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

Uterine 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 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

Uterine adenofibroma

  • AKA adenofibroma of the uterus.

General

  • Uncommmon.
  • Benign looking lesions can reoccur.[4]

Microscopic

Features:

  • Moderately demarcated lesion with:
    • Pale stroma and epithelioid/spindle cells.
    • Simple cuboidal (or columnar) epithelium with eosinophilic cytoplasm.

Note:

DDx:

  • Adenosarcoma.

Adenomatoid tumour

Should not be confused with Adamantinoma - a bone tumour.

General

  • Grossly mimics leiomyoma.[5]
  • Benign tumour - derived from mesothelium.
  • May be seen paratesticular.[6]

Microscopic

Features:[7]

  • 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.[8][9]

Images:

DDx:

IHC

Features:[10]

  • Calretin +ve.
  • AE1/AE3 +ve.
  • CD31 -ve.
  • CK7 +ve.[11]

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.[12]
  • Can be subclassified into four groups - as per Stanford.

Management:

  • Long-term follow-up.[12]

Microscopic

Features associated with recurrence:[12]

  • Nuclear atypia.

IHC

Features associated with recurrence:[12]

  • 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;[13] in one series 5 year survival ~= 30-35%.[14]
  • Considered to be a poorly differentiated endometrial carcinoma with metaplastic changes.[15]
  • Case reports of MMMT in ovary and fallopian tube.

Microscopic

Features:[16]

Images:

Adenosarcoma

General

Features:[17]

  • 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:[18][17]

  • "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.[17][19]

DDx:

Notes:

  • Cambium layer - seen in: adenosarcoma, botryoid RMS.[19]

Uterine 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.

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:[20]

  • 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.[21]

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.[22]
  • 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.

Notes:

  • Vaguely resembles the stroma of proliferative endometrium.

DDx:

Images:

IHC

Features:[24]

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

Molecular

May be associated a recurrent translocation:[25]

  • t(7;17)(p15;q21).
    • JAZF1 - chromosome 7.[26]
    • SUZ12 - chromosome 17.[27]

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

Trophoblastic tumours

Uterine tumors resembling ovarian sex cord tumours

  • Abbreviated UTROSCT.

General

  • Super rare.

Microscopic

Features:

  • Look like sex cord tumour:[28]
    • 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.[29]
  • Reproductive age women.

Gross

  • Lower uterine segment.

Microscopic

Features:[30]

  • 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:

Images:

IHC

Features (glandular component):[29]

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

Significant negative (glandular component):[29]

  • CK20 -ve.
  • CEA -ve.

See also

References

  1. http://www3.interscience.wiley.com/journal/119360394/abstract
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  3. STC. 25 February 2009.
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