A long survival of a patient with brain metastasis of unknown site of the primary tumor.

Eighty percent of brain metastases (BM) are diagnosed in patients with known primary site of cancer. BM of unknown primary represents a difficult diagnosis. In up to 15% of patients with BM, the site of the primary tumor will not be detected despite investigations. The prognosis of this entity is very poor. We report here a case of a long survival of a patient with brain metastasis of unknown primary. The conclusion that can be drawn is that within BM of unknown primary exist patients with a very good prognosis that must be collected and published in order to base recommendations.

Subdural hematoma during therapy of gastro-intestinal stromal tumor (GIST) with Imatinib mesylate.

Imatinib mesylate is a widely used tyrosine-kinase inhibitor (TKI) in chronic myeloid leukemia (CML) treatment. Imatinib has contributed to complete and prolong cytogenetic responses so that it is now the standard treatment of CML. Recently, Imatinib mesylate has shown a significantly prolonged progression-free survival and overall survival in metastatic and locally advanced c-Kit positive gastro-intestinal stromal tumors (GISTs) and more recently a prolonged disease-free survival in operated high risk GIST. Imatinib is a welltolerated treatment with few side effects mainly gastro-intestinal symptoms (nausea, vomiting and diarrhea), headaches, rash and periorbital edema. Hemorrhage incidents are rare in patients treated with Imatinib. They are more frequently seen in CML patients. Hemorrhage incidents in CML include in many cases upper gastro-intestinal (GI) tract bleeding and central nervous system bleeding in rare ones. In GIST patients treated with Imatinib, hemorrhage incidents are exclusively made of upper GI tract bleeding consecutive to tumor perforation or necrosis. In our observation, we present the case of a subdural hematoma occurring in a patient treated with adjuvant Imatinib for a high risk localized gastric GIST. No other case of subdural hematoma in GIST treated with Imatinib has been reported in literature.

[Desmoplastic fibroma of the skull in an infant]. / Fibrome desmoplastique du crâne chez un enfant.

Desmoplastic fibromas are uncommon osseous tumors that seldom involve the skull. These tumors are histologically benign but locally aggressive with a propensity for recurrence if resection is partial. To our knowledge, only 16 cases have been reported in the literature, seven of which concerned children. We report a further case of a desmoplastic fibroma of the skull in a 3-year-old boy who presented with a right parietal mass. The CT scan showed a lytic mass with brain compression and cortical destruction. The patient underwent a craniectomy and complete mass resection. Histological diagnosis was desmoplastic fibroma. Postoperative progress was normal without recurrence 6 months later.

[14 cases of communicating syringomyelia associated with Chiari I malformation in children]. / 14 cas de syringomyélie communicante associée à une malformation de Chiari type I chez l'enfant.

The authors review their experience of 14 children with syringomyelia and associated Chiari I malformation observed during the last 21 years. Initial symptoms were motor weakness (57%), sensory signs (50%), scoliosis (50%) and bladder dysfunction (21.5%), while signs of admission were pyramidal signs (78.5%) with motor deficit (43%), spinal deformities (64.2%), syringomyelic syndrome (36%) and sensory deficit (21.5%). Four children were investigated with myelography, three with myelography and spinal CT Scan and the last seven cases with MRI. Ten children (71.4%) underwent a decompression of the Chiari malformation with or without drainage of the cyst and shunting of an associated hydrocephalus. Syringo-peritoneal shunts were used in 3 children and laminectomy with syringo-subarachnoid shunt in 1 case. The neurological symptoms improved in 57% of the cases, 2 patients deteriorated and 4 patients were lost to follow-up. The authors discuss the clinical symptomatology, the MRI study, the controversies concerning the treatment and concluded with their attitude concerning this pathology in children.

[Pseudo-oligodendrogliomatous meningioma. Report of 2 cases and review of the literature]. / Méningiome pseudo-oligodendrogliomateux. Etude de deux cas et revue de la littérature.

We report two cases of oligodendroglioma-like meningioma revealed by symptoms of increased intracranial pressure, progressive hemiparesia and partial epileptic seizures. Brain CT-scan or scintigraphy and carotid arteriography were suggestive of a convexity meningioma. One patient had received radiation treatment for scalp tinea capitis 25 years previously. In spite of complete surgical removal, the tumor recurred in both cases respectively 17 years and 18 months later. The two patients were operated again, and one underwent a complementary radiotherapy. Pathologic diagnosis was particularly difficult in the first case where the pattern at conventional histologic technics was that of oligodendroglioma. On the occasion of recurrence, immuno-histochemistry and ultrastructural studies were performed. The tumor was positive for epithelial membrane antigen (E.M.A) and cytokeratin, but was negative for glial fibrillary acidic (G.F.A.) protein, S 100 protein (S 100), neuron-specific enolase (N.S.E.), vimentin, anti-LEU-7 (N.H.K.1), and neurofilaments (N.F.). Electron microscopy showed closely adjacent cells with tonofilaments and numerous desmosomes. These findings permitted to establish the diagnosis of oligodendroglioma-like meningioma instead of oligodendroglioma. In the second case, the histologic pattern was also reminiscent of oligodendroglioma, but presence of few cellular whorls in some part of the tumor permitted the correct diagnosis. The pathogenesis of this atypical form of meningioma, its tendency for recurrence, and usefulness of radiotherapy are discussed and literature is reviewed.