Inconclusive benefit of adjuvant 90Yttrium hydroxyapatite to radiosynovectomy for diffuse-type tenosynovial giant-cell tumour of the knee.

Aims: Intra-articular 90Yttrium (90Y) is an adjunct to surgical treatment by synovectomy for patients with diffuse-type tenosynovial giant-cell tumour (dtTGCT) of the knee, with variable success rates. Clinical information is, however, sparse and its value remains unclear. We investigated the long-term outcome of patients who underwent synovectomy with and without adjuvant treatment with 90Yttrium. Patients and Methods: All patients with dtTGCT of the knee who underwent synovectomy between 1991 and 2014 were included in the study. Group A patients underwent synovectomy and an intra-articular injection of 90Yttrium between six and eight weeks after surgery. Group B patients underwent surgery alone. Results: There were 34 patients in group A and 22 in group B. Recurrence of dtTGCT was identified by MRI, which was undertaken in patients with further symptoms. At a mean follow-up of 7.3 years (2.5 to 25.4), there was residual disease in 15 patients in group A and 11 in group B (p < 0.363). The mean Musculoskeletal Tumor Society (MSTS) score at final follow-up was 85% and 83%, respectively (p < 0.91). Conclusion: There were no significant differences in outcome between patients treated surgically for dtTGCT of the knee with or without an adjuvant intra-articular injection of 90Yttrium. We were unable to provide conclusive evidence of any benefits derived from the adjuvant treatment. Cite this article: Bone Joint J 2018;100-B:984-8.

The radiological evaluation of the hip joint after prosthetic arthroplasty of the proximal femur in patients with a tumour using a bipolar femoral head.

Excision of the proximal femur for tumour with prosthetic reconstruction using a bipolar femoral head places a considerable load on the unreplaced acetabulum. We retrospectively reviewed the changes which occur around the affected hip joint by evaluating the post-operative radiographs of 65 consecutive patients who underwent proximal prosthetic arthroplasty of the femur, and in whom an acetabular component had not been used. There were 37 men and 28 women with a mean age of 57.3 years (17 to 93). Radiological assessment included the extent of degenerative change in the acetabulum, heterotopic ossification, and protrusio acetabuli. The mean follow-up was 9.1 years (2 to 11.8). Degenerative changes in the acetabulum were seen in three patients (4.6%), Brooker grade 1 or 2 heterotopic ossification in 17 (26%) and protrusion of the prosthetic head in nine (13.8%). A total of eight patients (12.3%) needed a revision. Five were revised to the same type of prosthesis and three (4.6%) were converted to a total hip arthroplasty. We conclude that radiological evidence of degenerative change, heterotopic ossification and protrusion occur in a few patients who undergo prosthetic arthroplasty of the proximal femur for tumour. The limited extent of these changes and the lack of associated symptoms do not justify the routine arthroplasty of the acetabulum in these patients.

Hypofractionated adjuvant radiation therapy of soft-tissue sarcoma achieves excellent results in elderly patients.

OBJECTIVE: Adjuvant radiation therapy (RT) is an essential part of combined limb-sparing treatment of soft-tissue sarcoma (STS). Elderly or medically unfit patients often have difficulty in completing 6-7 weeks of standard fractionated daily treatment. Our aim was to evaluate the efficacy of a hypofractionated adjuvant approach with RT for STS in elderly and debilitated patients. METHODS: 21 elderly patients were treated with a short course of adjuvant RT (39-48 Gy, 3 Gy per fraction) for STS. The medical records of the patients were retrospectively reviewed for local or distant recurrence and side effects of RT. RESULTS: At a mean 26 months of follow-up, three local recurrences (14%) were detected. Eight patients (38%) had lung metastases during the observed period. Three of them died from metastatic disease. The hypofractionated radiation was well tolerated with minimum long-term side effects. CONCLUSION: Hypofractionated adjuvant radiation appears to be an effective treatment in terms of local control in elderly and debilitated patients. ADVANCES IN KNOWLEDGE: The results of this study might provide an alternative to commonly used standard fractionation of radiotherapy in sarcoma patients.

Fifteen years of bone tumor cryosurgery: a single-center experience of 440 procedures and long-term follow-up.

BACKGROUND: This summary of a single center's extensive cumulative experience in bone tumor cryosurgery assesses the long-term outcome of bone conservation surgery in which adjuvant cryosurgery plays a pivotal role. MATERIALS AND METHODS: We performed 440 cryosurgical procedures between January 1988 and December 2002. Two-thirds of the series comprised a variety of primary benign-aggressive and low-grade malignant lesions, and one-third were primary high-grade and metastatic bone tumors. The anatomical locations included almost every bone of the skeleton. Two methods of bone cryosurgery were used: Marcove's "open" direct-pour system using liquid nitrogen (1988-1997) and Meller's "closed" argon-based system (1998 to the present). RESULTS: The study cohort consisted of 214 males and 191 females (age range 5-82 years). The median follow-up was 7 years (range 3-18). The overall local recurrence rate was 8%: fractures=1%, infections=2% and skin burns=1.3%. There were three cases of transient nerve palsies in areas other than the sacrum, and four cases of late osteoarthritis of an adjacent joint. The functional outcome for the 372 patients with no evidence of disease was almost 100% "good" and "excellent" (American Musculo-skeletal Tumor Society System). Only two patients needed secondary amputations. CONCLUSIONS: Bone cryosurgery is a safe and effective limb-, joint- and even epiphysis-sparing surgical technique in suitable types of bone tumors, temporarily or permanently obviating the need for resection surgery.

Synovial sarcoma of the extremities and trunk: a long-lasting disease.

Synovial sarcoma (SS) of an extremity or trunk is relatively rare and is approached by limb sparing surgery (LSS), radiation therapy (RT) and chemotherapy. We conducted a retrospective analysis of the clinical and histopathological data of 73 patients with proven SS. At a median follow-up time of 6 years, local recurrence was seen in 17.8 and systemic recurrence 35.6% of patients (local-only, 6.8; systemic-only, 24.6; combined, 11%). The 10-year local recurrence-free survival (LRFS), systemic recurrence-free survival (SRFS) and overall survival (OS) rates were 78, 68 and 61%, respectively. LRFS was significantly better in patients treated with isolated limb perfusion (ILP); SRFS was influenced by the delay until diagnosis. The practical aspects of our observations are the need for long-term follow-up in order to diagnose recurrences, the fact that not all local or distant recurrences are necessarily associated with a shortening of OS time and the important role of induction ILP with TNF in cases of extremity SS.

A combined use of a free vascularised flap and an external fixator for reconstruction of lower extremity defects in children.

PURPOSE: To describe a combined use of a free vascularised flap and an external fixator for reconstruction of lower extremity defects in children, and correction of equinus contracture developed after removal of the external fixator using a circular dynamic frame. METHODS: Seven children (4 males) aged 4 to 12 (mean, 8) years were treated with 9 free vascularised flaps for 8 limbs (bilaterally in one patient and for a failed flap in another). Patient pathologies included: 3 soft tissue degloving injuries, one soft tissue and bone avulsion, one severe burn contracture, one resurfacing of soft tissue and bone necrosis, and one osteosarcoma resection defect. Free flap reconstruction was delayed in 6 patients (range, 3 weeks to 4 years). Static external fixators were used to stabilise the free vascularised flaps at the time of reconstruction, with the ankle in a neutral position. RESULTS: The mean follow-up was 5 (1-10) years. All flaps but one survived; the failed one was immediately reconstructed with a contralateral, latissimus dorsi flap. One anastomosis following a Kirschner-wire injury was successfully revised. Six patients had pin tract infections and were treated with oral antibiotics. Two patients developed equinus contracture 6 and 3 years later, after removal of the external fixator, and were corrected by distraction, using a dynamic Ilizarov frame. CONCLUSION: The combined use of a free flap and an external fixator for salvage of lower extremities is useful in children. Late development of equinus contracture can be safely corrected by distraction, without compromising flap viability.

Liposarcoma in adult limbs treated by limb-sparing surgery and adjuvant radiotherapy.

Between December 1995 and March 2003, 38 adult patients with intermediate or high-grade liposarcoma in a limb were treated by limb-sparing surgery and post-operative radiotherapy. The ten-year local recurrence-free survival was 83%, the ten-year metastasis-free survival 61%, the ten-year disease-free survival 51% and the ten-year overall survival 67%. Analysis of failure and success showed no association with the age of the patients, gender, the location of the primary tumour, the type of liposarcoma and the quality of resection. Our results indicate that liposarcoma may recur even ten years after the end of definitive therapy and may spread to unexpected sites as for soft-tissue sarcoma.

Closed argon-based cryoablation of bone tumours.

We report our experience with a new technique for cryosurgical ablation of bone tumours which allows accurate determination of the temperature and freezing time within a cavity of any geometrical shape. Between 1997 and 2000, 58 patients diagnosed with 13 malignant and 45 aggressive benign bone tumours underwent argon-based cryoablation. This technique includes removal of the tumour by curettage and filling the cavity with a gel medium into which metal probes are inserted. Argon gas is delivered through the metal probes and both time and temperature are computer-controlled. After formal reconstruction, all patients were followed for more than two years. None had skin necrosis, infection, neurapraxia or thromboembolic complication. Fractures occurred in two patients (3.4%) and the tumour recurred in two patients (3.4%).

Association between telomerase activity and outcome in patients with nonmetastatic Ewing family of tumors.

PURPOSE: Telomerase is considered a molecular marker for malignancy. The aim of this study was to determine telomerase activity (TA) as a prognostic factor at diagnosis and as a marker for minimal residual disease during therapy and follow-up in nonmetastatic Ewing family of tumors (EFT). PATIENTS AND METHODS: Primary tumor specimens and 97 peripheral blood (PBL) samples from 31 EFT patients were analyzed for TA by the Telomeric Repeat Amplification Protocol (TRAP assay). The telomerase catalytic subunit (human telomerase reverse transcriptase [hTERT]) gene expression was evaluated by quantitative reverse transcriptase polymerase chain reaction (RT-PCR) and telomere length was determined by Southern blotting. The presence of the EFT chimeric transcripts was analyzed by RT-PCR. Correlations with progression-free survival were evaluated. RESULTS: At diagnosis, TA in primary tumors did not correlate with outcome. During therapy and follow-up, highly significant correlation was observed between high TA in PBL samples and adverse prognosis (P <.0001). None of the patients harboring low TA progressed, with a long follow-up (median, 60 months) and a progression-free survival (PFS) of 100%. In nine patients, high TA actually could predict relapse, long before overt clinical relapse. The group of patients with high TA and positive RT-PCR had the most adverse outcome; PFS of 20% (P =.0025). TA was found to be a better prognostic factor than RT-PCR and histopathologic response at surgery. CONCLUSION: The results suggest that TA is a significant prognostic variable, superior to the established clinical prognostic parameters during therapy and tumor surveillance. It could be used in combination with RT-PCR for a new risk classification.

The use of surgery and yttrium 90 in the management of extensive and diffuse pigmented villonodular synovitis of large joints.

OBJECTIVE: The surgical treatment of extensive diffuse pigmented villonodular synovitis (PVNS) of large joints alone is unsatisfactory, with high rates of local recurrence. Post-synovectomy adjuvant treatment with external beam radiation therapy or intra-articular injection of yttrium 90 (90Y) yielded better results. We report our experience with 10 cases treated with debulking surgery followed by intra-articular injection of 90Y. PATIENTS AND METHODS: Between January 1989 and June 1998, 10 patients (eight males and two females aged 15-49 yr) with extensive diffuse PVNS were treated. In six patients the knee joint, in three patients the ankle joint, and in one patient the hip joint were involved. The 10 patients underwent 15 operations, one patient had three surgical procedures, and three patients underwent two surgeries (the intervals between re-operations for local recurrence were 2-4 yr). All patients had an intra-articular injection of 15-25 mCi (555-925 MBq) 90Y, 6-8 weeks after the last surgery. RESULTS: Mean follow-up time was 6 yr (range 2.5-12 yr). All patients were followed using repeated computerized tomography (CT) scans, magnetic resonance imaging (MRI), plain X-ray films and bone scans semi-annually. In nine patients, neither evidence of disease nor progression of bone or articular destruction were noted. In one patient, stabilization of disease was achieved with no further evidence of bony or articular damage. No complications were noticed after surgery or after the intra-articular 90Y injection. CONCLUSION: A combination of debulking surgery with intra-articular injection of 90Y for extensive diffuse PVNS of major joints is a reliable treatment method, with good results.

Desmoid tumour of the hand.

A rare case of an extra-abdominal desmoid tumour in the hand is reported. Its treatment and a short survey of the literature is given.

ErbB-4 expression in limb soft-tissue sarcoma: correlation with the results of neoadjuvant chemotherapy.

ErbB-4 is a recently described growth factor receptor. Relatively little is known about its expression in human tumours. In this study, we assessed the possible role of erbB-4 as a tissue marker for soft-tissue sarcomas (STS) and its correlation with the response to chemotherapy. The histological specimen of 29 patients with STS of a limb who had received preoperative doxorubicin (ADR)-based chemotherapy were studied for the degree of necrosis and the expression of erbB-4 (by an avidin-biotin-peroxidase technique). ErbB-4 expression in the preoperative tissue samples was compared with the expression in the postchemotherapy resected tumour. The true objective response rate to preoperative chemotherapy was 34%. Wide resection of the tumour was done in 12 patients, marginal in 14, amputation in 2 and no surgery in 1. The tumour necrosis was above 90% in 9 patients, 60-90% in 12, and less than 60% in 7 patients. An increase in erbB-4 expression was more common in cases with no response to chemotherapy, while no change or a decrease in erbB-4 was more common in responsive tumours (P=0.004). No correlation could be found between the degree of necrosis or the chemotherapeutic regimen and the change in expression of erbB-4. The median disease-free survival (DFS) was longer for patients with a decrease or no change in expression of erbB-4 than for patients with increased expression. It is believed that postchemotherapy new expression or no downregulation of the erbB-4 molecule represents tumour aggressiveness and increased capability of growth and spread.

Reconstruction of the extensor mechanism after proximal tibia endoprosthetic replacement.

The proximal tibia is a difficult area in which to perform a wide resection of a bone tumor. This difficulty is due to the intimate relationship of tumor in this location to the nerves and blood vessels of the leg, inadequate soft tissue coverage after endoprosthetic reconstruction, and the need to reconstruct the extensor mechanism. Competence of the extensor mechanism is the major determinant of functional outcome of these patients. Between 1980 and 1997, 55 patients underwent proximal tibia resection with endoprosthetic reconstruction for a variety of malignant and benign-aggressive tumors. Reconstruction of the extensor mechanism included reattachment of the patellar tendon to the prosthesis with a Dacron tape, reinforcement with autologous bone-graft, and attachment of an overlying gastrocnemius flap. All patients were followed for a minimum of 2 years; 6 patients (11%) had a transient peroneal nerve palsy, 4 patients (7.2%) had a fasciocutaneous flap necrosis, and 2 patients (3.6%) had a deep wound infection. Full extension to extension lag of 20 degrees was achieved in 44 patients, and 8 patients required secondary reinforcement of the patellar tendon. Function was estimated to be good to excellent in 48 patients (87%). Reattachment of the patellar tendon to the prosthesis and reinforcement with an autologous bone-graft and a gastrocnemius flap are reliable means to restore extension after proximal tibia endoprosthetic reconstruction.

Continuous intra-articular patient-controlled analgesia in a cancer patient with a pathological hip fracture. A case report.

Pain management is one of the major goals to achieve in treating terminal patients. Managing severe pain with drug therapy by using a combination of narcotics, non-narcotics, and adjuvant drugs provides the best results for most patients. In patients with fractures due to bone metastases there is a relative indication for operative treatment if the patient is expected to survive the operation and to recover in the postoperative period. In this case report a terminal 48 year-old female with a pathological femoral neck fracture is presented. No operative treatment was offered because of her bad general status. Pain control was maintained by continuous administration of local analgesics directly into the intra-articular femoral space, through a system of patient-controlled analgesia (PCA). The visual-analogue pain scale (VAS), which was maximal before beginning of this treatment, was markedly reduced during the treatment, and was again elevated to maximum after the intra-articular catheter was removed. Although continuous regional analgesia to a fractured bone which involves the joint is not a generally acceptable method and carries some risks it has a role in a selected group of patients.

Effect of P-glycoprotein expression on outcome in the Ewing family of tumors.

This study was designed to determine the prognostic significance of multidrug resistance, mediated by P-glycoprotein (Pgp) expression, in Ewing sarcoma. The clinical and laboratory features, treatment protocol, and outcome of 75 patients with Ewing sarcoma or peripheral neuroectodermal tumor treated between 1972 and 1997 were reviewed. Pgp expression was tested with the monoclonal antibody JSB-1. Thirty-four (64%) of the 53 tissue samples from untreated patients stained positive for Pgp. Progression-free and overall survival were 44 and 59%, respectively, in patients with negative findings, and 28 and 41% in those with positive findings; neither difference was significant. Of the 12 relapsed patients, 6 (50%) expressed more Pgp after chemotherapy than at diagnosis and 4 (33%) expressed less. Within these subgroups, 5 out of 6 and 3 out of 4 died from the disease. No correlation was found between Pgp and known prognostic factors of Ewing tumors. Pgp expression is probably an intrinsic factor of Ewing tumors but has no correlation to prognosis.

Palliative forequarter amputation for metastatic carcinoma to the shoulder girdle region: indications, preoperative evaluation, surgical technique, and results.

BACKGROUND AND OBJECTIVES: Uncontrolled metastatic carcinoma of the shoulder girdle is a difficult oncologic problem. This study reviews our experience with palliative forequarter amputation with emphasis on patient selection criteria, preoperative radiologic assessment, surgical technique, epineural postoperative analgesia, and clinical outcome. METHODS: Eight patients who underwent palliative forequarter amputation for metastatic carcinoma between 1980 and 1999 were analyzed retrospectively. Diagnoses included breast carcinoma (n = 3), squamous cell carcinoma (n = 2), hypernephroma (n = 2), and carcinoma of unknown origin (n = 1). All patients presented with severe, intractable pain and a useless extremity. Venography demonstrated obliteration of the axillary vein in each of the patients in whom this procedure was performed. Exploration of the brachial plexus confirmed tumor encasement and unresectability in all patients. Epineural catheters for bupivacaine infusion were placed for postoperative pain control. RESULTS: All patients experienced dramatic pain relief and improved mobility and overall function. Life-threatening hemorrhage and sepsis were alleviated. There were no instances of phantom limb pain or adverse psychological reactions, and no complications related to epineural analgesia. CONCLUSIONS: Palliative forequarter amputation is relatively safe and reliable and provides effective pain relief for selected patients with unresectable metastatic carcinoma to the axilla and bony shoulder girdle in whom radiotherapy and/or chemotherapy has not been effective. The triad of pain, motor loss, and an obliterated axillary vein is indicative of brachial plexus infiltration and unresectability.

Multiple primary malignancies in association with soft tissue sarcomas.

BACKGROUND: Modern cancer treatment has increased the survival of patients with various malignancies substantially. One of the late sequelae of successful treatment is the development of a second malignant tumor. However, in many cases of second primary tumors, exposure to chemotherapy or radiation therapy is not evident, and it should be postulated that the putative mechanism for the development of the second tumor is different. In the current series, the association between soft tissue sarcoma (STS) in adults and the development of other primary malignancies was studied. METHODS: A retrospective search of the data files of 610 patients with STS or bone sarcomas who were treated at the study institution between January 1995 and December 1999 was performed. All files regarding patients with STS who developed a second malignant tumor were retrieved for analysis. RESULTS: Of 375 patients with STS, 28 (7.5%) developed other malignant neoplasms either before or after the diagnosis of STS. STS as the first tumor occurred in 14 patients (ages 16-72 years). Only three patients were treated with chemotherapy for their sarcoma. Radiation therapy was administered to five patients as an adjuvant to surgery for the first tumor. The second tumor types mainly included STS and renal cell carcinoma. The time interval between the diagnosis of the STS and the second malignancy was 0 (for synchronous tumors) to 21 years. Three patients developed a third primary tumor within 3 years after the diagnosis of the second tumor. The median overall survival was > 78 months. Fourteen patients (ages 35-87 years) had a first primary tumor other than STS (mainly breast carcinoma and genitourinary malignancies). The second tumors (mainly STS) appeared within 0 (for synchronous tumors) to 27 years. The median overall survival for the 14 patients in this group from the time of diagnosis of the first tumor was > 102 months. CONCLUSIONS: The phenomenon of two or three primary neoplasms developing in patients in whom one of the tumors was STS occurs at a rate of 7.5%, a significantly higher rate than that reported for the occurrence of STS among the general cancer population (1%). The majority of cases occur incidentally. The clinical implication includes the need to search for an occult second primary tumor in patients with STS as an integral part of their follow-up. This is especially true in patients with primary malignant fibrous histiocytoma who demonstrate a risk for developing a renal cell carcinoma.

Is forequarter amputation justified for palliation of intractable cancer symptoms?

BACKGROUND: Limb-sparing surgery has replaced the radical surgical approach for treating limb sarcomas in most cases. Amputation has been advocated as a palliative procedure for symptomatic locally advanced disease that has already failed to respond to radiation therapy, chemotherapy and limited surgery. METHODS: Twelve patients with advanced malignant tumors involving the shoulder girdle or the proximal humerus underwent forequarter amputation (FQA) for palliative purposes. The tumor-related local problems were severe pain, limb dysfunction, tumor fungation, bleeding (requiring emergency FQA in one case) and infection. The preoperative Karnofsky performance status (KPS) in our series ranged from 30 to 70%. RESULTS: No perioperative mortality was observed. The morbidity was well tolerated by the patients. The KPS improved in most of the patients, and was assessed as 90-100% in 9 of the 12 patients. Overall, quality of life was reported to be at least moderately improved by 2 out of 3 patients. Survival was measured in months (3-24 months), but ultimately had no meaning since the procedure was palliative. Lung metastases were the dominant cause of death in our patients. CONCLUSIONS: The results of FQA in our series point to its feasibility and the gain in quality of life and performance status in severely ill patients with advanced malignancies. Local symptoms and signs were controlled, and quality of life was restored.

Metastatic renal cell carcinoma of bone: indications and technique of surgical intervention.

PURPOSE: We describe the efficacy of surgical excision of metastatic renal cell carcinoma of bone for achieving local tumor control, pain control and functional outcome with emphasis on the indications and techniques of surgical intervention as well as oncological outcome. MATERIALS AND METHODS: Between 1980 and 1997 we performed surgery on 45 patients (56 lesions) with metastatic renal cell carcinoma of bone. Indications for surgery were solitary bone metastasis, intractable pain, or impending or present pathological fracture. Surgery involved wide excision in 29 cases, marginal excision with adjunctive liquid nitrogen in 25 and amputation in 2. RESULTS: None of the patients had significant bleeding intraoperatively. Mean hospital stay was 9.8 days, during which there was no flap necrosis, deep wound infection, nerve palsy or thromboembolic complication. Postoperatively pain was significantly relieved in 91% of patients, while 89% achieved a good to excellent functional outcome, and 94% with metastatic lesions of the pelvic girdle and lower extremities were ambulatory. Local recurrence developed in only 4 of the 56 lesions (7.1%), including 3 after marginal resection. Survival was more than 2 years in 22 patients (49%) and more than 3 in 17 (38%). CONCLUSIONS: Surgical excision is safe and reliable for restoring mechanical bone stability, relieving pain and providing good function in most patients with metastatic renal cell carcinoma who meet the criteria for surgical intervention. Relatively prolonged survival in these cases justifies considering surgical intervention when feasible.