Gerard J. Powell

ACCURACY OF COMPUTED TOMOGRAPHY GUIDED CORE NEEDLE BIOPSY OF MUSCULOSKELETAL TUMOURS

Background:  The accurate diagnosis of musculoskeletal tumours is important for successful treatment. Image guided biopsy is gaining increasing acceptance for obtaining tissue for diagnosis. The aim of the present study is to assess the accuracy of computed tomography (CT)‐guided core needle biopsy of musculoskeletal tumours.

Gender-specific activity of chemotherapy correlates with outcomes in chemosensitive cancers of young adulthood

Good evidence indicates that adolescents and young adults (AYAs) with cancer do badly compared with children with similar cancers. The reasons are poorly understood. Australian registry data on 14,824 cancers of adolescence and young adulthood seen between 1982 and 2002 were reviewed. A detailed substudy of clinical characteristics was analyzed from 179 AYAs with Hodgkin lymphoma (HL), Ewing sarcoma (ES) or osteosarcomas (OS) treated at a single institution. Despite significant improvements in survival for both groups over the period in question, for acute lymphoblastic leukaemia, rhabdomyosarcoma, ES, OS and HL, survival for AYAs was worse than for children. For ES, OS and HL, the survival gap occurred almost entirely in males (Hazard ratios compared with female AYAs of 1.8 [p < 0.01], 1.4 [p = 0.03] and 1.5 [p < 0.01] respectively). Survival outcomes from ES, OS and HL for female AYAs were not significantly different from children of either sex. For brain tumors and thyroid cancers, which are primarily treated surgically, there were no gender‐related differences in outcomes. Although no differences in tumor stage or compliance were identified, male AYAs experienced less toxicity and lower response rates to chemotherapy (p = 0.008). Young males account almost entirely for excess mortality from chemosensitive cancers of adolescence and young adulthood compared to children, which may be due to relative underdosing with current chemotherapy dosing algorithms.

A phase Ib/II translational study of sunitinib with neoadjuvant radiotherapy in soft-tissue sarcoma.

Background:Preoperative radiotherapy (RT) is commonly used to treat localised soft-tissue sarcomas (STS). Hypoxia is an important determinant of radioresistance. Whether antiangiogenic therapy can ‘normalise’ tumour vasculature, thereby improving oxygenation, remains unknown.Methods:Two cohorts were prospectively enrolled. Cohort A evaluated the implications of hypoxia in STS, using the hypoxic tracer 18F-azomycin arabinoside (FAZA-PET). In cohort B, sunitinib was added to preoperative RT in a dose-finding phase 1b/2 design.Results:In cohort A, 13 out of 23 tumours were hypoxic (FAZA-PET), correlating with metabolic activity (r2=0.85; P<0.001). Two-year progression-free (PFS) and overall (OS) survival were 61% (95% CI: 0.44–0.84) and 87% (95% CI: 0.74–1.00), respectively. Hypoxia was associated with radioresistance (P=0.012), higher local recurrence (Hazard ratio (HR): 10.2; P=0.02), PFS (HR: 8.4; P=0.02), and OS (HR: 41.4; P<0.04). In Cohort B, seven patients received sunitinib at dose level (DL): 0 (50 mg per day for 2 weeks before RT; 25 mg per day during RT) and two patients received DL: −1 (37.5 mg per day for entire period). Dose-limiting toxicities were observed in 4 out of 7 patients at DL 0 and 2 out of 2 patients at DL −1, resulting in premature study closure. Although there was no difference in PFS or OS, patients receiving sunitinib had higher local failure (HR: 8.1; P=0.004).Conclusion:In STS, hypoxia is associated with adverse outcomes. The combination of sunitinib with preoperative RT resulted in unacceptable toxicities, and higher local relapse rates.

Radiation therapy in the treatment of desmoid tumours reduces surgical indications

BACKGROUND While several modalities have been proposed for the treatment of desmoid tumour/aggressive fibromatosis, high local recurrence rates have been reported. We present a retrospective study of including patients treated with radiation therapy, some of them in combination with surgical resection. PATIENTS AND METHODS Thirty-four consecutive patients were included (mean age 40+/-16 years, 9 male). Complete follow-up was available in 31 patients (51+/-36 months). Seventeen patients (50%) were treated with radiation therapy alone, 17 patients with radiation therapy and surgery. Radiation therapy (external beam) was applied in most cases to a total dose of 50.4 Gy in 28 fractions. The lesion was located in the upper extremity in 11 patients, in the lower extremity in 14 cases and on the trunk in 9 cases. RESULTS Overall recurrence/progression free survival was 88.5% at 5 years and 77.5% at 10 years. Recurrence free survival of the subset of patients undergoing combined treatment with radiation therapy and surgical resection was 83.6% at 5 years and 10 years. In patients who did not receive surgery but only radiation therapy, MRI showed a complete response in 20%, a partial response in 20%, and stable disease in 53% of cases. In this subset, two-third of patient had a metabolic response to radiotherapy (i.e. decrease uptake on the thallium-210 scan after radiotherapy compared to pre-therapy levels). CONCLUSION Low recurrence rates can be achieved with the use of radiation therapy alone in selected cases. Patients with a metabolic response (decrease) to radiotherapy may be treated with a non-surgical approach. Surgery might be considered in patients with a poor metabolic response to radiotherapy.

Outcomes of preoperative radiotherapy and resection of retroperitoneal sarcoma.

Preoperative radiotherapy (RT) is an important component of the management of retroperitoneal sarcoma (RPS). We aimed to establish the feasibility of this approach by determining the accuracy of computed tomography (CT)‐guided core biopsy, proportion of patients completing treatment, rates of acute toxicity and surgical complications, and treatment outcomes.

Multidisciplinary approach to diagnosis and management of osteosarcoma - a review of the St Vincent's Hospital experience.

BackgroundOsteosarcoma is the most common primary malignant bone tumour in children and young adults. Despite advances in the diagnosis and management of osteosarcoma, there have been few recent studies describing the experiences of tertiary referral centres. This paper aims to describe and discuss the clinical features, pre-operative work-up, management and outcomes of these patients at St Vincents Hospital (Melbourne, Australia).MethodsRetrospective study of fifty-nine consecutive patients managed for osteosarcoma at St Vincents Hospital between 1995 and 2005.ResultsMedian age at diagnosis was 21 (range, 11–84) years. Gender distribution was similar, with thirty-one male and twenty-eight female patients.Twenty-five patients had osteosarcoma in the femur, eleven each were located in the humerus and tibia, six were identified in the pelvis, and one each in the clavicle, maxilla, fibula, sacrum, ulna and radius.Pre-operative tissue diagnosis of osteosarcoma was obtained through computed tomography-guided percutaneous biopsy in over ninety percent of patients.Following initial therapy, over fifty percent of patients remained relapse-free during the follow-up period, with twelve percent and twenty-seven percent of patients documented as having local and distant disease recurrence, respectively. Of patients with recurrent disease, sixty-two percent remained disease-free following subsequent surgical intervention (most commonly, pulmonary metastatectomy).ConclusionPatient outcomes can be optimised through a multidisciplinary approach in a tertiary referral centre. At St Vincents Hospital, survival and relapse rates of patients managed for osteosarcoma compare favourably with the published literature.

Tuberculosis masquerading as malignancy: a multimodality approach to the correct diagnosis – a case report

BackgroundExtrapulmonary tuberculosis is one of the great mimickers of medicine, and often masquerades as malignancy. As a result, patients may be referred to oncologists and surgeons for further evaluation and management, delaying the institution of appropriate anti-tuberculous drug therapy.Case presentationWe present the case of a 21 year old man with tuberculous osteomyelitis, who was referred to the Bone and Soft Tissue Sarcoma Service at our institution with a provisional diagnosis of malignancy. Further investigation revealed extensive retroperitoneal abdominal and pelvic lymphadenopathy. The recognition of certain patterns on imaging, and finally the isolation of Mycobacterium tuberculosis from tissue samples obtained under image guidance, enabled the correct diagnosis to be made.ConclusionThis case highlights the importance of remaining cognisant of the protean manifestations of extrapulmonary tuberculosis, and illustrates the advantage of a clinically directed multi-modality imaging approach to diagnosis.

Value of PET scan in patients with retroperitoneal sarcoma treated with preoperative radiotherapy

PURPOSE Preoperative radiotherapy provides advantages in the management of retroperitoneal sarcoma (RPS). We describe our experience treating a cohort who underwent pre- and post-radiotherapy functional imaging with FDG-PET scan. METHODS AND MATERIALS Consecutive patients presenting between January 1999 and December 2009 with a diagnosis of either primary or recurrent RPS were identified from the hospital patient record database using ICD codes, and cross-referenced with the completed radiotherapy course database. Those patients suitable for preoperative radiotherapy and surgery who underwent both pre- and post-radiotherapy FDG-PET were included. Exclusions included presence of metastatic disease, age under 18 years and/or paediatric histology, and treatment with palliative intent. RESULTS Eleven patients were included, of whom six were male. Median age was 63 years (range, 38-78 years). The majority of patients had Stage T2b, high-grade disease. Ten patients were treated at initial presentation and one at first local recurrence. A malignant diagnosis was confirmed in all patients who underwent CT-guided core biopsy; a diagnosis of sarcoma was reached in 91%. Sensitivity of FDG-PET imaging was 100%. Metabolic partial or complete response did not correlate with change in tumour size, nor pathological response assessment. Pulmonary and hepatic metastatic disease was detected in one patient on post-treatment imaging. All patients in the cohort completed preoperative radiotherapy. There was no grade 3 or 4 toxicity. Sixty-four percent proceeded to radical resection. Complete macroscopic excision was achieved in all cases. There was no perioperative mortality. CONCLUSION Combined therapy with preoperative radiotherapy and surgery has acceptable levels of toxicity. CT-guided core biopsy is an accurate means of confirming a diagnosis of RPS prior to definitive treatment. Utility of PET scan in the management of RPS is evolving and further investigation is warranted.

Pigmented villonodular synovitis of the hip mimicking soft-tissue sarcoma: a case report

Pigmented villonodular synovitis is a rare and benign but potentially locally aggressive disease that should be considered in younger patients who present with monoarticular joint symptoms and pathology. We present a 30-year-old Sudanese woman with a huge mass arising from the right hip joint. A multimodality radiological approach to investigation and diagnosis is demonstrated and discussed. Histopathological examination of the resected specimen confirmed the diagnosis of pigmented villonodular synovitis with the mass consisting of a proliferation of fibrohistiocytic cells, abundant haemosiderin, foamy histiocytes, and occasional giant cells. The patient made a good recovery, with mobility aided by arm crutches and a hip abduction brace.

Magnetic resonance imaging of bone tumors and joints.

Purpose: In this invited review, the main subtypes of commoner bone tumors will be reviewed. Background and general concepts on pathology and surgery will be given with magnetic resonance imaging features of bone tumors. Text: The main malignant bone tumors being: osteogenic sarcoma, Ewings sarcoma, and chondrosarcoma are reviewed. Malignant intra-articular tumors are mentioned. The differential diagnoses including tumor mimickers are also reviewed. Conclusion: Magnetic resonance imaging allows for basic anatomical review and characterization of tumor type for diagnosis, therapy monitoring, and preoperative planning. A team approach to tumor imaging and treatment is optimal.