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Adult rhabdomyosarcoma

Maureen O'Sullivan
Since its foundation by remarkably talented and insightful individuals, prominently including Pepper Dehner, pediatric soft tissue tumor pathology has developed at an immense rate. The morphologic classification of tumoral entities has extensively been corroborated, but has also evolved with refinement or realignment of these classifications, through accruing molecular data, with many derivative ancillary diagnostic assays now already well-established. Tumors of unclear histogenesis, classically morphologically undifferentiated, are prominent amongst pediatric sarcomas, however, the classes of undifferentiated round- or spindle-cell-tumors-not-otherwise-specified are being dismantled gradually with the identification of their molecular underpinnings...
September 5, 2016: Seminars in Diagnostic Pathology
Sila Datta, Jay Gopal Ray, Tushar Deb, Santanu Patsa
Rhabdomyosarcoma is the malignant neoplasm of striated muscle and a relatively uncommon tumor of the oral cavity. Embryonal variety is the most common subtype, observed in children below 10 years of age but occasionally seen in adolescents and young adults. The present report describes a case of embryonal rhabdomyosarcoma in the left posterior buccal mucosa, with extension in the adjacent alveolus, soft palate, oropharynx and nasopharynx of a 17-year-old female.
September 2016: Journal of Oral and Maxillofacial Pathology: JOMFP
Maryam Sadat Hosseini, Tahereh Ashrafganjoei, Ainaz Sourati, Morteza Tabatabeifar, Mahdiss Mohamadianamiri
INTRODUCTION: Rhabdomyosarcoma has known as a highly malignant soft tissue sarcoma. It has been the most common soft tissue sarcoma in childhood, accounting for about 3 to 4 % of all cases of childhood cancer. Rhabdomyosarcoma was rare in adults, accounting for 3% of all soft-tissue sarcomas. embryonal rhabdomyosarcoma of female genital tract including uterine cervix in an adult was rare. CASE PRESENTATION: This study has reported a 33-year-old woman presented with abnormal vaginal discharge...
June 2016: Iranian Journal of Cancer Prevention
C L Tinkle, I Fernandez-Pineda, Y Li, C H Hua, M D Neel, S C Kaste, T E Merchant, L E Kun, S L Spunt, M Krasin
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
A Raldow, A Jacobson, S Goldberg, H Wang, E Choy, G Cote, F J Hornicek, T F DeLaney, Y L E Chen
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
Richard Lock, Hernan Carol, John M Maris, E Anders Kolb, Richard Gorlick, C Patrick Reynolds, Min H Kang, Stephen T Keir, Jianrong Wu, Andrei Purmal, Andrei Gudkov, Dias Kurmashev, Raushan T Kurmasheva, Peter J Houghton, Malcolm A Smith
BACKGROUND: CBL0137 is a novel drug that modulates FAcilitates Chromatin Transcription (FACT), resulting in simultaneous nuclear factor-κB suppression, heat shock factor 1 suppression and p53 activation. CBL0137 has demonstrated antitumor effects in animal models of several adult cancers and neuroblastoma. PROCEDURES: CBL0137 was tested against the Pediatric Preclinical Testing Program (PPTP) in vitro cell line panel at concentrations ranging from 1.0 nM to 10...
September 21, 2016: Pediatric Blood & Cancer
Xinyan Wang, Liyuan Song, Vincent Chong, Yongzhe Wang, Jing Li, Junfang Xian
PURPOSE: To identify magnetic resonance imaging (MRI) features of sinonasal rhabdomyosarcoma in adults, including diffusion-weighted (DW) and dynamic contrast-enhanced (DCE) MRI features as compared with carcinomas. MATERIALS AND METHODS: Sixty-four patients were included in this study, including 12 sinonasal rhabdomyosarcomas and 52 sinonasal carcinomas. MRI was completed in all 64 patients with a 3T MR scanner. Conventional MR (nonenhanced and static contrast-enhanced) imaging features, DCE-MRI parameters, and the apparent diffusion coefficients (ADCs) were analyzed by two authors independently (X...
September 20, 2016: Journal of Magnetic Resonance Imaging: JMRI
Geneviève Bouchard-Fortier, Raymond H Kim, Lisa Allen, Abha Gupta, Taymaa May
OBJECTIVE: To report three cases of embryonal rhabdomyosarcoma (ERMS) of the cervix in young women successfully treated with fertility-sparing surgery and chemotherapy. METHODS: Between January 2014 and December 2015, three cases of ERMS of the cervix were confirmed in young women at a single tertiary cancer center. All cases were managed by a pediatric oncologist and a gynecologic oncologist with a combination of surgery and chemotherapy. Fertility-sparing surgeries (cervical conization or robotic-assisted radical trachelectomy) were offered to patients depending on the tumor size...
November 2016: Gynecologic Oncology Reports
B Febrero, I Oviedo, A Ríos, J M Rodríguez
INTRODUCTION: Rhabdomyoscarcoma (RMS) is the most common soft-part tumour in children, however, it is infrequent in adults. Thyroid involvement is exceptional and only four cases have been reported: two primary RMS in children and another two cases in young adults with RMS metastasis to the thyroid. We report the first case in the literature of a primary adult RMS with thyroid involvement and superior vena cava syndrome. CASE PRESENTATION: A 67-year-old male was admitted for an oedema of the arm extending to the laterocervical region...
August 29, 2016: European Annals of Otorhinolaryngology, Head and Neck Diseases
Andrea Ferrari, Uta Dirksen, Stefan Bielack
The definition of soft tissue and bone sarcomas include a large group of several heterogeneous subtypes of mesenchymal origin that may occur at any age. Among the different sarcomas, rhabdomyosarcoma, synovial sarcoma, Ewing sarcoma and osteosarcoma are aggressive high-grade malignancies that often arise in adolescents and young adults. Managing these malignancies in patients in this age bracket poses various clinical problems, also because different therapeutic approaches are sometimes adopted by pediatric and adult oncologists, even though they are dealing with the same condition...
2016: Progress in Tumor Research
Emmanuel Desandes, Daniel P Stark
To design the services for adolescents and young adults (AYAs) with cancer, we need to understand the patterns of disease and the other clinical and managerial challenges of the patient group. Cancer occurring between the ages of 15 and 39 years is 4 times less rare than cancer occurring during the first 15 years of life and consists of 2% of all invasive cancer in Europe, about 66,000 patients in Europe each year. AYAs have a unique distribution of cancer types, including the peak in incidence of Hodgkin lymphoma (HL) or germ cell tumors...
2016: Progress in Tumor Research
Ismail Yağmur, Burak Turna, Ali Tekin, Emir Akıncıoğlu, Bnu Sarsık, İbrahim Ulman
INTRODUCTION: Benign prostatic hyperplasia (BPH) is generally common in men older than 50 years of age but is extremely rare in childhood. In the literature to date, fewer than five cases have been reported under 18 years of age. Owing to the limited number of cases, the etiology and management of BPH in the first two decades is not clear. OBJECTIVE: We herein report a 17-year-old boy who presented with acute urinary retention due to BPH and was treated with endoscopic transurethral resection of the prostate (TUR-P)...
August 2016: Journal of Pediatric Urology
Robert J Canter
Since preoperative chemotherapy has been clearly shown to improve outcomes for patients with Ewing sarcoma, rhabdomyosarcoma, and osteosarcoma, practitioners have attempted to extend the use of adjuvant/neoadjuvant chemotherapy to other types of adult soft tissue sarcoma. Given the high risk of distant recurrence and disease-specific death for patients with soft tissue sarcoma tumors larger than 10 cm, these patients should be considered candidates for neoadjuvant chemotherapy as well as investigational therapies...
October 2016: Surgical Oncology Clinics of North America
Xiaolong Yu, Yong Yang, Bin Zhang, Hucheng Liu, Runsheng Guo, Min Dai
Pleomorphic rhabdomyosarcoma (PRMS) is a rare type of soft tissue tumor accounting for <2% of all adult sarcomas. The present study describes a case of a 28-year-old male patient with primary PRMS of the right thigh. The patient was initially diagnosed with a schwannoma and underwent conservative therapy at a local hospital. At the 6-month follow-up, the patient reported a marked increase in the size of the mass. Finally, the patient underwent fine-needle aspiration and total tumor resection. The tumor measured 11×9×5 cm(3) in size and was located in the vastus intermedius muscle...
September 2016: Oncology Letters
Vinodh Pillai, David M Dorfman
Many epithelial neoplasms can be analyzed by flow cytometry (FC), particularly from serous cavity effusion samples, using EpCAM, a cell adhesion molecule expressed on most normal epithelial cells and expressed at a higher level in most epithelial neoplasms. A simple 3-color flow cytometric panel can provide a high sensitivity and specificity compared to cytomorphology. FC provides more rapid immunophenotyping than conventional immunohistochemical staining, can identify rare malignant cells that could be missed by a cytological exam alone, and can be utilized to evaluate limited samples such as cerebrospinal fluid or fine-needle aspiration samples...
2016: Acta Cytologica
Mai Watanabe, Shin-Ichi Ansai, Itaru Iwakiri, Takaya Fukumoto, Masahiro Murakami
Rhabdomyosarcoma (RMS) is a malignant mesenchymal tumor usually occurring in children, young adults, and predominantly arising in the head and neck or deep soft tissue of the extremities. Pleomorphic RMS (PRMS) arising on subcutaneous tissue in an adult patient's trunk is extremely rare. We present a case of primary PRMS occurring on the back of a 50-year-old man. The tumor was diagnosed by histological and immunohistochemical findings of the resected specimen. One month after the surgery, axillary lymph node metastases were found...
August 29, 2016: Journal of Dermatology
N Sood, N Sehrawat
Rhabdomyosarcoma (RMS) is a fast growing, malignant tumour arising from immature mesenchymal cells, committed to skeletal muscle differentiation. It is more often seen in the paediatric population and constitutes less than 1% of all malignancies and less than 3% of all soft tissue tumours. RMS of the paranasal sinuses constitutes 10-15% of adult head and neck RMS, ethmoidal and maxillary sinuses being the most common. We report a 56-year-oldman presenting with left nasal obstruction, epistaxis on and off and left cheek swelling...
August 2016: Malaysian Journal of Pathology
Kenya Kobayashi, Fumihiko Matsumoto, Makoto Kodaira, Taisuke Mori, Naoya Murakami, Akihiko Yoshida, Daisuke Maki, Masanori Teshima, Masahiko Fukasawa, Jun Itami, Masahiro Asai, Seiichi Yoshimoto
: Adult rhabdomyosarcoma (RMS) is a highly aggressive tumor. Multidisciplinary treatment is important. However, the role of surgery is controversial. The purpose of this study was to reveal the role of a delayed primary excision (DPE) after induction chemotherapy (IC) in localized nonmetastatic adult head and neck RMS. We retrospectively reviewed 24 adult head and neck RMS. Treatment was classified into the following two groups: the DPE group, who received IC followed by surgery, postoperative radiotherapy, and adjuvant chemotherapy (17 patients); the chemoradiotherapy (CRT) group, who received IC followed by chemoradiotherapy (seven patients)...
August 26, 2016: Cancer Medicine
Gregory W Charville, Sushama Varma, Erna Forgó, Sarah N Dumont, Eduardo Zambrano, Jonathan C Trent, Alexander J Lazar, Matt van de Rijn
Rhabdomyosarcoma, the most common soft tissue malignancy of childhood, is a morphologically variable tumor defined by its phenotype of skeletal muscle differentiation. The diagnosis of rhabdomyosarcoma often relies in part on the identification of myogenic gene expression using immunohistochemical or molecular techniques. However, these techniques show imperfect sensitivity and specificity, particularly in scant tissue biopsies. Here, we expand the toolkit for rhabdomyosarcoma diagnosis by studying the expression of PAX7, a transcriptional regulator of mammalian muscle progenitor cells implicated in the pathogenesis of rhabdomyosarcoma...
October 2016: American Journal of Surgical Pathology
Leora Witkowski, W Glenn McCluggage, William D Foulkes
The introduction of new sequencing technologies has resulted in the discovery of commonly mutated genes in uncommon cancers, including non-epithelial ovarian neoplasms and other rare gynaecological tumours, such as cervical embryonal rhabdomyosarcoma. In some of these neoplasms, mutations in certain genes are both frequent and specific enough for the genomic mutations and sometimes their associated protein loss or overexpression to be used as an aid to diagnosis. In this review, we contrast previous gene identification methods with newer ones, and discuss how the new sequencing technologies (collectively referred to as 'next-generation sequencing') have permitted the identification of specific molecular events that characterize several rare gynaecological neoplasms...
August 9, 2016: Histopathology
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