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.

Recurrent subcutaneous metastasis of a convexity meningioma: a case report and literature review.

Subcutaneous metastasis in the operative route after intracranial meningioma resection is extremely rare. Here we describe the case of a 69-year-old male who was operated on in our department for a convexity meningioma that spread a few years after surgery in the soft tissues next to the craniotomy site. Twenty-one other cases have been found in the medical literature. In this article, we discuss mechanisms of growth, presentation and management of subcutaneous meningiomas.

Cranio-cervical decompression associated with non-instrumented occipito-C2 fusion in children with mucopolysaccharidoses: Report of twenty-one cases.

Background: Mucopolysaccharidosis (MPS) is a multisystemic storage disorder of glycosaminoglycan deposits. Infiltration of the dura mater and supporting ligaments caused spinal cord compression and consecutive myelopathy, especially at the cranio-cervical junction (CCJ). Craniocervical instability and posterior decompression often raise the problem of fixation in children. The main purpose of this paper was to report the result of an original technique of occipito-cervical arthrodesis using a cranial halo-cast system in pediatric population. Methods: We recorded 21 patients with cervical myelopathy. All of them had spinal cord decompression by enlargement of the foramen magnum, C1 laminectomy, and occipito-C2 fusion using corticocancellous bone graft. Only one child has an extended laminectomy from C1 to C3. The occiput-C2 arthrodesis was stabilized by the cranial halo-cast system. This immobilization was performed preoperatively and kept for three months then switched to rigid cervical collar. Clinical assessment, including the Goel grade and mJOA, radiographs and magnetic resonance imaging were performed before surgery. The occipito-cervical arthrodesis was controlled by standard X-rays and CT scan. Results: According to the type of mucopolysaccharidosis, the patients were divided into MPS type I: n= 3, II: n=7, IV: n=11. The mean age of patients at surgery was 6.76 years. All mucopolysaccharidoses cases required a foramen magnum decompression by craniectomy, C1 laminectomy and occipito-C2 arthrodesis. As major complications, a child had immediate post-operative paraplegia due to spinal cord ischemia. The postoperative follow-up ranged from 1.5 to 4 years, with an average of 3.3 years. The average preoperative mJOA score was 8.9, and it improved to 14 points at the last follow-up. Conclusions: Satisfactory fusion and good clinical results were obtained with the 2-stage approach to CCJ anomalies.

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.

Hypopituitarism secondary to a pituitary metastasis in a young woman with an invasive breast carcinoma.

Hypopituitarism secondary to a pituitary metastasis is rare and difficult to diagnose since its symptoms are nonspecific. The presence of visual deficits and nerve palsies should suggest the presence of a pituitary metastasis in a cancer patient.

Acute Paraplegia Revealing a Hemorrhagic Cauda Equina Paragangliomas.

Cauda equina paragangliomas are rare neuroendocrine benign and slow-growing tumors. Acute paraplegia, occurring because of sudden intratumoral hemorrhage, represents an extremely rare clinical picture of this disease. We report the case of a 64-year-old male presenting with a 5-day acute lower back pain, sciatica, and leg weakness. Spinal imaging showed an intradural mass of the cauda equina region and an emergent surgical treatment was achieved. The lesion was removed "en bloc," and subarachnoid blood was noticed during surgery. The postoperative course was uneventful, with complete regression of pain and progressive motor recovery. The histological study revealed typical microscopic and immunohistochemical features of paragangliomas.

Hodgkin Lymphoma revealed by epidural spinal cord compression.

CONTEXT: Hodgkin Lymphoma is rarely diagnosed as spinal cord compression syndrome. Caused by an epidural mass, this complication is often encountered in a late stage of the disease. We report the case of a 40-year-old man presenting with symptoms of low thoracic spinal cord compression due to an epidural tumor on the MRI. FINDINGS: Emergent surgery was undertaken on this patient, consisting in laminectomy and tumor resection. After surgery, pain relief and mild neurological improvement were noticed. The histological study revealed a Hodgkin Lymphoma and the patient was referred to chemotherapy and radiotherapy. CONCLUSION: Though chemotherapy is the gold standard treatment for Hodgkin Lymphoma, surgical spinal decompression may be required in epidural involvement of the disease. Diagnosis may be suspected in the presence of lymphadenopathy and general health decay.

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.