Eric L. Staals

Malignancy in giant cell tumor of bone

The term malignant giant cell tumor embraces multiple entities and therefore can be confusing. The goals of the current study were to define the clinicopathologic and histologic features of malignancy in giant cell tumors and to clarify the terminology.

Synovial sarcoma: retrospective analysis of 250 patients treated at a single institution.

The optimal treatment for synovial sarcoma remains controversial. Treatment, outcome, and prognostic factors in patients treated in a single institution were examined.

Percutaneous CT-guided biopsy of the spine: results of 430 biopsies

Biopsies of lesions in the spine are often challenging procedures with significant risk of complications. CT-guided needle biopsies could lower these risks but uncertainties still exist about the diagnostic accuracy. Aim of this retrospective study was to evaluate the diagnostic accuracy of CT-guided needle biopsies for bone lesions of the spine. We retrieved the results of 430 core needle biopsies carried out over the past fifteen years at the authors’ institute and examined the results obtained. Of the 430 biopsies performed, in 401 cases the right diagnosis was made with the first CT-guided needle biopsy (93.3% accuracy rate). Highest accuracy rates were obtained in primary and secondary malignant lesions. Most false negative results were found in cervical lesions and in benign, pseudotumoral, inflammatory, and systemic pathologies. There were only 9 complications (5 transient paresis, 4 haematomas that resolved spontaneously) that had no influence on the treatment strategy, nor on the patient’s outcome. In conclusion we can assert that this technique is reliable and safe and should be considered the gold standard in biopsies of the spine.

Prognostic significance of immunohistochemical expression of ezrin in non-metastatic high-grade osteosarcoma.

Ezrin is a membrane‐cytoskeleton linker protein involved in regulating growth and metastatic behaviour of cancer cells. The study evaluated ezrin expression and its prognostic value in patients with non‐metastatic osteosarcoma.

Periosteal Osteosarcoma A Single-Institution Experience

Periosteal osteosarcoma is a rare variant of osteosarcoma. Wide surgical removal is the mainstay of treatment, but controversy remains about the role of chemotherapy. The objective of this study was to review and analyze the clinical and treatment‐related factors that influence the survival of patients with periosteal osteosarcoma who received treatment in a single institution.

Tenosynovial giant cell tumour/pigmented villonodular synovitis: Outcome of 294 patients before the era of kinase inhibitors

BACKGROUND Tenosynovial giant cell tumour/pigmented villonodular synovitis (TGCT/PVNS) is a benign neoplasm of synovium and tendon sheath. We conducted a retrospective pooled analysis in three major referral centers. METHODS Patients treated between 1998 and 2008 were examined. Only patients presenting with primary disease or first relapse were included. 5-year local failure free survival (5-year-LFFS) was analysed. RESULTS 294 patients were included: 254 with new diagnosis and 40 in 1st local recurrence (171 F/123 M; median age: 36 years; tumour size ⩽2 cm in 27% of patients, >2 to ⩽5 cm in 41%, and >5 cm in 32%). A diffuse pattern was reported in 69%, localised in 31%. No metastases were documented. Local failure (LF) was reported in 28% of patients: 36% in diffuse pattern, 14% in localised (p = 0.002); median time to LF: 16 months. With a median follow-up of 4.4 years, 5-year-LFFS was 66%, with multiple (up to five) local recurrences in 40% of relapsed patients. Size <2 cm, macroscopically complete resection, female gender and new diagnosis were associated with a better local control. After multivariate analysis, a previous relapse was independently associated with local failure. CONCLUSIONS This study underlines the propensity of TGCT/PVNS to multiple local recurrences. In absence of clinical factors, biological studies are needed to identify prognostic factors of local failure. After a first local recurrence, surgery does not seem to have a curative potential. In these high risk patients, studies addressing the role of target therapies are needed.

Hemangioendothelioma of the Spine

A review of the patient files from our institution’s oncology department showed that between 1950 and 2000, 11 patients were diagnosed with hemangioendothelioma of the spine, a rare, low-grade malignant vascular tumor. After reevaluation of the diagnoses by orthopaedic oncologists and pathologists, three patients were excluded; therefore eight patients formed the study group. The eight lesions were located in the thoracic (three) or lumbar spine (five, one in each vertebral level). Computed tomography scans revealed expansile lytic process. All lesions involved the vertebral body, but only one was diffuse with spinous process involvement. Treatment included: external beam irradiation alone (one patient), curettage and external beam irradiation (one patient), laminectomy and external beam irradiation (two patients), anterior resection only (two patients), and anterior resection with postoperative external beam irradiation (two patients). Patients without spinal stabilization had chronic low back pain; no patient with spinal stabilization had such pain. One patient who had surgical resection and radiation therapy had a radiation-induced sarcoma develop after 4 years. One patient who was treated with radiation therapy alone had a metastatic lung lesion develop. No other patient had tumor recurrence or progression. Therefore, patients with hemangioendothelioma of the spine may be treated with radiation therapy alone, surgery alone, or a combination thereof. However, because the number of patients in the current series was small, no definitive recommendations regarding treatment may be made. Resection of large lesions and stabilization of vertebral collapse may decrease back pain.

High-grade surface osteosarcoma: a review of 25 cases from the Rizzoli Institute.

High‐grade surface osteosarcoma is a rare variant of surface osteosarcoma and is associated with a poor prognosis.

Parosteal osteosarcoma dedifferentiating into telangiectatic osteosarcoma: importance of lytic changes and fluid cavities at imaging

PurposeThis study was performed to assess the imaging findings in cases of parosteal osteosarcoma dedifferentiated into telangiectatic osteosarcoma. Parosteal osteosarcoma is a low-grade well-differentiated malignant tumor. Dedifferentiation into a more aggressive lesion is frequent and usually visible on imaging as a central lytic area in a sclerotic mass. Only one case of differentiation into a telangiectatic osteosarcoma has been reported. As it has practical consequences, with a need for aggressive chemotherapy, we looked for this rather typical imaging pattern.Materials and methodsReview of 199 cases of surface osteosarcomas (including 86 parosteal, of which 23 were dedifferentiated) revealed lesions suggesting a possible telangiectatic osteosarcoma on imaging examinations in five cases (cavities with fluid). Histology confirmed three cases (the two other only had hematoma inside a dedifferentiated tumor). There were three males, aged 24, 28, and 32. They had radiographs and CT, and two an MR examination.ResultsLesions involved the distal femur, proximal tibia, and proximal humerus. The parosteal osteosarcoma was a sclerotic, regular mass, attached to the cortex. A purely lytic mass, partially composed of fluid cavities was easily detected on CT and MR. It involved the medullary cavity twice, and remained outside the bone once. Histology confirmed the two components in each case. Two patients died of pulmonary metastases and one is alive.ConclusionKnowledge of this highly suggestive pattern should help guide the initial biopsy to diagnose the two components of the tumor, and guide aggressive treatment.

Sex- and Age-Related Chemotherapy Toxicity in Patients with Non-Metastatic Osteosarcoma

Abstract The influence of age and sex on chemotherapy-related toxicity was evaluated in children and adults with non metastatic osteosarcoma. Treatment consisted of methotrexate (MTX, 12 g/m2), cisplatin (CDP 120 mg/m2) and doxorubicin (ADM 75-90 mg/m2) and high-dose ifosfamide (HDIFO). Toxicity data from 1,051 courses (295 with MTX, 756 based on doxorubicin, cisplatin and high-dose ifosfamide) were analyzed. Children (4-14 yrs) and females showed a higher incidence of grade 4 neutropenia and thrombocytopenia and were more frequently hospitalized for neutropenic fever compared to adolescents and young adults (AYA, 15-19 yrs) and adults (>20-40 yrs). Delayed MTX excretion was higher in adults than AYA and children. Adults (up to 40 years) can be treated with pediatric protocols for osteosarcoma and they experience lower hematologic toxicity compared to pediatric population. Further investigations on sex-related susceptibility to chemotherapy in osteosarcoma patients are recommended.