 2 weeks postoperatively. The patient remained free from recurrence for 3 years postoperatively. Conclusion: Extended ETS may offer a minimally invasive option for the posterior fossa NC, extensively occupying the ventral space of the brainstem.

EPID-15. AGE AND DOSIMETRIC IMPACTS ON RADIOTHERAPY ASSOCIATED GROWTH IMPAIRMENT FOR PATIENTS WITH INTRACRANIAL GERM CELL TUMORS (GCTs)

PURPOSE Intracranial germ cell tumors (GCTs) are rare pediatric central nervous system (CNS) tumors. Growth impairment induced by radiation treatments was rarely evaluated. We aimed to study the impacts of radiotherapy on height development and identify the dosimetric constraints. MATERIALS AND METHODS A total of 148 pediatric patients diagnosed with GCTs were retrospectively analyzed. Sex, age at irradiation, physical doses and biologically effective dose (BED), height, and endocrine status were obtained from patient records. The cumulative change in height was assessed using age-matched normalized height (ANH). Variables were assessed for correlations and statistical significance. RESULTS Cumulative physical doses and BEDs for the whole brain and pituitary were derived via DVHs and BEDVHs. In contrast to patients &gt;11.5 yr, linear correlations between ANH and cumulative physical doses as well as BEDs to the whole brain and pituitary were identified in patients≤11.5 yr. Dosimetric constrains to the pituitary was 36 Gy for physical dose (AUC=0.70 [95% CI, 0.54-0.86], P&lt; 0.05) and 63 Gy2 BED (AUC=0.69 [95% CI, 0.53-0.86], P&lt; 0.05). Intriguingly, no significant differences in ANH were found among different CSI dose groups in patients ≤11.5 yr. (all P&gt;0.05). Impaired hormone secretions in terms of GH and TSH were identified following cranial irradiation (both P&lt; 0.001), particularly for those with tumors at the suprasellar region (GH: P&lt; 0.01, TSH: P&lt; 0.001). CONCLUSION Our study revealed the impacts of age, dosimetrics and tumor locations for growth impairment. This study will contribute to the optimization of radiation treatment planning and facilitate more individualized therapeutic strategies in pediatric patient with GCTs.

Endoscopic Endonasal Supraoptic and Infraoptic Approaches for Complex “Parasuprasellar” Lesions: Surgical Anatomy, Technique Nuances, and Case Series

Surgical management of lesions involving the lateral area of the suprasellar region, including the lateral aspect of the planum sphenoidale and a tight junction region of the optic canal (OC), the anterior clinoid process (ACP), and the internal carotid artery (ICA) and its dural rings, is extremely challenging. Here, the authors introduce two novels endoscopic endonasal supraoptic (EESO) and endoscopic endonasal infraoptic (EEIO) approaches to access these regions, namely, “parasuprasellar” area. Surgical simulation of the EESO and EEIO approaches to the parasuprasellar area was conducted in 5 silicon-injected specimens. The same techniques were applied in 12 patients involving the parasuprasellar area.The EESO and EEIO approaches can be used independently or in combination, but are more often employed as a complement to the endoscopic endonasal midline approach and transcavernous approach. In clinical application, the EESO and EEIO approaches were successfully performed in 12 patients harboring tumors and multiple aneurysms involving the parasuprasellar area. Gross total and subtotal tumor resection were achieved in 9 patients and 1 patient, respectively. For two patients with multiple aneurysms, the lesions were clipped selectively according to location and size. Visual acuity improved in 7 patients, remained stable in 4, and deteriorated in only 1. No postoperative intracranial infection or ICA injury occurred in this series. The EESO and EEIO approaches can be combined with the current endoscopic endonasal midline approach and transcavernous approach to remove extensive pathologies involving the intrasellar, suprasellar, sphenoid, and cavernous sinuses and even bifurcation of the ICA.

Il craniofaringioma

Craniopharyngioma (CP) is a rare epithelial low-grade tumour that develops in the sellar/suprasellar region of the brain, along the craniopharyngeal duct. It has a bimodal distribution and the first peak occurs in paediatric age almost exclusively consisting of the adamantinomatous subtype. A second peak occurs in adulthood after the fifth decade of life and is more likely to be of the papillary subtype. Therapy is based on surgical removal, so the best approach is the endonasal approach, which is sometimes associated with radiation therapy. Molecular target drugs are a promising novelty, indeed they are already in use in adults and are being tested in children. Although CP is considered a low malignancy tumour, its localization and close relationships with important structures such as the optical pathways, the hypothalamic-pituitary axis and the thalamus burden it with important complications (visual disturbances, central obesity, dysendocrinopathies) that can interfere with the patient’s quality of life.

Failure to Thrive Revealing a Pilomyxoid Astrocytoma: An Uncommon Case Report with Literature Review

Pilomyxoid astrocytoma (PMA) is a freshly described figure of low-grade neoplasms encountered in early childhood. Nevertheless, its precise classification by the World Health Organization (WHO) is still debatable. Making an exact diagnosis relies on histological and immunohistochemical pathognomonic features with specific radiological findings. PMA behaves aggressively with a shorter progression-free survival, and its management is unfortunately still arguable. We describe a rare case of PMA involving the suprasellar region who displays symptoms consistent with diencephalic syndrome. The diagnosis was made by magnetic resonance imaging (MRI) focused on the hypothalamic-pituitary axis, and the patient underwent a subtotal tumor resection combined with chemotherapy. Diagnosis of brain tumors should be kept in mind in young children with generalized and severe unexplained loss of subcutaneous fat with failure to thrive after ruling out classical causes.

Psychotic Features as Initial Presentation of Intracranial Epidermoid of Suprasellar Origin

Intracranial epidermoids are rare congenital lesions which account for around 1% of all intracranial tumors. They present most commonly in cerebello-pontine angle followed by suprasellar region, along with different intracranial areas. The most common presentation includes headache, cranial nerve deficiets, seizures, raised intracranial pressure. It is extremely uncommon for epidermoid tumors to present with psychiatric symptoms. We present a case of 38 year old female with history of psychosis for 2 years with personality change, disorganised behavior and delusions. An episode of increased sedation and semi-consciousness led to neuroimaging which revealed a large epidermoid tumor of suprasellar region and interpeduncular region. Surgical resection of the epidermoid was done and patient was maintained on drug treatment. Keywords: Epidermoid tumour, Psychosis

Endoscopic management of periventricular and intraventricular arachnoid cysts

Introduction The most successful surgical management of periventricular and intraventricular cysts is still a matter of debate. Up to the early 1990s, open cyst fenestration and cystoperitoneal shunts were the only options available. Recently, different endoscopic approaches to these lesions have gained popularity. Methods Eighteen endoscopic procedures were performed for the treatment of arachnoid cysts in 18 patients. Sylvian fissure arachnoid cysts were excluded from this study. Ten of them were females and eight were males with their ages ranging from 6 months to 50 years with a mean of 16 years. All patients were prospectively observed. Results Seven of the arachnoid cysts were in the suprasellar region, 5 in the quadrigeminal region, 2 in the posterior fossa, 2 parietal, and 2 intraventricular. Seventeen cases (94%) had hydrocephalus. The main presenting manifestations were those of increased intracranial pressure. All fenestrations were done in the lateral ventricle except for the 2 cases with posterior fossa arachnoid cysts, the fenestrations were done to the fourth ventricle. Endoscopic ventriculocytostomies (VC) were performed in 14 cases without operative complications and no stents were placed. Endoscopic cystocisternostomies (C) were done in all suprasellar cases. Endoscopic cystoventriculostomies (CV) were done in 4 cases. The mean surgical time was 1 h. The mean follow-up period was 15 months. Symptoms improved in 17 cases. Seventeen cases demonstrated a significant decrease of the cysts’ size in the postoperative computed tomography (CT) and magnetic resonance imaging (MRI). A single case developed a complication in the form of subdural hygroma which required no intervention and was managed conservatively. Conclusion Endoscopic management of periventricular and intraventricular arachnoid cysts is a useful safe option in the management of arachnoid cysts related to the ventricular system.