G. De Pinieux

Primary cutaneous Ewing sarcoma: a systematic review focused on treatment and outcome.

Summary Background  Despite intensive treatment, the overall prognosis of Ewing sarcoma of the bone remains poor. Primary cutaneous Ewing sarcoma very rarely occurs and the prognosis has been reported to be better in some small series. All cases of Ewing sarcoma are currently treated in the same way, regardless of their location.

Coloration noire des os du crâne et de la face

INTRODUCTION We report the case of a patient with a craniofacial black bone disease. This was discovered accidentally during a coronal approach. CASE REPORT A 38-year-old patient was referred to our unit for facial palsy having appeared 10 years before. Rehabilitation of the facial palsy was performed with a lengthening temporal myoplasty and lengthening of the upper eyelid elevator. An unusual black color of the skull was observed at incision of the coronal approach. Subperiostal dissection of skull and malars confirmed the presence of a black bone disease. A postoperative history revealed minocycline intake (200mg per day) during 3 years. DISCUSSION This craniofacial black bone disease was caused by minocycline intake. The originality of this case is to see directly the entire craniofacial skeleton black. This abnormal pigmentation may affect various organs or tissues. Bone pigmentation is irreversible unlike that of the mouth mucosa or of the skin. This abnormal pigmentation is usually discovered accidentally.

Nævus de Becker et hypoplasie hémimaxillaire : association fortuite ou syndrome ?

INTRODUCTION Beckers nevus is a dermatological disease sometimes associated with a malformation such as hemifacial bone dysplasia. OBSERVATION A 11-year-old male patient presented with a delayed growth of the left maxillary and dental impactions. At 15 years of age, Beckers nevus appeared on the upper torso, both shoulders and cheeks. Le Fort I segmented osteotomy with an iliac bone graft was complicated because of maxillary dysplasia. DISCUSSION The chronology of our patients cutaneous and maxillary abnormalities was similar to previously reported cases. Conversely, the Beckers nevus-bone dysplasia association has been reported only twice. It may not be a coincidence and could belong to a Beckers nevus syndrome.

Estrategia diagnóstica, anatomía patológica y clasificación de los tumores óseos primarios

La conducta medica inicial ante el hallazgo de un tumor oseo primario es fundamental, pues permite definir la mejor estrategia diagnostica y terapeutica. Desde luego, la asistencia medica ha de ser multidisciplinaria. Esto conduce a la eleccion del metodo de biopsia diagnostica y a la via de acceso mas adecuada. En el menor tiempo posible, la biopsia se enviara en «estado fresco» al patologo, quien ha de repartir el material (fijacion, congelacion, etc.) segun la cantidad, la calidad o los presuntos diagnosticos. Se conocen unas 50 formas de tumores oseos primarios, distribuidas en 12 clases principales. Algunas de estas formas, sobre todo los condrosarcomas, cuentan con una clasificacion histopronostica especifica.