Malignant giant cell tumor of bone or soft tissue treated by surgery with or without radiotherapy.

Malignant giant cell tumor of bone or soft tissue (MGCTBS) is one of the malignant tumors associated with poor prognosis. However, it remains controversial whether the combined treatment of both surgery and radiotherapy (surgery + RT) leads to better survival outcomes than surgical treatment alone (surgery alone) does for patients diagnosed with MGCTBS. We compared the two treatment strategies using the data provided by Surveillance, Epidemiology, and End Results (SEER) program. About 357 patients with MGCTBS who received either surgery + RT or surgery alone between 1975 and 2016 in the USA were identified and then matched based on their propensity scores estimated using the patients' baseline characteristics. We also performed a subgroup analysis for patients with high-grade and regional/distant tumor extension. Unadjusted Kaplan-Meier curves suggested that the surgery alone group had a better 10-year survival profile than the surgery + RT group. After propensity score matching, there was no statistical difference between the two treatment groups with respect to the 10-year cancer-specific survival and overall survival distributions. A subsequent subgroup analysis demonstrated that the surgery alone group has a similar 10-year survival comparing with the surgery + RT group for patients with high-grade and regional/distant tumor extension. The results of our study suggest that RT should not be recommended as a regular therapeutic method for MGCTBS, even for patients with high-grade histology and distant metastasis Clinical Significance: This study may provide better decision making for surgeons dealing with malignant giant cell tumor of bone or soft tissue. Type of study: Observation study. Level of evidence: Level III.

A multidisciplinary approach to giant cell tumors of tendon sheath and synovium--a critical appraisal of literature and treatment proposal.

Giant cell tumors deriving from synovium are classified into a localized (GCT of tendon sheath; GCT-TS) and diffuse form (diffuse-type GCT, Dt-GCT). We propose a multidisciplinary management based upon a systematic review and authors' opinion. Open excision for GCT-TS and open synovectomy (plus excision) for Dt-GCT is advised to reduce the relatively high recurrence risk. External beam radiotherapy should be considered in severe cases, as Dt-GCT commonly extends extra-articular.

Giant cell tumor of the cervical spine: review and case report / Tumor de células gigantes da coluna cervical: revisão e relato de caso

The giant cell tumor of bone is a primary neoplasm, which can be locally aggressive, benign or low grade malignant tumors, that is uncommon in the vertebrae above the sacrum and even more rare in the cervical spine. Tumor radical excision, ?en bloc? is considered the ideal treatment, however frequently not doable, mainly in the cervical spine due to critical neurovascular structures involvement. Adjuvant radiotherapy can be used in cases of subtotal resection or tumor relapse, lowering recurrence rates of the tumor. Case report: female patient, 25 years-old, presenting with cervical pain e sensitivity disturbance in her left arm, with diagnosis of bone neoplasm in C3-C5. She underwent subtotal resection of the lesion, confirming the diagnostic of giant cell tumor of bone, cervical spine arthrodesis and adjuvant radiotherapy. On 30-month follow-up, she was out of pain complains with total recovery of the left arm sensibility.

O tumor de células gigantes da coluna é uma neoplasia primária que pode ser localmente agressiva, benigna ou com baixo grau de malignidade, sendo incomum em vértebras acima do sacro e ainda mais raro na coluna cervical. A excisão radical do tumor, ?em bloco?, é considerada o tratamento ideal, entretanto não é sempre factível, principalmente na coluna cervical pelo envolvimento de estruturas neurovasculares críticas. Radioterapia adjuvante pode ser usada em casos de ressecção subtotal ou recidiva tumoral, com redução da recorrência da neoplasia. Relato de caso: paciente do sexo feminino, de 25 anos, com quadro de cervicalgia e disestesia em membro superior esquerdo, com diagnóstico de processo expansivo afetando os corpos vertebrais de C3-C5. Foi submetida à exérese subtotal da lesão, com diagnóstico de tumor de células gigantes, artrodese de coluna cervical e radioterapia adjuvante. Sem recidivas no seguimento em 30 meses.

Radiation therapy for infiltrative giant cell tumor of the tendon sheath.

PURPOSE: Giant cell tumor of the tendon sheath (GCTTS) has a high recurrence after excision and can be a management challenge. Although experience with radiation therapy for GCTTS is limited, it is purported to control infiltrative cases and prevent recurrence. We describe our approach to primary and recurrent GCTTS, as well as our identification of infiltrative cases and their treatment with radiation therapy. METHODS: We reviewed 58 patients (32 men and 26 women) with GCTTS in the hand excised at 1 center between 1998 and 2009. Mean age at the time of excision at our center was 50 years. A total of 14 patients who had undergone primary excision at other centers were referred to our center with recurrent disease. Intraoperatively, we found infiltrative disease in 4 patients undergoing primary excision at our center and in 10 of the 14 patients referred to our institution with recurrent disease. All infiltrative (4 primary and 10 recurrent) cases were referred for radiation therapy. RESULTS: Of 14 patients with infiltrative tumors, 10 received radiation therapy (3 patients declined and 1 had a major comorbidity that precluded therapy). Radiation dose was either 35 Gy in 14 fractions or 48 Gy in 24 fractions. At 3.1 years' follow-up, none of the 10 patients treated with radiation therapy had recurrence. No long-term complications were associated with radiation therapy, and hand function was not adversely affected. We identified 4 recurrences, 2 of which were in patients with primary tumors without infiltrative features. Of the 4 patients who were referred for radiation but did not receive it, 2 patients developed recurrence by 2 years after referral. CONCLUSIONS: In cases of infiltrative GCTTS, radiation therapy may provide local tumor control with preservation of hand function. Radiation therapy may be particularly helpful when further surgery is not a good option. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Long-term outcome of the treatment of high-risk tenosynovial giant cell tumor/pigmented villonodular synovitis with radiotherapy and surgery.

BACKGROUND: The treatment of diffuse tenosynovial giant cell tumor (TGCT) requires extensive surgical resection of the hypertrophic synovium and multiple soft tissue masses yet still may result in high rates of local failure. The authors of this report examined their experience in treating patients with advanced/multiply recurrent TGCT with a combination of surgery and external-beam radiotherapy. METHODS: Fifty patients who were treated for TGCT with radiotherapy and surgery from 1972 to 2006 were identified. Patient demographics, radiotherapy treatment parameters, surgical treatment, and oncologic and functional outcomes were evaluated. All patients had pathologic review at presentation and required at least 1 year follow-up. RESULTS: Forty-nine patients had diffuse TGCT with both intra-articular and extra-articular disease (1 had malignant TGCT). Twenty-eight patients (56%) were referred after at least 1 local recurrence. Thirty patients (60%) underwent at least 2 operations before radiotherapy. The mean dose of radiation delivered was 39.8 gray. At a mean follow-up of 94 months (range, 19-330 months), 47 patients (94%) had not developed a recurrence or had stable disease/signal characteristics on serial cross-sectional imaging (for those patients who had gross residual disease at the time of radiotherapy). Two patients required subsequent total hip arthroplasty because of progressive osteoarthritis, and there were 4 cases of avascular necrosis (only 1 post-treatment). Forty-one patients had good/excellent function. CONCLUSIONS: For patients with extensive or multiple local relapses or when surgery alone would result in a large burden of residual disease or major functional loss, the addition of moderate-dose adjuvant radiotherapy provided excellent local control while maintaining good function with low treatment-related morbidity.

Giant cell tumour of the tendon sheath of the hand.

PURPOSE: To review outcomes of 106 patients after complete excision of the giant cell tumour of the tendon sheath of the hand, with or without postoperative radiotherapy. METHODS: Records of 77 women and 29 men aged 11 to 61 (mean, 31) years who underwent excision of giant cell tumours of the tendon sheath of the hand were reviewed. All patients presented with gradually progressive swelling; only 3 of them also presented with pain. The lesions were located on the dorsal aspect (n=11), the palmar aspect (n=66), both aspects (n=10), or circumferentially (n=14). No patient had multiple lesions. Five patients had bony erosion and 3 had neurovascular bundle involvement. RESULTS: The mean follow-up period was 12 (range, 4-22) years. 56 patients with well-encapsulated giant cell tumour in the palmer and/or dorsal aspects were classified as at low risk of recurrence. The remaining 50 patients were classified as at high risk of recurrence and underwent postoperative radiotherapy. None of the patients at low risk had any recurrence; 4 of those at high risk had recurrence despite radiotherapy. No complication was attributable to the irradiation, except that 3 patients had some dark pigmentation around the wound scar. CONCLUSION: Postoperative radiotherapy may have a role in reducing recurrence of the giant cell tumour of the tendon sheath of the hand.

[Helical tomotherapy for axial and paraspinal tumours: experience of Institut Bergonié (14 cases)]. / Expérience de l'institut Bergonié à propos de 14 cas de tomothérapie hélicoïdale de tumeurs axiales et para-axiales.

PURPOSE: To evaluate the feasibility of helical tomotherapy intensity-modulated radiotherapy for the treatment of axial and paraspinal tumours. PATIENTS AND METHODS: Fourteen consecutive patients with axial and paraspinal tumours at the Bergonié Institute between June 2007 and June 2009 were analysed. Various localisations were observed: four paravertebral tumours, five primitive vertebral tumours and six iliosacral or sacral tumours. RESULTS: The dose prescribed to the median of the planning target volume (PTV) varied from 45 to 68Gy depending on histology. The maximum dose delivered to the PTV (Dnear max or D2%) was 60.6Gy (range 46.3-69.1). In the five cases where the tumour was located close to the spinal cord, the median value of Dnear max was 40.1Gy (range 37.8-45.4). In the seven where the tumour was situated close to the cauda equina, the median value of Dnear max was 46.5Gy (range 39.2-60). For two cases in whom the sciatic nerve was the nervous structure at risk, the Dnear max was 64.4 and 67Gy. Six patients were in complete remission, six patients suffered a relapse at a median follow-up of 14.5 months (4-24), two died following local tumour progression and metastasis, tumoral stabilisation had been obtained in two patients. Treatment was well tolerated and was never interrupted in all patients. CONCLUSIONS: Results from this preliminary series are encouraging, particularly concerning the good tolerance of this new intensity-modulated radiotherapy presently more available than proton therapy.

Multidisciplinary management of primary tumors of the vertebral column.

OPINION STATEMENT: Primary spinal neoplasms are rare tumors that can lead to significant morbidity secondary to local bone destruction and invasion into adjacent neurological and vascular structures. These tumors represent a clinical challenge to even the most experienced physicians and require a multidisciplinary approach to ensure optimal patient outcomes. This review will discuss the most common primary bone tumors and focus on recent surgical, medical, and radiation treatment advances.

Giant cell tumor of the tendon sheath treated by brachytherapy (surface mold) technique-A technical illustration.

BACKGROUND: Giant cell tumors of the tendon sheath (GCTTS) are rare tumors involving diverse locations and have a tendency to recur postoperatively. Hence, radiotherapy (RT) plays a pivotal role as an adjuvant modality to prevent local recurrences. Although RT is an attractive treatment modality, treating these tumors in complex anatomical locations, such as the thumb with external beam radiation (EBRT), and ensuring adequate dosimetric coverage can be challenging. Literature regarding the optimal RT technique to treat GCTTS is limited in view of the rarity of these lesions and their complex anatomical locations. In this report, we describe a case of GCTTS of the thumb treated successfully with surface mold radiotherapy using a customized thermoplastic mold for the thumb. The patient tolerated the treatment well with excellent local control and functional outcome. METHODS AND MATERIALS: We developed a brachytherapy mold technique using a customized thermoplastic thumb mold with interstitial catheters and irradiated the target volume homogeneously using microselectron iridium-192 high-dose-rate (Nucletron B.V., Veenandaal, The Netherlands) sources. CONCLUSION: The index patient treated with surface mold therapy illustrates that this technique is safe and practical method to achieve excellent local tumor control with good functional outcome.

Malignant giant cell tumor of the skull base originating from clivus and sphenoid bone.

We present a case report of a giant cell tumor located in the skull base orginating from clivus and sphenoid bone treated by surgery and external beam radiotherapy (EBRT).

[Lung dissemination of giant cell tumor of femur. The case report]. / Rozsiew do pluc guza olbrzymiokomórkowego kosci udowej. Opis przypadku.

The case of 32 years old patient suffering for giant cell bone tumor of left femur was reported. After surgery (curettage and filling of tumor bed with bone cement followed by arthroplasty), a dissemination to lungs was found. Patient was treated by palliative lungs radiotherapy (10 x 1.1 Gy) and six cycles of chemotherapy (every four weeks) based on cisplatin (35 mg/m2) and doxorubicin (30 mg/m2) obtaining significant regression of metastases. Because of four persistent lung metastases, the extracranial radiosurgery using one fraction of 16 Gy was done.

[The role of radiotherapy in the treatment of bone neoplasms]. / Stellung der Strahlentherapie in der Behandlung der Knochentumoren.

Primary malignant bone neoplasms are relatively rare. The most common bone tumors are osteosarcoma,Ewing's sarcoma,chondrosarcoma, fibrosarcoma,malignant fibrous histiocytoma of bone, giant cell tumor, aneurysmal bone cyst and chordoma. These tumors are generally considered to be a radioresistant entities, but it has been suggested that radiotherapy may be effective in a palliative and in some curative situations, if a sufficient dose is given to an adequate volume. Only for the management of primary Ewing's sarcoma the radiation therapy is an essential part in the multimodal therapy concept. The most common bone neoplasms and the role of the radiotherapy are discussed in these chapter.

Proton radiotherapy in management of pediatric base of skull tumors.

PURPOSE: Primary skull base tumors of the developing child are rare and present a formidable challenge to both surgeons and radiation oncologists. Gross total resection with negative margins is rarely achieved, and the risks of functional, structural, and cosmetic deficits limit the radiation dose using conventional radiation techniques. Twenty-nine children and adolescents treated with conformal proton radiotherapy (proton RT) were analyzed to assess treatment efficacy and safety. MATERIALS AND METHODS: Between July 1992 and April 1999, 29 patients with mesenchymal tumors underwent fractionated proton (13 patients) or fractionated combined proton and photon (16 patients) irradiation. The age at treatment ranged from 1 to 19 years (median 12); 14 patients were male and 15 female. Tumors were grouped as malignant or benign. Twenty patients had malignant histologic findings, including chordoma (n = 10), chondrosarcoma (n = 3), rhabdomyosarcoma (n = 4), and other sarcomas (n = 3). Target doses ranged between 50.4 and 78.6 Gy/cobalt Gray equivalent (CGE), delivered at doses of 1.8-2.0 Gy/CGE per fraction. The benign histologic findings included giant cell tumors (n = 6), angiofibromas (n = 2), and chondroblastoma (n = 1). RT doses for this group ranged from 45.0 to 71.8 Gy/CGE. Despite maximal surgical resection, 28 (97%) of 29 patients had gross disease at the time of proton RT. Follow-up after proton RT ranged from 13 to 92 months (mean 40). RESULTS: Of the 20 patients with malignant tumors, 5 (25%) had local failure; 1 patient had failure in the surgical access route and 3 patients developed distant metastases. Seven patients had died of progressive disease at the time of analysis. Local tumor control was maintained in 6 (60%) of 10 patients with chordoma, 3 (100%) of 3 with chondrosarcoma, 4 (100%) of 4 with rhabdomyosarcoma, and 2 (66%) of 3 with other sarcomas. The actuarial 5-year local control and overall survival rate was 72% and 56%, respectively, and the overall survival of the males was significantly superior to that of the female patients (p = 0.002). Of the patients with benign tumors, 1 patient (giant cell tumor) had local failure at 10 months. The other 8 patients continued to have local tumor control; all 9 patients were alive at last follow-up (actuarial 5-year local control and overall survival rate of 89% and 100%, respectively). Severe late effects (motor weakness and sensory deficits) were observed in 2 (7%) of 29 patients. CONCLUSION: Proton RT for children with aggressively recurring tumors after major skull base surgery can offer a considerable prospect of tumor control and survival. Longer follow-up is necessary to assess the real value of protons, in particular with regard to bone growth and cosmetic outcome.

Radiosensitive giant cell tumour of the sphenoid bone.

Giant cell tumours rarely occur in the cranial region. We encountered a radiosensitive giant cell tumour of the sphenoid in a 12-year-old girl. After a two-stage operation, the residual tumour regrew rapidly. The adjuvant radiotherapy subsequent to additional surgery has suppressed the growth of the residual tumour for 5 years.

Giant-cell tumour of the tendon sheath. Is radiotherapy indicated to prevent recurrence after surgery?

Giant-cell tumour of the tendon sheath, also called pigmented villonodular synovitis, is a benign tumour with a high incidence of recurrence. We have tried to identify risk factors for recurrence. Of the 48 patients included in the study, 14 received radiotherapy after surgery. Only two (4%) had a recurrence. This compares favourably with previously reported incidences of between 25% and 45%.

Neurological recovery in a patient with recurrent aggressive giant cell tumour of the axis--a case report.

A rare case of an aggressive recurrent giant cell tumour of axis is presented. The problems encountered in diagnosis and management are discussed. High dose dexamethasone was found to be useful managing this inoperable aggressive tumour which was compressing the cord. Early diagnosis would facilitate wide excision of the tumour with good prognosis.