Ovarian Sarcoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Maneesha Nandimandalam, M.B.B.S.[2]

Overview

Ovarian carcinosarcoma, which is also known as a malignant mixed mullerian tumor (MMMT) of the ovary, is a rare, aggressive cancer of the ovary with two distinct characteristic cancer types i.e carcinoma and sarcoma.Primary ovarian sarcomas occur as pure sarcomas or mixed müllerian tumors (MMTs).Ovarian sarcoma is one of the least common gynecologic malignancy, constituting approximately 1% of all ovarian malignancies.Prognosis is generally poor, and the 5-year survival rate of patients with ovarian sarcoma is approximately 28.2%.Most of the women are asymptomatic, when present, symptoms may include,pain in the abdomen or pelvic area, bloating or swelling of the abdomen, quickly feeling full when eating, other digestive problems. An elevated concentration of CA-125 in serum is seen in some patients of ovarian sarcoma.Biopsy is the study of choice.Findings on MRI suggestive of ovarian sarcoma include the following.Surgery is the mainstay of treatment for ovarian sarcoma.Among all chemotherapeutic regimens that are being used to treat ovarian sarcoma, they are divided into two groups like platinum containing regimens and non-platinum regimens. Cisplatin, carboplatin are commonly used.


Historical Perspective

There is limited information available about the historical perspective of ovarian sarcoma

Classification

  • There is no established system for the classification of ovarian Sarcoma.[1][2][3]
  • Primary ovarian sarcomas occur as pure sarcomas or mixed müllerian tumors (MMTs).
  • Pure sarcomas are comprised of a single malignant mesenchymal element and are further categorized as:
    • Stromal cell sarcomas
    • Fibrosarcomas
    • Leiomyosarcomas
    • Neurofibrosarcomas
    • Rhabdomyosarcomas
    • Chondrosarcomas
    • Angiosarcomas
    • Liposarcomas
  • On the other hand mixed mullerian tumors(MMTs) are defined by the presence of both carcinomatous and sarcomatous elements and are more common than pure sarcomas.
  • Ovarian MMTs can be further classified as homologous or heterologous on the basis of the tissue components present.
  • Homologous tumors contain elements that are native to the ovary whereas heterologous tumors contain elements that normally are not present in the ovary.

The staging of [malignancy name] is based on the [staging system].

OR

There is no established system for the staging of [malignancy name].

Pathophysiology

  • The exact pathogenesis of ovarian sarcoma is not fully understood[4][3]
  • Clonal loss of the wild-type BRCA2 allele as well as the same somatic mutation of the TP53 gene was evident in histologic components
Types of ovarian cancer according to origin,Vargas AN. Natural history of ovarian cancer. Ecancermedicalscience. 2014;8:465. Published 2014 Sep 25. doi:10.3332/ecancer.2014.465,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4176445/

Causes

The exact causes of ovarian sarcoma have not been identified.

Differentiating ovarian sarcoma from Other Diseases

Epidemiology and Demographics

Risk Factors

There are no established risk factors for ovarian sarcoma

Screening

There is insufficient evidence to recommend routine screening for ovarian sarcoma.

Natural History, Complications, and Prognosis

  • Ovarian carcinosarcomas follow a distinct natural history compared to other more common epithelial carcinomas[10][3][11][12]
  • Ovarian carcinosarcomas are aggressive neoplasms with a predilection towards early dissemination.
  • Prognostic factors for this tumor type remain unclear because of its rarity.
  • Prognosis is generally poor, and the 5-year survival rate of patients with ovarian sarcoma is approximately 28.2%.
  • Some possible factors such as age and menopausal status have been proposed.

Diagnostic Study of Choice

  • There are no established criteria for the diagnosis of ovarian sarcoma
  • Biopsy is the study of choice

History and Symptoms

Most of the women are asymptomatic, when present, symptoms may include:[13][14][15]

  • Pain in the abdomen or pelvic area
  • Bloating or swelling of the abdomen
  • Quickly feeling full when eating
  • Other digestive problems

Physical Examination

Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].

OR

Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

The presence of [finding(s)] on physical examination is diagnostic of [disease name].

OR

The presence of [finding(s)] on physical examination is highly suggestive of [disease name].

Laboratory Findings

  • An elevated concentration of CA-125 in serum is seen in some patients of ovarian sarcoma.[16][17][18]
  • There are no diagnostic laboratory findings associated with ovarian sarcoma.

Electrocardiogram

There are no ECG findings associated with ovarian sarcoma.

X-ray

There are no x-ray findings associated with ovarian Sarcoma.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with ovarian Sarcoma.

CT scan

  • CT scan may be helpful in the diagnosis of ovarian sarcoma . Findings on CT scan suggestive of ovarian sarcoma include:[19][20][21]

MRI

  • MRI may be helpful in the diagnosis of ovarian sarcoma. Findings on MRI suggestive of ovarian sarcoma include the following:[22][19][21][23][20]

Other Imaging Findings

There are no other imaging findings associated with ovarian sarcoma

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

There is no medical treatment for ovarian sarcoma, the mainstay of therapy is surgery and chemotherapy.

Surgery

  • Surgery is the mainstay of treatment for ovarian sarcoma.
  • The management is similar to that of epithelial carcinoma of ovary, consisting of cytoreductive surgery followed by adjuvant chemotherapy.

Chemotherapy

  • Chemotherapy with various regimens has been used in different centers without defined conclusions on efficacy[14][15][24][25][26]
  • Multiple chemotherapeutic regimens have been evaluated with modest response rates ranging from 12% to 100%.
  • Among all chemotherapeutic regimens that are being used, they are divided into two groups like platinum containing regimens and non-platinum regimens.
    • Platinum containing chemotherapy regimens
  1. Carboplatin and ifosfamide (Carbo-I)
  2. Carboplatin (Carbo)
  3. Cyclophosphamide, adriamycin and cisplatin (CAP)
  4. Carboplatin and cyclophosphamide (Carbo-C)
  5. Epirubicin, carboplatin and 5FU (E-Carbo-F)
  6. Epirubicin, cisplatin and 5FU (ECF)
  7. Taxol and Carboplatin (T-Carbo)
  • Other chemotherapy regimens
  1. Doxorubicin (A)
  2. Doxorubicin and cyclophosphamide (AC)
  3. Cyclophosphamide (IV)
  4. Cyclophosphamide (oral)
  5. Melphalan

Primary Prevention

There are no established measures for the primary prevention of ovarian sarcoma.

Secondary Prevention

There are no established measures for the secondary prevention of ovarian sarcoma.

References

  1. ↑ 1.0 1.1 Harris, M A; Delap, L M; Sengupta, P S; Wilkinson, P M; Welch, R S; Swindell, R; Shanks, J H; Wilson, G; Slade, R J; Reynolds, K; Jayson, G C (2003). "Carcinosarcoma of the ovary". British Journal of Cancer. 88 (5): 654–657. doi:10.1038/sj.bjc.6600770. ISSN 0007-0920.
  2. ↑ Makris GM, Siristatidis C, Battista MJ, Chrelias C (2015). "Ovarian carcinosarcoma: a case report, diagnosis, treatment and literature review". Hippokratia. 19 (3): 256–9. PMC 4938474. PMID 27418786.
  3. ↑ 3.0 3.1 3.2 Rauh-Hain JA, Growdon WB, Rodriguez N, Goodman AK, Boruta DM, Schorge JO; et al. (2011). "Carcinosarcoma of the ovary: a case-control study". Gynecol Oncol. 121 (3): 477–81. doi:10.1016/j.ygyno.2011.02.023. PMID 21420726.
  4. ↑ Vargas AN (2014). "Natural history of ovarian cancer". Ecancermedicalscience. 8: 465. doi:10.3332/ecancer.2014.465. PMC 4176445. PMID 25371706.
  5. ↑ Ferlay, Jacques; Soerjomataram, Isabelle; Dikshit, Rajesh; Eser, Sultan; Mathers, Colin; Rebelo, Marise; Parkin, Donald Maxwell; Forman, David; Bray, Freddie (2015). "Cancer incidence and mortality worldwide: Sources, methods and major patterns in GLOBOCAN 2012". International Journal of Cancer. 136 (5): E359–E386. doi:10.1002/ijc.29210. ISSN 0020-7136.
  6. ↑ Ferlay, Jacques; Shin, Hai-Rim; Bray, Freddie; Forman, David; Mathers, Colin; Parkin, Donald Maxwell (2010). "Estimates of worldwide burden of cancer in 2008: GLOBOCAN 2008". International Journal of Cancer. 127 (12): 2893–2917. doi:10.1002/ijc.25516. ISSN 0020-7136.
  7. ↑ Parkin DM, Bray F, Ferlay J, Pisani P (2005). "Global cancer statistics, 2002". CA Cancer J Clin. 55 (2): 74–108. PMID 15761078.
  8. ↑ Cresanta JL (1992). "Epidemiology of cancer in the United States". Prim Care. 19 (3): 419–41. PMID 1410056.
  9. ↑ Bray, Freddie; Ferlay, Jacques; Soerjomataram, Isabelle; Siegel, Rebecca L.; Torre, Lindsey A.; Jemal, Ahmedin (2018). "Global cancer statistics 2018: GLOBOCAN estimates of incidence and mortality worldwide for 36 cancers in 185 countries". CA: A Cancer Journal for Clinicians. 68 (6): 394–424. doi:10.3322/caac.21492. ISSN 0007-9235.
  10. ↑ Barnholtz-Sloan JS, Morris R, Malone JM, Munkarah AR (2004) Survival of women diagnosed with malignant, mixed mullerian tumors of the ovary (OMMMT). Gynecol Oncol 93 (2):506-12. DOI:10.1016/j.ygyno.2004.02.016 PMID: 15099970
  11. ↑ Sood AK, Sorosky JI, Gelder MS, Buller RE, Anderson B, Wilkinson EJ et al. (1998) Primary ovarian sarcoma: analysis of prognostic variables and the role of surgical cytoreduction. Cancer 82 (9):1731-7. PMID: 9576296
  12. ↑ Cicin İ, Özatlı T, Türkmen E, Özturk T, Özçelik M, Çabuk D et al. (2016) Predictive and Prognostic Factors in Ovarian and Uterine Carcinosarcomas. Balkan Med J 33 (5):517-524. DOI:10.5152/balkanmedj.2016.151268 PMID: 27761279
  13. ↑ Dai Y, Shen K, Lang JH, Huang HF, Pan LY, Wu M; et al. (2011). "Primary sarcoma of the ovary: clinicopathological characteristics, prognostic factors and evaluation of therapy". Chin Med J (Engl). 124 (9): 1316–21. PMID 21740740.
  14. ↑ 14.0 14.1 del Carmen MG, Birrer M, Schorge JO (2012). "Carcinosarcoma of the ovary: a review of the literature". Gynecol Oncol. 125 (1): 271–7. doi:10.1016/j.ygyno.2011.12.418. PMID 22155675.
  15. ↑ 15.0 15.1 Kim HJ, Lee HM, Kim MK, Lee YK, Lee IH, Lee KH; et al. (2017). "Prognostic assessment of sarcomatous histologic subtypes of ovarian carcinosarcoma". Obstet Gynecol Sci. 60 (4): 350–356. doi:10.5468/ogs.2017.60.4.350. PMC 5547082. PMID 28791266.
  16. ↑ Shakuntala P, Umadevi K, Usha A, Abhilasha N, Bafna U (2012). "Primary ovarian adenosarcoma with elevated Ca-125 levels and normal ascitic fluid cytology: a case report and review of literature". Ecancermedicalscience. 6: 284. doi:10.3332/ecancer.2012.284. PMC 3530409. PMID 23304240.
  17. ↑ Mogensen O, Mogensen B, Jakobsen A (1990). "Tumour-associated trypsin inhibitor (TATI) and cancer antigen 125 (CA 125) in mucinous ovarian tumours". Br J Cancer. 61 (2): 327–9. doi:10.1038/bjc.1990.64. PMC 1971406. PMID 2310684.
  18. ↑ Van Gorp T, Cadron I, Despierre E, Daemen A, Leunen K, Amant F; et al. (2011). "HE4 and CA125 as a diagnostic test in ovarian cancer: prospective validation of the Risk of Ovarian Malignancy Algorithm". Br J Cancer. 104 (5): 863–70. doi:10.1038/sj.bjc.6606092. PMC 3048204. PMID 21304524.
  19. ↑ 19.0 19.1 Miccò M, Sala E, Lakhman Y, Hricak H, Vargas HA (2015). "Imaging Features of Uncommon Gynecologic Cancers". AJR Am J Roentgenol. 205 (6): 1346–59. doi:10.2214/AJR.14.12695. PMC 5502476. PMID 26587944.
  20. ↑ 20.0 20.1 Bell DJ, Pannu HK (2011). "Radiological assessment of gynecologic malignancies". Obstet Gynecol Clin North Am. 38 (1): 45–68, vii. doi:10.1016/j.ogc.2011.02.003. PMID 21419327.
  21. ↑ 21.0 21.1 Forstner R, Graf A (1999). "[Diagnostic imaging in staging of gynecologic carcinomas]". Radiologe. 39 (7): 610–8. PMID 10472090.
  22. ↑ Manoharan D, Das CJ, Aggarwal A, Gupta AK (2016). "Diffusion weighted imaging in gynecological malignancies - present and future". World J Radiol. 8 (3): 288–97. doi:10.4329/wjr.v8.i3.288. PMC 4807338. PMID 27027614.
  23. ↑ Alt CD, Brocker KA, Eichbaum M, Sohn C, Arnegger FU, Kauczor HU; et al. (2011). "Imaging of female pelvic malignancies regarding MRI, CT, and PET/CT: Part 2". Strahlenther Onkol. 187 (11): 705–14. doi:10.1007/s00066-011-4002-z. PMID 22037656.
  24. ↑ Brown E, Stewart M, Rye T, Al-Nafussi A, Williams AR, Bradburn M; et al. (2004). "Carcinosarcoma of the ovary: 19 years of prospective data from a single center". Cancer. 100 (10): 2148–53. doi:10.1002/cncr.20256. PMID 15139057.
  25. ↑ Harris MA, Delap LM, Sengupta PS, Wilkinson PM, Welch RS, Swindell R; et al. (2003). "Carcinosarcoma of the ovary". Br J Cancer. 88 (5): 654–7. doi:10.1038/sj.bjc.6600770. PMC 2376340. PMID 12618869.
  26. ↑ Cicin I, Saip P, Eralp Y, Selam M, Topuz S, Ozluk Y; et al. (2008). "Ovarian carcinosarcomas: clinicopathological prognostic factors and evaluation of chemotherapy regimens containing platinum". Gynecol Oncol. 108 (1): 136–40. doi:10.1016/j.ygyno.2007.09.003. PMID 17936342.

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