[Desmoid tumor of the thoracic wall. A case manifesting as painful stiffness of the shoulder]. / Tumeur desmoïde de la paroi thoracique. Un cas révélé par un enraidissement douloureux de l'épaule.

BACKGROUND: Desmoid tumors are uncommon fibromatous tumors arising from musculoaponeurotic tissue characterized by spindle cell fibroblast and myofibroblast proliferation. The cause is unknown (trauma, hormonal factors, genetic anomaly...). Locally invasive, they tend to recur increasing morbidity or even mortality. CASE REPORT: Stiff shoulder resulting from blockage of the scapulothoracic articulation was the inaugural sign. Three-phase bone scintigraphy demonstrated early uptake in the soft tissue in contact with the scapula. Magnetic resonance imaging and biopsy confirmed the diagnosis of desmoid tumor. Complete resection was performed. No adjuvant radiotherapy nor hormone treatment were given since no antiestrogen receptor antibodies were identified on the surgical specimen. DISCUSSION: The clinical manifestation (stiff shoulder) and the scapular localization observed in this case are unusual for desmoid tumors. Initially tendinopathy of the shoulder, acromio-clavicular arthropathy and capsular retraction of the shoulder joint had been entertained. Early diagnosis and wide surgical resection are indicated in desmoid tumors.

Education for outpatients with rheumatic diseases. Evaluation of short-term and medium-term results.

This prospective study was conducted to evaluate the short- and medium-term impact of the outpatient education sessions that have been available at the Cochin Teaching Hospital since 1992. Each patient was asked to complete a ten-item anonymous questionnaire at the beginning of the education session (evaluation 0), immediately after the session (evaluation 1), after six months (evaluation 2) and after 12 months (evaluation 3). Mean numbers of correct answers per patient were calculated. Eight education sessions were evaluated (osteoarthritis, osteoporosis, rheumatoid arthritis, back pain, conservative treatments, surgical treatments, low-calorie diets and high-calcium diets). One hundred twenty-four patients completed the first two questionnaires, 94 (75.80%) completed the six-month questionnaire and 75 (60.5%) completed the 12-month questionnaire. Mean numbers of correct answers were as follows: 5.7 before the session, 7.3 after the session (p = 0.0001), 7.2 after six months (p = 0.0001), and 7.9 after 12 months (p = 0.0001). These results demonstrate that the education sessions significantly improved patient knowledge in the short and medium term. Their impact on quality of life is being evaluated.

[Idiopathic massive osteolysis of the femur. Syndrome called Gorham-Stout syndrome]. / Ostéolyse massive idiopathique du fémur. Syndrome dit de Gorham-Stout.

A case of massive osteolysis (disappearing bone disease, phantom bone, Gorham's massive osteolysis) was observed in a young african man with two lesions of the femur. This is a very rare syndrome characterized by an acute spontaneous resorption of bone, without any sign of malignant or infectious disorder. The lesion may develop in any part of the skeleton, with extensive bone loss, but is benign. Bone is replaced by haemangiomatous tissue. The diagnosis lead on the association of clinical, radiological and histological signs. The pathogenesis is still unknown and the treatment remains controversial. Osteolysis stops after a few years, but this is unpredictable; in our case, we have proposed prosthetic replacement, associated with bone grafting and irradiation.

[Essential osteolysis. Study of 3 cases and a classification trial]. / L'ostéolyse essentielle. Etude de 3 cas et essai de classification.

Disappearing bone disease is a rare syndrome, characterized by an idiopathic and spontaneous progressive resorption of bone, for which different etiologies must be considered. The classification of this disease is difficult; acro-osteolysis involves the phalanges or the carpus and tarsus. Massive osteolysis corresponds to diffuse multicentric cystic hemangiomatosis and Gorham and Stout's disease. We describe two cases of acro-osteolysis and one of Gorham and Stout's disease. The mechanism of disappearing bone disease remains unknown and no treatment has yet been found to stop its evolution.