title: Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT): A review of 47 cases.
abstract: OBJECTIVE
  Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT) is a rare disease with a poor prognosis. SCCOHT has recently been shown to be associated with SMARCA4 gene mutations as well as molecular and genetic similarities to malignant rhabdoid tumors (MRT). The objective of our study is to describe the clinical characteristics, treatment modalities and outcomes of 47 patients with SCCOHT.
  
  
  METHODS
  We performed a retrospective analysis of 47 patients with SCCOHT evaluated at MD Anderson Cancer Center between 1990 and 2014. Medical records were reviewed for demographic information, pathologic findings, treatment regimens and outcomes.
  
  
  RESULTS
  Median age at diagnosis was 30 years (range 5-46). All patients underwent surgery with unilateral salpingo-oophorectomy (USO) performed in 26 patients (55%), and hysterectomy with bilateral salpingooophorectomy (BSO) in 21 patients (45%). Sixteen patients (34.0%) had stage I disease, six (12.8%) stage II, 23 (48.9%) stage III, and two patients (4.3%) had stage IV disease. Information on adjuvant treatment was available for 43 patients: 83.3% received chemotherapy alone, 9.5% chemotherapy followed by radiotherapy, 2.4% chemoradiation, and 4.8% did not receive any adjuvant therapy. Median follow-up was 13.2 months (range, 0.1 to 210.7) with a median overall survival of 14.9 months. Multi-agent chemotherapy and radiotherapy were associated with a better prognosis.
  
  
  CONCLUSIONS
  Our findings suggest that aggressive therapy including multi-agent chemotherapy and possibly radiotherapy may extend survival. Further study is needed to improve outcomes in these patients including the adoption of systemic therapies used in MRT as well as the development of novel agents targeting specific mutations.
authors.affiliation: Department of Medical Oncology, Hospital Israelita Albert Einstein, São Paulo, Brazil; Division of Gynecologic Oncology, Universidade Federal de São Paulo, São Paulo, Brazil.
authors.forename: D
authors.initials: D
authors.lastname: Callegaro-Filho
authors.affiliation: Department of Gynecologic Oncology and Reproductive Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: D M
authors.initials: DM
authors.lastname: Gershenson
authors.affiliation: Department of Gynecologic Oncology and Reproductive Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: A M
authors.initials: AM
authors.lastname: Nick
authors.affiliation: Department of Biostatistics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: M F
authors.initials: MF
authors.lastname: Munsell
authors.affiliation: Department of Gynecologic Oncology and Reproductive Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: P T
authors.initials: PT
authors.lastname: Ramirez
authors.affiliation: Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: P J
authors.initials: PJ
authors.lastname: Eifel
authors.affiliation: Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: E D
authors.initials: ED
authors.lastname: Euscher
authors.affiliation: Division of Gynecologic Oncology, Universidade Federal de São Paulo, São Paulo, Brazil.
authors.forename: R M
authors.initials: RM
authors.lastname: Marques
authors.affiliation: Division of Gynecologic Oncology, Universidade Federal de São Paulo, São Paulo, Brazil.
authors.forename: S M
authors.initials: SM
authors.lastname: Nicolau
authors.affiliation: Department of Gynecologic Oncology and Reproductive Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. Electronic address: kschmele@mdanderson.org.
authors.forename: K M
authors.initials: KM
authors.lastname: Schmeler
mesh_terms: D000293:Adolescent; D000328:Adult; D018288:Carcinoma, Small Cell / Q000097:blood / Q000473:pathology* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006934:Hypercalcemia / Q000097:blood / Q000473:pathology* / Q000628:therapy*; D008875:Middle Aged; D010051:Ovarian Neoplasms / Q000097:blood / Q000473:pathology* / Q000628:therapy*; D012189:Retrospective Studies; D055815:Young Adult
keywords: Hypercalcemic type; Malignant rhabdoid tumor; Radiotherapy; SMARCA4; Small cell carcinoma of the ovary; VPCBAE