The Ewing's family of tumors (EFT) are malignant neoplasms affecting children and young adults. Most cases arise in the long bones or the pelvis. Primary EFT of head and neck is uncommon and primary sinonasal EFT is even rarer. Previous studies have not focused on the sinonasal region specifically, and the published literature on sinonasal EFT consists of sporadic case reports. Fourteen cases of sinonasal EFT were available and had H&Es for review and immunohistochemical stains for CD99, S100, keratins, synaptophysin and desmin. FISH or RT-PCR was performed for EWSR1 abnormalities on 8 cases. The 14 identified patients included 5 males and 9 females, ranging from 7-70 years of age (mean 32.4 years). Tumors involved nasal cavity (5), sinuses (5) or both (4). Five patients had dural, orbital or brain involvement. The majority involved bone radiologically and/or microscopically. All cases were composed of small cells with variable cytoplasmic clearing. Focal or prominent nesting was noted in most cases. All cases were positive for CD99. Keratins (AE1/3 and/or CAM5.2), S100 and synaptophysin were positive in 4, 3 and 5 cases, respectively. All cases were negative for desmin. The 8 cases tested by FISH or RT-PCR were positive for EWSR1 abnormalities. Follow-up in 8 patients ranged from 1-168 months (average 11.3 m) showing 1 death due to metastatic disease, 1 death due to local disease, 1 patient alive with metastases and 5 patients disease-free at last follow-up. Interestingly, however, an analysis of the literature suggests a better prognosis for sinonasal EFT than EFT overall.

Background Bony tumors of the foot account for approximately 3% of all osseous tumors. Diagnosis is frequently delayed as a result of lack of clinician familiarity and as a result of their rarity. The reasons for the delays, however, are unclear. Questions/purposes We therefore determined (1) how hindfoot tumors present and the specific reasons for delay in diagnosis; (2) whether the spectrum of disease varies between the talus and calcaneus; and (3) how these patients were treated. Methods We retrospectively reviewed the medical notes and imaging for all patients with 34 calcaneal and 23 talar tumors recorded in the Scottish Bone Tumour Registry. Demographics, presentation, investigation, histology, management, recurrence, and mortality were recorded.

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Background Bizarre parosteal osteochondromatous proliferation (BPOP) is a benign lesion of bone, and numerous questions remain unresolved regarding its etiology, diagnosis, and treatment. Questions/purposes We present the Scottish Bone Tumour Registry experience of this rare lesion. Patients and MethodsWe performed a retrospective analysis of the Scottish Bone Tumour Registry records. Histologic specimens were reexamined by a musculoskeletal pathologist. Radiographs were reevaluated by a musculoskeletal radiologist.Results From 1983 to 2009, 13 cases (13 patients; six male, seven female) were identified. Their ages ranged from 13 to 65 years. All patients presented with localized swelling. Pain was present in five. Antecedent trauma was present in two. Nine lesions affected the hand, three the foot, and one the tibial tuberosity. Twelve lesions were excised and one was curetted. There were seven recurrences of which six were excised. One lesion recurred a second time and was excised. There were no metastases. Radiographs showed densely mineralized lesions contiguous with an uninvolved cortex. Cortical breakthrough was present in one case and scalloping in another. Histologic analysis characteristically showed hypercellular cartilage with pleomorphism and calcification/ossification without atypia, bone undergoing maturation, and a spindle cell stroma. Conclusions BPOP is a rare benign lesion that probably is neoplastic, with no gender predilection, and affecting patients over a wide age range. Previously trauma was considered an etiologic factor, but this no longer seems to be the case. The rate of recurrence was 50%, which may indicate a more extensive resection is required for this locally aggressive lesion. No metastases were reported.

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