Large aplasia cutis congenita of the vertex conservative management.

BACKGROUND: Aplasia cutis congenita (ACC) of the vertex with bone defect is a rare and begnin anomaly that can involve the epidermis, dermis, and subcutaneous tissues of the scalp with significant bone defect Bajpai and Pal (J Pediatr Surg 38(2):e4, 2003). When associated with skull defect, this rare malformation carries the risk of severe complications such as rupture of the superior sagittal sinus or infections. METHODS AND RESULTS: We report a case of aplasia cutis congenita of the scalp with skull defect measuring 9 × 10 cm and an exposed sagittal sinus in a newborn. Both conservative and surgical methods have been proposed to treat this condition. In our case, conservative treatment was planned led to complete epithelization and the patient was healing well at 5 years of follow-up. CONCLUSIONS: ACC of the vertex with a large scalp defects present a management dilemma Rocha et al. (Clin Case Rep 3(10):841-4, 2015). Based on a review of the literature, we report this case to demonstrate that even for the largest skin and bone defects, an initial conservative approach may allow for complete wound closure without the need for early surgical intervention.

Intramedullary spinal cord tumors: A retrospective multicentric study.

CONTEXT: Intramedullary tumors are neoformations taking part on the spinal cord, and they are a rare pathology. Due to the rarity of such lesions, clinical studies take years to ensure a decent feedback with a significant number of cases. DESIGN: Our study is retrospective and descriptive. PARTICIPANTS: We share a Tunisian multicentric experience of 27 years through a retrospective study of 120 cases of spinal cord tumors that have been operated in six different centers. OUTCOME MEASURES: The clinical, radiological, and histological findings have been analyzed along with postoperative results and tumoral progression so that we could conclude to some factors of prognosis concerning the management of these tumors. RESULTS: The mean age of our patients is 33.84 years. We had 57 males and 63 females. The most frequent revealing symptom was motor trouble presented as frequent as 77.5% of the patients. Glial tumors were represented in 81 of the cases (67.5%) and nonglial by 39 cases (32.5%). Glial tumors we found were essentially 39 ependymomas and 35 astrocytomas. Surgical resection is key in the management of these lesions; the quality of tumoral resection was a significant factor of disease progression as subtotal resection is correlated to more important progression than total one. CONCLUSION: We conclude this work with some statements. In terms of functional results, age is not a significant factor. Presurgical functional state, the histological type, and the extent of surgical resection are the important factors.

Pediatric Cerebellar Pilomyxoid Astrocytoma: Clinical and Radiological Findings in Three Cases.

Pilomyxoid astrocytomas (PMA) is a particular form of glial tumors distinct from pilocytic astrocytomas (PA). On the last 2016 WHO classification for CNS tumours, no definite grade assignment was proposed for these lesions. They may be more aggressive with a different clinical course compared to PA due to their greater propensity for local recurrence and cerebrospinal dissemination. Most cases arise from the hypothalamic region. Only few studies reported cerebellar localization of the lesion. We report 3 pediatric cases treated for pediatric PMA of the posterior fossa. Clinical, radiological, and prognostic features were reviewed. The age of our patients was between 1 and 9 years old. Signs of intracranial hypertension were found in all patients. One of them presented an increased head circumference and the 2 others had a cerebellar syndrome. Brain CT-scan and MRI displayed a large wellcircumscribed intra-axial solid and cystic posterior fossa tumor. Total surgical resection was performed for all tumors. After a 2 years follow up, no signs of recurrence were noticed. In the literature, PMA been reported with overwhelming majority in children aged between 2 months and 4 years. Despite of many pathological similarities with PAs, PMAs have some specific features in histology, leading to their identification as independent type of glioma. Radiological differential diagnosis between PMAs and Pas can be made using arterial spin labeling imaging, which shows low perfusion parameters in PAs. Clinical and radiological follow up are mandatory do to different natural history and higher rates of local recurrence of this tumor compared to PA. Prognosis is favorable when complete surgical exeresis is possible.

Distinguishing Falcine Chondrosarcomas from Their Mimics and Management.

BACKGROUND: Primary intracranial chondrosarcoma is an extremely rare malignant tumor of the central nervous system, which accounts for <0.16% of all primary intracranial tumors. This rare tumor has a high associated morbidity from the tumor itself as well as from treatment modalities. CASE DESCRIPTION: A 33-year-old man presented with a diffuse headache of 3 months' duration. He was admitted to our department with weakness in the right extremities that had persisted for more than a month. Findings of the neurologic examination revealed right hemiparesis. Cranial magnetic resonance imaging demonstrated a well-demarcated, parasagittal left frontal mass, which compressed to the lateral ventricle. It was hypointense on T1-weighted and hyperintense on T2-weighted images without creating edema in the surrounding tissue. A left frontoparietal craniotomy with complete excision of the mass was performed. The postoperative period was uneventful, and patient was discharged on the fourth postoperative day without any neurologic deficit. Histopathology showed a morphology that was in favor of chondrosarcoma grade 1. CONCLUSIONS: Dural chondrosarcoma is a possible entity in the differential diagnosis of a presumed meningioma, particularly when atypical features are present. We report a grade 1 intracranial chondrosarcoma of the classical subtype without any neurologic problems after complete surgical excision. The patient did not receive any adjuvant therapy and at 26 months' follow-up showed no recurrence.

Embryonal tumor with multilayered rosettes: illustrative case and review of the literature.

BACKGROUND: Embryonal tumor with multilayered rosettes (ETMR) is a very rare entity and has seldom been reported. It has been newly defined tumor entity included in the latest update (revised fourth edition) of WHO 2016 Classification of Tumors of the Central Nervous System which portends a uniform dismal prognosis and survival even with the best of multimodality approaches. ILLUSTRATIVE CASE: This report documents the presentation of a 2-year-old girl with voluminous intracranial ETMR in the right parieto-occipital region. We describe clinical diagnosis, histological aspects, radiological features, and current management of this very aggressive tumor. CONCLUSION: Pediatric intracranial ETMR is a highly aggressive neoplasm, and it should be considered in the differential diagnosis of pediatric brain tumors.

Pediatric Intracranial Anaplastic Gangliogliomas: Illustrative Case and Systematic Review.

OBJECTIVE: We present an illustrative case of pediatric intracranial anaplastic ganglioglioma and systematically reviewed the current reported data of anaplastic ganglioglioma in the pediatric population. METHODS: A comprehensive literature search for our review was conducted using PubMed, Scopus, Web of Science, PsycINFO, Cochrane, and Embase databases. The search terms included "ganglioglioma," "anaplastic," "pediatrics," "children," and "intracranial." We used no limitations for date, type, or language. Reports of pediatric patients (age <19 years) with intracranial anaplastic gangliogliomas were included. Baseline patient demographic characteristics, clinical presentations, imaging characteristics, management strategies, and outcomes data were extracted. RESULTS: We included 24 studies involving 34 patients in the quantitative synthesis. The mean patient age was 9.18 ± 5.46 years (range, 0.16-18). A male predominance was observed (approximate male/female ratio, 1.61:1). Increased intracranial pressure was the most common symptom (n = 19; 55%), followed by seizures (n = 11; 32%). These tumors were most often found in the supratentorial compartment (n = 26; 76%). All 34 patients had undergone surgical removal. The mean follow-up was 22.2 months (range, 2-72). The mean overall survival duration was 43 months (95% confidence interval, 32.45%-55.31%; 1- and 3-year overall survival, 76.6% and 45.5%, respectively). The median event-free survival was 34 months (95% confidence interval, 10.6%-57.3%; 1- and 3-year event-free survival rate, 76.6% and 42.4%, respectively). CONCLUSIONS: Our results contribute to our understanding of the characteristics of this rare malignant tumor and show that anaplastic ganglioglioma should be considered in the differential diagnosis of intracranial tumors in pediatric patients.

A case of adult anaplastic cerebellar ganglioglioma.

BACKGROUND: Anaplastic posterior fossa ganglioglioma in adults is exceedingly rare. To date, only one case of adult anaplastic posterior fossa ganglioglioma has been reported in the English literature and none has been described at the cerebellum. To our knowledge, this report is the third case of malignant posterior fossa ganglioglioma in adults and the first at the cerebellum. In general, this entity can be misdiagnosed preoperatively as a primary posterior fossa neoplasm, and by reporting our clinical and radiographic observations we want to add to the existing literature on this rare entity. CASE DESCRIPTION: A 40-year-old man presented with a history of headaches and dizziness and progressive gait disturbance and was diagnosed with anaplastic ganglioglioma in the posterior fossa. CONCLUSIONS: Although rare, our case demonstrates that anaplastic ganglioglioma should be considered in the differential diagnosis of infratentorial tumors in adult patients.

Spinal epidural angiolipomas: Clinical characteristics, management and outcomes.

PURPOSE: The spinal epidural angiolipomas are rare expansive processes made of mature lipomatous and angiomatous elements. They often have a benign character. Their etiology, pathogenesis remains uncertain, and it is a cause of spinal cord compression. The magnetic resonance imaging is the most important neuroradiological examination. Histological examination is the only examination to confirm the diagnosis. Surgery is the treatment of choice. METHODS: A retrospective study of all patients operated on for a spinal epidural angiolipoma at the Department of Neurosurgery at the National Institute of Neurology of Tunis between January 2000 and December 2014 (15 years) was performed. The aim of this study is to describe the clinical, radiological, histological characteristics and the treatment of this tumor. RESULTS: A total of nine patients were operated from January 01, 2000 to November 30, 2014. The average age of our patients was 51 years with ages that ranged from 29 to 65 with a male predominance. The period between onset of symptoms and diagnosis ranged from 24 months with an average 12 months. Posterior localization of the tumor was seen in all patients. Surgical resection was performed for all cases. The postoperative course has been satisfactory, with a complete recovery of neurological functions in all patients. CONCLUSIONS: The spinal epidural angiolipomas is rare expansive process causing spinal cord compression. Treatment is exclusively surgical resection. The functional outcome of spinal epidural angiolipomas is particularly favorable with a complete neurological recovery is if the patient was quickly operated.

Secondary Chondrosarcoma of the Upper Thoracic Costovertebral Junction with Neural Foraminal Extension and Compressing the Spinal Cord.

BACKGROUND: Chondrosarcoma is a rare malignant tumor of bone. This family of tumors can be primary malignant tumors or a secondary malignant transformation of an underlying benign cartilage tumor. Secondary chondrosarcoma arising from a benign solitary costal osteochondroma is extremely rare. Data show that the reported incidence of costal osteochondroma is very low and they are usually found in the anterior region at the costochondral junction. To our knowledge, however, there have been no previous reports, in English literature, describing osteochondroma malignant transformation located in the thoracic costovertebral junction. CASE DESCRIPTION: We report the case of a man with chondrosarcoma arising from the malignant degeneration of an osteochondroma at the right first thoracic costovertebral junction with neural foraminal extension and compressing the spinal cord. CONCLUSIONS: Although it is rare in solitary osteochondromas of rib, malignant transformation must always be considered.

Intradural Extramedullary Capillary Hemangioma in the Upper Cervical Spine: First Report.

BACKGROUND: The occurrence of intradural extramedullary capillary hemangiomas is exceedingly rare. To date, only 39 cases of intradural extramedullary capillary hemangiomas have been reported in the English literature, and all of these cases have been described at the lumbar and thoracic spinal levels. To our knowledge, this report is the first case of capillary hemangiomas of the cervical spine in the literature. In general, this entity is misdiagnosed preoperatively as a neoplasm. CASE DESCRIPTION: A 29-year-old man presented with neck pain and progressive gait disturbance, and was diagnosed with an intradural extramedullary capillary hemangioma in the cervical region. CONCLUSIONS: Although rare, our case demonstrates that capillary hemangioma should be considered in the differential diagnosis of intradural extramedullary tumor of the cervical spine.

Primary sporadic Burkitt lymphoma of the orbit, clinical characteristics, management, and outcomes: a case study.

BACKGROUND: Involvement of the orbit with Burkitt's lymphoma is a very rare presentation of extra-nodal lymphoma. ILLUSTRATIVE CASE: We report a case of a 2-year-old female presented an unusual location of sporadic Burkitt's lymphoma arising in the orbital region. Diagnostic magnetic resonance imagining identified an oval-shaped mass on the lateral rectus of the right orbit that caused dislocation of eyeball, for which she underwent a biopsy from the periorbital swellings. The mass was histologically confirmed as Burkitt's lymphoma, and postoperative aggressive chemotherapy was initiated. We describe clinical diagnosis, histological aspects, radiological features, and current management of this rapidly growing malignant tumor. CONCLUSION: Because of the rapid progression of Burkitt lymphoma, and considering that it responds well to treatment, early recognition and appropriate management are important factors for survival and to preserve visual function.

Hydatid cyst of the cranial vault.

Only 0.5 to 2% of hydatid cysts are localized in the skeleton and of these, 3 to 4% are found in the skull. In this paper, the authors report a case of primary hydatidosis involving the cranial vault revealed by a bulging mass of the forehead and symptoms of raised intracranial pressure that occurred in a 22-year-old woman who came from a rural area. Through this case and literature review, the authors analyse the epidemiological, clinical and radiological aspects of skull hydatidosis. They conclude that hydatid cyst should be considered in the differential diagnosis of any soft tissue swelling or osteolytic lesion in the scalp of patients living in endemic areas.