Uterine tumours

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This article deals with uterine tumours, excluding 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).

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

Common benign

Leiomyomas

Main article: Leiomyoma

General

  • Often called "fibroids".
  • Extremely common... 40% of women by age 40.
  • Benign.
    • Can be a cause of AUB (abnormal uterine bleeding).
    • Large & multiple associated with infertility.

Gross

  • 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 assoc. 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).
  • Ki-67 (-ve).
  • SMA (+ve).
  • Desmin (+ve).

Uncommon benign

Adenomatoid tumour

General

  • Grossly mimics leiomyoma.[4]
  • Benign tumour - derived from mesothelium.

Notes:

Microscopy

Features:[5]

  • Well-circumscribed lesion; however, not encapsulated.
  • Small tubulocystic spaces lined by cytologically normal mesothelium.

Images:

DDx:

  • Lymphangioma.
  • Leiomyoma.

IHC

Features:[6]

  • Calretin +ve.
  • AE1/AE3 +ve.
  • CD31 -ve.
  • CK7 +ve.[7]

Uncertain malignant potential

Smooth muscle tumour of uncertain malignant potential (STUMP)

General

  • Like ASAP and ASCUS - a waffle category... when one isn't sure it is a leiomyoma vs. leiomyosarcoma.
  • Clinical behaviour: usually benign.[8]
  • Can be subclassified into four groups - as per Stanford.

Management:

  • Long-term follow-up.[8]

Microscopic

Features associated with recurrence:[8]

  • Nuclear atypia.

IHC

Features associated with recurrence:[8]

  • p16 +ve.
  • p53 +ve.

Malignant

Carcinosarcoma

  • AKA malignant mixed muellerian tumour, abbreviated MMMT.

General

  • Associated with previous radiation exposure.
  • Metstasize as adenocarcinoma.
  • Aggressive/poor prognosis;[9] in one series 5 year survival ~= 30-35%.[10]
  • Considered to be a poorly differentiated endometrial carcinoma with metaplastic changes.[11]
  • Case reports of MMMT in ovary and fallopian tube.

Microscopic

Features:[12]

  • Biphasic tumour:
    1. Malignant glandular component (adenocarcinoma).
    2. Malignant stromal component (one of the following):
      • Skeletal muscle.
      • Smooth muscle.
      • Cartilage.
      • Bone.
      • Undifferentiated sarcoma.

Image: MMMT (WC).

Adenosarcoma

General

Features:[13]

  • 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:[14][13]

  • "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.[13][15]

DDx:

  • Benign polyp.

Notes:

  • Cambium layer - seen in: adenosarcoma, botryoid RMS.[15]

Leiomyocarcoma

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:

  1. Cellular atypia - common.
  2. Necrosis.
    • Should be patchy/multifocal.
    • Zonal necrosis is suggestive of vascular cause.
  3. Mitoses - key feature.
    • 10 mitoses/HPF.
    • 5 mitoses/HPF - if epithelioid.
    • 2 mitoses/HPF - if myxoid.

IHC

  • CD10 -ve.
  • Positive for SMC markers.
    • Desmin - present in all three types of muscle.
    • Caldesmon.
    • Smooth muscle myosin.

Endometrial stromal tumours

This grouping includes the gamut from benign to malignant.

Overview

WHO classification:[16]

  • 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.[17]

Endometrial stromal nodule

  • Abbreviated ESN.

General

  • Benign. (???)

Microscopic

Features:

  • 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.[18]
  • No vascular invasion.

Notes:

  • Myometrial invasion or vascular invasion = ESS or UES.

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.

Image(s):

Notes:

  • Vaguely resembles the stroma of proliferative endometrium.

Undifferentiated endometrial sarcoma

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:

Weird stuff

Uterine tumors resembling ovarian sex cord tumours (UTROSCT)

  • Look like sex cord tumour:[20]
    • May have: anastomosing cords, trabeculae, small nests and/or tubules.

See also

References

  1. ↑ 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. ↑ 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.
  5. ↑ 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.
  6. ↑ 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.
  7. ↑ LAE. 9 December 2009.
  8. ↑ 8.0 8.1 8.2 8.3 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.
  9. ↑ 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.
  10. ↑ 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.
  11. ↑ 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.
  12. ↑ 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.
  13. ↑ 13.0 13.1 13.2 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.
  14. ↑ 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.
  15. ↑ 15.0 15.1 URL: http://www.medilexicon.com/medicaldictionary.php?t=48297. Accessed on: 9 August 2011.
  16. ↑ 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.
  17. ↑ 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.
  18. ↑ 18.0 18.1 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.
  19. ↑ 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.
  20. ↑ URL: http://www.nature.com/modpathol/journal/v19/n1/full/3800475a.html. Accessed on: 5 August 2010.
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