Osteosarcoma Arising as a Secondary Malignancy following Treatment for Hematologic Cancer: A Report of 33 Affected Patients from the Cooperative Osteosarcoma Study Group (COSS).

PURPOSE: Osteosarcoma may arise as a secondary cancer following leukemias or lymphomas. We intended to increase the knowledge about such rare events. PATIENTS AND METHODS: We searched the Cooperative Osteosarcoma Study Group's database for individuals who developed their osteosarcoma following a previous hematological malignancy. The presentation and treatment of both malignancies was investigated, and additional neoplasms were noted. Outcomes after osteosarcoma were analyzed and potential prognostic factors were searched for. RESULTS: A total of 33 eligible patients were identified (male: 23, female: 10; median age: 12.9 years at diagnosis of hematological cancer; 20 lymphomas, 13 leukemias). A cancer predisposition syndrome was evident in one patient only. The hematological cancers had been treated by radiotherapy in 28 (1 unknown) and chemotherapy in 26 cases, including bone-marrow transplantation in 9. The secondary bone sarcomas (high-grade central 27, periosteal 2, extra-osseous 2, undifferentiated pleomorphic sarcoma of bone 2) arose after a median lag-time of 9.4 years, when patients were a median of 19.1 years old. Tumors were considered radiation-related in 26 cases (1 unknown). Osteosarcoma-sites were in the extremities (19), trunk (12), or head and neck (2). Metastases at diagnosis affected eight patients. Information on osteosarcoma therapy was available for 31 cases. All of these received chemotherapy. Local therapy involved surgery in 27 patients, with a good response reported for 9/18 eligible patients. Local radiotherapy was given to three patients. The median follow-up was 3.9 (0.3-12.0) years after bone tumor diagnosis. During this period, 21 patients had developed events as defined, and 15 had died, resulting in 5-year event-free and overall survival rates of 40% (standard error: 9%) and 56% (10%), respectively. There were multiple instances of additional neoplasms. Several factors were found to be of prognostic value (p < 0.05) for event-free (osteosarcoma site in the extremities) or overall (achievement of a surgical osteosarcoma-remission, receiving chemotherapy for the hematologic malignancy) survival. CONCLUSIONS: We were able to prove radiation therapy for hematological malignancies to be the predominant risk factor for later osteosarcomas. A resulting overrepresentation of axial and a tendency towards additional neoplasms affects prognosis. Still, selected patients may become long-term survivors with appropriate therapies, which is an argument against therapeutic negligence.

Acute abdomen in pediatric radiology. / Das akute Abdomen in der Kinderradiologie.

BACKGROUND: Imaging is a common part of the workup for acute abdomen in childhood and adolescence. Rapid diagnosis is crucial for adequate therapy, especially in young children. METHOD: This review is intended to provide an overview of the typical causes of acute abdomen in children and adolescents and is specifically aimed at radiologists who do not regularly work in pediatric radiology. RESULTS AND CONCLUSION: Age-specific peculiarities make a division into three age groups useful: Newborns, infants and toddlers, and schoolchildren. Possible causes of acute abdomen in children range from congenital lesions, particularly in newborns, to entities well known in adult radiology, more commonly in school-aged children. Since younger children have a higher sensitivity to radiation and often a limited ability to cooperate, sonography and conventional X-ray are the primary imaging modalities used. Decisive advantages of sonography, especially in younger children, include its widespread availability, the lack of need for sedation, and the possibility of bedside examination. Supplementary cross-sectional imaging, usually MRI, is reserved for special clinical questions. Close interdisciplinary cooperation between attending clinicians and radiologists is essential for efficient diagnostics in this patient group. KEY POINTS: · Age-specific features must be observed. · Sonography and radiography are the most important modalities. · Both congenital and acquired pathologies play a role. · Good interdisciplinary cooperation is essential in this patient group. CITATION FORMAT: · Lochbühler N, Bölle P, Müller-Abt P. Acute abdomen in pediatric radiology. Fortschr Röntgenstr 2024; 196: 451 - 462.

Primary osteosarcoma of the ribs: A report from the Cooperative Osteosarcoma Study Group.

PURPOSE: Primary rib osteosarcoma has not been investigated extensively, and clinical characteristics and optimal therapeutic strategies have not been defined. The authors used the database of the Cooperative Osteosarcoma Study Group (COSS) to analyze this tumor-site in depth. METHODS: The COSS database was searched for treatment-naive, high-grade osteosarcomas of the rib. Affected patients were analyzed for demographic and tumor-related factors, treatments, and outcomes. RESULTS: A total of 44 patients (23 males, 21 females; median age, 23 years [range, 6-59]) were identified. Primary metastases were detected in six of 44 (14%) patients. Surgery was performed in 40 of 44 (91%) patients and rendered 35 of 44 (80%) patients macroscopically disease-free. Chemotherapy was known to have been administered in 43 of 44 (98%) patients and radiotherapy in seven of 42 (17%) (no data for two patients). A good response to chemotherapy was only noted in five (33%) of those 15 evaluable patients who had received any preoperative chemotherapy. After a median follow-up of 2.49 (0.22-40.35) years for all patients and 6.61 (0.25-40.35) years for 26 survivors (21 of these in first complete remission), 5-year actuarial overall and event-free survival were 53.0% (8.5%) and 42.2% (8.1%), respectively. Incomplete tumor surgery was the most notable negative prognostic factor. Osteoblastic histology and a poor response to chemotherapy may have contributed. CONCLUSION: This large series provides evidence that patients with costal primaries are older than the average osteosarcoma patient, but appear to share the similar tumor biology and-if treated according to standard protocols-prognostic factors with tumors of other sites. Early, preoperative diagnosis and permanent, definitive local control remain major challenges and should contribute to improved outcomes.

Cervical spine involvement in patients with juvenile idiopathic arthritis - MRI follow-up study.

BACKGROUND: To describe MRI and clinical findings in patients with juvenile idiopathic arthritis with cervical spine involvement at onset and follow-up under therapy. METHODS: 13 patients with signs of cervical spine involvement in juvenile idiopathic arthritis with a median disease duration of 1.7 years were included in the study. Clinical records and MR images were retrospectively analyzed according to symptoms and findings concerning the cervical spine. RESULTS: At the onset of cervical spine involvement all patients showed limited range of motion, whereas only 5 of them complained of pain. In MR images joint hyperintensity, contrast enhancement, malalignment, ankylosis, erosion and narrowing of the spinal canal at cranio-cervical junction were found at 28, 32, 15, 2, 2 and 3 sites in 12 (93%), 13 (100%), 8 (62%), 2 (15%), 2 and 3 (20%) patients respectively. 3 of the 5 patients with pain (60%) showed ankylosis, erosions or narrowing of the spinal canal at cranio-cervical junction on MRI. At follow-up - after a median disease duration of cervical spine arthritis of 2.1 years and a variable duration of treatment with methotrexate (all patients) and biological agents (12 patients) - joint hyperintensity, enhancement and malalignment decreased to 15, 19 and 6 sites in 10 (77%), 11 (85%) and 3 (20%) patients respectively whereas ankylosis, erosion and narrowing of the spinal canal at cranio-cervical junction increased to 7, 6 and 4 sites in 3 (20%), 4 (31%) and 4 patients respectively. Pain was no longer reported, but 9 of 13 (69%) patients still had a limited range of motion with 6 of them (46%) showing skeletal changes on MRI. CONCLUSIONS: This first MRI based follow-up study shows that cervical spine arthritis can follow a severe disease course in juvenile arthritis. While malalignments and inflammation sites decreased osseous changes with erosions, ankylosis, and narrowing of the spinal canal increased under treatment despite only minor subjective complaints. Therefore close MRI monitoring of these patients appears to be reasonable.

Reduced artefacts and improved assessment of hyperintense brain lesions with BLADE MR imaging in patients with neurofibromatosis type 1.

BACKGROUND: Assessment of small brain lesions in children is often compromised by pulsation, flow or movement artefacts. MRI with a rotating blade-like k-space covering (BLADE, PROPELLER) can compensate for these artefacts. OBJECTIVE: We compared T2-weighted FLAIR images that were acquired with different k-space trajectories (conventional Cartesian and BLADE) to evaluate the impact of BLADE technique on the delineation of small or low-contrast brain lesions. MATERIALS AND METHODS: The subject group comprised 26 children with neurofibromatosis type 1 (NF 1), who had been routinely scanned at 1.5 T for optic pathway gliomas with both techniques and who had the typical hyperintense brain lesions seen in NF 1. Four experienced radiologists retrospectively compared unlabelled 4-mm axial images with respect to the presence of artefacts, visibility of lesions, quality of contour and contrast. RESULTS: Both techniques were comparable in depicting hyperintense lesions as small as 2 mm independent of contrast and edge definition. Pulsation and movement artefacts were significantly less common with BLADE k-space trajectory. In 7 of 26 patients (27%), lesions and artefacts were rated as indistinguishable in conventional FLAIR, but not in BLADE FLAIR images. CONCLUSION: BLADE imaging significantly improved the depiction of lesions in T2-W FLAIR images due to artefact reduction especially in the posterior fossa.