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Background: Osteosarcoma is a malignant tumor of mesenchymatous origin that chiefly affects the metaphysis of long bones. The extraskeletal form of the disease is rare, and even rarer is a cutaneous site, whether metastatic or primary. Herein, we report a new case of primary cutaneous osteosarcoma., Patients and Methods: A 54-year-old woman presented a hard subcutaneous nodular tumor on her left arm noted 1 year earlier. The diagnosis of cutaneous osteosarcoma was made on the basis of histological analysis of the lesion, which showed a sarcomatous dermal-hypodermal proliferation secreting osteoid. Clinical and radiological staging ruled out any extra-cutaneous spread, particularly to bone, thus confirming the primary cutaneous nature of the osteosarcoma., Discussion: Primary cutaneous osteosarcoma is a rare tumor, diagnosis of which is normally based on histopathological features., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Central nervous system medulloepithelioma is a rare, highly malignant childhood tumor. It might be confused with medulloblastoma or other primitive neuroectodermal tumors, but it is quite particular by its clinical, radiological, and pathological features. The mean survival varies depending on whether or not a gross-total resection is possible. Adjuvant radiochemotherapy is often indicated. Only two reported cases in the literature survived beyond 4 years after treatment by gross total resection and radiotherapy without chemotherapy. We report three cases of supratentorial medulloepithelioma occurring in three children aged 11-17 years. Two patients underwent a gross-total resection followed by radiotherapy and survived more than 4 years after treatment. The third case had, however, recurred twice within the 1st postoperative month despite a complete resection each time and metastasis to the lung developed. Chemotherapy was then carried out after the third procedure and the patient died 7 months later., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Most hemangiopericytomas (HPC) are located in the musculoskeletal system and the skin, while the location in the central nervous system (CNS) is rare. The latter represents 2 to 4% in large series of meningeal tumors, thus accounting for less than 1% of all CNS tumors. In the central nervous system, tumors with a hemangiopericytomatous histolopathological pattern can be either hemangiopericytomas or solitary fibrous tumors. CNS-HPCs have a relentless tendency for local recurrence and metastases outside the CNS. Metastasis can also appear many years after adequate treatment of the primary tumor. We present a pathological study of eight patients with CNS-HPC and compare our results with corresponding published data. The CNS-HPC group consisted of three males and five females with a mean age of 36.75 years. The tumors were supratentorial in four cases, infratentorial in two cases, tentorial in one case and located in the spinal cord in the last one. Histologically, CNS-HPCs were similar to their soft tissue counterparts. One case demonstrated increased cellularity, marked nuclear hyperchromasia and marked cellular pleomorphism with infiltration of the cerebellum. All patients underwent surgery with gross-total resection in all cases. No patients received postoperative radiation therapy. Only four patients recurred locally after six, seven and eight months, and five years. Our study presents the pathological features of CNS-HPC as a distinct entity from both meningioma and solitary fibrous tumors. A comparative review of literature with our results is discussed.

Sacrococcygeal teratomas are rare congenital tumors generally discovered at birth. Exceptionally these tumors are observed in adults. Radical resection must be performed to avoid potentially malignant recurrence even if the primary lesion was benign. We report a case of a benign sacroccygeal teratoma diagnosed in a 25-year-old male and discuss clinical features, pathological considerations and treatment.

Chordoid meningioma is an uncommon histopathological variant of meningioma frequently confused with chordoma. Recurrence is not uncommon. The aim of our study is to present the clinical and pathological characteristics of this entity and a review of the literature. We report two cases of chordoid meningioma occurring in two women aged 47 and 63 years. Both women presented neurological signs without systemic disease. Radiological findings were temporoparietal and temporofrontal lesions with intense enhancement after contrast injection. Complete surgical resection was performed. Histopathologic findings were consistent with chordoid meningioma.

Extraneural diffusion of glioblastoma is very unusual. Extracranial metastases generally occur after craniotomy or a long survival time. Metastases usually involve the lungs, pleura, lymph nodes and occasionally the bone. We report herein a case of bone dissemination occurring 18 month after the initial diagnosis and treatment of supratentorial glioblastoma.

Ganglioglioma is usually a well differentiated slowly growing mixed neuronal and glial neoplasm corresponding to WHO grade I or II. However, some gangliogliomas are considered to be WHO grade III because they exhibit anaplastic features in their glial component. Finally there are exceptionally rare cases of newly diagnosed gangliogliomas with grade IV changes in the glial component. We report a case of a 25-year-old woman with a family history of neurofibromatosis who presented initially with a World Health Organization grade IV anaplastic ganglioglioma (a mixed ganglion cell multiform tumor glioblastoma). Despite aggressive management, the patient died of disease in a relatively short period. Histologically, two cell populations were noted: a predominant glial component consisting in a multiform glioblastoma and ganglion cells supporting a diagnosis of ganglioglioma. Immunohistochemical analysis clearly distinguished the two tumor cell populations. Although other cases of grade III gangliogliomas and twelve cases of grade IV gangliogliomas have been reported, the present case is exceptional in that, to our knowledge, it is the second case of a patient who presented initially with a composite grade IV ganglioglioma and who was clinically followed up to the time of death. This case allows direct comparison between the histological findings in a multiform glioblastoma and a ganglioglioma. It also documents the aggressive biologic behavior of this complex neoplasm.

Introduction: The association of Behçet's disease and of Crohn's disease is exceptional, and raises a nosological problem., Case Report: A 24 year-old female developed since 1996 chronic diarrhea, abdominal pain, recurrent oral and genital ulcer and polyarthralgia. Endoscopic colonoscopy examination showed a diffuse colitis. Histological examination revealed epitheloid granuloma without vasculitis compatible with a Crohn's disease. The patient was treated with oral prednisone (1mg/kg/day). In November 1998, she was admitted for diarrhea. Many pseudofolliculitis lesions, uveitis and positive pathergy test were noted. The HLA was B 51. The diagnosis of Crohn's disease associated with Behçet's disease was made. She was treated with high doses of prednisone (1mg/kg/day and 6 monthly intravenous pulses of cyclophosphamide). Skin lesions and diarrhea improved within few days, and cleared completely within five months. Presently, the patient remains clinically free of disease., Conclusion: While being of different pathogenic origin, Behçet's disease and Crohn's disease may coexist within one and the same patient and cause diagnostic and therapeutic problems.

The association of Behçet's disease and non-Hodgkin's lymphoma is very rare. The first case of primary intestinal lymphoma with intestinal Behçet's disease is reported. A 37 year-old woman had been treated for Behçet's disease with colchicine for 2 years. In July 1997, she developed uveitis and was treated by monthly intravenous pulses of cyclophosphamide for 6 months, but uveitis persisted; so cyclophosphamide was replaced by cyclosporin 300 mg/day. One month later, she suffered from diarrhea. Colonoscopy showed ileocoecal ulcerations. Histological examination of surgical biopsy revealed B large cell type lympocytic malignant lymphoma and vasculitis lesions compatible with intestinal Behçet's disease. Cyclosporin was stopped and treatment with prednisone was instituted. The relationship between non-Hodgkin's lymphoma and immunosuppressive drugs in Behçet's disease is discussed.

We report a case of minimal deviation adenocarcinoma of the uterine cervix in a 32 year old women who present a Peutz Jeghers syndrome. This patient also had fibroadenoma of the breast and dystrophic mastopathy. This case represent an example of predisposition to developing tumors of the Peutz Jeghers syndrome.

The synovial lipoma are uncommon articular tumors of unknown origin. We report two cases of synovial lipoma arborescens arisen in a man and a woman respectively 41 and 27 years old. Both had already consulted for a tumefaction progressively increasing in volume. It was accompanied in the first case by a laxity in the knee joint. Radiography and arthroscopy showed an important hyperplasia of the articular synovia. A total synovectomy was performed in both cases. The synovia had a hairy aspect and was extremely thickened. It weighted 1.5 kg in the first case and its section had a fatty aspect. The histological examination confirmed the diagnosis of synovial lipoma arborescens. The observed aspect of the two tumors and particularly their volume, which was very important in the first case are arguments in favor of their tumoral nature.

Tumors of ceruminous glands, called "ceruminoma" are rare. They arise from the ceruminous glands of the external auditory canal. They realise a group of glandular tumors diversified in histologic pattern and prognosis. Three patients with ceruminous gland tumors are reported: one case of ceruminous adenoma, one case of adenocarcinoma and adenoid cystic carcinoma in the third one.

Digestive manifestations are uncommon in Behçet's disease. The authors reported 3 male patients between 20 and 30 years with Behçet's disease who developed surgical abdomen. Emergency surgical intervention found perfored ileal ulceration. Histological study of resection piece showed mainly intestinal angitis with periphlebitis, venous thrombosis and obliterant endarteritis.

The authors report a case of aspergillus granuloma of the brain, in a 28 year old woman, simulating a meningioma. Preoperative diagnosis of aspergilloma is difficult. However, it may be suspected in a patient who has associated pulmonary and paranasal sinus fungal infection. Peroperatively it may be confused with a brain tumour. The lesions usually are in the frontal lobes. Diagnosis can be made only by surgical biopsy with identification of fungal elements. Granuloma induce a good host response, and a high capacity to elaborate antibodies.

The clinical, follow up and diagnostic features of a case of chemodectoma (paraganglioma) of the orbit are reviewed. 30 cases are reported in the literature. Chemodectoma may arise in any area of the body where non chromaffin paraganglion structures are situated. These are localized in the orbit of the chimpanzee but not in the orbit of man. Malignant behavior of chemodectoma has been a controversial subject, with most authors considering them to be benign tumors. Some authors have diagnosed locally recurrent and infiltrative tumors as malignant. The authors report one case of chemodectoma of the orbit. A 5 year old child has had right exophthalmos. She was treated by tumorectomy. The follow-up is marked by recurrence of the tumor and infiltration of the right hemi-face.