Giant cell tumour of a phalanx in the foot: a case report

Giant cell tumours are uncommon benign osseous neoplasias with an obscure origin. They mostly occur in the epiphyses of long bones after skeletal maturity. Phalangeal bones are a very rare primary site of involvement. The authors report a case of giant cell tumour involving a phalangeal bone in the foot and review the presentation, distinctive features and treatment of this tumour when occurring in this location. A 28-year-old female patient was seen with an aggressive giant cell tumour of the first phalangeal bone of the third ray of her left foot. En bloc resection of the third ray was performed without bone grafting. The patient has now been free from disease for 12 years. When giant cell tumour occurs in such a location, it appears to represent a distinct, more aggressive form of tumour. Because of the higher risks for local recurrence, treatment should be aggressive.     

Surgical strategy in primary retroperitoneal tumours

Sixty-nine patients with primary retroperitoneal tumours (17 benign, 52 malignant including 4 malignant tumours of uncertain origin) were reviewed to determine the best form of surgical strategy. Total resection was performed in 88 per cent of benign cases and in 65 per cent of malignant cases. In 62 per cent of the total resections for malignant tumours, en bloc excision included adjacent organs or anatomical structures. Operative mortality rate (in terms of the total number of operations performed) was 5 per cent. Postoperative complications occurred in 14 per cent and recurrences in 35 per cent. The overall 5-year survival rate was 67 per cent in patients with totally resected tumours and zero in patients whose tumours were treated by partial resection or biopsy. An aggressive surgical approach aimed at total excision of the tumour is the best form of therapy currently available. In the totally resected retroperitoneal tumour, the use of adjuvant radiotherapy and/or chemotherapy depends on the grade of the malignancy and clearance as assessed histologically. Careful follow-up based on the use of computerized axial tomography and ultrasound allows early identification of recurrence at a stage when the recurrence is amenable to total resection.     

Endoprosthetic replacement of the distal humerus following resection of bone tumours

Between 1988 and 2006, 18 patients had a custom-made endoprosthetic replacement of the distal humerus for bone tumours at our institution. There were 11 primary malignant neoplasms, six secondary deposits, and one benign aggressive tumour. The mean follow-up was for 4.4 years (1 to 18.2). Complications occurred in nine patients and included aseptic loosening in three (16.6%), local recurrence in two (11%), infection in two (11%), neuropraxia of the radial nerve in one (5.5%) and a peri-prosthetic fracture in one (5.5%). Excision was inadequate in four patients (22%), all of which developed local recurrence and/or metastases. There were seven deaths from the primary disease after a mean of 2.3 years (1 to 5), one of whom had an above-elbow amputation for local recurrence seven months before death. The remaining six had satisfactory elbow function at their last follow-up. The 11 living patients were evaluated using the Musculoskeletal Tumour Society and Toronto Extremity Salvage scoring systems. The mean scores achieved were 76% (67% to 87%) and 73% (59% to 79%), respectively. Overall, 17 of 18 patients had significant improvement in the degree of their pain following operation. Custom-made endoprosthetic reconstruction of the elbow for bone tumours is a viable treatment in carefully selected patients. It maintains satisfactory function and provides good pain relief.     

Metachronous multicentric giant cell tumour of the upper extremity in a skeletally immature girl : A rare presentation

Giant Cell tumour (GCT) or Osteoclastoma is a benign locally aggressive tumour with a tendency for local recurrence. Long tubular bones (75-90%) are frequent sites of involvement. GCT constitutes 5% of all primary bone tumours. Metachronous multicentric giant cell tumour of bone is a rare entity. Multicentric GCT, in contrast to unifocal GCT, has a tendency to involve the small bones of hands and feet, involving the metaphysis/diaphysis of long bones and tends to occur in a slightly younger population. We report a young girl presenting with metachronous multicentric recurrent benign GCT, with the lesions involving the ipsilateral right hand and distal humerus. She was successfully treated with an aggressive surgical approach (en-bloc resection).     

Management of the giant-cell tumours of the distal radius

BACKGROUND: Giant-cell tumour of the distal radius is a rare neoplasm that affects the peri-articular metaphysial region of the bone. Curettage alone or with bone grafting has been reported to be associated with high incidence of local recurrence in these tumours. In the present series, we report the results of curettage only as the treatment for primary giant-cell tumour of the distal radius carried out at a single centre. PATIENTS AND RESULTS: A total of 287 patients with giant-cell tumour have been referred to us for treatment over the last 28 years; 24 of these were found to have lesion in the distal radius. One patient underwent endoprosthetic replacement of the distal radius. The remaining 23 patients underwent curettage of the primary neoplasm. Four out of the 23 (17%) patients developed local recurrence of disease, The mean time to local recurrence was 17 months (range, 9-27 months). Complications such as collapse of the articular cartilage are more common in patients with an extensive soft tissue component of the tumour. CONCLUSIONS: Curettage alone is adequate treatment for the majority of patients with giant-cell tumours of the distal radius but some form of stabilisation may be required in the presence of extensive bone destruction.     

Clinical and immunohistochemical characteristics of benign giant cell tumour of bone with pulmonary metastases: case series

PURPOSE: Giant cell tumour of bone with pulmonary metastases is rare. However, some patients die of pulmonary metastases, and histological examination cannot distinguish between benign tumour and malignant metastases. In this study, we present clinical and immunohistochemical findings associated with giant cell tumour of bone with pulmonary metastases. METHODS: Five patients with benign giant cell tumour of bone with pulmonary metastases (one man and 4 women) were studied. Patients' ages ranged between 20 and 23 years (mean age, 21.8 years). Tumours were in the distal femur in 2 cases, and in the proximal tibia, distal tibia, and lumbar spine in one case each. The tissue specimens from primary tumours, recurrent tumours, and pulmonary metastases were studied using immunohistochemical techniques. RESULTS: Three of the 5 primary tumours were of the spontaneous regression or growth cessation type, or the continuously slow-growing type, showing 4.2% to 6.2% of positive cells for Ki-67 after immunohistochemical staining. However, 2 patients with the rapid-growing type of disease died of pulmonary metastases; their primary, recurrent, and metastatic tumour specimens contained 9.0% to 11.5% of positive cells for Ki-67. CONCLUSION: Three of the 5 primary tumours had a benign clinical pattern and immunohistochemistry. Two of the 5 patients died of pulmonary metastases, which had an aggressive clinical pattern and a high prevalence of positive cells in Ki-67. Examination of Ki-67 should be carried out for aggressive type of giant cell tumour.     

Metatarsal giant cell tumour in a 7-year-old child: a case report

Giant cell tumours are uncommon benign osseous neoplasias with an obscure origin. They mostly occur in the epiphyses of long bones after skeletal maturity. Occurrence in a metatarsal bone in a child less than 10 years old is quite exceptional. The authors report a case of a 7-year-old girl with an aggressive giant cell tumour of the first metatarsal bone of her right foot. Intralesional excision by curettage and grafting with morselised iliac cortical and cancellous bone was performed. The girl is now disease free since 7 years. Giant cell tumours in this location and age group are rare; they appear to represent a distinct, more aggressive form of tumour. They should be considered in the differential diagnosis of a destructive bony lesion in skeletally immature patients. Curettage and bone grafting with morcelised iliac cortical and cancellous bone remains a treatment option, despite a high potential for recurrence.     

Neuroendocrine tumour of the inferior vena cava

Malignant tumours of the inferior vena cava (IVC) are rare. Secondary involvement of the IVC by adjacent retroperitoneal tumours is more common than the occurrence of leiomyosarcomas of the IVC. The authors present a case of a secondary neuroendocrine tumour of the infrarenal vena cava that presented with pulmonary emboli. To their knowledge this presentation has not been reported previously. The tumour was technically resectable and reconstruction of the IVC was not required. No primary tumour was identified. As palliative treatment, an aggressive surgical approach is recommended when the primary tumour is controlled or is not readily identified.     

Adenocarcinoma of the urinary bladder

We report 40 patients seen over a 15-year period with a histological diagnosis of adenocarcinoma of the bladder; 18 patients had primary adenocarcinoma of the bladder, arising either from the urachus or from glandular metaplasia of the urothelium, and the other 22 had secondary lesions representing invasion from adjacent structures, notably prostate, colon and ovary. In this latter group symptoms related to the primary lesion were variable. The distinction between primary and secondary neoplasm is an important one and was rarely made on the basis of endoscopic or clinical findings alone. Urachal tumours were more common in females, whereas primary and secondary vesical adenocarcinomas were more common in males. The urachal tumours also occurred in a younger age group. Most of the adenocarcinomas, urachal or primary, were already advanced at the time of diagnosis. All tumours were palpable bimanually after resection and were at least T2 or T3. In the urachal carcinomas the results of partial cystectomy were disappointing because of the high rate of local recurrence and death from metastases. Primary non-urachal vesical adenocarcinoma carried an even poorer prognosis if non-radical surgery was carried out. The mean survival was 13 months. Radiotherapy was not effective in urachal and primary adenocarcinomas as these tumours are generally radioresistant. The treatment of secondary adenocarcinoma was governed by the primary site of the tumour. Radical surgery combined with chemotherapy and radiotherapy appeared to give the longest survival in the colonic tumours. Patients with prostatic cancer had a poorer survival rate than those with the same stage tumour but without bladder involvement, with renal failure secondary to obstructive uropathy being the commonest cause of death.     

The impact of pathological fractures on therapy outcome in patients with primary malignant bone tumours

The primary objective of this study was to investigate the implications of pathological fractures on therapy outcome in patients with primary malignant bone tumours and to determine whether limb salvage can be safely performed. A retrospective analysis of 447 patients with primary malignant bone tumours, treated between 1985 and 2005, was performed. Multivariate Cox regression analysis was used to investigate the influence of pathological fractures and further independent variables on survival rate. In 52 of the 447 patients, the primary malignant bone tumour was complicated by a pathological fracture. These fractures were more common in malignant fibrous histiocytoma (MFH) of the bone and in the tumour stages IIa/b and III. Ablative surgery was performed in ten patients and limb salvage surgery in 42 patients. The mortality risk for patients with pathological fractures was significantly increased by a factor of 1.82 (p = 0.015), and overall duration of survival was significantly lower in the fracture group, with a median of 6.2 years (p < 0.00001). In univariate and multivariate analysis, fracture, higher tumour stages and resection margins remained a significant predictor of worse survival. Overall survival, rate of local recurrence and distant metastases were not affected by the type of surgical treatment selected; there was no difference between the patients who underwent limb salvage and those who underwent an amputation. Pathological fracture in patients with primary malignant bone tumours is a predictor of worse survival and significantly increases mortality risk. Reconstructive surgery did not influence the survival rate, showing that limb salvage therapy is safe when adequate resection margins are achieved.     

Is bone grafting necessary?

Summary Twenty cases of giant-cell tumour of bone treated over an 11-year period were analysed. The minimum follow-up was 2 years. Primary treatment consisted of a detailed curettage alone, without bone grafting. A recurrence rate of 30% (six patients) was recorded. Cases of recurrence were suitable for resection and endoprosthetic replacement. We conclude that detailed curettage alone, without a bone graft, is an effective primary treatment for patients with giant-cell tumour of bone.     

Lower recurrence rate in chondroblastoma using extended curettage and cryosurgery

Purpose

Various methods for the treatment of chondroblastoma of bone have been used including simple curettage, or combined with bone grafting, in addition to the use of adjuvant therapy. However, local recurrence still represents a challenge in the management of this aggressive tumour. This study focuses on evaluating the role of intraregional extended curettage together with the use of adjuvant cryotherapy and autogenous bone grafting in the treatment of benign chondroblastoma of bone aiming to decrease the recurrence rate.

Methods

All patients with chondroblastoma included in this study underwent intralesional extended curettage, adjuvant cryotherapy using liquid nitrogen, and autogenous iliac crest bone grafting. Follow up for healing of chondroblastoma lesions and detection of any local recurrence was assessed on clinical and radiological bases. The functional outcome was assessed by the Musculoskeletal Tumour Society scoring system.

Results

The mean follow-up period was 49 months. The average time for bone healing was 7.4 months. Our rate of local recurrence is 7.1 %. Two patients (14.3 %) developed physeal growth arrest. One patient had superficial skin sloughing (7.1 %). None of the cases had pathological fracture. The mean Musculoskeletal Tumour Society functional score was 92.7 %.

Conclusion

Chondroblastoma is an aggressive benign bone tumour with a high rate of recurrence. The use of high-speed burr combined with adjuvant intralesional cryotherapy and iliac crest autogenous bone grafting is a reliable method of treatment with a low rate of recurrence.     

Arthroscopic management of Juxta-articular chondroblastoma with bone substitutes – A case report

Chondroblastoma, a rare benign bone tumour arising from the epiphysis, accounts for approximately 1% of all primary bone tumours and is known to be locally aggressive with potential for metastases and local recurrence. Open surgical curettage is associated with high risk of recurrence and potential for damage to the physis resulting in growth disturbances. We report a 14-year-old girl with chondroblastoma involving the distal femoral epiphysis in whom an arthroscopic approach was employed for thorough curettage of the lesion under direct vision followed by cavity management using bone graft substitute. At seven years follow-up she is asymptomatic, has normal knee function and her radiographs have shown good osteointegration of the synthetic graft with no evidence of recurrence. By combining the principles of intra-articular and endosteal arthroscopy in select patients, both tumour excision and cavity management can be done arthroscopically.     

Intracranial hemangiopericytoma: study of 12 cases

Summary  Most hemangiopericytomas (HPCs) are located in the musculoskeletal system and the skin, while the intracranial location is rare. They represent 2 to 4% in large series of meningeal tumours, thus accounting for less than 1% of all intracranial tumours. Many authors have argued about the true origin of this tumour. The current World Health Organization classification of Central Nervous System tumours distinguishes HPC as an entity of its own, and classified it into the group of “mesenchymal, non-meningothelial tumours”. Radical surgery is the treatment of choice, but must be completed with postoperative radiotherapy, which has proved to be the therapy most strongly related to the final prognosis. HPCs have a relentless tendency for local recurrence and metastases outside the central nervous system which can appear even many years after diagnosis and adequate treatment of the primary tumour.  Twelve patients with intracranial HPC were treated at our Unit between 1978 and 1999. There were 10 women and 2 men. Ten tumours were supratentorial and most located at frontoparietal parasagittal level. The most common manner of presentation was a focal motor deficit. All tumours were hyperdense in the basal Computed Tomography scans and most enhanced homogeneously following intravenous contrast injection. In 50% of cases, tumour margins were irregular or lobulated. Seven tumours were studied with Magnetic Resonance Imaging, being six of them iso-intense with the cortical gray matter on T1-weighted and T2-weighted images. Twenty operations were performed in the 12 patients. In 10 cases radical excision could be achieved with no operative mortality. Total recurrence rate was 33.3%. Eight patients were treated with external radiotherapy at some time through the course of their disease. Eight out of the 12 patients in this series are disease-free (Glasgow Outcome Scale categories 1 and 2) after a mean follow up of 52 months.     

Percutaneous radiofrequency ablation for unresectable pulmonary metastases from renal cell carcinoma

OBJECTIVE

To evaluate the clinical utility of lung radiofrequency ablation (RFA) in patients with unresectable pulmonary metastasis from renal cell carcinoma (RCC).

PATIENTS AND METHODS

We retrospectively examined 39 patients with unresectable metastases from RCC who were treated with lung RFA. Patients with six or fewer lung metastases measuring ≤6 cm that were confined in the lung, had all lung tumours ablated (curative ablation). Patients with extrapulmonary lesions, seven or more lung tumours, or large tumours of >6 cm, had mass reduction (palliative ablation). The primary endpoints was the overall survival, secondary endpoints were safety, local tumour progression rate, and recurrence‐free survival in the curative ablation group.

RESULTS

There were significant differences in the overall survival rates between the curative and palliative groups at 1 year (100% vs 90%), 3 years (100% vs 52%) and 5 years (100% vs 52%) (P < 0.05). The maximum lung tumour diameter was also a significant prognostic factor. There was local tumour progression in 13 patients (33%) during the mean follow‐up of 25 months. The recurrence‐free survival rates were 92% at 1 year, 23% at 3 years and 23% at 5 years in the curative ablation group. Pneumothorax requiring chest tube placement (six of 89, 7%) and pneumonia (one of 89, 1%) were major complications.

CONCLUSION

Lung RFA is a safe and effective treatment for prolonging survival in patients with unresectable RCC lung metastases.     

Giant cell tumour around the foot and ankle

Giant cell tumour (GCT) has been known to behave unpredictably when located in the foot and ankle bones. Involvement of foot and ankle bones is rare and comprises of less than 4% of all the GCTs. Out of 175 cases of GCT documented in the Scottish Bone Tumour Registry, 8 cases arose from foot and ankle. The mean age was 28.8 years (13–47). These included six males and two females. Pain and swelling was the common mode of presentation. All the cases had minimum follow-up of 36 months (36–180) and had definitive treatment within the first 3 months of initial presentation. The Campannacci radiographic staging system was used in grading the tumours. Seven of eight cases had intralesional procedure, while the remaining one had primary below knee amputation. One case had local recurrence within 2 years of the initial treatment requiring further surgery. Five out of seven cases with curettage involved the distal tibia or fibula. We had one recurrence of the three cases involving the tarsal bones, which reflects the difficulty in achieving thorough curettage in small bone tumours.Thorough curettage and bone grafting with adjuvant therapy has a low recurrence rate and should be considered when possible particularly in the long bones. Use of bone cement may be a better option in small bone involvement in view of difficulty in achieving thorough curettage. Regular follow-up helps detect early recurrence.     

Management of T1G3 tumours of the bladder

T1G3 tumours are the most aggressive superficial tumours of the bladder, with a high risk of recurrence and progression. Complete endoscopic resection of the tumour is the first diagnostic and therapeutic step in T1G3 management. A second resection should be done at 1 month to avoid residual tumour and misdiagnosis of a muscle infiltrative cancer. As a result of treatment by instillations of Calmette and Guérin bacillus following endoscopic resection, a 5-year survival rate of 80% has been reported, with 50 to 60% of bladder preservation. BCG is the only conservative treatment that has proven effectiveness on both tumour recurrence and progression. Long term protocols seem to give the best results. Endovesical chemotherapy is not commonly used as its impact on progression has not been demonstrated. Radical cystectomy can be chosen as first line treatment in patients with particularly aggressive tumours. Long term and close surveillance should be achieved in every patient.     

Osteoarticular allograft reconstruction of the distal radius after bone tumour resection

Resection of the distal end of the radius is indicated in the treatment of locally aggressive primary benign and malignant bone tumours. The aim of this study was to evaluate the technique of osteoarticular allograft reconstruction of this bone defect. We analysed 12 patients retrospectively with a minimum follow-up of 2 years (range 26-145 months, median 52 months). Three patients had a malignant tumour and nine had a giant cell tumour. The patients ages ranged from 13 to 65 years. The mean resected length of the radius was 6.6 (range 4-14)cm. Non-union of the osteotomy line was diagnosed 6 months after surgery in one case and needed bone grafting. Distal radio-ulnar joint instability was observed in eight cases. Subchondral bone alterations and joint narrowing were present in all cases but were painful in only one patient. The mean range of motion was 51 degrees of flexion and 37 degrees of extension.     

Malignant tumours of bone: clinical aspects and natural course.

An accurate pathological diagnosis must be made prior to treatment of a primary malignant bone tumour. Consideration must be given to the clinical and radiologic aspects as well as the histology. Both benign and malignant tumours occur more frequently in certain decades. A search should be made for precursor lesions such as Paget's disease. The presenting manifestations of pain, a mass and dysfunction are not specific for tumours. Laboratory tests may be helpful, especially in distinguishing tumours from infections and metabolic diseases. Metastasis is usually via the blood stream to the lungs and bones. The low survival rate following amputation for osteosarcoma and radiation therapy for Ewing's sarcoma has been improved by chemotherapy. The lower-grade tumours such as aggressive giant cell tumour and low-grade chondrosarcoma can often by treated successfully by resection and insertion of an autograft, an allograft or a metallic implant.     

Intracranial epidermoid tumours: thirty-seven years of diagnosis and treatment

The clinical, operative and pathological characteristics of 102 consecutive cases of intracranial epidermoid tumours are reported. These cases constituted 1.1% of all intracranial tumours and 13.5% of congenital neoplasma admitted to our service in the period 1951-88. Of 91 (89.2%) intradural tumours, 74 (72.5%) were in the subarachnoid cisterns, especially in the cerebellopontine angle cistern (31 cases). The tumours were solid in 69 cases (67.6%) and cystic in 26 (25.5%); the remaining 7 cases were not recorded. There were no specific clinical features with which to identify the nature and extent of the tumour. Diagnosis and treatment are described, with an evaluation of CT and MRI. Before 1981, total removal rate of tumour was 29.3%; after that date it was 72.7% with the aid of microsurgery. The overall surgical mortality rate was 4.9% (5 cases), but there were no deaths in the microsurgical group. Follow-up for a period of 0.5-37 years (mean 13 years) was carried out in 68 patients (70.1%). Among 24 patients with incomplete removal, recurrence of tumour was verified in 4 cases; 3 underwent reoperation with excellent or good results. One patient refused operation and died. All the remaining 20 cases, save 3 who died of other diseases, returned to their normal activities without recurrent manifestations. The results suggest that the primary treatment of epidermoid tumours should be surgical removal including the contents and capsule of a tumour. With the aid of microsurgical technique, complete removal is possible. Patients with incomplete removal can also obtain a long-term favourable result.