Leiomyosarcoma differential diagnosis: Difference between revisions

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==Overview==
==Overview==
Leiomyosarcoma must be differentiated from other soft tissue tumors, some variants of leimyoma resembles malignant tumor. Other possible differential diagnosis of leimyosarcoma include, epithelioid endometrial stromal sarcoma, alveolar soft part sarcoma, epithelioid angiosarcoma, pleomorphic rhabdomyosarcoma.
Leiomyosarcoma must be differentiated from other soft tissue tumors, some variants of [[leiomyoma]] resembles malignant tumor. Other possible differential diagnosis of leimyosarcoma include, epithelioid endometrial stromal sarcoma, epithelioid [[angiosarcoma]].


==Differentiating Leiomyosarcoma from other Diseases==
==Differentiating Leiomyosarcoma from other Diseases==
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Uterine]] Leiomyosarcoma<ref name="SantosCunha2015">{{cite journal|last1=Santos|first1=Pedro|last2=Cunha|first2=Teresa Margarida|title=Uterine sarcomas: clinical presentation and MRI features|journal=Diagnostic and Interventional Radiology|volume=21|issue=1|year=2015|pages=4–9|issn=13053825|doi=10.5152/dir.2014.14053}}</ref><ref name="HataHata1990">{{cite journal|last1=Hata|first1=Kohkichi|last2=Hata|first2=Toshiyuki|last3=Makihara|first3=Ken|last4=Aoki|first4=Showa|last5=Takamiya|first5=Osamu|last6=Kitao|first6=Manabu|last7=Harada|first7=Yuji|last8=Nagaoka|first8=Saburo|title=Sonographic Findings of Uterine Leiomyosarcoma|journal=Gynecologic and Obstetric Investigation|volume=30|issue=4|year=1990|pages=242–245|issn=1423-002X|doi=10.1159/000293278}}</ref><ref name="RhaByun2003">{{cite journal|last1=Rha|first1=Sung Eun|last2=Byun|first2=Jae Young|last3=Jung|first3=Seung Eun|last4=Lee|first4=Soo Lim|last5=Cho|first5=Song Mee|last6=Hwang|first6=Seong Su|last7=Lee|first7=Hae Giu|last8=Namkoong|first8=Sung-Eun|last9=Lee|first9=Jae Mun|title=CT and MRI of Uterine Sarcomas and Their Mimickers|journal=American Journal of Roentgenology|volume=181|issue=5|year=2003|pages=1369–1374|issn=0361-803X|doi=10.2214/ajr.181.5.1811369}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Uterine]] Leiomyosarcoma<ref name="SantosCunha2015">{{cite journal|last1=Santos|first1=Pedro|last2=Cunha|first2=Teresa Margarida|title=Uterine sarcomas: clinical presentation and MRI features|journal=Diagnostic and Interventional Radiology|volume=21|issue=1|year=2015|pages=4–9|issn=13053825|doi=10.5152/dir.2014.14053}}</ref><ref name="HataHata1990">{{cite journal|last1=Hata|first1=Kohkichi|last2=Hata|first2=Toshiyuki|last3=Makihara|first3=Ken|last4=Aoki|first4=Showa|last5=Takamiya|first5=Osamu|last6=Kitao|first6=Manabu|last7=Harada|first7=Yuji|last8=Nagaoka|first8=Saburo|title=Sonographic Findings of Uterine Leiomyosarcoma|journal=Gynecologic and Obstetric Investigation|volume=30|issue=4|year=1990|pages=242–245|issn=1423-002X|doi=10.1159/000293278}}</ref><ref name="RhaByun2003">{{cite journal|last1=Rha|first1=Sung Eun|last2=Byun|first2=Jae Young|last3=Jung|first3=Seung Eun|last4=Lee|first4=Soo Lim|last5=Cho|first5=Song Mee|last6=Hwang|first6=Seong Su|last7=Lee|first7=Hae Giu|last8=Namkoong|first8=Sung-Eun|last9=Lee|first9=Jae Mun|title=CT and MRI of Uterine Sarcomas and Their Mimickers|journal=American Journal of Roentgenology|volume=181|issue=5|year=2003|pages=1369–1374|issn=0361-803X|doi=10.2214/ajr.181.5.1811369}}</ref>
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Abnormal vaginal bleeding (Postmenopausal or intermenstrual bleeding or bleeding from uterus or vagina)
* Abnormal [[vaginal bleeding]] ([[Postmenopausal]] or intermenstrual [[bleeding]] or bleeding from [[uterus]] or vagina)
* Pressure or pain pelvic or abdomen
* Pressure or [[pain]] [[pelvic]] or [[abdomen]]
* Vaginal dyscharge
* [[Vaginal discharge]]
* Change in bladder or bowel habits
* Change in [[bladder]] or [[bowel]] habits
* Fatigue
* [[Fatigue]]
* Fever
* [[Fever]]
* Malaise
* [[Malaise]]
* Weight loss
* [[Weight loss]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Lump protruding from vagina
* [[Lump]] protruding from [[vagina]]


* Mass in abdomen or pelvic
* Mass in [[abdomen]] or [[pelvic]]


* Tendenrness on palpating pelvic or Abdomen
* [[Tenderness]] on palpating [[pelvic]] or [[abdomen]]


* Lymphadenopathy
* [[Lymphadenopathy]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* ↓ RBC or Hemoglobin may be seen on CBC.  
* ↓ [[RBC]] or [[Hemoglobin]] may be seen on [[CBC]].
* In uterine leiomyosarcomas (LMSs) [[p16]] is overexpressed compared with [[leiomyoma]].  
| style="background: #F5F5F5; padding: 5px;" |
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* Deep myometrial invasion
* Deep [[myometrial]] invasion


*
*
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Massive uterine enlargement
* Massive [[uterine]] enlargement
* Irregular central zones of low attenuation,
* Irregular central zones of low attenuation,
* Extensive necrosis and hemorrhage
* Extensive [[necrosis]] and [[hemorrhage]]
| style="background: #F5F5F5; padding: 5px;" |Features such as tumor localization, irregular or nodular margins, necrosis, rapid growth, intense contrast enhancement, and restriction at diffusion-weighted imaging can suggest the diagnosis and help differentiate from more common leiomyomas and endometrial carcinoma.
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* Features such as tumor localization, irregular or nodular margins, [[necrosis]], rapid growth, intense contrast enhancement, and restriction at [[diffusion-weighted imaging]] can suggest the diagnosis and help differentiate from more common [[leiomyoma]]<nowiki/>s and [[endometrial carcinoma]].
| style="background: #F5F5F5; padding: 5px;" |
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* '''Gross histology:'''
* '''Gross histology:'''
* Large, solitary, poorly circumscribed masses (average 10 cm)   
* Large, solitary, poorly circumscribed masses (average 10 cm)   
* Typically display a fleshy variegated cut surface with areas of hemorrhage or necrosis
* Typically display a fleshy variegated cut surface with areas of [[hemorrhage]] or [[necrosis]]
| style="background: #F5F5F5; padding: 5px;" |Histology and biopsy
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* [[Histology]] and [[biopsy]]
| style="background: #F5F5F5; padding: 5px;" |
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* Women with sarcoma vs women with fibroids, tend to be older
* Women with [[sarcoma]] vs women with [[fibroids]], tend to be older
* More likely to be postmenopausal
* More likely to be [[postmenopausal]]
* More likely to have a history of another nonuterine malignancy
* More likely to have a history of another nonuterine [[malignancy]]
* Women with sarcoma were more likely to have masses that are subserosal
* Women with [[sarcoma]] were more likely to have masses that are subserosal
* Solitary rather than multiple uterine mass
* Solitary rather than multiple [[uterine]] mass
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Leimyoma (mitotically active type)
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''[[Leiomyoma]]''' ('''mitotically active type''')<ref name="WoźniakWoźniak2017">{{cite journal|last1=Woźniak|first1=Andrzej|last2=Woźniak|first2=Sławomir|title=Ultrasonography of uterine leiomyomas|journal=Menopausal Review|volume=16|issue=4|year=2017|pages=113–117|issn=1643-8876|doi=10.5114/pm.2017.72754}}</ref><ref name="BodnerBodner-Adler2016">{{cite journal|last1=Bodner|first1=Klaus|last2=Bodner-Adler|first2=Barbara|last3=Kimberger|first3=Oliver|last4=Czerwenka|first4=Klaus|last5=Mayerhofer|first5=Klaus|title=Bcl-2 Receptor Expression in Patients With Uterine Smooth Muscle Tumors: An Immunohistochemical Analysis Comparing Leiomyoma, Uterine Smooth Muscle Tumor of Uncertain Malignant Potential, and Leiomyosarcoma|journal=Journal of the Society for Gynecologic Investigation|volume=11|issue=3|year=2016|pages=187–191|issn=1071-5576|doi=10.1016/j.jsgi.2003.10.003}}</ref><ref name="pmid27190823">{{cite journal |vauthors=Adaikkalam J |title=Lipoleiomyoma of Cervix |journal=J Clin Diagn Res |volume=10 |issue=4 |pages=EJ01–2 |date=April 2016 |pmid=27190823 |doi=10.7860/JCDR/2016/16505.7531 |url=}}</ref><ref name="HouserCarrasco1979">{{cite journal|last1=Houser|first1=L. Murray|last2=Carrasco|first2=C. H.|last3=Sheehan|first3=C. R.|title=Lipomatous tumour of the uterus: radiographic and ultrasonic appearance|journal=The British Journal of Radiology|volume=52|issue=624|year=1979|pages=992–993|issn=0007-1285|doi=10.1259/0007-1285-52-624-992}}</ref><ref name="KeriakosMaher2013">{{cite journal|last1=Keriakos|first1=Remon|last2=Maher|first2=Mark|title=Management of Cervical Fibroid during the Reproductive Period|journal=Case Reports in Obstetrics and Gynecology|volume=2013|year=2013|pages=1–3|issn=2090-6684|doi=10.1155/2013/984030}}</ref><ref name="pmid10775744">{{cite journal |vauthors=Coronado GD, Marshall LM, Schwartz SM |title=Complications in pregnancy, labor, and delivery with uterine leiomyomas: a population-based study |journal=Obstet Gynecol |volume=95 |issue=5 |pages=764–9 |date=May 2000 |pmid=10775744 |doi= |url=}}</ref>
| style="background: #F5F5F5; padding: 5px;" |
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* [[Vaginal bleeding]]
* [[Vaginal bleeding]]


* [[Pelvic pain]]
* [[Pelvic pain]]
* [[Urinary retention]]
* [[Constipation]]
* [[Infertility]]
* [[Bowel obstruction]]
* [[Vaginal discharge]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Large,irregular [[Pelvic masses|pelvic mass]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* CBC Suggestive of Anemia
* [[CBC]] suggestive of [[anemia]]
* [[Bcl-2]] expression is more frequent and strong marker in [[leiomyoma]]<nowiki/>s compared with leiomyosarcoma.
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* Enlarged uterus with multiple whorled mass lesions, largest of size 8.6×7.1 cm, not extending into the abdomen
* Enlarged [[uterus]] with multiple whorled mass lesions, largest of size 8.6×7.1 cm, not extending into the [[abdomen]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* An enlarged uterus and a deformed uterine contour are the most common CT findings of leiomyomas
* An enlarged [[uterus]] and a deformed uterine contour are the most common [[CT]] findings of leiomyomas
* Leiomyomas usually have a uniformly solid consistency
* [[Leiomyoma]]<nowiki/>s usually have a uniformly solid consistency
| style="background: #F5F5F5; padding: 5px;" |  
| style="background: #F5F5F5; padding: 5px;" |  
'''T2-weighted [[MRI]]''':
* Hypointense masses
* Homogeneous
enhancement
* Red degeneration
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| style="background: #F5F5F5; padding: 5px;" |
* Abscence of cytologic atypia
* Abscence of cytologic [[atypia]]


* Mild nuclear atypia, up to 10-20 mitosis
* Mild nuclear atypia, up to 10-20 [[mitosis]]


* No tumor cell necrosis
* No tumor cell [[necrosis]]
| style="background: #F5F5F5; padding: 5px;" |Biopsy and histology
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" | -
* [[Biopsy]] and [[histology]]
| style="background: #F5F5F5; padding: 5px;" |  
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Hemorrhagic]] cellular leiomyomas (apoplectic leiomyoma)<ref name="pmid4073354">{{cite journal |vauthors=Myles JL, Hart WR |title=Apoplectic leiomyomas of the uterus. A clinicopathologic study of five distinctive hemorrhagic leiomyomas associated with oral contraceptive usage |journal=Am. J. Surg. Pathol. |volume=9 |issue=11 |pages=798–805 |date=November 1985 |pmid=4073354 |doi= |url=}}</ref><ref>{{cite journal|doi=10.1097/PAS.0000000000000569.}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Hemorrhagic]] cellular [[leiomyoma]]<nowiki/>s (apoplectic [[leiomyoma]])<ref name="pmid4073354">{{cite journal |vauthors=Myles JL, Hart WR |title=Apoplectic leiomyomas of the uterus. A clinicopathologic study of five distinctive hemorrhagic leiomyomas associated with oral contraceptive usage |journal=Am. J. Surg. Pathol. |volume=9 |issue=11 |pages=798–805 |date=November 1985 |pmid=4073354 |doi= |url=}}</ref><ref>{{cite journal|doi=10.1097/PAS.0000000000000569.}}</ref>
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Abdominal pain
* [[Abdominal pain]]
* [[Vaginal bleeding]]
 
* [[Pelvic pain]]
* [[Urinary retention]]
* [[Constipation]]
* [[Infertility]]
* [[Bowel obstruction]]
* [[Vaginal discharge]]
*  
*  
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Large,irregular [[Pelvic masses|pelvic mass]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* ↓ [[RBC]] or [[Hemoglobin]] on [[CBC]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" | -
* Irregularly enlarged [[uterus]], usually hypoechoic but can be isoechoic and even hyperechoic as well.
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
* Enlarged [[uterus]] with irregular border
| style="background: #F5F5F5; padding: 5px;" | '''T2-weighted MRI''':
* Hypointense masses
| style="background: #F5F5F5; padding: 5px;" |'''On microscopic analysis:'''
| style="background: #F5F5F5; padding: 5px;" |'''On microscopic analysis:'''
* Zones of recent hemorrhage within nodules of hypercellular smooth muscle.  
* Zones of recent [[hemorrhage]] within nodules of hypercellular [[smooth muscle]].  


* Coagulative necrosis (red degeneration)
* [[Coagulative necrosis]] (red degeneration)
* Mitotic figure not exceeding 2/10 HPF, mostly located in the perihemorrhagic areas               '''On gross examination:'''
[[Mitotic]] figure not exceeding 2/10 HPF, mostly located in the perihemorrhagic areas
* Features of multiple hemorrhagic area
 
* Necrosis
'''On gross examination:'''
* Cyst formation
* Features of multiple [[hemorrhagic]] area
* Softening, or color different than the usual leiomyoma  
* [[Necrosis]]
| style="background: #F5F5F5; padding: 5px;" |Biopsy specimen and histology
* [[Cyst]] formation
* Softening, or color different than the usual [[leiomyoma]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
|-
* [[Biopsy]] specimen and [[histology]]
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Endometrial cancer|Endometrial Cancer]]
| style="background: #F5F5F5; padding: 5px;" |
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* Abnormal Vaginal bleeding or post coital bleeding
| style="background: #F5F5F5; padding: 5px;" |Enlarged uterus
| style="background: #F5F5F5; padding: 5px;" |CBC and Clotting studies to r/o anemia and coagulopathy
| style="background: #F5F5F5; padding: 5px;" |Thickened endometrial line on ultrasound.
| style="background: #F5F5F5; padding: 5px;" |Thickened endometrial line on CT
| style="background: #F5F5F5; padding: 5px;" |Thickened endometrial line on MRI
| style="background: #F5F5F5; padding: 5px;" |In well-differentiated forms, endometrioid adenocarcinoma produces small, round back-to-back glands without intervening stroma with varying degrees of glandular complexity are demonstrated by luminal infolding, budding, papillae (with or without psammoma bodies), and cribriforming. In grade 1 lesions, nuclei of the lining epithelial cells are uniform and oval to cylindrical, with minimal atypia and small discrete nucleoli. The cellular axes are perpendicular to the basement membrane, and stratification may or may not be present. Typically, high-grade tumors (with significant solid components) display an increased amount of nuclear atypia, as demonstrated by pleomorphism, irregular chromatin clumping, and prominent nucleoli 
| style="background: #F5F5F5; padding: 5px;" |Biopsy under hysteroscopic guidance
| style="background: #F5F5F5; padding: 5px;" | -
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |''[[PEComa]]''<ref name="TanZhang2013">{{cite journal|last1=Tan|first1=Y.|last2=Zhang|first2=H.|last3=Xiao|first3=E.-H.|title=Perivascular epithelioid cell tumour: Dynamic CT, MRI and clinicopathological characteristics—Analysis of 32 cases and review of the literature|journal=Clinical Radiology|volume=68|issue=6|year=2013|pages=555–561|issn=00099260|doi=10.1016/j.crad.2012.10.021}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |''[[PEComa]]''<ref name="TanZhang2013">{{cite journal|last1=Tan|first1=Y.|last2=Zhang|first2=H.|last3=Xiao|first3=E.-H.|title=Perivascular epithelioid cell tumour: Dynamic CT, MRI and clinicopathological characteristics—Analysis of 32 cases and review of the literature|journal=Clinical Radiology|volume=68|issue=6|year=2013|pages=555–561|issn=00099260|doi=10.1016/j.crad.2012.10.021}}</ref><ref name="KangSeo2014">{{cite journal|last1=Kang|first1=Jeong Ba|last2=Seo|first2=Jin Won|last3=Park|first3=Young-Han|last4=Jang|first4=Pong Rheem|title=Malignant Perivascular Epithelioid Cell Tumor of the Uterus with Lung Metastasis|journal=The Korean Journal of Pathology|volume=48|issue=6|year=2014|pages=454–457|issn=1738-1843|doi=10.4132/KoreanJPathol.2014.48.6.454}}</ref><ref>{{cite journal|title=PEComa of the Uterus: A Rare Mesenchymal Tumor Displaying a ≪Snowstorm≫ Pattern at Magnetic Resonance Imaging|journal=Journal of the Belgian Society of Radiology|volume=100|issue=1|year=2016|issn=1780-2393|doi=10.5334/jbr-btr.926}}</ref>
| style="background: #F5F5F5; padding: 5px;" |
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* Palpable [[Abdomen|abdominal]] masses
* Palpable [[Abdomen|abdominal]] masses
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* Dull [[Abdomen|abdominal]] [[pain]] in [[RUQ|right upper quadrant]] ([[liver]])
* Dull [[Abdomen|abdominal]] [[pain]] in [[RUQ|right upper quadrant]] ([[liver]])
| style="background: #F5F5F5; padding: 5px;" |
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* Lower [[abdominal pain]] with a palpable [[mass]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* PECs typically stain for [[Melanocyte|melanocytic]] markers ([[HMB-45]], HMSA-1, Melan A (Mart 1), microophthalmia [[transcription factor]] (Mitf), [[myogenic]] markers ([[actin]]), and less commonly [[desmin]]
* PECs typically stain for [[Melanocyte|melanocytic]] markers ([[HMB-45]], HMSA-1, Melan A (Mart 1), micro-ophthalmia [[transcription factor]] (Mitf), [[myogenic]] markers ([[actin]]), and less commonly [[desmin]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Granulomatous]] ovoid [[uterine]] [[tumor]] with a rich vascular network
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Mean tumor diameter of 5 cm, well-defined margins, hypodense mass  
* Mean tumor diameter of 5 cm, well-defined margins, hypodense mass  
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Low and high signal intensity on T1- and T2-weighted MRI
* Low and high signal intensity on T1- and T2-weighted [[MRI]]
* Strong, heterogeneous enhancement
* Strong, heterogeneous enhancement
| style="background: #F5F5F5; padding: 5px;" |'''Microscopic analysis:'''
* [[Perivascular cell|Perivascular]] location
* Epithelioid, elongated and spindle shaped, or vacuolated ([[adipocyte]]-like).
* Clear to granular, lightly [[eosinophilic]] cytoplasm
* [[Nuclear]] atypia
*
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* [[Biopsy]]
| style="background: #F5F5F5; padding: 5px;" |
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* Association with [[lymphangiomyomatosis]] and [[tuberous sclerosis]]
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Epitheloid [[angiosarcoma]]'''<ref>{{cite journal|doi=10.1043/1543-2165-135.2.268.}}</ref><ref name="HwangLim2013">{{cite journal|last1=Hwang|first1=Jae Pil|last2=Lim|first2=Sang Moo|title=Uterine Epithelioid Angiosarcoma on F-18 FDG PET/CT|journal=Nuclear Medicine and Molecular Imaging|volume=47|issue=2|year=2013|pages=134–137|issn=1869-3474|doi=10.1007/s13139-013-0191-y}}</ref><ref name="ChenFirth2018">{{cite journal|last1=Chen|first1=Innie|last2=Firth|first2=Bianca|last3=Hopkins|first3=Laura|last4=Bougie|first4=Olga|last5=Xie|first5=Ri-hua|last6=Singh|first6=Sukhbir|title=Clinical Characteristics Differentiating Uterine Sarcoma and Fibroids|journal=JSLS : Journal of the Society of Laparoendoscopic Surgeons|volume=22|issue=1|year=2018|pages=e2017.00066|issn=1086-8089|doi=10.4293/JSLS.2017.00066}}</ref>
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* Association with lymphangiomyomatosis and tuberous sclerosis
* [[Painful]], enlarging soft tissue masses
 
* Long [[bone fractures]]
* Arteriovenous [[shunting]] and subsequent high-output [[cardiac failure]] symptoms such as [[dyspnea]] on exertion.
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* [[Peritoneal]] bleeding in some cases
* Painful, enlarging soft tissue masses
| style="background: #F5F5F5; padding: 5px;" |
* Strongly positive for [[vimentin]]
* Positive for [[cytokeratin]]
* [[CD31]], [[CD34]]
| style="background: #F5F5F5; padding: 5px;" |
* Nonspecific
 
* Bulky or normal size [[uterus]]
 
* Intratumoral blood flow on [[Doppler]]
| style="background: #F5F5F5; padding: 5px;" |
* Enhanced huge enhanced round heterogenous solid [[mass]] with internal multifocal cysts on [[CT scan]]
| style="background: #F5F5F5; padding: 5px;" |'''T2-weighted MRI:'''
* Focal areas of high signal intensity, known as the “cauliflower-like appearance” on gadolinium-enhanced [[MRI]]
| style="background: #F5F5F5; padding: 5px;" |'''Microscopic histology:'''
* [[Nodules]], and trabeculae of infiltrative epithelioid to spindled cells
* [[Eosinophilic]] [[cytoplasm]].
| style="background: #F5F5F5; padding: 5px;" |
* [[Biopsy]] and [[histology]]
| style="background: #F5F5F5; padding: 5px;" |
* Epithelioid [[angiosarcoma]] has a male predilection
* Most often arises in the deep [[soft tissue]]<nowiki/>s (usually intramuscular) of the extremities, but a variety of primary sites, including the [[thyroid gland]] can be involved.
* Early nodal and solid organ [[metastasis]], especially to the [[lungs]], [[bone]], [[soft tissue]], and [[skin]].
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Epitheloid angiosarcoma'''<ref>{{cite journal|doi=10.1043/1543-2165-135.2.268.}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''[[Intravenous leiomyomatosis]]'''<ref name="pmid7820530">{{cite journal| author=Nakayama Y, Kitamura S, Kawachi K, Kawata T, Fukutomi M, Hasegawa J et al.| title=Intravenous leiomyomatosis extending into the right atrium. | journal=Cardiovasc Surg | year= 1994 | volume= 2 | issue= 5 | pages= 642-5 | pmid=7820530 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7820530  }}</ref><ref name="pmid22340462">{{cite journal |vauthors=Kang LQ, Zhang B, Liu BG, Liu FH |title=Diagnosis of intravenous leiomyomatosis extending to heart with emphasis on magnetic resonance imaging |journal=Chin. Med. J. |volume=125 |issue=1 |pages=33–7 |date=January 2012 |pmid=22340462 |doi= |url=}}</ref><ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref>
| style="background: #F5F5F5; padding: 5px;" |
* [[Symptoms]] varies depending on [[tumor]] location.
* [[Symptoms]] related to [[uterine leiomyoma]] such as [[abdominal pain]], [[abnormal uterine bleeding]], and [[abdominal distension]]
* [[Dyspnea]], [[lower extremity edema]] in case of [[cardiac]] involvement
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Painful, enlarging soft tissue masses
* [[Jugular venous distension]]
 
* [[Lower extremity edema]]
* Long bone fractures
* [[Abdominal mass]]
* Arteriovenous shunting and subsequent high-output cardiac failure symptoms such as dypnea on exertion.
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* No specific lab finding
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Vascularized [[thrombi]] within the [[pelvic]] [[veins]] and [[inferior vena cava]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Irregular [[mass]] exhibiting [[heterogeneous]] enhancement with [[extension]] into adjacent [[veins]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Leiomyomatous [[lesion]] or a distorted [[uterus]] with projections into [[vasculature]].
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |'''Microscopic histology:'''
* [[Benign]], well-differentiated [[tumor]] with [[smooth muscle]] growing within [[veins]] as worm-like projections.
* Nodules, and trabeculae of infiltrative epithelioid to spindled cells
* Eosinophilic cytoplasm.
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Biopsy]] and [[Histology]]
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[Echocardiography]] may show hyperechoic elongated mobile [[mass]] extending from the [[IVC]] and an irregular lesion in the [[right atrium]] with protrusion into the [[right ventricle]]
|}
|}



Latest revision as of 20:03, 23 October 2019

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

Overview

Leiomyosarcoma must be differentiated from other soft tissue tumors, some variants of leiomyoma resembles malignant tumor. Other possible differential diagnosis of leimyosarcoma include, epithelioid endometrial stromal sarcoma, epithelioid angiosarcoma.

Differentiating Leiomyosarcoma from other Diseases

The table below summarizes the findings that differentiate Leiomyosarcoma from other conditions that may cause similar signs and symptoms.[1][2][3]

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histology
Ultrasound CT scan MRI
Uterine Leiomyosarcoma[4][5][6]
  • Gross histology:
  • Large, solitary, poorly circumscribed masses (average 10 cm)
  • Typically display a fleshy variegated cut surface with areas of hemorrhage or necrosis
  • Women with sarcoma vs women with fibroids, tend to be older
  • More likely to be postmenopausal
  • More likely to have a history of another nonuterine malignancy
  • Women with sarcoma were more likely to have masses that are subserosal
  • Solitary rather than multiple uterine mass
Leiomyoma (mitotically active type)[7][8][9][10][11][12]
  • CBC suggestive of anemia
  • Bcl-2 expression is more frequent and strong marker in leiomyomas compared with leiomyosarcoma.
  • Enlarged uterus with multiple whorled mass lesions, largest of size 8.6×7.1 cm, not extending into the abdomen
  • An enlarged uterus and a deformed uterine contour are the most common CT findings of leiomyomas
  • Leiomyomas usually have a uniformly solid consistency

T2-weighted MRI:

  • Hypointense masses
  • Homogeneous

enhancement

  • Red degeneration
  • Mild nuclear atypia, up to 10-20 mitosis
Hemorrhagic cellular leiomyomas (apoplectic leiomyoma)[13][14]
  • Irregularly enlarged uterus, usually hypoechoic but can be isoechoic and even hyperechoic as well.
  • Enlarged uterus with irregular border
T2-weighted MRI:
  • Hypointense masses
On microscopic analysis:

Mitotic figure not exceeding 2/10 HPF, mostly located in the perihemorrhagic areas

On gross examination:

PEComa[15][16][17]
  • Mean tumor diameter of 5 cm, well-defined margins, hypodense mass
  • Low and high signal intensity on T1- and T2-weighted MRI
  • Strong, heterogeneous enhancement
Microscopic analysis:
Epitheloid angiosarcoma[18][19][20]
  • Painful, enlarging soft tissue masses
  • Peritoneal bleeding in some cases
  • Painful, enlarging soft tissue masses
  • Nonspecific
  • Enhanced huge enhanced round heterogenous solid mass with internal multifocal cysts on CT scan
T2-weighted MRI:
  • Focal areas of high signal intensity, known as the “cauliflower-like appearance” on gadolinium-enhanced MRI
Microscopic histology:
Intravenous leiomyomatosis[21][22][23]
  • No specific lab finding

References

  1. Cotton PB, Shorvon PJ (1984) Analysis of endoscopy and radiography in the diagnosis, follow-up and treatment of peptic ulcer disease. Clin Gastroenterol 13 (2):383-403. PMID: 6378443
  2. Wanebo HJ, Kennedy BJ, Chmiel J, Steele G, Winchester D, Osteen R (1993) Cancer of the stomach. A patient care study by the American College of Surgeons. Ann Surg 218 (5):583-92. PMID: 8239772
  3. Kimura T, Kamiura S, Yamamoto T, Seino-Noda H, Ohira H, Saji F (2004) Abnormal uterine bleeding and prognosis of endometrial cancer. Int J Gynaecol Obstet 85 (2):145-50. DOI:10.1016/j.ijgo.2003.12.001 PMID: 15099776
  4. Santos, Pedro; Cunha, Teresa Margarida (2015). "Uterine sarcomas: clinical presentation and MRI features". Diagnostic and Interventional Radiology. 21 (1): 4–9. doi:10.5152/dir.2014.14053. ISSN 1305-3825.
  5. Hata, Kohkichi; Hata, Toshiyuki; Makihara, Ken; Aoki, Showa; Takamiya, Osamu; Kitao, Manabu; Harada, Yuji; Nagaoka, Saburo (1990). "Sonographic Findings of Uterine Leiomyosarcoma". Gynecologic and Obstetric Investigation. 30 (4): 242–245. doi:10.1159/000293278. ISSN 1423-002X.
  6. Rha, Sung Eun; Byun, Jae Young; Jung, Seung Eun; Lee, Soo Lim; Cho, Song Mee; Hwang, Seong Su; Lee, Hae Giu; Namkoong, Sung-Eun; Lee, Jae Mun (2003). "CT and MRI of Uterine Sarcomas and Their Mimickers". American Journal of Roentgenology. 181 (5): 1369–1374. doi:10.2214/ajr.181.5.1811369. ISSN 0361-803X.
  7. Woźniak, Andrzej; Woźniak, Sławomir (2017). "Ultrasonography of uterine leiomyomas". Menopausal Review. 16 (4): 113–117. doi:10.5114/pm.2017.72754. ISSN 1643-8876.
  8. Bodner, Klaus; Bodner-Adler, Barbara; Kimberger, Oliver; Czerwenka, Klaus; Mayerhofer, Klaus (2016). "Bcl-2 Receptor Expression in Patients With Uterine Smooth Muscle Tumors: An Immunohistochemical Analysis Comparing Leiomyoma, Uterine Smooth Muscle Tumor of Uncertain Malignant Potential, and Leiomyosarcoma". Journal of the Society for Gynecologic Investigation. 11 (3): 187–191. doi:10.1016/j.jsgi.2003.10.003. ISSN 1071-5576.
  9. Adaikkalam J (April 2016). "Lipoleiomyoma of Cervix". J Clin Diagn Res. 10 (4): EJ01–2. doi:10.7860/JCDR/2016/16505.7531. PMID 27190823.
  10. Houser, L. Murray; Carrasco, C. H.; Sheehan, C. R. (1979). "Lipomatous tumour of the uterus: radiographic and ultrasonic appearance". The British Journal of Radiology. 52 (624): 992–993. doi:10.1259/0007-1285-52-624-992. ISSN 0007-1285.
  11. Keriakos, Remon; Maher, Mark (2013). "Management of Cervical Fibroid during the Reproductive Period". Case Reports in Obstetrics and Gynecology. 2013: 1–3. doi:10.1155/2013/984030. ISSN 2090-6684.
  12. Coronado GD, Marshall LM, Schwartz SM (May 2000). "Complications in pregnancy, labor, and delivery with uterine leiomyomas: a population-based study". Obstet Gynecol. 95 (5): 764–9. PMID 10775744.
  13. Myles JL, Hart WR (November 1985). "Apoplectic leiomyomas of the uterus. A clinicopathologic study of five distinctive hemorrhagic leiomyomas associated with oral contraceptive usage". Am. J. Surg. Pathol. 9 (11): 798–805. PMID 4073354.
  14. . doi:10.1097/PAS.0000000000000569. Check |doi= value (help). Missing or empty |title= (help)
  15. Tan, Y.; Zhang, H.; Xiao, E.-H. (2013). "Perivascular epithelioid cell tumour: Dynamic CT, MRI and clinicopathological characteristics—Analysis of 32 cases and review of the literature". Clinical Radiology. 68 (6): 555–561. doi:10.1016/j.crad.2012.10.021. ISSN 0009-9260.
  16. Kang, Jeong Ba; Seo, Jin Won; Park, Young-Han; Jang, Pong Rheem (2014). "Malignant Perivascular Epithelioid Cell Tumor of the Uterus with Lung Metastasis". The Korean Journal of Pathology. 48 (6): 454–457. doi:10.4132/KoreanJPathol.2014.48.6.454. ISSN 1738-1843.
  17. "PEComa of the Uterus: A Rare Mesenchymal Tumor Displaying a ≪Snowstorm≫ Pattern at Magnetic Resonance Imaging". Journal of the Belgian Society of Radiology. 100 (1). 2016. doi:10.5334/jbr-btr.926. ISSN 1780-2393.
  18. . doi:10.1043/1543-2165-135.2.268. Check |doi= value (help). Missing or empty |title= (help)
  19. Hwang, Jae Pil; Lim, Sang Moo (2013). "Uterine Epithelioid Angiosarcoma on F-18 FDG PET/CT". Nuclear Medicine and Molecular Imaging. 47 (2): 134–137. doi:10.1007/s13139-013-0191-y. ISSN 1869-3474.
  20. Chen, Innie; Firth, Bianca; Hopkins, Laura; Bougie, Olga; Xie, Ri-hua; Singh, Sukhbir (2018). "Clinical Characteristics Differentiating Uterine Sarcoma and Fibroids". JSLS : Journal of the Society of Laparoendoscopic Surgeons. 22 (1): e2017.00066. doi:10.4293/JSLS.2017.00066. ISSN 1086-8089.
  21. Nakayama Y, Kitamura S, Kawachi K, Kawata T, Fukutomi M, Hasegawa J; et al. (1994). "Intravenous leiomyomatosis extending into the right atrium". Cardiovasc Surg. 2 (5): 642–5. PMID 7820530.
  22. Kang LQ, Zhang B, Liu BG, Liu FH (January 2012). "Diagnosis of intravenous leiomyomatosis extending to heart with emphasis on magnetic resonance imaging". Chin. Med. J. 125 (1): 33–7. PMID 22340462.
  23. Fasih, Najla; Prasad Shanbhogue, Alampady K.; Macdonald, David B.; Fraser-Hill, Margaret A.; Papadatos, Demetrios; Kielar, Ania Z.; Doherty, Geoffrey P.; Walsh, Cynthia; McInnes, Matthew; Atri, Mostafa (2008). "Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations". RadioGraphics. 28 (7): 1931–1948. doi:10.1148/rg.287085095. ISSN 0271-5333.


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