Well-differentiated Liposarcoma Research Articles

Investigation of radiomics and deep convolutional neural networks approaches for glioma grading

Purpose. To determine glioma grading by applying radiomic analysis or deep convolutional neural networks (DCNN) and to benchmark both approaches on broader validation sets. Methods. Seven public datasets were considered: (1) low-grade glioma or high-grade glioma (369 patients, BraTS’20) (2) well-differentiated liposarcoma or lipoma (115, LIPO); (3) desmoid-type fibromatosis or extremity soft-tissue sarcomas (203, Desmoid); (4) primary solid liver tumors, either malignant or benign (186, LIVER); (5) gastrointestinal stromal tumors (GISTs) or intra-abdominal gastrointestinal tumors radiologically resembling GISTs (246, GIST); (6) colorectal liver metastases (77, CRLM); and (7) lung metastases of metastatic melanoma (103, Melanoma). Radiomic analysis was performed on 464 (2016) radiomic features for the BraTS’20 (others) datasets respectively. Random forests (RF), Extreme Gradient Boosting (XGBOOST) and a voting algorithm comprising both classifiers were tested. The parameters of the classifiers were optimized using a repeated nested stratified cross-validation process. The feature importance of each classifier was computed using the Gini index or permutation feature importance. DCNN was performed on 2D axial and sagittal slices encompassing the tumor. A balanced database was created, when necessary, using smart slices selection. ResNet50, Xception, EficientNetB0, and EfficientNetB3 were transferred from the ImageNet application to the tumor classification and were fine-tuned. Five-fold stratified cross-validation was performed to evaluate the models. The classification performance of the models was measured using multiple indices including area under the receiver operating characteristic curve (AUC). Results. The best radiomic approach was based on XGBOOST for all datasets; AUC was 0.934 (BraTS’20), 0.86 (LIPO), 0.73 (LIVER), (0.844) Desmoid, 0.76 (GIST), 0.664 (CRLM), and 0.577 (Melanoma) respectively. The best DCNN was based on EfficientNetB0; AUC was 0.99 (BraTS’20), 0.982 (LIPO), 0.977 (LIVER), (0.961) Desmoid, 0.926 (GIST), 0.901 (CRLM), and 0.89 (Melanoma) respectively. Conclusion. Tumor classification can be accurately determined by adapting state-of-the-art machine learning algorithms to the medical context.

A CASE STUDY ON RETROPERITONEAL MASSES

Background 
 Retroperitoneal masses are a large group of uncommon tumours which originate in the retroperitoneal space in the abdomen. Their diverse presentations and locations make them difficult to diagnose by surgeons and need precise radiological diagnosis. Most of the diagnosed tumours are in advanced size and stage and are malignant in nature. Some of them are radio/chemotherapy sensitive but the definite management should be by complete resections of the tumours.
 Objectives 
 The primary aim of our study is to identify patients diagnosed with retroperitoneal masses among elective cases in two surgical hospitals in Sulaimani governorate/ Iraq over four years. All underwent combined surgical management with chemo/radiotherapy and followed up.
 Materials and Methods
 Seven patients with retroperitoneal masses are reported in this paper, five of whom are male and two females. Their ages range from 3 to 72 years, and the mean age is 40.4. In addition, other surgeons who practiced in different specialties brought some of the cases to us. Over four years, they were hospitalized and treated at Sulaimani Teaching Hospital and Soma Private Hospital. The patients underwent surgery following a thorough history, physical examination, and imaging, and the histological findings determined the definitive diagnosis. In addition to direct questioning the patients and patients’ parents (as one case was a child) to obtain their consent, information was gathered from the medical records of both hospitals.
 Results
 Complete resection of retroperitoneal tumours by surgical approach was reported in five patients included in the study, and residual tumours were reported in two. An uneventful post-operative period in all of them, and recurrence was recorded in the follow-up period in three of them. Histopathological findings were: two well-differentiated liposarcomas, one dedifferentiated liposarcoma, one Ewing sarcoma, one rhabdomyosarcoma, one ganglioneuroma, and one lipoma. Mortality was reported in three of the patients. 
 Conclusion
 Retroperitoneal tumours are rare and require precise diagnosis by physical examination and imaging modalities. The primary management method in our study was surgical resection combined with neoadjuvant chemo/radiotherapy and regular follow-up.

Organ Infiltration and Patient Risk After Multivisceral Surgery for Primary Retroperitoneal Liposarcomas.

The extent of histological organ involvement (HOI) to organs and structures of a retroperitoneal liposarcoma may have prognostic implications. This study investigated incidence, characteristics, and risk association of HOI in these patients. Data of patients who underwent multivisceral resection for primary liposarcoma (2009-2014) were retrospectively analyzed. HOI was the variable of interest and was classified into four degrees: absent (HOI-0), perivisceral (HOI-1), initial (HOI-2), and advanced (HOI-3). Primary endpoint was overall survival (OS). Secondary endpoint was disease-free survival (DFS). The prognostic value of HOI was adjusted for preoperative treatment and the Sarculator nomogram score. A total of 109 patients were included. HOI-0, HOI-1, HOI-2, and HOI-3 were detected in 9 (8.3%), 11 (10.1%), 43 (39.4%), and 46 (42.2%) patients. Median follow-up was 8.4 years [interquartile range (IQR) 7.2-9.6 years]. There were 68 recurrences and 50 patient deaths observed, resulting in a 10-year OS and DFS of 51.1% [95% confidence interval (CI) 41.9-62.1%] and 34.1% (95% CI 25.2-46.1%), respectively. Clinically relevant HOIs (HOI-2 and HOI-3) were found in 35/45 (77.8%) and 54/64 (84.4%) cases of well- and de-differentiated liposarcomas, respectively. On multivariable survival analysis, patients with HOI-3 had significantly shorter OS (HOI-3 vs HOI-0/HOI-1 HR 2.92; p = 0.012) and DFS (HOI-3 vs HOI-0/HOI-1 HR 2.23; p = 0.045), independently of the nomogram score (OS: HR 2.93; p < 0.001; DFS: HR 1.78; p = 0.003). Initial and advanced HOIs are frequently detected in both well-differentiated and de-differentiated liposarcomas, supporting that multivisceral resection may be needed. HOI stratifies the risk of patients with primary retroperitoneal liposarcoma.

Rare paratesticular localization of dedifferentiated liposarcoma: Case report and review of the literature.

Dedifferentiated liposarcoma is defined as a malignant tumor that changes its shape from a well-differentiated liposarcoma to a non-liposarcomatous form. Most paratesticular liposarcomas manifest as an inguinal, painless, slow-growing mass. The standard treatment is extensive surgical excision, radiotherapy being proposed for cases with positive margins, those with recurrence, or in cases of the existence of unfavorable prognostic factors. We present the case of a young patient diagnosed initially with left hydrocele, which after 2 years proved to mask a differentiated liposarcoma of the spermatic cord. The initial clinical manifestations were represented by the increase in volume of the left groin-scrotal region and pain at this level. Microscopic examination in hematoxylin-eosin staining highlighted the presence of lipoblasts and fibroblasts in association with areas of hemorrhage and tumor necrosis. The performed immunohistochemical tests confirmed the diagnosis of dedifferentiated liposarcoma. To support and confirm the presence of the mouse double minute 2 homolog gene mutation, chromogenic in situ hybridization analysis was performed. The initial treatment was the surgical one. After 2 weeks, the patient received zolendronic acid for hypercalcemia which was caused by the osseous metastasis. The patient died secondary to acute renal failure caused by hypercalcemia despite the treatment received. This case underlines the importance of both the correct management of oncological patients, as well as immunohistochemical and genetic tests in the identification of prognostic factors, with the ultimate goal of administering an appropriate oncological treatment.

Clinical and molecular features of four Brazilian families with multiple endocrine neoplasia type 1.

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant syndrome characterized by its clinical variability and complexity in diagnosis and treatment. We performed both clinical and molecular descriptions of four families with MEN1 in a follow-up at a tertiary center in Brasília. From a preliminary review of approximately 500 medical records of patients with pituitary neuroendocrine tumor (PitNET) from the database of the Neuroendocrinology Outpatient Clinic of the University Hospital of Brasília, a total of 135 patients met the criteria of at least two affected family members. From this cohort, we have identified 34 families: only four with a phenotype of MEN1 and the other 30 families with the phenotype of familial isolated pituitary adenoma (FIPA). Eleven patients with a clinical diagnosis of MEN1 from these four families were selected. Variants in MEN1 gene were identified in all families. One individual from each family underwent genetic testing using targeted high-throughput sequencing (HTS). All patients had primary hyperparathyroidism (PHPT), and the second most common manifestation was PitNET. One individual had well-differentiated liposarcoma, which has been previously reported in a single case of MEN1. Three variants previously described in the database and a novel variant in exon 2 have been found. The study allowed the genotypic and phenotypic characterization of families with MEN1 in a follow-up at a tertiary center in Brasília.

Protocol for the 2ND-STEP study, Japan Clinical Oncology Group study JCOG1802: a randomized phase II trial of second-line treatment for advanced soft tissue sarcoma comparing trabectedin, eribulin and pazopanib

BackgroundSoft tissue sarcomas (STS) are a rare type of malignancy comprising a variety of histological diagnoses. Chemotherapy constitutes the standard treatment for advanced STS. Doxorubicin-based regimens, which include the administration of doxorubicin alone or in combination with ifosfamide or dacarbazine, are widely accepted as first-line chemotherapy for advanced STS. Trabectedin, eribulin, pazopanib, and gemcitabine plus docetaxel (GD), which is the empirical standard therapy in Japan, are major candidates for second-line chemotherapy for advanced STS, although clear evidence of the superiority of any one regimen is lacking. The Bone and Soft Tissue Tumor Study Group of the Japan Clinical Oncology Group (JCOG) conducts this trial to select the most promising regimen among trabectedin, eribulin, and pazopanib for comparison with GD as the test arm regimen in a future phase III trial of second-line treatment for patients with advanced STS.MethodsThe JCOG1802 study is a multicenter, selection design, randomized phase II trial comparing trabectedin (1.2 mg/m2 intravenously, every 3 weeks), eribulin (1.4 mg/m2 intravenously, days 1 and 8, every 3 weeks), and pazopanib (800 mg orally, every day) in patients with unresectable or metastatic STS refractory to doxorubicin-based first-line chemotherapy. The principal eligibility criteria are patients aged 16 years or above; unresectable and/or metastatic STS; exacerbation within 6 months prior to registration; histopathological diagnosis of STS other than Ewing sarcoma, embryonal/alveolar rhabdomyosarcoma, well-differentiated liposarcoma and myxoid liposarcoma; prior doxorubicin-based chemotherapy for STS, and Eastern Cooperative Oncology Group performance status 0 to 2. The primary endpoint is progression-free survival, and the secondary endpoints include overall survival, disease-control rate, response rate, and adverse events. The total planned sample size to correctly select the most promising regimen with a probability of > 80% is 120. Thirty-seven institutions in Japan will participate at the start of this trial.DiscussionThis is the first randomized trial to evaluate trabectedin, eribulin, and pazopanib as second-line therapies for advanced STS. We endeavor to perform a subsequent phase III trial comparing the best regimen selected by this study (JCOG1802) with GD.Trial registrationThis study was registered with the Japan Registry of Clinical Trials (jRCTs031190152) on December 5, 2019.

Prognostic factors and outcomes of adult spermatic cord sarcoma. A study from the French Sarcoma Group

PurposeTo evaluate the outcomes of adult patients with spermatic cord sarcoma (SCS). MethodsAll consecutive patients with SCS managed by the French Sarcoma Group from 1980 to 2017 were analysed retrospectively. Multivariate analysis (MVA) was used to identify independent correlates of overall survival (OS), metastasis-free survival (MFS), and local relapse-free survival (LRFS). ResultsA total of 224 patients were recorded. The median age was 65.1 years. Forty-one (20.1%) SCSs were discovered unexpectedly during inguinal hernia surgery. The most common subtypes were liposarcoma (LPS) (73%) and leiomyosarcoma (LMS) (12.5%). The initial treatment was surgery for 218 (97.3%) patients. Forty-two patients (18.8%) received radiotherapy, 17 patients (7.6%) received chemotherapy. The median follow-up was 5.1 years. The median OS was 13.9 years. In MVA, OS decreased significantly with histology (HR, well-differentiated LPS versus others = 0.096; p = 0.0224), high grade (HR, 3 versus 1–2 = 2.7; p = 0.0111), previous cancer and metastasis at diagnosis (HR = 6.8; p = 0.0006). The five-year MFS was 85.9% [95% CI: 79.3–90.6]. In MVA, significant factors associated with MFS were LMS subtype (HR = 4.517; p < 10-4) and grade 3 (HR = 3.664; p < 10-3). The five-year LRFS survival rate was 67.9% [95% CI: 59.6–74.9]. In MVA, significant factors associated with local relapse were margins and wide reresection (WRR) after incomplete resection. OS was not significantly different between patients with initial R0/R1 resection and R2 patients who underwent WRR. ConclusionsUnplanned surgery affected 20.1% of SCSs. A nonreducible painless inguinal lump should suggest a sarcoma. WRR with R0 resection achieved similar OS to patients with correct surgery upfront.

Management of Recurrent Retroperitoneal Sarcoma

Recurrence after resection of retroperitoneal sarcoma is common and varies by histological subtype. Pattern of recurrence is similarly affected by histology (e.g., well-differentiated liposarcoma is more likely to recur locoregionally, whereas leiomyosarcoma is more likely to develop distant metastases). Radiotherapy may provide effective locoregional control in limited circumstances and the data on the impact of chemotherapy are scant. Surgery for locally recurrent disease is associated with the greatest survival benefit; however, data are retrospective and from a highly selected subgroup of patients. Limited retrospective data have also suggested a survival association with the resection of limited distant metastases. Given the complexity of these patients, multidisciplinary evaluation at a high-volume sarcoma center is critical.

Development and validation of a nomogram for predicting morbidity in surgically resected primary retroperitoneal sarcoma

BackgroundSurgery is the cornerstone of the treatment for primary retroperitoneal sarcoma (RPS). The purpose of this study was to establish a nomogram predictive model for predicting postoperative morbidity in primary RPS.MethodsClinicopathological data of patients who underwent radical resection from 2009 to 2021 were retrospectively analyzed. Risk factor analysis was performed using a logistic regression model, and modeling variables were selected based on Akaike Information Criterion. The nomogram prediction model was built on the basis of a binary logistic regression model and internally validated by calibration curves and concordance index.ResultsA total of 319 patients were enrolled, including 162 males (50.8%). 22.9% (n = 73) were over 65 years of age, and 70.2% (n = 224) had tumors larger than 10 cm. The most common histologic subtypes were well-differentiated liposarcoma (38.2%), dedifferentiated liposarcoma (25.1%) and leiomyosarcoma (7.8%). According to the Clavien–Dindo Classification, 96 (31.1%) and 31 (11.6%) patients had grade I–II complications and grade III–V complications, respectively. Age, tumor burden, location, operative time, number of combined organ resections, weighted resected organ score, estimated blood loss and packed RBC transfusion was used to construct the nomogram, and the concordance index of which was 0.795 (95% CI 0.746–0.844). and the calibration curve indicated a high agreement between predicted and actual rates.ConclusionsNomogram, a visual predictive tool that integrates multiple clinicopathological factors, can help physicians screen RPS patients at high risk for postoperative complications and provide a basis for early intervention.

Clinical analysis of 5-year survival and recurrence in giant retroperitoneal liposarcoma after surgery.

Clinical analysis of 5-year survival and recurrence in giant retroperitoneal liposarcoma after surgery.

A Case of Giant Well-Differentiated Liposarcoma Requiring Extensive Resection to the Deep Lateral Neck

頭頸部領域の軟部肉腫は全頭頸部腫瘍中でも非常にまれな疾患であり，その中で脂肪肉腫は最も頻度が高い。頸部に発生する場合，解剖学的観点より再発時の完全切除が難しく比較的予後は悪いとされる。今回われわれは，腫瘍を完全切除するために頭頸部外科医が扱う頸部郭清の範囲を超え，側頸深部まで広範囲な切除を要した巨大脂肪肉腫を経験した。症例は50歳男性。上背部脂肪腫で7回腫瘍摘出術を施行されているが再発を繰り返し，7回目の手術で病理組織学的に高分化型脂肪肉腫と診断され当科に紹介された。腫瘍は左頸部から後頸部，背部に及び，頭頸科で頸部郭清術を行い，整形外科にて深頸筋膜深葉を超えて腫瘍を切除した。腫瘍切除後は，形成外科により広背筋有茎皮弁を用いて再建した。高分化型脂肪肉腫では辺縁切除が許容される場合もあるが，再発を繰り返している症例では脱分化すると遠隔転移のリスクが高いといわれている。頭頸部領域など再手術時のリスクが高い領域に発生した症例では，完全切除するために広範切除が必要である。ただし救済手術は困難になることが多く，初回治療時から計画性のある切除態度で臨むことが大切である。

Case Report: Resection of Giant Mediastinal Liposarcoma Using the Partial Sternotomy Incision

Primary mediastinal liposarcomas are rare. There are four types of liposarcoma: well differentiated/atypical lipomatous tumor, dedifferentiated, myxoid, pleomorphic. In this study we report a rare case of a mediastinal well-differentiated liposarcoma that was successfully surgically removed. Because of the noninvasive growth pattern of most liposarcomas, patients often present with few symptoms, which was the case in our patient presenting only with dry cough. Normally these tumors are diagnosed by radiologic imaging. Common symptoms include cough, dyspnea, wheezing, chest pain, and weight loss. There are different histologic variants of liposarcoma, the most frequent one is well-differentiated liposarcoma. In our case we couldn’t confirm the histology with fine needle aspiration but with the frozen section we confirmed the diagnosis of well-differentiated liposarcoma. An optimal approach is very important to remove mediastinal liposarcoma because of the diversity of size and location of lesions.

Silver In Situ Hybridization for the Rapid Assessment of MDM2 Amplification in Soft Tissue and Bone Tumors. Validation Based on an Audit of 192 Consecutive Cases Evaluated by Silver In Situ Hybridization and Fluorescence In Situ Hybridization.

The discovery of almost invariable mouse double minute 2 (MDM2) amplification among atypical lipomatous tumors (ALT)/well-differentiated liposarcoma and dedifferentiated liposarcoma is incorporated into the contemporary diagnostic workup of fatty lesions. MDM2 amplifications are also found frequently in intimal sarcomas and in low-grade osteogenic sarcoma. At present, fluorescence in situ hybridization (FISH) is the reference test for MDM2 assessment. We are interested in evaluating silver in situ hybridization (SISH) for this purpose. Between October 2016 and May 2020, in 192 consecutive cases requiring MDM2 FISH, SISH was also performed concurrently, including 77 (40.1%) core biopsies and 115 (58.9%) surgical specimens. The mean patient age was 61.0 years. SISH results were available overnight or within 48 hours if repeat testing was required. FISH results were available within 2 to 5 weeks. The cost of SISH was one third of FISH. FISH demonstrated MDM2 amplification in 44 cases (23.6%), was negative in 144 cases (74.4%) and nondiagnostic in 4 decalcified cases (2.0%). SISH showed MDM2 amplification in 33 cases (17.2%), no amplification in 119 cases (62.0%), and indeterminate results because of poor signal in 40 (20.8%) cases. All 33 (100%) SISH-amplified tumors and 113 of 119 (95.0%) nonamplified results were confirmed by FISH. There were no clear differences in the performance of SISH on NCB versus surgical specimens. The overall performance indices of SISH are sensitivity 75%, specificity 78.5%, positive predictive value 100%, and negative predictive value 95.8%. FISH is not required when SISH is clearly amplified. This is clinically useful and improves efficiency. Nonamplified SISH results provide early indications of the likely FISH findings, but there is a 4.2% chance of FISH being positive. At present, the main drawback of SISH is the high rate of nondiagnostic tests. Optimization of SISH signal detection to reduce the proportion of indeterminate results is our current focus.

GLI1 Immunohistochemistry Distinguishes Mesenchymal Neoplasms With GLI1 Alterations From Morphologic Mimics.

Glioma-associated oncogene 1 ( GLI1 ) alterations have been described in pericytoma with t(7;12), gastroblastoma, plexiform fibromyxoma, and an emerging class of GLI1 -rearranged or amplified mesenchymal neoplasms including "nested glomoid neoplasm". The immunophenotype of these tumor types is nonspecific, making some cases difficult to diagnose without sequencing. The utility of GLI1 immunohistochemistry (IHC) in distinguishing nested glomoid neoplasms and pericytomas with t(7;12) from morphologic mimics is unknown. To investigate the diagnostic value of GLI1 IHC, we determined its sensitivity and specificity in a "test cohort" of 23 mesenchymal neoplasms characterized by GLI1 alterations, including 12 nested glomoid neoplasms (7 GLI1 -rearranged, 4 GLI1 amplified, and 1 unknown GLI1 status), 9 pericytomas with t(7;12), 1 gastroblastoma, and 1 malignant epithelioid neoplasm with PTCH1 :: GLI1 fusion. GLI1 IHC was 91.3% sensitive in this cohort; all tumors except 2 pericytomas with t(7;12) expressed GLI1. GLI1 was also expressed in 1 of 8 (12%) plexiform fibromyxomas. Nineteen of 22 GLI1-positive tumors showed nuclear and cytoplasmic staining, while 3 showed nuclear staining only. GLI1 IHC was 98.0% specific; among morphologic mimics [40 well-differentiated neuroendocrine tumors, 10 atypical lung carcinoids, 20 paragangliomas, 20 glomus tumors, 20 solitary fibrous tumors, 10 Ewing sarcomas, 10 alveolar rhabdomyosarcomas (ARMS), 10 BCOR -altered sarcomas, 10 myoepitheliomas, 9 myopericytomas, 9 epithelioid schwannomas, 9 ossifying fibromyxoid tumors, 10 biphasic synovial sarcomas, 10 PEComas, 31 gastrointestinal stromal tumors, 10 inflammatory fibroid polyps, 11 pseudoendocrine sarcomas], 5 of 249 tumors expressed GLI1 (2 well-differentiated neuroendocrine tumors, 1 ARMS, 1 Ewing sarcoma, 1 BCOR -altered sarcoma). GLI1 IHC was also performed on a separate cohort of 13 molecularly characterized mesenchymal neoplasms in which GLI1 copy number gain was identified as a putatively secondary event by DNA sequencing (5 dedifferentiated liposarcoma [DDLPS], 2 adenosarcomas, 2 unclassified uterine sarcomas, 1 leiomyosarcoma, 1 ARMS, 1 intimal sarcoma, 1 osteosarcoma); 2 DDLPS, 1 ARMS, and 1 unclassified uterine sarcoma expressed GLI1. Lastly, because pleomorphic sarcomas sometimes show GLI1 amplification or copy number gain, GLI1 IHC was performed on a separate "pleomorphic sarcoma" cohort: GLI1 was expressed in 1 of 27 DDLPS, 1 of 9 leiomyosarcomas, and 2 of 10 pleomorphic liposarcomas, and it was negative in 23 well-differentiated liposarcomas and 9 unclassified pleomorphic sarcomas. Overall, GLI1 IHC was 91.3% sensitive and 98.0% specific for mesenchymal tumor types with driver GLI1 alterations among morphologic mimics. GLI1 expression was less frequent in other tumor types with GLI1 copy number gain. Given its specificity, in the appropriate morphologic context, GLI1 IHC may be a useful diagnostic adjunct for mesenchymal neoplasms with GLI1 alterations.

Mandibular Myxoid Liposarcoma: a Case Study.

Liposarcomas are described as soft tissue sarcomas derived from adipose tissue. The finding of this tumor in the mandibular region is exceedingly rare. As of now, it has been described mainly in case reports and small series. A multidisciplinary approach is required to offer optimal treatment and may involve surgery, radiation and systemic therapies. Surgical repair of these defects represents a major challenge in oral and maxillofacial reconstructive surgery. We present the case of a 54-year-old man referred to our center with a progressively increasing mass in the anterior portion of the mandible. Biopsy revealed a well-differentiated myxoid liposarcoma. Resection of the tumor was performed with an additional primary reconstruction.

Well-Differentiated Liposarcoma as a Case: The Competence of Surgical Treatment was Questioned

Sarcomas are malignant tumors arising from skeletal and non-skeletal connective tissue, as well as the peripheral nervous system. Sarcoma is detected in the majority of patients who have a primary retroperitoneal, extra-visceral, unifocal soft tissue mass. Liposarcoma and leiomyosarcoma are the most prevalent histologic types of retroperitoneal sarcoma in adults. Most retroperitoneal liposarcomas are known as low to mild grade tumors. Well differentiated (low grade) liposarcomas are the most prevalent kind and have no potential to metastasize. Atypical lipomatous tumors are defined as those that develop on the body wall, trunk, or extremity. However, the same tumors are called well-differentiated liposarcomas due to their tendency for local recurrence and located in the retroperitoneum, mediastinum, and spermatic cord. In our case, we aimed to present a case that occupies most of the intra-abdominal volume, has associated gastrointestinal system complaints, and eventually was diagnosed as well-differentiated liposarcoma following surgical treatment.

Targeting Autophagy With the Synergistic Combination of Chloroquine and Rapamycin as a Novel Effective Treatment for Well-differentiated Liposarcoma.

Liposarcoma is a type of soft-tissue sarcoma arising from fat tissue. It is relatively common among soft-tissue sarcomas. Chloroquine (CQ), an antimalarial drug, can inhibit autophagy and induce apoptosis in cancer cells. Rapamycin (RAPA) is an inhibitor of mTOR. The combination of RAPA and CQ is a strong inhibitor of autophagy. Previously, we showed that the combination of RAPA and CQ was effective against a de-differentiated liposarcoma patient-derived orthotopic xenograft (PDOX) mouse model. In the present study, we investigated the mechanism of efficacy of the combination of RAPA and CQ to target autophagy in a well-differentiated liposarcoma (WDLS) cell line in vitro. The human WDLS cell line 93T449 was used. The WST-8 assay was used to test the cytotoxicity of RAPA and CQ. Western blotting was used to detect microtubule-associated protein light chain 3-II (LC3-II) which is a component of autophagosomes. Immunostaining of LC3-II was also performed for autophagosome analysis. Τhe TUNEL assay was used to detect apoptotic cells, and apoptosis-positive cells were counted in three randomly selected microscopic fields for statistical validation. RAPA alone and CQ alone inhibited the viability of 93T449 cells. The combination of RAPA and CQ inhibited 93T449 cell viability significantly more than either drug alone and increased the number of autophagosomes which led to extensive apoptosis. The combination of RAPA and CQ increased the number of autophagosomes which led to apoptosis in 93T449 WDLS cells, suggesting novel effective treatment for this recalcitrant cancer by targeting autophagy.

Large soft-tissue mass in the extremity is not necessarily a sarcoma: A case report of a giant lipoma of the thigh

Lipoma is the most common benign adipose tissue tumor. Giant lipomas are those that measure more than 10 cm in length or more than 1000 g in weight. There is a possibility of sarcomatous change in them but it is not always the rule. Lipoma and liposarcoma can be differentiated on the magnetic resonance imaging features of tumor size, stranding, nodularity, T2 signal, and contrast enhancement. The existence of thin septa helps identify lipomas and distinguishes them from well-differentiated liposarcomas, which have thick and irregular septa and fat necrosis at times. The open surgical approach is the best for the complete excision of giant lipomas without any recurrence.

Giant Liposarcoma of the Lumbar Fossa: A Case Report

Retroperitoneal sarcomas are rare tumors that develop in the mesenchymal tissue, where liposarcoma is the most frequently encountered subtype. The symptoms they induce are often vague and nonspecific, thus requiring thorough evaluation. Their diagnosis primarily relies on imaging techniques such as CT scans and MRI, and is confirmed by biopsy. Despite excision surgery, which remains the main treatment aiming for total resection, local recurrences remain a frequent challenge. Although radiotherapy and chemotherapy in the perioperative period are sometimes considered, their effectiveness remains subject to debate. The histological type of the tumor is of paramount importance in prognosis, with well-differentiated liposarcomas being associated with better outcomes than myxoid or dedifferentiated forms. Optimal management of these tumors requires a multidisciplinary approach, involving close collaboration between surgeons, oncologists, radiologists, and pathologists to ensure adequate and personalized treatment. Hence, the interest of a case of giant retroperitoneal liposarcoma in a 65-year-old man that we managed and report here.

Clinicopathological Features of Intrathoracic Liposarcoma-A Systematic Review with an Illustrative Case.

Liposarcoma (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. A 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. 123 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. Dyspnea, 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%).