Difference between revisions of "Uterine tumours"

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This article deals with '''uterine tumours''', excluding tumours that arise from the endometrium.
This article deals with '''[[uterus|uterine]] tumours''', with the exception of the tumours that arise from the [[endometrium]].  
Uterine tumours are like water in the sea - very very common.  Many hysterectomies are done for 'em... the most common are leiomyomata ([[AKA]] fibroids).
 
Uterine tumours are like water in the sea - very very common.  Many hysterectomies are done for them. The most common are leiomyomata ([[AKA]] fibroids).


Pre-malignant [[endometrium]] and endometrial tumours are dealt with in the articles, ''[[endometrial hyperplasia]]'' and ''[[endometrial carcinoma]]''.
Pre-malignant [[endometrium]] and endometrial tumours are dealt with in the articles, ''[[endometrial hyperplasia]]'' and ''[[endometrial carcinoma]]''.
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=Common benign=
=Common benign=
==Uterine leiomyoma==
==Uterine leiomyoma==
{{Main|Leiomyoma}}
*Often called ''fibroids''.
*Often called "fibroids".
{{Main|Uterine leiomyoma}}
===General===
*Extremely common... 40% of women by age 40.
*Benign.
**Can be a cause of abnormal uterine bleeding (commonly abbreviated ''AUB'').
*Large & multiple associated with infertility.
 
===Gross===
Feature:
* Sharply circumscribed.
* Gray-white.
* Whorled appearance.
 
Factor that raise concern for leiomyosarcoma:
* Haemorrhage.
* Cystic degeneration.
* [[Necrosis]].
 
===Microscopic===
Features:
* Spindle cells arranged in fascicles.
** Fascicular appearance: adjacent groups of cells have their long axis perpendicular to one another; looks somewhat like a braided hair that was cut.
* Whorled arrangement of cells.
 
Negatives:
* Necrosis (low power) - suggestive of leiomyosarcoma.
* Hypercellularity.
* Nuclear atypia seen at low power.
* Few mitoses.
 
Images:
*[http://commons.wikimedia.org/w/index.php?title=File:Cutaneous_leiomyosarcoma_-_a_-_intermed_mag.jpg Fascicular pattern - leiomyosarcoma (WC)].
 
====Variants====
*Lipoleiomyoma - with adipose tissue.
**Image: [http://commons.wikimedia.org/wiki/File:Lipoleiomyoma1.jpg Lipoleiomyoma - low mag. (WC)].
*Hypercellular leiomyoma - hypercellularity assoc. with more mutations.<ref>[http://www3.interscience.wiley.com/journal/119360394/abstract http://www3.interscience.wiley.com/journal/119360394/abstract]</ref>
*Atypical leiomyoma (AKA ''symplastic leiomyoma'') - leiomyoma with nuclear atypia.
**Image: [http://commons.wikimedia.org/wiki/File:Atypical_leiomyoma_intermed_mag.jpg Atypical leiomyoma (WC)].
*Benign metastasizing leiomyoma.<ref>{{Cite journal  | last1 = Patton | first1 = KT. | last2 = Cheng | first2 = L. | last3 = Papavero | first3 = V. | last4 = Blum | first4 = MG. | last5 = Yeldandi | first5 = AV. | last6 = Adley | first6 = BP. | last7 = Luan | first7 = C. | last8 = Diaz | first8 = LK. | last9 = Hui | first9 = P. | title = Benign metastasizing leiomyoma: clonality, telomere length and clinicopathologic analysis. | journal = Mod Pathol | volume = 19 | issue = 1 | pages = 130-40 | month = Jan | year = 2006 | doi = 10.1038/modpathol.3800504 | PMID = 16357844 |url=http://www.nature.com/modpathol/journal/v19/n1/full/3800504a.html}}</ref>
**This is just what it sounds like.  Some believe these are low grade [[leiomyosarcoma]]s.
 
===IHC===
Work-up of suspicious leiomyomas:<ref>STC. 25 February 2009.</ref>
*CD10 (+ve).
*Ki-67 (-ve).
*SMA (+ve).
*Desmin (+ve).


=Uncommon benign=
=Uncommon benign=
==Uterine adenofibroma==
==Uterine adenofibroma==
*[[AKA]] ''adenofibroma of the uterus''.
*[[AKA]] ''[[adenofibroma]] of the uterus''.
===General===
===General===
*Uncommmon.
*Uncommmon.
*Benign looking lesions can reoccur.<ref name=pmid2351327>{{Cite journal  | last1 = Seltzer | first1 = VL. | last2 = Levine | first2 = A. | last3 = Spiegel | first3 = G. | last4 = Rosenfeld | first4 = D. | last5 = Coffey | first5 = EL. | title = Adenofibroma of the uterus: multiple recurrences following wide local excision. | journal = Gynecol Oncol | volume = 37 | issue = 3 | pages = 427-31 | month = Jun | year = 1990 | doi =  | PMID = 2351327 }}</ref>
*Benign looking lesions can reoccur.<ref name=pmid2351327>{{Cite journal  | last1 = Seltzer | first1 = VL. | last2 = Levine | first2 = A. | last3 = Spiegel | first3 = G. | last4 = Rosenfeld | first4 = D. | last5 = Coffey | first5 = EL. | title = Adenofibroma of the uterus: multiple recurrences following wide local excision. | journal = Gynecol Oncol | volume = 37 | issue = 3 | pages = 427-31 | month = Jun | year = 1990 | doi =  | PMID = 2351327 }}</ref>
**It has been proposed that these lesions are in fact well-differentiated ''adenosarcomas''.<ref name=pmid18941402>{{Cite journal  | last1 = Gallardo | first1 = A. | last2 = Prat | first2 = J. | title = Mullerian adenosarcoma: a clinicopathologic and immunohistochemical study of 55 cases challenging the existence of adenofibroma. | journal = Am J Surg Pathol | volume = 33 | issue = 2 | pages = 278-88 | month = Feb | year = 2009 | doi = 10.1097/PAS.0b013e318181a80d | PMID = 18941402 }}</ref>


===Microscopic===
===Microscopic===
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**Pale stroma and epithelioid/spindle cells.
**Pale stroma and epithelioid/spindle cells.
**Simple cuboidal (or columnar) epithelium with eosinophilic cytoplasm.  
**Simple cuboidal (or columnar) epithelium with eosinophilic cytoplasm.  
*Low mitotic rate.
*Nuclear atypia minimal.
Note:
*Appearance similar to ''[[fibroadenoma]]''.


DDx:
DDx:
*Adenosarcoma.
*Adenosarcoma.
==Adenomatoid tumour==
:Should '''not''' be confused with ''[[Adamantinoma]]'' - a bone tumour.
===General===
*Grossly mimics leiomyoma.<ref name=pmid8543111>{{Cite journal  | last1 = Huang | first1 = CC. | last2 = Chang | first2 = DY. | last3 = Chen | first3 = CK. | last4 = Chou | first4 = YY. | last5 = Huang | first5 = SC. | title = Adenomatoid tumor of the female genital tract. | journal = Int J Gynaecol Obstet | volume = 50 | issue = 3 | pages = 275-80 | month = Sep | year = 1995 | doi =  | PMID = 8543111 }}
</ref>
*Benign tumour - derived from mesothelium.
*May be seen paratesticular.<ref name=pmid20223139>{{Cite journal  | last1 = González Resina | first1 = R. | last2 = Carranza Carranza | first2 = A. | last3 = Congregado Córdoba | first3 = J. | last4 = Conde Sánchez | first4 = JM. | last5 = Congregado Ruiz | first5 = CB. | last6 = Medina López | first6 = R. | title = [Paratesticular adenomatoid tumor: a report of nine cases]. | journal = Actas Urol Esp | volume = 34 | issue = 1 | pages = 95-100 | month = Jan | year = 2010 | doi =  | PMID = 20223139 }}
</ref>
===Microscopic===
Features:<ref>{{Ref GP|346}}</ref>
*Well-circumscribed lesion; however, ''not'' encapsulated.
*Small tubulocystic spaces lined by cytologically normal mesothelium.
**These pseudotubular spaces are crossed by "thread-like bridging strands" - '''key feature'''.<ref name=pmid19543245>{{Cite journal  | last1 = Sangoi | first1 = AR. | last2 = McKenney | first2 = JK. | last3 = Schwartz | first3 = EJ. | last4 = Rouse | first4 = RV. | last5 = Longacre | first5 = TA. | title = Adenomatoid tumors of the female and male genital tracts: a clinicopathological and immunohistochemical study of 44 cases. | journal = Mod Pathol | volume = 22 | issue = 9 | pages = 1228-35 | month = Sep | year = 2009 | doi = 10.1038/modpathol.2009.90 | PMID = 19543245 }}</ref><ref name=pmid14571427>{{Cite journal  | last1 = Hes | first1 = O. | last2 = Perez-Montiel | first2 = DM. | last3 = Alvarado Cabrero | first3 = I. | last4 = Zamecnik | first4 = M. | last5 = Podhola | first5 = M. | last6 = Sulc | first6 = M. | last7 = Hora | first7 = M. | last8 = Mukensnabl | first8 = P. | last9 = Zalud | first9 = R. | title = Thread-like bridging strands: a morphologic feature present in all adenomatoid tumors. | journal = Ann Diagn Pathol | volume = 7 | issue = 5 | pages = 273-7 | month = Oct | year = 2003 | doi =  | PMID = 14571427 }}</ref>


Images:
Images:
*[http://commons.wikimedia.org/wiki/File:Adenomatoid_tumour_-_low_mag.jpg Adenomatoid tumour - low mag. (WC)].
*[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5496288/figure/F2/ Adenofibroma (nlm.nih.gov)].<ref>{{cite journal |authors=Chawla L, Vatsa R, Roy KK, Kumar S |title=Uterine Adenofibroma: An Unsual Cause of Nonpuerperal Uterine Inversion in Postmenopausal Female |journal=J Midlife Health |volume=8 |issue=2 |pages=95–97 |date=2017 |pmid=28706412 |pmc=5496288 |doi=10.4103/jmh.JMH_27_17 |url=}}</ref>
*[http://commons.wikimedia.org/wiki/File:Adenomatoid_tumour_-_intermed_mag.jpg Adenomatoid tumour - intermed. mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Adenomatoid_tumour_-b-_very_high_mag.jpg Adenomatoid tumour - very high mag. (WC)].


DDx:
==Adenomatoid tumour==
*Lymphangioma.
:Should '''not''' be confused with the [[bone tumour]] ''[[adamantinoma]]''.
*Leiomyoma.
{{Main|Adenomatoid tumour}}
 
===IHC===
Features:<ref name=pmid16548294>{{Cite journal  | last1 = Canedo-Patzi | first1 = AM. | last2 = León-Bojorge | first2 = B. | last3 = de Ortíz-Hidalgo | first3 = C. | title = [Adenomatoid tumor of the genital tract. Clinical, pathological and immunohistochemical study in 9 cases] | journal = Gac Med Mex | volume = 142 | issue = 1 | pages = 59-66 | month =  | year =  | doi =  | PMID = 16548294 }}
</ref>
*Calretin +ve.
*AE1/AE3 +ve.
*CD31 -ve.
*CK7 +ve.<ref>LAE. 9 December 2009.</ref>


=Uncertain malignant potential=
=Uncertain malignant potential=
==Smooth muscle tumour of uncertain malignant potential==
==Smooth muscle tumour of uncertain malignant potential==
*Abbreviated ''STUMP''.
*Abbreviated ''STUMP''.
===General===
{{Main|Smooth muscle tumour of uncertain malignant potential}}
*Like ''[[ASAP]]'' and ''[[ASCUS]]'' - a [[waffle diagnosis|waffle category]]... when one isn't sure it is a ''leiomyoma'' vs. ''leiomyosarcoma''.
*Clinical behaviour: usually benign.<ref name=pmid19417585>{{cite journal |author=Ip PP, Cheung AN, Clement PB |title=Uterine smooth muscle tumors of uncertain malignant potential (STUMP): a clinicopathologic analysis of 16 cases |journal=Am. J. Surg. Pathol. |volume=33 |issue=7 |pages=992–1005 |year=2009 |month=July |pmid=19417585 |doi=10.1097/PAS.0b013e3181a02d1c |url=}}</ref>
*Can be subclassified into four groups - as per Stanford.
 
Management:
*Long-term follow-up.<ref name=pmid19417585/>
 
===Microscopic===
Features associated with recurrence:<ref name=pmid19417585/>
*Nuclear atypia.
 
===IHC===
Features associated with recurrence:<ref name=pmid19417585/>
*p16 +ve.
*p53 +ve.


=Malignant=
=Malignant=
==Uterine carcinosarcoma==
==Uterine carcinosarcoma==
* [[AKA]] ''malignant mixed muellerian tumour'', abbreviated ''MMMT''.
* [[AKA]] ''malignant mixed muellerian tumour'', abbreviated ''MMMT''.
{{Main|Uterine carcinosarcoma}}


===General===
==Adenosarcoma of the uterus==
* Associated with previous radiation exposure.
*[[AKA]] ''uterine adenosarcoma''.  
* Metstasize as adenocarcinoma.
{{Main|Adenosarcoma of the uterus}}
* Aggressive/poor prognosis;<ref name=pmid15688674>{{Cite journal  | last1 = Ivy | first1 = JJ. | last2 = Unger | first2 = JB. | title = Malignant mixed mullerian sarcomas of the uterus--the LSUHSC Shreveport experience. | journal = J La State Med Soc | volume = 156 | issue = 6 | pages = 324-6 | month =  | year =  | doi =  | PMID = 15688674 }}</ref> in one series 5 year survival ~= 30-35%.<ref name=pmid14967435>{{Cite journal  | last1 = Callister | first1 = M. | last2 = Ramondetta | first2 = LM. | last3 = Jhingran | first3 = A. | last4 = Burke | first4 = TW. | last5 = Eifel | first5 = PJ. | title = Malignant mixed Müllerian tumors of the uterus: analysis of patterns of failure, prognostic factors, and treatment outcome. | journal = Int J Radiat Oncol Biol Phys | volume = 58 | issue = 3 | pages = 786-96 | month = Mar | year = 2004 | doi = 10.1016/S0360-3016(03)01561-X | PMID = 14967435 }}</ref>
*Considered to be a poorly differentiated endometrial carcinoma with metaplastic changes.<ref name=pmid19853898>{{Cite journal  | last1 = D'Angelo | first1 = E. | last2 = Prat | first2 = J. | title = Uterine sarcomas: a review. | journal = Gynecol Oncol | volume = 116 | issue = 1 | pages = 131-9 | month = Jan | year = 2010 | doi = 10.1016/j.ygyno.2009.09.023 | PMID = 19853898 }}</ref>
*Case reports of MMMT in [[ovary]] and [[fallopian tube]].
 
===Microscopic===
Features:<ref name=Ref_WMSP428>{{Ref WMSP|428}}</ref>
*Biphasic tumour:
*#Malignant glandular component (adenocarcinoma).
*#Malignant stromal component (one of the following):
*#*Homologous type (tissue native to uterus):
*#*#Smooth muscle ([[leiomyosarcoma]]).
*#*#Fibrous tissue ([[fibrosarcoma]]).
*#*Heterologous type (tissue not native to the uterus):
*#*#Skeletal muscle ([[rhabdomyosarcoma]]).
*#*#Cartilage ([[chondrosarcoma]]).
*#*#Bone ([[osteosarcoma]]).
*#*Undifferentiated sarcoma ([[pleomorphic undifferentiated sarcoma]]).
 
Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Carcinosarcoma_-_low_mag.jpg MMMT - low mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Carcinosarcoma_-_high_mag.jpg MMMT - high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:MalignantMixedMullerianTumor.JPG MMMT - crappy (WC)].
*www:
**[http://path.upmc.edu/cases/case227.html MMMT - case 1 - several images (upmc.edu)].
**[http://path.upmc.edu/cases/case328.html MMMT - case 2 - several images (upmc.edu)].
 
==Adenosarcoma==
===General===
Features:<ref name=pmid20179434>{{Cite journal  | last1 = McCluggage | first1 = WG. | title = Mullerian adenosarcoma of the female genital tract. | journal = Adv Anat Pathol | volume = 17 | issue = 2 | pages = 122-9 | month = Mar | year = 2010 | doi = 10.1097/PAP.0b013e3181cfe732 | PMID = 20179434 }}</ref>
*Uncommon.
*May prolapse through cervical os and thus present as [[cervical polyp]].
*Most commonly uterine corpus, occasionally cervix and ovary, rarely in the vagina, fallopian tube, peritoneal surfaces, intestine.
*Typically 30-40 years old.
 
Treatment:
*TAH-BSO.
**Tumours are estrogen responsive.
===Microscopic===
Features:<ref name=Ref_PBoD1089>{{Ref PBoD|1089}}</ref><ref name=pmid20179434/>
*"Malignant stroma" - '''key feature'''.
**Stromal nuclear pleomorphism - usu. low grade.
**WHO criteria: 2+ mitoses / 10 HPF -- definition suffers from [[HPFitis]].
*Benign glands with an abnormal shape.
*"Cambium layer" = increased cellularity around the epithelial elements.<ref name=pmid20179434/><ref name=medilexicon_cambium/>
 
DDx:
*Benign polyp.
 
Notes:
*''Cambium layer'' - seen in: adenosarcoma, botryoid [[RMS]].<ref name=medilexicon_cambium>URL: [http://www.medilexicon.com/medicaldictionary.php?t=48297 http://www.medilexicon.com/medicaldictionary.php?t=48297]. Accessed on: 9 August 2011.</ref>


==Uterine leiomyosarcoma==
==Uterine leiomyosarcoma==
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===Microscopic===
===Microscopic===
Features:
Features:
*Smooth muscle differentiation - '''key feature'''.
*Smooth muscle differentiation - '''essential'''.
**Fascicular architecture.
**Fascicular architecture.
***Whorled look at low power.
***Whorled look at low power.
***Groups of spindle cells cut peripendicular to their long axis adjacent to groups of spindle cells cut in the plane of their long axis.
***Groups of spindle cells cut peripendicular to their long axis adjacent to groups of spindle cells cut in the plane of their long axis.
**May rely on [[IHC]] - if poorly differentiated.
**May rely on [[IHC]] - if poorly differentiated.
*Malignant histomorphologic features - all three required:
*Malignant histomorphologic features - two of three required - '''key features''':<ref name=pmid21865091>{{Cite journal  | last1 = Ip | first1 = PP. | last2 = Cheung | first2 = AN. | title = Pathology of uterine leiomyosarcomas and smooth muscle tumours of uncertain malignant potential. | journal = Best Pract Res Clin Obstet Gynaecol | volume = 25 | issue = 6 | pages = 691-704 | month = Dec | year = 2011 | doi = 10.1016/j.bpobgyn.2011.07.003 | PMID = 21865091 }}</ref>
*#[[Nuclear pleomorphism]].
*#[[Nuclear pleomorphism]].
*#[[Necrosis]].
*#Coagulative tumour cell [[necrosis]]  
*#*Should be patchy/multifocal.
*#*Should be patchy/multifocal.
*#*Zonal necrosis is suggestive of vascular cause and may be a degenerative change.
*#*Zonal necrosis is suggestive of vascular cause and may be a degenerative change.
*#**Zonal necrosis may be seen in (benign) leiomyomas.
*#**Zonal necrosis may be seen in (benign) leiomyomas.
*#Mitoses.
*#Mitoses.
*#*10 mitoses/HPF.
*#*10 mitoses/10 HPF.
*#*5 mitoses/HPF - if epithelioid.
*#*5 mitoses/10 HPF - if epithelioid.
*#*2 mitoses/HPF - if [[myxoid]].
*#*2 mitoses/10 HPF - if [[myxoid]].
 
Note:
*The mitotic rate seems to be a relatively weak predictor; a modest rate may be malignant and a high rate benign.<ref name=pmid9388868>{{Cite journal  | last1 = Guo | first1 = L. | last2 = Liu | first2 = T. | last3 = Huang | first3 = H. | title = [Reappraisal of the pathological criteria for uterine leiomyosarcoma]. | journal = Zhonghua Bing Li Xue Za Zhi | volume = 25 | issue = 5 | pages = 266-9 | month = Oct | year = 1996 | doi =  | PMID = 9388868 }}</ref>
 
DDx:
*[[Endometrial stromal sarcoma]].
*[[Uterine carcinosarcoma]].
*[[Undifferentiated endometrial sarcoma]].
*[[Smooth muscle tumour of uncertain malignant potential]].
*[[Uterine leiomyoma]].


===IHC===
===IHC===
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**Caldesmon.
**Caldesmon.
**Smooth muscle myosin.
**Smooth muscle myosin.
*p16 +ve.<ref name=pmid18156978>{{Cite journal  | last1 = Gannon | first1 = BR. | last2 = Manduch | first2 = M. | last3 = Childs | first3 = TJ. | title = Differential Immunoreactivity of p16 in leiomyosarcomas and leiomyoma variants. | journal = Int J Gynecol Pathol | volume = 27 | issue = 1 | pages = 68-73 | month = Jan | year = 2008 | doi = 10.1097/pgp.0b013e3180ca954f | PMID = 18156978 }}</ref>
**Useful for differentiation from leiomyoma.


=Endometrial stromal tumours=
=Endometrial stromal tumours=
Line 238: Line 120:


===General===
===General===
*Benign. (???)
*Benign.  


===Microscopic===
===Microscopic===
Features:
Features:
*Well-circumscribed - '''key feature'''.
*Well-circumscribed - '''key feature'''.
**The interface of lesion may not have more than three finger-like irregularities/projections into the surround myometrium that are >= 3 mm.<ref name=pmid17347285>{{Cite journal  | last1 = Baker | first1 = P. | last2 = Oliva | first2 = E. | title = Endometrial stromal tumours of the uterus: a practical approach using conventional morphology and ancillary techniques. | journal = J Clin Pathol | volume = 60 | issue = 3 | pages = 235-43 | month = Mar | year = 2007 | doi = 10.1136/jcp.2005.031203 | PMID = 17347285 | url = http://jcp.bmj.com/content/60/3/235.full }}</ref>
**The interface of the lesion may not have more than three finger-like irregularities/projections into the surround myometrium that are >= 3 mm.<ref name=pmid17347285>{{Cite journal  | last1 = Baker | first1 = P. | last2 = Oliva | first2 = E. | title = Endometrial stromal tumours of the uterus: a practical approach using conventional morphology and ancillary techniques. | journal = J Clin Pathol | volume = 60 | issue = 3 | pages = 235-43 | month = Mar | year = 2007 | doi = 10.1136/jcp.2005.031203 | PMID = 17347285 | url = http://jcp.bmj.com/content/60/3/235.full }}</ref>
*No vascular invasion.
***If it does... it is an [[ESS]].
*No [[vascular invasion]].


Notes:
DDx:
*Myometrial invasion or vascular invasion = ESS or UES.
*[[Endometrial stromal sarcoma]] (ESS), [[UES]] - myometrial invasion or [[vascular invasion]].


Images:
Images:
*[http://en.wikipedia.org/wiki/File:EndometrialStromalNodule.JPG ESN (WC)].
*[http://commons.wikimedia.org/wiki/File:EndometrialStromalNodule.JPG ESN (WC)].


==Endometrial stromal sarcoma==
==Endometrial stromal sarcoma==
*Abbreviated ESS.
*Abbreviated ESS.
*[[AKA]] ''low-grade endometrial stromal sarcoma''.
*Previously known as ''low-grade endometrial stromal sarcoma''.
{{Main|Endometrial stromal sarcoma}}
 
==Undifferentiated endometrial sarcoma==
*Abbreviated as ''UES''.
*Previously known as ''high-grade endometrial stromal sarcoma''.<ref name=pmid24146786>{{Cite journal  | last1 = Feng | first1 = W. | last2 = Malpica | first2 = A. | last3 = Skaland | first3 = I. | last4 = Gudlaugsson | first4 = E. | last5 = Robboy | first5 = SJ. | last6 = Dalen | first6 = I. | last7 = Hua | first7 = K. | last8 = Zhou | first8 = X. | last9 = Baak | first9 = JP. | title = Can proliferation biomarkers reliably predict recurrence in world health organization 2003 defined endometrial stromal sarcoma, low grade? | journal = PLoS One | volume = 8 | issue = 10 | pages = e75899 | month =  | year = 2013 | doi = 10.1371/journal.pone.0075899 | PMID = 24146786 }}</ref>
===General===
===General===
*Rare.<ref name=pmid20179433> {{Cite journal  | last1 = Chew | first1 = I. | last2 = Oliva | first2 = E. | title = Endometrial stromal sarcomas: a review of potential prognostic factors. | journal = Adv Anat Pathol | volume = 17 | issue = 2 | pages = 113-21 | month = Mar | year = 2010 | doi = 10.1097/PAP.0b013e3181cfb7c2 | PMID = 20179433 }}</ref>
*Malignant.
*Rare.
*This can be thought of as ''[[pleomorphic undifferentiated sarcoma]]'' in the [[uterus]].


===Microscopic===
===Microscopic===
Features:
*Highly cellular Islands with a wavy irregular border.
**Border has finger-like projections/[[tongue]]-like projections.
**Benign uterine smooth muscle between islands of tumour cells.
*Epithelioid cells.
*High [[NC ratio]].
*Thin blood vessels within islands of cells.
**Tumour cells pallisade around the vessels.
Notes:
*Vaguely resembles the stroma of proliferative endometrium.
DDx:
*[[Leiomyoma]].
*Endometrial stromal nodule.
Images:
*[http://commons.wikimedia.org/wiki/File:EndometrialStromalSarcoma.JPG ESS (WC)].
*[http://commons.wikimedia.org/wiki/File:EndometrialStromalSarcomaLowGrade.JPG Endometrial stromal sarcoma - low grade (WC)].
*[http://jcp.bmj.com/content/60/3/235/F5.large.jpg ESS in an article with many crappy images].<ref name=pmid17347285>{{Cite journal  | last1 = Baker | first1 = P. | last2 = Oliva | first2 = E. | title = Endometrial stromal tumours of the uterus: a practical approach using conventional morphology and ancillary techniques. | journal = J Clin Pathol | volume = 60 | issue = 3 | pages = 235-43 | month = Mar | year = 2007 | doi = 10.1136/jcp.2005.031203 | PMID = 17347285 | url = http://jcp.bmj.com/content/60/3/235.full }}</ref>
===IHC===
Features:<ref name=pmid14751141>{{Cite journal  | last1 = Zhu | first1 = XQ. | last2 = Shi | first2 = YF. | last3 = Cheng | first3 = XD. | last4 = Zhao | first4 = CL. | last5 = Wu | first5 = YZ. | title = Immunohistochemical markers in differential diagnosis of endometrial stromal sarcoma and cellular leiomyoma. | journal = Gynecol Oncol | volume = 92 | issue = 1 | pages = 71-9 | month = Jan | year = 2004 | doi =  | PMID = 14751141 }}</ref>
*CD10 +ve.
*h-caldesmin -ve.
**[[Leiomyoma]]s +ve.
*PR +/-ve.
*ER +/-ve.
===Molecular===
May be associated a recurrent [[translocation]]:<ref name=pmid12648605>{{Cite journal  | last1 = Amant | first1 = F. | last2 = Moerman | first2 = P. | last3 = Cadron | first3 = I. | last4 = Hagemeijer | first4 = A. | last5 = Vergote | first5 = I. | last6 = Debiec-Rychter | first6 = M. | title = Endometrial stromal sarcoma with a sole t(X;17) chromosome change: report of a case and review of the literature. | journal = Gynecol Oncol | volume = 88 | issue = 3 | pages = 459-62 | month = Mar | year = 2003 | doi =  | PMID = 12648605 | URL =  http://www.sciencedirect.com/science/article/pii/S0090825802000963 }}</ref>
*t(7;17)(p15;q21).
**JAZF1 - chromosome 7.<ref name=omim606246>{{OMIM|606246}}</ref>
**SUZ12 - chromosome 17.<ref name=omim606245>{{OMIM|606245}}</ref>
==Undifferentiated endometrial sarcoma==
Features:
Features:
#Marked nuclear atypia.
#Marked nuclear atypia.
Line 307: Line 161:
DDx:
DDx:
*[[Leiomyosarcoma]].
*[[Leiomyosarcoma]].
*Carcinosarcoma.
*[[Uterine carcinosarcoma|Carcinosarcoma]].
*[[Rhabdomyosarcoma]].
*[[Rhabdomyosarcoma]].
*[[Melanoma]].
*[[Melanoma]].
===IHC===
Features:<ref name=pmid21464730>{{Cite journal  | last1 = Abeler | first1 = VM. | last2 = Nenodovic | first2 = M. | title = Diagnostic immunohistochemistry in uterine sarcomas: a study of 397 cases. | journal = Int J Gynecol Pathol | volume = 30 | issue = 3 | pages = 236-43 | month = May | year = 2011 | doi = 10.1097/PGP.0b013e318200caff | PMID = 21464730 }}</ref>
*SMA ~50% +ve.
Typically negative:<ref name=pmid21464730/>
*Smooth muscle markers: desmin, h-caldesmon.
*Skeletal muscle markers: Myf4, actin.
*Melanoma: S100, HMB-45.
*GIST: CD117.


=Weird stuff=
=Weird stuff=
Line 317: Line 181:
*[[Placental site trophoblastic tumour]].
*[[Placental site trophoblastic tumour]].


==Uterine tumors resembling ovarian sex cord tumours==
==Uterine tumour resembling an ovarian sex cord stromal tumour==
*Abbreviated ''UTROSCT''.
*Abbreviated ''UTROSCT''.
 
{{Main|Uterine tumour resembling an ovarian sex cord stromal tumour}}
===General===
*Super rare.
 
===Microscopic===
Features:
*Look like sex cord tumour:<ref>URL: [http://www.nature.com/modpathol/journal/v19/n1/full/3800475a.html http://www.nature.com/modpathol/journal/v19/n1/full/3800475a.html]. Accessed on: 5 August 2010.</ref>
**May have: anastomosing cords, [[trabeculae]], small nests and/or tubules.


==Atypical polypoid adenomyoma of the uterus==
==Atypical polypoid adenomyoma of the uterus==
*Abbreviated ''APA''.
*Abbreviated ''APA''.
*[[AKA]] ''atypical polypoid adenomyoma''.
*[[AKA]] ''atypical polypoid adenomyoma''.
===General===
{{Main|Atypical polypoid adenomyoma of the uterus}}
*Very rare.<ref name=pmid21684185>{{Cite journal  | last1 = Terada | first1 = T. | title = Atypical polypoid adenomyoma of the uterus: an immunohistochemical study on 5 cases. | journal = Ann Diagn Pathol | volume = 15 | issue = 5 | pages = 338-41 | month = Oct | year = 2011 | doi = 10.1016/j.anndiagpath.2011.03.008 | PMID = 21684185 }}</ref>
*Reproductive age women.
 
===Gross===
*Lower uterine segment.
 
===Microscopic===
Features:<ref name=pmid17050990>{{Cite journal  | last1 = Jakus | first1 = S. | last2 = Edmonds | first2 = P. | last3 = Dunton | first3 = C. | last4 = Holland | first4 = G. | title = Atypical polypoid adenomyoma mimicking cervical adenocarcinoma. | journal = J Low Genit Tract Dis | volume = 6 | issue = 1 | pages = 33-8 | month = Jan | year = 2002 | doi =  | PMID = 17050990 }}</ref>
*Glands with irregular (non-ovoid) shapes.
*Benign smooth muscle around the glands.
*Morular squamous metaplasia - balls of squamous cells - very common.
*Nuclear atypia (mild).
 
DDx:
*[[Endometrioid endometrial carcinoma]].
*Endocervical adenocarcinoma.
 
Images:
*[http://www.pathologypics.com/PictView.aspx?ID=1220 APA (pathologypics.com)].
 
===IHC===
Features (glandular component):<ref name=pmid21684185/>
*AE1/AE3 +ve.
*CK7 +ve.
*ER +ve.
*PR +ve.
 
Significant negative (glandular component):<ref name=pmid21684185/>
*CK20 -ve.
*CEA -ve.


=See also=
=See also=

Latest revision as of 15:26, 10 January 2022

This article deals with uterine tumours, with the exception of the tumours that arise from the endometrium.

Uterine tumours are like water in the sea - very very common. Many hysterectomies are done for them. The most common are leiomyomata (AKA fibroids).

Pre-malignant endometrium and endometrial tumours are dealt with in the articles, endometrial hyperplasia and endometrial carcinoma.

Common benign

Uterine leiomyoma

  • Often called fibroids.
Main article: Uterine leiomyoma

Uncommon benign

Uterine adenofibroma

General

  • Uncommmon.
  • Benign looking lesions can reoccur.[1]
    • It has been proposed that these lesions are in fact well-differentiated adenosarcomas.[2]

Microscopic

Features:

  • Moderately demarcated lesion with:
    • Pale stroma and epithelioid/spindle cells.
    • Simple cuboidal (or columnar) epithelium with eosinophilic cytoplasm.
  • Low mitotic rate.
  • Nuclear atypia minimal.

Note:

DDx:

  • Adenosarcoma.

Images:

Adenomatoid tumour

Should not be confused with the bone tumour adamantinoma.
Main article: Adenomatoid tumour

Uncertain malignant potential

Smooth muscle tumour of uncertain malignant potential

  • Abbreviated STUMP.
Main article: Smooth muscle tumour of uncertain malignant potential

Malignant

Uterine carcinosarcoma

  • AKA malignant mixed muellerian tumour, abbreviated MMMT.
Main article: Uterine carcinosarcoma

Adenosarcoma of the uterus

  • AKA uterine adenosarcoma.
Main article: Adenosarcoma of the uterus

Uterine leiomyosarcoma

Main article: Leiomyosarcoma

General

  • Poor prognosis.
  • Do not (generally) arise from leiomyomas.
  • Often singular, i.e. one tumour; unlike leiomyomas (which are often multiple).

Gross

Features:

  • "Fleshy" appearance.
  • Necrosis.
  • Large size.
  • Often singular, i.e. one lesion; leiomyomata are often multiple.

Microscopic

Features:

  • Smooth muscle differentiation - essential.
    • Fascicular architecture.
      • Whorled look at low power.
      • Groups of spindle cells cut peripendicular to their long axis adjacent to groups of spindle cells cut in the plane of their long axis.
    • May rely on IHC - if poorly differentiated.
  • Malignant histomorphologic features - two of three required - key features:[4]
    1. Nuclear pleomorphism.
    2. Coagulative tumour cell necrosis
      • Should be patchy/multifocal.
      • Zonal necrosis is suggestive of vascular cause and may be a degenerative change.
        • Zonal necrosis may be seen in (benign) leiomyomas.
    3. Mitoses.
      • 10 mitoses/10 HPF.
      • 5 mitoses/10 HPF - if epithelioid.
      • 2 mitoses/10 HPF - if myxoid.

Note:

  • The mitotic rate seems to be a relatively weak predictor; a modest rate may be malignant and a high rate benign.[5]

DDx:

IHC

  • CD10 -ve.
  • Positive for SMC markers.
    • Desmin - present in all three types of muscle.
    • Caldesmon.
    • Smooth muscle myosin.
  • p16 +ve.[6]
    • Useful for differentiation from leiomyoma.

Endometrial stromal tumours

This grouping includes the gamut from benign to malignant.

Overview

WHO classification:[7]

  • Endometrial stromal nodule - not a tumour.
  • Endometrial stromal sarcoma (ESS), low grade.
  • Undifferentiated endometrial sarcoma (UES).

Notes:

  • Some believe in a "high grade ESS"... some don't.[8]

Endometrial stromal nodule

  • Abbreviated ESN.

General

  • Benign.

Microscopic

Features:

  • Well-circumscribed - key feature.
    • The interface of the lesion may not have more than three finger-like irregularities/projections into the surround myometrium that are >= 3 mm.[9]
      • If it does... it is an ESS.
  • No vascular invasion.

DDx:

Images:

Endometrial stromal sarcoma

  • Abbreviated ESS.
  • Previously known as low-grade endometrial stromal sarcoma.
Main article: Endometrial stromal sarcoma

Undifferentiated endometrial sarcoma

  • Abbreviated as UES.
  • Previously known as high-grade endometrial stromal sarcoma.[10]

General

Microscopic

Features:

  1. Marked nuclear atypia.
  2. Mitoses+++.
  3. Poorly differentiated - key feature
    • Looks nothing like low grade endometrial stromal sarcoma.
    • Negative for smooth muscle markers (to exclude leiomyosarcoma).

Notes:

  • Need IHC to diagnose.

DDx:

IHC

Features:[11]

  • SMA ~50% +ve.

Typically negative:[11]

  • Smooth muscle markers: desmin, h-caldesmon.
  • Skeletal muscle markers: Myf4, actin.
  • Melanoma: S100, HMB-45.
  • GIST: CD117.

Weird stuff

Trophoblastic tumours

Uterine tumour resembling an ovarian sex cord stromal tumour

  • Abbreviated UTROSCT.
Main article: Uterine tumour resembling an ovarian sex cord stromal tumour

Atypical polypoid adenomyoma of the uterus

  • Abbreviated APA.
  • AKA atypical polypoid adenomyoma.
Main article: Atypical polypoid adenomyoma of the uterus

See also

References

  1. ↑ Seltzer, VL.; Levine, A.; Spiegel, G.; Rosenfeld, D.; Coffey, EL. (Jun 1990). "Adenofibroma of the uterus: multiple recurrences following wide local excision.". Gynecol Oncol 37 (3): 427-31. PMID 2351327.
  2. ↑ Gallardo, A.; Prat, J. (Feb 2009). "Mullerian adenosarcoma: a clinicopathologic and immunohistochemical study of 55 cases challenging the existence of adenofibroma.". Am J Surg Pathol 33 (2): 278-88. doi:10.1097/PAS.0b013e318181a80d. PMID 18941402.
  3. ↑ Chawla L, Vatsa R, Roy KK, Kumar S (2017). "Uterine Adenofibroma: An Unsual Cause of Nonpuerperal Uterine Inversion in Postmenopausal Female". J Midlife Health 8 (2): 95–97. doi:10.4103/jmh.JMH_27_17. PMC 5496288. PMID 28706412. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5496288/.
  4. ↑ Ip, PP.; Cheung, AN. (Dec 2011). "Pathology of uterine leiomyosarcomas and smooth muscle tumours of uncertain malignant potential.". Best Pract Res Clin Obstet Gynaecol 25 (6): 691-704. doi:10.1016/j.bpobgyn.2011.07.003. PMID 21865091.
  5. ↑ Guo, L.; Liu, T.; Huang, H. (Oct 1996). "[Reappraisal of the pathological criteria for uterine leiomyosarcoma].". Zhonghua Bing Li Xue Za Zhi 25 (5): 266-9. PMID 9388868.
  6. ↑ Gannon, BR.; Manduch, M.; Childs, TJ. (Jan 2008). "Differential Immunoreactivity of p16 in leiomyosarcomas and leiomyoma variants.". Int J Gynecol Pathol 27 (1): 68-73. doi:10.1097/pgp.0b013e3180ca954f. PMID 18156978.
  7. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 426. ISBN 978-0781765275.
  8. ↑ Amant F, Vergote I, Moerman P (November 2004). "The classification of a uterine sarcoma as 'high-grade endometrial stromal sarcoma' should be abandoned". Gynecol. Oncol. 95 (2): 412–3; author reply 413. doi:10.1016/j.ygyno.2004.07.021. PMID 15491769. http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6WG6-4DF46J8-3&_user=1166899&_coverDate=11%2F01%2F2004&_rdoc=1&_fmt=high&_orig=search&_sort=d&_docanchor=&view=c&_searchStrId=1204975755&_rerunOrigin=google&_acct=C000051839&_version=1&_urlVersion=0&_userid=1166899&md5=d6ec1eee2941460a085d1dac6615b5a5.
  9. ↑ Baker, P.; Oliva, E. (Mar 2007). "Endometrial stromal tumours of the uterus: a practical approach using conventional morphology and ancillary techniques.". J Clin Pathol 60 (3): 235-43. doi:10.1136/jcp.2005.031203. PMID 17347285. http://jcp.bmj.com/content/60/3/235.full.
  10. ↑ Feng, W.; Malpica, A.; Skaland, I.; Gudlaugsson, E.; Robboy, SJ.; Dalen, I.; Hua, K.; Zhou, X. et al. (2013). "Can proliferation biomarkers reliably predict recurrence in world health organization 2003 defined endometrial stromal sarcoma, low grade?". PLoS One 8 (10): e75899. doi:10.1371/journal.pone.0075899. PMID 24146786.
  11. ↑ 11.0 11.1 Abeler, VM.; Nenodovic, M. (May 2011). "Diagnostic immunohistochemistry in uterine sarcomas: a study of 397 cases.". Int J Gynecol Pathol 30 (3): 236-43. doi:10.1097/PGP.0b013e318200caff. PMID 21464730.
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