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Testicular cancers are classified in germ cell and non-germ cell tumors, as well as, liposarcomas. We report the case of a patient with a large testicular liposarcoma, submitted to surgical treatment with excision of scrotal pouch and segment of the spermatic cord, and the left testicle, showing a good evolution. This report presents one of the first cases of a sclerosing variant of well-differentiated testicular liposarcoma, large in size and with no association with another cancer. Due to their location, the diagnosis is difficult and unusual. Complete tumor resection and regular medical follow-up show a good prognosis, less recurrence, and little cellular differentiation. RESUMEN Los cánceres de testículo se clasifican en tumores de células germinales y células no germinales, así como en liposarcomas. Presentamos el caso de un paciente con un gran liposarcoma testicular, sometido a tratamiento quirúrgico con exéresis de la bolsa escrotal y segmento de cordón espermático y testículo izquierdo, con buena evolución. Este informe presenta uno de los primeros casos de una variante esclerosante de liposarcoma testicular bien diferenciado, de gran tamaño y sin asociación con otro cáncer. Debido a su ubicación, el diagnóstico es difícil e inusual. La resección completa del tumor y el seguimiento médico regular muestran un buen pronóstico, menor recidiva, y poca diferenciación celular. RESUMO Os tumores testiculares são classificados em células germinativas e não germinativas, assim como os lipossarcomas. Relatamos o caso de um paciente com lipossarcoma testicular de grande dimensão submetido a tratamentos cirúrgicos com ressecção de bolsa escrotal e segmento do cordão espermático e do testículo esquerdo, apresentando boa evolução do quadro. O relato traz um dos primeiros casos de lipossarcoma testicular bem diferenciado com variante esclerosante, de grande dimensão e sem associação a outra neoplasia. Devido à localização, apresenta diagnóstico difícil e pouco habitual. Com a ressecção total do tumor e o acompanhamento médico regular, o paciente apresenta bom prognóstico, menor recidiva e pouca diferenciação celular.

Aim To evaluate results of the surgical treatment of liposarcomas during the period of 2005 and 2017 in LUHSH KC. Objectives To evaluate the main demographic data (gender, age) of patients, that undergone surgical liposarcoma treatment, to evaluate liposarcomas characteristics, patients‘ survival rate Methodology Retrospective clinical study analysing patients who had liposarcoma surgery during the period of 2005 - 2017 in the LUHSCH KC. Results Ratio between female and male patients was 1,2:1. Patients‘ mean age was 59,52. Most commonly diagnosed liposarcoma was myxoid (34,5 %). The most commonly diagnosed liposarcoma in age group of 80 year and above was differentiated liposarcoma. Myxoid liposarcoma most commonly diagnosed in age group of 40-60 years. Dedifferentiated liposarcomas diagnosed significantly more often to male than female patients. Localization of liposarcomas was: 39,9 % limbs, 34,5% retroperitoneum. 21,8% atypical localizations – internal organs, chest, brain area. Liposarcomas were diagnosed in lower limbs more frequently. Majority of diagnosed liposarcomas were third diferentiantion degree. Average tumor size was 14,34 ± 11,75 cm. Liposarcomas size correlated with patients survival time – the bigger liposarcoma was the shorter survival time. The best 5 years overall survival was related to differentiated liposarcomas. Relation between radicality of surgery and patients overall survival was observed. Overall survival was significantly better when liposarcoma was removed radically. Conclusion During the period of 2005-2017 in LUHSH KC 55 patients undergone liposarcoma surgery. Patients average age was 59 years, patients mostly were women. Most commonly diagnosed type of liposarcoma was myxoid liposarcoma. Majority of sarcomas were detected in lower limbs. The average lipsarcoma size was - 14,34 ± 11,75cm, diferentiation degree– G3. There was relation between patients overall survival and radicality of surgery and liposarcomas differentiation degree (p

International audience; Introduction: Among lipomatous soft tissue tumor, the noninvasive diagnosis between benign lipomas, borderline well differentiated liposarcomas (WDL)/atypical lipomatous tumors (ALT) and high grade subtype of liposarcomas is crucial since it directly drive the therapy strategy. Our aim is to develop a MRI-based radiomic method to classify between these three forms of tumors.Methods: 105 subjects with lipomatous soft tissue tumors with histology and fat-suppressed T1w contrast enhanced MR images available were retrospectively enrolled to constitute the database. According to histology, 3 groups have been constituted: benign (including deep lipomas, n = 23); intermediate (including ALT and WDL, n = 41); and malignant (including high grade liposarcomas: myxoid, dedifferentiated, and pleomorphic, n = 41). MR images were obtained from 56 different centers with non-uniform protocols (3 fields: 1.0T, 1.5T, and 3.0T, with 18 different MR systems commercialized by 4 vendors). Images were automatically loaded on an in-house software and tumor was manually segmented by two observers blinded to histology to extract the radiome. The radiome included 87 features (size, shape, intensity distribution, image domain and frequency domain textures features). Radiomes were next mined with a supervised machine learning approach to build a decisional algorithm based on two radiomic models.Results: To classify between benign and (intermediate + malignant) groups, the 12th order model gave the best performance (AUROC: 0.959; sensitivity: 89%; specificity: 95.7). To classify between intermediate and malignant groups the 17th order model gave the best diagnosis performances (AUROC: 0.907; sensitivity: 85.4%; specificity: 90.2%).Conclusion: These results show that the evaluation of lipomatous tumor malignancy is feasible using a routinely used MRI acquisition in clinical practice. These encouraging results need to be further confirmed on another prospective or existing application cohort.

The article shows the literature review of the biology, prognostic factors and the results of liposarcomas treatment according to the histological subtypes and the localization of the primary tumor. We are also discussing the modern options of systemic therapy, including the original chemotherapy regimens, developed in N.N.Blokhin Russian Cancer Research Center, and new effective therapeutic agents.

Sarcomas are a heterogeneous group of mesenchymal tumors arising from soft tissue or bone, with an uncertain etiology and difficult classification. Soft tissue sarcomas (STSs) account for around 1% of all adult cancers. Till date, more than 50 histologic subtypes have been identified. Adipocyte sarcoma or liposarcoma (LPS) is one of the most common STS subtypes, accounting for 15% of all sarcomas, with an incidence of 24% of all extremity STSs and 45% of all retroperitoneal STSs. The new World Health Organization classification system has divided LPS into four different subgroups: atypical lipomatous tumor/well-differentiated LPS, dedifferentiated LPS, myxoid LPS, and pleomorphic LPS. These lesions can develop at any location and exhibit different aggressive potentials reflecting their morphologic diversity and clinical behavior. Patients affected by LPS should be managed in specialized multidisciplinary cancer centers. Whereas surgical resection is the mainstay of treatment for localized disease, the benefits of adjuvant and neoadjuvant chemotherapy are still unclear. Systemic treatment, particularly chemotherapy, is still limited in metastatic disease. Despite the efforts toward a better understanding of the biology of LPS, the outcome of advanced and metastatic patients remains poor. The advent of targeted therapies may lead to an improvement of treatment options and clinical outcomes. A larger patient enrollment into translational and clinical studies will help increase the knowledge of the biological behavior of LPSs, test new drugs, and introduce new methodological studies, that is, on treatment response.

Benign adipocytic tumors (AT) are mainly represented by lipomas whereas most malignant AT are Atypical Lipomatous Tumors/Well-differentiated liposarcomas (ALT/WDLPS) and dedifferentiated liposarcomas (DDLPS). HMGA2 gene (High Mobility Group A2) is rearranged in some lipomas and amplified in ALT/WDLPS and DDLPS. Thus, we hypothesized that HMGA2 played a fundamental role in benign and malignant AT genesis. In favor of this hypothesis, we observed a constant overexpression of HMGA2 in amplified ALT/WDLPS and DDLPS, and in rearranged lipomas. In a case of lipomatosis, that is a pathological proliferation of the adipocytic tissu without rearrrangement of HMGA2, the overexpression of HMGA2 was asssociated with an inhibition of the expression of several let-7 microRNAs. However, we did not find a leading role of let-7 microRNAs in the deregulation of HMGA2 expression in AT. We also studied partner fusion genes of HMGA2 in lipomas and have specifically identified a new fusion involving PPAP2B (Phosphatidic Acid Phosphatase type 2B) which is located in 1p32. We also confirmed the role of NFIB gene (9p22) in lipomas. Finally, we have established prognostic correlations in a series of 116 ALT/WDLPS and DDLPS: HMGA2 amplification was associated with ALT/WDLPS histotype and a longer survival whereas respective CDK4 and JUN amplification were associated with DDLPS and shorter survival. Thus, our data support the hypothesis of an early and major role of HMGA2 in the genesis well differentiated AT., Les tumeurs adipocytaires (TA) bénignes sont majoritairement constituées par les lipomes, alors que les TA malignes sont principalement des Tumeurs Lipomateuses Atypiques (TLA)/ liposarcome (LPS) bien différenciés (LBD) et les LPS dédifférenciés (LDD). Le gène HMGA2 (High Mobility Group A2) est remanié dans certains lipomes et amplifié dans les TLA/LBD et LDD. Ainsi, nous avons émis l’hypothèse que HMGA2 jouait un rôle fondamental dans la genèse des TA bénignes et malignes. En faveur de cette hypothèse, nous avons observé une surexpression constante de HMGA2 dans les TLA/LBD et LDD avec amplification de HMGA2 et les lipomes avec remaniement de HMGA2. Dans un cas de lipomatose, hypertrophie pathologique du tissu adipeux sans anomalie du gène HMGA2, une surexpression de HMGA2 était associée à une inhibition de l’expression de plusieurs microARN let-7. En revanche, nos travaux ne sont pas en faveur d’un rôle prépondérant des microARN let7 dans la surexpression de HMGA2 dans les TA. Nous nous sommes également intéressés aux gènes partenaires de fusion avec HMGA2 dans les lipomes et avons notamment identifié une nouvelle fusion impliquant PPAP2B (Phosphatidic Acid Phosphatase type 2B) localisé en 1p32. Nous avons aussi confirmé le rôle du gène NFIB (9p22) dans les lipomes. Enfin, nous avons établi des corrélations pronostiques dans une grande série de 116 TLA/LBD et LDD : l’amplification de HMGA2 était associée à l’histotype TLA/LBD et à une survie longue alors que les amplifications de CDK4 et JUN sont associées au type LDD et une survie courte. Ainsi, nos données confortent l’hypothèse d’un rôle précoce et majeur de HMGA2 dans la genèse des TA bien différenciées.

Background Rhabdomyolysis is an uncommon side effect of trabectedin which is used for the second line therapy of metastatic sarcoma after anthracycline and ifosfamide failure. This side effect may be due to pharmacokinetic interactions caused by shared mechanisms of metabolism involving the cytochrome P450 (CYP) system in the liver. Here, for the first time in literature, we describe the unexpected onset of heavy toxicity, including rhabdomyolysis, after the fourth course of trabectedin in a patient with retroperitoneal liposarcoma who at the same time was taking an alternative herbal medicine suspected of triggering this adverse event. Case presentation This is the case of a 56 year old Caucasian man affected by a relapsed de-differentiated liposarcoma who, after the fourth cycle of second-line chemotherapy with trabectedin, complained of sudden weakness, difficulty walking and diffuse muscle pain necessitating complete bed rest. Upon admission to our ward the patient showed grade (G) 4 pancytopenia and a marked increase in liver lytic enzymes, serum levels of myoglobin, creatine phosphokinase (CPK) and lactate dehydrogenase. No cardiac or kidney function injuries were present. Based on these clinical and laboratory features, our conclusive diagnosis was of rhabdomyolysis induced by trabectedin. The patient did not report any trauma or muscular overexertion and no co-morbidities were present. He had not received any drugs during treatment with trabectedin, but upon further questioning the patient informed us he had been taking a folk medicine preparation of chokeberry (Aronia melanocarpa) daily during the last course of trabectedin and in the 2 subsequent weeks. One week after hospitalization and cessation of intake of chokeberry extract, CPK and other markers of myolysis slowly returned to standard range, and the patient noted a progressive recovery of muscle strength. The patient was discharged on day 14 when a blood transfusion and parenteral hydration gradually lowered general toxicity. Progressive mobilization of the patient was obtained as well as a complete normalization of the laboratory findings. Conclusions The level of evidence of drug interaction leading to the adverse event observed in our patient was 2 (probable). Thus our case underlines the importance of understanding rare treatment-related toxicities such as trabectedin-induced rhabdomyolysis and the possible role of the drug-drug interactions in the pathogenesis of this rare side effect. Furthermore, this report draws attention to a potential problem of particular concern, that of nutritional supplements and complementary and alternative drug interactions. These are not widely recognized and can cause treatment failure.

Background To investigate the expression of E-cadherin, beta-catenin and topoisomerase-II alpha and examine their clinical relevance in liposarcomas. Materials and methods The expression of E-cadherin, beta-catenin and topoisomerase II alpha was examined immunohistochemically on formalin-fixed paraffin-embedded tissue specimens from 71 patients who underwent surgical treatment for liposarcomas of the extremities or the retroperitoneum in two major cancer reference centres between 1990 and 2000. Detailed medical notes were available for all patients who were followed for median 82 months (range 5 to 215 months). Obtained expression data were weighted against clinical and pathology parameters of clinical relevance. Results Patients were mostly male (59%), median age was 56 years for the liposarcomas of the extremities and 60 years for the retroperitoneal liposarcomas. The tumours were of diverse histology, grade and size (median diameters 7 and 17 cm for tumours of the extremities and retroperitoneum respectively). Expression of β-catenin protein was weakly detected in 15 cases (21.1%). Similarly weak expression of topoisomerase II-alpha was detected in 14 (19.7%) cases of which only two had more than 20% of tumor cells stained positive. E-cadherin was not detected in the studied cohort of liposarcomas. We did not detect associations between the expression of the above proteins by liposarcoma cells and clinical outcome. Conclusions Liposarcomas do not express E-cadherin, which matches the absence of epithelioid differentiation in this sarcoma subtype, and have low topoisomerase II-alpha expression, which justifies to some extend their resistance to anthracycline-based chemotherapy.

The present work occupies itself with the immunhistochemical reaction patterns of three new antibodies against lipid-associated proteins. Adipophilin (ADFP) is a protein, that in undifferentiated adipocytes, but, to find also in numerous other cell types. TIP 47 is a protein, that also found in different cells. Perilipin can be found only in adipocytes and steroidogenic cells. It was examined the occurrence and behavior of adipophilin, perilipin and tip47 in human normal tissues, in pathologically changed tissues, in liposarcomas and in other soft tissue sarcomas. The immunhistochemical examination took place with the ABC-technique at paraffinembedded material. Liposarcomas usually were most positive in different size, for adipophilin. Perilipin-positive were 70% of the liposarcomas and all others soft tissues sarcomas were negative. With the antibody agaimst tip 47 the reaction were different. For the first time, in the present study could be characterized three new lipid-associated antigens in liposarcomas and other soft tissues sarcomas., Die vorliegende Arbeit beschäftigt sich mit den immunhistochemischen Reaktionsmustern von neuen Antikörpern gegen drei lipid-assoziierte Proteine. Adipophili (ADFP) ist ein Protein, das in undifferenzierten Adipozyten, aber auch in zahlreichen anderen Zelltypen zu finden ist. TIP 47 ist ein dem Adipophilin sehr nahe stehendes Protein, das ebenfalls in verschiedenen Zellen angetroffen wird. Perilipin lässt sich dagegen nur in Adipozyten und steroidhaltigen Zellen finden. Untersucht wude das Vorkommen und Verhalten von Adipophilin, Perilipin und TIP 47 in normalen menschlichen, in pathologisch veränderten Gewebe, in Liposarkomen und anderen nicht-lipomatösen Weichteilsarkomen. Die immunhistochemische Untersuchung erfolgte mit der ABC-Methode an parafineingebettetem Material. Die meisten Liposarkome waren in unterschiedlichen Ausmaß positiv für Adipophilin. Perilipin-positiv waren 70% der Liposarkome, alle nicht-lipomatösen Weichteilsarkome waren negativ. Mit dem Antikörper gegen TIP 47 fand sich eine unterschiedliche Reaktion. In der vorliegenden Studie konnte erstmals das Vorkommen von drei neuen lipid-assoziierten Antigenen in Liposarkomen und anderen nicht- lipomatösen Weichteilsarkomen charakterisiert werden.

Summary Objective To describe the MRI features of liposarcomas of the extremities and correlate them with data from the histologic subtypes. Material and methods Retrospective study of 20 cases of liposarcoma of the extremities identified on MRI, surgically removed and confirmed at pathology. On MRI examination, T1- and T2-weighted fat-suppressed and non-fat-suppressed images were acquired then gadolinium-enhanced fat-suppressed T1-weighted images were obtained in at least two orthogonal planes. Results Sixteen female and four male patients aged 12 to 77 years old at presentation, in 16 cases, with a slowly enlarging painless mass (demonstrating no associated local or general inflammatory components), located in the lower extremity (16 cases) and predominantly located in the thigh (13 cases). Pathologic examination revealed three cases of well-differentiated liposarcoma, 12 cases of myxoid liposarcoma one of which with round cells, three cases of pleomorphic liposarcoma, one case of dedifferentiated liposarcoma and one case of mixed-type liposarcoma. MR images mostly showed well-circumscribed tumors (19 cases) . Well-differentiated liposarcomas typically demonstrated a very specific diagnostic appearance as a predominantly adipose mass containing nonlipomatous components seen as thick septa that may show nodularity. Other subtypes of liposarcoma demonstrated a small amount of adipose tissue thus producing a marbled textural pattern on T1-weighted images particularly in myxoid liposarcomas (9 cases), or even nonlipomatous elements in high-grade liposarcomas (in round cell liposarcoma and in two out of three pleomorphic liposarcomas). The myxoid subtype has also a relatively characteristic appearance as a low signal intensity noted on T1-weighted images and a marked high signal intensity on T2. The dedifferentiated liposarcoma has a very specific radiologic appearance as a nonlipomatous component within a predominantly adipose mass, simulating that of well-differentiated liposarcomas. Conclusion MRI of extremity liposarcomas is a highly reliable and sensitive method to characterize liposarcomas. Besides its value in the diagnosis of liposarcoma and locoregional extension control, it allows proper identification of the specific histologic subtypes of liposarcoma. Level of evidence Level IV. Retrospective diagnostic study.