Uterine tumours

From Libre Pathology
Revision as of 17:52, 28 March 2013 by Michael (talk | contribs) (w)
Jump to navigation Jump to search

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

Main article: Leiomyoma
  • Often called "fibroids".

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:

Variants

  • Lipoleiomyoma - with adipose tissue.
  • Hypercellular leiomyoma - hypercellularity associated with more mutations.[1]
  • Atypical leiomyoma (AKA symplastic leiomyoma) - leiomyoma with nuclear atypia.
  • Benign metastasizing leiomyoma.[2]
    • This is just what it sounds like. Some believe these are low grade leiomyosarcomas.

IHC

Work-up of suspicious leiomyomas:[3]

  • CD10 +ve.[4]
  • SMA +ve.
  • Desmin +ve.
  • Ki-67 -ve.

Others:

  • p16 usually -ve.[5]
    • Often +ve in leiomyosarcoma.
  • H-caldesmon +ve.[4]

Sign out

UTERUS, UTERINE TUBES AND LEFT OVARY, TOTAL HYSTERECTOMY, BILATERAL SALPINGECTOMY AND RIGHT OOPHRECTOMY:
- LEIOMYOMATA WITH FOCAL CALCIFICATION AND HYALINE CHANGE.
- SECRETORY PHASE ENDOMETRIUM.
- RIGHT OVARY WITHIN NORMAL LIMITS.
- UTERINE TUBES WITHIN NORMAL LIMITS.
- UTERINE CERVIX WITHIN NORMAL LIMITS.

Myomectomy

UTERINE MASSES ("FIBROIDS"), MYOMECTOMY:
- LEIOMYOMATA.

Uncommon benign

Uterine adenofibroma

General

  • Uncommmon.
  • Benign looking lesions can reoccur.[6]
    • It has been proposed that these lesions are in fact well-differentiated adenosarcomas.[7]

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 Adamantinoma - a bone tumour.

General

  • Grossly mimics leiomyoma.[8]
  • Benign tumour - derived from mesothelium.
  • May be seen paratesticular.[9]

Microscopic

Features:[10]

  • 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.[11][12]

Images:

DDx:

IHC

Features:[13]

  • Calretinin +ve.
  • AE1/AE3 +ve.
  • CD31 -ve.
  • CK7 +ve.[14]

Uncertain malignant potential

Smooth muscle tumour of uncertain malignant potential

  • Abbreviated STUMP.

General

  • Like ASAP and ASCUS - a waffle category... when one isn't sure it is a leiomyoma vs. leiomyosarcoma.
  • Clinical behaviour: usually benign.[15]
  • Can be subclassified into four groups - as per Stanford.

Management:

  • Long-term follow-up.[15]

Microscopic

Features associated with recurrence:[15]

  • Nuclear atypia.

DDx:

IHC

Features associated with recurrence:[15]

  • p16 +ve.
  • p53 +ve.

Malignant

Uterine carcinosarcoma

  • AKA malignant mixed muellerian tumour, abbreviated MMMT.

General

  • Associated with previous radiation exposure.
  • Metstasize as adenocarcinoma.
  • Aggressive/poor prognosis;[16] in one series 5 year survival ~= 30-35%.[17]
  • Considered to be a poorly differentiated endometrial carcinoma with metaplastic changes.[18]
  • Case reports of MMMT in ovary and fallopian tube.

Microscopic

Features:[19]

DDx:

Images:

Adenosarcoma of the uterus

  • AKA uterine adenocarcinoma.

General

Features:[20]

  • 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.

Clinical:[21]

  • Most common presentations of Müllerian adenosarcoma (percentages based on series of 41 individuals[22]):
    • Vaginal bleeding ~ 70%.
    • Pelvic mass ~ 40%.
    • Uterine polyp ~ 30%.
  • Prognosis (based on series of ~500 individuals[23]):
    • Favourable outcome - most detected at an early stage.
      • ~80% five year survival for stage I tumours.
    • Outcome better than carcinosarcoma.

Treatment:

  • TAH-BSO.
    • Tumours are estrogen responsive.
  • Chemotherapy (platin-based).[22]

Microscopic

Features:[24][20]

  • "Malignant stroma" - key feature.
    • Stromal nuclear pleomorphism - usually low grade.
    • WHO criteria: 2+ mitoses / 10 HPF -- definition suffers from HPFitis.
      • Mitotic rate criteria often ignored as mitotically inactive tumours metastasize.[20]
  • Benign glands with an abnormal shape.
  • "Cambium layer" = increased cellularity around the epithelial elements.[20][25]

Notes:

DDx:

Images:

IHC

  • CD10 +ve.[20]
  • ER +ve.
  • PR +ve.

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 - key feature.
    • 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 - all three required:
    1. Nuclear pleomorphism.
    2. 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/HPF.
      • 5 mitoses/HPF - if epithelioid.
      • 2 mitoses/HPF - if myxoid.

DDx:

IHC

  • CD10 -ve.
  • Positive for SMC markers.
    • Desmin - present in all three types of muscle.
    • Caldesmon.
    • Smooth muscle myosin.
  • p16 +ve.[5]
    • Useful for differentiation from leiomyoma.

Endometrial stromal tumours

This grouping includes the gamut from benign to malignant.

Overview

WHO classification:[26]

  • 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.[27]

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.[28]
      • If it does... it is an ESS.
  • No vascular invasion.

DDx:

Images:

Endometrial stromal sarcoma

  • Abbreviated ESS.
  • AKA low-grade endometrial stromal sarcoma.

General

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:

Images:

IHC

Features:[4]

  • CD10 +ve.
  • h-caldesmin -ve.
  • PR +/-ve.
  • ER +/-ve.

Molecular

May be associated a recurrent translocation:[30]

  • t(7;17)(p15;q21).
    • JAZF1 - chromosome 7.[31]
    • SUZ12 - chromosome 17.[32]

Undifferentiated endometrial sarcoma

  • Abbreviated as UES.

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:[33]

  • SMA ~50% +ve.

Typically negative:[33]

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

Weird stuff

Trophoblastic tumours

Uterine tumors resembling ovarian sex cord tumours

  • Abbreviated UTROSCT.

General

  • Super rare.

Microscopic

Features:

  • Look like sex cord tumour:[34]
    • May have: anastomosing cords, trabeculae, small nests and/or tubules.

Atypical polypoid adenomyoma of the uterus

  • Abbreviated APA.
  • AKA atypical polypoid adenomyoma.

General

  • Very rare.[35]
  • Benign.[36]
  • Reproductive age women.

Gross

  • Lower uterine segment.

Microscopic

Features:[36]

  • Glands with irregular (non-ovoid) shapes.
  • Benign smooth muscle around the glands - key feature.
  • Morular squamous metaplasia - balls of squamous cells - very common.
  • Nuclear atypia (mild).

DDx:

Images:

IHC

Features (glandular component):[35]

  • AE1/AE3 +ve.
  • CK7 +ve.
  • ER +ve.
  • PR +ve.

Significant negative (glandular component):[35]

  • CK20 -ve.
  • CEA -ve.

See also

References

  1. ↑ Pandis, N.; Heim, S.; Willén, H.; Bardi, G.; Flodérus, U-M.; Mandahl, N.; Mitelman, F. (Jan 1991). "Histologic—cytogenetic correlations in uterine leiomyomas.". International Journal of Gynecological Cancer 1 (4): 163-68. http://www3.interscience.wiley.com/journal/119360394/abstract.
  2. ↑ Patton, KT.; Cheng, L.; Papavero, V.; Blum, MG.; Yeldandi, AV.; Adley, BP.; Luan, C.; Diaz, LK. et al. (Jan 2006). "Benign metastasizing leiomyoma: clonality, telomere length and clinicopathologic analysis.". Mod Pathol 19 (1): 130-40. doi:10.1038/modpathol.3800504. PMID 16357844. http://www.nature.com/modpathol/journal/v19/n1/full/3800504a.html.
  3. ↑ STC. 25 February 2009.
  4. ↑ 4.0 4.1 4.2 Zhu, XQ.; Shi, YF.; Cheng, XD.; Zhao, CL.; Wu, YZ. (Jan 2004). "Immunohistochemical markers in differential diagnosis of endometrial stromal sarcoma and cellular leiomyoma.". Gynecol Oncol 92 (1): 71-9. PMID 14751141.
  5. ↑ 5.0 5.1 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.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ Huang, CC.; Chang, DY.; Chen, CK.; Chou, YY.; Huang, SC. (Sep 1995). "Adenomatoid tumor of the female genital tract.". Int J Gynaecol Obstet 50 (3): 275-80. PMID 8543111.
  9. ↑ González Resina, R.; Carranza Carranza, A.; Congregado Córdoba, J.; Conde Sánchez, JM.; Congregado Ruiz, CB.; Medina López, R. (Jan 2010). "[Paratesticular adenomatoid tumor: a report of nine cases].". Actas Urol Esp 34 (1): 95-100. PMID 20223139.
  10. ↑ Nucci, Marisa R.; Oliva, Esther (2009). Gynecologic Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 346. ISBN 978-0443069208.
  11. ↑ Sangoi, AR.; McKenney, JK.; Schwartz, EJ.; Rouse, RV.; Longacre, TA. (Sep 2009). "Adenomatoid tumors of the female and male genital tracts: a clinicopathological and immunohistochemical study of 44 cases.". Mod Pathol 22 (9): 1228-35. doi:10.1038/modpathol.2009.90. PMID 19543245.
  12. ↑ Hes, O.; Perez-Montiel, DM.; Alvarado Cabrero, I.; Zamecnik, M.; Podhola, M.; Sulc, M.; Hora, M.; Mukensnabl, P. et al. (Oct 2003). "Thread-like bridging strands: a morphologic feature present in all adenomatoid tumors.". Ann Diagn Pathol 7 (5): 273-7. PMID 14571427.
  13. ↑ Canedo-Patzi, AM.; León-Bojorge, B.; de Ortíz-Hidalgo, C.. "[Adenomatoid tumor of the genital tract. Clinical, pathological and immunohistochemical study in 9 cases]". Gac Med Mex 142 (1): 59-66. PMID 16548294.
  14. ↑ Latta, E. 9 December 2009.
  15. ↑ 15.0 15.1 15.2 15.3 Ip PP, Cheung AN, Clement PB (July 2009). "Uterine smooth muscle tumors of uncertain malignant potential (STUMP): a clinicopathologic analysis of 16 cases". Am. J. Surg. Pathol. 33 (7): 992–1005. doi:10.1097/PAS.0b013e3181a02d1c. PMID 19417585.
  16. ↑ Ivy, JJ.; Unger, JB.. "Malignant mixed mullerian sarcomas of the uterus--the LSUHSC Shreveport experience.". J La State Med Soc 156 (6): 324-6. PMID 15688674.
  17. ↑ Callister, M.; Ramondetta, LM.; Jhingran, A.; Burke, TW.; Eifel, PJ. (Mar 2004). "Malignant mixed Müllerian tumors of the uterus: analysis of patterns of failure, prognostic factors, and treatment outcome.". Int J Radiat Oncol Biol Phys 58 (3): 786-96. doi:10.1016/S0360-3016(03)01561-X. PMID 14967435.
  18. ↑ D'Angelo, E.; Prat, J. (Jan 2010). "Uterine sarcomas: a review.". Gynecol Oncol 116 (1): 131-9. doi:10.1016/j.ygyno.2009.09.023. PMID 19853898.
  19. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 428. ISBN 978-0781765275.
  20. ↑ 20.0 20.1 20.2 20.3 20.4 20.5 McCluggage, WG. (Mar 2010). "Mullerian adenosarcoma of the female genital tract.". Adv Anat Pathol 17 (2): 122-9. doi:10.1097/PAP.0b013e3181cfe732. PMID 20179434.
  21. ↑ Abu, J.; Ireland, D.; Brown, L. (Apr 2007). "Adenosarcoma of an endometrial polyp in a 27-year-old nulligravida: a case report.". J Reprod Med 52 (4): 326-8. PMID 17506376.
  22. ↑ 22.0 22.1 Verschraegen, CF.; Vasuratna, A.; Edwards, C.; Freedman, R.; Kudelka, AP.; Tornos, C.; Kavanagh, JJ.. "Clinicopathologic analysis of mullerian adenosarcoma: the M.D. Anderson Cancer Center experience.". Oncol Rep 5 (4): 939-44. PMID 9625851.
  23. ↑ Arend, R.; Bagaria, M.; Lewin, SN.; Sun, X.; Deutsch, I.; Burke, WM.; Herzog, TJ.; Wright, JD. (Nov 2010). "Long-term outcome and natural history of uterine adenosarcomas.". Gynecol Oncol 119 (2): 305-8. doi:10.1016/j.ygyno.2010.07.001. PMID 20688363.
  24. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1089. ISBN 0-7216-0187-1.
  25. ↑ 25.0 25.1 URL: http://www.medilexicon.com/medicaldictionary.php?t=48297. Accessed on: 9 August 2011.
  26. ↑ 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.
  27. ↑ 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.
  28. ↑ 28.0 28.1 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.
  29. ↑ Chew, I.; Oliva, E. (Mar 2010). "Endometrial stromal sarcomas: a review of potential prognostic factors.". Adv Anat Pathol 17 (2): 113-21. doi:10.1097/PAP.0b013e3181cfb7c2. PMID 20179433.
  30. ↑ Amant, F.; Moerman, P.; Cadron, I.; Hagemeijer, A.; Vergote, I.; Debiec-Rychter, M. (Mar 2003). "Endometrial stromal sarcoma with a sole t(X;17) chromosome change: report of a case and review of the literature.". Gynecol Oncol 88 (3): 459-62. PMID 12648605.
  31. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 606246
  32. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 606245
  33. ↑ 33.0 33.1 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.
  34. ↑ URL: http://www.nature.com/modpathol/journal/v19/n1/full/3800475a.html. Accessed on: 5 August 2010.
  35. ↑ 35.0 35.1 35.2 Terada, T. (Oct 2011). "Atypical polypoid adenomyoma of the uterus: an immunohistochemical study on 5 cases.". Ann Diagn Pathol 15 (5): 338-41. doi:10.1016/j.anndiagpath.2011.03.008. PMID 21684185.
  36. ↑ 36.0 36.1 Jakus, S.; Edmonds, P.; Dunton, C.; Holland, G. (Jan 2002). "Atypical polypoid adenomyoma mimicking cervical adenocarcinoma.". J Low Genit Tract Dis 6 (1): 33-8. PMID 17050990.
Retrieved from "https://librepathology.org/w/index.php?title=Uterine_tumours&oldid=20908"