scholarly journals Diagnosis and staging of malignant bone tumours in children: what is due and what is new?

2021 ◽  
Vol 15 (4) ◽  
pp. 312-321 ◽  
Author(s):  
Marta Salom ◽  
Catharina Chiari ◽  
Jean Maria Gómez Alessandri ◽  
Madeleine Willegger ◽  
Reinhard Windhager ◽  
...  

Purpose Although malignant bone tumours in children are infrequent, it is important to know how to properly diagnose and stage them, in order to establish an adequate treatment. Methods We present a review of the diagnostic workflow of malignant bone tumours in children, including history and clinical examination, imaging, laboratory tests and biopsy techniques. Moreover, the two most commonly used staging systems are reviewed. Results History, clinical examination and laboratory tests are nonspecific for diagnosing malignant bone tumours in children. Radiographs remain the mainstay for initial diagnosis, with MRI the modality of choice for local assessment and staging. Fluorine-18 labelled fluoro-deoxy-glucose-positron emission tomography scans provide a noninvasive method to assess the aggressiveness of the tumour and to rule out metastasis and is replacing the use of the bone scintigraphy. Biopsy must be always performed under the direction of the surgeon who is to perform the surgical treatment and after all diagnostic evaluation has been done. Staging systems are useful to study the extent of the tumour and its prognosis. They are expected to evolve as we better understand new molecular and genetic findings. Conclusion When a malignant bone tumour is suspected in a child, it is essential to make a correct diagnosis and referral to an experienced centre. Following an appropriate workflow for diagnosis and staging facilitates, prompt access to treatment improves outcomes. Level of Evidence Level V Expert opinion

2017 ◽  
Vol 99 (7) ◽  
pp. 568-572 ◽  
Author(s):  
P Yang ◽  
S Evans ◽  
N Bali ◽  
A Ramasamy ◽  
R Evans ◽  
...  

Introduction Malignant osseous foot tumours are uncommon. Their oncological outcomes have been poorly documented in the literature so far. The aim of this study was to establish the incidence and to evaluate the oncological outcomes of such patients. Methods Our large orthopaedic oncology database was used to review 70 malignant osseous foot tumour patients. Results The age at diagnosis of malignant osseous foot tumours demonstrated a bimodal distribution peaking in the second and eighth decades of life. Overall, 55 primary malignant bone tumours of the foot (79%) were identified. The median duration from onset of symptoms to diagnosis was 52 weeks (interquartile range [IQR]: 17–104). Eight primary tumours (15%) underwent an accidental excision (ie intralesional excision of a malignant bone tumour where some of the tumour has been left behind, also known as a ‘whoops procedure’) prior to referral to our unit. Forty-six patients (84%) underwent surgery overall and thirteen of these developed recurrence or metastases. Seven of eight patients with a previous accidental excision underwent amputation. Fifteen osseous metastatic foot lesions were identified. The median length of foot symptoms to diagnosis was 24 weeks (IQR: 20–36 weeks). The median time to death following diagnosis of osseous foot metastases was 20.1 months (IQR: 11.3–27.8 months). Conclusions A high index of suspicion and awareness of clinical features of malignant osseous foot tumours are both essential to avoid diagnostic delays. Amputation is associated with a respectable outcome for patients who have undergone previous accidental excisions.


2021 ◽  
Vol 15 (4) ◽  
pp. 322-330
Author(s):  
Lizz van der Heijden ◽  
Germán L. Farfalli ◽  
Inês Balacó ◽  
Cristina Alves ◽  
Marta Salom ◽  
...  

Purpose The main challenge in reconstruction after malignant bone tumour resection in young children remains how and when growth-plates can be preserved and which options remain if impossible. Methods We describe different strategies to assure best possible long-term function for young children undergoing resection of malignant bone tumours. Results Different resources are available to treat children with malignant bones tumours: a) preoperative planning simulates scenarios for tumour resection and limb reconstruction, facilitating decision-making for surgical and reconstructive techniques in individual patients; b) allograft reconstruction offers bone-stock preservation for future needs. Most allografts are intact at long-term follow-up, but limb-length inequalities and corrective/revision surgery are common in young patients; c) free vascularized fibula can be used as stand-alone reconstruction, vascularized augmentation of structural allograft or devitalized autograft. Longitudinal growth and joint remodelling potential can be preserved, if transferred with vascularized proximal physis; d) epiphysiolysis before resection with continuous physeal distraction provides safe resection margins and maintains growth-plate and epiphysis; e) 3D printing may facilitate joint salvage by reconstruction with patient-specific instruments. Very short stems can be created for fixation in (epi-)metaphysis, preserving native joints; f) growing endoprosthesis can provide for remaining growth after resection of epi-metaphyseal tumours. At ten-year follow-up, limb survival was 89%, but multiple surgeries are often required; g) rotationplasty and amputation should be considered if limb salvage is impossible and/or would result in decreased function and quality of life. Conclusion Several biological and technological reconstruction options must be merged and used to yield best outcomes when treating young children with malignant bone tumours. Level of Evidence Level V Expert opinion


2010 ◽  
Vol 35 (5) ◽  
pp. 354-361 ◽  
Author(s):  
D. Baumhoer ◽  
G. Jundt

Malignant bone tumours of the hands are uncommon. Although almost all lesions that occur in other parts of the skeleton can also affect the hands, their frequency, distribution and clinical characteristics differ. This review focusses on the histology of these tumours and gives an overview of the main differential diagnoses. Close correlation to radiologic and clinical features usually leads to the correct diagnosis.


2021 ◽  
Vol 14 (4) ◽  
pp. e242802
Author(s):  
Muthuvel Balasubramaniyan ◽  
Sanjay Verma ◽  
Sumeet Rajendra Dhawan ◽  
Akshay Kumar saxena

Osteoarticular tuberculosis of flat bones of the chest wall such as sternum, scapula and rib is extremely rare in children. Because of its atypical clinical presentation mimicking malignant bone tumours, diagnosis remains a challenge. Histological and microbiological diagnosis remains confirmatory. Antitubercular therapy is the cornerstone in management.


2021 ◽  
Vol 15 (4) ◽  
pp. 337-345
Author(s):  
Ilkka J. Helenius ◽  
Andreas H. Krieg

Purpose Axial malignant bone tumours are rare in children and adolescents, and their prognosis is still relatively poor due to non-specific symptoms, such as back or groin pain, which may result in late hospital presentation. Therefore, it is very important to raise awareness regarding this pathology. Methods We performed a narrative review, including scientific publications published in English. We searched Medline and Google Scholar databases for information on the incidence and prognosis of axial malignant bone tumours in children and adolescents (< 18 years). Outcomes of different surgical management strategies and reconstruction options were assessed. Results The incidence of primary malignant bone tumours before the age of 18 years is approximately five per one million population; around 25% of these tumours are located in the axial skeleton. With a five-year survival rate of 50%, tumours in an axial location (chest cage, spine, pelvis) are associated with a poorer prognosis than tumours in more peripheral locations. En bloc excision with clear margins has been shown to improve local control and overall survival, even though obtaining adequate surgical margins is difficult due to the close location of large neurovascular structures and other major organs. Spinal reconstruction options include instrumented fusion with allograft or expandable cage. Pelvic reconstruction is needed in internal hemipelvectomy, and the options include biological, endoprosthetic reconstructions, hip transposition, arthrodesis or creation of pseudoarthrosis and lumbopelvic instrumentation. Conclusion Early diagnosis, a timely adequate multidisciplinary management, appropriate en bloc excision, and reconstruction improve survival and quality of life in these patients. Level of Evidence V


Author(s):  
M. Hajizadeh ◽  
R. Falak ◽  
M. Tavakoli ◽  
R. Hosseinzadeh ◽  
M. Alipour ◽  
...  

Toxoplasma gondii (T. gondii) is an intracellular parasite that infects humans and seroprevalence of its infection varies from about 10 to 80 percent in different countries with a higher prevalence in warmer and humid regions. In this study, the rate of acute and chronic toxoplasmosis in patients with benign or malignant bone tumours was investigated. Fifty-three patients who suffered from various bone tumours, as well as sixty-five healthy controls with an unknown serological profile for anti-Toxoplasma antibodies, were enrolled in this cross-sectional study. Anti-toxoplasma antibodies were detected in serum samples using enzyme-linked immunosorbent assay (ELISA) and blood samples of them were used for real-time PCR. Thirty-two (60.32%) and twenty-one (39.63%) of patients had malignant tumours and benign tumours, respectively. The results showed a higher and significant seropositivity rate of IgM antibodies in primary bone tumour patients compared to the control group and Toxoplasma DNA became positive in 18.86% of patients with primary bone tumours and 6.15% of controls. Surprisingly, the high presence of parasite DNA was detected in patients with malignant tumours. The seroprevalence of T. gondii IgM antibody and DNA positivity among the cancer patients were significantly higher than healthy individuals. Also, chronic toxoplasmosis (it was shown with IgG positive) appears to be more common in people with benign cancers than malignancies. The study showed a relatively high seroprevalence of anti-T. gondii antibodies in patients with primary bone cancer. However, the considerable rate of positive blood samples for the presence of parasite’s DNA should not be ignored. A key to the effective management of diseases in immunosuppressed individuals is prompt and accurate diagnosis of toxoplasmosis. Moreover, it seems that PCR tests may be more reliable than serological methods and it could be considered as a precise method for diagnosis of acute toxoplasmosis. 


Author(s):  
Roberta Bovenzi ◽  
Matteo Conti ◽  
Rocco Cerroni ◽  
Mariangela Pierantozzi ◽  
Alessandro Stefani ◽  
...  

Abstract Background Adult-onset sporadic chorea includes a wide and heterogeneous group of conditions whose differential diagnosis and treatments are often challenging and extensive. Objectives To analyse retrospectively cases of adult-onset sporadic chorea from a single Italian centre to provide insights for a practical approach in the management of these patients. Methods A total of 11,071 medical charts from a 9-year period (2012–2020) were reviewed, identifying 28 patients with adult-onset sporadic chorea (genetic forms excluded). All available data regarding phenomenology, diagnostic workup, aetiology, treatments, and long-term outcome from this cohort were collected and analysed. Results Adult-onset sporadic chorea occurred more frequently in females and presented with an acute-subacute onset. Cerebrovascular diseases accounted for 68% of aetiology; further causes were structural brain lesions, internal diseases, and other movement disorder syndromes. Clinical course was mild, with spontaneous resolution or minimal disturbances in 82% of cases. Neuroimaging was fundamental to diagnose 76% of adult-onset sporadic chorea, an appropriate clinical examination contributed to the 14% of diagnoses, whereas basic laboratory tests to the 10%. Conclusions Revision of real-world data of adult-onset sporadic chorea patients from a single Italian cohort suggests that an accurate clinical examination, neuroimaging, and routine laboratory tests are useful to identify those cases underlying potentially severe but treatable conditions. Although in the majority of cases adult-onset sporadic chorea has mild clinical course and good response to symptomatic treatments, it is essential to run a fast diagnostic workup.


2021 ◽  
pp. 112070002110015
Author(s):  
Riccardo Zucchini ◽  
Andrea Sambri ◽  
Claudio Giannini ◽  
Michele Fiore ◽  
Carlotta Calamelli ◽  
...  

Introduction: Periacetabular reconstruction after resection of primary bone tumour is a very demanding procedure. They are frequently associated with scarce functional results and a high rate of complications. We report a series of patients with periacetabular resections for primary bone tumours and reconstruction with a porous tantalum (PT) acetabular cup (AC). Materials and methods: 27 patients (median age 30 years) were included, being affected by primary bone tumours of the pelvis and treated with peri-acetabular resection and reconstruction with a PT AC. The diagnoses were 13 osteosarcomas, 7 chondrosarcomas and 7 Ewing sarcomas. Function was assessed with the Harris Hip Score and complications were classified according to Zeifang. Results: The median follow-up was 70 months. 1 patient required removal of the PT AC because of implant associated infection 55 months after surgery. There was 1 hip dislocation and no case of aseptic loosening. At final follow-up, the median HHS was 81 points (range 48–92). Conclusions: The used PT AC had good medium-term survival rates and good functional results. This technique is a viable reconstructive option after resections of periacetabular primary bone sarcomas.


2021 ◽  
pp. 205141582110328
Author(s):  
Abisola Oliyide ◽  
Ijeoma Chibuzo ◽  
Magda Kujawa

Thrombosed urethral prolapse is a rare clinical condition. In this context, we describe our experience and compare our findings with the literature, following presentation of five consecutive cases over 2 years. This will hopefully improve awareness and appropriateness of specialty referrals as a correct diagnosis is rarely established prior to the patient being seen by a urologist. We also wish to highlight a case presentation of thrombosed urethral prolapse outside the bimodal age distribution which has been recorded in the literature. Level of Evidence: Level 4


2005 ◽  
Vol 29 (4) ◽  
pp. 255-259 ◽  
Author(s):  
M. Laitinen ◽  
J. Hardes ◽  
H. Ahrens ◽  
C. Gebert ◽  
B. Leidinger ◽  
...  

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