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Mesenchymal chondrosarcoma

Min Hyun Cho, Sung Han Kim, Weon Seo Park, Jae Young Joung, Ho Kyung Seo, Jinsoo Chung, Kang Hyun Lee
BACKGROUND: Sarcomatoid urothelial carcinoma (SUC) is a rare malignant neoplasm of the urinary bladder comprising 0.2-0.6 % of all histological bladder tumor subtypes. It presents as a high-stage malignancy and exhibits aggressive biological behavior, regardless of the treatment employed. It is defined as histologically indistinguishable from sarcoma and as a high-grade biphasic neoplasm with malignant epithelial and mesenchymal components. The mean age of patients presenting with SUC is 66 years, and the male-to-female ratio is 3:1...
October 20, 2016: World Journal of Surgical Oncology
Petra Balogh, Rita Bánusz, Monika Csóka, Zsófia Váradi, Edit Varga, Zoltán Sápi
BACKGROUND: Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin and comprises the largest category of soft-tissue sarcomas both in children and adolescents. From a pediatric oncology point of view, RMS has traditionally been classified into alveolar (ARMS) and embryonal (ERMS) subtypes. The anatomical localization of the tumor may vary, but commonly involve the head/neck regions, male and female urogenital tract or the trunk and extremities. CASE PRESENTATION: Here, we report two challenging cases involving 17- and 9-years-olds males where diffuse and multiplex bone lesions suggested either a hematological disease or a primary bone tumor (mesenchymal chondrosarcoma)...
October 18, 2016: Diagnostic Pathology
Sumit Majumdar, Rajyalakshmi Boddepalli, Divya Uppala, A Kameswara Rao
Mesenchymal chondrosarcomas (MC) are rare and aggressive forms of chondrosarcoma. They are distinct tumors arising in unicentric or multicentric locations from both skeletal and extraskeletal tissues. The most affected region is the facial skeleton, especially the jaws. In this report, we present a case of MC primarily involving the mandible in a 60-year-old female patient.
September 2016: Journal of Oral and Maxillofacial Pathology: JOMFP
Nihat Demirhan Demirkiran, Olcay Akdeniz, Onur Hapa, Hasan Havıtçıoğlu
INTRODUCTION: Arthroscopic fixation of tibial spine fracture without damage to the growth plate is very important in patients with open physis. The present article describes a simple and effective technique being used for the first time to treat this condition. CASE REPORT: A 16-year-old boy sustained avulsion fractures of tibial spine while playing. He was treated arthroscopically with excellent result. CONCLUSION: Arthroscopic fixation of tibial spine fracture in patients with open physis with two cannulated screws perpendicular to each other is a very simple technique which provides strong construct, and allows early mobilization without risk of damage to the growth plate...
April 2016: Journal of Orthopaedic Case Reports
Chenthuran Deivaraju, H Thomas Temple, Norman Block, Philip Robinson, Andrew V Schally
AIM: Luteinizing hormone releasing hormone (LHRH) is a neurohormone, secreted by the hypothalamus, which regulates the secretion of gonadotropins, luteinizing hormone (LH) and follicle stimulating hormone (FSH) from the pituitary. LHRH acts by binding to receptors located in the pituitary gland. These receptors (LHRH receptors) have also been found in the cytoplasm of many tumor cells that involve both the reproductive and non-reproductive organs. These receptors have been demonstrated in prostate and breast cancers, endometrial carcinomas, renal cell carcinoma, lymphoma, carcinoma of liver, pancreas and skin...
August 17, 2016: Hormone Molecular Biology and Clinical Investigation
Yvonne de Jong, Annemiek M van Maldegem, Adrian Marino-Enriquez, Danielle de Jong, Johnny Suijker, Inge H Briaire-de Bruijn, Alwine B Kruisselbrink, Anne-Marie Cleton-Jansen, Karoly Szuhai, Hans Gelderblom, Jonathan A Fletcher, Judith V M G Bovée
Mesenchymal chondrosarcomas are rare and highly aggressive sarcomas occurring in bone and soft tissue, with poor overall survival. Bcl-2 expression was previously shown to be upregulated in mesenchymal chondrosarcomas. We here report on a newly derived mesenchymal chondrosarcoma cell line, MCS170, in which we investigated treatment with the BH3 mimetic ABT-737 alone or in combination with conventional chemotherapy as a possible new therapeutic strategy. The presence of the characteristic HEY1-NCOA2 fusion was confirmed in the MCS170 cell line using FISH, RT-PCR, and sequencing...
October 2016: Laboratory Investigation; a Journal of Technical Methods and Pathology
Min-Huan Wu, Pei-Han Huang, Mingli Hsieh, Chun-Hao Tsai, Hsien-Te Chen, Chih-Hsin Tang
Chondrosarcoma is a malignant tumor of mesenchymal origin predominantly composed of cartilage-producing cells. This type of bone cancer is extremely resistant to radiotherapy and chemotherapy. Surgical resection is the primary treatment, but is often difficult and not always practical for metastatic disease, so more effective treatments are needed. In particular, it would be helpful to identify molecular markers as targets for therapeutic intervention. Endothelin-1 (ET-1), a potent vasoconstrictor, has been shown to enhance chondrosarcoma angiogenesis and metastasis...
September 2, 2016: Oncotarget
N V Vasil'ev
Lymph node metastasis of osteosarcomas is a rather rare phenomenon; according to different authors, the incidence of lymph node metastasis is 4 to 11%. The detection of lymph node metastases in osteosarcoma is associated with a significant reduction in the 5-year survival of patients and allows its classification as clinical stage IV tumor. The risk factors for lymph node metastases in patients with bone sarcomas are age (≥64 years), gender (female), nosological entity (undifferentiated pleomorphic sarcoma, osteosarcoma, chondrosarcoma), tumor depth (muscle, bone), and the size of primary tumor (>5 сm)...
July 2016: Arkhiv Patologii
Xiangmin Shi, Zhuo Liang, Jian Li, Jianping Guo, Zhaoliang Shan, Yutang Wang
Ventricular tachycardia (VT) and premature contraction originating from the right ventricular outflow tract (RVOT) usually appear in healthy individuals. Radiofrequency ablation (RFA) is highly effective at resolving this type of arrhythmia. Refractory VT of RVOT is uncommon and occasionally results from cardiac metastasis of extraskeletal mesenchymal chondrosarcomas (ESMC). ESMC is a rare malignant tumor arising from soft tissues. The current study presents the case of a 25-year-old male with severe VT arising from RVOT due to metastasis of an ESMC that originally occurred in the retroperitoneum...
September 2016: Experimental and Therapeutic Medicine
Xing Bao, Tingting Ren, Yi Huang, Shidong Wang, Fan Zhang, Kuisheng Liu, Bingxin Zheng, Wei Guo
BACKGROUND: In addition to treating acute promyelocytic leukemia, arsenic trioxide (ATO) suppresses other solid tumors, including chondrosarcoma. However, the effects of ATO on metastasis in chondrosarcoma cells, and the underlying molecular mechanisms remain unclear. METHODS: The effects of ATO on the migratory and invasive capacities of chondrosarcoma cells were investigated by Wound healing, Transwell and EMT assays. The expression of miR-125b in human chondrosarcoma tissues and cell lines was detected by real-time PCR analysis...
2016: Journal of Experimental & Clinical Cancer Research: CR
Nishanth Sadashiva, Anil Sharma, Dhaval Shukla, Poyuran Rajalakshmi, Anita Mahadevan, Bhagavatula Indira Devi
BACKGROUND: Intracranial extraskeletal mesenchymal chondrosarcomas (MCSs) are rare tumors accounting for <0.16% of intracranial tumors. They are usually described as occurring in the age group of 20-30 years and are commonly found in the frontoparietal region arising from the falx and surrounding dura. We describe 3 cases at varying ages, locations, and outcomes. CASE DESCRIPTION: A 42-year-old woman with anterior one third falx-based lesion; a 7-year-old boy with mid one third falcine lesion with hyperostosis of bone, presenting in comatose stage; and a 52-year-old woman with left posterior lateral frontal dural-based lesion are presented...
August 23, 2016: World Neurosurgery
Jarish N Cohen, David A Solomon, Andrew E Horvai, Sanjay Kakar
Mesenchymal chondrosarcoma (MC) is an aggressive small round blue cell tumor with chondrogenic differentiation that typically arises in bony sites. Approximately a third of these tumors develop in extra-skeletal sites such as the meninges, and somatic soft tissue. MCs are well-circumscribed, lobulated masses, with focal calcification. Histologically, two distinct populations of neoplastic cells characterize MC: sheets of primitive small, round, blue cells surrounding islands of well-developed hyaline cartilage with mature chondrocytes in lacunae...
August 17, 2016: Human Pathology
Adrián Mariño-Enríquez, Judith V M G Bovée
Sarcomas are infrequent mesenchymal neoplasms characterized by notable morphological and molecular heterogeneity. Molecular studies in sarcoma provide refinements to morphologic classification, and contribute diagnostic information (frequently), prognostic stratification (rarely) and predict therapeutic response (occasionally). Herein, we summarize the major molecular mechanisms underlying sarcoma pathogenesis and present clinically useful diagnostic, prognostic and predictive molecular markers for sarcoma...
September 2016: Surgical Pathology Clinics
Manisha Sharma, Manas Madan, Mridu Manjari, Harleen Kaur
Chondrosarcoma is a mesenchymal tumor composed of tumor cells producing cartilage. It is more common in older age and often affects the axial skeleton. We report a rare case of chondrosarcoma mimicking a sellar and suprasellar mass with parasellar extension. A 40 yr woman presented with decreasing visual acuity and headache. Magnetic resonance (MR) image revealed a cystic sellar and suprasellar mass with parasellar extension showing mild enhancing solid component. It favored the diagnosis of craniopharyngioma...
2016: Iranian Journal of Pathology
Shudong Chen, Yufeng Wang, Guoyi Su, Bolai Chen, Dingkun Lin
BACKGROUND: Mesenchymal chondrosarcoma is a rare malignant tumor arising from bone or soft tissues. Instraspinal dumbbell-shaped mesenchymal chondrosarcoma is even rarer; however, it should not be neglected by clinicians. CASE PRESENTATION: A 26-year-old female was referred to our hospital with a 1.5-month history of sciatic pain and numbness in the left leg. Computed tomography and magnetic resonance imaging scans revealed an intraspinal dumbbell-shaped mass which had distinguishing features of neurogenic tumors, surprisingly, with massive calcifications, and no tumor metastasis was found...
2016: World Journal of Surgical Oncology
Yin P Hung, Christopher Dm Fletcher, Jason L Hornick
A distinct subset of round cell sarcomas harbors capicua transcriptional repressor (CIC) rearrangement. Diagnosing these sarcomas can be difficult owing to their resemblance to Ewing sarcoma and other 'small round blue cell tumors'; molecular techniques are generally required. Recent gene expression studies of CIC-rearranged sarcomas identified the upregulation of ETV4. We assessed the sensitivity and specificity of ETV4 and WT1 immunohistochemistry for CIC-rearranged sarcoma. We evaluated whole-tissue sections from 40 CIC-rearranged sarcomas, 40 Ewing sarcomas, 4 BCOR-CCNB3 sarcomas, 6 unclassified round cell sarcomas, and 150 histologic mimics...
July 22, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Hideo Morioka, Shunji Takahashi, Nobuhito Araki, Hideshi Sugiura, Takafumi Ueda, Mitsuru Takahashi, Tsukasa Yonemoto, Hiroaki Hiraga, Toru Hiruma, Toshiyuki Kunisada, Akihiko Matsumine, Michiro Susa, Robert Nakayama, Kazumasa Nishimoto, Kazutaka Kikuta, Keisuke Horiuchi, Akira Kawai
BACKGROUND: Trabectedin is reported to be particularly effective against translocation-related sarcoma. Recently, a randomized phase 2 study in patients with translocation-related sarcomas unresponsive or intolerable to standard chemotherapy was conducted, which showed clinical benefit of trabectedin compared with best supportive care (BSC). Extraskeletal myxoid chondrosarcoma (EMCS) and Mesenchymal chondrosarcoma (MCS) are very rare malignant soft tissue sarcomas, and are associated with translocations resulting in fusion genes...
2016: BMC Cancer
Pan Ma, Wenjuan Yan, Ye Tian, Jingya Wang, Jian Q Feng, Chunlin Qin, Yi-Shing Lisa Cheng, Xiaofang Wang
During endochondral ossification, chondrocytes embed themselves in a proteoglycan-rich matrix during the proliferation-maturation transition. Accumulating evidence shows that proteoglycans are essential components for chondrocyte proliferation and differentiation. When we conditionally inactivated FAM20B (Family with sequence similarity 20 member-B), which is a newly identified xylose kinase essential for glycosaminoglycan (GAG) formation on the protein core of proteoglycans, from the dental mesenchyme using Osr2-Cre, which is also strongly expressed in joint cartilage, we found chondrosarcoma in the knee joint and remarkable defects of postnatal ossification in the long bones...
2016: Scientific Reports
Chan Kim, Eun Kyung Kim, Hun Jung, Hong Jae Chon, Jung Woo Han, Kyoo-Ho Shin, Hyuk Hu, Kyung Sik Kim, Young Deuk Choi, Sunghoon Kim, Young Han Lee, Jin-Suck Suh, Joong Bae Ahn, Hyun Cheol Chung, Sung Hoon Noh, Sun Young Rha, Soo Hee Kim, Hyo Song Kim
BACKGROUND: The PD-1/PD-L1 axis plays a paramount role in the immune escape of tumor cells by negative regulation of T-cell functions. The aim of the present study was to characterize the PD-L1 expression pattern and its clinical implication in soft-tissue sarcomas (STS). METHODS: We analyzed PD-L1 expression in 82 STS patients with 5 subtypes: rhabdomyosarcoma, synovial sarcoma, Ewing sarcoma, epithelioid sarcoma, and mesenchymal chondrosarcoma. RESULTS: The median age at diagnosis was 26 (range: 1-78) and the male to female ratio was 1...
2016: BMC Cancer
Vittoria Colia, Salvatore Provenzano, Nadia Hindi, Paolo G Casali, Silvia Stacchiotti
This review highlights the data currently available on the activity of systemic therapy in chondrosarcoma, chordoma, giant cell tumour of the bone (GCTB) and solitary fibrous tumour, i.e., four rare sarcomas amongst mesenchymal malignancy arising from the skull base.
July 2016: Reports of Practical Oncology and Radiotherapy
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