Introduction Ewing’s sarcoma family of tumors was first described by James Ewing in 1921. It is a rare tumor and no one center can hope to accrue sufficient numbers over a reasonable period of time to accurately review them.

Objectives A retrospective analysis of all Ewing’s sarcomas of the upper limb from the Scottish Bone Tumor registry (1940–2000), is presented. Attempts have been made to elaborate on prognostic factors, and to document improvements in survival with trends in local therapy over the study period.

Results The registry included 23 patients with Ewing’s sarcoma of the upper limb bones. Age at diagnosis ranged from 7–58 Yrs (mean −21.6 Yrs). 48% of lesions occurred in the second decade and 26 % in the third decade. Male: Female ratio was 2.8: 1. The commonest site involved was the proximal humerus (30%), followed by scapula (21%), radius (17%) and hand bones (13%).Resectable lesions underwent excision and adjuvant radio/chemo therapy. Cumulative 5 year survival for the series was 52%. Forearm and hand lesions which achieved complete excision had best results (75% five year survival). Proximal humeral lesions had 57 % survival. Scapular lesions had the worst prognosis (20% survival). Death was usually due to metastatic disease (diagnosed mean 12mts after initial presentation). Lesions presenting with pathological fractures, or having metastasis at diagnosis had extremely poor survival. There was an increasing use of surgery, and changes to chemotherapy (Euro-Ewing protocol) during the latter part of the study period. There were no toxic deaths reported.

Discussion Ewing’s sarcoma is a rare lesion. Any of the upper limb bones can be involved. Majority of lesions occur within the first 3 decades of life (87%). Survival is highly dependent on initial presentation. The key prognostic factor is the presence of detectable metastasis at diagnosis. Increased use of surgery and newer chemotherapeutic agents have decreased the local relapse rate over the period of study.

Background Osteosarcoma is the most common bone sarcoma, and the 3rd most common malignancy in children and adolescents. It accounts for 20% of primary malignant bone tumors.

Methods A retrospective review of osteosarcomas from the Scottish National Bone Tumor Registry (1940–2000) involving the upperlimb bones is presented. Patient demography, type and location of lesions, treatment options, recurrence and survival rates, and metastasis have been analysed.

Results 75 cases were identified from the registry. Sex incidence showed a slight male preponderance with male: female ratio 1.14: 1.Age at presentation ranged from 4–88 Yrs (mean 28.44 Yrs). 46.7% sarcomas occurred in the second decade (11–20 Yrs). The humerus was the bone most frequently involved (78.6% of lesions), and the proximal humerus the commonest site (60%). The scapula was involved in 9.3% and the forearm in 8%.A rare solitary lesion of the clavicle was encountered.17% presented with pathological fractures at diagnosis. Patients typically present with dull aching pain of weeks to months. All patients underwent radiological studies and diagnostic biopsy. Treatment modalities included amputation, limb-sparing surgery, adjuvant/neoadjuvant chemotherapy and radiotherapy. The cumulative 5 year survival for the series was 32%.Death was usually due to pulmonary and skeletal metastasis, and the mean survival in such patients was 21.5 mts. Patients presenting with metastatic pulmonary disease had poor prognosis. Limb-sparing surgery with wide margins does not compromise survival. Results with custom endoprosthesis are encouraging.

Discussion Osteosarcomas require a multidisciplinary approach to diagnosis and treatment to optimise survival. During the first half of the study period amputation was the mainstay of treatment with high incidence of mortality due to metastatic disease. Recent advances in neoadjuvant/adjuvant chemotherapy have improved the ability to perform limb –sparing resections, and disease free and overall survival rates have improved. Regular, long follow-up is indicated in these patients.

Introduction: Frozen shoulder is a general term denoting all causes of motion loss in the shoulder. As the syndrome is very common, many patients do not undergo detailed imaging studies before treatment. Some patients are managed by their GPs for long periods before being referred to a hospital.

Objectives: A series of 15 patients with primary neoplasms of the shoulder girdle mimicking frozen shoulder syndrome is presented.

Methods: A retrospective review of the Scottish bone tumor registry.

Results: There were 6 male and 9 female patients. The common presentation was pain and stiffness of the shoulder joint. Mean age at diagnosis was 46.63Yrs (range 23 – 71 Yrs). 73% were less than 50 Yrs of age. Only 2 gave history of trauma. Most received local steroids and physiotherapy before diagnosis. There were 10(66.6%) proximal humeral lesions and 5(33.3%) scapular lesions. Humeral lesions included chondrosarcoma (2), Ewing’s (2), lymphoma (2), chondroma (2) and osteoblastoma (1). Scapular lesions included chondrosarcoma (3), lymphoma (1) and fibromatosis (1).Scapular tumors involved older individuals. The mean delay in diagnosis after onset of symptoms was 15.8 mts (range 2 weeks- 48 months). All patients had X rays and CT/MRI. Treatment included a combination of surgery, chemotherapy and radiation. 3 patients with humeral lesions died at a mean of 20.6 mts, and 3 patients with scapular lesions died at a mean of 4.3 mts after diagnosis. The common cause of death was pulmonary metastasis.

Discussion: Many so called frozen shoulders are joints inhibited by pain rather than by true contracture. The commonest lesion to mimic a frozen shoulder is a slow growing low/middle grade chondrosarcoma. Young patients presenting with persistent pain or night pain must be examined for this possibility. Consideration should be given for further investigation before instituting treatment. Delay in diagnosis adversely affects survival. Surgeons are reminded that although rare, a tumor should be suspected when clinical presentations are unusual.

Background: The late effect of ionizing radiation on the development of sarcomas within the field of radiation is referred to as Post Irradiation Sarcoma (PRS).

Methods: A retrospective study from the Scottish Bone Tumor Registry (1940–2000) of PRS of the upper limb. The diagnostic criterion of Cahan was strictly followed and all patients followed up for 5 years.

Results: 11 female patients with previous history of radiotherapy (XRT) for carcinoma breast were identified as having PRS. The mean age at diagnosis was 68.7 years (51–80y). The latent period between irradiation and diagnosis ranged from 8 to 36 years (mean 15.6 y). All lesions occurred on the same side as breast malignancy. 6 lesions involved the proximal humerus and 5 the scapula. Histologically there were 7 osteosarcomas (65%), 1 chondrosarcoma, 1 spindlecell sarcoma and 2 dedifferentiated sarcomas. 2 humeral lesions underwent forequarter amputation, 2 excisions and endoprosthesis and 2 chemotherapy. Cumulative 5 year survival for humeral lesions was 33%. Survival was better with patients who achieved complete surgical margins. Majority of scapular lesions presented with either pulmonary/chest wall secondaries and were unresectable. Cumulative survival was poor ranging from 1 to 12 months (mean 6.2 months). De-differentiated lesions had the worst prognosis. Both orthovoltage and megavoltage XRT were implicated. It was not possible to analyze the relationship between total irradiation dose, individual fraction dose and incidence of PRS.

Discussion: PRS is an uncommon tumor with an incidence less than 1%, for patients who survive 5 years after XRT. Delay in diagnosis, aggressive nature, truncal location making extirpative surgery difficult and elderly patients all make the prognosis worse than that of primary sarcomas. As treatment of carcinoma of the breast evolves towards greater breast conservation it will be important to monitor the frequency of this complication more closely.

Background: Tumors and tumor-like lesions of the clavicle are uncommon and often present with a diagnostic problem. Although almost every type of tumor has been reported from this location the true incidence of primary tumors is 0.5–1%.

Materials and Methods: A retrospective review of the Scottish Bone Tumor Registry (1940–2000). All histologically confirmed lesions with minimum 5 year follow-up are included.

Results: 32 lesions were identified from the registry. The age at presentation varied from 4 years to 84 years. There were 7 myelomas, 10 lymphomas, 2 Ewing sarcomas,1 osteosarcoma, 1 chondrosarcoma, 1 Giant Cell tumor, 1 Aneurismal bone cyst, 1 chondroma, 1 unicameral bone cysts, 1 non-ossifying fibroma, and 6 eosinophilic granulomas. 3 cases presented with pathological fractures. Majority lesions involved the medial third. Malignant lesion underwent excision and adjuvant chemo-radiotherapy. The cumulative 5 year survival was 80% when adequate surgical margins were achieved. Death was usually due to soft tissue and skeletal metastasis. Benign lesions were treated with curettage and bone grafting or by partial excision of the clavicle. 3 cases of eosinophilic granulomas and the solitary chondroma required no further treatment after biopsy.

Discussion: Metastatic tumors are more common than primary tumors in this region. Among primary lesions, malignant tumors are more common than benign. All ages can be affected. The subcutaneous location of the clavicle usually aids in early diagnosis. Anatomically, the medial third was the commonest site involved. Prognosis is good if adequate surgical margins can be achieved and if there is no distal spread. Partial or total claviclectomy usually results in good functional outcomes.

Background: Chondrosarcoma is the second most frequent primary malignant tumor of bone. The biologic evolution of these tumors is slow, requiring long follow up for meaningful survival analysis.

Methods: The clinicopathologic profiles of 84 (41 male, 45 female/M: F, 1:1.09) chondrosarcomas of the upper limb from the Scottish Bone Tumor Registry (1940–2000) are presented.

Results: The mean age at presentation was 54.8Yrs (range 12–85yrs).The proximal humerus was the most frequent anatomical site (30.2%).21% lesions involved the scapula, and 34% small bones of the hand. Local pain was the most frequently reported initial symptom. All patients were followed up for a minimum 60 mts. Radiographically; chondrosarcomas had a characteristic appearance of bone expansion and cortical thickening. The cumulative 5 year survival was 82.5%. Local recurrence developed in19.7 % (mean 40 mts after initial presentation), and distal metastasis in 16.2%.Hand tumors rarely had metastasis. Tumor breach at time of surgery increased risk of local recurrence. Histological grade was an important factor in predictor of local recurrence and metastasis.

Discussion: Chondrosarcomas are highly diverse tumors ranging from slow growing non- metastasizing lesions to highly aggressive metastasizing sarcomas. With adequate initial surgical intervention, chondrosarcoma is primarily a local disease with a low metastatic rate. Hand lesions have best prognosis.

Introduction: Types of cancer occurring in children are very different from those occurring in adults. Reliable data on incidence and mortality of childhood cancers is sparse.

Methods: A review of all primary malignant bone tumors in children (0–14 Yrs) from the Scottish National Bone Tumor Registry (1940–2000) is presented. Epidemiology, clinical presentation, pathology, radiological characteristics, treatment options, recurrence rates, geographic distribution and incidence are discussed.

Results: Excluding myelomas and lymphomas, 154 patients were identified. 122 (80.2%) lesions were benign, and 30 (19.7%) malignant. There were 20 osteosarcomas (66%), 8 Ewing’s sarcomas (26%), 1 chondrosarcoma and 1 fibrosarcoma.

Osteosarcoma – Age at presentation ranged from 4–14 Yrs (mean 10.3Yrs). 70 % involved 10–14 Yrs. Male: Female incidence was 1.5:1. 75% of lesions involved the proximal humerus.15 % presented with pathological fractures. The mean cumulative 5 year survival was 20%. Death was usually due to pulmonary metastasis.65% had pulmonary metastasis at a mean 6.3 mts after diagnosis. Mean survival in these patients was 14mts. Survival was superior with adjuvant chemotherapy and wide excision.

Ewing’s Sarcomas- Age at presentation ranged from 7–14 (mean 11.2Yrs).71.4% involved 10–14Yrs. Male: Female was 1.6:1. 62.5% lesions involved the humerus and 25 % the radius and 12.5% the scapula. The mean cumulative 5 year survival was 37.5%.Death was due to pulmonary or skeletal metastasis (mean 21.5mts). All patients had radiotherapy and chemotherapy.

Chondrosarcoma- A rare low-grade chondrosarcoma of the proximal humerus was encountered. Excision and grafting yielded good results.

Discussion: Majority of bone lesions in this age group are benign. Osteosarcomas and Ewing’s sarcomas predominate among the malignant (93%).The peak incidence occurs with adolescent growth spurt. Mean age is lower for osteosarcomas, and the sex incidence for both show male preponderance. Survival rates for Ewing’s was higher than for osteosarcomas. Pulmonary involvement at presentation had worst prognosis.

Introduction: Musculoskeletal tumours and lesions resembling tumours present exceptionally difficult diagnostic and treatment problems in children. Presenting symptoms are usually a poor guide to diagnosis, and are often misleading.

Methods: A review of benign bone lesions of the upper limb in the peadiatric age group (0–14 yrs) from the Scottish Bone Tumour Registry is presented.

Results: Excluding myelomas and lymphomas, the registry included 154 lesions. 122 (80.2%) were benign including, 25 unicameral bone cysts, 22 aneurysmal bone cysts, 31 chondromas, 15 osteochondromas, 12 diaphyseal aclasis, 7 osteoidosteomas, 2 chondroblastomas, 2 nonossifying fibromas, and 1 each of giant cell tumour, eosinophilic granuloma and chondromyxoid fibroma.

Unicameral cysts- Age at presentation ranged from 3–14 yrs (mean 9.4).Male: Female incidence was 7.3:1. 92% involved the proximal humerus. 88 % presented with fracture. Recurrence rate after curettage was 20% at a mean of 10.8 mts.

Aneurysmal cysts – Age at presentation ranged from 5–14 yrs (mean 9.3). 55% involved the proximal humerus, and 20% hand bones. 45% presented with fracture. Recurrance rate after curettage was 31.8% at a mean of 15.1mts.

Chondromas- Age at presentation ranged from 3–14 yrs (mean 10.4).20 lesions were solitary and 11 multiple. Among solitary lesions, 16 were central (75% hand) and 4 parosteal.

Osteochondromas- Age at presentation ranged from 1–14 yrs (mean 10.1).Male: female ratio was 2.75:1. 53.3% involved the proximal humerus.

Osteoid Osteoma- Age at presentation ranged from 5–13 yrs (mean 10.14). Male: female ratio was 1:6. 42% involved the hand.

Discussion: Benign tumours compose majority of bone neoplasms in this age group. Unicameral and aneurysmal cysts, along with enchondromas were most frequent. Although the age at presentation shows variations with tumour type, the incidence seems to peak at 9–10 Yrs. Careful analysis of the clinical presentation, radiological characteristics and histology are required to distinguish them from malignant lesions.

Background: Bizarre parosteal osteochondromatous proliferation [Nora’s lesion] is a rare tumourous lesion with aggressive growth that affects primarily the small tubular bones of extremities and often recurs after excision. It is often confused with chondrosarcomas, parosteal osteosarcomas and florid reactive periostitis.

Materials and Methods: The clinical and pathological findings in eight cases of Nora’s lesion from the Scottish bone tumour registry are presented.

Results: There were eight lesions reported in the registry. (5 female & 3 male). Anatomically there was 1 metacarpal lesion, 2 proximal phalanx lesions, 3 middle phalanx lesions and 2 distal phalanx lesions. Age at presentation ranged from 25 – 65 years (mean 41y). The common mode of presentation was pain and swelling for a period of 6 months to 2 years. There was no history of trauma. Two patients were initially misdiagnosed, one as synovial chondromatosis and the other as an osteochondroma. Treatment was in the form of an excision biopsy. The recurrence rate after excision was 62.5%. 60 % of recurrences occurred within 12 mts of excision. One phalangeal lesion recurred twice after excision. There was no distal metastasis.

Discussion: Bizarre parosteal osteochondromatous proliferation is histologically composed of bony, chondroid and fibrous proliferation, with a high cellular density and bizarre chondrocytes but is devoid of cellular atypia or necrosis. It is a rare pseudotumorous lesion, with typical clinical, radiographic and histopathology findings similar to those of other benign and malignant tumours. The rarity of this lesion and the high local recurrence rate necessitate an accurate histological diagnosis and complete excision.

Background: Osteoid Osteomas were first described by Bergstrand (1930), and were later elaborated by Jaffe (1935). They account for 12% of all benign bone tumors. Approximately 6–8% of lesions occur in the hand, and can be extremely difficult to diagnose.

Aims: A retrospective review of osteoid osteomas from the Scottish Bone Tumor Registry is presented focusing on the unusual clinical features of lesions of the carpus and hand.

Results: 19 patients (8 male, 11 female) were identified from the registry. The mean age at presentation was 27.5 Yrs (10–56 Yrs). 78% of lesions occurred within 2nd–3rd decade of life. Pain over the involved area most noticeably at night, was the usual presentation. 21% gave history of associated trauma. Phalangeal lesions accounted for 68.4% tumors with the proximal phalanx predominating.4 lesions involved the distal phalanx. There were 3 metacarpal and 3 carpal lesions. Carpal bones involved included the Capitate (2), and the Trapezium (1). Clubbing was noticed in distal phalanx lesions. The mean time from presentation to diagnosis was 5.6 months. 9 patients were initially misdiagnosed as having either a traumatic or infective condition.3 patients required a bone scan for diagnosis. All patients underwent surgical excision, with 2 requiring bone grafting. Follow up ranged from 1–9 Yrs (mean 3Yrs). There were no instances of recurrence or spontaneous regression.

Discussion: Osteoid Osteoma is an infrequent tumor of the hand. Absence of typical pain pattern, unobtrusive radiographic features (absence of reactive bone, inapparent nidus), and variations in the histological picture make diagnosis in this region difficult. Lesions are easily misdiagnosed and successful treatment often delayed. Isotope bone scan is diagnostic and CT accurately visualizes the nidus. Awareness of these characteristics may aid early diagnosis. Surgical excision is usually curative and has excellent prognosis.