[Fibrous dysplasia of the rib. Ten case reports]. / Dysplasie fibreuse costale. A propos de dix cas.

PURPOSE OF THE STUDY: Fibrous dyplasia is a rare sporadic disease accounting for 0.8% of primary bone tumors. This benign pseudotumor results from proliferation of fibrous tissue in bone and the production of immature bone tissue without an osteoblastic crown. The disease can involve one or more bones, ribs are rarely involved. We report a series of 10 cases of costal fibrous dysplasia. CASE REPORTS: This series of 10 cases was collected over a period of 10 years (1996-2005). There were five men and five women, mean age 38.4 years, range 27-52 years. One rib was involved in eight patients, two ribs in two. Pain was the most frequent symptom. Plain X-rays showed signs suggestive of fibrous dysplasia. To confirm the diagnosis, rib resection was performed in all ten patients. Pathology examination provided the diagnosis. The postoperative period was uneventful in all patients and all are recurrence free at mean 50 months follow-up. DISCUSSION: Management of fibrous dysplasia can be simple surveillance in the majority of patients. Nevertheless, in the event of a single focus, particularly in a rib, fibrous dysplasia can raise a difficult problem of differential diagnosis with malignant tumors. Surgical resection is therefore required in selected cases.

[Desmoid fibromatosis of the posterior mediastinum]. / Fibromatose de type desmoïde du médiastin postérieur.

Fibromatosis are uncommon connective tissue tumours arising from musculo-aponeurotic tissue and characterised by spindle cell fibroblastic and myofibroblastic proliferation. The exact aetiology is unknown but several factors are considered to be positively correlated with their development and growth (genetic and hormonal factors and trauma). Although they are considered histologically benign they behave aggressively locally and relapse repeatedly after surgical excision. Mediastinal localisation is very rare. We describe a case of de novo fibromatosis of the posterior mediastinum in a 61 year old man with no history of thoracotomy or trauma. Although mediastinal fibromatosis is very uncommon physicians should be aware of this disease in order to ensure appropriate surgical treatment.