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OBJECTIVES: The revised European Society of Musculoskeletal Radiology (ESSR) consensus guidelines on soft tissue tumor imaging represent an update of 2015 after technical advancements, further insights into specific entities, and revised World Health Organization (2020) and AJCC (2017) classifications. This second of three papers covers algorithms once histology is confirmed: (1) standardized whole-body staging, (2) special algorithms for non-malignant entities, and (3) multiplicity, genetic tumor syndromes, and pitfalls. MATERIALS AND METHODS: A validated Delphi method based on peer-reviewed literature was used to derive consensus among a panel of 46 specialized musculoskeletal radiologists from 12 European countries. Statements that had undergone interdisciplinary revision were scored online by the level of agreement (0 to 10) during two iterative rounds, that could result in 'group consensus', 'group agreement', or 'lack of agreement'. RESULTS: The three sections contain 24 statements with comments. Group consensus was reached in 95.8% and group agreement in 4.2%. For whole-body staging, pulmonary MDCT should be performed in all high-grade sarcomas. Whole-body MRI is preferred for staging bone metastasis, with [18F]FDG-PET/CT as an alternative modality in PET-avid tumors. Patients with alveolar soft part sarcoma, clear cell sarcoma, and angiosarcoma should be screened for brain metastases. Special algorithms are recommended for entities such as rhabdomyosarcoma, extraskeletal Ewing sarcoma, myxoid liposarcoma, and neurofibromatosis type 1 associated malignant peripheral nerve sheath tumors. Satisfaction of search should be avoided in potential multiplicity. CONCLUSION: Standardized whole-body staging includes pulmonary MDCT in all high-grade sarcomas; entity-dependent modifications and specific algorithms are recommended for sarcomas and non-malignant soft tissue tumors. CLINICAL RELEVANCE STATEMENT: These updated ESSR soft tissue tumor imaging guidelines aim to provide support in decision-making, helping to avoid common pitfalls, by providing general and entity-specific algorithms, techniques, and reporting recommendations for whole-body staging in sarcoma and non-malignant soft tissue tumors. KEY POINTS: An early, accurate, diagnosis is crucial for the prognosis of patients with soft tissue tumors. These updated guidelines provide best practice expert consensus for standardized imaging algorithms, techniques, and reporting. Standardization can improve the comparability examinations and provide databases for large data analysis.
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Chordoma is a malignant bone tumor originating from notochordal remnants, most commonly occurring at the sacrococcygeal junction. We present a case of a 70-year-old male with chronic pain in the lower lumbar spine. MRI performed elsewhere revealed a large tumor that involved S4, S5, and the coccyx with a presacral soft tissue component. The lesion was heterogeneously hyperintense on T2-weighted images with a thick hypointense rim anteriorly. On T1-weighted images, the lesion showed a native hyperintense signal centrally probably due to hemorrhage. Based on this MRI, the diagnosis of chordoma was suggested. A spontaneous marked reduction in size was observed on a 4-week interval MRI performed at our institution before biopsy. Due to spontaneous tumor shrinkage along with peripheral enhancement, a differential diagnosis of infection or bleeding in a retrorectal cyst was proposed. This case teaches us that chordomas may contain a large hemorrhagic component, which is hyperintense on T1-weighted images and shows peripheral rim enhancement. Spontaneous shrinkage of a tumor may occur due to the resolution of a hematoma within weeks. Biopsy is key to obtain the correct diagnosis. Understanding the typical and more rare features of chordomas is key for MSK radiologists as well as pathologists. Chordomas are typically slow-growing tumors, but radiologists should be aware that intratumoral hemorrhage can lead to rapid changes in tumor size, which may be mistaken for either regression or progression of tumor. This case highlights the importance of considering hemorrhagic events within chordomas in the differential diagnosis when observing size fluctuations on imaging.
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Small cell osteosarcoma (SCOS), a variant of conventional high-grade osteosarcoma (COS), may mimic fusion-driven round cell sarcomas (FDRCS) by overlapping clinico-radiological and histomorphological/immunohistochemical characteristics, hampering accurate diagnosis and consequently proper therapy. We retrospectively analyzed decalcified formalin-fixed paraffin-embedded (FFPE) samples of 18 bone tumors primarily diagnosed as SCOS by methylation profiling, fusion gene analysis, and immunohistochemistry.In eight cases, the diagnosis of SCOS was maintained, and in 10 cases it was changed into FDRCS, including three Ewing sarcomas (EWSR1::FLI1 in two cases and no identified fusion gene in the third case), two sarcomas with BCOR alterations (KMT2D::BCOR, CCNB3::BCOR, respectively), three mesenchymal chondrosarcomas (HEY1::NCOA2 in two cases and one case with insufficient RNA quality), and two sclerosing epithelioid fibrosarcomas (FUS::CREBL3 and EWSR1 rearrangement, respectively).Histologically, SCOS usually possessed more pleomorphic cells in contrast to the FDRCS showing mainly monomorphic cellular features. However, osteoid was seen in the latter tumors as well, often associated with slight pleomorphism. Also, the immunohistochemical profile (CD99, SATB2, and BCOR) overlapped.Clinically and radiologically, similarities between SCOS and FDRCS were observed, with by imaging only minimal presence or lack of (mineralized) osteoid in most of the SCOSs.In conclusion, discrimination of SCOS, epigenetically related to COS, versus FDRCS of bone can be challenging but is important due to different biology and therefore therapeutic strategies. Methylation profiling is a reliable and robust diagnostic test especially on decalcified FFPE material. Subsequent fusion gene analysis and/or use of specific immunohistochemical surrogate markers can be used to substantiate the diagnosis.
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Neoplasias Ósseas , Osteossarcoma , Sarcoma de Células Pequenas , Sarcoma , Humanos , Estudos Retrospectivos , Sarcoma/genética , Sarcoma de Células Pequenas/genética , Neoplasias Ósseas/patologia , Osteossarcoma/genética , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/análise , Proteínas de Fusão Oncogênica/genéticaRESUMO
OBJECTIVES: Early, accurate diagnosis is crucial for the prognosis of patients with soft tissue sarcomas. To this end, standardization of imaging algorithms, technical requirements, and reporting is therefore a prerequisite. Since the first European Society of Musculoskeletal Radiology (ESSR) consensus in 2015, technical achievements, further insights into specific entities, and the revised WHO-classification (2020) and AJCC staging system (2017) made an update necessary. The guidelines are intended to support radiologists in their decision-making and contribute to interdisciplinary tumor board discussions. MATERIALS AND METHODS: A validated Delphi method based on peer-reviewed literature was used to derive consensus among a panel of 46 specialized musculoskeletal radiologists from 12 European countries. Statements were scored online by level of agreement (0 to 10) during two iterative rounds. Either "group consensus," "group agreement," or "lack of agreement" was achieved. RESULTS: Eight sections were defined that finally contained 145 statements with comments. Overall, group consensus was reached in 95.9%, and group agreement in 4.1%. This communication contains the first part consisting of the imaging algorithm for suspected soft tissue tumors, methods for local imaging, and the role of tumor centers. CONCLUSION: Ultrasound represents the initial triage imaging modality for accessible and small tumors. MRI is the modality of choice for the characterization and local staging of most soft tissue tumors. CT is indicated in special situations. In suspicious or likely malignant tumors, a specialist tumor center should be contacted for referral or teleradiologic second opinion. This should be done before performing a biopsy, without exception. CLINICAL RELEVANCE: The updated ESSR soft tissue tumor imaging guidelines aim to provide best practice expert consensus for standardized imaging, to support radiologists in their decision-making, and to improve examination comparability both in individual patients and in future studies on individualized strategies. KEY POINTS: ⢠Ultrasound remains the best initial triage imaging modality for accessible and small suspected soft tissue tumors. ⢠MRI is the modality of choice for the characterization and local staging of soft tissue tumors in most cases; CT is indicated in special situations. Suspicious or likely malignant tumors should undergo biopsy. ⢠In patients with large, indeterminate or suspicious tumors, a tumor reference center should be contacted for referral or teleradiologic second opinion; this must be done before a biopsy.
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Tenosynovial giant cell tumour (TGCT) is a rare soft-tissue tumour originating from synovial lining of joints, bursae and tendon sheaths. The tumour comprises two subtypes: the localised-type (L-TGCT) is characterised by a single, well-defined lesion, whereas the diffuse-type (D-TGCT) consists of multiple lesions without clear margins. D-TGCT was previously known as pigmented villonodular synovitis. Although benign, TGCT can behave locally aggressive, especially the diffuse-type. Magnetic resonance imaging (MRI) is the modality of choice to diagnose TGCT and discriminate between subtypes. MRI can also provide a preoperative map before synovectomy, the mainstay of treatment. Finally, since the arrival of colony-stimulating factor 1-receptor inhibitors, a novel systemic therapy for D-TGCT patients with relapsed or inoperable disease, MRI is key in assessing treatment response. As recurrence after treatment of D-TGCT occurs more often than in L-TGCT, follow-up imaging plays an important role in D-TGCT. Reading follow-up MRIs of these diffuse synovial tumours may be a daunting task. Therefore, this educational review focuses on MRI findings in D-TGCT of the knee, which represents the most involved joint site (approximately 70% of patients). We aim to provide a systematic approach to assess the knee synovial recesses, highlight D-TGCT imaging findings, and combine these into a structured report. In addition, differential diagnoses mimicking D-TGCT, potential pitfalls and evaluation of tumour response following systemic therapies are discussed. Finally, we propose automated volumetric quantification of D-TGCT as the next step in quantitative treatment response assessment as an alternative to current radiological assessment criteria.
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Tenosynovial giant cell tumour (TGCT) is a rare, locally aggressive, mesenchymal tumor arising from the joints, bursa and tendon sheaths. TGCT comprises a nodular- and a diffuse-type, with the former exhibiting mostly indolent course and the latter a locally aggressive behavior. Although usually not life-threatening, TGCT may cause chronic pain and adversely impact function and quality of life (QoL). CSFR1 inhibitors are effective with benefit on symptoms and QoL but are not available in most countries. The degree of uncertainty in selecting the most appropriate therapy and the lack of guidelines on the clinical management of TGCT make the adoption of new treatments inconsistent across the world, with suboptimal outcomes for patients. A global consensus meeting was organized in June 2022, involving experts from several disciplines and patient representatives from SPAGN to define the best evidence-based practice for the optimal approach to TGCT and generate the recommendations presented herein.
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Tumor de Células Gigantes de Bainha Tendinosa , Qualidade de Vida , Humanos , Consenso , Tumor de Células Gigantes de Bainha Tendinosa/tratamento farmacológico , Tumor de Células Gigantes de Bainha Tendinosa/patologiaRESUMO
PURPOSE: A major component of cells in tenosynovial giant cell tumor (TGCT) consists of bystander macrophages responding to CSF1 that is overproduced by a small number of neoplastic cells with a chromosomal translocation involving the CSF1 gene. An autocrine loop was postulated where the neoplastic cells would be stimulated through CSF1R expressed on their surface. Here, we use single-cell RNA sequencing (scRNA-seq) to investigate cellular interactions in TGCT. EXPERIMENTAL DESIGN: A total of 18,788 single cells from three TGCT and two giant cell tumor of bone (GCTB) samples underwent scRNA-seq. The three TGCTs were additionally analyzed using long-read RNA sequencing. Immunofluorescence and IHC for a range of markers were used to validate and extend the scRNA-seq findings. RESULTS: Two recurrent neoplastic cell populations were identified in TGCT that are highly similar to nonneoplastic synoviocytes. We identified GFPT2 as a marker that highlights the neoplastic cells in TCGT. We show that the neoplastic cells themselves do not express CSF1R. We identified overlapping MAB features between the giant cells in TGCT and GCTB. CONCLUSIONS: The neoplastic cells in TGCT are highly similar to nonneoplastic synoviocytes. The lack of CSF1R on the neoplastic cells indicates they may be unaffected by current therapies. High expression of GFPT2 in the neoplastic cells is associated with activation of the YAP1/TAZ pathway. In addition, we identified expression of the platelet-derived growth factor receptor in the neoplastic cells. These findings suggest two additional pathways to target in this tumor.
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Tumor de Células Gigantes de Bainha Tendinosa , Humanos , Tumor de Células Gigantes de Bainha Tendinosa/genética , Tumor de Células Gigantes de Bainha Tendinosa/metabolismo , Tumor de Células Gigantes de Bainha Tendinosa/patologia , Translocação GenéticaRESUMO
PURPOSE OF REVIEW: Giant cell tumors of bone (GCTB) are intermediate, locally aggressive primary bone tumors. For conventional GCTB, surgery remains treatment of choice. For advanced GCTB, a more important role came into play for systemic therapy including denosumab and bisphosphonates over the last decade. RECENT FINDINGS: In diagnostics, focus has been on H3F3A (G34) driver mutations present in GCTB. The most frequent mutation (G34W) can be detected using immunohistochemistry and is highly specific in differentiating GCTB from other giant cell containing tumors. PD-L1 expression can be used as biological marker to predict higher recurrence risks in GCTB patients.The use of bisphosphonate-loaded bone cement is under investigation in a randomized controlled trial. A new technique consisting of percutaneous microwave ablation and bisphosphonate-loaded polymethylmethacrylate cementoplasty was proposed for unresectable (pelvic) GCTB.Increased experience with use of denosumab raised concern on elevated recurrence rates. However, conclusions of meta-analyses should be interpreted with risk of indication bias in mind. Several small studies are published with short-course denosumab (varying from 3 to 6 doses). One small trial directly compared denosumab and zoledronic acid, with no statistical differences in radiological and clinical outcome, and nonsignificantly higher recurrence rate after denosumab. As bisphosphonates directly target neoplastic stromal cells in GCTB, larger directly comparative trials are still warranted. SUMMARY: Neoadjuvant denosumab is highly effective for advanced GCTB, and a short-course is advised to facilitate surgery, whereas increased recurrence rates remain of concern. Randomized controlled trials are conducted on bisphosphonate-loaded bone cement and on optimal dose and duration of neoadjuvant denosumab. PD-L1 could be a potential new therapy target in GCTB.
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Conservadores da Densidade Óssea , Neoplasias Ósseas , Tumor de Células Gigantes do Osso , Antígeno B7-H1 , Cimentos Ósseos/uso terapêutico , Conservadores da Densidade Óssea/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/genética , Denosumab/uso terapêutico , Tumor de Células Gigantes do Osso/tratamento farmacológico , Tumor de Células Gigantes do Osso/genética , Tumor de Células Gigantes do Osso/cirurgia , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , Ácido Zoledrônico/uso terapêuticoRESUMO
BACKGROUND AND OBJECTIVES: Surgery is the mainstay of treatment for tenosynovial giant cell tumors (TGCTs). However, achieving a cure through surgery alone remains challenging, especially for the diffuse-type (D-TGCT). METHODS: Our goal was to describe the surgical management of patients with D-TGCT related to large joints, treated between 2000 and 2020. We analyzed the effect of (in)complete resections and the presence of postoperative tumor (POT) on magnetic resonance imaging (MRI) on radiological and clinical outcomes. RESULTS: A total of 144 patients underwent open surgery for D-TGCT, of which 58 (40%) had treatment before. The median follow-up was 65 months. One hundred twenty-five patients underwent isolated open surgeries, in which 25 (20%) patients' D-TGCT was intentionally removed incompletely. POT presence on the first postoperative MRI was observed in 64%. Both incomplete resections and POT presence were associated with higher rates of radiological progression (73% vs. 44%; Kaplan-Meier [KM] analysis p = 0.021) and 59% versus 7%; KM analysis p < 0.001), respectively. Furthermore, patients with POT presence clinically worsened more often than patients without having POT (49% vs. 24%; KM analysis p = 0.003). CONCLUSIONS: D-TGCT is often resected incompletely and tumor presence is commonly observed on the first postoperative MRI, resulting in worse radiological and clinical outcomes. Therefore, surgeons should try to remove D-TGCT in toto and consider other multimodal therapeutic strategies.
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Tumor de Células Gigantes de Bainha Tendinosa , Neoplasias Embrionárias de Células Germinativas , Neoplasias Testiculares , Estudos de Coortes , Tumor de Células Gigantes de Bainha Tendinosa/patologia , Tumor de Células Gigantes de Bainha Tendinosa/cirurgia , Humanos , MasculinoRESUMO
INTRODUCTION: Tenosynovial giant cell tumor (TGCT) is a mono-articular, benign or locally aggressive and often debilitating neoplasm. Systemic therapies are becoming part of the multimodal armamentarium when surgery alone will not confer improvements. Since TGCT is characterized by colony-stimulating factor-1 (CSF1) rearrangements, the most studied molecular pathway is the CSF1 and CSF1 receptor (CSF1R) axis. Inhibiting CSF1-CSF1R interaction often yields considerable radiological and clinical responses; however, adverse events may cause treatment discontinuation because of an unfavorable risk-benefit ratio in benign disease. Only Pexidartinib is approved by the US FDA; however, the European Medicines Agency has not approved it due to a uncertain risk-benefit ratio. Thus, there is a need for safer and effective therapies. AREAS COVERED: Light is shed on disease mechanisms and potential drug targets. The safety and efficacy of different systemic therapies are evaluated. EXPERT OPINION: The CSF1-CSF1R axis is the principal drug target; however, the effect of CSF1R inhibition on angiogenesis and the role of macrophages, which are essential in the postoperative course, needs further elucidation. Systemic therapies have a promising role in treating mainly diffuse-type, TGCT patients who are not expected to clinically improve from surgery. Future drug development should focus on targeting neoplastic TGCT cells.
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Tumor de Células Gigantes de Bainha Tendinosa , Tumor de Células Gigantes de Bainha Tendinosa/tratamento farmacológico , Tumor de Células Gigantes de Bainha Tendinosa/metabolismo , Tumor de Células Gigantes de Bainha Tendinosa/patologia , Humanos , Macrófagos/metabolismo , Biologia MolecularRESUMO
BACKGROUND: Atypical cartilaginous tumour (ACT) and grade II chondrosarcoma (CS2) of long bones are respectively managed with watchful waiting or curettage and wide resection. Preoperatively, imaging diagnosis can be challenging due to interobserver variability and biopsy suffers from sample errors. The aim of this study is to determine diagnostic performance of MRI radiomics-based machine learning in differentiating ACT from CS2 of long bones. METHODS: One-hundred-fifty-eight patients with surgically treated and histology-proven cartilaginous bone tumours were retrospectively included at two tertiary bone tumour centres. The training cohort consisted of 93 MRI scans from centre 1 (n=74 ACT; n=19 CS2). The external test cohort consisted of 65 MRI scans from centre 2 (n=45 ACT; n=20 CS2). Bidimensional segmentation was performed on T1-weighted MRI. Radiomic features were extracted. After dimensionality reduction and class balancing in centre 1, a machine-learning classifier (Extra Trees Classifier) was tuned on the training cohort using 10-fold cross-validation and tested on the external test cohort. In centre 2, its performance was compared with an experienced musculoskeletal oncology radiologist using McNemar's test. FINDINGS: After tuning on the training cohort (AUC=0.88), the machine-learning classifier had 92% accuracy (60/65, AUC=0.94) in identifying the lesions in the external test cohort. Its accuracies in correctly classifying ACT and CS2 were 98% (44/45) and 80% (16/20), respectively. The radiologist had 98% accuracy (64/65) with no difference compared to the classifier (p=0.134). INTERPRETATION: Machine learning showed high accuracy in classifying ACT and CS2 of long bones based on MRI radiomic features. FUNDING: ESSR Young Researchers Grant.
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Neoplasias Ósseas , Condrossarcoma , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Condrossarcoma/diagnóstico por imagem , Condrossarcoma/patologia , Humanos , Aprendizado de Máquina , Imageamento por Ressonância Magnética/métodos , Estudos RetrospectivosRESUMO
Hibernomas are rare lipomatous tumors composed of brown adipocytes. The relative paucity of reported cases involving the bones accounts for the poor understanding of this entity, which is known to affect almost exclusively the axial skeleton. We present a case of intraosseous hibernoma of the humerus, which was found incidentally in a 52-year-old woman and initially misinterpreted as a cartilaginous tumor on magnetic resonance imaging (MRI). The lesion was unchanged in size and morphology at short interval follow-up but increased in size during follow-up over 6 years with an 11 mm increase in the largest diameter. Given the patient's concerns and lesion growth, curettage was performed. Pathology analysis revealed brown fat in keeping with the diagnosis of intraosseous hibernoma. Radiological and pathological findings and pitfalls are herein highlighted to enforce knowledge on this lesion rarely affecting the long bones. Radiologists should think of intraosseous hibernoma if they come across a sclerotic lesion on X-ray or computed tomography, which contains macroscopic fat and shows enhancement on contrast-enhanced MRI. In addition, an intraosseous hibernoma may be picked up incidentally on positron emission tomography-computed tomography due to high fluorodeoxyglucose avidity.
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Lipoma , Tecido Adiposo Marrom/patologia , Diagnóstico Diferencial , Feminino , Fluordesoxiglucose F18 , Humanos , Úmero , Lipoma/diagnóstico por imagem , Lipoma/patologia , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Although curative surgery remains the cornerstone of the therapeutic strategy in patients with soft tissue sarcomas (STS), neoadjuvant radiotherapy and chemotherapy (NART and NACT, respectively) are increasingly used to improve operability, surgical margins and patient outcome. The best imaging modality for locoregional assessment of STS is MRI but these tumors are mostly evaluated in a qualitative manner. OBJECTIVE: After an overview of the current standard of care regarding treatment for patients with locally advanced STS, this review aims to summarize the principles and limitations of (i) the current methods used to evaluate response to neoadjuvant treatment in clinical practice and clinical trials in STS (RECIST 1.1 and modified Choi criteria), (ii) quantitative MRI sequences (i.e., diffusion weighted imaging and dynamic contrast enhanced MRI), and (iii) texture analyses and (delta-) radiomics.
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Sarcoma , Neoplasias de Tecidos Moles , Humanos , Imageamento por Ressonância Magnética/métodos , Terapia Neoadjuvante/métodos , Estudos Retrospectivos , Sarcoma/tratamento farmacológico , Sarcoma/terapia , Neoplasias de Tecidos Moles/tratamento farmacológico , Neoplasias de Tecidos Moles/terapia , Resultado do TratamentoRESUMO
BACKGROUND: Histotype specific neoadjuvant therapy response data is scarce in soft tissue sarcomas. This study aimed to assess the impact of a moderate radiotherapy (RT) dose on resectability and to correlate MRI parameters to pathological treatment response in Myxoid Liposarcoma (MLS). METHODS: This prospective, multicenter, single-arm, phase 2 trial assessed the radiological effects of 36 Gy of preoperative radiotherapy in primary non-metastatic MLS (n=34). Distance of the tumor to the neurovascular bundle, tumor dimensions, fat fraction, enhancing fraction were determined on MRI scans at baseline, after 8 and 16 fractions, and preoperatively. Pathological response was established by central pathology review. RESULTS: Preoperative radiotherapy resulted in a median increase of 2 mm (IQR 0 to 6) of the distance of the tumor to the neurovascular bundle. As compared to baseline, the median change of the tumor volume, craniocaudal diameter and axial diameter at preoperative MRI were -60% (IQR -74 to -41), -19% (IQR -23 to -7) and -20% (IQR -29 to -12), respectively. The median fat fraction of 0.1 (IQR 0.0-0.1) and enhancing fraction of 0.8 (IQR 0.6 to 0.9) at baseline, changed to 0.2 (IQR 0.1 to 0.5) and to 0.5(IQR 0.4 to 0.9) preoperatively, respectively. Radiological signs of response in terms of volume, enhancing fraction and fat fraction were correlated with specific pathological signs of response like hyalinization, necrosis and fatty maturation. CONCLUSIONS: A moderate dose of preoperative radiotherapy may improve resectability in MLS and could facilitate achievement of clear margins and function preservation. MRI features which were predictive for expressions of pathological response, can play a role in further personalization of neoadjuvant treatment strategies in order to improve outcome in MLS.
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Lipossarcoma Mixoide/diagnóstico por imagem , Lipossarcoma Mixoide/radioterapia , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/radioterapia , Tecido Adiposo/diagnóstico por imagem , Adiposidade , Adulto , Vasos Sanguíneos/diagnóstico por imagem , Fracionamento da Dose de Radiação , Feminino , Humanos , Lipossarcoma Mixoide/patologia , Lipossarcoma Mixoide/cirurgia , Imageamento por Ressonância Magnética , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Terapia Neoadjuvante , Neoplasia Residual , Nervos Periféricos/diagnóstico por imagem , Período Pré-Operatório , Estudos Prospectivos , Radioterapia Adjuvante , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Carga TumoralRESUMO
INTRODUCTION: Oligometastatic disease and/or oligoprogression in myxoid liposarcoma(oMLS) triggers discussions on local treatment options and delay of systemic treatments. We hypothesized that satisfactory local control and postponement of systemic therapy could be achieved with a modest radiotherapy(RT) dose in oMLS. METHODS: The DOREMY trial is a multicenter, phase 2 trial evaluating efficacy and toxicity of a modest RT dose in both localized and oMLS; this report presents the data of the oMLS cohort treated with 36 Gy in 12-18 fractions with optional subsequent metastasectomy. The primary endpoint was local progression free survival(LPFS). Secondary endpoints included postponement of systemic therapy, symptom reduction, radiological objective response, and toxicity. RESULTS: Nine patients with a total of 25 lesions were included, with a median follow-up of 23 months. The median number of lesions per patient was three and the trunk wall and bone were the most frequently affected sites. In lesions treated with definitive RT(n = 21), LPFS rates at 1, 2, and 3 years were respectively 73%, 61%, and 40%. Radiological objective response and clinical symptom reduction were achieved in 8/15(53%) and 9/10(90%) of the evaluable lesions, respectively. No local recurrences occurred in lesions treated with RT and metastasectomy(n = 4). For the entire study population, the median postponement of systemic therapy was 10 months. Grade ≥ 2 toxicity was observed in 2/9(22%) of patients. CONCLUSIONS: This trial suggests that 36 Gy could possibly be effective to achieve local control, postpone systemic therapy and reduce symptoms in oMLS. Given the minimal toxicity this treatment could be reasonably considered in oMLS.
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Lipossarcoma Mixoide , Radioterapia (Especialidade) , Adulto , Terapia Combinada , Humanos , Lipossarcoma Mixoide/radioterapia , Recidiva Local de Neoplasia , Estudos RetrospectivosRESUMO
Giant cell tumours of bone (GCTB) are benign giant cell-rich tumours typically occurring in the epi-metaphysis of skeletally mature patients. Despite their benign classification, GCTB may be locally aggressive with local recurrence as a challenging issue. Denosumab is a human monoclonal antibody that inhibits osteolysis via the RANK-RANK ligand pathway. There is currently no consensus on optimal treatment duration or imaging modality for monitoring patients on denosumab therapy. This review illustrates the radiological findings of GCTB on denosumab treatment seen on plain radiographs, CT, MRI, PET-CT and DEXA, with reference to the current literature. Recognizing imaging features indicative of a positive response to denosumab is important for therapeutic decision-making. Imaging findings with respect to duration of denosumab treatment, tumour upregulation during treatment, tumour recurrence and malignant transformation are discussed. The development of a sclerotic neocortex and varying degrees of matrix osteosclerosis are seen on plain radiographs. Reconstitution of subarticular bone and articular surface irregularity are optimally evaluated on CT which can also quantify tumour density. MRI demonstrates heterogeneous low signal matrix and is useful to assess decrease in size of cystic and/or soft tissue components of GCTB. A fat-suppressed fluid-sensitive MR sequence is important to detect tumour reactivation. Reduction in 18F-FDG-PET avidity represents an early sensitive sign of response to denosumab treatment. Regardless of imaging modality, close follow-up in a specialist centre and careful evaluation of nonresponders is necessary as local recurrence after cessation of denosumab treatment and malignant transformation of GCTB have been described.
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Conservadores da Densidade Óssea , Neoplasias Ósseas , Tumor de Células Gigantes do Osso , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/tratamento farmacológico , Denosumab/uso terapêutico , Tumor de Células Gigantes do Osso/diagnóstico por imagem , Tumor de Células Gigantes do Osso/tratamento farmacológico , Humanos , Recidiva Local de Neoplasia , Tomografia por Emissão de Pósitrons combinada à Tomografia ComputadorizadaRESUMO
Liposarcomas are the most common soft tissue sarcoma. They occur mainly in the thigh or retroperitoneum. Due to their size, lipomatous tumours can herniate either through the abdominal wall or in the groin. The part of the tumour that herniates represents only the 'tip of the iceberg', as the main part of the tumour is not detectable clinically and is often underestimated. Due to their deep location, lipomatous tumours are often large at the time of presentation and therefore their surgical management can be challenging. Furthermore, due to their delayed presentation, there is a higher risk of de-differentiation. In this pictorial review, we discuss different presentations of herniating lipomatous tumours according to the location of the abdominal wall defects. We aim to cover a wide spectrum of hernia defects including inguinal, ventral, lumbar, sciatic and ischiorectal hernias. We also present cases of tumours within the psoas compartment 'herniating' from the pelvis into the thigh. In case of a palpable lump, the first diagnostic step is to perform an ultrasound. If the herniating tissue is not fully accessible with ultrasound, additional cross-sectional imaging by CT or MRI is warranted. In this article, CT and MRI findings in lipomatous tumours are addressed and the use of contrast enhanced sequences in MRI is discussed. Patients' outcome depends not only on adequate diagnosis but also on the correct route of tissue sampling for histology and oncological resection to prevent local recurrence and loss of function. Therefore, referral to a specialised sarcoma treatment centre is key and needs to be done before biopsy.