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1.
JCO Clin Cancer Inform ; 8: e2300234, 2024 Aug.
Article in English | MEDLINE | ID: mdl-39094063

ABSTRACT

PURPOSE: Cure models are a useful alternative to Cox proportional hazards models in oncology studies when there is a subpopulation of patients who will not experience the event of interest. Although software is available to fit cure models, there are limited tools to evaluate, report, and visualize model results. This article introduces the cureit R package, an end-to-end pipeline for building mixture cure models, and demonstrates its use in a data set of patients with primary extremity and truncal liposarcoma. METHODS: To assess associations between liposarcoma histologic subtypes and disease-specific death (DSD) in patients treated at Memorial Sloan Kettering Cancer Center between July 1982 and September 2017, mixture cure models were fit and evaluated using the cureit package. Liposarcoma histologic subtypes were defined as well-differentiated, dedifferentiated, myxoid, round cell, and pleomorphic. RESULTS: All other analyzed liposarcoma histologic subtypes were significantly associated with higher DSD in cure models compared with well-differentiated. In multivariable models, myxoid (odds ratio [OR], 6.25 [95% CI, 1.32 to 29.6]) and round cell (OR, 16.2 [95% CI, 2.80 to 93.2]) liposarcoma had higher incidences of DSD compared with well-differentiated patients. By contrast, dedifferentiated liposarcoma was associated with the latency of DSD (hazard ratio, 10.6 [95% CI, 1.48 to 75.9]). Pleomorphic liposarcomas had significantly higher risk in both incidence and the latency of DSD (P < .0001). Brier scores indicated comparable predictive accuracy between cure and Cox models. CONCLUSION: We developed the cureit pipeline to fit and evaluate mixture cure models and demonstrated its clinical utility in the liposarcoma disease setting, shedding insights on the subtype-specific associations with incidence and/or latency.


Subject(s)
Liposarcoma , Humans , Liposarcoma/pathology , Liposarcoma/therapy , Male , Female , Middle Aged , Aged , Proportional Hazards Models , Software , Adult
2.
Medicine (Baltimore) ; 103(33): e39282, 2024 Aug 16.
Article in English | MEDLINE | ID: mdl-39151522

ABSTRACT

RATIONALE: Mucinous liposarcoma myxoid liposarcoma is a malignant mucoid soft tissue tumor derived from undifferentiated stromal cells in perivascular, subbody cavity and intermuscular space, and composed of cells at different stages of differentiation from preadipocytes to mature cells. In rare cases, it may change from lipoma malignancy. The main manifestations is painless mass, relatively slow growth, the course can last decades, the prevalence of liposarcoma in the population is 14% to 18%, mainly in adults, male prevalence is higher than women, but not significant. The main good hair part is the thigh, have mucinous sex, high differentiation type, dedifferentiation type, polymorphic type. Clinical diagnosis is difficult, and there are no obvious symptoms in the early stage, so the diagnosis should be combined with B ultrasound, MRI, CT, and other auxiliary examinations. The gold standard is pathological examination. In December 2023, our department admitted a patient with a mucinous abdominal mass. The report is as follows. PATIENT CONCERNS: Does liposarcoma metastasize? Is any chemotherapy required after surgery? Will it ever relapse in the future? What is the survival period after surgery? DIAGNOSIS: Mucinous liposarcoma. INTERVENTIONS: Surgical resection of the sarcoma. RESULTS: The nodule sample was 33 * 28 * 13 cm, with complete capsule, gray and yellow sections, fine texture, soft, gray, red, grayish, and yellow mucoid nodules in some areas, and the maximum diameter of the nodules was 21cm. Immunohistochemistry was: CD34 (+), CDK 4 (+), CK (-), Desmin (weak +), Ki67 (index 5%), MDM 2 (-), p16 (weak +), S-100P (+), Vimentin (+), BCL-2 (+). He was also sent to the Department of Pathology of Peking Union Medical College Hospital for consultation with Professor Lu Zhaohui, whose consultation opinion was in line with myxoliposarcoma. CONCLUSION: Retroperitoneal liposarcoma is a common retroperitoneal tumor, but it is relatively rare in clinical practice; the overall morbidity is low, mainly manifested as abdominal pain and abdominal distension, abdominal distension, and a long course of disease; it is not sensitive to radiotherapy and chemotherapy, and should be closely follow up by CT examination to understand the recurrence and metastasis.


Subject(s)
Liposarcoma, Myxoid , Humans , Male , Liposarcoma, Myxoid/pathology , Liposarcoma, Myxoid/diagnosis , Liposarcoma, Myxoid/surgery , Liposarcoma, Myxoid/diagnostic imaging , Middle Aged , Abdominal Cavity/pathology , Abdominal Cavity/diagnostic imaging , Liposarcoma/pathology , Liposarcoma/diagnosis , Liposarcoma/surgery
3.
J Pak Med Assoc ; 74(8): 1538-1540, 2024 Aug.
Article in English | MEDLINE | ID: mdl-39160731

ABSTRACT

Spermatic cord malignancies are a scarce modality and liposarcoma of spermatic cord is even a rarer condition encountered. Liposarcoma is usually a slowly progressive, non-tender, well circumscribed mass of variable shapes owing to conformity to fascial compartments. We are reporting a case of 65-year-old male, with a two-month history of initially tender and later non-tender mass in the scrotum, above the testis. Ultrasonography showed a right mid inguinal mass measuring 6x3x3 cm at the superior pole of the right testis and small fluid around the right testis. Fine needle aspiration cytology (FNAC) of the right inguinal mass revealed a spindle cell neoplasm. The patient underwent right inguinal radical orchiectomy with local wide excision of the sarcoma of the spermatic cord origin. Final histopathology confirmed dedifferentiated liposarcoma. No adjuvant treatment was offered and the patient was put on surveillance. Follow-up of more than 10 months has not revealed any local recurrence, regional or non-regional lymph nodes, or systemic metastasis.


Subject(s)
Genital Neoplasms, Male , Liposarcoma , Orchiectomy , Spermatic Cord , Humans , Male , Liposarcoma/surgery , Liposarcoma/pathology , Liposarcoma/diagnostic imaging , Spermatic Cord/pathology , Spermatic Cord/diagnostic imaging , Spermatic Cord/surgery , Aged , Genital Neoplasms, Male/pathology , Genital Neoplasms, Male/surgery , Genital Neoplasms, Male/diagnostic imaging , Orchiectomy/methods , Biopsy, Fine-Needle
4.
Am J Dermatopathol ; 46(8): 509-511, 2024 Aug 01.
Article in English | MEDLINE | ID: mdl-39133219

ABSTRACT

ABSTRACT: Among liposarcomas, well-differentiated liposarcoma and dedifferentiated liposarcoma are the most common. The majority of these tumors are found in deep retroperitoneum or extremities. When found outside the retroperitoneum, these adipose-derived tumors are known as atypical lipomatous tumors (ALT). Superficial ALT are particularly rare; thus, little is known about their clinical presentation, genomic status, and management. Here, we present the case of a 54-year-old man with an intermittently bothersome, slowly growing mass on his left upper back for over 2 years, which was incidentally diagnosed as ALT. This patient's ALT, however, showed a profound degree of pleomorphism with MDM2 and control centromere 12 (CEP12) coamplification and negative CD34 and S100 and RB1 expression, unlike most other ALT described in the literature. This case report details the diagnostic workup and histopathological findings for adipose tumors and summarizes the different subtypes, including atypical spindle cell/pleomorphic lipomatous tumor, pleomorphic liposarcoma, and spindle cell/pleomorphic lipoma, with brief discussion on management.


Subject(s)
Liposarcoma , Humans , Male , Middle Aged , Liposarcoma/pathology , Liposarcoma/genetics , Lipoma/pathology , Biomarkers, Tumor/analysis , Soft Tissue Neoplasms/pathology , Soft Tissue Neoplasms/genetics , Proto-Oncogene Proteins c-mdm2/genetics , Immunohistochemistry
5.
Clin Nucl Med ; 49(9): 873-876, 2024 Sep 01.
Article in English | MEDLINE | ID: mdl-39102813

ABSTRACT

ABSTRACT: Atypical spindle cell/pleomorphic lipomatous tumor is categorized as a benign lipomatous tumor, but various MRI findings pose accurate diagnostic challenges. In our case, both MRI and PET/CT scans indicated the possibility of atypical lipomatous tumor/well-differentiated liposarcoma or dedifferentiated liposarcoma. Needle biopsy suggested benign to low-grade malignancy; hence, we opted for the wide resection. The final diagnosis of atypical spindle cell/pleomorphic lipomatous tumor was confirmed through histopathology analysis, including immunohistochemistry and fluorescence in situ hybridization. Since achieving an accurate diagnosis solely through imaging can be challenging, histopathology remains essential.


Subject(s)
Lipoma , Magnetic Resonance Imaging , Thigh , Humans , Thigh/diagnostic imaging , Thigh/pathology , Diagnosis, Differential , Lipoma/diagnostic imaging , Lipoma/pathology , Positron Emission Tomography Computed Tomography , Male , Liposarcoma/diagnostic imaging , Liposarcoma/pathology , Middle Aged , Tomography, X-Ray Computed
6.
J Cancer Res Clin Oncol ; 150(7): 343, 2024 Jul 09.
Article in English | MEDLINE | ID: mdl-38981922

ABSTRACT

PURPOSE: Radical resection of retroperitoneal liposarcoma (RLPS) may necessitate vascular resection and reconstruction. The study was conducted to assess surgical outcomes of surgery for RLPS with major vascular involvement. METHODS: Patients with RLPS who underwent surgical resection at the Sarcoma Center of Peking University Cancer Hospital between April 2011 and December 2022 were identified from a prospectively maintained database. Patients were classified into two groups: vascular resection and non-vascular resection groups. A propensity score matching analysis was performed to eliminate baseline differences between the groups. Surgical details and postoperative outcomes were analyzed. Furthermore, prognostic factors for local recurrence-free survival (LRFS) and overall survival (OS) were assessed. RESULTS: Overall, 199 patients were identified and the median follow-up period was 48 (interquartile range [IQR] 45-69) months. Vascular resection was performed in 42 (21%) patients, 25 of whom had vascular infiltration. A total of 39 patients had vascular replacement and 3 patients underwent partial resection (side-wall resection). Vascular resection was burdened by higher rates of major morbidity (38% vs. 14%, p < 0.001) and 30-day mortality (7.1% vs. 1.3%, p = 0.005). After propensity-matched analysis, patients who underwent vascular resection had 5-year LRFS and OS rates comparable to those without vascular involvement. Major vascular resection was not an independent risk factor for LRFS or OS. CONCLUSIONS: Although accompanied by increased risks of major morbidity and mortality, the major vascular resection enabled radical resection in patients with advanced RLPS, affording comparable 5-year LRFS and OS rates compared to those who did not.


Subject(s)
Liposarcoma , Propensity Score , Retroperitoneal Neoplasms , Humans , Retroperitoneal Neoplasms/surgery , Retroperitoneal Neoplasms/pathology , Retroperitoneal Neoplasms/mortality , Male , Female , Liposarcoma/surgery , Liposarcoma/pathology , Liposarcoma/mortality , Middle Aged , Aged , Vascular Surgical Procedures/methods , Adult , Retrospective Studies , Treatment Outcome , Prognosis , Hospitals, High-Volume
8.
BMC Cancer ; 24(1): 855, 2024 Jul 18.
Article in English | MEDLINE | ID: mdl-39026264

ABSTRACT

BACKGROUND: Retroperitoneal liposarcoma (RLPS) constitutes the majority of retroperitoneal sarcomas. While surgical resection remains the sole curative approach, determining the optimal surgical strategy for RLPS remains elusive. This study addresses the ongoing debate surrounding the optimal surgical strategy for RLPS. METHODS: We recruited 77 patients with RLPS who underwent aggressive surgical policies. Patients were categorized into three surgical subtypes: suprapancreatic RLPS, pancreatic RLPS, and subpancreatic RLPS. Our standardized surgical strategy involved resecting macroscopically uninvolved adjacent organs according to surgical subtypes. We collected clinical, pathological and prognostic data for analyses. RESULTS: The median follow-up was 45.5 months. Overall survival (OS) and recurrence-free survival (RFS) were significantly correlated with multifocal RLPS, pathological subtype, recurrent RLPS and histological grade (P for OS = 0.011, 0.004, 0.010, and < 0.001, P for RFS = 0.004, 0.001, < 0.001, and < 0.001, respectively). The 5-Year Estimate OS of well-differentiated liposarcoma (WDLPS), G1 RLPS, de novo RLPS and unifocal RLPS were 100%, 89.4%, 75.3% and 69.1%, respectively. The distant metastasis rate was 1.4%. The morbidity rates (≥ grade III) for suprapancreatic, pancreatic, and subpancreatic RLPS were 26.7%, 15.6%, and 13.3%, respectively. The perioperative mortality rate is 2.6%. CONCLUSIONS: Standardized aggressive surgical policies demonstrated prognostic benefits for RLPS, particularly for G1 RLPS, WDLPS, unifocal RLPS, and de novo RLPS. This approach effectively balanced considerations of adequate exposure, surgical safety, and thorough removal of all fat tissue. G1 RLPS, WDLPS, unifocal RLPS, and de novo RLPS could be potential indications for aggressive surgical policies.


Subject(s)
Liposarcoma , Retroperitoneal Neoplasms , Humans , Liposarcoma/surgery , Liposarcoma/pathology , Liposarcoma/mortality , Retroperitoneal Neoplasms/surgery , Retroperitoneal Neoplasms/pathology , Retroperitoneal Neoplasms/mortality , Male , Female , Middle Aged , Aged , Adult , Prognosis , Follow-Up Studies , Neoplasm Recurrence, Local/surgery , Retrospective Studies , Aged, 80 and over
9.
Int J Mol Sci ; 25(14)2024 Jul 16.
Article in English | MEDLINE | ID: mdl-39063036

ABSTRACT

BACKGROUND: As a common soft tissue sarcoma, liposarcoma (LPS) is a heterogeneous malignant tumor derived from adipose tissue. Due to the high risk of metastasis and recurrence, the prognosis of LPS remains unfavorable. To improve clinical treatment, a robust risk prediction model is essential to evaluate the prognosis of LPS patients. METHODS: By comprehensive analysis of data derived from GEO datasets, differentially expressed genes (DEGs) were obtained. Univariate and Lasso Cox regressions were subsequently employed to reveal distant recurrence-free survival (DRFS)-associated DEGs and develop a prognostic gene signature, which was assessed by Kaplan-Meier survival and ROC curve. GSEA and immune infiltration analyses were conducted to illuminate molecular mechanisms and immune correlations of this model in LPS progression. Furthermore, a correlation analysis was involved to decipher the therapeutic significance of this model for LPS. RESULTS: A six-gene signature was developed to predict DRFS of LPS patients and showed higher precision performance in more aggressive LPS subtypes. Then, a nomogram was further established for clinical application based on this risk model. Via GSEA, the high-risk group was significantly enriched in cell cycle-related pathways. In the LPS microenvironment, neutrophils, memory B cells and resting mast cells exhibited significant differences in cell abundance between high-risk and low-risk patients. Moreover, this model was significantly correlated with therapeutic targets. CONCLUSION: A prognostic six-gene signature was developed and significantly associated with cell cycle pathways and therapeutic target genes, which could provide new insights into risk assessment of LPS progression and therapeutic strategies for LPS patients to improve their prognosis.


Subject(s)
Gene Expression Regulation, Neoplastic , Liposarcoma , Tumor Microenvironment , Humans , Liposarcoma/genetics , Liposarcoma/immunology , Liposarcoma/pathology , Liposarcoma/mortality , Prognosis , Tumor Microenvironment/immunology , Tumor Microenvironment/genetics , Transcriptome , Gene Expression Profiling , Biomarkers, Tumor/genetics , Nomograms , Male , Female , Kaplan-Meier Estimate , ROC Curve
10.
Ultrasound Med Biol ; 50(9): 1472-1478, 2024 Sep.
Article in English | MEDLINE | ID: mdl-38955624

ABSTRACT

OBJECTIVE: Lipomatous soft tissue tumors (STT), ranging from benign lipomas to malignant liposarcomas, require accurate differentiation for timely treatment. Complementary to MRI, Contrast-enhanced ultrasound (CEUS) is emerging as a promising tool, providing insight into tumor microperfusion in real-time. This study aims to explore the potential of preoperative CEUS in differentiating benign lipomatous tumors from malignant liposarcoma subtypes. METHODS: Eighty-seven patients with lipomatous STT scheduled for surgery were enrolled. Clinical and MRI assessments were conducted to obtain general tumor characteristics. CEUS was used for a standardized tumor perfusion evaluation. Perfusion analysis included peak enhancement, rise time, wash-in perfusion index, and wash-out rate, reflecting the perfusion kinetics. Histopathological results were obtained for every STT and compared to perfusion characteristics. RESULTS: In total, 48 lipoma, 23 ALT and 11 liposarcoma were identified. Significant differences in tumor microperfusion were demonstrated, with higher perfusion levels indicating higher malignancy (Peak enhancement [a.u.] of Lipoma: 145 ± 238; ALT: 268 ± 368; Liposarcoma: 3256 ± 4333; p (ALT vs. Liposarcoma) < 0.001). A perfusion-based identification of a benign lipoma or ALT versus sarcoma resulted in a positive predictive value of 93%. Patient-related factors (age, gender, BMI, ASA score, smoking status) had no significant impact on the CEUS-based perfusion parameters. CONCLUSION: Our study suggests CEUS as a capable non-invasive tool for improving preoperative assessment of lipomatous STT. It can assist in the distinction between benign and malignant STT, accelerating treatment decisions and enhancing patient outcomes. Significant correlations between CEUS-derived parameters and malignancy highlight its risk assessment potential.


Subject(s)
Contrast Media , Lipoma , Liposarcoma , Ultrasonography , Humans , Liposarcoma/diagnostic imaging , Female , Male , Middle Aged , Lipoma/diagnostic imaging , Aged , Ultrasonography/methods , Adult , Diagnosis, Differential , Image Enhancement/methods , Aged, 80 and over , Soft Tissue Neoplasms/diagnostic imaging , Prospective Studies
11.
Pathol Res Pract ; 260: 155376, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38875758

ABSTRACT

Lipogenic and fibrous tumors are thought to originate from CD34-positive stromal fibroblastic/fibrocystic cells. Well-differentiated lipogenic tumors typically express CD34, whereas dedifferentiated liposarcoma (DDLPS) often loses it. We conducted survival analyses involving 59 patients with DDLPS. Males comprised 53% of the cohort, and the median age at the time of wide resection of primary DDLPS was 60 years. Loss of CD34 expression was defined as when ≥50% of the dedifferentiated area was immunohistochemically negative for CD34. As a result, 39 of the 59 patients showed loss of CD34 expression during the initial operation for DDLPS. In the univariate analyses, the tumor site in the retroperitoneum/abdominal cavity and loss of CD34 expression were significantly associated with poor overall survival. In the multivariate analyses, loss of CD34 expression (HR = 2.26; 95% CI = 1.02-5.02; p = 0.04) and the tumor site in the retroperitoneum/abdominal cavity (HR = 3.11; 95% CI = 1.09-8.86; p = 0.03) were retained as independent prognostic factors. Six CD34-positive cases lost CD34 expression when they developed metastasis and/or local recurrence, suggesting that the loss was associated with the later stage of the tumor. Therefore, an association existed between the loss of CD34 expression and clinicopathological behaviors such as poorer prognoses and recurrence.


Subject(s)
Antigens, CD34 , Biomarkers, Tumor , Liposarcoma , Humans , Male , Liposarcoma/pathology , Liposarcoma/metabolism , Middle Aged , Female , Antigens, CD34/metabolism , Aged , Adult , Biomarkers, Tumor/analysis , Biomarkers, Tumor/metabolism , Prognosis , Aged, 80 and over , Immunohistochemistry , Neoplasm Recurrence, Local/pathology , Neoplasm Recurrence, Local/metabolism , Retroperitoneal Neoplasms/pathology , Retroperitoneal Neoplasms/metabolism
12.
Rev. esp. patol ; 57(2): 128-132, Abr-Jun, 2024. ilus
Article in English | IBECS | ID: ibc-232418

ABSTRACT

Primary hepatic liposarcoma is an extremely rare malignant tumour derived from adipocytes and is part of the group of mesenchymal tumours. We present the case of a 43-year-old Hispanic male patient with a pleomorphic hepatic liposarcoma and absence of MDM2 gene amplification. Two years and six months after surgery, the patient is asymptomatic. The present case is the first report of this entity with positive immunohistochemical testing for p16, p53, S100, vimentin and absence of MDM2 gene amplification. (AU)


El liposarcoma hepático primario es un tumor maligno extremadamente raro, derivado de adipocitos, y forma parte del grupo de tumores mesenquimales. Presentamos el caso de un paciente masculino de 43 años con diagnóstico de liposarcoma hepático pleomorfo con ausencia de amplificación del gen MDM2. Dos años y 6 meses después de la cirugía el paciente se encuentra asintomático. El presente caso es el primer informe de esta entidad con estudio inmunohistoquímico positivo para p16, p53, S100, vimentina y ausencia de amplificación del gen MDM2. (AU)


Subject(s)
Humans , Male , Adult , Liposarcoma , Neoplasms , Adipocytes , Mesenchymal Stem Cells , Vimentin
13.
Respirar (Ciudad Autón. B. Aires) ; 16(2): 183-192, Junio 2024.
Article in Spanish | LILACS, UNISALUD, BINACIS | ID: biblio-1556174

ABSTRACT

Se presenta a un paciente con liposarcoma mediastinal gigante con dolor torácico, disnea, cuyos estudios por imágenes revelaban la presencia de una gran tumoración de 42 cm en su diámetro mayor que abarcaba todo el mediastino, comprometía ambas cavidades torácicas, rechazaba los pulmones, corazón y grandes vasos. La biopsia con aguja cortante bajo guía ecográfica fue informada como liposarcoma. El paciente tuvo resección completa del tumor mediante la incisión Clamshell. En el post operatorio inmediato, presentó shock circulatorio más disfunción multiorgánica (DOMS): plaquetopenia, insuficiencia renal aguda con necesidad de soporte dialítico, injuria hepática. El soporte y monitoreo especializado en la Unidad de Cuidados Intensivos (UCI) permitió mejoría clínica y buena evolución. Salió de alta en buenas condiciones.


We present a patient with giant mediastinal liposarcoma with chest pain, dyspnea, whose imaging studies revealed the presence of a large tumor measuring 42 cm in its greatest diameter that covered the entire mediastinum, involved both thoracic cavities, rejected the lungs, heart and big glasses. The sharp needle biopsy under ultrasound guidance was reported as liposarcoma. The patient had complete resection of the tumor through the Clamshell incision. In the immediate postoperative period, he presented circulatory shock plus multiple organ dysfunction (DOMS): plateletopenia, acute renal failure with the need for dialytic support, liver injury. Specialized support and monitoring in the Intensive Care Unit (ICU) allowed clinical improvement and good evolution. He was discharged in good condition.


Subject(s)
Humans , Male , Adult , Thoracotomy , Liposarcoma/surgery , Mediastinal Neoplasms/diagnosis , Shock , Chest Pain , Tomography , Cough , Critical Care , Drug Therapy , Dyspnea , Image-Guided Biopsy , Multiple Organ Failure/surgery
14.
ESMO Open ; 9(7): 103487, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38943735

ABSTRACT

BACKGROUND: Locally advanced (unresectable) or metastatic dedifferentiated liposarcoma (DDLPS) is a common presentation of liposarcoma. Despite established diagnostic and treatment guidelines for DDLPS, critical clinical gaps remain driven by diagnostic challenges, symptom burden and the lack of targeted, safe and effective treatments. The objective of this study was to gather expert opinions from Europe and the United States on the management, unmet needs and expectations for clinical trial design as well as the value of progression-free survival (PFS) in this disease. Other aims included raising awareness and educate key stakeholders across healthcare systems. MATERIALS AND METHODS: An international panel of 12 sarcoma key opinion leaders (KOLs) was recruited. The study consisted of two rounds of surveys with pre-defined statements. Experts scored each statement on a 9-point Likert scale. Consensus agreement was defined as ≥75% of experts scoring a statement with ≥7. Revised statements were discussed in a consensus meeting. RESULTS: Consensus was reached on 43 of 55 pre-defined statements across disease burden, treatment paradigm, unmet needs, value of PFS and its association with overall survival (OS), and cross-over trial design. Twelve statements were deprioritised or merged with other statements. There were no statements where experts disagreed. CONCLUSION: This study constitutes the first international Delphi panel on DDLPS. It aimed to explore KOL perception of the disease burden and unmet need in DDLPS, the value of PFS, and its potential translation to OS benefit, as well as the relevance of a cross-over trial design for DDLPS therapies. Results indicate an alignment across Europe and the United States regarding DDLPS management, unmet needs, and expectations for clinical trials. Raising awareness of critical clinical gaps in relation to DDLPS can contribute to improving patient outcomes and supporting the development of innovative treatments.


Subject(s)
Consensus , Delphi Technique , Liposarcoma , Progression-Free Survival , Humans , Liposarcoma/therapy , Liposarcoma/mortality , Liposarcoma/pathology , Clinical Trials as Topic , Europe , Research Design
15.
Clin Nucl Med ; 49(8): e412-e414, 2024 Aug 01.
Article in English | MEDLINE | ID: mdl-38861420

ABSTRACT

ABSTRACT: A 58-year-old man with resected liposarcoma exhibited persistent irregular interlobular septal thickening of lung on CT concerning lymphangitic metastasis. FDG PET/CT was positive in these foci. There was a narrowing of the pulmonary veins, which had not been seen on the imaging conducted prior to radiofrequency ablation therapy for atrial fibrillation. Lung perfusion scanning demonstrated more extensive hypoperfusion. This case highlights the diagnostic pitfalls of interstitial thickening of the lung in cancer patients. Meticulous history review and nuclear imaging helped distinguish the postablation pulmonary venous stenosis from metastatic spread to the lung interstitium.


Subject(s)
Liposarcoma , Stenosis, Pulmonary Vein , Humans , Male , Middle Aged , Liposarcoma/diagnostic imaging , Diagnosis, Differential , Stenosis, Pulmonary Vein/diagnostic imaging , Lung Neoplasms/diagnostic imaging , Positron Emission Tomography Computed Tomography , Radiofrequency Ablation , Pulmonary Veins/diagnostic imaging
16.
Sci Rep ; 14(1): 14087, 2024 06 18.
Article in English | MEDLINE | ID: mdl-38890407

ABSTRACT

Canine liposarcoma is an uncommon tumor that shares morphological similarities with its human counterpart. In dogs, the genetic features of this tumor are unknown and, based on immunohistochemical studies, amplification of the gene MDM2 and the mutation of TP53 are suspected. In this study 51 cases of primary liposarcomas were immunohistochemically stained for MDM2 and p53 and subjected to fluorescent in situ hybridization and next-generation sequencing to detect MDM2 amplification and TP53 mutations, respectively. MDM2 and p53 were expressed in 21 and 6 cases, respectively. MDM2 amplification and TP53 mutations were identified in 10 and 15 cases, respectively. Statistical analysis revealed an association of the myxoid subtype and the mitotic count with p53 expression and TP53 mutation. No association was found between MDM2 amplification and MDM2 expression or tumor subtype. These results suggest that despite morphological similarities, canine liposarcoma differs from its human counterpart, for which MDM2 amplification is diagnostic for well differentiated and de-differentiated variants, and TP53 mutations are more common in pleomorphic liposarcoma rather than the myxoid one as occur in our cases. Furthermore, canine myxoid liposarcoma likely represents a distinct disease rather than a mere morphological variant.


Subject(s)
Dog Diseases , Liposarcoma , Proto-Oncogene Proteins c-mdm2 , Tumor Suppressor Protein p53 , Proto-Oncogene Proteins c-mdm2/genetics , Proto-Oncogene Proteins c-mdm2/metabolism , Dogs , Animals , Liposarcoma/genetics , Liposarcoma/veterinary , Liposarcoma/pathology , Liposarcoma/metabolism , Tumor Suppressor Protein p53/genetics , Tumor Suppressor Protein p53/metabolism , Dog Diseases/genetics , Dog Diseases/pathology , Mutation , Female , Male , In Situ Hybridization, Fluorescence , High-Throughput Nucleotide Sequencing , Gene Amplification , Immunohistochemistry
17.
Korean J Gastroenterol ; 83(6): 243-246, 2024 Jun 25.
Article in English | MEDLINE | ID: mdl-38918037

ABSTRACT

This case report presents the successful endoscopic submucosal dissection (ESD) of a well-differentiated esophageal liposarcoma in a 51-year-old male with persistent dysphagia. The cause was initially diagnosed as a 10 cm pedunculated lesion extending from the upper esophageal sphincter to the mid-esophagus. An ESD was chosen over traditional surgery because it is less invasive. The procedure involved a precise submucosal injection and excision with special techniques to manage bleeding from a central vessel. Despite the extraction challenges owing to the size of the lesion, it was successfully removed orally. A histopathological examination of the 8.3×4.2×2.3 cm specimen revealed the characteristic features of a well-differentiated liposarcoma, including MDM2 and CDK4 positivity. The follow-up revealed no recurrence, and active surveillance has been performed since. This report highlights the versatility of ESD in treating significant esophageal tumors and provides evidence for its efficacy as a minimally invasive alternative.


Subject(s)
Endoscopic Mucosal Resection , Esophageal Neoplasms , Liposarcoma , Humans , Male , Esophageal Neoplasms/surgery , Esophageal Neoplasms/pathology , Esophageal Neoplasms/diagnosis , Middle Aged , Liposarcoma/surgery , Liposarcoma/pathology , Liposarcoma/diagnosis , Tomography, X-Ray Computed , Cyclin-Dependent Kinase 4/metabolism , Proto-Oncogene Proteins c-mdm2/metabolism , Esophagoscopy
18.
Medicina (Kaunas) ; 60(6)2024 Jun 07.
Article in English | MEDLINE | ID: mdl-38929567

ABSTRACT

Liposarcomas are some of the most challenging soft tissue tumors and are subclassified into multiple subtypes with special histologic and molecular features. The peculiarities of each histopathological subtype influence the clinical behavior, management, and treatment of these neoplasms. For instance, well-differentiated liposarcomas are common soft tissue malignancies and usually display a favorable outcome. On the other hand, pleomorphic liposarcoma is the rarest, yet the most aggressive subtype of liposarcoma. This histopathological diagnosis may be challenging due to the scarce available data and because pleomorphic liposarcomas can mimic other pleomorphic sarcomas or other neoplasms of dissimilar differentiation. Nevertheless, the correct diagnosis of pleomorphic liposarcoma is of utmost importance as such patients are prone to develop local recurrences and metastases. Treatment usually consists of surgical excision along with radiotherapy and follow-up of the patients. Therefore, this review aims to assess the complex clinical, histological, and immunohistochemical features of liposarcomas in order to establish how these characteristics influence the management and prognosis of the patients, emphasizing the particularities of pleomorphic liposarcoma.


Subject(s)
Immunohistochemistry , Liposarcoma , Humans , Liposarcoma/diagnosis , Liposarcoma/therapy , Immunohistochemistry/methods , Biomarkers, Tumor/analysis , Soft Tissue Neoplasms/therapy , Soft Tissue Neoplasms/pathology , Soft Tissue Neoplasms/diagnosis , Prognosis
19.
Cancer Med ; 13(11): e7324, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38847519

ABSTRACT

OBJECTIVE: We have developed explainable machine learning models to predict the overall survival (OS) of retroperitoneal liposarcoma (RLPS) patients. This approach aims to enhance the explainability and transparency of our modeling results. METHODS: We collected clinicopathological information of RLPS patients from The Surveillance, Epidemiology, and End Results (SEER) database and allocated them into training and validation sets with a 7:3 ratio. Simultaneously, we obtained an external validation cohort from The First Affiliated Hospital of Naval Medical University (Shanghai, China). We performed LASSO regression and multivariate Cox proportional hazards analysis to identify relevant risk factors, which were then combined to develop six machine learning (ML) models: Cox proportional hazards model (Coxph), random survival forest (RSF), ranger, gradient boosting with component-wise linear models (GBM), decision trees, and boosting trees. The predictive performance of these ML models was evaluated using the concordance index (C-index), the integrated cumulative/dynamic area under the curve (AUC), and the integrated Brier score, as well as the Cox-Snell residual plot. We also used time-dependent variable importance, analysis of partial dependence survival plots, and the generation of aggregated survival SHapley Additive exPlanations (SurvSHAP) plots to provide a global explanation of the optimal model. Additionally, SurvSHAP (t) and survival local interpretable model-agnostic explanations (SurvLIME) plots were used to provide a local explanation of the optimal model. RESULTS: The final ML models are consisted of six factors: patient's age, gender, marital status, surgical history, as well as tumor's histopathological classification, histological grade, and SEER stage. Our prognostic model exhibits significant discriminative ability, particularly with the ranger model performing optimally. In the training set, validation set, and external validation set, the AUC for 1, 3, and 5 year OS are all above 0.83, and the integrated Brier scores are consistently below 0.15. The explainability analysis of the ranger model also indicates that histological grade, histopathological classification, and age are the most influential factors in predicting OS. CONCLUSIONS: The ranger ML prognostic model exhibits optimal performance and can be utilized to predict the OS of RLPS patients, offering valuable and crucial references for clinical physicians to make informed decisions in advance.


Subject(s)
Liposarcoma , Machine Learning , Retroperitoneal Neoplasms , SEER Program , Humans , Retroperitoneal Neoplasms/mortality , Retroperitoneal Neoplasms/pathology , Male , Female , Liposarcoma/mortality , Liposarcoma/pathology , Middle Aged , China/epidemiology , Aged , Risk Factors , Proportional Hazards Models , Prognosis , Adult
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