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soft tissue sarcoma of head and neck

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
N H Alotaibi, Aurélie Bornand, Nicolas Dulguerov, M Becker, Pavel Dulguerov
INTRODUCTION: Synovial sarcoma (SS) is uncommon high grade soft tissue sarcoma, accounting for less than 10% of all head and neck sarcomas. Also, about 10% of SS occur within the Head & Neck. In the pediatric population, SS is an extremely rare head & neck malignancy. PRESENTATION OF CASE: We present a case of sixteen years old boy diagnosed with SS situated of the hypopharynx treated by surgical excision and post operative radio-chemotherapy. DISCUSSION: This anatomical location brings additional functional challenges (swallowing, phonation, respiration), especially in the pediatric population...
September 3, 2016: International Journal of Surgery Case Reports
Preyas J Vaidya, Anil Heroor, Sitaram Prasad, Rajat Bhargava, Nanadan Arulvannan, Jay Mehta, Boman N Dhabhar, Prashant N Chhajed
Synovial cell sarcoma is an extremely rare tumor of mesenchymal origin. It commonly affects the soft tissues of the extremities but could possibly origin from the head and neck, heart, lung, pleura, mediastinum, esophagus, abdominal wall and the mesentery, and retroperitoneum. Primary synovial sarcoma of pleura, mediastinum, and lung have been reported. Primary synovial sarcoma of the diaphragm has not been reported to the best of our knowledge. We report a case of primary synovial cell sarcoma of the diaphragm presenting as a recurrent pleural effusion and pain in the left hypochondrium managed with multimodality approach...
April 2016: Journal of Cancer Research and Therapeutics
Shalini Koppisetty, Ricardo C Alessio, Atul Rajpurkar
Malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare soft tissue sarcomas of ectomesenchymal origin. They are commonly seen in association with neurofibromatosis type 1 (NF-1), but can also occur without a history of NF (isolated MPNST). MPNSTs are most commonly located on the extremities (brachial and sacral plexus), head and neck, and trunk regions and are rarely reported in genitourinary organs. These tumors are aggressive, with a high recurrence rate and distant metastases. MPNST involving the kidney is extremely rare, and review of the literature using PubMed from 2001 to 2014 revealed eight cases of MPNST involving the kidney (seven, primarily involving the kidney and one metastatic MPNST of the kidney)...
July 2016: Urology Annals
Seung Cheol Ha, Jungsu S Oh, Jong-Lyel Roh, Hyojeong Moon, Jae Seung Kim, Kyung-Ja Cho, Seung-Ho Choi, Soon Yuhl Nam, Sang Yoon Kim
PURPOSE: Head and neck soft tissue sarcoma (HNSTS) is a rare type of tumor with various histological presentations and clinical behaviors. (18)F-FDG PET/CT is being increasingly used for staging, grading, and predicting treatment outcomes in various types of human cancers, although this modality has been rarely studied in the survival prediction of HNSTS. Here we examined the prognostic value of tumor metabolic parameters measured using (18)F-FDG PET/CT in patients with HNSTS. METHODS: This study included 36 consecutive patients with HNSTS who underwent (18)F-FDG PET/CT scanning prior to treatment at our institution...
July 23, 2016: European Journal of Nuclear Medicine and Molecular Imaging
Lucas K Vitzthum, Lindsay C Brown, Jessica W Rooney, Robert L Foote
Head and neck soft tissue sarcomas (HNSTSs) are rare and heterogeneous cancers in which radiation therapy (RT) has an important role in local tumor control (LC). The purpose of this study was to evaluate outcomes and patterns of treatment failure in patients with HNSTS treated with RT. A retrospective review was performed of adult patients with HNSTS treated with RT from January 1, 1998, to December 31, 2012. LC, locoregional control (LRC), disease-free survival (DFS), overall survival (OS), and predictors thereof were assessed...
June 28, 2016: Rare Tumors
David Thorn, Christoph Mamot, Fatime Krasniqi, Frank Metternich, Sven Prestin
The Ewing sarcoma family of tumors (ESFT) encompasses a group of highly aggressive, morphologically similar, malignant neoplasms sharing a common spontaneous genetic translocation that affect mostly children and young adults. These predominantly characteristic, small round-cell tumors include Ewing's sarcoma of the bone and soft tissue, as well as primitive neuroectodermal tumors (PNETs) involving the bone, soft tissue, and thoracopulmonary region (Askin's tumor). Extraosseous ESFTs are extremely rare, especially in the head and neck region, where literature to date consists of sporadic case reports and very small series...
2016: Sarcoma
Dan Wang, Xin He, Hong Zheng
Synovial sarcoma is a rare tumour found in soft tissue; it is a mesenchymal spindle cell tumour that is not related to the synovial membrane. This tumour has a low incidence, and the most frequent place of occurrence is the lower extremities in young adults. Synovial sarcoma of the head and neck accounts for 3%-5% of sarcomas in this anatomical region. The tumor in the nasal cavity is less than 1%. The treatment of choice for synovial sarcoma of the head and neck is complete surgical excision of the tumour mass followed by adjuvant radiotherapy...
February 2016: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
Margaret von Mehren, R Lor Randall, Robert S Benjamin, Sarah Boles, Marilyn M Bui, Ernest U Conrad, Kristen N Ganjoo, Suzanne George, Ricardo J Gonzalez, Martin J Heslin, John M Kane, Henry Koon, Joel Mayerson, Martin McCarter, Sean V McGarry, Christian Meyer, Richard J O'Donnell, Alberto S Pappo, I Benjamin Paz, Ivy A Petersen, John D Pfeifer, Richard F Riedel, Scott Schuetze, Karen D Schupak, Herbert S Schwartz, William D Tap, Jeffrey D Wayne, Mary Anne Bergman, Jillian Scavone
Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for Soft Tissue Sarcoma (available at provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumor, desmoid tumors, and rhabdomyosarcoma...
June 2016: Journal of the National Comprehensive Cancer Network: JNCCN
Patrick C Feasel, Alison L Cheah, Karen Fritchie, Brody Winn, Melissa Piliang, Steven D Billings
BACKGROUND: Clear cell sarcoma typically arises in deep soft tissues of the foot/ankle. Primary head and neck clear cell sarcoma is rare. We report three molecularly confirmed primary head and neck clear cell sarcoma and review the literature. METHODS: Head and neck clear cell sarcoma with no primary elsewhere were retrieved from archival/consultation files. English language literature review of all reported head and neck clear cell sarcoma was performed. RESULTS: Three cases were identified...
October 2016: Journal of Cutaneous Pathology
Arvind Krishnamurthy, G C Raghunandhan, Urmila Majhi
Schwannomas are benign, slow growing nerve sheath tumours of Schwann cell origin. They predominantly are known to involve the head, neck and the flexor surfaces of the extremities, retroperitoneum and the posterior spinal roots. The chest wall is a relatively uncommon location for a schwannoma, the vast majority of which are intra-thoracic, which are usually located in the posterior mediastinum and bulge into the thoracic cavity. Schwannomas arising from the lateral chest wall are relatively uncommon (<5 %)...
September 2015: Indian Journal of Surgical Oncology
Anuradha Ananthaneni, Puneeth Horatti Kuberappa, G Vijay Srinivas, Mohammad Asif Kiresur
Rhabdomyosarcoma (RMS), a malignant neoplasm of skeletal muscle origin, is the most common soft tissue sarcoma seen in childhood and adolescence. The most frequent site is the head and neck accounting for 40% of all cases and other involved sites are genitourinary tract, retroperitoneum, and to a lesser extent, the extremities. RMS is relatively uncommon in the oral cavity and the involvement of the jaws is extremely rare. Here, we report a case of 50-year-old female with oral RMS involving maxillary alveolar region with clinical, radiological, histopathological and immunohistochemical findings...
January 2016: Journal of Oral and Maxillofacial Pathology: JOMFP
Sandhya Shrivastava, Sushruth K Nayak, Prachi Nayak, Sourabh Sahu
Fibrosarcoma is a malignant tumor of fibroblasts. At one time, it was considered one of the most common soft tissue sarcomas. However, the diagnosis of fibrosarcoma is made much less frequently today because of the recognition and separate classification of other spindle cell lesions that have similar microscopic features. Of all the fibrosarcomas occurring in humans, only 0.05% occur in the head and neck region. Here, we present a case of 22-year-old female patient with the swelling on the left anterior aspect of the face...
January 2016: Journal of Oral and Maxillofacial Pathology: JOMFP
Anita P Javalgi, Mahesh H Karigoudar, Katyayani Palur
Ewing's sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing's sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail history and examination she was further referred to detail pathological and radiological investigations...
April 2016: Journal of Clinical and Diagnostic Research: JCDR
Jingjing Xu, Jiawei Wang, Minming Zhang, Baizhou Li
Sclerosing epithelioid fibrosarcoma (SEF) is an unusual variant of fibrosarcoma that was previously considered to be a low-grade tumor with an indolent course. The tumor occurs most commonly in the soft tissue of the limb, trunk, head and neck, and occasionally in the bone and visceral organs. The skull is a rare primary site for SEF, with only 3 cases reported to date. The current study reports a case of SEF occurring in the occipital bone of a 24-year-old man, who lacked neurological symptoms. Imaging revealed a large mass emanating from the occipital bone and involving the superior sagittal sinus, torcular herophili and adjacent brain tissue...
May 2016: Oncology Letters
Navid Ayoobian, Akbar Sarabi Asl, Hosein Poorbaygi, Mohammad Reza Javanshir
High-dose-rate (HDR) brachytherapy is a popular modality for treating cancers of the prostate, cervix, endometrium, breast, skin, bronchus, esophagus, and head and neck as well as soft-tissue sarcomas. Because of different source designs and licensing issues, there is a need for specific dosimetry dataset for each HDR source model. The main objective of the present work is to measure 2D relative dose distribution around a new prototype 192Ir source, referred to as IRAsource-HDR, in PMMA phantom in the framework of AAPM TG-43 and TG-55 recommendations for radial distances of 0...
2016: Journal of Applied Clinical Medical Physics
Souvagya Panigrahi, Sudhansu S Mishra, Sanjib Mishra, Srikant Das
Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma. The most common anatomical sites include the upper and lower extremities and trunk and less commonly the head and neck. To our knowledge, few patients with a cranial or facial MPNST have been reported. We report such a lesion in a 35-year-old woman who presented with left sided rapidly progressive proptosis and visual loss due to an orbital lump extending up to the temporal lobe. Cranial imaging showed a huge mass invading the orbital wall and temporal bone...
April 2016: Asian Journal of Neurosurgery
Yueyuan Yu, Jin Xiao, Lan Wang, Guiqiang Yang
Low-grade myofibroblastic sarcoma (LGMS) represents an atypical myofibroblastic tumor characterized by a diffusely infiltrating pattern of spindle-shaped tumor cells. It was classified as a distinct soft tissue tumor by the World Health Organization in 2002. LGMS occurs mostly in adult patients and has a predilection for the head and neck region. So far, only a few cases of LGMS located in the mandible have been reported. Aggressive surgical resection with clear margins is the primary treatment for LGMS. Because of its rarity, reports of radiation therapy are limited, and the therapeutic effect is still controversial...
July 2016: Journal of Oral and Maxillofacial Surgery
S A Khurram, D Biswas, M Fernando
A 63 year old male presented with a three month history of dysphagia, neck swelling and an oropharyngeal swelling on examination. Initial fine needle aspiration cytology and magnetic resonance imaging (done at a peripheral hospital) suggested a salivary gland neoplasm with lymph node metastasis. An infra-temporal approach was employed to excise the tumour mass and a modified radical neck dissection undertaken to remove the cervical metastasis. Histopathological examination with immunohistochemistry confirmed this to be a soft tissue chordoma...
September 2016: Head and Neck Pathology
Yu-Chien Kao, Yun-Shao Sung, Lei Zhang, Shih-Chiang Huang, Pedram Argani, Catherine T Chung, Nicole S Graf, Dale C Wright, Stewart J Kellie, Narasimhan P Agaram, Kathrin Ludwig, Angelica Zin, Rita Alaggio, Cristina R Antonescu
Soft tissue undifferentiated round cell sarcoma (URCS) occurring in infants is a heterogenous group of tumors, often lacking known genetic abnormalities. On the basis of a t(10;17;14) karyotype in a pelvic URCS of a 4-month-old boy showing similar breakpoints with clear cell sarcoma of kidney (CCSK), we have investigated the possibility of shared genetic abnormalities in CCSK and soft tissue URCS. Most CCSKs are characterized by BCOR exon 16 internal tandem duplications (ITDs), whereas a smaller subset shows YWHAE-NUTM2B/E fusions...
August 2016: American Journal of Surgical Pathology
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