Complete response to exemestane in a patient with a desmoid tumor.

Desmoid tumors are rare mesenchymal neoplasms without metastatic potential. Despite the benign nature of this condition, some patients develop disease progression despite all locoregional options for care. Aggressive forms of desmoid tumors may induce morbidity that can lead to physical impairment and mortality that is occasionally observed as a result of local infiltrative growth and tissue invasion, in particular with abdominal disease. Few therapeutic options are available for patients with recurrent/unresectable desmoid tumors. Several studies have suggested the potential benefit of antiestrogens such as tamoxifen in this setting. Here we report the first description of the efficacy of an aromatase inhibitor in a patient with a desmoid tumor.

Synovium CD20 expression is a potential new predictor of bone erosion progression in very-early arthritis treated by sequential DMARDs monotherapy -- a pilot study from the VErA cohort.

OBJECTIVE: Because available biomarkers (rheumatoid factors [RF], anti-cyclic citrullinated autoantibodies [anti-CCP2], erythrocyte sedimentation rate at 1st hour [ESR]/C-reactive peptide [CRP] and bone erosions) are insufficient to predict rheumatoid arthritis (RA) structural damage, to determine whether synovium expression of greater or equal to 1 markers could constitute new prognostic factor(s). METHOD: The study was conducted on 18 prospectively enrolled disease-modifying anti-rheumatic drug (DMARD)- and glucocorticoid-naïve, VErA cohort patients with very-early arthritis (median duration: 4months). Recorded at baseline were: clinical and biological (serum ESR, CRP, RF-isotypes, anti-CCP2, osteoprotegerin, receptor activator of nuclear κB-ligand [RANK-L] and cartilage oligomeric matrix protein [COMP] levels) data; synovium expression (HLA-DR, CD163, CD3, CD20, VEGF, osteoprotegerin, RANK-L, Bcl2 and global inflammation index) for a metacarpophalangeal joint-synovium biopsy. Baseline and 3-year hand-and-foot X-rays were graded with the van der Heijde-modified-Sharp score; the judgment criterion was its progression during follow-up. Pearson's product moment correlation statistics were used to test for association between paired samples. RESULTS: A baseline, a significant relationship was found between erosive damage and markers of B-cell activation, notably the synovium CD20 expression (r=0.68; P=0.0001). Quantified by the modified-Sharp erosion score variation, the 3-year structural damage progression was significantly correlated with: serum levels of RF-IgG (r=0.75; P=0.0003), -IgM (r=0.69; P=0.001), anti-CCP2 (r=0.53; P=0.02) and RANK-L (r=0.61; P=0.007); synovium CD20 expression (r=0.70; P=0.001). CONCLUSION: This analysis of the prognostic value of a large panel of synovium markers in a limited sample of prospectively followed, well-documented patients suggested that both synovial CD20 and serum RANK-L levels might be new predictors of structural damage progression in very-early RA.

[PEComas: report of five abdominopelvic cases]. / PEComes abdomino-pelviens: à propos d'une série de cinq cas.

We report five cases of abdomino-pelvic PEComas diagnosed in the last 10 years in the Rouen University Hospital. Four are hepatic and one is in a pelvic location which is unusual due to its strongly pigmented aspect. The tumors derived from "perivascular epithelioid cells" are rare. They are characterized by spindle or epithelioid cells in an immediate perivascular location. The immunochemistry is positive for HMB45, MelanA and smooth muscle Actin. The criteria for malignancy are infiltrative growth pattern, necrosis, high cellularity, high nuclear grade and mitotic activity. There are 8% of recurrence and 20% of metastasis (lung, bones, liver). This study presents the clinical, pathologic, immunohistochemical and molecular aspects of these PEComas and discusses the main differential diagnosis of the pigmented one.

Anatomical basis of the suprascapular nerve entrapment, and clinical relevance of the supraspinatus fascia.

INTRODUCTION: The entrapment of the suprascapular nerve (SSN) is commonly considered at the level of the suprascapular notch and more rarely in the spinoglenoid notch. Recent per-operative findings showed a compression of the SSN along its course in the supraspinatus fossa. The removal of a fascia for releasing the nerve between the suprascapular notch and spinoglenoid notch led us to purchase an anatomical study. MATERIALS AND METHODS: 30 cadaver shoulders have been dissected. The morphological features about the suprascapular notch, the supraspinatus fascia, and the spinoglenoid notch have been observed. Histological studies of the fascia and the spinoglenoid ligament have been performed. Morphometric parameters such as shape of the suprascapular notch, diameters of the SSN before and after the suprascapular notch, distance between the two notches, length of the course of the SSN into the supraspinatus fossa, diameters of the spinoglenoid notch have been measured. RESULTS: The shape of the suprascapular notch could be seen as "U"- or "V" as previously reported. The fascia was quite constant (completely identified in 29 shoulders) and was the lateral extension of the supraspinatus fascia. The SSN coursed between the bone and the fascia and was surrounded by fat tissue. This fascia was thickened at the level of the spinoglenoid notch and joined the infraspinatus fascia. The spinoglenoid ligament was seen in 28 shoulders. DISCUSSION AND CONCLUSION: In pathologic and post-trauma conditions, the fascia can be retracted or thickened and the SSN may be entrapped along its course in the supraspinatus fossa, between the suprascapular notch and the spinoglenoid notch and without any compression in any notch. These anatomical data lead us to consider that a tunnel syndrome may concern the SSN.

[Chordomas]. / Les chordomes.

Chordoma is a rare bone tumor, believed to derive from notochordal rests, which generally arises at the two extremities of axial skeleton. We present a literature review on chordomas. Diagnosis has been greatly improved by MRI and immunohistochemistry. Conversely, complementary immunohistochemistry, cytometry and cytogenetic techniques have failed to improve prognosis evaluation. Radical surgery with free surgical margins is the most accurate curative treatment. Progress in radiotherapy should offer new therapeutic perspectives in the future. The recognition of new entities such as giant notochordal rest or hamartoma, and notochordal cells benign tumor, can lead to confusion since there is no consensus regarding their nature and whether or not they correspond to chordoma precursors. Prudence should be the rule in order to avoid overtreatment.

[Recurring bone epithelioid hemangioma]. / Hémangiome épithélioïde osseux récidivant.

Bone vascular tumors are very rare. Epithelioid types are described according to their architecture, their degree of vascular differentiation, and their cytonuclear atypia. The include epithelioid hemangioma, epithelioid hemangioendothelioma, and angiosarcoma. We report a case of L4 corpus vertebral bone epithelioid hemangioma. The patient was a 25-year-old man with a tumor that recurred twice. The lesion was characterized by a vascular lumen lined by cells with regular nuclei and inflammatory infiltrates. Capillaries were lined by prominent epithelioid endothelial cells, associated with CD31+ and cytokeratin-.

PAS inclusions, immunoreactive tenascin and proliferative activity in low-grade chondrosarcomas.

To distinguish between chondrosarcoma (grade 1--borderline histology) and enchondroma, we examined six chondrosarcomas (grade 1--borderline histology) which looked like benign lesions. Their diagnosis, albeit based on clinical, radiologic and pathologic examinations, was not easily reached. Moreover, we examined six enchondromas and 11 chondrosarcomas, the diagnoses of which were straightforward. All cartilaginous tumors were studied, placing emphasis on PAS-positive intracytoplasmic globules. Anti-Ki67 proliferation-associated nuclear antigen antibody and tenascin antibody were applied. The following features were observed in low-grade chondrosarcomas: (1) masses of hyalin and/or myxoid cartilage invading spaces around the tumor, (2) host lamellar bone trabeculae surrounded by cartilage on all sides, (3) tumoral resorption of bone trabeculae. Intracytopasmic hyalin globules (ICG) were more frequently found in malignant than in benign neoplasm (p = 0.042). Moreover, tenascin matrix immunoreactivity was more likely to be observed in benign than in malignant neoplasm (p = 0.029). Ki67 immunoreactivity was more frequent in characterized than in low-grade chondrosarcomas or in enchondromas, where it was null (p = 0.0044).

Septic arthritis of the hip caused by Yersinia enterocolitica: a case report.

We report a case of bacteriologically documented hip infection caused by Yersinia enterocolitica. A 67-year-old male with a history of valvular disease was admitted for pain and motion range limitation in the left hip with a fever. No organisms were recovered by needle aspiration, but Yersinia enterocolitica grew in joint fluid obtained by surgical arthrotomy. Investigations of the gastrointestinal tract were normal, and there was no evidence of endocarditis. After 6 weeks of appropriate antibiotic therapy and immobilization with transtibial traction, the clinical and laboratory test abnormalities improved. However, the patient died from an intercurrent condition. Y. enterocolitica, a well-known cause of reactive arthritis, can cause septic arthritis.