Intraosseous schwannoma is a rare benign neoplasm, which most commonly arises in the head and neck region particularly the mandible, due to the long intraosseous path of sensory nerves in the mandible. We present a 27-year-old lady with an unusual presentation of an intraosseous schwannoma of the first metatarsal. There is only one report published previously of an intraossous schwannoma of the lesser metatarsal bone of the foot. A 27-year-old woman presented with painful left forefoot following a trip while walking. Plain radiographs demonstrated a pathological fracture through a lytic lesion of the first metatarsal of the left foot. MRI scan using axial T1-weighted spin echo and axial and sagittal T2-weighted gradient echo showed an amorphous mass occupying the medulla of the bone but with a breach of the plantar aspect of cortex with apparent localised destruction. Ultrasound-guided biopsy was performed. Haematoxylin and Eosin stained specimen sections showed a proliferation of spindle cells of alternating hypercellularity and hypocellularity. This case was managed by curettage and grafting with autograft and synthetic bone substitute. At two-year follow-up, the radiographs showed complete graft incorporation and a healed cyst. The patient was clinically asymptomatic with return of full functions. There were no clinico-radiological findings to suggest any recurrence. Due to rarity and non-specific clinico-radiological features, this case illustrates the necessity of a multi-disciplinary approach with an accurate histological diagnosis in combination with radiological and clinical appearances.
Between 1948 and 2004, we report 34 patients with Ewing’s sarcoma of pelvis accrued from Scottish Bone Tumour Registry, aiming to identify the prognostic factors and the influence of various treatment modalities on outcome. There were 19 male and 15 female patients at a mean age of 19 years (range, 3 to 48 years). The Pain was main presenting symptom in 30, swelling in 12 and restriction of hip movements in 11 patients. The commonest anatomical site was ilium. Local control was achieved by surgery, radiotherapy (n=25), chemotherapy (n=23) or a combination. The survival correlated significantly with chemotherapy protocols in favour of the group that received ifosamide (p<
0.01). Metastases at presentation was the most important factor determining survival (P<
0.01). Among the patients who presented without metastases (n=25), there was no statistically significant difference in survival based on the anatomical location of the tumour, age or sex. The mean time to lung metastases from the date of presentation was 13 months, while bone metastases presented at an average of 20 months. None of the patients with the metastasis or local recurrence survived. There were 5 local and 17 systemic (metastatic) relapses. The mean duration of survival was 13 months. With advances in imaging, aggressive chemotherapy, surgery and conformal radiotherapy which can deliver high dose of radiation with precision, it is possible to achieve a cure rate of more that 50% in non-metastatic pelvic Ewing’s sarcoma. The results of this study favour a middle-path regime combining all treatment modalities.
Limb salvage surgery includes all of the surgical procedures designed to accomplish removal of a malignant tumour and reconstruction of the limb in order to achieve an acceptable oncological, functional, and cosmetic result. The aim of this study was to evaluate the functional outcome following endoprosthetic replcament for primary upper &
lower extremity musculoskeletal neolplasms. Between 1983 and 2004, we found 68 patients from the Scottish Tumor Register having had an endoprosthetic reconstruction for upper and lower-extremity malignant musculoskeletal tumours, of which 32 were alive for performing functional assessment (lower extremity-26, upper extremity-6). The clinical, radiological and oncological outcomes were evaluated. The functional outcome was measured by the Musculoskeletal Tumor Society and Toronto Extremity Salvage Score. The average follow-up was 59 months (range, 1 to 21 years). There were 19 female and 13 male patients. These were anatomically distributed as around the knee (n=18 cases), hip (n=8) and shoulder (n=4). The most common diagnosis was chondrosarcoma (n=10) and osteosarcoma (n=11). Most of the patients were completely satisfied with their condition, with a decreased walking distance as the only notable restriction. There was no correlation between the functional outcome and the type or site of resection. Complications occurred in ten cases, including two cases of aseptic loosening and one case each of recurrent instability, sciatic nerve palsy and femoral nerve palsy. The median functional score using the Musculoskeletal Tumour Society system was 56% and Toronto Extremity Salvage Score was 72%. Limb salvage for malignant musculoskeletal tumours continues to pose therapeutic and oncological challenges with considerable functional issues, but the good function and local tumour control in most patients justifies its continued use.
Primary lymphomas of bone are uncommon malignancies with involvement of spine, long bones, pelvis, ribs and skull in decreasing order of frequency. We describe an unusual case who presented with recurrent knee effusions turned out to be a high grade B-cell non-Hodgkin lymphoma. A 34-year-old man is presented with painful recurrent knee effusions without any systemic aberration. Multiple aspirations were carried out to help knee pain and swelling with immediate response, but had negative cytology. Hematological and biochemical parameters were normal. Plain radiographic examination of the knee failed to show any bony abnormalities. An MRI scan of the knee showed extensive marrow oedema of the distal femur with breach in the anterior cortex. Subsequently, open biopsy of the lesion was carried out which confirmed the diagnosis of a high grade B-cell Non-Hodgkin lymphoma. Staging CT-scan of the chest, abdomen and pelvis failed to show any other lesions elsewhere. He was subsequently treated with the help of multi-drug chemotherapy and radiotherapy. At 7 months post treatment, the patient had no relapse and a repeat MRI at 7 months showed reduction in the size of lesion. This case illustrates the limitations of plain radiograph and the usefulness of MRI in the diagnosis of bony lymphoma. The diagnostic dilemmas while investigating recurrent knee effusions due to primary skeletal Non Hodgkin lymphoma should be resolved by timely MRI scan and histological diagnosis.
Does the type of implant have any correlation with critical fusion time of hallux metatarsophalangeal joint? There are few cadaveric biomechanical studies published in the literature assessing the strength and rigidity of different fixation methods. Although it is still unclear whether the amount of metal affects the fusion rate, the aim of this study was to assess whether using a supplementary dorsal ¼ tubular plate in addition to a compression screw gives any added rigidity to the fusion area leading to an earlier fusion. A retrospective analysis was conducted on the first metatarsophalangeal joint fusion in 26 consecutive patients (34 feet) between April 1998 to February 2002 comparing using single screw versus a screw supplemented with a dorsal ¼ tubular plate. There were 18 females and 8 males with a mean age of 51.5 years and a mean follow-up of 2.9 years. The final fusion was assessed clinically and radiologically by trans-articular trabeculation. There was a fusion rate of 97%. All patients except one had solid fusion. One case had non-union. Four cases had superficial wound infection, which settled down with appropriate antibiotic therapy. Paraesthesia over the dorso-medial aspect of the big toe in three patients and transfer metatarsalgia in two patients were documented. The type of implant did not show any direct correlation with the complication rate. There is no evidence to suggest in this study relating the amount of implant to final outcome. Therefore the choice of implant fixation can be at the discretion of the operating surgeon.
Necrotising fasciitis is a rapidly spreading, life-threatening condition. We report a case of necrotising fasciitis in a 71-year-old woman after a primary total hip replacement which became deeply infected as a result.
There are few reports describing dislocation of the metacarpophalangeal joint of the thumb in children. This study describes the clinical features and outcome of 37 such dislocations and correlates the radiological pattern with the type of dislocation. The mean age at injury was 7.3 years (3 to 13). A total of 33 children underwent closed reduction (11 under general anaesthesia). Four needed open reduction in two of which there was soft-tissue interposition. All cases obtained a good result. There was no infection, recurrent dislocation or significant stiffness. So-called ‘simple complete’ dislocations that present with the classic radiological finding of the joint at 90° dorsal angulation may be ‘complex complete’ injuries and require open reduction.
This study aimed to analyse clinical, radiological and histological features of 29 cases of pathological fractures of the long bones either presented as or occurred during the course of sarcomatous degeneration in Paget’s disease of bone utilising Scottish Bone Tumour Registry between 1950 to 2000. The mean age was 67.2 (range, 53–81) years with 16 males and 13 females. The commonest bone affected was the femur (n=13) followed by humeral (n=8), tibial (n=5), ilium (n=2) and os calcis (n=1) fracture. The mean duration of symptoms prior to presentation was two months. Fourteen patients presented with pain associated with a pathological fracture. One patient presented with painless lump and eleven with painful swelling followed by a pathological fracture. In the remaining three, the patients reported with a delayed or non-union of the pathological fracture. Nine patients presented acutely as pathological fracture being their main presenting symptom. The typical radiological appearance was a lytic (n=22). Histology showed predominantly osteosarcoma (n=17) followed by malignant fibrous histiocytoma (n=7) and pleomorphic sarcoma (n=5). Amputation was carried out in 16, internal fixation in 5 and replacement arthroplasty in two (1-cemented bipolar and 1-Charnley’s total hip arthroplasty). In addition, adjuvant radiotherapy in 13, adjuvant chemotherapy in 3 and combined radiotherapy-chemotherapy in 2 patients was instituted. The mean survival was 19.3 months (humeral=4.6, pelvifemoral=30, tibiocalcaneal=23.3 months). In conclusion, the majority of the pathological fractures in Paget’s sarcoma behave differently from their counterparts (non-Pagetic pathological fractures) with regard to their limb ablation treatment rather than stabilisation operation. The actual occurrence of a pathological fracture did not show to significantly affect the overall survivorship.
Between 1944 to 2003, eighty nine cases were registered with a diagnosis of Paget’s sarcoma in the Scottish Bone and Soft Tissue Tumour Registry. We found thirteen cases of sarcomatous degeneration of the spine (0.26% of the total bone tumour registry case) which were analysed in this study elaborating clinical, radiological and histopathological features. The mean age was 66.9 years (range 56 to 79 years). There were ten males and three females. There were seven cases involving sacral spine (63.6%), three cases involving lumbar vertebrae and two affecting dorsal spine. One case had diffuse dorso-lumbar involvement from D11 to L3 vertebrae. The mode of presentation was increasing low back pain (in all 13), unilateral sciatica (6, left sided-5, right sided-1), bilateral sciatica (2), lower limb weakness (8) and autonomic dysfunction (4, presented as chronic cauda equina syndrome). The majority of the cases (69.23%) were osteosarcomas. Out of these osteosarcomas, two showed giant cell rich matrix and one revealed predominant telengiectatic areas. Rest of the histological types was shared by chondrosarcoma, fibrosarcoma and malignant fibrous histiocytoma. Decompression laminectomy was performed in three cases. Eight patients had received radiotherapy. The mean survival was 3.93 months (range, 1 week to 7 months), nearly half to the whole Scottish Paget’s sarcoma series with a mean survival of 7.5 months. We found a constellation of symptomatology due to radiculo-medullary compression with a fatal evolution, predominantly lumbosacral involvement, predominantly osteosarcomatous histopathology with a poorest prognosis of all Paget’s sarcoma. Although, decompression laminectomy and adjuvant radiotherapy provided reasonable pain relief and palliation; however, there was no significant influence on the overall prognosis of the patients with Paget’s sarcoma of spine in the last six decades.
Ten cases of histologically proven chondromyxoid fibroma (CMF) of the Foot and Ankle with a mean follow-up of 6.1 years were reviewed. The mean age was 19 years (12.8 for skeletally immature and 25.2 for remaining skeletally mature patients). There were 6 males and 4 females. There were 5 phalangeal (3-proximal, 1-middle and 1-terminal phalanx), 3 metatarsal, 1 tarsal affecting body of os calcis and one case of distal tibial involvement. The mean delay in presentation was 16.7 months. The common modes of presentation were pain only (n=4), painful lump (n=4) and painless lump (n=2). Typical x-ray finding was an expansile, lobulated, cystic metaphyeal lesion. Cortical erosion was documented in 50% patients. Histology confirmed by several pathologists due to difficulty in diagnosis. There were two atypical CMF. In four cases, curettage alone was carried out, while 5 underwent curettage along with autogenous bone grafting. One case of distal phalangeal CMF had primary toe amputation. Postoperatively, calcaneal CMF had infection (treated with wound debridement and re-curettage). Two cases observed recurrences after 9 and 16 months from their initial operation. Both of them were male, had preliminary proximal phalangeal CMF, showed to have cortical erosion and were treated with curettage only. Re-curettage was done for one, while toe amputation through the metatarsal neck was performed for another recurrence. Foot phalangeal CMF which were initially treated with curettage only should be kept under close follow-up as we observed 20% recurrence rate within two year period. Cases with cortical erosion should be supplemented with autogenous bone grafting.
A 68 year old lady presented with one year history of painful right heel. She noticed slowly growing swelling resulting in broadening of the heel and difficulty in putting on her shoe-wear for a period of two months. On physical examination, there was a diffuse swelling over the posterior aspect of the right heel. The mass was firm to hard, mildly tender on palpation, fixed to bone and deep-seated. There was no erythema with increased warmth over the area of the mass. Plain x-ray of the affected heel revealed a pathological avulsion fracture of the posterosuperior corner of the calcaneus with typical Pagetoid features in the form of diffuse cortical thickening, coarsened trabeculae, loss of corticotrabecular differentiation and poorly demarcated osteolytic destruction. The lateral radiograph of the left heel showed normal radiological appearance of the os calcis. Chest x-ray was normal. The histological examination showed malignant fibrous histiocytoma characterised by the abundance of pleomorphic cells, spindle cells, lipid containing macrophages and touton type giant cells. The microvascular invasion and amount of mitosis declared the tumour as a high grade anaplastic, pleomorphic tumour. A below knee amputation was done approximately 3 weeks from the date of biopsy and after full staging. The patient died after surviving for a period of 6 years and 8 months from the date of biopsy. It is important to recognise the fact that an avulsion fracture can be pathological in nature and secondary to Paget’s sarcoma in patients with Paget’s disease. This case highlights that a high index of suspicion should be observed in patients presenting with a chronic heel pain who are shown to have pre-existing Paget’s disease on radiological grounds and who may have an avulsion fracture or other localising signs of underlying sarcomatous degeneration.
We describe a 65 year old gentleman presented with a three month history of painful lump in the right forearm which was subsequently confirmed to be osteosarcomatous degeneration in the Paget’s disease of the radius. A 65 year old gentleman presented with a three month history of painful lump in the right forearm with overlying redness thought by the patient to be the result of recent sun exposure. On physical examination, there was an 8 x 10 cm sized mass over the radial aspect of the right forearm. The mass was soft to firm, mildly tender to palpation, fixed to bone and deep-seated. There was erythema with increased warmth over the area of the mass. There was significant restriction of forearm supination and pronation. Neurovascular examination was normal. Chest x-ray was normal. Plain x-ray of the affected forearm revealed a mixed pattern (both sclerotic and lytic appearance) affecting the entire diaphyseal portion of the radius with soft tissue extension in the mid-shaft. There were alterations in the trabecular pattern of the bone, thickened cortices, encroachment of the medullary space and an increase in the size and deformity of the entire length of the radius with cortical destruction sparing the ulna. Bone scan showed multiple hot foci with an increase uptake in the sacrum, left hemipelvis, L3 vertebra and intensely increased uptake over the right radius. On the 10th day of presentation, an open incisional biopsy was done. Microscopy showed a high grade sarcomatous tumour with areas of osteoid formation. There was evidence of vascular invasion. The presence of preexisting Paget’s disease of bone was confirmed in the specimen. An above elbow amputation was done 4 weeks from the date of biopsy and after full staging. The patient died 16 months with multiple metastases from the date of diagnosis. Any patient with Paget’s disease who develops new pain in a previously pain-free area of Paget’s disease, or worsening of pain, or a painful progressive mass or a lytic area in sclerotic Paget’s bone should be evaluated for possible sarcomatous degeneration.
Fourteen cases of pigmented villonodular synovitis (PVNS) of the foot and ankle (between January 1957 and December 1999) accrued from Scottish bone tumor registry are presented with an average follow-up of 4.6 years aimed to analyse the clinical, radiological and histopathological features in order to investigate the clinical behaviour of PVNS in the foot and ankle, and to determine the factors influencing recurrence. The mean age was 26.4 years (range, 8 to 52 years). There were eight females and six males. The mean delay in presentation was 10.3 months. The anatomical sites were foot phalanges (n=2), tarso-metatarsal area (n=3) and hindfoot (n=9). Hindfoot cases comprised of 6 extra-articular soft tissue swelling around the ankle, two affecting the ankle joint and one involving the subtalar joint. There were eight (57.1%) cases presented with painless lump, five (35.7%) patients with painful lumps and one case with a lump associated with toe deformity. The clinical suspicion were ganglion, gout, soft tissue swelling (? tumour) and exostosis. Peri-articular tissue invasion and cortical infiltration was found in one third on plain films. CT scan showed multiple lytic lesions and MRI scan findings were consistent with extensive low signal soft tissue hypertrophy and bone erosion, two of which were suspected with synovial sarcoma. Excision of the lump was done in 4 cases with a complete recovery. Foot phalangeal PVNS were treated with toe amputation through metatarsophalangeal joint and no cases had recurrence of the lesion. There were two recurrences affecting the ankle and the subtalar joint. Recurrent ankle PVNS was treated with re-exploration, open synovectomy, curettage of talar cyst and autogenous bone grafting. The second recurrent case involving subtalar joint was treated with re-excision and curettage. Both recurred cases were primarily treated with intralesional excision for their diffuse variety. There were no recurrences in the nodular variety. Complete recovery was achieved in 85.7% case (12/14). A high index of suspicion for PVNS should be observed for cases presenting with a painless or painful mass in the foot and ankle region. Complete recovery can be achieved in the majority by complete excision. Toe amputation may be considered for foot phalangeal PVNS.