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.

Historical Perspective

[Disease name] was first discovered by [name of scientist], a [nationality + occupation], in [year]/during/following [event].

The association between [important risk factor/cause] and [disease name] was made in/during [year/event].

In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].

In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].

There have been several outbreaks of [disease name], including -----.

In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].

Classification

  • There is no established system for the classification of ovarian Sarcoma.[1]
  • 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
  • Clonal loss of the wild-type BRCA2 allele as well as the same somatic mutation of the TP53 gene was evident in histologic components


Causes

Disease name] may be caused by [cause1], [cause2], or [cause3].

OR

Common causes of [disease] include [cause1], [cause2], and [cause3].

OR

The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].

OR

The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.

Differentiating ovarian sarcoma from Other Diseases

[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Age of onset Symptoms Physical examination
Lab Findings Imaging Immunohistopathology
pelvic/abdominal pain or pressure vaginal bleeding/discharge GI dysturbance Fever Tenderness CT scan/US MRI
Serous cystadenoma/carcinoma
[2][3][4][5]
  • >55 y/o
+/– +/–
  • In US we may see simple or multiloculated cyst
  • In serous cystadenocarcinoma we may see papillary projection inside the cyst
  • In serous cystadenocarcinoma we may see ascites
  • In Serous cystadenoma we may see a simple cyst with beak sign, hypointense on T1 and hyperintense on T2
  • In serous cystadenocarcinoma we may see some Solid malignant components inside the cyst with intermediate signal on T1 and T2
Mucinous cystadenoma/carcinoma
[6][7][8]
  • >55 y/o
+/– +/–
  • Stained glass appearance due to variable signal intensity on T1 and T2
  • The more mucin we have, there is more intensity on T1
  • and less intensity on T2
Endometrioma
[9][10][11]
+ + +/– +
  • hyperintensity on T1-weighted images and a hypointensity on T2-weighted images
  • Powder burn hemorrhages
Teratoma
[12][13][14][15]


  • 10-30 y/o
+/– +/–
  • We may see evidence of fat components
Granulosa cell tumor
[16][17][18][19]
  • 50-60 y/o
+ +/–
Sertoli-leydig cell tumor
[20][21]
  • 15 to 35 y/o
+/–
  • In US we may see unilateral Well-defined hypoechoic lesion
  • Low T2 signal intensity
  • areas of high signal intensity
Tubal tubo-ovarian abscess
[22][23][24][25]
+ + + +
  • hypointense in T1 and heterogeneous in T2
Fallopian tube carcinoma
[26]
  • >60 y/o
+ + + +/–
  • Low signal on T1
  • In case of hemorrhage inside the tumor we may see high signal intensity on T1
  • Low or of intermediate signal on T2
  • Based on the tumor type we may have different biopsy finding
Uterine Leiomyoma
[27][28]
+ + +/–
  • Low to intermediate signal intensity on T1 and T2
  • In case of necrosis inside the mass, there might be some high signal lesions on T2
Choriocarcinoma
[29][30][31][32]
+ + +/– +
  • We may see an infiltrative uterine mass and thickening of uterine wall
Leiomyosarcoma
[33][34][35][36][37]
  • >55 y/o
+ + +/–
  • Increased uterine size
  • Irregular central zones of low signal intensity (tumor necrosis)


OR

[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].

Epidemiology and Demographics

  • Ovarian sarcoma is one of the least common gynecologic malignancy, constituting approximately 1% of all ovarian malignancies.[1][38][39]
  • In 2012, approximately 239,000 women were diagnosed with ovarian cancer worldwide, and 152,000 died of the disease.
  • Patients of all age groups may develop sarcoma of the ovary both postmenopausal and premenopausal women.
  • The incidence of ovarian Sarcoma increases with age; the median age at diagnosis is 50-60 years.
  • There is no racial predilection to ovarian Sarcoma

Risk Factors

There are no established risk factors for [disease name].

OR

The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].

OR

Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

OR

Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.

Screening

There is insufficient evidence to recommend routine screening for [disease/malignancy].

OR

According to the [guideline name], screening for [disease name] is not recommended.

OR

According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].

Natural History, Complications, and Prognosis

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.


Diagnostic Study of Choice

The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].

OR

The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].

OR

The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].

OR

There are no established criteria for the diagnosis of [disease name].

History and Symptoms

Most of the women are asymptomatic, when present, symptoms may include:[40][41][42]

  • 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/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].

OR

Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

OR

[Test] is usually normal among patients with [disease name].

OR

Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].

OR

There are no diagnostic laboratory findings associated with [disease name].

Electrocardiogram

There are no ECG findings associated with [disease name].

OR

An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

X-ray

There are no x-ray findings associated with [disease name].

OR

An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

There are no CT scan findings associated with [disease name].

OR

[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

OR

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

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

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 treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Surgery

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

Primary Prevention

There are no established measures for the primary prevention of [disease name].

OR

There are no available vaccines against [disease name].

OR

Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

OR

[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].

References

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