Sexcord/ stromal ovarian tumors surgery

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Sexcord/ stromal ovarian tumors Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Surgical intervention is not recommended for the management of [disease name].

OR

Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]

OR

The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].

OR

The feasibility of surgery depends on the stage of [malignancy] at diagnosis.

OR

Surgery is the mainstay of treatment for [disease or malignancy].

Indications

  • Surgical intervention is not recommended for the management of [disease name].

OR

  • Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either:
    • [Indication 1]
    • [Indication 2]
    • [Indication 3]
  • The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either:
    • [Indication 1]
    • [Indication 2]
    • [Indication 3]

Surgery

Primary surgery:

  • Surgery is the mainstay of treatment for sexcord/ stromal ovarian tumors[1][2][3][4][5][6][7][8][9]
  • Both benign and malignant ovarian sex cord-stromal tumors are managed surgically
  • The schematic approach to malignant sexcord/ stromal ovarian tumors is decribed below
 
 
 
 
 
 
 
 
 
 
 
Malignant sexcord-stromal tumors
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Stage IA/IC: fertility desired
 
 
 
All others
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Fertility sparing surgery with complete staging
 
 
 
Complete staging
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Stage I, low risk
 
Stage I high risk(eg, ruptured stage IC or poorly differentiated stage I) or Intermediate risk(eg, heterologous elements
 
Stage II-IV
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Observe
 
Observe or consider platinum based chemotherapy
 
platinum based chemotherapy or radiotherapy for limited disease
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Surveillance
 
Surveillance
 
Surveillance
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
If clinical relapse: Clinical trial, consider secondary cytoreductive surgery, or recurrence therapy

1.Non-fertility-sparing surgery:

  • Treatment in all postmenopausal and pre-menopausal women with bilateral involvement of ovaries includes total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH-BSO)
  • The main difference between sex cord-stromal versus other ovarian neoplasms is that lymph node metastasis is rare
  • Thus, most clinicians prefer not to perform pelvic and paraaortic lymphadenectomy in most women with malignant sex cord-stromal neoplasms
  • However, lymphadenectomy is required for women with palpable nodal enlargement

2.Fertility-sparing surgery:

  • Unilateral salpingo-oophorectomy (USO) with preservation of the contralateral ovary and the uterus is considered to be adequate surgical treatment for the majority of pre-menopausal patients with granulosa cell tumors
Surveillance for malignant sexcord-stromal ovarian tumors
0-2 yrs After 2 yrs
Physical exam As clinically indicated based on stage

(i.e, 6-12 mo if early stage and low-risk disease,4-6 mo if high-risk disease)

As clinically indicated based on stage

(i.e, 6-12 mo if early stage and low-risk disease,4-6 mo if high-risk disease)

Serum tumor markers
  • Testing as clinically indicated, if applicable
  • If done frequency based on stage (i.e, 6-12 mo if early stage and low-risk disease,4-6 mo if high-risk disease)
  • Testing as clinically indicated, if applicable
  • If done frequency based on stage (i.e, 6-12 mo if early stage and low-risk disease,4-6 mo if high-risk disease)
Radiological imaging Reserved for patients with symptoms, elevated biomarkers, or suspicious findings on physical exam Reserved for patients with symptoms, elevated biomarkers, or suspicious findings on physical exam

Treatment of recurrence:

  • There is no much data available on the optimal treatment of patients with recurrence of disease[10][11][12][13][14]
  • For women who undergo complete resection and/or have no residual disease at the end of surgery, systemic therapy is often used
  • However, if the recurrence seems to be resectable, then surgical treatment may offer a survival advantage
  • Medical therapy is generally recommended for women who are:
    • Not candidates for surgery
    • Have residual disease after surgery
    • Experience multiple recurrences
  • Bleomycin, etoposide, and cisplatin (BEP) and paclitaxel plus carboplatin are the most commonly utilized platinum-based regimens for these patients.

Chemotherapy

  • Adjuvant chemotherapy is usually considered for patients with poor prognosis factors such as: [9][12][13]
    • Large tumour size
    • High mitotic activity-index or
    • Ruptured tumours
  • BEP(bleomycin, etoposide, cisplatin) is the most accepted regimen even for recurrent disease that is refractory to hormone therapy

Hormonal therapy

  • Hormone treatment is usually added for advanced granulosa cell tumors(GrCTs), given their frequent association with oestrogen dependence and usually indolent course
  • Bone densitometry monitoring is indicated for patients receiving aromatase inhibitors

Contraindications

References

  1. Gurumurthy M, Bryant A, Shanbhag S (April 2014). "Effectiveness of different treatment modalities for the management of adult-onset granulosa cell tumours of the ovary (primary and recurrent)". Cochrane Database Syst Rev (4): CD006912. doi:10.1002/14651858.CD006912.pub2. PMID 24753008.
  2. Gremeau AS, Bourdel N, Jardon K, Rabischong B, Mage G, Pouly JL, Canis M (January 2014). "Surgical management of non-epithelial ovarian malignancies: advantages and limitations of laparoscopy". Eur. J. Obstet. Gynecol. Reprod. Biol. 172: 106–10. doi:10.1016/j.ejogrb.2013.10.023. PMID 24315353.
  3. Schultz KA, Schneider DT, Pashankar F, Ross J, Frazier L (May 2012). "Management of ovarian and testicular sex cord-stromal tumors in children and adolescents". J. Pediatr. Hematol. Oncol. 34 Suppl 2: S55–63. doi:10.1097/MPH.0b013e31824e3867. PMID 22525408.
  4. Gershenson DM (June 2012). "Current advances in the management of malignant germ cell and sex cord-stromal tumors of the ovary". Gynecol. Oncol. 125 (3): 515–7. doi:10.1016/j.ygyno.2012.03.019. PMID 22426486.
  5. Färkkilä A, Haltia UM, Tapper J, McConechy MK, Huntsman DG, Heikinheimo M (August 2017). "Pathogenesis and treatment of adult-type granulosa cell tumor of the ovary". Ann. Med. 49 (5): 435–447. doi:10.1080/07853890.2017.1294760. PMID 28276867.
  6. Uma Devi K, Purushotham N, Jayashree N (2015). "Management of Ovarian Cancer In Younger Women". Rev Recent Clin Trials. 10 (4): 263–9. PMID 26411956.
  7. Qian Q, You Y, Yang J, Cao D, Zhu Z, Wu M, Chen J, Lang J, Shen K (April 2015). "Management and prognosis of patients with ovarian sex cord tumor with annular tubules: a retrospective study". BMC Cancer. 15: 270. doi:10.1186/s12885-015-1277-y. PMC 4408581. PMID 25886261.
  8. Chatziioannidou K, Botsikas D, Tille JC, Dubuisson J (May 2015). "Preservation of fertility in non-Peutz-Jegher syndrome-associated ovarian sex cord tumour with annular tubules". BMJ Case Rep. 2015. doi:10.1136/bcr-2014-207841. PMC 4434316. PMID 25969483.
  9. 9.0 9.1 Nasioudis D, Orfanelli T, Frey MK, Chapman-Davis E, Caputo TA, Witkin SS, Holcomb K (March 2019). "Role of adjuvant chemotherapy in the management of non-granulosa cell ovarian sex cord-stromal tumors". J Gynecol Oncol. 30 (2): e19. doi:10.3802/jgo.2019.30.e19. PMC 6393626. PMID 30740951.
  10. Sehouli J, Drescher FS, Mustea A, Elling D, Friedmann W, Kühn W, Nehmzow M, Opri F, Klare P, Dietel M, Lichtenegger W (2004). "Granulosa cell tumor of the ovary: 10 years follow-up data of 65 patients". Anticancer Res. 24 (2C): 1223–9. PMID 15154651.
  11. Mangili G, Sigismondi C, Frigerio L, Candiani M, Savarese A, Giorda G, Lauria R, Tamberi S, Greggi S, Lorusso D (July 2013). "Recurrent granulosa cell tumors (GCTs) of the ovary: a MITO-9 retrospective study". Gynecol. Oncol. 130 (1): 38–42. doi:10.1016/j.ygyno.2013.04.047. PMID 23623833.
  12. 12.0 12.1 Gershenson DM, Morris M, Burke TW, Levenback C, Matthews CM, Wharton JT (April 1996). "Treatment of poor-prognosis sex cord-stromal tumors of the ovary with the combination of bleomycin, etoposide, and cisplatin". Obstet Gynecol. 87 (4): 527–31. doi:10.1016/0029-7844(95)00491-2. PMID 8602303.
  13. 13.0 13.1 Homesley HD, Bundy BN, Hurteau JA, Roth LM (February 1999). "Bleomycin, etoposide, and cisplatin combination therapy of ovarian granulosa cell tumors and other stromal malignancies: A Gynecologic Oncology Group study". Gynecol. Oncol. 72 (2): 131–7. doi:10.1006/gyno.1998.5304. PMID 10021290.
  14. Brown J, Shvartsman HS, Deavers MT, Burke TW, Munsell MF, Gershenson DM (September 2004). "The activity of taxanes in the treatment of sex cord-stromal ovarian tumors". J. Clin. Oncol. 22 (17): 3517–23. doi:10.1200/JCO.2004.12.074. PMID 15337800.

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