Aim

Giant cell tumour (GCT) of bone is a benign but locally aggressive tumour. Although topical adjuvants have been used in the past, local recurrence following intralesional excision of GCT of bone continues to remain a problem. The use of bisphosphonates as an anti-osteoclastic agent in the management of osteolytic bone metastases is well accepted. Therefore our study aims to retrospectively demonstrate whether the administration of bisphosphonate as an adjuvant can control aggressive local recurrence of GCT and prevent wide resections of bones or amputations.

Background

Cryosurgery is a well established modality in the treatment of benign aggressive and low grade malignant tumours. In this setting it allows for intra-lesional resection and preservation of function without compromising oncological outcome. Here we present the outcome of 87 patients treated with cryosurgery for low-grade chondrosarcoma of bone.

Multiple myeloma may be associated with extensive bone destruction, impending or present pathological fracture, and intractable pain. Chemotherapy and radiotherapy are usually effective, but surgical intervention may sometimes be required.

We analyzed the surgical technique and the functional and oncological outcomes of patients with multiple myeloma who underwent surgery in our services between 1993-2004.

There were 19 males and 15 females (age range 49– 75 years) who had destructive bone lesions located at the humerus (n=17), acetabulum (n=5), femur (n=5), or tibia (n=7). Indications for surgery included pathological fracture (n=20), impending pathological fracture (n=11), and intractable pain (n=3). Nineteen patients underwent marginal tumor resection, reconstruction with cemented hardware, and adjuvant radiation therapy and 15 patients underwent wide tumor resection with endoprosthetic reconstruction. All patients reported immediate and substantial postoperative pain relief. Function was good/excellent in 23 patients (68%), moderate in eight (23%), and poor in three (9%). Two patients (5.9%) had local tumor recurrence treated with local excision and adjuvant radiotherapy, with no evidence of further recurrence at 21 and 26 months, respectively. Thirty one (91%) patients survived > 1 year, 23 (68%) > 2 years, and 15 (44%) > 3 years postoperatively. All reconstructions remained stable at the most recent follow-ups.

The relatively prolonged survival of patients with multiple myeloma justifies an aggressive surgical approach, which is safe and associated with good local tumor control and functional outcome.

Introduction: Cryosurgery of bone tumors using direct pour of liquid nitrogen has the advantage of joint preservation associated with good local tumor control. However, this technique does not allow accurate control of the temperature or of the overall time of freezing. Additionally, this is a gravity-dependent procedure that cannot be applied in all shapes and locations of tumor cavities. The authors report their experience with a novel cryosurgical technique that allows accurate determination of the temperature and freezing time as well as freezing of any geometry of tumor cavity.

Materials and Methods: From 1997 to 2000, 58 patients who were diagnosed with 13 malignant and 45 benign-aggressive bone tumors underwent argon-based cryoablation. This technique included tumor removal by means of curettage and burr-drilling, filling the tumor cavity with a gel medium, insertion of metal probes into this medium, and computer-controlled delivery of argon gas through the metal probes, and reconstruction of the tumor cavity with cemented hardware. All patients were followed for more than two years.

Results: None had skin necrosis, infection, thromboembolic complication, or neurapraxia. Fractures occurred in two patients (3.4%) and local tumor recurrence in two patients (3.4%), who were successfully treated with a second closed cryoablation.

Conclusions: The current study focuses on the concept and surgical technique of argon-based and computer-controlled, closed cryoablation of bone tumors. The main advantages of this system are the ability to control the freezing temperature and overall freezing time and the use of a gel medium, which evenly conducts the cold temperature throughout the tumor cavity and allows cryoablation of various cavital geometry and positions. The current technique of argon-based cryoablation is simple and easy to perform. It achieves good local tumor control and is associated with a low rate of complication. The authors recommend its use as an alternative to the traditional direct pour technique of cryosurgery.

Introduction: Metastatic bone disease of the humerus may be associated with disabling pain and loss of function. Surgery must provide good local tumor control, immediate mechanical stability, and a short rehabilitation period. Between 1980 and 2000, the authors operated 59 patients with metastatic disease of the humerus. The current study summarizes the principles of the surgical technique and functional and oncological outcomes.

Materials and Methods: There were 33 females and 26 males. Indications for surgery included pathological fracture (40), impending fracture (11), and intractable pain (6). Anatomic locations of humeral metastases included: around the proximal humeral metaphysic and head (Type I) – 18, humeral diaphysis (Type II) – 39, and humeral condyles (Type III) – 2. Types I and III metastases were treated with resection and endoprosthetic reconstruction. Type II metastases were treated with intralesional tumor removal and cemented nailing. Postoperatively, 31 patients were treated with radiation therapy, 35 with chemotherapy, and 14 with immunotherapy.

Follow-up of the study patients included physical examination, radiological evaluation and functional evaluation according to the American Musculoskeletal Tumor Society system.

Results: Patients who had cemented nailing had better overall function, emotional acceptance, hand positioning, and lifting ability than patients who underwent endoprosthetic reconstruction. Pain alleviation and dexterity were comparable in both groups. All patients had a stable extremity and overall function of 56 patients (95%) was > 68% of normal upper extremity function. Only two patients (3%) had a local tumor recurrence.

Conclusions: An aggressive surgical approach in patients who have humeral metastases and meet the criteria for surgical intervention is beneficial; it provides durable reconstruction and is associated with good function and local tumor control in most patients.

Introduction: Diffused pigmented villonodular synovitis (PVNS) is a locally aggressive lesion for which surgery provides only marginal resection. An adjuvant treatment modality is therefore required to prevent local tumor recurrence. The authors describe their experience with intra-articular injection of Yttrium⁹⁰ (Y⁹⁰), a radioisotope, as an adjuvant for tumor resection.

Materials and Methods: Between 1989 and 2002, 20 patients with diffuse PVNS were treated with post-operative, intraarticular injection of Y⁹⁰. There were 15 male and 5 female patients who ranged in age from 13 to 67 years (mean, 35 years). Anatomic locations of the affected joints included: knee – 15, ankle – 4, hip – 1. Tumor resection was initially done in all patients: 13 patients required open arthrotomy, the remaining 7 underwent arthroscopic tumor resection. Ten patients were referred for treatment after having operation for a local tumor recurrence: 6 patients had one, 2 had two, 1 had three, and the remaining one had five local recurrences. Six to eight weeks after surgery, intraarticular injection of 15–25 mCi of Y⁹⁰ was done. These procedures were conducted in the operating room under local anesthesia and fluoroscopic guidance. All patients were followed for a minimum of two years (range, 25–168 months; mean, 65 months).

Results: Following Y⁹⁰ injection, all patients reported mild pain around the affected joint. This pain was well controlled with the use of NSAID’s and typically resolved within a few days or weeks. Three patients had superficial skin inflammation and associated blisters around the site of injection, probably the result of Y⁹⁰ effect on the soft-tissues. All were treated conservatively with complete resolution of their symptoms. All patients gained their pre-injection range-of-motion within 4–6 weeks. At the most recent follow-up, five patients had transient post-radiation skin changes (discoloration of the skin and dry and scaly skin) and local recurrence occurred in only one patient (5%) with PVNS around the knee; additional Y⁹⁰ injections were unsuccessful and he eventually underwent knee arthrodesis.

Conclusion: Y⁹⁰ injection is a reliable adjuvant for surgery in the management of diffused PVNS. Local tumor control and good function, associated with only mild morbidity are achieved in the majority of the patients.

Multiple myeloma may be associated with extensive bone destruction, impending or present pathological fracture, and intractable pain. However, surgical intervention is rarely indicated since local bone crises are effectively managed with chemotherapy and radiotherapy in the majority of the patients. The current retrospective analysis of patients who eventually required surgical intervention emphasized indications for surgery, surgical technique, and functional and oncological outcomes.

Materials and Methods: Between 1982 and 2000, the authors operated on 18 patients with multiple myeloma. There were 11 females and 7 males whose age ranged from 4 to 67 years (median, 59 years). Anatomic locations: proximal humerus – 5, proximal femur – 4, distal femur – 5, proximal tibia – 3. One patient had total femur involvement. Preoperatively, 11 patients were treated with chemotherapy and 4 received radiotherapy. Seven patients were referred with a bone lesion as their initial presentation and, therefore, did not receive pre-operative treatment.

Indications for surgery: pathological fractures – 11 patients, impending pathological fractures – 5 patients, and intractable pain in 2 patients. Surgeries included 12 marginal resections with cryosurgery and 6 wide resections with endoprosthetic reconstructions. Postoperative radiotherapy was given to three patients and chemotherapy to 11. Follow-up included physical and radiological evaluation and functional evaluation according to the American Musculoskeletal Tumor Society System.

Results: Fifteen patients (83%) survived more than 1 year and 12 patients (66%) survived more than 2 years after surgery. There were no postoperative deep wound infections, thromboembolic complications, or local tumor recurrences. Functional outcome was good to excellent in 14 patients (78%), moderate in 3 (16%), and poor in one patient (6%).

Conclusions: Multiple myeloma rarely may require surgical intervention because of impending or present pathological fracture or intractable pain. The relatively prolonged survival of patients with multiple myeloma justifies an aggressive surgical approach. Resection of these tumors was shown to be safe, reliable, and associated with good local tumor control and functional outcome.

Introduction: Tumors of the axilla impose a surgical difficulty because they are usually large at presentation and in close proximity to the major neurovascular bundle of the upper extremity. Attempted tumor resection via the base of the axilla is difficult because of limited exposure of the axillary content and neurovascular bundle. The authors have used a safe and reliable exposure for these situations.

Methods: Between 1980 and 1997, 35 patients underwent extensile exposure of an axillary tumor. Diagnoses included 19 primary and 16 metastatic tumors of the axilla. The axillary cavity was fully exposed via the deltopectoral groove after detachment and reflection of two layers of muscles: first, the pectoralis major and, second, the coracoid origin of the pectoralis minor, cora-cobrachialis, and the short head of the biceps muscle. This surgical approach allowed full tumor visualization and determination of the exact anatomic relation of the tumor to the neurovascular bundle and as a result, tumor respectability. Following resection, the pectoralis minor and conjoined tendons were reattached to the coracoid process with a nonabsorbable suture, and the pectoralis major was reattached to its insertion site on the proximal humerus in the same manner.

Results: Exposure revealed a safe plane of dissection between the tumor and the major neurovascular bundle in 23 patients and invasion of the major neurovascular bundle in 12 patients who subsequently underwent a forequarter amputation. At the most recent follow-up, none of these patients had functional limitation, which could be attributed to the extensile approach itself. All patients gained their presurgical pectoralis major and biceps function.

Complications in the group of patients that underwent tumor resection included three (13%) superficial wound infections. Due to intended enbloc resection of an involved nerve with the tumor, two nerve palsies (8.7%) were documented. None of the remaining 21 patients had numbness, paresthesias, or nerve pain. There were three (13%) local recurrences; two were managed with wide excision and adjuvant radiation therapy and one necessitated amputation.

Conclusions: The extensile exposure of the axilla allows full visualization of axillary tumors. It allows determination of tumor respectability and safe and reliable resection, when indicated. This exposure is associated with good functional outcome and an acceptable morbidity and is recommended in the management of axillary tumors.

The presentation of this huge monoinstitutional cumulative experience in bone tumor cryosurgery serves as an illustration of our basic phylosophycal concent: “No man-made implants are even close to God’s natural implants yet”. The result is the concept of conservation surgery in which cryotherapy plays the major role.

Materials and Methods: During the period of 1/88 to 12/02 (15 years, FUT 2→17 years, median=6 years) 440 cryosurgical procedures were performed in 405 people. There were 214 male and 191 female patients. The age range was 5 to 80 years but most were between 20 and 59 years. 2/3 of the series included a variety of primary benign aggressive and low grade malignant lesions and 1/3 included primary high grade and metastatic bone tumors. The anatomical location is highly variable and includes almost every bone of the skeleton.

Two methods of bone cryosurgery will be presented (including combinations of them and other adjuvants or techniques): The open system according to Marcove and our Closed System.

Results and Complications: Overall local recurrence (LR) rate=8%, fractures=1%, infections=2%, skin burns= 1.3%.

There were 3 cases of temporary nerve palsies and 2 cases of late OA of an adjacent joint. Functional outcome in 372 NED patients is almost 100% good and excellent (AMSTS, Enneking’s scoring system).

Summary: Bone cryosurgery is a safe, reliable and inexpensive technique of conservative limb, joint and even epiphysis sparing surgery in the suitable types of bone tumors, eliminating temporarily or permanently the need for resection surgery.

Introduction: Megaprosthetic failures around the knee and especially those who are infection-related are difficult to manage. Although most of these cases are effectively managed with a two-stage prosthetic revision, selected cases eventually require sacrifice of the knee joint. The authors present their experience with knee-arthrodesis using a vascularized fibula and allograft reinforcement.

Materials and Methods: Between 1998 and 2002, eight patients with failed knee prosthesis were referred for resection-arthrodesis; all patients had at least two previous revision attempts using a spacer or a new implant. Knee-arthrodesis included resection of the distal femur and proximal tibia and reconstruction with a free micro-vascularized fibular graft and allograft reinforcement. Fibular grafts were harvested with a large musculocutaneous flap to facilitate soft-tissue coverage and monitor flap viability. Following surgery, patients were kept non-weight-bearing for 3 months. Radiographs were performed 6 and 12 weeks postoperatively to establish fibular graft incorporation. If healing had progressed satisfactorily, weight-bearing was gradually allowed.

Results: At the most recent follow-up’ all eight patients had a stable and painless reconstruction, associated with radiological evidence of solid fibular graft union. The latter was typically observed between 6 to 12 weeks from surgery. Complications included one emergent surgery for anastamotic rupture in one patient and surgical debridement with skin grafting of musculocutaneous flap necrosis in another patient.

Conclusions: Knee-arthrodesis using microvascularized fibula and allograft reinforcement is a safe and reliable salvage procedure in end-stage failures of megaprosthetic knee implants.

Introduction: Surgical removal by means of curettage is the mainstay of treatment of enchondromas of the hand. Methods of reconstruction after tumor removal usually entail no reconstruction or filling of the tumor cavity with a bone graft. These techniques necessitate a prolonged period of protected activity until bone healing of the tumor cavity occurs. The authors have utilized hardware and bone cement for the purpose of reconstruction of the tumor cavity. This technique provides immediate mechanical stability and allows early mobilization.

Methods: Between 1986 and 1999 the authors treated 13 patients (8 females, 4 males) who ranged in age from 23 to 58 years (median, 32 years) and diagnosed with enchondroma of the hand. Eight patients presented with a pathological fracture. Anatomic locations included: metacarpal bones – 5, proximal phalanx – 4, and middle phalanx – 4. Tumors were approached through the retained thinned or destroyed cortex to minimize additional bone loss. Surgery included removal of all gross tumor with hand curettes; this was followed by high speed burr drilling of the inner reactive bone shell. Reconstruction included intramedullary metal wire along the longitudinal axis of the cavity and polyme-hylmethacrylate (PMMA). Full activity as tolerated was allowed immediately after surgery. All patients were followed for more than 2 years.

Follow-up included physical and radiological evaluation and functional evaluation.

Results: Following surgery, all patients returned to their presurgical functional capability within two weeks. At the last follow-up, none of the patients had local tumor recurrence and although three patients had 15° to 20° decrease in flexion of the metacarpophalangeal joint, none reported a functional limitation. There were no postoperative infections or fractures.

Conclusions: Reconstruction of the tumor cavity, remaining after curettage of enchondroma of the hand, with intramedullary hardware and PMMA provides immediate mechanical stability and allows early mobilization. This technique is associated with good short- and long-term functional outcomes.

Introduction: Chromosomal analysis is becoming increasingly useful in the diagnosis and management of bone and soft-tissue sarcomas. The identification of chromosomal aberrations such as translations, deletions, additions of a part or whole chromosome, and other markers are associated with specific tumor subtypes.

Material and Methods: Between 1998 and 2000, 78 bone and soft-tissue tumors were analyzed. Cytogenetic analysis was carried on a short-term cultured tissues by G-banding FISH and SKY procedures, as needed. Histopathological diagnoses included osteosarcoma – 16, Ewing’s sarcoma – 13, synovial sarcoma – 4, rhabdomyosarcoma – 4 (alveolar – 3, embryonal – 1), liposarcoma – 3, extra-abdominal fibromatosis – 3, alveolar soft part sarcoma – 12, and other soft-tissue sarcoma – 12. Other diagnoses included 8 hematological malignancies and 13 benign tumors.

Results: Eight of the 16 osteosarcomas studies demonstrated complex hyperploid karyotypes compatible with the diagnosis of high-grade osteosarcoma. In most Ewing’s sarcoma, including three cases with a typical t(11;22) translocation, other chromosomal abnormalities such as trisomies of chromosomes 5,6,8, and 14 were observed. Three of the four synovial sarcomas had the typical t(X;18)(p11.2;q11.2) translocation. One of the synovial sarcomas was initially diagnosed on a histopathological basis as Ewing’s sarcoma but the cytogenetic analysis showed a complex X;18 translocation and led to change in diagnosis and related treatment. Only one of the alveolar rhabdomyosarcomas demonstrated the typical t(2;13)(q35;q14) translocation, while hypertetraploid set with double minutes (dmin) was detected in the other two cases. By using SKY, chromosome 1 was determined as the origin of one of the dmins, suggesting that PAX7 amplification could be involved in the pathogenesis of this tumor.

Conclusions: Cytogenetic analysis of bone and soft-tissue tumors are of important clinical value for accurate diagnosis of tumor type. It can also provide information suggesting the pathogenesis of these tumors.

Introduction: Modern cancer treatment has substantially increased the survival of patients with various malignancies. One of the late sequelae of a successful treatment is the development of a second malignant tumor. However, in many cases of second primary cancers, exposure to chemotherapy or radiation therapy is not evident, and it should be postulated that the putative mechanism for the development of the second cancer is different.

Material and Methods: Retrospective search of data files of 610 patients with soft-tissue or bone sarcomas that were treated by the authors from January 1995 through December 1999 were performed.

Results: Out of 375 patients with soft-tissue sarcoma (STS), 28 (7.5%) developed other malignant neoplasm either before or after its diagnosis. The second tumor types included mainly STS and renal cell carcinoma. The time interval between the diagnosis of STS and the second malignancy was o to 21 years. Three patients developed a third primary tumor within 0–3 years after the diagnosis of the second tumor. The median overall survival was > 78 months.

Conclusions: The phenomenon of two or three primary neoplasms in patients in whom one of the tumors was STS occurs in a rate of 7.5% – a significantly higher rate than the occurrence of STS among the general cancer population (1%). Most cases are detected incidentally. The clinical implications are the need to search for an occult second primary in patients with STS as an integral part of their follow-up. It is especially true in patients with primary MFH who show increased risk for developing a renal cell carcinoma.

Introduction: Soft-tissue sarcomas (STS) in children and young adults are rare. This is a heterogeneous group of tumors, which is traditionally divided to rhabdomyo-sarcomas and non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS). These tumors are further classified to high- and low-grade tumors.

Material and Methods: Between 1988 and 1999, the authors treated 50 patients (25 males, 25 females) under the age of 20 who were diagnosed with a soft-tissue sarcoma.

Histopathological Diagnoses: rhabdomyosarcoma – 11, synovial sarcoma – 6, other high-grade STS (extraskeletal Ewing’s sarcoma, epitheloid sarcoma, neurofibrosarcoma, hemangiopericytoma, fibrosarcoma, and unclassified sarcoma) – 17. Seven patients were diagnosed with low-grade STS and 9 patients with an aggressive desmoid tumor.

Anatomic Location: Lower extremities – 30, upper extremities – 9, shoulder girdle – 2, trunk – 4, pelvic girdle – 5.

Preoperative Treatment: Thirty patients received neo-adjuvant chemotherapy, four patients underwent isolated limb perfusion with TNF and melphalan, and one patient received preoperative radiation therapy. Surgery: Forty-seven underwent limb-sparing resections and 3 underwent primary amputation. Wide margins were achieved in 37 patients and marginal margins in 10. Intralesional resection was performed in 3 patients.

Postoperative Treatment: Thirty-seven patients received adjuvant chemotherapy and 34 received radiation therapy.

Oncological Status: At the most recent follow-up, 24 patients of the 37 patients with high-grade STS have no evidence of disease, three are alive with disease, and seven are dead. Fourteen of the 16 patients with low-grade tumors have no evidence of disease and 2 are alive with disease. There were 4 secondary amputations due to local tumor recurrence.

Conclusions: Management of soft-tissue sarcomas in children and young adults requires the judgmental use of pre- and postoperative treatment modalities. Local tumor control can be achieved in the majority of the patients. A longer follow-up is required to determine the overall survival of these patients.

Introduction: Total scapular resection causes a significant functional loss because of the sacrifice of the glenoid, which serves as a stable base for shoulder motion. The authors analyze their experience with two types of reconstructions following total scapular resection; suspension of the humeral head from the clavicle without endoprosthetic reconstruction of the scapula and endoprosthetic scapular reconstruction.

Materials and Methods: Between 1979 and 1997, the authors treated 23 patients with scapular tumors that required total scapular resection. Patients were diagnosed with 14 bone and 9 soft-tissue tumors. Resection included total scapulectomy in 12 patients and enbloc resection of the scapula and humeral head in 11 patients.

Reconstruction: All eleven patients who had resection of their humeral head underwent reconstruction of the humerus with endoprosthesis. Scapular endoprosthesis was further installed in 7 patients and suspension of the humeral head from the clavicle with a Dacron tape was performed in 16 patients (Suspension of the prosthetic humeral head from the clavicle – 4 patients; suspension of the native humeral head from the clavicle – 12 patients). Endoprosthetic reconstruction of the scapula was feasible only when the periscapular musculature was sufficient for endoprosthetic attachment and coverage. The scapular prosthesis was attached to the prosthetic humeral head with a Goretex® sleeve, which served as an artificial joint capsule. All patients were followed for a minimum of 2 years; follow-up included physical examination, radiological evaluation and functional evaluation according to the American Musculoskeletal Tumor Society system.

Results: Elbow range-of-motion and hand dexterity were similar in the two groups of patients. However, compared with patients who undergone humeral suspension, those who had scapular endoprosthesis had better abduction (60°–90° vs. 10°–20°) of the shoulder joint. Moreover, these patients had better cosmetic appearance of the shoulder girdle. There were no deep wound infections, prosthetic failures, or secondary amputations. Overall, 6 patients who had scapular prosthesis (86%) and 10 patients who had humeral suspension (62%) had a good-to-excellent functional outcome.

Conclusions: The number of patients who underwent a scapular endoprosthetic reconstruction is small and does not allow a valid statistical analysis; however, the authors feel that scapular endoprosthesis reconstruction is associated with better functional and cosmetic outcomes, when compared to humeral suspension. The authors recommend reconstruction of the scapula with endoprosthesis when periscapular musculature, remaining after tumor resection allows attachment and coverage of the prosthesis.

Introduction: Telomerase is a ribonucleoprotein that adds TTA GGG nucleotide repeated into the ends of eukaryotic chromosomes to maintain their integrity. Most of the normal somatic cells do not express telomerase while telomerase is expressed in the vast majority of malignant tumor cells. Contradictory and limited data have been reported concerning the telomerase expression in soft-tissue sarcomas. The current study evaluates telomerase expression in a single histologic type of a high-grade soft-tissue sarcoma.

Materials and Methods: A non-radioactive in situ hybridization (ISH) method was used to study the expression of the RNA component of human telomerase in 55 paraffin embedded archives tissue samples of patients who were diagnosed with synovial sarcoma, the diagnosis of which was based on morphologic, immunohistochemical, and cytogenetic characteristics. The intensity and distribution of telomerase RNA was scored by two different investigators. Intensity was graded as weak, moderate, or intensive. These parameters were further correlated to the oncologic status of the patient.

Results: The majority of the investigated specimens demonstrated moderate to intensive telomerase RNA intensity with a diffuse distribution throughout the specimen. A positive correlation was found between telomerase intensity and progression of the underlying disease.

Conclusions: Results of the current series suggest that upregulating of telomerase expression may play a role in the pathogenesis and biological activity of synovial sarcoma. This upregulation as detected by ISH assay may be a useful prognostic tool in the evaluation of these patients.

Background: The c-ebB-2 gene and its products (also designated HER-2 and c-neu) encode for a 185-kd transmembrane glycoprotein with intracellular tyrosine kinase activity. C-erbB-2 belongs to the epidermal growth factor receptor family, of which there are four known members, and has molecular homology to the epidermal growth factor receptor. It seems that this family is critical in control of growth, differentiation, and mobility of many normal and transformed epithelial cell types.

Materials and Methods: We have looked for over expression of c-erbB-2 gene product in 230 cases of soft tissue sarcoma, in order to establish a possible new prognostic marker, and a potentially new treatment option.

Results: In all the cases, irrespective of the sarcoma histological type, the immunostaining for erbB-2 was negative.

Conclusions: Applications of erbB-2 for prognostication as well as the option of receptor targeting by trastuzumab monoclonal antibodies were aborted.

Background: We have recently observed that many of our sarcoma patients presented also with thyroid disorders. Literature data are almost unavailable on this topic.

Materials and Methods: Retrospective analysis of files of patients with sarcoma and clinically overt thyroid disorders.

Results: Out of 375 patients with soft tissue sarcomas (STS) and 235 with bone sarcoma (BS) including small blue round cell tumors (SBRC), 28 patients (4.6%) had an associated significant thyroid disorder. The types of sarcoma were mainly liposarcoma followed by malignant fibrous histiocytoma, leiomyosarcoma and bone sarcoma. The primary sites were mainly limb and trunk. The interval between the diagnosis of the thyroid disorder and the sarcoma varied between {−14} years (thyroid first) and {+16.5} years (thyroid later) with a median of {−0.2} years. Thyroid disorders included goiter, thyroiditis and carcinoma.

Conclusions: There are basic-science and clinical evidences to a possible common pathway that leads to the association between overt thyroid disorders and sarcomas of bone or soft tissues. Oncogene erbA activity is related to thyroid receptors to T3 and to development of sarcoma. Cross talk of the sarcoma oncogene and the erbA might contribute to the development of sarcoma. The thyroid hormone receptor and the highly related viral oncoprotein v-erbA are found exclusively in the nucleus as stable constituents of chromatin. It has been shown that v-erbA can block the spontaneous differentiation in erythroid cells transformed by various retroviral oncogenes. V-erbA can alter the spectrum of neoplasia induced by the v-src oncogene, which causes predominantly sarcomas and erythroblastosis in chicks. The erbA can cooperate with other oncogenes such as v-erbB or with v-fms, v-ras, and c-kit. Cooperation with v-myc may play a role in the development of rhabdomyosarcoma especially in thyroid hormone deficiency state. The possible clinical implications are the need to screen patients with sarcoma to thyroid disorders, and patients with thyroid disorders for malignant diseases.

Introduction: Despite advances in limb-sparing techniques, the proximal tibia remains a difficult area in which to perform a wide resection of extensive bone tumors due to the intimate relationship to the nerves and blood vessels, inadequate soft-tissue coverage, and the need to reconstruct the extensor mechanism. The current long-term follow-up study, based on the experience with 55 patients who underwent proximal tibia endoprosthetic reconstruction emphasizes reconstruction of the extensor mechanism.

Materials and Methods: Between 1980 and 1997, 55 patients underwent proximal tibia resection with endoprosthetic reconstruction. There were 34 males and 21 females whose age ranged from 8 to 56 years (median, 27 years. Diagnoses were: primary bone sarcomas – 48, benign aggressive lesions – 6, and failure of previous osteoarticular allograft reconstruction – 1. Intra-articular resection with en bloc removal of the tibial tuberosity was performed in all cases. Endoprosthetic reconstruction was performed with 39 modular, 16 custom-made prostheses. Reconstruction of the extensor mechanism included reattachment of the patellar tendon to the prosthesis with a Dacron tape and reinforcement with a gastrocnemius flap and bone grafting of the patellar tendon-prosthesis interface. Rehabilitation emphasized prolonged immobilization of knee joint in full extension.

Results: All patients were followed for a minimum of 2 years (range 24–235 months, median – 75.5 months). Full extension to extension lag of 20° was achieved in 44 patients (78%), extension lag of 20° to 30° was found in 10 patients (19%), and extension lag of 40° was found in 1 patient (3%). Eight patients required an additional procedure which involved reinforcement of the patellar tendon with either combined quadriceps tendon and Goretex graft construct (seven patients) or simple plication of the tendon (one patient). Seven of these patients gained an extension lag of less than 20°. Overall, function was estimated to be good to excellent in 48 patients, fair in 6, and poor in one patient.

Discussion: Extension lag of up to 20° is considered compatible with activities of daily living. Emphasis on reattachment of the patellar tendon to the prosthesis and its reinforcement with a gastrocnemius flap and bone graft achieved that goal in the majority of the patients.

Secondary reinforcement of the patellar tendon is recommended for extension lag of more than 20°.

Background: The surgical treatment of extensive diffuse Pigmented Villonodular Synovitis (PVNS) of large joints alone, is unsatisfactory, with high rates of local recurrence. Postsynovectomy adjuvant treatment with external beam radiation therapy or intraarticular injection of Yttrium90 (Y90) yielded better results.

Aims: Experience with 10 cases treated with debulking surgery followed by intraarticular injection of Y90 is reported.

Methods: Between January 1989 and June 1998, 10 patients (8 males and 2 females aged 15049 years) with extensive diffuse PVNS were treated. In 6 patients the knee joint, in 3 patients the ankle joint, and in 1 patient the hip joint were involved. The 10 patients underwent 15 operations, 1 patient had 3 surgical procedures, and 3 patients underwent 2 surgeries (interval between re-operations for local recurrence were 2–4 years). All patients had an intraarticular injection of 15–25 mCi of Y90, 6–8 weeks after the last surgery.

Results: Follow up time was 2.5–12 years (mean 6 years). All patients were followed by repeated computerized tomography (CT) scans, magnetic resonance imaging (MRI), plain X-ray films and bone scans semi-annually. In 9 patients no evidence of disease and no progression of bone or articular destruction have been noted. In 1 patient stabilization of disease was achieved with no further evidence of bony or articular damage. No complications were noticed after surgery, nor after the intraarticular Y90 injection.

Conclusions: A combination of debulking surgery with intraarticular injection of Y90 for extensive diffuse PVNS of major joints is a reliable way of treatment with good results.