RESUMEN
Adipocytic tumors are the most common mesenchymal tumors in soft tissues. Among them, a diagnostic challenge relies in the distinction between lipoma and atypical lipomatous tumor (ALT)/well differentiated liposarcoma (WDLPS), as both entities are often undistinguishable not only from a radiological point of view, but also at the microscopic level and particularly when dealing with small tumor specimen. Thus, detection of recurrent MDM2 amplifications may be the only criteria to discriminate malignant tumors from lipomas. In this study, we report the case of a patient diagnosed with a well differentiated, adipocytic tumor located in the inferior limb and lacking MDM2 amplification, whose diagnosis was reclassified for ALT/WDLPS after identification of an alternative MDM4 amplification by comparative genomic hybridization profiling, whole exome sequencing and fluorescence in situ hybridization (FISH). Screening of a cohort of 37 large, deep-seated, well-differentiated adipocytic tumors previously classified as lipomas using RT-qPCR and FISH failed to detect other cases of MDM4-amplified ALT/WDLPS. This report shows that MDM4 amplification is an exceptional molecular event alternative to MDM2 amplification in ALT/WDLPS. This alteration should be considered and looked for in suspicious adipocytic tumors to optimize their surgical management.
Asunto(s)
Lipoma , Liposarcoma , Humanos , Liposarcoma/diagnóstico , Liposarcoma/genética , Liposarcoma/patología , Amplificación de Genes , Hibridación Fluorescente in Situ , Hibridación Genómica Comparativa , Proteínas Proto-Oncogénicas c-mdm2/genética , Proteínas Proto-Oncogénicas c-mdm2/metabolismo , Lipoma/diagnóstico , Lipoma/genética , Lipoma/patología , Biomarcadores de Tumor/genética , Proteínas Proto-Oncogénicas/genética , Proteínas de Ciclo Celular/genéticaRESUMEN
BACKGROUND: Differentiating atypical lipomatous tumors (ALTs) and well-differentiated liposarcomas (WDLs) from benign lipomatous lesions is important for guiding clinical management, though conventional visual analysis of these lesions is challenging due to overlap of imaging features. Radiomics-based approaches may serve as a promising alternative and/or supplementary diagnostic approach to conventional imaging. PURPOSE: The purpose of this study is to review the practice of radiomics-based imaging and systematically evaluate the literature available for studies evaluating radiomics applied to differentiating ALTs/WDLs from benign lipomas. REVIEW: A background review of the radiomic workflow is provided, outlining the steps of image acquisition, segmentation, feature extraction, and model development. Subsequently, a systematic review of MEDLINE, EMBASE, Scopus, the Cochrane Library, and the grey literature was performed from inception to June 2022 to identify size studies using radiomics for differentiating ALTs/WDLs from benign lipomas. Radiomic models were shown to outperform conventional analysis in all but one model with a sensitivity ranging from 68 to 100% and a specificity ranging from 84 to 100%. However, current approaches rely on user input and no studies used a fully automated method for segmentation, contributing to interobserver variability and decreasing time efficiency. CONCLUSION: Radiomic models may show improved performance for differentiating ALTs/WDLs from benign lipomas compared to conventional analysis. However, considerable variability between radiomic approaches exists and future studies evaluating a standardized radiomic model with a multi-institutional study design and preferably fully automated segmentation software are needed before clinical application can be more broadly considered.
Asunto(s)
Lipoma , Liposarcoma , Humanos , Liposarcoma/patología , Lipoma/diagnóstico por imagenRESUMEN
This review examines findings of musculoskeletal neoplasms whose equivocal imaging and/or histopathologic features make it difficult to determine if they will show aggressive behavior. We include both intermediate tumors as defined by the World Health Organization (WHO), and a single low-grade malignancy, low-grade central osteosarcoma, which mimics a benign lesion on imaging and histology. Intermediate tumors are a broad category and are subdivided into tumors that have risk of local recurrence only, and ones that have a risk of distant limb and pulmonary metastases. Difficult intermediate musculoskeletal lesions include atypical cartilaginous tumor/grade 1 chondrosarcoma, atypical lipomatous tumor/grade 1 liposarcoma, and solitary fibrous tumor. We review diagnostic criteria, differential diagnosis, and recommendations for surveillance.
Asunto(s)
Neoplasias Óseas , Lipoma , Liposarcoma , Neoplasias de los Tejidos Blandos , Humanos , Lipoma/patología , Neoplasias de los Tejidos Blandos/diagnóstico por imagen , Neoplasias de los Tejidos Blandos/patología , Liposarcoma/patología , Huesos/patología , Diagnóstico Diferencial , Neoplasias Óseas/diagnóstico por imagenRESUMEN
BACKGROUND: According to guidelines, every soft tissue tumor (STT) larger than 3 cm should be biopsied before definitive resection. Advances in magnetic resonance imaging (MRI) improve the possibility to give a provisional diagnosis of the tumor's entity. Can lipomas and atypical lipomatous tumors (ALTs) of the extremities therefore be primarily marginally resected based on interpretation of MR images without a previous biopsy?. METHODS: In this retrospective, single-center study, 240 patients with the suspicion of a lipomatous tumor in MRI and surgical treatment in our institution between 2011 and 2020 were included. MR imaging was performed before surgery. All resected specimens underwent histopathological analysis. RESULTS: The collective comprised 142 tumors that were suspected as lipoma or ALT by the radiologist and underwent primary marginal resection (PMR). One case had myxoid liposarcoma that was underestimated on MRI and needed radical follow-up resection. One-hundred forty-one patients were cured after PMR. Ninety-eight patients were biopsied initially and in 93 cases resected afterwards according to the necessary oncological margins. CONCLUSION: In our institution, PMR is performed if a lipoma or ALT is suspected on MR imaging. Our treatment method and the diagnostic algorithm are presented. Primary resection spares patients from one surgical procedure, but a slight risk for underestimation of the tumor remains.
Asunto(s)
Lipoma , Liposarcoma , Neoplasias de los Tejidos Blandos , Adulto , Biopsia , Diagnóstico Diferencial , Humanos , Lipoma/diagnóstico , Lipoma/patología , Lipoma/cirugía , Liposarcoma/cirugía , Estudios Retrospectivos , Neoplasias de los Tejidos Blandos/cirugíaRESUMEN
OBJECTIVE: This study aimed to assess the efficacy of using MRI findings for differentiating musculoskeletal dedifferentiated liposarcoma (DDLP) from atypical lipomatous tumor (ALT). MATERIALS AND METHODS: This study included 22 patients with histopathologically proven DDLP and 35 with ALT in the musculoskeletal areas. All DDLPs were immunohistochemically positive for MDM2. MRI findings for both pathologies were retrospectively reviewed and compared. RESULTS: The maximum lesion diameter was significantly lower in DDLPs than in ALTs (p < 0.01). Ill-defined margin, peritumoral edema, and tail sign were more frequently observed in DDLPs than in ALTs (p < 0.01, respectively). The fatty component was less frequently observed in DDLPs than in ALTs (27 vs. 100%; p < 0.01), whereas the non-fatty component was more frequently observed in DDLPs than in ALTs (100 vs. 11%; p < 0.01). The occupation rate by non-fatty components was significantly higher in DDLPs than in ALTs (p < 0.01). No significant differences were observed in imaging findings associated with fatty component; however, necrosis within the non-fatty component on the contrast-enhanced image was more frequently observed in DDLPs than in ALTs (72 vs. 0%, p < 0.05). CONCLUSION: DDLPs always had a non-fatty component, whereas ALTs always had fatty component. Ill-defined margin, peritumoral edema, tail sign, and necrosis within non-fatty components were useful MRI features for differentiating musculoskeletal DDLP from ALT.
Asunto(s)
Liposarcoma , Humanos , Estudios Retrospectivos , Liposarcoma/diagnóstico por imagen , Liposarcoma/patología , Imagen por Resonancia Magnética/métodos , NecrosisRESUMEN
PURPOSE: Benign, intermediate-grade and malignant tumors sometimes have overlapping imaging and clinical characteristics. The purpose of this study was to evaluate the added value of contrast-enhanced sequences (dynamic contrast enhancement (DCE)), diffusion-weighted imaging (DWI), and chemical shift imaging (CSI) to noncontrast MRI sequences for the characterization of indeterminate lipomatous tumors. MATERIALS AND METHODS: Thirty-two consecutive patients with histologically proven peripheral lipomatous tumors were retrospectively evaluated. Two musculoskeletal radiologists recorded the MRI features in three sessions: (1) with noncontrast T1-weighted and fluid-sensitive sequences; (2) with addition of static pre- and post-contrast 3D volumetric T1-weighted sequences; and (3) with addition of DCE, DWI, and CSI. After each session, readers recorded a diagnosis (benign, intermediate/atypical lipomatous tumor (ALT), or malignant/dedifferentiated liposarcoma (DDL)). Categorical imaging features (presence of septations, nodules, contrast enhancement) and quantitative metrics (apparent diffusion coefficient values, CSI signal loss) were recorded. RESULTS: For 32 tumors, the diagnostic accuracy of both readers did not improve with the addition of contrast-enhanced sequences, DWI, or CSI (53% (17/32) session 1; 50% (16/30) session 2; 53% (17/32) session 3). Noncontrast features, including thick septations (p = 0.025) and nodules ≥ 1 cm (p < 0.001), were useful for differentiating benign tumors from ALTs and DDLs, as were DWI (p = 0.01) and CSI (p = 0.009) metrics. CONCLUSION: The addition of contrast-enhanced sequences (static, DCE), DWI, and CSI to a conventional, noncontrast MRI protocol did not improve diagnostic accuracy for differentiating benign, intermediate-grade, and malignant lipomatous tumors. However, we identified potentially useful imaging features by DCE, DWI, and CSI that may help distinguish these entities.
Asunto(s)
Medios de Contraste , Aumento de la Imagen/métodos , Lipoma/diagnóstico por imagen , Liposarcoma/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Neoplasias de los Músculos/diagnóstico por imagen , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Diagnóstico Diferencial , Imagen de Difusión por Resonancia Magnética , Femenino , Humanos , Masculino , Persona de Mediana Edad , Reproducibilidad de los Resultados , Estudios Retrospectivos , Sensibilidad y Especificidad , Adulto JovenRESUMEN
Hibernomas are soft tissue tumors derived from remnants of brown fat. They are rare masses that can have variable presentations ranging from incidental asymptomatic masses to pain due to nerve compression. We present the case of a 52-year-old male presenting with an atypical lipomatous mass on ultrasound and magnetic resonance imaging. The mass was excised and sent for pathology with the result being a hibernoma. We should be vigilant in the treatment of such tumor presentations as they may be a low grade liposarcoma in disguise. Surgical biopsy or excision is the best treatment for achieving a definite diagnosis.
Asunto(s)
Lipoma , Liposarcoma , Neoplasias de los Tejidos Blandos , Diagnóstico Diferencial , Humanos , Lipoma/diagnóstico por imagen , Lipoma/cirugía , Liposarcoma/diagnóstico por imagen , Liposarcoma/cirugía , Masculino , Persona de Mediana Edad , Hombro/patología , Neoplasias de los Tejidos Blandos/diagnóstico por imagen , Neoplasias de los Tejidos Blandos/cirugíaRESUMEN
OBJECTIVE. Diagnostic accuracy of core needle biopsy (CNB) for adipocytic tumors can be low because of sampling error from these often large, heterogeneous lesions. The purpose of this study was to evaluate the diagnostic accuracy of image-guided CNB for various adipocytic tumors in comparison with excisional pathology. MATERIALS AND METHODS. Adipocytic tumors (n = 77) of all adult patients undergoing image-guided CNB and subsequent surgical excision of an adipocytic tumor at a tertiary referral center between 2005 and 2019 were studied. To determine concordance, we compared pathologic diagnoses based on CNB to the reference standard of pathologic diagnoses after surgical excision. Tumors were divided into three categories (benign lipomatous tumors [lipoma, lipoma variants, hibernomas], atypical lipomatous tumors [ALTs] or well-differentiated liposarcomas [WDLs], and higher grade liposarcomas [myxoid, dedifferentiated, pleomorphic]), and diagnostic accuracy was calculated for each category. RESULTS. In 73 of 77 adipocytic tumors (95%), diagnosis at CNB and diagnosis after excision were concordant. Accuracy of diagnosis was poorer for ALTs and WDLs than for the other two categories, and the difference was statistically significant (p < .002). For the 29 benign lipomatous tumors and the 27 higher-grade liposarcomas, diagnoses at CNB and after excision were concordant in all cases (100%). Seventeen of the 21 tumors (81%) diagnosed as ALTs or WDLs at CNB had a concordant diagnosis after excision; four of the 21 were upgraded (dedifferentiated liposarcoma, n = 3; myxoid liposarcoma, n = 1). CONCLUSION. CNB provides high diagnostic accuracy for adipocytic tumors, particularly for benign lipomatous tumors and higher grade liposarcomas. However, though still high at 81%, diagnostic accuracy of CNB is not as high for tumors diagnosed as ALTs or WDLs. Awareness of this limitation is important when determining management, particularly of cases of ALT or WDL for which surgery is not planned.
Asunto(s)
Biopsia con Aguja Gruesa/métodos , Biopsia Guiada por Imagen/métodos , Neoplasias de Tejido Adiposo/patología , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Lipoma/diagnóstico por imagen , Lipoma/patología , Liposarcoma/diagnóstico , Liposarcoma/patología , Masculino , Persona de Mediana Edad , Neoplasias de Tejido Adiposo/diagnóstico , Reproducibilidad de los Resultados , Estudios RetrospectivosRESUMEN
After Evans and colleagues identified the lipomatous tumor with a well-differentiated liposarcoma in a subcutaneous location or within a muscle layer, namely, atypical lipomatous tumor (ALT), this malignancy has been investigated to clarify the characteristics of clinical behavior and genomic changes. As one of the important issues for clinicians, it is a hot topic of how to distinguish ALT from benign lipoma in the clinical aspect. Recent studies revealed novel findings to clarify the risk factor for the diagnosis of ALT and molecular targets for the treatment of ALT. Clinical characteristics of superficial-type ALT well reflect the subcutaneous location of the tumor and are slightly different compared to deep-type ALT, such as tumor size. In addition, there has been a recent discovery of novel findings in ALT-related genes, namely, HMG2A (high mobility group protein 2a), YEATS4 (YEATS domain containing 4), and CPM (Carboxypeptidase M). Recent updates on treatment for advanced ALT are well developed including immunotherapy and conducting clinical trials. Finally, this review introduces one of the hot topics of ALT research focused on epigenetic changes: their attention in recent updates on clinical characteristics and the novel discovery of related genes, treatment, and epigenetic modifications in atypical lipomatous tumors.
Asunto(s)
Liposarcoma/genética , Animales , Antineoplásicos/uso terapéutico , Epigénesis Genética , Predisposición Genética a la Enfermedad , Humanos , Liposarcoma/diagnóstico por imagen , Liposarcoma/tratamiento farmacológico , Liposarcoma/patologíaRESUMEN
BACKGROUND: Lipomatous masses are the most common soft tissue tumors. While the majority are benign lipomas, it is important to identify those masses that are malignant prior to excision. Current guidelines recommend core needle biopsy (CNB) for all lipomatous masses larger than 3-5 cm. The objective of this study was to determine if routine preoperative CNB based on mass size is necessary, or if radiographic features can guide the need for CNB. MATERIALS AND METHODS: Patients who underwent excision of extremity or truncal lipomatous masses at a single institution from October 2014 to July 2017 were retrospectively reviewed. By protocol, preoperative imaging was routinely obtained for all masses larger than 5 cm. High-risk radiographic features (intramuscular location, septations, nonfat nodules, heterogeneity, and ill-defined margins) and surgical pathology were evaluated to determine patients most likely to benefit from preoperative CNB. RESULTS: Of 178 patients, 2 (1.1%) had malignant tumors on surgical pathology. All masses smaller than 5 cm were benign and, if imaging was obtained, had two or fewer high-risk radiographic features. Both of the patients with malignant tumors had masses larger than 5 cm, preoperative imaging that showed at least four high-risk radiographic features, and underwent CNB prior to excision. CONCLUSIONS: The overall rate of malignancy is very low. The results of this study suggest that lipomatous masses smaller than 5 cm without concerning clinical characteristics do not require preoperative imaging or CNB. Conversely, lipomatous masses larger than 5 cm should undergo routine MRI with subsequent CNB if multiple high-risk radiographic features are present.
Asunto(s)
Lipoma/diagnóstico , Liposarcoma/diagnóstico , Cuidados Preoperatorios/normas , Neoplasias de los Tejidos Blandos/diagnóstico , Adulto , Biopsia con Aguja Gruesa/normas , Biopsia con Aguja Gruesa/estadística & datos numéricos , Diagnóstico Diferencial , Femenino , Humanos , Lipoma/patología , Lipoma/cirugía , Liposarcoma/cirugía , Imagen por Resonancia Magnética/normas , Imagen por Resonancia Magnética/estadística & datos numéricos , Masculino , Persona de Mediana Edad , Guías de Práctica Clínica como Asunto , Cuidados Preoperatorios/métodos , Estudios Retrospectivos , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/cirugía , Carga TumoralRESUMEN
Atypical and malignant lipomatous tumors are infrequent in the pediatric population. Within this uncommon cohort, the morphologically and genetically related spectrum of atypical lipomatous tumor/well-differentiated liposarcoma/dedifferentiated liposarcoma (ALT/WDL/DDLS) is markedly rare. Their shared characteristic molecular aberration is a genomic amplicon of a region of chromosome 12q, including the oncogenes MDM2 and CDK4. We present an unusual case of a pediatric patient with an ALT, with recurrence after 2 years in the form of a bone-forming mass, radiologically and pathologically mimicking parosteal osteosarcoma, a tumor also molecularly characterized by amplification of MDM2 and CDK4. However, with ample histologic sampling, a single focus of lipogenic differentiation was identified, thus representing the first near complete low-grade osteosarcomatous dedififferentation reported within ALT/WDL/DDLS and the first ever in pediatric patient. The case serves a reminder of a diagnosis differential and pitfalls within MDM2-amplified tumors.
Asunto(s)
Liposarcoma/diagnóstico , Liposarcoma/patología , Recurrencia Local de Neoplasia/diagnóstico , Recurrencia Local de Neoplasia/patología , Osteosarcoma/diagnóstico , Adolescente , Desdiferenciación Celular , Diagnóstico Diferencial , Femenino , Amplificación de Genes , Humanos , Liposarcoma/genética , Clasificación del Tumor , Recurrencia Local de Neoplasia/genética , Proteínas Proto-Oncogénicas c-mdm2/genética , Neoplasias de los Tejidos Blandos/diagnóstico , Neoplasias de los Tejidos Blandos/genética , Neoplasias de los Tejidos Blandos/patologíaRESUMEN
OBJECTIVES: The objectives of the study are (1) to distinguish lipoma (L) from atypical lipomatous tumor (ALT) using MRI qualitative features, (2) to assess the value of contrast enhancement, and (3) to evaluate the reproducibility and confidence level of radiological readings. MATERIALS AND METHODS: Patients with pathologically proven L or ALT, who underwent MRI within 3 months from surgical excision were included in this retrospective multicenter international study. Two radiologists independently reviewed MRI centrally. Impressions were recorded as L or ALT. A third radiologist was consulted for discordant readings. The two radiologists re-read all non-contrast sequences; impression was recorded; then post-contrast images were reviewed and any changes were recorded. RESULTS: A total of 246 patients (135 females; median age, 59 years) were included. ALT was histopathologically confirmed in 70/246 patients. In multivariable analysis, in addition to the lesion size, deep location, proximal lower limb lesions, demonstrating incomplete fat suppression, or increased architectural complexity were the independent predictive features of ALT; but not the contrast enhancement. Post-contrast MRI changed the impression in a total of 5 studies (3 for R1 and 4 for R2; 2 studies are common); all of them were incorrectly changed from Ls to ALTs. Overall, inter-reader kappa agreement was 0.42 (95% CI 0.39-0.56). Discordance between the two readers was statistically significant for both pathologically proven L (p < 0.001) and ALT (p = 0.003). CONCLUSION: Most qualitative MR imaging features can help distinguish ALTs from BLs. However, contrast enhancement may be limited and occasionally misleading. Substantial discordance on MRI readings exists between radiologists with a relatively high false positive and negative rates.
Asunto(s)
Lipoma/diagnóstico por imagen , Liposarcoma/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Medios de Contraste , Femenino , Humanos , Lipoma/patología , Liposarcoma/patología , Masculino , Persona de Mediana Edad , Reproducibilidad de los Resultados , Estudios RetrospectivosRESUMEN
Adipocytic tumors are rare in children and are mostly benign. Less than 25 cases of pediatric well-differentiated liposarcoma (WDLPS), atypical lipomatous tumors (ALT), and dedifferentiated liposarcoma (DDLPS) have been reported. Among them, only three cases were genetically analyzed. We describe the genetic features of a rapidly growing adipose tumor that occurred in the thigh of a 7-year-old girl. Histologically, it was composed of mature adipocytic cells with a few atypia. Molecular analysis showed high-level amplification of the 12q13-21 region including MDM2 among 64 amplified genes. MDM2 amplification is a diagnostic hallmark of ALT/WDLPS/DDLPS. In adult cases, it is typically located in ring or giant marker chromosomes. In the present case, extra-copies of MDM2 were located on double minute chromosomes (dmin). This raised the hypothesis of dmin being precursors of adult's rings and giant markers and may provide indications for a better understanding of the mechanisms of adipose tumor oncogenesis.
Asunto(s)
Amplificación de Genes , Liposarcoma/genética , Proteínas Proto-Oncogénicas c-mdm2/genética , Neoplasias de los Tejidos Blandos/genética , Adipocitos/metabolismo , Adipocitos/patología , Niño , Cromosomas Humanos Par 12/genética , Femenino , Humanos , Liposarcoma/patología , Proteínas Proto-Oncogénicas c-mdm2/metabolismo , Neoplasias de los Tejidos Blandos/patologíaRESUMEN
BACKGROUND: To evaluate the diagnostic value of MR imaging for the differentiation of lipomas and atypical lipomatous tumors (ALT) in comparison with histology and MDM2 amplification status. METHODS: Patients with well-differentiated lipomatous tumors (n = 113), of which 66 were diagnosed as lipoma (mean age 53 years (range, 13-82); 47% women) and 47 as atypical lipomatous tumor (ALT; mean age 60 years (range, 28-88); 64% women), were included into this study using histology and MDM2 amplification status by fluorescence in situ hybridization (FISH) as standard of reference. Preoperative MR images were retrospectively assessed by two radiologists for the following imaging features: maximum tumor diameter (mm) as well as the affected compartment (intramuscular, intermuscular or subcutaneous), septa (absent, thin (< 2 mm) or thick septa (> 2 mm) with nodular components); contrast enhancing areas within the lipomatous tumor (< 1/3 of the tumor volume, > 1/3 of the tumor volume); RESULTS: Of the 47 patients with ALT, 40 (85.1%) presented thick septa (> 2 mm) and this finding significantly increased the likelihood of ALT (OR 6.24, 95% CI 3.36-11.59; P < 0.001). The likelihood of ALT was increased if the tumor exceeded a maximum diameter of 130.0 mm (OR 2.74, 95% CI 1.82-4.11, P < 0.001). The presence of contrast enhancement in lipomatous tumors significantly increased the likelihood of ALT (Odds ratio (OR) 2.95, 95% confidence interval (CI) 2.01-4.31; P < 0.001). Of the lipomas, 21.1% were located subcutaneously, 63.6% intramuscularly and 15.2% intermuscularly. On the other hand, none of the ALTs were located subcutaneously, the majority was located intermuscularly (87.3%) and a small number of ALTs was located intramuscularly (12.7%). CONCLUSIONS: Our results suggest that using specific morphological MR imaging characteristics (maximum tumor diameter, thick septa and contrast enhancement) and the information on the localization of the lipomatous tumor, a high sensitivity and substantial specificity can be achieved for the diagnosis of lipomas and ALTs.
Asunto(s)
Amplificación de Genes , Lipoma/diagnóstico por imagen , Liposarcoma/diagnóstico por imagen , Proteínas Proto-Oncogénicas c-mdm2/genética , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Diagnóstico Diferencial , Femenino , Humanos , Hibridación Fluorescente in Situ , Lipoma/genética , Liposarcoma/genética , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Sensibilidad y Especificidad , Adulto JovenRESUMEN
Myxoid adipocytic tumors encompass a broad heterogeneous group of benign and malignant adipocytic tumors, which are typically myxoid (e.g. myxoid liposarcoma, lipoblastoma and lipoblastoma-like tumor of the vulva) or may occasionally appear predominantly myxoid (e.g. pleomorphic liposarcoma, atypical lipomatous tumor, dedifferentiated liposarcoma, chondroid lipoma, spindle cell/pleomorphic lipoma, atypical spindle cell lipomatous tumor and atypical pleomorphic lipomatous tumor). There have been significant advances in recent years in classification and understanding the pathogenesis of adipocytic tumors, based on the correlation of histologic, immunohistochemical, and cytogenetic/molecular findings. Despite these advances, the morphologic diagnosis and accurate classification of a myxoid adipocytic tumor can be challenging due to major morphologic overlap between myxoid adipocytic and non-adipocytic tumors. This article will provide a review on the currently known morphological, immunohistochemical and molecular features of myxoid adipocytic tumors and their differential diagnosis.
Asunto(s)
Neoplasias de Tejido Adiposo/diagnóstico , Neoplasias de Tejido Adiposo/patología , Neoplasias de los Tejidos Blandos/diagnóstico , Neoplasias de los Tejidos Blandos/patología , Diagnóstico Diferencial , HumanosRESUMEN
Liposarcoma is the single most common soft tissue sarcoma accounting for up to 35 % of sarcomas. It represents a histologically diverse group of soft tissue tumors that demonstrate a wide range of imaging appearances with varied behavior patterns. Correspondingly, more aggressive histological subtypes often require management that includes a combination of surgery, chemotherapy, and radiation therapy. Distinguishing among liposarcoma subtypes has important therapeutic and prognostic implications. In this manuscript, we review the liposarcoma subtypes and their histologic and MRI findings, prognostic implications, and differential diagnostic considerations.
Asunto(s)
Liposarcoma/diagnóstico , Liposarcoma/patología , Liposarcoma/terapia , Diagnóstico Diferencial , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética , PronósticoRESUMEN
BACKGROUND: The role of adjuvant radiotherapy (RT) in the management of atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WD-LPS) remains controversial. METHODS: Two hundred eighty-three patients with operable ALT/WD-LPS, no history of previous cancer, chemotherapy (CT) or RT, treated between 1984 and 2011 registered in the Conticabase database were included and described. Overall (OS), progression-free survival (PFS) and time to local relapse (TTLR) were evaluated from the time of first treatment. RESULTS: Three of 20 centers enrolled 58% of the patients. Median age at diagnosis was 61 (range 25-94) years, 147 patients (52%) were males, 222 (78%) patients had their primary tumor located in an extremity while 36 (13%) and 25 (9%) had tumors involving the girdle and the trunk wall, respectively. The median size of primary tumors was 17 cm (range 2-48 cm). Adjuvant RT was given to 132 patients (47%). Patients who received adjuvant RT had larger tumors (P = 0.005), involving more often the distal limbs (P < 0.001). Use of adjuvant RT varied across centers and along the study period. Other characteristics were balanced between the two groups. Median follow-up was 61.7 months. None of the patients developed metastasis during follow-up. The 5-year local relapse-free survival rates were 98.3% versus 80.3% with and without adjuvant RT, respectively (P < 0.001). Once stratified on time period (before/after 2003), adjuvant RT, tumor site and margin status (R0 versus other) were independently associated with TTLR. No OS difference was observed (P = 0.105). CONCLUSION: In this study, adjuvant RT following resection of ALT/WD-LPS was associated with a reduction of LR risk.
Asunto(s)
Liposarcoma/mortalidad , Liposarcoma/radioterapia , Adulto , Anciano , Anciano de 80 o más Años , Supervivencia sin Enfermedad , Femenino , Humanos , Liposarcoma/cirugía , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia/radioterapia , Radioterapia AdyuvanteRESUMEN
Segmentation and image intensity discretization impact on radiomics workflow. The aim of this study is to investigate the influence of interobserver segmentation variability and intensity discretization methods on the reproducibility of MRI-based radiomic features in lipoma and atypical lipomatous tumor (ALT). Thirty patients with lipoma or ALT were retrospectively included. Three readers independently performed manual contour-focused segmentation on T1-weighted and T2-weighted sequences, including the whole tumor volume. Additionally, a marginal erosion was applied to segmentations to evaluate its influence on feature reproducibility. After image pre-processing, with included intensity discretization employing both fixed bin number and width approaches, 1106 radiomic features were extracted from each sequence. Intraclass correlation coefficient (ICC) 95% confidence interval lower bound ≥ 0.75 defined feature stability. In contour-focused vs. margin shrinkage segmentation, the rates of stable features extracted from T1-weighted and T2-weighted images ranged from 92.68 to 95.21% vs. 90.69 to 95.66% after fixed bin number discretization and from 95.75 to 97.65% vs. 95.39 to 96.47% after fixed bin width discretization, respectively, with no difference between the two segmentation approaches (p ≥ 0.175). Higher stable feature rates and higher feature ICC values were found when implementing discretization with fixed bin width compared to fixed bin number, regardless of the segmentation approach (p < 0.001). In conclusion, MRI radiomic features of lipoma and ALT are reproducible regardless of the segmentation approach and intensity discretization method, although a certain degree of interobserver variability highlights the need for a preliminary reliability analysis in future studies.
Asunto(s)
Lipoma , Imagen por Resonancia Magnética , Variaciones Dependientes del Observador , Humanos , Lipoma/diagnóstico por imagen , Lipoma/patología , Imagen por Resonancia Magnética/métodos , Femenino , Masculino , Reproducibilidad de los Resultados , Persona de Mediana Edad , Estudios Retrospectivos , Anciano , Adulto , Procesamiento de Imagen Asistido por Computador/métodos , RadiómicaRESUMEN
Background/Aim: Lipomatous tumors, including lipomas, atypical lipomatous tumors (ALTs), myxoid liposarcomas (MLs), and dedifferentiated liposarcomas (DLs), are often diagnosed using magnetic resonance imaging (MRI). Differential diagnosis of lipomas and ALTs by MRI is often challenging. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) has recently been used for the diagnosis and evaluation of tumor staging and recurrence of soft tissue tumors. The maximum standardized uptake value (SUVmax) is positively associated with malignant grade in several cancers. This study aimed to evaluate SUVmax of 18F-FDG PET/CT in the differential diagnosis of lipomatous tumors. Patients and Methods: Patients who underwent 18F-FDG PET/CT for the diagnosis of lipomatous tumors between January 2013 and September 2021 were included in the study. Patients with lipomatous tumors, confirmed by pathological diagnosis or surgical specimens, were evaluated for lipomatous tumor SUVmax. Results: This study included 44 patients with lipomas (n=19), ALTs (n=12), MLs (n=9), and DLs (n=4). The mean SUVmax of lipomas, ALTs, MLs, and DLs was 0.99±1.41, 1.92±0.95, 5.21±4.94, and 9.29±1.43, respectively. Lipomas showed a significantly lower SUVmax than did ALTs, MLs, and DLs (p<0.05). ALTs demonstrated a significantly lower SUVmax than did MLs and DLs (p<0.05). No significant differences were observed between MLs and DLs. Conclusion: Lipomas or ALTs had a significantly lower SUVmax than lipomatous sarcomas. Lipomas had a significantly lower SUVmax than ALTs, aiding in their preoperative differentiation. 18F-FDG-PET/CT could serve as a potent tool for the differential diagnosis of lipomatous tumors.
RESUMEN
Background: Liposarcomas originating in the supraclavicular fossa are exceptionally rare, with only a few documented cases in the medical literature. Methods: We present a unique case involving a remarkably large indolent, atypical lipomatous tumor or well-differentiated liposarcoma situated in the right supraclavicular region, measuring 18 cm × 18 cm × 17 cm. To our knowledge, this represents the largest liposarcoma found in the supraclavicular fossa reported in the literature. This unique case highlights the effective management of a remarkably large supraclavicular liposarcoma through complete surgical resection. Result and Conclusion: Our findings further support the existing consensus that complete surgical removal is often curative for well-differentiated liposarcoma.