Multiple Bone Involvement Research Articles

Multiple Bone Involvement in Low-grade Myofibroblastic Sarcoma Demonstrated on 18F-FDG PET/CT.

A 68-year-old woman with low back pain for 2 months was admitted. T2-weighted fat-saturated imaging revealed hyperintense lesions in multiple lumbar regions, suggesting the possibility of bone metastases. Multiple osteolytic and mixed osteolytic-osteoblastic lesions with significant 18F-fluorodeoxyglucose (18F-FDG) uptake, as well as multiple osteoblastic lesions with mild 18F-FDG uptake, were observed on subsequent 18F-FDG positron emission tomography/computed tomography without an identifiable primary lesion. This patient was pathologically diagnosed with low-grade myofibroblastic sarcoma (LGMS) after biopsy and surgery. Although multiple bone involvement in LGMS is extremely rare, this case suggests that it should be considered in the differential diagnosis of multiple bone metastases.

Stratified Treatment in Pediatric Anaplastic Large Cell Lymphoma: Result of a Prospective Open-Label Multiple-Institution Study.

The risk stratification of pediatric anaplastic large cell lymphoma (ALCL) has not been standardized. In this study, new risk factors were included to establish a new risk stratification system for ALCL, and its feasibility in clinical practice was explored. On the basis of the non-Hodgkin's lymphoma Berlin-Frankfurt-Munster 95 (NHL-BFM-95) protocol, patients with minimal disseminated disease (MDD), high-risk tumor site (multiple bone, skin, liver, and lung involvement), and small cell/lymphohistiocytic (SC/LH) pathological subtype were enrolled in risk stratification. Patients were treated with a modified NHL-BFM-95 protocol combined with an anaplastic lymphoma kinase inhibitor or vinblastine (VBL). A total of 136 patients were enrolled in this study. The median age was 8.8 years. The 3-year event-free survival (EFS) and overall survival of the entire cohort were 77.7% (95% confidence interval [CI], 69.0% to 83.9%) and 92.3% (95% CI, 86.1% to 95.8%), respectively. The 3-year EFS rates of low-risk group (R1), intermediate-risk group (R2), and high-risk group (R3) patients were 100%, 89.5% (95% CI, 76.5% to 95.5%), and 67.9% (95% CI, 55.4% to 77.6%), respectively. The prognosis of patients with MDD (+), stage IV cancer, SC/LH lymphoma, and high-risk sites was poor, and the 3-year EFS rates were 45.3% (95% CI, 68.6% to 19.0%), 65.7% (95% CI, 47.6% to 78.9%), 55.7% (95% CI, 26.2% to 77.5%), and 70.7% (95% CI, 48.6% to 84.6%), respectively. At the end of follow-up, one of the five patients who received maintenance therapy with VBL relapsed, and seven patients receiving anaplastic lymphoma kinase inhibitor maintenance therapy did not experience relapse. This study has confirmed the poor prognostic of MDD (+), high-risk site and SC/LH, but patients with SC/LH lymphoma and MDD (+) at diagnosis still need to receive better treatment (ClinicalTrials.gov number, NCT03971305).

Elevated skull fractures: an institutional experience and individual participant data meta-analysis.

Elevated skull fracture (ESF) is a rare but potentially life-threatening type of skull fracture. The literature on this topic is relatively sparse. Herein, we conducted a meta-analysis of all the patients reported in the literature with ESFs with respect to their clinical management to better inform practice. On 20th of January 2023, we conducted a systematic search of literature to find all published cases of ESF. We also conducted a retrospective review of ESF cases from our institution. The data collection and analysis were conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. After screening, 28 studies met the inclusion criteria. A total of 104 individual patients were included in the meta-analysis, with a median age of 24 years and 85.7% of whom were males. 11 patients (11.2%) had an unfavorable outcome while 37 (35.2%) had one or more complications. We found that GCS on admission is an independent predictor of poor outcome in ESF (odds ratio (95% confidence interval) = 1.605 (1.110-2.315), p value = 0.012). Regarding complications, dural injury (odds ratio (95% confidence interval) = 66.667 (7.407-500.00), p value < 0.001) and multiple bone involvement (odds ratio (95% confidence interval) = 6.849 (2.127-22.222), p value = 0.001) were independent predictors of complication. ESFs represent a rare yet consequential form of cranial injury, carrying potentially life-threatening implications if not promptly addressed. In this study, we present the meta-analysis of outcomes and complications within this patient cohort, offering a comprehensive synthesis of existing literature on this pathology. However, further investigation is imperative to provide higher-quality evidence and address lingering uncertainties in the classification and management of ESFs.

Letterer–Siwe disease: a case of multisystem Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is an idiopathic uncommon haematological condition affecting infants and young children but can occur at any age group characterized by clonal proliferation of abnormal Langerhans cells. Langerhans cells express an immunophenotype positive for S100 protein, CD1a and Langerin (CD207). Here we present a case of 2-year-old male patient presented with a multiple raw area associated with oozing, bleeding and crusted lesions were present over scalp, trunk and post-auricular areas. Radiographic examination revealed multiple osteolytic bone involvement. Histopathological examination revealed Langerhans cell histiocytosis. Immunophenotyping was positive for vimentin, CD1a, and S100. Based on radiological, histological and immunophenotyping findings suggestive of multisystem LCH. The purpose of this report is to describe a rare case of LCH in the 2-year-old male child with multisystem LCH involving skin, soft tissue and bones and to discuss clinical, radiological and histopathological features of LCH.

Multiple solitary plasmocytoma with multifocal medullary asymptomatic presentation for diabetic patient (Case Report)

Multiple solitary plasmocytoma with multiple bone involvement, it’s very rare cases, in the literature, special skull vault associated with other medullary involvement, In our case is the first case report for multiple skull vault, with multiple focal medullary, asymptomatic for diabetic patient, also highlighted the accurate diagnostic of plasmocytoma and management.

A rare case report of multiple bone involvement with avascular necrosis due to micro thrombi in a known case of COVID-19 can it be post sequale?

A rare case report of multiple bone involvement with avascular necrosis due to micro thrombi in a known case of COVID-19 can it be post sequale?

Paradigm shift in vertebral eosinophilic granuloma

Objective: Eosinophilic granuloma is the most common type of Langerhans cell histiocytosis. Vertebral bone involvement of eosinophilic granuloma is a rare disease. There are few studies in the literature on patients with vertebral bone involvement diagnosed with eosinophilic granuloma. There is still no clear protocol for the treatment of patients with vertebral involvement with eosinophilic granuloma. This article is a summary of the clinical follow-ups of our 6 cases diagnosed with spinal eosinophilic granuloma. We changed the chemotherapy protocol or extended the duration of chemotherapy in patients with progressive disease.Methods: In this study, there are six patients (4 male and 2 female) with vertebral involvement of eosinophilic granuloma in our hospital between September 2017 and August 2022. The clinical findings, radiological images, treatments and follow-up results of the patients were analyzed retrospectively.Results: In the follow-ups, a new vertebral bone involvement was observed in two patients and a femoral bone involvement was observed in another patient. One patient with neurological deficit underwent surgical treatment. Chemotherapy treatment was given to four patients with multiple bone involvement or recurrence. No recurrence was observed in three patients who started chemotherapy treatment after recurrence.Conclusions: Surgical treatment should be considered in patients with neurological deficits and spinal instability. According to the results of our study, patients with multiple bone or systemic involvement and no neurological deficit should receive chemotherapy treatment. We recommend changing the chemotherapy protocol or extending the chemotherapy duration in patients with progressive disease.

Age-related skull fracture patterns in infants after low-height falls.

Prior research and experience has increased physician understanding of infant skull fracture prediction. However, patterns related to fracture length, nonlinearity, and features of complexity remain poorly understood, and differences across infant age groups have not been previously explored. To determine how infant and low-height fall characteristics influence fracture patterns, we collected data from 231 head CT 3D reconstructions and quantified length and nonlinearity using a custom image processing code. Regression analysis was used to determine the effects of age and fall characteristics on nonlinearity, length, and features of fracture complexity. While impact surface had an important role in the number of cracks present in a fracture, younger infants and greater fall heights significantly affected most features of fracture complexity, including suture-to-suture spanning and biparietal involvement. In addition, increasing fracture length with increasing fall height supports trends identified by prior finite-element modeling. Finally, this study yielded results supporting the presence of soft tissue swelling as a function of fracture location rather than impact site. Age-related properties of the infant skull confer unique fracture patterns following head impact. Further characterization of these properties, particularly in infants <4 months of age, will improve our understanding of the infant skull's response to trauma. Younger infant age and greater fall heights have significant effects on many features of fracture complexity resulting from low-height falls. Incorporating multiple crack formation and multiple bone involvement into computational models of young infant skull fractures may result in increased biofidelity. Drivers of skull fracture complexity are not well understood, and skull fracture patterns in real-world data across infant age groups have not been previously described. Understanding fracture complexity relative to age in accidental falls will improve the understanding of accidental and abusive head trauma.

Pediatric Musculoskeletal Coccidioidomycosis in Central California: A Single Center Experience.

Published literature on musculoskeletal coccidioidomycosis is sparse and limited to case reports and case series. This is one of the largest case series to describe the clinical presentation, diagnosis, medical and surgical management and outcomes of pediatric musculoskeletal coccidioidomycosis at a tertiary care hospital. A retrospective case review was performed on patients ≤ 21 years old who were followed at a tertiary care center with a diagnosis of musculoskeletal coccidioidomycosis from January 1, 2007, to December 31, 2020. Descriptive data are expressed as medians and interquartile range (IQR) for continuous variables or as frequency and percentage for categorical variables. Categorical values were compared using the χ2 test. Forty-one patients were identified. The median age was 12.8 years, and most were male (71%), Latinx (66%) and healthy (71%). Limb swelling (66%), bone pain (54%) and joint pain (46%) were the most common presenting symptoms. Multiple bone involvement was present in 29% while 12% had the joint disease, and craniofacial (n = 10) and metacarpal/metatarsal bones (n=9) were the most commonly involved sites. Elevated Coccidioides complement fixation (CF) titers ≥1:32 were seen in 90% of the patients. Thirty-three patients (81%) required surgical interventions and of these 16 (48%) required additional surgical procedures. Eleven patients (27%) had disease relapse. Children >13 years of age were more likely to have > 1 organ involvement (16 vs. 7, P = 0.04), multiple bone involvement (10 vs. 2, P = 0.004) and maximum Coccidioides CF titers >1:128 (13 vs. 6, P = 0.02). In endemic areas, musculoskeletal coccidioidomycosis causes a substantial disease burden in children and should be considered in the differential diagnosis of those presenting with bone and joint pain or swelling. Early diagnosis and treatment are essential to minimize long-term morbidity and mortality.

Pathological humerus fracture due to anti-interferon-gamma autoantibodies: A case report

BACKGROUNDVarious etiologies contribute to pathological fractures, including bone infections. Recently, non-tuberculosis Mycobacterium-related bone infections among patients with anti-interferon-gamma autoantibody-induced adult-onset immunodeficiency has raised concerns in Southeast Asia, with the common presentations including osteomyelitis. However, it also rarely manifests as traumatic fractures, as reported in this case.CASE SUMMARYA diabetic female fractured her humerus after a traumatic accident and received fixation surgery. Abnormal necrotic bone tissue and abscess formation were noted, and she was diagnosed with a pathological fracture due to non-tuberculosis Mycobacterium infection. Multiple bone involvement was also revealed in a bone scan. Anti-interferon-gamma autoantibodies were then checked due to an unexplained immunocompromised status and found to be positive. Her humerus fracture and multiple bone infections healed after steroid and anti-non-tuberculosis Mycobacterium medication treatment following fixation surgery.CONCLUSIONComprehensive preoperative evaluations may help identify pathological fractures and guide the treatment course.

Clinical study of MAP2K1-mutated Langerhans cell histiocytosis in children.

To analyze the genetic and clinical features of children with MAP2K1-mutated Langerhans cell histiocytosis (LCH). We compared the clinical features of 37 children with MAP2K1-mutated LCH with those of the BRAFV600E mutation group (n = 133) and no known mutation group (n = 59) in the same period. We found 13 mutations of the MAP2K1 gene, which were mainly concentrated at p.53-62 and p.98-103. The most common mutation site was c.172_186del (12/37). Compared with the BRAFV600E mutation group, the patients with MAP2K1 mutations were mainly characterized by single-system multiple bone involvement (P = 0.022), with later disease onset (P = 0.029) as well as less involvement of risk organs, especially liver (P = 0.024). There was no significant difference in clinical features compared with the no known mutation group. The 2-year progression-free survival rate of first-line treatment (ChiCTR1900025783, 07/09/2019) in MAP2K1-mutated patients was 65.6% ± 9.5%. The prognosis of patients with lung involvement was poor [HR (95% CI) = 6.312 (1.769-22.526), P = 0.005]. More progression or relapses could be found in patients with bony thorax involvement (8/17 vs. 2/20, P = 0.023), yet involvements in other sites of bones, such as craniofacial bone involvement (8/26 vs. 2/11, P = 0.688) and limb bone involvement (5/12 vs. 5/25, P = 0.240), were not correlated to disease progression or relapse. The children with MAP2K1-mutated LCH have specific clinical features requiring clinical stratification and precise treatment. MAP2K1-mutated patients with lung involvement (especially with bony thorax involvement) had poor prognosis.

Primary sacral lymphoma with multiple bone involvement evaluated using 18F-FDG PET/CT.

Primary sacral lymphoma with multiple bone involvement evaluated using 18F-FDG PET/CT.

Bone pain at leukemia diagnosis and other risk factors for symptomatic osteonecrosis in children with acute lymphoblastic leukemia.

Osteonecrosis is a major cause of acute and long-lasting complications of acute lymphoblastic leukemia (ALL) therapy in children. Our study aimed to evaluate the prevalence, characteristics, risk factors, and outcome of osteonecrosis in children with ALL. The cohort included 559 children aged 1-20years diagnosed with ALL between 2003 and 2018 at two tertiary medical centers in Israel and enrolled in two consecutive protocols: ALL-IC BFM 2002 and AIEOP-BFM ALL 2009. Symptomatic osteonecrosis was prospectively captured as an adverse event. Osteonecrosis occurred in 51 patients (9.1%). Ninety-four percent of the events were graded as moderate or severe (grades 3-4, Ponte di Legno Toxicity Working Group classification) and multiple bone involvement was common. Full resolution of osteonecrosis was documented in only 16% of the children (median follow-up 4.2years). Stepwise logistic regression identified five risk factors for osteonecrosis, with a high predictive value (AUC=0.88): older ageat ALL diagnosis, high-risk ALL group, T-cell immunophenotype, female gender, and a novel risk factor: bone pain at the time of leukemia diagnosis. In addition, osteonecrosis was less common among children of Arab ethnicity. Thrombophilia and an elevated age-adjusted body mass index were not confirmed as risk factors for osteonecrosis. Due to the low rates of osteonecrosis resolution and its debilitating long-term impact, the identification of patients at high risk for osteonecrosis is important for their inclusion in further studies evaluating potential therapeutic adjustments.

Surgical management of proximal femoral metastasis: Fixation or hip replacement? A 309 case series

IntroductionThe proximal femur is the most frequent operative site for metastasis, but there is no consensus between internal fixation and hip replacement. The present multicenter retrospective observational study sought: (1) to compare early clinical results between internal fixation and hip replacement for proximal femoral metastasis (PFM), and (2) to assess events affecting survival. HypothesisThe study hypothesis was that internal fixation and hip replacement give comparable clinical results, operative site complications rates and survival. Material and methodsThe series comprised 309 cases, 10 of which were bilateral, in 182 females and 117 males, with a mean age of 67.2±11.5 years and 62.5±13.2 years, respectively. Primaries were mainly breast (118; 38.2%), lung (85; 25.5%) or kidney (40; 12.9%). PFM was revelatory in 114 cases (36.9%). There was visceral involvement in 142 patients (46%), multiple peripheral bone involvement in 212 (68.6%), and spinal involvement in 134 (43.4%). There were 124 pathologic fractures (40%), 51 of which were revelatory. Metastases were cervicocephalic in 135 cases (43.7%), metaphyseal in 166 (53.7%) and both in 8 (2.6%). PFM was osteolytic in 90% of cases, managed by hip replacement in 161 cases and internal fixation in 148 (12 screwed plates, 136 nails). Seventy-seven patients had postoperative radiation therapy. ResultsAfter hip replacement (n=144), walking was normal in 35 cases (24.3%), impaired but unassisted in 53 (36.8%), with 1 forearm crutch in 24 (16.6%), 2 crutches or a frame in 26 (18%), and impossible in 6 (4.1%). After nailing (n=125), results were respectively 38 (30.4%), 47 (37.6%), 15 (12%), 18 (14.4) and 7 (5.6%). Recovery of normal walking capacity did not significantly differ according to technique (p=0.162); nor did pain or function. Recovery of normal walking capacity was better after preventive surgery (p<0.001). Perioperative complications comprised: 10 cases of severe blood loss, 7 pulmonary embolisms, 6 digestive hemorrhages, and 5 lung infections. Secondarily, there were 11 infections (7 after hip replacement, 2 after nailing, 2 after plate fixation), 7 progressive osteolyses and 5 fractures. Complications rates were significantly higher with plate fixation, with no difference between nailing and hip replacement. Survival did not significantly differ between hip replacement (12 months [95% CI: 7–19]), nailing (7 months [95% CI: 6–11]) and plate fixation (16 months [95% CI: 6-not calculable]). ConclusionsClinical results and survival were comparable between hip replacement and nailing, confirming the study hypothesis, in agreement with the literature. Each technique has its indications. Patients with severely impaired walking capacity benefited greatly from surgery. The importance of preventive surgery was highlighted. Level of evidenceIV, retrospective study.

Pleural effusion caused by polyostotic fibrous dysplasia involving the ribs, the sternum and the thoracic spine associated with multiple cystic degenerations – a case report

Introduction. Fibrous dysplasia is a noninherited benign skeletal disorder associated with abnormal bone development. Single bone involvement, the monostotic form, accounts for 70 ? 80% of cases, while the polyostotic form, with multiple bone involvement, accounts for 20 ? 30% of cases. Cystic degeneration and occasional aneurysmal bone cyst formation may be found in fibrous dysplasia lesions, particularly in the costal lesions. Case Report. A 51-year-old man presented with acute shortness of breath after sustaining simple chest wall injury. Chest computed tomography showed multiple massive osteolytic rib lesions, as well as a massive left-sided pleural effusion with compression atelectasis of the lung parenchyma. Osteolytic lesions of the anterior 2nd and 7th thoracic vertebral body were found, along with a well defined osteolytic lesion in the body of the sternum. Video-assisted thoracoscopy of the left pleural space was performed and frozen sections, collected using endoscopic biopsy forceps of the cystic wall and solid parts of the tumors, were sent for ex tempore histopathological analysis. Results showed fibrous dysplasia with suspected malignancy. Talc pleurodesis was performed based on the obtained results. At present, the patient is asymptomatic with his daily routine uninterrupted by his medical condition. Conclusion. Treatment of pleural effusion caused by a cyst rupture of unresectable degenerated polyostotic fibrous dysplasia of the ribs represents a surgical challenge. Surgical drainage of the cysts followed by chemical pleurodesis seems to be a reasonable solution in cases where pulmonary functions are impacted by combined effects of pleural effusion and cystic compression.

Mandibular and cheek swelling revealing the diagnosis of localized amyloidosis

Mandibular and cheek swelling revealing the diagnosis of localized amyloidosis

Polyosteotic Hydatidosis with Pathological Fracture: A Case Report

Introduction: A Hydatid disease of the bone is a rare presentation of hydatid disease since it accounts for 4% of the infection rate, which mostly involve the lungs and liver, the human being is infected with contaminated food that contain eggs and it represent the intermediate stage of the disease transmission. Case presentation: A 55 years old male who was a known case of hydatid disease of the tibia and distal femur presented with gradual onset of right hip pain which made him bed ridden, serological test proved the infection, MRI showed heavy infection of the proximal femur. Discussion: Bone involvement is very slow process owing to the compact nature, the parasites replaces the hard tissue of the bone and eventually breaches the outer cortex and spread to the soft tissue around the bone, multiple bone involvement is rare, since there are few reports of coincedence existence of pelvis and proximal femur. Skeletal hydatid disease is notorious for its high recurrence rate especially when it involve the coxofemoral and pelvic region, so it is wise to offer radical treatment from the start in a way similar to bone tumors with adjuvant chemotherapy and radiotherapy, however extensive surgeries in this region carries a high risk of complications. Conclusion: Multiple bony hydatid cyst involvement with pathological fracture is a rare presentation in the lower limb, the importance of early diagnosis is paramount to avoid major complication especially in the coxal region of the femur, skeletal survey is an important step to avoid missing skip lesions.

Radiological Imaging Findings of Craniofacial Fibrous Dysplasia.

To present the radiological findings of fibrous dysplasia (FD) patients who had computed tomography (CT) and/or magnetic resonance imaging (MRI) scans. This study included a total of 25 patients (17 female and 8 male) who were found to have FD based on CT examinations between March 2010 and July 2018. Involved bones, type of involvement (single or multiple) and CT appearance features (ground-glass, sclerotic, cystic or mixed type) were evaluated. Age range of the patients with FD was 14-55 (mean: 29.92 ± 12.63) years. Sixteen patients had single bone and nine patients had multiple bones affected. Single bone most frequently involved maxillary bones. Multiple bone involvement affected up to four bones, sphenoid bone being the most frequent. Fifteen lesions were mixed type, eight were ground-glass and two were sclerotic on CT. Intense contrast-enhancement was observed in four patients on MRI. Craniofacial FD is more frequent in young adults and women, and more commonly involves single bone, mostly maxillary and sphenoid bones. Craniofacial FDs could be of different types on radiological examinations depending upon their compositions.

JCES 01.20 Primary Tumor Resection versus Maintenance Therapy for Patients with Oligometastatic Non-Small Cell Lung Cancer

To evaluate (1) the potential effect of primary tumor resection, an aggressive local consolidative therapy, for patients with oligometastatic NSCLC on 3 year overall survival; (2) the surgical outcomes in the treatment of patients with oligometastatic NSCLC; (3) the potential clinical factors predicting survival in order to better select patients for surgery. According to the extent of pulmonary resection, the patients were divided into two subgroups. A. intent to cure (ITC: removal of total or primary pulmonary lesions); B. intent to biopsy (ITB: preservation of major lesions, only diagnostic biopsy via minimally invasive approach). M stage classified based on 8th UICC/AJCC TNM M categories. From Jan 2002 through Dec 2015, a total of 115 consecutive metastatic NSCLC patients were enrolled from Peking University Cancer Hospital. The 3-year overall survival (OS) of ITC and ITB were 64.3% and 34.9% (log-rank p = 0.0009), respectively. Multivariate cox proportional regression analysis identified multiple station lymph nodes (LN) and bone involvement may be prognostic indicators. The current findings suggest that aggressive surgical therapy can extend the survival in selected stage IV NSCLC patients, and should be further explored in phase 3 trials as a standard treatment option in this clinical scenario.

Bone Metastases in Follicular Carcinoma of Thyroid.

Though the association between follicular carcinoma and bone metastasis is well established, the site-wise distribution is not known. One hundred seventy-three patients of follicular carcinoma presenting between 2003 and 2011 were selected from 1093 patients of follicular lesions presenting at a single institution. Of these, 59 (34%) with bone metastasis were included in the study. Fifty of the 59 patients (84.7%) had metastasis at presentation, while 9 developed bone metastasis during follow-up. Sixty-one percent had solitary metastasis, 15 (25.4%) had multiple bone involvement, while 8 patients (13.6%) had synchronous lung metastasis. Overall, the spine was the commonest site of bone metastases, seen in 20 patients (33.9%), followed by the pelvis, skull, long bones and sternum. Bone metastasis is a known phenomenon in follicular carcinoma. The spine is the commonest site followed by the pelvis.