Primary intrathoracic liposarcoma: a clinical analysis of 31 cases.
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ABSTRACT:
SUBMITTER: Fu Z
PROVIDER: S-EPMC6444813 | biostudies-literature | 2019 Apr
REPOSITORIES: biostudies-literature
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Primary intrathoracic liposarcoma: a clinical analysis of 31 cases.
Fu Zhixue Z Yang Kun K Yang Xu X Chen Shulan S Wang Wenqing W Chen Dongfu D Zhao Jun J Li Zhishan Z Feng Qinfu Q Zhou Zongmei Z Wang Luhua L Gao Shugeng S Liang Jun J
Cancer communications (London, England) 20190402 1
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Project description:IntroductionMerkel cell carcinoma (MCC) is a rare, aggressive cutaneous neuroendocrine cancer that typically arises in sun-exposed areas of the skin, especially in the elderly. Significant advances have recently been made regarding skin cancers, but data on cases of MCC are rather limited as these patients are frequently grouped together with other non-melanoma skin cancers (NMSC). Here, we performed an analysis of the clinical profile of patients with MCC in Poland to identify major factors influencing the prognosis.MethodsApproximately 13,000 pathology and medical records were examined to identify patients with MCC diagnosed between 2010 and 2019. The management and outcomes of patients with histologically confirmed MCC were retrospectively evaluated.ResultsThirty-one patients diagnosed with MCC were identified. The tumor occurred predominantly in women (61.3%) and in the elderly (mean 75.6 years). Twenty-nine patients had locoregional MCC and two had metastatic MCC at the time of diagnosis. Patients in stage I disease had excellent prognosis. In stages II and III, respectively 22.2% and 50.0% of patients developed metastases. Among patients who received chemotherapy with cisplatin and etoposide, 17% achieved partial remission with progression-free survival (PFS) of 8.0 months, and a further 50% achieved stable disease with PFS of 4.0, 4.5, and 4.5 months respectively. In 6 (19.4%) patients MCC coexisted with chronic lymphocytic leukemia (CLL). In all six cases CLL preceded MCC development.ConclusionsFemale gender, tumor-free resection margins, and local disease were found to be independent prognostic factors in MCC progression. Patients with hematological malignancies, immunosuppression, and those with immune deficiencies should be closely followed up as they are predisposed to develop MCC.
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Project description:BackgroundLiposarcoma (LPS) is the second most common type of soft tissue sarcoma, accounting for approximately 15-20% of all the sarcomas. Primary intrathoracic LPS, however, is quite rare. LPS is a malignant mesenchymal tumor, comprised of lipogenic tissue with varying degrees of atypia. It can be subclassified into well-differentiated LPS (WDLPS), myxoid LPS (MLPS)/round cell LPS, pleomorphic LPS (PLPs), and dedifferentiated LPS (DDLPS), based on the histology.Case presentationA 76-year-old male patient initially presented to the emergency room with a complaint of precordial chest pain for one month. Computed tomography (CT) of his chest showed a large, 8 cm × 8 cm x 10 cm, supradiaphragmatic, complex solid mass in the lower left hemithorax, along the anterior chest wall. Chest wall mass excision revealed dedifferentiated LPS, with excision of margins. Positron emission tomography (PET) scan did not show metastatic disease. Seven months later, he presented with shortness of breath, and CT of the chest showed large, left pleural-based masses, causing compression of surrounding structures. He was not a candidate for surgical resection. This patient subsequently failed chemotherapy and opted for hospice.ConclusionIntrathoracic LPS is a rare tumor. Recurrence is higher with dedifferentiated histology forms. Radical surgery with excision of margins is the primary recommended treatment.
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Project description:Since primary retroperitoneal liposarcoma (PRPLS) is rare in the clinic, related clinical studies are lacking. The present study was designed to investigate the predictive factors of short-term (≤1 year) recurrence (STR) and construct a novel nomogram of local recurrence-free survival (LRFS) for surgically resected PRPLS. A total of 128 PRPLS cases who underwent radical surgery were retrospectively analyzed. Based on the interval from the operation to tumor recurrence, the predictors of STR were screened using univariate and multivariate logistic regression analyses. Cox proportional hazard regression models were applied to identify the predictors of LRFS. Furthermore, the independent predictors acquired from multivariate analyses were used to construct a nomogram. Multivariate logistic regression analysis revealed that age ≥55 years [odds ratio (OR)=5.607, P=0.010], operative time ≥260 min (OR=9.716, P=0.005) and tumor necrosis (OR=3.781, P=0.037) were independent risk factors of STR for PRPLS. In the Cox regression analysis, clinical symptoms [hazard ratio (HR)=1.746, P=0.017], resection method (OR=0.370, P=0.021) and de-differentiated histological subtype (HR=1.975, P=0.048) were identified as independent predictors of LRFS. Subsequently, the independent predictors acquired from multivariate analyses were used to construct a nomogram for LRFS. Age, operative time, tumor necrosis, clinical symptoms, resection method and histological subtype were related to recurrence for surgically resected PRPLS and a novel nomogram was constructed based on the above predictors.
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Project description:BackgroundLiposarcoma (LPS) is one of the most common soft-tissue sarcomas. However, intrathoracic LPS is rare, as only 1% of all LPS cases are found in the thorax.MethodsA systematic literature review through PubMed and Embase databases was performed. Only eligible case reports and case series reporting intrathoracic LPS in adult patients were included. Kaplan-Meier curves were calculated to evaluate the survival rate of included patients based on the histological subtype of LPS.Results123 studies reporting 197 patients were included. We added a case of a 69-year-old female patient with recurrent giant intrathoracic LPS. The primary tumor measured 15.1cm × 22.9 cm × 21.9 cm and weighed 3100 g. Six months later, the patient was admitted to the hospital with another intrathoracic tumor measuring 9.5 cm × 9 cm× 1.4 cm. The immunohistochemical studies showed expression of murine double minute 2 (MDM2) antigen in both primary and recurrent tumor cells.ConclusionsDyspnea, chest pain, and cough were the most common symptoms reported in included studies. Overall, the 5-year survival rate was 62%. The highest survival was observed in well-differentiated LPS patients (80%) and the lowest in myxoid LPS (31%).
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Project description:BackgroundRetroperitoneal liposarcoma (RLS) is a type of rare malignant tumor. The aim of the present study was to study the correlations between the clinicopathological characteristics and the prognostic factors of RLSso as to delineate independent pathological indicators affecting prognosis.MethodsThe RLS pathological specimens were collected at Beijing Shijitan Hospital Affiliated to Capital Medical University from 2008 to 2018, and were used to study the histopathological features, including tumor size, histological types, lymphatic metastasis, tumor emboli in the blood and lymph vessels, and nerve invasion; immunohistochemical features, including Ki-67 and P53 proteins; and molecular pathological features, such as MDM2 (murine double minute2) gene amplification. Clinical treatment and follow-up data were collected, and the survival rate was calculated by Kaplan-Meier method. Univariate and multivariate survival analyses were performed to study the independent pathological factors.ResultsOf the 124 RLS cases, 63 were males (50.8%) and 61 were females (49.2%), with a median age of 52 years (range, 22-80 years). There were 51 cases (41.1%) of well-differentiated liposarcoma, 73 cases (57.3%) of dedifferentiated liposarcoma (DDL); 3 cases (2.4%) with lymph node metastases; 4 cases (3.2%) with tumor emboli in the vessels; 21 cases (16.9%) with Ki-67 label index <20% and 103 cases (83.1%) with Ki-67 label index ≥20%; and 81 cases (65.3%) with P53 <60% and 43 cases (24.7%) with P53 ≥60%. Fluorescence in situ hybridization studies revealed MDM2 gene amplification. Univariate analysis found the following pathological factors were correlated with prognosis: histopathological classification, recurrence, mitosis, tumor necrosis, Ki-67 label index, and P53. Multivariate analysis by Cox proportional hazard model found the following three independent pathological prognostic factors: age [hazard ratio (HR): 1.798, P=0.018], tumor site (HR: 1.263, P=0.002), tumor necrosis (HR: 1.984, P=0.024), and recurrence (HR: 2.161, P=0.049).ConclusionsAge, tumor necrosis, tumor site, and recurrence could be independent pathological prognostic factors for RLS.
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Project description:Background: Primary intrathoracic synovial sarcoma (SS) is a rare entity. The objective of this study was to evaluate survival outcomes for patients with intrathoracic SS presenting with localized disease at diagnosis. Methods: We conducted a retrospective review of 63 patients diagnosed with intrathoracic SS between 1997 and 2020. The Kaplan-Meier method and log-rank test were used to estimate the progression-free survival (PFS), overall survival (OS), local recurrence-free survival (LRFS), and metastasis-free survival (MFS). The hazard ratios were estimated by using Cox proportional hazards regression. Median follow-up time, age-at-diagnosis, and primary tumor size were 31 months (range: 4-218 months), 43 years (range: 18-77), and 7 cm (range: 1-23), respectively. Results: Sixty-two of sixty-three (98%) patients had their primary tumor resected, from whom eighteen (29%) and forty-three (69%) had received neo/adjuvant radiotherapy and chemotherapy, respectively. Median PFS, OS, and MFS were 1.2, 3.0, and 1.1 years, respectively. Based on multivariable analyses, patients with ≥5 cm tumor size had poorer OS (versus < 5 cm; HR: 2.66; 95% CI: 1.16, 6.11; LR-p = 0.014). Importantly, the receipt of neo/adjuvant chemotherapy was the only factor associated with both a more favorable PFS (HR: 0.33; 95% CI: 0.17, 0.65; LR-p = 0.0002) and a more favorable MFS (median 1.33 years versus no chemo 0.5 years; HR: 0.35; 95% CI: 0.17, 0.73; LR-p = 0.005). Conclusions: Outcomes associated with intrathoracic SS remain poor. Factors associated with poorer outcomes include larger tumors and omission of chemotherapy in the management of localized disease. We recommend providing perioperative chemotherapy to all patients with ≥5 cm tumor size to improve progression and metastasis-free survival.
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Project description:IntroductionPrimary intrathoracic liposarcoma is extremely rare, and most published series lack genetic analyses. The aim of our study is to better understand the clinicopathologic and genetic features of these rare lesions.Materials and methodsForty-three primary intrathoracic liposarcomas were identified and most cases were analyzed by systematic genetic studies, including fluorescence in situ hybridization (FISH), whole-exome sequencing (WES), and Sanger sequencing.ResultsThis series included 27 males and 16 females (ratios, 1.68:1) aged 24-73 years (median, 53 years). Tumors mainly occurred in the mediastinum (n=23, 53.5%), followed by pleural cavity (n=16, 37.2%) and lung (n=4, 9.3%). The study included 21 well-differentiated liposarcomas (WDLs), 19 dedifferentiated liposarcomas (DDLs), 2 myxoid pleomorphic liposarcomas (MPLs) and 1 pleomorphic liposarcoma (PL), without identification of myxoid liposarcoma. FISH analysis identified MDM2 amplification in 17 of 18 WDLs (94.4%) and all DDLs (16/16, 100.0%). The MDM2-nonamplified WDL was CDK4-nonamplified but FRS2-amplified. WES and Sanger sequencing found somatic TP53 mutation in the 2 MPLs. Follow-up information was available for 33 of 38 cases (86.8%). Thirteen patients (39.4%) showed no evidence of disease, 10 patients (30.3%) were alive with disease, and 8 patients (24.2%) died of disease. Fourteen cases developed recurrence and 1 with metastasis.ConclusionsWDL/DDL was the overwhelming subtype in this location, followed by MPL and PL. Analysis of the FRS2 gene, in combination with MDM2 and other genes of 12q13-15, may more precisely characterize WDL/DDLs. MPL is the most fatal subtype of this site. Further studies are needed to explore the role of TP53 in the pathogenesis of MPL.
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Project description:Leiomyomas associated with hereditary leiomyomatosis and renal cell carcinoma syndrome and leiomyomas with bizarre nuclei often show overlapping morphological features, in particular cells with prominent eosinophilic nucleoli, perinucleolar halos, and eosinophilic cytoplasmic inclusions. Although hereditary leiomyomatosis and renal cell carcinoma syndrome is defined by fumarate hydratase (FH) germline mutations, resulting in S-(2-succino)-cysteine (2SC) formation, it is unknown whether leiomyomas with bizarre nuclei show similar alterations. In this study, we evaluated the morphology and FH/2SC immunoprofile of 31 leiomyomas with bizarre nuclei. DNA from tumor and normal tissues from 24 cases was subjected to massively parallel sequencing targeting 410 key cancer genes. Somatic genetic alterations were detected using state-of-the-art bioinformatics algorithms. No patient reported a personal history of renal neoplasia or cutaneous leiomyomas, but one had a family history of renal cell carcinoma while another had a family history of uterine leiomyomas. Aberrant FH/2SC expression was noted in 17 tumors (16 FH-negative/2SC-positive, 1 FH-positive/2SC-positive). On univariate analysis, staghorn vessels, eosinophilic cytoplasmic inclusions, diffuse distribution of prominent eosinophilic nucleoli with perinucleolar halos, and an 'alveolar pattern of edema' were associated with an abnormal immunoprofile, but only staghorn vessels remained significant on multivariate analysis. Massively parallel sequencing analysis (n=24) revealed that 13/14 tumors with aberrant FH/2SC immunoprofile harbored somatic FH somatic genetic alterations, including homozygous deletions (n=9), missense mutations coupled with loss of heterozygosity (n=3), and a splice site mutation (n=1), whereas no somatic FH mutations/deletions were found in tumors with normal immunoprofile (n=10; P<0.0001). Leiomyomas with bizarre nuclei with normal FH/2SC staining pattern more frequently harbored TP53 and/or RB1 alterations than those with aberrant FH/2SC immunoprofile (60 vs 14%; P=0.032). These data demonstrate that leiomyomas with bizarre nuclei are morphologically and genetically heterogeneous and that hereditary leiomyomatosis and renal cell carcinoma syndrome-related morphological features, abnormal FH/2SC staining, and somatic FH mutations/deletions can be seen in a subset of sporadic tumors.
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Project description:ObjectiveThis study intended to retrospectively analyze the data of patients with primary retroperitoneal liposarcoma in a single Asian large-volume sarcoma center and to establish nomograms focused on PRLPS for predicting progression-free survival (PFS) and overall survival (OS).MethodsA total of 211 patients treated surgically for primary, non-metastatic retroperitoneal liposarcoma during 2009-2021 were identified, and clinicopathologic variables were analyzed. PFS and OS nomograms were built based on variables selected by multivariable analysis. The discriminative and predictive ability of the nomogram was assessed by concordance index and calibration curve.ResultsThe median follow-up time was 25 months. A total of 117 (56%) were well-differentiated, 78 (37%) were dedifferentiated, 13 (6%) were myxoid, and 3 (1%) were pleomorphic morphology. Compared to the western population cohort reported by the Memorial Sloan-Kettering Cancer Center, the median age of patients in this cohort was younger (57 vs. 63 years), the tumor burden was lower (20 vs. 26 cm), and the proportion of patients with R0 or R1 resection was higher (97% vs. 81%). The 5-year PFS rate was 49%, and factors independently associated with PFS were symptoms at visit, preoperative needle biopsy, histologic subtypes, and postoperative hospital stay. The 5-year OS rate was 72%. American Society of Anesthesiologists Physical Status and Clavien-Dindo classification were independently associated with OS. The concordance indexes for PFS and OS nomograms were 0.702 and 0.757, respectively. The calibration plots were excellent.ConclusionsThe proposed nomogram provided a favorable reference for the treatment of primary retroperitoneal liposarcoma patients.
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Project description:BackgroundCost and compliance are 2 factors that can significantly affect the outcomes of non-operative and operative treatment of trigger finger (TF) and both may be influenced by social factors. The purpose of this study was to investigate socioeconomic disparities in the surgical treatment for TF.MethodsAdult patients (≥18 years old) were identified using International Classification of Diseases 9 and 10 Clinical Modification diagnostic codes for TF and Current Procedural Terminology (CPT) procedural codes (CPT: 26055) in the New York Statewide Planning and Research Cooperative System database. Each diagnosis was linked to procedure data to determine which patients went on to have TF release. A multivariable logistic regression was performed to assess the likelihood of receiving surgery. The variables included in the analysis were age, sex, race, social deprivation index (SDI), Charlson Comorbidity Index, and primary insurance type. A P-value < .05 was considered significant.ResultsOf the 31 411 TF patients analyzed, 8941 (28.5%) underwent surgery. Logistic regression analysis showed higher odds of receiving surgery in females (odds ratio [OR]: 1.108) and those with workers compensation (OR: 1.7). Hispanic (OR: 0.541), Asian (OR: 0.419), African American (OR: 0.455), and Other race (OR: 0.45) had decreased odds of surgery. Medicaid (OR: 0.773), Medicare (OR: 0.841), and self-pay (OR: 0.515) reimbursement methods had reduced odds of receiving surgery. Higher social deprivation was associated with decreased odds of surgery (OR: 0.988).ConclusionsThere are disparities in demographic characteristics among those who receive TF release for trigger finger related to race, primary insurance, and social deprivation.
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