Bone Sarcoma Of The Pelvis: A Multicentric Retrospective Study Of One Hundred-Fourteen Cases

Background: The aim of this study was to assess the oncologic outcome of pelvic bone sarcomas (PBS) and to identify prognosis factors.Methods: We report a multicentric cohort of patients treated for a PBS from 2000 to 2020. Data from 12 hospitals were analysed. Patients treated for primary PBS were included. Alive patients with less than 6 months of follow up were excluded. The primary outcome was survival.Results: One hundred and fourteen patients (67 males and 48 females) were reviewed with a mean follow up of 32±46,5 (1 to 216) months. The mean patient and doctor diagnosis delays were respectively 8,5±10,2 (1 to 60) and 3±4,3 (0 to 24) months. Sixty-eight patients (59,6%) died after a mean time from diagnosis of 15,9±22,8 (1 to 120) months. The overall survival rates at 5 and 10 years were respectively 38,4% and 27,6%. Chondrosarcoma histological type (HR=3,64), metastasis (HR=3,55) and surgery (HR=0,12) were identified as significant survival factors. Surgery was also associated to a decreased risk of metastasis (OR=0,03, 95% CI: 0,01 – 0,1). Among the 76 patients (66,7%) who underwent surgery, local recurrence was observed in 19 patients (25%) with a mean time from surgery to onset of 11,05 (±17,5) months. Conclusions: This nation-wide20-year-cohort study shows that surgery is the most effective treatment option in PBS regardless the histological type of the tumour. Efforts have to be done to decrease the diagnosis delay in order to start treatment when surgery is still feasible.

Recurrent Bilateral Lower Motor Neuron Type of Facial Palsy with Hearing Impairment: Hyperphosphatemic Familial Tumoral Calcinosis

Hyperphosphatemic familial tumoral calcinosis (HFTC) presents with varied neurological manifestations that have been reported in the literature like facial palsy, vision and hearing impairment, stroke, and headache. In this article, we reported a 12-year-old girl child patient with recurrent facial weakness with bilateral hearing impairment and multiple ulcerative lesions on lower limbs and elbows. On examination, she had lower motor neuron (LMN) facial palsy with conductive hearing loss. The investigations showed hyperphosphatemia (9.3 mg/dL) with normal serum calcium (10.4 mg/dL), alkaline phosphatase (147.9 U/L), parathyroid hormone (23.12 pg/mL), and renal function tests. Elevated serum calcium and phosphorus product (96.72 mg2/mL2) and elevated renal tubular reabsorption of phosphate (TMPxGFR) value (9.16) were noted. Skeletal survey showed hyperostosis in the long bone diaphysis, vertebrae, ribs, pelvic bone, skull, and facial bones with narrowing of cranial ostium, characteristically without any peri-articular soft tissue calcifications. An angiogram showed multiple intravascular calcifications. She was managed with a low-phosphate diet, sevelamer, niacinamide, acetazolamide, sucroferric oxyhydroxide to lower serum phosphate level, and topical sodium thiosulfate ectopic cutaneous calcification. Exome sequencing showed novel homozygous inframe deletion of ACG in FGF23 gene exon 3 at c.374_376 delins position (p. Asp125del) in the proband and a mutation in the heterozygous state in the mother and elder sibling, thus confirming a molecular diagnosis of HFTC. Our case had a unique neurological presentation of recurrent bilateral lower motor nerve facial palsy, hearing loss, multiple ectopic cutaneous calcifications without peri-articular deposits, multiple intravascular, intracranial, and vertebral endplate calcification, which has not been reported earlier. The proband showed a novel pathogenic variant suggesting an expanding phenotype of HFTC.

Correlation Between Changes of Pelvic Bone Marrow Fat Content and Hematological Toxicity in Concurrent Chemoradiotherapy for Cervical Cancer

Objectives: To quantify the pelvic bone marrow (PBM) fat content changes receiving different radiation doses of concurrent chemoradiotherapy for cervical cancer and to determine association with peripheral blood cell counts. Methods: Fifty-four patients were prospectively collected. Patients underwent MRI iterative decomposition of water and fat with echo asymmetrical and least squares estimation (IDEAL IQ) scanning at RT-Pre, RT mid-point, RT end, and six months. The changes in proton density fat fraction (PDFF%) at 5–10 Gy, 10–15 Gy, 15–20 Gy, 20–30 Gy, 30–40 Gy, 40–50 Gy, and >50 Gy doses were analyzed. Spearman’s rank correlations were performed between peripheral blood cell counts versus the differences in PDFF% at different dose gradients before and after treatment. Results: The lymphocytes (ALC) nadirs appeared at the midpoint of radiotherapy, which was only 27.6% of RT-Pre; the white blood cells (WBC), neutrophils (ANC), and platelets (PLT) nadirs appeared at the end of radiotherapy which was 52.4%, 65.1%, and 69.3% of RT-Pre, respectively. At RT mid-point and RT-end, PDFF% increased by 46.8% and 58.5%, respectively. Six months after radiotherapy, PDFF% decreased by 4.71% under 5–30 Gy compared to RT-end; while it still increased by 55.95% compared to RT-Pre. There was a significant positive correlation between PDFF% and ANC nadirs at 5–10 Gy (r = 0.62, P = 0.006), and correlation was observed between PDFF% and ALC nadirs at 5–10 Gy (r = 0.554, P = 0.017). Conclusion: MRI IDEAL IQ imaging was a non-invasive approach to evaluate and track the changes of PBM fat content with concurrent chemoradiotherapy for cervical cancer. The limitation of low-dose bone marrow irradiation volume in cervical cancer concurrent chemoradiotherapy should be paid more attention.

Evaluation of the impact of teaching on delineation variation during a virtual stereotactic ablative radiotherapy contouring workshop

Introduction: Variation in delineation of target volumes/organs at risk (OARs) is well recognised in radiotherapy and may be reduced by several methods including teaching. We evaluated the impact of teaching on contouring variation for thoracic/pelvic stereotactic ablative radiotherapy (SABR) during a virtual contouring workshop. Materials and methods: Target volume/OAR contours produced by workshop participants for three cases were evaluated against reference contours using DICE similarity coefficient (DSC) and line domain error (LDE) metrics. Pre- and post-workshop DSC results were compared using Wilcoxon signed ranks test to determine the impact of teaching during the workshop. Results: Of 50 workshop participants, paired pre- and post-workshop contours were available for 21 (42%), 20 (40%) and 22 (44%) participants for primary lung cancer, pelvic bone metastasis and pelvic node metastasis cases, respectively. Statistically significant improvements post-workshop in median DSC and LDE results were observed for 6 (50%) and 7 (58%) of 12 structures, respectively, although the magnitude of DSC/LDE improvement was modest in most cases. An increase in median DSC post-workshop ≥0·05 was only observed for GTVbone, IGTVlung and SacralPlex, and reduction in median LDE > 1 mm was only observed for GTVbone, CTVbone and SacralPlex. Post-workshop, median DSC values were >0·7 for 75% of structures. For 92% of the structures, post-workshop contours were considered to be acceptable or within acceptable variation following review by the workshop faculty. Conclusions: This study has demonstrated that virtual SABR contouring training is feasible and was associated with some improvements in contouring variation for multiple target volumes/OARs.

A Case Report of Osteopoikilosis in the ribs, pelvic region and spine

Osteopoikilosisis a rare inherited benign bone dysplasia incidentally found on radiological exams. It ischaracterized by a specific radiological pattern which consists in diffuse, round or oval, symmetrically shaped sclerotic bone areas distributed throughout the skeleton. It is important to do a correct diagnosis because these lesions could be easily confused with bone metastasis. We reported a case of an osteopoikilosis patient presenting to our clinic with transient loss of consciousness and without any numbness, tingling and weakness in the legs or other parts of the body. The CT scan showed multiple small sclerotic foci bone islands, scattered throughout the thoracic and lumbar spine, ribs, pelvic bone, sacrum and bilateral proximal femur. No significant increase in the activity was detected in Technetium-99m (Tc-99m) whole body bone scintigraphy. The patient was diagnosed with characteristic radiological findings of osteopoikilosis and was followed up.