Ovarian Sarcoma: Difference between revisions

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==Screening==
==Screening==
There is insufficient evidence to recommend routine screening for [disease/malignancy].
There is insufficient evidence to recommend routine screening for ovarian sarcoma.
 
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==
==Natural History, Complications, and Prognosis==

Revision as of 19:55, 30 July 2019


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][40][41][42]
  • 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 ovarian sarcoma.

Natural History, Complications, and Prognosis

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

  • 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

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:[43][44][45]

  • 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 ovarian Sarcoma.

Echocardiography or Ultrasound

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

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 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[44][45][46][47][48]
  • 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

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