Document Detail

A case of primary alveolar soft part sarcoma of the uterine cervix and a review of the literature.
MedLine Citation:
PMID:  21519815     Owner:  NLM     Status:  MEDLINE    
Alveolar soft part sarcoma (ASPS) that originates from the uterine cervix is extremely rare, with only thirteen cases reported. The participation of the ASPL-TFE3 chimeric gene, translocation (X; 17) (p11; q25), has been demonstrated in ASPS. Here, we report a case of cervical ASPS, along with a review of the literature. The patient, a 56-year-old woman, was referred for a 70 × 80 mm cervical tumor. She underwent a hysterectomy and bilateral salpingo-oophorectomy, and remained disease free for 66 months without adjuvant therapy. Pathological examination revealed features consistent with ASPS. In addition, the present case demonstrated strong positive nuclear staining for TFE3, and ASPL-TFE3 fusion gene type 1 was detected by RT-PCR. In a review of fourteen cases of this tumor (including the present case), the immunohistochemical expression patterns of myogenic or neuroendocrine markers were somewhat varied among cases. In all cases except for the present case, the patients were under 40 years of age, and the tumor sizes were under 5 cm. The prognosis of ASPS in the cervix was considerably better than that of ASPS in soft tissues. Complete resection with adequate margins is thought to be important, although the appropriate surgical method, including lymph node dissection, is uncertain. The role of chemotherapy or radiotherapy as adjuvant therapy has not been defined. Cervical ASPS is extremely rare, making case series the most viable option for understanding their natural history and for developing a treatment strategy, including an optimal surgical procedure and adjuvant therapy.
Kiyoshi Hasegawa; Ryoko Ichikawa; Risa Ishii; Shuko Oe; Rina Kato; Yoichi Kobayashi; Makoto Kuroda; Yasuhiro Udagawa
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2011-04-26
Journal Detail:
Title:  International journal of clinical oncology     Volume:  16     ISSN:  1437-7772     ISO Abbreviation:  Int. J. Clin. Oncol.     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2011-12-14     Completed Date:  2012-05-15     Revised Date:  2012-12-13    
Medline Journal Info:
Nlm Unique ID:  9616295     Medline TA:  Int J Clin Oncol     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  751-8     Citation Subset:  IM    
Department of Obstetrics and Gynecology, Fujita Health University School of Medicine, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi 470-1192, Japan.
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MeSH Terms
Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / genetics
Child, Preschool
Middle Aged
Oncogene Proteins, Fusion / genetics
Sarcoma, Alveolar Soft Part / genetics,  pathology*,  surgery*
Translocation, Genetic
Uterine Cervical Neoplasms / genetics,  pathology*,  surgery*
Reg. No./Substance:
0/ASPSCR1 protein, human; 0/Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; 0/Oncogene Proteins, Fusion; 0/TFE3 protein, human

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