Vertebral solitary bone plasmacytoma in a young adult with Trisomy 21: A case report.

Context: Solitary bone plasmacytoma (SBP) are rare lesions, accounting for less than 5% of all plasma cell proliferations. We describe a case of a 21-year-old female with Trisomy 21 presenting with cauda equina compression from an SBP. Findings: Solitary bone plasmacytoma (SBP) is a rare primary bone tumor. It is characterized by monoclonal proliferation of malignant plasma cells localized to a bone segment, without signs of systemic invasion. The vertebral location is the most common. It preferentially affects men during their 5th or 6th decade. Clinical relevance: We report the first association between solitary bone plasmacytoma and Trisomy 21.

Ewing's sarcoma of the cranial vault: a case report.

Ewing's sarcoma is a malignant tumor that mainly affects young patients. It represents 10% of primary malignant tumors of the bone and 3% of malignant tumors of the child. Cranial localization is extremely rare representing less than 1% of all the localizations. We report a case of a 10-year-old girl who presented with an intracranial hypertension syndrome with left parietal mass of progressive installation. The X-ray skull showed a lytic lesion with irregular margins involving the left parietal bone. Brain magnetic resonance imaging revealed extensive parietal bone destruction involving both the inner and outer tables. The girl was operated in emergency. Histological examination concluded to Ewing's Sarcoma. The resection was incomplete (R1). The girl received induction's chemotherapy. The cerebral scanner evaluation showed no abnormalities. Then, she received consolidation's chemotherapy with concomitant local radiation therapy. Currently, the girl is in complete remission with a seven-month decline.

[Cerebral gliosarcoma: clinico-pathologic study of 8 cases]. / Gliosarcome cérébral: etude clinico-pathologique de 8 cas.

BACKGROUND: Gliosarcomas are biphasic neoplasms composed of a glioblastoma admixed to a sarcomatous component with different lines of differentiation. Histogenesis of these tumors is still discussed. AIM: Our objective is to specify clinical and pathological characteristics of this rare neoplasm and to discuss its histogenesis. METHODS: Retrospective study of eight cases of gliosarcomas diagnosed between January 1998 and December 2004. Clinical, radiological, therapeutic and follow-up data were reviewed. Histological features and immunohistochemical results were also included in this review. RESULTS: Five patients were male, three women with a median age of 50.7 (range 31-74 years). Symptoms were dominated by intracranial hypertension and paralysis. The most common location was parietal or temporo-parietal (5 cases: 62.5%). Pathological exam including histochemical and immunohistochemical study confirmed the diagnosis of gliosarcoma in all cases. Sarcomatous component had features of fibrosarcoma in 5 cases, osteosarcoma in 2 cases and malignant fibrous histiocytoma in 1 case. All patients were treated by surgical excision (complete in five cases and partial in three cases). Adjuvant radiotherapy was received in three cases. One patient was lost on follow-up. Two patients died from postoperative. complications and the five remaining patients died with a medium follow up of 9 months (extremes: 2-24 months). CONCLUSION: Clinical, radiological and follow-up features of gliosarcomas share great similarities with glioblastomas. Histopathological, histochemical and immunohistochemical studies are helpful in accuracy diagnosis. Recent cytogenetic and molecular data support a monoclonal origin for these tumors.

Gliosarcoma with osteosarcomatous features: a short illustrated review.

INTRODUCTION: Gliosarcoma is a rare, malignant, biphasic brain tumor formed by both glioblastoma and sarcomatous components. Various lines of differentiation are described in the latter component, but most commonly fibrosarcomatous and pleomorphic sarcoma are present. Osteosarcomatous features are exceedingly rare. OBJECTIVE: We report a case of gliosarcoma with osteosarcomatous features in a 33-year-old woman. METHODS: Histologically, the sarcomatous portion displayed a typical pattern of fibrosarcoma associated with areas of osteoid formation. RESULTS AND CONCLUSION: Immunohistochemical glial fibrillary acid protein (GFAP) expression was seen only in the glioblastoma portion. Clinicopathological characteristics and radiological data of this rare condition were reviewed. Possible differential diagnoses and potential histogenesis were also discussed.

[Epithelioid schwannoma of the acoustic nerve: a case report]. / Schwannome épithélioïde du nerf acoustique: une observation.

Epithelioid schwannomas mainly affect peripheral nerves and are frequently malignant. They are rarely encountered intracranially. Only five cases involving the eight nerve have been reported in the literature. We report the sixth case of epithelioid schwannoma of the acoustic nerve diagnosed in a 57 year-old woman operated for neurinoma of the right cerebellopontine angle. The patient was readmitted for local recurrence nine months later. Histologically, the tumour was composed of round or polygonal atypic cells associated with spindle cell areas. In our case, the local recurrence, nuclear pleomorphism and especially the high percentage of positive cells with p53 (80%) and Ki67 (30%) antibodies support the aggressive nature of the lesion.