Acute respiratory failure as initial manifestation of conventional osteosarcoma rich in giant cells: a case report

Background Osteosarcoma is a malignant tumor of the bone. The giant cell-rich osteosarcoma (GCRO) is a rare histological variant of the conventional osteosarcoma, accounting for 3% of all osteosarcomas. It has a variable clinical presentation, ranging from asymptomatic to multiple pathological fractures, mainly involving long bones, and less frequently the axial skeleton and soft tissues. Case presentation We present the case of a 25-year-old Hispanic woman, previously healthy, with a 1-month history of dyspnea on exertion, intermittent dry cough, hyporexia, and intermittent unquantified fever. She presented to the emergency department with a sudden increase in dyspnea during which she quickly entered ventilatory failure and cardiorespiratory arrest with pulseless electrical activity. Resuscitation maneuvers and orotracheal intubation were initiated, but effective ventilation was not achieved despite intubation and she was transferred to the intensive care unit of our institution. The chest radiograph showed a mediastinal mass that occluded and displaced the airway. The chest tomography showed a large mediastinal mass that involved the pleura and vertebral bodies. A thoracoscopic biopsy was performed that documented a conventional giant cell-rich osteosarcoma. The patient was considered to be inoperable due to the size and extent of the tumor and subsequently died. Conclusions The giant cell-rich osteosarcoma is a very rare histological variant of conventional osteosarcoma. Few cases of this type of osteosarcoma originating from the spine have been reported in the literature, and to our knowledge none of the reported cases included invasion to the chest cavity with airway compression and fatal acute respiratory failure that was present our case. Radiological and histological features of the GCRO must be taken into account to make a prompt diagnosis.

Lymphocyte-monocyte ratio as predictive factors for huvos scores in osteosarcoma extremities treated by neoadjuvan chemotherapy (cisplatin and doxorubicin)

Background: Chemotherapy is a vital treatment in osteosarcoma but it’s responses are difficult to predict, and there are still no biomarkers that can estimate the prognosis of chemotherapy. Lymphocytes and monocytes are key immune cells which are examined on complete blood count test before chemotherapy and often associated with prognosis of various malignancies. Low lymphocyte-monocyte ratio (LMR) are associated with poor prognostics in some cancers.Methods: This study is cross-sectional retrospective analytics that was conducted at Sanglah Hospital from June to August 2018. The research subject was medical records of intramedullary conventional osteosarcoma patients which fulfil inclusion and exclusion criteria. In this study, LMR as independent variable and Huvos score as dependent variable.Results: The result in this study showed positive correlation between LMR before neoadjuvan Chemotherapy and Huvos score (r=0.500) with p<0.05.Conclusions: A positive correlation was found between LMR and Huvos scores. Low LMR before neoadjuvan chemotheraphy (<2.81) were correlated with low huvos scores (grade I and II).

Cytokeratin-Positive Osteosarcoma Simulating Sarcomatoid Metastatic Carcinoma

Osteosarcoma, the most common primary malignant bone tumor, rarely stains positive for epithelial markers such as cytokeratin on immunohistochemical analysis. We describe a 52-year-old man with a destructive distal femoral metaphyseal lesion who was initially treated for metastatic sarcomatoid carcinoma based on extensive radiographic and histopathologic evaluation. Ultimately, wide resection of the distal femur was performed, revealing high-grade conventional osteosarcoma with intense and diffuse cytokeratin positivity. Such immunohistochemical staining in osteosarcoma is rare, making it difficult to distinguish cytokeratin-positive osteosarcoma from metastatic carcinoma. The presence of a cytokeratin-positive bone neoplasm with malignant osteoid formation on histological studies as well as integration with clinical and radiologic data can help confirm osteosarcoma as the ultimate diagnosis.

Tumour volume as a predictor of metastases in patients presenting with high-grade conventional osteosarcoma of the extremities

ABSTRACT BACKGROUND: The aim of this study was to compare the initial tumour volume in patients with and without pulmonary and/or skeletal metastases at time of presentation. The secondary aim was to compare the value of tumour volume in the prediction of metastases at time of presentation with known predictive factors, namely serum alkaline phosphatase (ALP) and lactate dehydrogenase (LDH). MATERIALS AND METHODS: A retrospective cross-sectional analysis was performed comparing the primary tumour volume in patients with and without metastases. All patients with histologically confirmed high-grade conventional osteosarcoma over a five-year period were included. RESULTS: The study comprised 61 patients. The mean age was 21 years (SD: 11.9, range 5-56) with an equal distribution of males and females (51% vs 49%). There was no correlation between tumour volume and age at presentation (p=0.31). There was no evidence of metastases in only 20% (n=12) of patients. Skeletal metastases were present in 28% (n=16) of the patients and pulmonary metastases were present in 44 cases (72%). There was no significant difference in the tumour volume at presentation between patients with and without pulmonary metastases (p=0.11). However, tumour volume did appear to predict the presence of skeletal metastases (p=0.02). A tumour volume of 1 383 cm3 had a negative predictive value (NPV) of 92% and positive predictive value (PPV) of 55% for the presence of skeletal metastases (area under curve [AUC]=0.76; sensitivity 66%; specificity 87%). A tumour volume of 480 cm3 had a 100% NPV for the presence of skeletal metastases (AUC=0.74). A tumour volume > 1 380 cm3 had an odds ratio (OR) of 13.6 (p<0.01; 95% CI 2.6-72.5) as an independent variable in relation to skeletal metastases. Multivariate analysis (with ALP and LDH) of tumour volume >1 380 cm3 yielded an OR of 8.6 (p=0.04; 95% CI 1.1-67) for presence of skeletal metastases. CONCLUSION: In this series of conventional high-grade osteosarcoma of the extremities, we found a very high rate of metastases at time of diagnosis. While there was no association with pulmonary metastases, increased tumour volume was associated with an increased risk for the presence of skeletal metastases. More studies in the developing world clinical setting are required to investigate this further; the high rate of metastases seen at time of diagnosis also requires further investigation Level of evidence: Level 4. Keywords: osteosarcoma, metastases, tumour volume, prognosis, staging

Local dissemination of osteosarcoma observed after massage therapy: a case report

Background Limited evidence is available regarding the dissemination of tumor tissues due to compression during massage therapy, a routine procedure in patients with various symptoms in Asian countries. Case presentation A 12-year-old male presented at a massage clinic with pain and swelling of his left knee, which worsened the same night. Consistent with conventional osteosarcoma, radiography revealed cortical bone destruction, osteoblastic changes, and periosteal reactions. Magnetic resonance imaging revealed a tumor in the distal femur, an extraskeletal mass, and an infiltrative lesion in the intramuscular and neurovascular areas surrounding the distal femur; this was considered as hemorrhage and dissemination of the tumor tissue. 18Fluorine-labelled fluorodeoxyglucose-positron emission tomography and computed tomography revealed multiple metastases in the spine, liver, and lung. Consistent with osteosarcoma, histopathological examination revealed tumor cell proliferation with extensive pleomorphism and mitoses. Despite undergoing chemotherapy, radiation therapy, and hip disarticulation, the patient died due to multiple metastases 13 months after the initial diagnosis. Conclusions The present case suggests association of massage therapy with the local dissemination of tumor tissues, although influence of massage therapy on metastatic lesions remains unclear. Massage therapists should be aware of the possibility for dissemination of hidden malignancies due to the procedure.

Improved osteosarcoma survival with addition of mifamurtide to conventional chemotherapy – a prospective single institution analysis.

Background Conventional osteosarcoma is rare disease. Current treatment approaches have been set decades ago and include combination of three drug chemotherapy schedule and surgery. Three and five years event free survival in localized disease is roughly 59-54%, respectively. An immunotherapy agent mifamurtide was introduced to clinical practice after registration study which reported survival benefit, but some methodological controversies have been unsufficient for Food and Drug Administration market authorization Methods We report here prospective single center analysis of mifamurtide addition to conventional therapy in 16 patients during 5.5 year enrollment period. Results Adverse event profile was as expected with no dose limiting toxicities. All patients were given mifamurtide according to planned treatment schedule. There were no local relapses observed, one patient died in first complete remission due to doxorubicine cardiotoxicity, one patient had pulmonary metastatic relapse. Observed three and five years event free survival was 82,6 (CI 70-100%) and 82,6% (CI 70-100%), progression free survival was 92,3% (CI 78,9-100%) and 92,3% (CI 78,9-100%), overall survival was 93,3 (CI 81,5-100) and 74,7 (CI 47,2-100), respectively. Conclusions Survival benefit of mifamurtide is reported herein. Addition of mifamurtide should be considered as best treatment option for patients with localized osteosarcoma.