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Arnaud Jannin, Stéphanie Espiard, Kanza Benomar, Christine Do Cao, Bénédicte Mycinski, Henri Porte, Michèle D'Herbomez, Nicolas Penel, Marie-Christine Vantyghem
The purpose of this study was to analyse the characteristics of 6 patients managed in a university hospital between 1996 and 2016 for non-islet cell tumor hypoglycemia (NICTH), a form of hypoglycaemia due to the paraneoplastic secretion of IGF-2 or its related substances. RESULTS: Three of these 6 patients (50%), aged over 69 years, including 2 with acromegaloid phenotype, presented with a pleural solitary fibrous tumor (SFT), with median diameter 20 cm (interquartile range, 12.5-20.5) with a low median SUV (3...
March 16, 2018: Annales D'endocrinologie
Alessandro De Vita, Federica Recine, Laura Mercatali, Giacomo Miserocchi, Chiara Liverani, Chiara Spadazzi, Roberto Casadei, Alberto Bongiovanni, Federica Pieri, Nada Riva, Dino Amadori, Toni Ibrahim
Background: Myxofibrosarcoma (MFS), formerly considered as a myxoid variant of malignant fibrous histiocytoma, is the most common sarcoma of the extremities in adults and is characterized by a high frequency of local recurrence. The clinical behavior of MFS is unpredictable and the efficacy of chemotherapy is still not well documented. Furthermore, given the relatively recent recognition of MFS as a distinct pathologic entity its cellular and molecular biology has still not been extensively studied in patient-derived preclinical models...
December 2017: Therapeutic Advances in Medical Oncology
Ji-Gang Wang, Baolei Wang, Yanjiao Hu, Ji-Hua Liu, Bing Liu, Hui Liu, Peng Zhao, Lijuan Zhang, Yu-Jun Li
Primary cardiac tumors are uncommon, and the majority of them are benign which are curable but can cause significant morbidity if not diagnosed and treated in a timely fashion. The objective of this study was to review the clinicopathologic features and surgical outcomes of patients with primary cardiac tumors in a single medical center in China. We have retrospectively reviewed 212 consecutive adult patients who underwent surgical resection of primary cardiac tumors at our center from January of 2001 to June of 2017...
January 6, 2018: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Heng Zhang, Da Zhu, Li Dong
Myxofibrosarcoma is a rare type of highly malignant sarcoma which easily occur lung metastasis. We report a rare metastatic myxofibrosarcoma which generated from the upper and middle lobe of the lung, invading the right superior pulmonary vein, extending to the left atrium. A single imaging examination may lead to misdiagnosis, so the combination of computed tomography imaging, magnetic resonance imaging, as well as positron emission tomographic scan are necessary for perioperative diagnosis and surgical planning...
February 2018: Echocardiography
Filippo Badaloni, Eugenio Pozzati, Gianluca Marucci, Pietro Fiaschi, Antonio Fioravanti
Herein we describe the case of a young patient who presented with a recent history of epilepsy due to multiple brain lesions; he did not complain about any cardiopulmonary impairments. The patient died as a consequence of hemorrhagic progression of brain metastatic disease. Regardless of a thorough investigation, the heart tumor remained occult. Primary cardiac tumors are very rare entities. Most of these are benign, but approximately 25% are malignant, and the majority of these are sarcomas. Myxofibrosarcoma and osteosarcoma are exceptionally rare...
November 21, 2017: Curēus
Haa-Na Song, Min Gyu Kang, Jeong Rang Park, Jin-Yong Hwang, Jung Hun Kang, Won Seop Lee, Gyeong-Won Lee
Myxofibrosarcoma is a rare tumor, refractory to cytotoxic chemotherapy and radiotherapy. Pembrolizumab is an innovative immunotherapy drug consisting of programmed death ligand receptor-1 (PD-L1) antibody proven to be useful for numerous types of cancer cells. A patient had been diagnosed with metastatic myxofibrosarcoma, refractory to radiotherapy and conventional cytotoxic chemotherapy. The patient achieved a partial response during palliative chemotherapy with pembrolizumab for 14 cycles. To the best of our knowledge, this is the first case report demonstrating the efficacy of pembrolizumab for refractory myxofibrosarcoma...
January 22, 2018: Cancer Research and Treatment: Official Journal of Korean Cancer Association
Nzechukwu Zimudo Ikeri, Andrea Oludolapo Akinjo, Olugbende O Ajayi, Adekunbiola Aina Fehintola Banjo
OBJECTIVE: There is scanty data on histologically diagnosed soft-tissue sarcomas in the Nigerian literature. This is due to paucity of facilities for ancillary testing as well as a dearth of specialist soft tissue pathologists. Knowledge however of the common soft-tissue sarcomas is vital for the establishment of an effective sarcoma service. The aim of this study, therefore, was to determine the histological spectrum of soft-tissue sarcomas in Lagos, Nigeria. MATERIALS AND METHODS: Archival haematoxylin and eosin (H and E)-stained slides were retrieved and reviewed by a team of soft-tissue pathologists at the Royal National Orthopaedic Hospital, London, UK...
October 2017: Nigerian Postgraduate Medical Journal
Jong Hui Suh, Do Yeon Kim, Jeong Seob Yoon, Eun Su Park, Chan Beom Park
We report an unusual malignant cardiac neoplasm that initially presented as pulmonary thromboembolism in a 78-year-old male. Despite anticoagulation, the pulmonary artery lesion progressed and a mass-like lesion developed in the right ventricular outflow tract. Venoarterial extracorporeal membrane oxygenation was applied before surgery due to the presence of severe right-side heart failure with pulmonary hypertension. A round mass was found in the right ventricle and separate lobulated masses were found in both pulmonary arteries, and these were consistent with low-grade myxofibrosarcoma...
December 2017: Journal of Thoracic Disease
Muhammad Shoaib Khan, Sahadev Reddy, Richard Lombardi, Pitti Isabel, Walter E Mcgregor, Bang Tang, George Gabriel, Robert W Biederman
Left atrial appendage mass can occasionally pose a serious challenge to physicians to identify the nature of the mass with the aid of imaging techniques. We present a case of 67-year-old man, who was evaluated for suspected left atria myxoma. Transesophageal echocardiography revealed a heterogeneous density originating from left atrial appendage, thought to be most consistent with a myxoma. Cardiac magnetic resonance imaging, uncharacteristically, gave an equivocal picture, suggesting the mass to be a myxoma on initial imaging and a thrombus with evidence of liquefaction necrosis following postcontrast enhancement...
December 27, 2017: Echocardiography
Brigitte C Widemann, Antoine Italiano
Undifferentiated pleomorphic sarcomas, myxofibrosarcomas, and malignant peripheral nerve sheath tumors are characterized by complex genomic characteristics and aggressive clinical behavior. Recent advances in the understanding of the pathogenesis of these tumors may allow for the development of more-effective innovative therapeutic strategies, including immunotherapies. This review describes the current knowledge of the epidemiology, clinical presentation, treatment, and pathogenesis of these tumors and highlights ongoing and future research...
January 10, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
B Joel Tjarks, Jennifer S Ko, Steven D Billings
BACKGROUND: Myxofibrosarcoma classically presents as a painless mass in the proximal extremities. Cutaneous myxofibrosarcomas arising in the head and neck and distal extremities are extremely uncommon. We present a series of 6 cases of myxofibrosarcoma presenting in the head and neck and acral locations. METHODS: Archives were searched using the term "myxofibrosarcoma" over a 6-year period (2009-2015). The clinicopathologic features of myxofibrosarcoma were recorded...
February 2018: Journal of Cutaneous Pathology
D-P Wu, X-B Yan, L-G Liu, C Tian, K Han, H Zhang, D-L Min
OBJECTIVE: Topoisomerase IIβ binding protein 1 (TopBP1) is involved in DNA damage and replication checkpoint and has been shown to be related to tumorigenesis in many cancer types. This study aimed to evaluate the biological role and clinicopathological significance of TopBP1 in OS. PATIENTS AND METHODS: TopBP1 expression in sarcoma patients was determined through the Oncomine database, and the prognostic role of TopBP1 expression was assessed in a retrospective cohort study...
September 2017: European Review for Medical and Pharmacological Sciences
Anastasiya Quimby, Abigail Estelle, Arun Gopinath, Rui Fernandes
Myxofibrosarcoma (MFS) is a malignant fibroblastic tumor that primarily affects the lower and upper extremities. It is usually described as a slow-growing tumor with high recurrence rates but low metastatic potential. The reported incidence of head and neck MFS is 2 to 4% and rarely presents with distant metastases. This report describes a case of maxillary MFS in a 72-year-old woman whose disease progression followed an atypical course with an extremely rapid rate of growth and early pulmonary and central nervous system lesions...
December 2017: Journal of Oral and Maxillofacial Surgery
Jeremy Lewin, Swati Garg, Beatrice Y Lau, Brendan C Dickson, Frank Traub, Nalan Gokgoz, Anthony M Griffin, Peter C Ferguson, Irene L Andrulis, Hao-Wen Sim, Suzanne Kamel-Reid, Tracy L Stockley, Lillian L Siu, Jay S Wunder, Albiruni R A Razak
There are limited data regarding the molecular characterization of undifferentiated pleomorphic sarcomas (UPS; formerly malignant fibrous histiocytoma). This study aimed to investigate the utility of next generation sequencing (NGS) in UPS to identify subsets of patients who harbour actionable mutations. Patients diagnosed with UPS underwent pathological re-evaluation by a pathologist specializing in sarcoma. Tumor DNA was isolated from archived fresh frozen tissue samples and genotyped using NGS with the Illumina MiSeq TruSeq Amplicon Cancer Panel (48 genes, 212 amplicons)...
January 1, 2018: International Journal of Cancer. Journal International du Cancer
Nicholas Latchana, Vincent C Daniel, Robert W Gould, Raphael E Pollock
BACKGROUND: Tumor embolisms (TE) are an underappreciated source of pulmonary embolisms in sarcoma. Most evidence in the literature is limited to case reports and none have described the presence of TE secondary to myxofibrosarcoma. We report the first case of myxofibrosarcoma TE and perform a review of the literature for TE secondary to bone and soft tissue sarcomas (STS). CASE PRESENTATION: A 36-year-old female presented with debilitating pain of the right upper extremity secondary to a recurrent soft tissue sarcoma...
August 30, 2017: World Journal of Surgical Oncology
Vittoria Colia, Marco Fiore, Salvatore Provenzano, Elena Fumagalli, Rossella Bertulli, Carlo Morosi, Angelo P Dei Tos, Marta Barisella, Alessandro Gronchi, Paolo G Casali, Roberta Sanfilippo
BACKGROUND: We report on the activity of anthracycline-based and high-dose prolonged-infusion ifosfamide chemotherapy in a retrospective series of patients affected by advanced myxofibrosarcoma treated at Istituto Nazionale Tumori in Milan, Italy, and within the Italian Rare Cancer Network (RTR). METHODS: Advanced myxofibrosarcoma patients treated with anthracycline + ifosfamide and high-dose prolonged-infusion ifosfamide as a single agent from November 2001 to December 2016 were retrospectively reviewed...
2017: Clinical Sarcoma Research
Kemal Kösemehmetoğlu, Ece Özoğul, Berrin Babaoğlu, Gaye Güler Tezel, Gökhan Gedikoğlu
OBJECTIVE: Programmed death ligand 1 (PD-L1) found on tumor cells has recently been reported to have a key role in the development and dissemination of many tumors, such as lung and breast carcinomas. In this study, we retrospectively analyzed PD-L1 expression among different types of sarcomas. MATERIAL AND METHOD: Tissue microarrays of 3-4 mm diameter were composed from paraffin blocks of 222 various sarcomas. Slides prepared from microarrays were stained for PD-L1 antibody (Cell Signaling, E1L3N®) using Leica Bond Autostainer...
2017: Türk Patoloji Dergisi
Hiroyuki Tsuchie, Mitsunori Kaya, Hiroyuki Nagasawa, Makoto Emori, Yasutaka Murahashi, Emi Mizushima, Naohisa Miyakoshi, Toshihiko Yamashita, Yoichi Shimada
PURPOSE: A clinical feature of myxofibrosarcoma is local recurrence, but knowledge about distant metastasis is sparse. We evaluated the tendency of clinical and histological features of metastasis in myxofibrosarcoma patients. METHODS: Fifty-eight patients with myxofibrosarcoma were treated in our hospitals, and a total of 16 consecutive patients with distant metastases were included in this retrospective study (9 males and 7 females, with a mean age of 77 years)...
August 2017: Upsala Journal of Medical Sciences
Ellen Heitzer, Sandra Sunitsch, Magdalena M Gilg, Birgit Lohberger, Beate Rinner, Karl Kashofer, Nicole Stündl, Peter Ulz, Joanna Szkandera, Andreas Leithner, Bernadette Liegl-Atzwanger
Myxofibrosarcomas are morphologically heterogeneous soft tissue sarcomas lacking a specific immunohistochemical expression profile and recurrent genetic changes. The study was designed to gain further insights into the molecular landscape of myxofibrosarcomas by targeted re-sequencing of known cancer driver hotspot mutations and the analysis of genomewide somatic copy number alterations. A well-defined group of myxofibrosarcomas, including myxofibrosarcomas G1 (n=6), myxofibrosarcomas G3 (n=7), myxofibrosarcomas with morphologically heterogeneous and independently selectable G1 and G3 areas within a tumor (n=8), and myxofibrosarcomas G3 with subsequent tumor recurrence (n=1) or metastatic disease (n=3) were evaluated...
December 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Neha Gupta, Samuel Kenan, Leonard B Kahn
Synovial sarcoma is a high-grade sarcoma commonly affecting young adults. The sites of involvement include soft tissue near joints, lung, pleura, mediastinum, larynx, kidney, and buttocks. Histologic types include monophasic, biphasic, and undifferentiated. We report a unique case of synovial sarcoma with low-grade histologic features mimicking pleomorphic hyalinizing angiectatic tumor (PHAT) with indolent behavior for a period of 10 years. The tumor showed angiectatic blood vessels with fibrinous cuffing, hypocellular and hypercellular spindle cell areas with rare mitoses, and focal atypia in a myxoid background...
August 1, 2017: International Journal of Surgical Pathology
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