Uterine tumours

From Libre Pathology
Jump to navigation Jump to search

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

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

Should not be confused with Adamantinoma - a bone tumour.

General

  • Grossly mimics leiomyoma.[4]
  • Benign tumour - derived from mesothelium.
  • May be seen paratesticular.[5]

Microscopic

Features:[6]

  • 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.[7][8]

Images:

DDx:

  • Lymphangioma.
  • Leiomyoma.

IHC

Features:[9]

  • Calretin +ve.
  • AE1/AE3 +ve.
  • CD31 -ve.
  • CK7 +ve.[10]

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.[11]
  • Can be subclassified into four groups - as per Stanford.

Management:

  • Long-term follow-up.[11]

Microscopic

Features associated with recurrence:[11]

  • Nuclear atypia.

IHC

Features associated with recurrence:[11]

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

Microscopic

Features:[15]

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

Images:

Adenosarcoma

General

Features:[16]

  • 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:[17][16]

  • "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.[16][18]

DDx:

  • Benign polyp.

Notes:

  • Cambium layer - seen in: adenosarcoma, botryoid RMS.[18]

Uterine leiomyocarcoma

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:[19]

  • 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.[20]

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.[21]
  • 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:[23]

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

Molecular

May be associated a recurrent translocation:[24]

  • t(7;17)(p15;q21).
    • JAZF1 - chromosome 7.[25]
    • SUZ12 - chromosome 17.[26]

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:[27]
    • 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. 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.
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. LAE. 9 December 2009.
  11. 11.0 11.1 11.2 11.3 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.
  12. 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.
  13. 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.
  14. 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.
  15. 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.
  16. 16.0 16.1 16.2 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.
  17. 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.
  18. 18.0 18.1 URL: http://www.medilexicon.com/medicaldictionary.php?t=48297. Accessed on: 9 August 2011.
  19. 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.
  20. 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.
  21. 21.0 21.1 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.
  22. 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.
  23. 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.
  24. 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.
  25. Online 'Mendelian Inheritance in Man' (OMIM) 606246
  26. Online 'Mendelian Inheritance in Man' (OMIM) 606245
  27. URL: http://www.nature.com/modpathol/journal/v19/n1/full/3800475a.html. Accessed on: 5 August 2010.