Peripheral type of primitive neuroectodermal tumour arising from the left orbital floor.

Primitive neuroectodermal tumours (PNETs) are rare tumours that originate from primitive neural crest cells. They are usually found in children below ten years of age. Peripheral PNETs (pPNETs) occur in soft tissues of the body, but have the same genetic changes as Ewing's sarcoma of the bone (now called soft tissue Ewing's sarcoma). They commonly present in the thoracopulmonary region, abdomen, pelvis and the extremities. The head and neck regions may also be involved. Our case demonstrates a PNET in the peripheral tissue arising from the left orbital floor and spreading locally to involve the left maxillofacial region, cheek and gum. The incidence of pPNETs is likely to be under-reported in the literature. Recent diagnostic advances, including cytogenetic and immunohistochemical analysis, have allowed these tumours to be distinguished from other small, poorly differentiated round cell tumours such as rhabdomyosarcoma, lymphoma and poorly differentiated synovial sarcoma.

Assessment of the efficacy of pamidronate in ankylosing spondylitis: an open prospective trial.

INTRODUCTION: Bisphosphonates have anti-inflammatory properties in arthritic conditions. This study was conducted to assess the therapeutic potential of intravenous pamidronate in nonsteroidal anti-inflammatory drug (NSAID) refractory/intolerant cases of ankylosing spondylitis (AS). METHODS: A total of 35 NSAID refractory/intolerant AS patients with Bath AS Disease Activity Index (BASDAI) score 4 or above were recruited for the study. Monthly pamidronate infusions (60 mg) were administered to the patients for six months. Treatment outcomes were assessed by comparing baseline values with the values after six infusions using BASDAI, Bath AS Functional Index (BASFI), Metrology Index (BASMI) and Global Score (BAS-G), C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). An improvement was defined according to the Assessments in Ankylosing Spondylitis (ASAS)-20 and BASDAI-50. RESULTS: 26 patients received all the six infusions. Of these, 22 (85 percent) achieved ASAS-20 and 20 (77 percent) achieved BASDAI-50 responses. Decrements were noted in the mean BASDAI (56.4 percent), BASFI (52.66 percent), BASMI (55.72 percent), BAS-G (66.71 percent), ESR (52.12 percent) and CRP (72.84 percent) after six months. The tender and swollen joint counts of 14 (54 percent) patients with peripheral arthritis were respectively reduced to a mean value of 0.85 and nil, from the baseline of 2.57 and 1.2. Early feel good response was noted in 16 (62 percent) patients within 48 hours of the first infusion. Fever, arthralgia and myalgia were observed in six cases after the first infusion, and in one case, after the second infusion. These symptoms resolved spontaneously within 24 hours. CONCLUSION: Intravenous pamidronate has good efficacy for the treatment of AS.