title: A case of primary alveolar soft part sarcoma of the uterine cervix and a review of the literature.
abstract: 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.
authors.affiliation: Department of Obstetrics and Gynecology, Fujita Health University School of Medicine, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi 470-1192, Japan. khase@fujita-hu.ac.jp
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Hasegawa
authors.forename: Ryoko
authors.initials: R
authors.lastname: Ichikawa
authors.forename: Risa
authors.initials: R
authors.lastname: Ishii
authors.forename: Shuko
authors.initials: S
authors.lastname: Oe
authors.forename: Rina
authors.initials: R
authors.lastname: Kato
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Kobayashi
authors.forename: Makoto
authors.initials: M
authors.lastname: Kuroda
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Udagawa
mesh_terms: D000328:Adult; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007044:Hysterectomy; D047908:Intracellular Signaling Peptides and Proteins; D008297:Male; D008875:Middle Aged; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D010052:Ovariectomy; D011379:Prognosis; D018234:Sarcoma, Alveolar Soft Part / Q000235:genetics / Q000473:pathology* / Q000601:surgery*; D014178:Translocation, Genetic; D002583:Uterine Cervical Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery*
chemical_list: C422743:ASPSCR1 protein, human; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; D047908:Intracellular Signaling Peptides and Proteins; D015514:Oncogene Proteins, Fusion; C069203:TFE3 protein, human