Mixed mullerian tumor: Difference between revisions

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{{CMG}} {{AE}} {{MV}}
{{CMG}} {{AE}} {{MV}}
   
   
{{SK}} Mixed Müllerian tumor; MMMT; Malignant mixed Müllerian tumor
{{SK}} Mixed Müllerian tumor; MMMT; Malignant mixed Müllerian tumor; Carcinosarcoma of the uterus; Sarcomatoid carcinoma of the uterus; Malignant mesodermal mixed tumor; Metaplastic carcinoma
 
==Overview==
==Overview==
'''Mixed Mullerian tumor''' (MMMT) is a rare [[uterine sarcoma]] or carcinosarcoma.


==Historical Perspective==
==Historical Perspective==
*[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
*Mixed Mullerian tumor was first described by Ferriera and colleagues in 1951.<ref name="pmid16051326">{{cite journal |vauthors=Wright JD, Rosenblum K, Huettner PC, Mutch DG, Rader JS, Powell MA, Gibb RK |title=Cervical sarcomas: an analysis of incidence and outcome |journal=Gynecol. Oncol. |volume=99 |issue=2 |pages=348–51 |year=2005 |pmid=16051326 |doi=10.1016/j.ygyno.2005.06.021 |url=}}</ref>
*In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
   
   
==Classification==
==Classification==
*[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
*Mixed Mullerian tumors are normally composed of both carcinomatous (epithelial) and sarcomatous (mesodermal) components.
:*[group1]
*Mixed Mullerian tumor may be classified according to pathology findings into 2 types:  
:*[group2]
'''Epitheloid subtype'''
:*[group3]
:*Endometroid adenocarcinoma (most common)
*Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
:*Clear cell carcinoma
:*Mucinous carcinoma
:*Papillary-serous carcinoma
'''Sarcomatoid subtype'''
:*Undifferentiated sarcoma
:*Rhabdomyosarcoma
 
*Mixed Mullerian tumor may also be classified according to antomical location into 7 types:
:*Uterine corpus
:*Cervix
:*Ovaries
:*Fallopian tubes
:*Vagina
:*Peritoneum
:*Extragenital sites
 
==Pathophysiology==
==Pathophysiology==
*The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
*The pathogenesis of mixed Mullerian tumor is characterized by  
*The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
*Mixed Mullerian tumor
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
*On gross pathology, a large cervical mass is a characteristic finding of mixed Mullerian tumor.
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
*On microscopic histopathological analysis, high-grade stromal sarcoma, poorly differentiated epithelial cells, and angiolymphatic invasion are characteristic findings of mixed Mullerian tumor.
   
   
==Causes==
==Causes==
* [Disease name] may be caused by either [cause1], [cause2], or [cause3].
* Mixed Mullerian tumor may be caused by precursor lesions, such as
* [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
* Mixed Mullerian tumor is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
* There are no established causes for [disease name].
* There are no established causes for mixed Mullerian tumor.
   
   
==Differentiating [disease name] from other Diseases==
==Differentiating mixed Mullerian tumor 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]
*Mixed Mullerian tumor must be differentiated from other diseases that cause abnormal vaginal bleeding, abdominal pain, and metrorrhagia, such as:
:*[Differential dx2]
:*Uterine leiomyosarcoma
:*[Differential dx3]
:*Adenocarcinoma of the uterus
:*Endometrial cancer
   
   
==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
* The prevalence of mixed Mullerian tumor is approximately [number or range] per 100,000 individuals worldwide.
* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
* In [year], the incidence of mixed Mullerian tumor was estimated to be [number or range] cases per 100,000 individuals in [location].
   
   
===Age===
===Age===
*Patients of all age groups may develop [disease name].
*The median age at diagnosis of Mixed Mullerian tumor is 66 years
*Mixed Mullerian tumor is more commonly observed among patients aged between 50 and 60 years old.
*[Disease name] is more commonly observed among patients aged [age range] years old.
*Mixed Mullerian tumor is more commonly observed among postmenopausal women
*[Disease name] is more commonly observed among [elderly patients/young patients/children].
===Gender===
*[Disease name] affects men and women equally.
*[Gender 1] are more commonly affected with [disease name] than [gender 2].
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
   
   
===Race===
===Race===
*There is no racial predilection for [disease name].
*There is no racial predilection for mixed Mullerian tumor.
*[Disease name] usually affects individuals of the [race 1] race.
*[Race 2] individuals are less likely to develop [disease name].
   
   
==Risk Factors==
==Risk Factors==
*Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
*Common risk factors in the development of mixed Mullerian tumor are exposure to radiation, excessive estrogen exposure, obesity, and nulliparity.<ref name="pmid20642852">{{cite journal |vauthors=Kanthan R, Senger JL, Diudea D |title=Malignant mixed Mullerian tumors of the uterus: histopathological evaluation of cell cycle and apoptotic regulatory proteins |journal=World J Surg Oncol |volume=8 |issue= |pages=60 |year=2010 |pmid=20642852 |pmc=2913917 |doi=10.1186/1477-7819-8-60 |url=}}</ref>
   
   
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*The majority of patients with [disease name] remain asymptomatic for [duration/years].
*Early clinical features include postmenopausal vaginal bleeding, abdominal pain, and metrorrhagia.
*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
*If left untreated, the majority of patients with mixed Mullerian tumor may progress quickly to develop lymph node invasion, metastasis, and death.  
*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*Common complications of mixed Mullerian tumor include  
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
*Prognosis is generally poor, and the 5-year survival rate of patients with Mixed Mullerian tumor is approximately 33% to 39%.
*Prognosis is generally poor, and the 5-year survival rate of patients with Mixed Mullerian tumor is approximately 33% to 39%.
*Findings associated with good prognosis includes p16 and Mcl-1 genetic expression.


== Diagnosis ==
== Diagnosis ==
===Diagnostic Criteria===
===Diagnostic Criteria===
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
*The diagnosis of mixed Mullerian tumor is made with biopsy.
:*[criterion 1]
*Biopsy findings of mixed Mullerian tumor, include:
:*[criterion 2]
:*Tumor with carcinomatous and sarcoma-like elements
:*[criterion 3]
:*Angiolymphatic invasion
:*[criterion 4]
 
=== Symptoms ===
=== Symptoms ===
*[Disease name] is usually asymptomatic.
*Mixed Mullerian tumor is usually asymptomatic.
*Symptoms of [disease name] may include the following:
*Symptoms of mixed Mullerian tumor may include the following:<ref name="pmid20642852">{{cite journal |vauthors=Kanthan R, Senger JL, Diudea D |title=Malignant mixed Mullerian tumors of the uterus: histopathological evaluation of cell cycle and apoptotic regulatory proteins |journal=World J Surg Oncol |volume=8 |issue= |pages=60 |year=2010 |pmid=20642852 |pmc=2913917 |doi=10.1186/1477-7819-8-60 |url=}}</ref>
:*[symptom 1]
*Early symptoms may include:  
:*[symptom 2]
:* Abnormal [[vaginal bleeding]], abnormal menstrual periods
:*[symptom 3]
:* [[Metrorrhagia]] in premenopausal women
:*[symptom 4]
:* Postmenopausal [[vaginal bleeding]] <ref name="pmid22513918">{{cite journal| author=Kong A, Johnson N, Kitchener HC, Lawrie TA| title=Adjuvant radiotherapy for stage I endometrial cancer. | journal=Cochrane Database Syst Rev | year= 2012 | volume= 4 | issue=  | pages= CD003916 | pmid=22513918 | doi=10.1002/14651858.CD003916.pub4 | pmc=PMC4164955 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22513918  }} </ref>
:*[symptom 5]
:* Postcoital bleeding
:*[symptom 6]
*Advanced symptoms may include:
:* [[Polyuria]] and [[dysuria]], If a tumor places pressure on the bladder or urethra
:* [[Pelvic pain]] and [[dyspareunia]]
:* [[Fatigue]] and unexplained [[weight loss]]
 
=== Physical Examination ===
=== Physical Examination ===
*Patients with [disease name] usually appear [general appearance].
*Patients with mixed Mullerian tumor may have a normal appearance.
*Physical examination may be remarkable for:
*Pelvic examination may be remarkable for:
:*[finding 1]
:*Vaginal bleeding
:*[finding 2]
:*Enlarged uterus (advanced stage)
:*[finding 3]
 
:*[finding 4]
:*[finding 5]
:*[finding 6]
=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].
*There are no specific laboratory findings associated with mixed Mullerian tumor.


*A  [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
===Imaging Findings===
===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].
*Enhanced CT scan and MRI is the imaging modalities of choice for mixed Mullerian tumor.
*On MRI, findings of mixed Mullerian tumor, may include: 
*[Imaging study 1] is the imaging modality of choice for [disease name].
:*T1: predominantly isointense to both myometrium (75%) and endometrium (70%)
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
:*T2:hyper-intense to myometrium (90%) either hypo-intense (55%) or isointense (41%) to endometrium.  
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
*On enhanced CT, findings of mixed Mullerian tumor, may include: 
:*Heterogeneously hypodense and ill defined mass
:*Dilatation of uterine cavity
   
   
=== Other Diagnostic Studies ===
=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Mixed Mullerian tumor may also be diagnosed using laparoscopy.  
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
   
   
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.
*There is no treatment for mixed Mullerian tumor; the mainstay of therapy is supportive care.
*The medical management for mixed Mullerian tumor, include:
*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
   
   
=== Surgery ===
=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*Surgery is the mainstay of therapy for mixed Mullerian tumor.
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*Total hysterectomy in conjunction with surgical staging is the most common approach to the treatment of mixed Mullerian tumor.
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].
*Different surgical procedures for the treatment of  mixed Mullerian tumor, include:
:*Total hysterectomy
:*Bilateral salpingo-oophorectomy
:*Pelvic and para-aortic lymph node dissection
:*Cytology of peritoneal washings
:*Omentectomy
:*Biopsies of peritoneal surfaces
   
   
=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for [disease name].
*There are no primary preventive measures available for mixed Mullerian tumor.
*Once diagnosed and successfully treated, patients with mixed Mullerian tumor are followed-up every 6 or 12 months.
*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
*Follow-up testing include pelvic examination, ultrasound, and biomarker monitorization.  
 
*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].  


==References==
==References==

Revision as of 19:19, 5 April 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Mixed Müllerian tumor; MMMT; Malignant mixed Müllerian tumor; Carcinosarcoma of the uterus; Sarcomatoid carcinoma of the uterus; Malignant mesodermal mixed tumor; Metaplastic carcinoma

Overview

Mixed Mullerian tumor (MMMT) is a rare uterine sarcoma or carcinosarcoma.

Historical Perspective

  • Mixed Mullerian tumor was first described by Ferriera and colleagues in 1951.[1]

Classification

  • Mixed Mullerian tumors are normally composed of both carcinomatous (epithelial) and sarcomatous (mesodermal) components.
  • Mixed Mullerian tumor may be classified according to pathology findings into 2 types:

Epitheloid subtype

  • Endometroid adenocarcinoma (most common)
  • Clear cell carcinoma
  • Mucinous carcinoma
  • Papillary-serous carcinoma

Sarcomatoid subtype

  • Undifferentiated sarcoma
  • Rhabdomyosarcoma
  • Mixed Mullerian tumor may also be classified according to antomical location into 7 types:
  • Uterine corpus
  • Cervix
  • Ovaries
  • Fallopian tubes
  • Vagina
  • Peritoneum
  • Extragenital sites

Pathophysiology

  • The pathogenesis of mixed Mullerian tumor is characterized by
  • Mixed Mullerian tumor
  • On gross pathology, a large cervical mass is a characteristic finding of mixed Mullerian tumor.
  • On microscopic histopathological analysis, high-grade stromal sarcoma, poorly differentiated epithelial cells, and angiolymphatic invasion are characteristic findings of mixed Mullerian tumor.

Causes

  • Mixed Mullerian tumor may be caused by precursor lesions, such as
  • Mixed Mullerian tumor is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
  • There are no established causes for mixed Mullerian tumor.

Differentiating mixed Mullerian tumor from other Diseases

  • Mixed Mullerian tumor must be differentiated from other diseases that cause abnormal vaginal bleeding, abdominal pain, and metrorrhagia, such as:
  • Uterine leiomyosarcoma
  • Adenocarcinoma of the uterus
  • Endometrial cancer

Epidemiology and Demographics

  • The prevalence of mixed Mullerian tumor is approximately [number or range] per 100,000 individuals worldwide.
  • In [year], the incidence of mixed Mullerian tumor was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • The median age at diagnosis of Mixed Mullerian tumor is 66 years
  • Mixed Mullerian tumor is more commonly observed among patients aged between 50 and 60 years old.
  • Mixed Mullerian tumor is more commonly observed among postmenopausal women

Race

  • There is no racial predilection for mixed Mullerian tumor.

Risk Factors

  • Common risk factors in the development of mixed Mullerian tumor are exposure to radiation, excessive estrogen exposure, obesity, and nulliparity.[2]

Natural History, Complications and Prognosis

  • Early clinical features include postmenopausal vaginal bleeding, abdominal pain, and metrorrhagia.
  • If left untreated, the majority of patients with mixed Mullerian tumor may progress quickly to develop lymph node invasion, metastasis, and death.
  • Common complications of mixed Mullerian tumor include
  • Prognosis is generally poor, and the 5-year survival rate of patients with Mixed Mullerian tumor is approximately 33% to 39%.
  • Findings associated with good prognosis includes p16 and Mcl-1 genetic expression.

Diagnosis

Diagnostic Criteria

  • The diagnosis of mixed Mullerian tumor is made with biopsy.
  • Biopsy findings of mixed Mullerian tumor, include:
  • Tumor with carcinomatous and sarcoma-like elements
  • Angiolymphatic invasion

Symptoms

  • Mixed Mullerian tumor is usually asymptomatic.
  • Symptoms of mixed Mullerian tumor may include the following:[2]
  • Early symptoms may include:
  • Advanced symptoms may include:

Physical Examination

  • Patients with mixed Mullerian tumor may have a normal appearance.
  • Pelvic examination may be remarkable for:
  • Vaginal bleeding
  • Enlarged uterus (advanced stage)

Laboratory Findings

  • There are no specific laboratory findings associated with mixed Mullerian tumor.

Imaging Findings

  • Enhanced CT scan and MRI is the imaging modalities of choice for mixed Mullerian tumor.
  • On MRI, findings of mixed Mullerian tumor, may include:
  • T1: predominantly isointense to both myometrium (75%) and endometrium (70%)
  • T2:hyper-intense to myometrium (90%) either hypo-intense (55%) or isointense (41%) to endometrium.
  • On enhanced CT, findings of mixed Mullerian tumor, may include:
  • Heterogeneously hypodense and ill defined mass
  • Dilatation of uterine cavity

Other Diagnostic Studies

  • Mixed Mullerian tumor may also be diagnosed using laparoscopy.

Treatment

Medical Therapy

  • There is no treatment for mixed Mullerian tumor; the mainstay of therapy is supportive care.
  • The medical management for mixed Mullerian tumor, include:

Surgery

  • Surgery is the mainstay of therapy for mixed Mullerian tumor.
  • Total hysterectomy in conjunction with surgical staging is the most common approach to the treatment of mixed Mullerian tumor.
  • Different surgical procedures for the treatment of mixed Mullerian tumor, include:
  • Total hysterectomy
  • Bilateral salpingo-oophorectomy
  • Pelvic and para-aortic lymph node dissection
  • Cytology of peritoneal washings
  • Omentectomy
  • Biopsies of peritoneal surfaces

Prevention

  • There are no primary preventive measures available for mixed Mullerian tumor.
  • Once diagnosed and successfully treated, patients with mixed Mullerian tumor are followed-up every 6 or 12 months.
  • Follow-up testing include pelvic examination, ultrasound, and biomarker monitorization.

References

  1. ↑ Wright JD, Rosenblum K, Huettner PC, Mutch DG, Rader JS, Powell MA, Gibb RK (2005). "Cervical sarcomas: an analysis of incidence and outcome". Gynecol. Oncol. 99 (2): 348–51. doi:10.1016/j.ygyno.2005.06.021. PMID 16051326.
  2. ↑ 2.0 2.1 Kanthan R, Senger JL, Diudea D (2010). "Malignant mixed Mullerian tumors of the uterus: histopathological evaluation of cell cycle and apoptotic regulatory proteins". World J Surg Oncol. 8: 60. doi:10.1186/1477-7819-8-60. PMC 2913917. PMID 20642852.
  3. ↑ Kong A, Johnson N, Kitchener HC, Lawrie TA (2012). "Adjuvant radiotherapy for stage I endometrial cancer". Cochrane Database Syst Rev. 4: CD003916. doi:10.1002/14651858.CD003916.pub4. PMC 4164955. PMID 22513918.
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