A tragical paediatric case history of intraorbital and intracranial epithelioid hemangioendothelioma.

Epithelioid hemangioendothelioma (EHE) is a rare tumor of intermediate malignancy. We report a case of intracranial and intraorbitar EHE. A 3-year-old girl presented with a 3-month history of progressive left exophthalmia. Neuroradiologic imaging (CT scan and MRI) showed an intraorbitar process with an intense enhancement extending to temporal fossa, ethmoidal bone, nasal fossa, maxillary sinus, and cavernous sinus. The angiogram was normal. The tumor was operated through subfrontal approach but only a partial resection was performed. The histological diagnosis was epithelioid hemangioendothelioma. The patient was neurologically intact 2 months after surgery without exophtalmia. However 4 months after surgery he displayed a fall of the right eye vision with intense headache. Control CT scan showed persistence of important tumoral residue. Epithelioid hemangioendothelioma is a hemorrhagic tumor. Total removal must be possible. Otherwise, we recommend a complementary chemoradiotherapy and close followup. We propose this interesting case history of a tragical evolution of EHE in contradiction with what has already been reported.

[Intracranial hydatid cysts in children: a report of 9 cases]. / Kystes hydatiques cérébraux de l'enfant (à propos de 9 cas).

PURPOSE: To illustrate the value of cross-sectional imaging (CT, MRI) for the diagnosis and follow-up of intracranial hydatid cysts in children. MATERIALS AND METHODS: Retrospective study of 9 cases of intracranial hydatid cysts in children seen over a period of 8 years. Precontrast and postcontrast 5 mm thick axial CT images were obtained in 7 cases. Noncontrast sagittal, axial and coronal T1W and T2W images were obtained in 2 cases. RESULTS: Mean patient age was 7.5 years. Intracranial hypertension was the main presenting clinical symptom. A single supratentorial cyst with significant mass effect upon the ventricular system and midline structures was observed in all cases. All patients underwent surgery with good outcome in all cases. CONCLUSION: CT is the imaging modality of choice for diagnosis and postoperative follow-up of intracranial hydatid cysts in children. MRI is most helpful for further characterization when multiple or atypical cysts are present to optimize management.

[Benign sacrococcygeal teratoma in a child: a case report with a review of the literature]. / Tératome sacrococcygien bénin chez l'enfant : revue de littérature à propos d'un cas.

Sacrococcygeal teratomas are rare congenital tumors, generally discovered at birth. These tumors are seldom observed in children. Radical resection must be performed to avoid potentially malignant recurrence even if the primary lesion is benign. We report a case in an 8-year-old girl who did not have a past medical history. Since the age of 2 years, she presented a progressive sacral tumefaction with no neurological deficit. The MRI showed a large sacrococcygeal cyst in hypointense-signal T1-weighted imaging with no contrast enhancement, and a hyperintense signal in T2-weighted imaging. At surgery, the tumor was totally removed. The intraoperative aspect was that of a viscous cyst. The histological study showed a sacrococcygeal teratoma.

[Giant cerebellar tuberculoma mimicking a malignant tumor]. / Tuberculome géant du cervelet simulant une tumeur maligne.

BACKGROUND: Isolated central nervous system (CNS) tuberculoma is rare. Central nervous system tuberculosis (TB) is associated with high morbidity and mortality despite modern methods of detection and treatment. The authors report a case of a giant cerebellar tuberculoma mimicking a malignant tumor and review the literature. OBSERVATION: A six-year-old girl, with no past medical history, vaccinated for her age, presented with a three-month history of occipitocervical cephalalgia, complicated by gait disturbances. The MRI showed a left cerebellar tumor suggestive of a medulloblastoma. At surgery, a nodular, avascular lesion was found and pathological examination confirmed tuberculoma. Intracranial tuberculoma is an uncommon variety of central nervous system tuberculosis. The prognosis is related to the rapidity of diagnosis, surgical resection and the complementary antituberculosis treatment. CONCLUSION: Intracranial tuberculoma is an uncommon variety of central nervous system infection. Prognosis is improved by a quick diagnosis, surgical removal, and associated antituberculoma therapy.

[Intracranial aneurysm associated with aortic coarctation: a case report and literature review]. / Anévrisme intracrânien associé à une coarctation de l'aorte: à propos d'un cas et revue de la littérature.

We report a new and rare case associating an intracranial aneurysm and aortic coarctation. Based on this case and a review of the literature we discuss the clinical aspects, the pathogenesis and the management of this disorder predominantly observed in the adolescent and young adult population.

[Symptomatic intramedullary arachnoid cyst. Report of two cases and literature review]. / Kyste arachnoïdien intramédullaire symptomatique. A propos de deux cas et résumé de la littérature.

OBJECTIVE: The objective of this work is to present and discuss the rare situation of curable medullary compression with favorable prognosis. MATERIAL AND METHOD: Two cases of thoracic intramedullary arachnoid cysts are described. Clinical, paraclinical, therapeutics and outcome features are discussed with a review of the literature. RESULTS: Two children, 4 and 8 years old, with an uneventful history were admitted for progressive spastic paraparesia. MRI demonstrated a thoracic intramedullary cystic lesion at level T3-T4 in both patients. The cyst was emptied with partial cyst wall resection via dorsomedial myelotomy. The pathology examination confirmed the diagnosis of arachnoid cyst. The initial clinical signs resolved completely in both patients. CONCLUSION: Thought in children, intramedullary arachnoidian cyst is a potential cause of medullary compression. Direct surgery is the treatment of choice.

Cerebellar dermoid cyst with occipital dermal sinus. Report of two pediatric cases.

Intracranial dermoid cyst is a rare entity accounting for 0.1-0.7% of all intracranial tumors. The most common location is in the posterior fossa, at or near the midline. We present 2 pediatric cases with dermal sinus. The first case presented with clinical signs of increased intracranial pressure and cerebellar symptoms. CT scan showed a large and regular midline posterior fossa cyst without contrast enhancement. The second case was revealed by recurrent meningitis. CT scan showed a midline vermis low-density mass with capsular contrast enhancement. Dermal sinus was found in 2 cases. Complete surgical removal was performed followed, in a second operation, by ventriculoperitoneal shunt in 2 cases. There was no postoperative complication in our patients. The aim of this study is to discuss the clinical aspects of dermoid cyst, especially in cases with dermal sinus, and to review the therapeutic strategies in case of associated hydrocephalus.

[Giant osteoid osteoma of the posterior skull base. A case report and literature review]. / Ostéome ostéoïde géant de l'étage postérieur de la base du crâne. A propos d'un cas et revue de la littérature.

BACKGROUND: Osteoid osteoma is a benign bone neoplasm which is seen in the long bones. It is rarely described in the cranium. Posterior skull base osteoma is extremely rare and has been anecdotally reported. OBSERVATION: We report a rare case of a large osteoid osteoma of the petro-occipital area in a 26-year-old man. He presented with clinical sign of raised intracranial pressure, cerebellar symptoms and large left retro-auricular swelling. Cerebral MRI shows a giant lesion of the posterior cerebral fossa with destruction of the left petrous bone and the left side of the occipital bone. Large excision was performed through a retro-sigmoid approach. In our knowledge, there is no similar case reported in the literature to date. CONCLUSION: Although benign and rare, osteoid osteoma can present with neurological deficit due to mass effect and involvement of nervous structures especially in the posterior skull base.

[Extradural thoracic arachnoid cyst. Case report and review of the literature]. / Kyste arachnoïdien extra-dural thoracique. A propos d'un cas et revue de la littérature.

OBJECTIVE: To report an unusual and asymptomatic cause of cord compression treated surgically with good clinical outcome. METHODS AND MATERIAL: We report a case of extradural arachnoid cyst in the thoracic region and review the literature on the clinical, neuroradiologic and therapeutic features. RESULTS: A 17-year-old man, with unremarkable past medical history was referred to our institution of Neurosurgery, CHU Mohammed-VI, Marrakech, with progressive spastic paraparesis. Magnetic resonance imaging (MRI) showed a posterior extradural cystic lesion in the thoracic region from T6-T7 thoracic vertebra. The cyst was completely removed by posterior approach. An arachnoid cyst was confirmed at the histological study. Neurological symptoms improved after surgery. CONCLUSION: Given its infrequency, the diagnosis of arachnoid cyst should be suspected when a cystic lesion causes cord compression. Surgery is the treatment of choice, providing good clinical outcome.

[Metastatic and hemorrhagic brain arteriovenous fistulae due to a choriocarcinoma. Case report]. / Fistule artérioveineuse cérébrale métastatique hémorragique d'origine choriocarcinomateuse.

Brain metastasis of choriocarinoma is uncommon. These tumors develop in women of childbearing age and commonly produce signs and symptoms of subarachnoid hemorrhage, intracerebral hemorrhage, or brain tumor. Diagnosis can be established by histologic study of operative swabs and bioassay of the patient's blood, urine and cerebrospinal fluid for chorionic gonadotropin. This condition is highly chemo- and radiosensitive. We report the case of a 36-year-old woman with intracranial neoplastic fistulae. Rupture occurred 3 days after spontaneous abortion at 3 months of pregnancy. The patient presented with hemiplegia, aphasia and unclear consciousness. Left fronto-parietal hematoma was diagnosed on the CT scan, and cerebral angiography showed an arteriovenous intracranial fistulae. The hematoma and angioma were surgically removed successfully. The histological examination showed a metastatic choriocarcinoma. Surgery was followed by chemotherapy and radiotherapy. After 6 years of follow-up, complete remission has been obtained.

Tumor-like tuberculosis of the sacrum.

Isolated tuberculosis of the sacrum in a 43-year-old woman manifested as functional impairment of the right lower limb. Sacral tuberculosis is rare in patients with no history of tuberculosis. Another unusual feature was the tumor-like aspect of the lesion, with diffuse, ill-defined osteolysis of a large part of the sacrum and extension to the presacral soft tissues responsible for rectal displacement.