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Adult rhabdomyosarcoma

Paula Marrano, Mary Shago, Gino R Somers, Paul S Thorner
Osteogenic sarcoma (OS) is the most common malignant bone tumor in children and adolescents. Despite advances in molecular genetic characterization of pediatric and adult tumors, the diagnosis of OS still depends almost entirely on light microscopy. The lack of consistent genetic changes in OS has greatly hindered the development of any diagnostic molecular test. Recently, whole-genome sequencing has shown that ~50% of cases of OS have a translocation involving the TP53 gene with breakpoints confined to the first intron...
March 14, 2018: American Journal of Surgical Pathology
Andrew J Jacobs, Carol D Morris, Adam S Levin
BACKGROUND: Reported rates of the incidence of lymph node metastasis in soft tissue sarcoma vary considerably. Many are based on single-institution series and small patient populations. Certain sarcoma subtypes, including synovial sarcoma, have been associated with a higher risk of lymph node involvement. Most single centers have insufficient numbers of patients to assess lymph node metastasis accurately, but larger national databases may allow a more accurate estimation. QUESTIONS/PURPOSES: We queried a large national database and asked the following questions: (1) What proportion of patients with soft tissue sarcoma have lymph node metastasis and distant metastasis? (2) What histologic subtypes are associated with increased risk of nodal metastasis? (3) What is the impact of lymph node metastases and histologic subtype on survival? (4) Does lymph node excision improve survival of patients with soft tissue sarcoma? METHODS: The National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) Program is a national database that covers a geographic cross-section representing approximately 28% of the US population across demographic groups...
March 2018: Clinical Orthopaedics and related Research
Briony K Varda, Patricia Cho, Andrew A Wagner, Richard S Lee
BACKGROUND: In adult urologic oncology the use of robotics has become commonplace; in pediatric urology it is rare. Herein, we describe a collaboration between an adult and a pediatric urologist performing robotic surgery for children and young adults with suspicious or cancerous genitourinary (GU) lesions. OBJECTIVES: To evaluate clinical and oncologic outcomes in children and young adults undergoing robotic surgery for suspicious or cancerous lesions of the GU tract; to describe our collaborative model between an adult and pediatric surgeon at a free-standing children's hospital...
March 2, 2018: Journal of Pediatric Urology
Samaneh Abbasi, Manoochehr Makvandi, Ali Teimoori, Alireza Samarbaf-Zadeh
BACKGROUND: Echovirus 6 (E6), is one of the main enteroviral serotypes, was initially isolated from patients with aseptic meningitis (AM) and is a major cause of hospitalization among children and adults worldwide. METHODS: A cerebrospinal fluid (CSF) sample was collected from patient with clinically suspected aseptic meningitis (AM) in August 2011. Following detection of a virus and subsequent virus serotyping, the whole genome sequence was determined. The sequence of the VP1 region of the isolated strain E6 RA/E6/Ahvaz/Iran/2011 showed 79% (>75%) nucleotide and 94% (>85%) amino acid homology with prototype strain D'Amori...
February 20, 2018: Journal of the Chinese Medical Association: JCMA
Donel A Sequea, Maria E Reese
Rhabdomyosarcoma (RMS) represents the most common sarcoma in childhood, yet is extremely rare in adults with only a handful of cases reported. Here we present a case of intraspinal spindle cell RMS in an adult who presented as a typical case of spinal stenosis. To our knowledge, this is the first reported case of lumbar intraspinal spindle cell RMS in an adult patient. Furthermore, RMS phenotypically presents more aggressively in adults compared to children.
February 13, 2018: PM & R: the Journal of Injury, Function, and Rehabilitation
Juneyoug Koh, Euiseok Jung, Se Jin Jang, Dong Kwan Kim, Byong Sop Lee, Ki-Soo Kim, Ellen Ai-Rhan Kim
Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 (GGT → GAT), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia...
January 2018: Korean Journal of Pediatrics
Uroosa Ibrahim, Amina Saqib, Farhan Mohammad, Juan Ding, Blerina Salman, Fady K Collado, Meekoo Dhar
Embryonal rhabdomyosarcoma (RMS) is a rare type of sarcoma, primarily seen in the pediatric and adolescent population. Three subtypes of embryonal RMS are described, with the botryoid type being the most common. The incidence of this disease in adult females is 0.4% to 1% with the affected age group being patients in the third to fourth decade of life. It is exceedingly rare in patients above 40 years of age. We describe the case of a 48-year-old female, gravida 9 para 5, who presented with abnormal vaginal bleeding and an exophytic mass on examination...
November 21, 2017: Curēus
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
Andre Pinto, Ryan M Kahn, Andrew E Rosenberg, Brian Slomovitz, Charles Matthew Quick, Michella K Whisman, Marilyn Huang
Rhabdomyosarcoma (RMS) is an aggressive mesenchymal tumor most commonly diagnosed in the pediatric population, and when occurring in adults, tends to develop in the deep soft tissue of the limbs. Primary uterine RMS comprises an even more restricted subset, with little known or reported when compared to most other gynecologic sarcomas. Our goal with this study was to retrospectively evaluate cases from two academic institutions and describe the main histopathologic findings of this rare gynecologic malignancy...
January 7, 2018: Human Pathology
Julia Taeubner, Triantafyllia Brozou, Nan Qin, Jasmin Bartl, Sebastian Ginzel, Joerg Schaper, Joerg Felsberg, Simone Fulda, Christian Vokuhl, Arndt Borkhardt, Michaela Kuhlen
The sonic hedgehog (SHH) signaling pathway has been shown to play important roles in embryogenesis, cell proliferation as well as in cell differentiation. It is aberrantly activated in various common cancers in adults, but also in pediatric neoplasms, such as rhabdomyosarcoma (RMS) and atypical teratoid/rhabdoid tumors (AT/RTs). Dysregulation and germline mutation in PATCHED1 (PTCH1), a receptor for SHH, is responsible for the Gorlin Syndrome, a familial cancer predisposing syndrome including RMS. Here, we report a newborn diagnosed with congenital embryonal RMS...
January 2018: European Journal of Human Genetics: EJHG
Trevan D Fischer, Shrawan G Gaitonde, Bradley C Bandera, Mehul V Raval, Sanjeev A Vasudevan, Kenneth W Gow, Elizabeth A Beierle, John J Doski, Adam B Goldin, Monica Langer, Jed G Nuchtern, Stacey Stern, Leland J Foshag, Melanie Goldfarb
BACKGROUND: Multimodal therapy is the standard treatment for pediatric rhabdomyosarcoma, but for adolescents and young adults (AYAs: ages 15-39) and older adults with rhabdomyosarcoma, the use of adjuvant therapy is variable, and survival is greatly decreased compared with younger patients. METHODS: All patients with rhabdomyosarcoma who had a curative operative were identified from the 1998-2012 National Cancer Database. Regression analyses identified independent factors relating to receipt of multimodal therapy (resection + chemotherapy + radiation) and the influence of multimodal therapy on 5-year overall survival...
February 2018: Surgery
Maha At Elsebaie, Zeinab Elsayed
PURPOSE: Recently, conservative approaches such as wide local excisions and neoadjuvant chemotherapy are being considered to select young adult females with gynecologic RMS who have strong desire to preserve fertility. This analysis aims to identify prognosticators affecting survival outcomes and defining potential candidacy for fertility-preservation. Another focus is to explore the role of chemotherapy in reducing the need for aggressive surgery and the role of radiotherapy in decreasing rates of local failure...
November 20, 2017: Archives of Gynecology and Obstetrics
Mohammed Abomelha
There is a paucity of data regarding testicular cancer among Saudis as well as the nonexistent of published national data. Furthermore, a substantial increase of the incidence of testicular cancer among Saudis was lately noted. The aim of the study is to determine the trends and patterns of testicular cancer among adult Saudis using national data over a period of 20 years. The national database of the Saudi Cancer Registry (SCR) on testicular cancer over the last two decades was studied including epidemiological and histological patterns...
October 2017: Urology Annals
Kentaro Igarashi, Kei Kawaguchi, Tasuku Kiyuna, Takashi Murakami, Shinji Miwa, Scott D Nelson, Sarah M Dry, Yunfeng Li, Arun S Singh, Hiroaki Kimura, Katsuhiro Hayashi, Norio Yamamoto, Hiroyuki Tsuchiya, Fritz C Eilber, Robert M Hoffman
Adult pleomorphic rhabdomyosarcoma (RMS) is a rare and recalcitrant, highly-malignant mesenchymal tumor in need of improved therapeutic strategies. Our laboratory pioneered the patient-derived orthotopic xenograft (PDOX) nude mouse model with the technique of surgical orthotopic implantation (SOI). We previously described the development of a PDOX model of adult pleomorphic RMS where the tumor behaved similar to the patient donor. A high-grade pleomorphic rhabdomyosarcoma from a striated muscle was previously grown orthotopically in the right biceps-femoris muscle of nude mice to establish the PDOX model...
September 29, 2017: Oncotarget
Arnaud Jannin, Benjamin Hennart, Antoine Adenis, Bruno Chauffert, Nicolas Penel
Alveolar rhabdomyosarcoma (AR) in adult patients is an exceptional malignancy. Management of AR is based on (neo)adjuvant chemotherapy combining ifosfamide, vincristine, and actinomycin D and local curative-intent surgery/radiotherapy. In cases of relapsing AR, the combination of temozolomide/irinotecan is regarded as a possible option. Here we describe life-threatening long-lasting toxicity related to the 1st cycle of irinotecan-based chemotherapy in a 56-year-old woman suffering from locally advanced and metastatic head and neck AR...
2017: Case Reports in Oncological Medicine
L Fenzl, M Mehrmann, K Kremp, G Schneider
Benign, intermediate and malignant soft tissue tumors can be differentiated histologically. Furthermore, the tumors can be subdivided according to their linear differentiation. In the new World Health Organization (WHO) classification of soft tissue tumors from 2013 changes have been made relating to the allocation of known entities, e. g. undifferentiated sarcomas have been formed into a new subgroup and are no longer assigned to the fibrohistiocytic tumors. The term malignant fibrous histiocytoma has been replaced by the undifferentiated sarcoma...
November 2017: Der Radiologe
M P Jokinen, D L Morgan, H C Price, R A Herbert, T Saddler, D Dixon
The use of immunohistochemical (IHC) staining in determining and/or confirming the cellular origin of poorly differentiated sarcomas was evaluated in this study. Sarcomatous neoplasms were evaluated in a research study conducted in 2 strains of p53+/- haploinsufficient mice. The most common neoplasms were undifferentiated sarcomas, followed by osteosarcomas and rhabdomyosarcomas (RMSs). The RMSs were poorly differentiated and appeared similar to the pleomorphic, or adult type, RMS of humans. All sarcomas stained positive by IHC for the mesenchymal cell intermediate filament vimentin...
August 2017: Toxicologic Pathology
Susan C Shelmerdine, Armando J Lorenzo, Abha A Gupta, Govind B Chavhan
Urinary bladder masses are rare in children, and the associated histologic features and prognoses in this population are different from those in adults. Most children with urinary bladder masses present with lower urinary tract symptoms, which may include hematuria, dysuria, frequent urination, and urgency to urinate. However, some of these masses may be identified incidentally or involve generic symptoms such as abdominal distention. In general, pediatric bladder tumors can be divided into those that originate from the bladder epithelium, known as urothelial neoplasms, and mesenchymal bladder neoplasms, which are more prevalent...
October 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Abdurrahman Aycan, Sebahattin Celik, Fetullah Kuyumcu, Mehmet Edip Akyol, Mehmet Arslan, Erkan Dogan, Harun Arslan
BACKGROUND AND OBJECTIVE: Spinal bone metastases are common. They are mostly localized to the lumbar, thoracic, and cervical spine. The most common primaries to result in spinal metastases include lung, breast, and prostate carcinomas in adults as opposed to leukemia, Ewing sarcoma, rhabdomyosarcoma, and neuroblastoma in children. In patients diagnosed with cancer, bone metastases are found in 40% and spinal metastases in 10%. In this study, we reviewed 25 patients diagnosed with a spinal metastasis of unknown primary who presented with low back pain or acute-onset neurologic deficits and underwent operative treatment...
January 2018: World Neurosurgery
Scott C Borinstein, Diana Steppan, Masanori Hayashi, David M Loeb, Michael S Isakoff, Odion Binitie, Andrew S Brohl, Julia A Bridge, Mark Stavas, Eric T Shinohara, William H Meyer, Damon R Reed, Lars M Wagner
Optimal treatment of rhabdomyosarcoma (RMS) requires multidisciplinary approach, incorporating chemotherapy with local control. Although current therapies are built on cooperative group trials, a comprehensive standard of care to guide clinical decision making has been lacking, especially for relapsed patients. Therefore, we assembled a panel of pediatric and adolescent and young adult sarcoma experts to develop treatment guidelines for managing RMS and to identify areas in which further research is needed...
February 2018: Pediatric Blood & Cancer
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