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Gynecological Sarcoma

Gynecological Sarcomas

Updated May 2018

Common Errors in the Diagnosis and Management of Gynecologic Malignancies

  1. Failure to perform a complete history and physical examination.    
  2. Over-reliance on diagnostic examinations and under-reliance on clinical suspicion and physical findings.    
  3. Failure to perform a pelvic examination as part of the initial assessment.

1. Staging

Uterine Sarcomas 

These uncommon tumours usually arise in the uterus but can occasionally originate in the ovary or broad ligament.

The most common subtypes are:
  • Low grade endometrial stromal (ESS)
  • High grade endometrial stromal (HGESS)
  • Undifferentiated uterine sarcoma (UUS)
  • Uterine leiomyosarcoma (uLMS)

WHO/ISGP Classification of Uterine Mesenchymal and Mixed Epithelial and Mesenchymal Tumour

Mesenchymal Tumours

Endometrial Stromal Tumours 

  1. Endometrial stromal nodule     
  2. Low-grade endometrial stromal sarcoma    
  3. High-grade endometrial stromal sarcoma
  4. Undifferentiated uterine sarcoma
  5. Uterine tumor resembling ovarian sex cord tumor

Miscellaneous Mesenchymal Tumours 

    f. Rhabdomyosarcoma     

Perivascular epithelioid cell tumor

Smooth Muscle Tumors

  1. Leiomyoma and benign leiomyoma-variants
  2. Smooth muscle tumor of uncertain malignant potential
  3. Leiomyosarcomatypical
    epithelioid
    myxoid
  4. Other smooth muscle tumors

Mixed epithelial-nonepithelial tumors

  1. Adenosarcoma
  2. Carcinosarcoma
* Uterine carcinosarcoma is classified as a carcinoma rather than a  sarcoma, though they have stromal differentiation, and is treated as such. Adenosarcomas are considered sarcomas.

Evaluation

Most uterine sarcomas are diagnosed based on pathologic evaluation after hysterectomy, only a minority of the cases is diagnosed at the time of endometrial sampling.

The role of pre-operative imaging to evaluate for uterine sarcomas is not well defined as most have non-specific appearances.

  • Known uterine sarcoma prior to surgery: CT C/A/P +/- MRI for staging purposes 
  • Post-operative evaluation: CT C/A/P +/- MRI for staging purposes 

2. Management

It is recommended that Uterine sarcomas be discussed at  a tumour board with pathology review.

Surgical Management

Diagnosis of uterine sarcoma is made at the time of uterine biopsy:

  • Operable cases: A total abdominal hysterectomy +/- bilateral salpingo-oophorectomy is recommended for all histological subtypes. Lymphnode metastatses are uncommon in sarcomas.  When disease is outside of the uterus surgical staging and cytoreduction can be performed provided that the surgeon believes the disease to be resectable.
  • Non surgical candidates: Can be offered systemic therapy +/- radiation therapy for local control.
Diagnosis of uterine sarcoma is made after hysterectomy

  • Patients with uterine sarcomas should be referred post diagnosis to BC Cancer for staging and an opinion regarding adjuvant treatment. 
  • Further surgical intervention is determined based on the histology, stage and type of primary surgery.

Adjuvant Management

Low grade endometrial stromal (ESS)

Pelvic and abdominal recurrence develops in one-third to one-half of patients. The interval to recurrence can vary with a mean of three years. Distant metastases are uncommon.

  • Stage I: surveillance vs. endocrine therapy. No role for radiation therapy.
  • Stage II/IV: endocrine therapy +/- radiation therapy.
    • Data to support administration of adjuvant endocrine therapy for stage II to IV ESS are limited. The decision to pursue adjuvant systemic treatment, drug choice, and duration of treatment is case dependent. 

High grade/undifferentiated or Leiomyosarcoma

  • Stage I:  observation vs adjuvant chemo
  • Stage II/III resected to zero residual disease: consideration for adjuvant chemo+/- radiation therapy
    • The role for adjuvant systemic therapy for adenosarcoma, leiomyosarcoma, HGESS, or UUS is controversial and should not be considered standard of care, given there is no overall survival benefit reflected in prospective adjuvant trials.
  • Stage III/IV suboptimally debulked, or nonoperable
    • Systemic therapy should be considered. Treatment choice and duration of treatment is case dependent. 

Recurrent/Metastatic Disease

There is a limited role for surgical resection.

Radiation therapy, outside of oligometastic, disease is reserved for symptomatic disease. Mainstay of treatment is systemic therapy.
  • Low grade stromal sarcoma
    • For ER/PR positive disease, hormonal therapy should be considered. Tamoxifen should be avoided as there is some evidence to suggest increased risk of recurrence with use. 
  • Leiomyosarcoma
    • Gemcitabine/docetaxel or doxorubin are reasonable choices for patients with good functional status.
    • Aromatase inhibitors have response rates <10%.  However, for some patients with ER/PR positive disease, low disease burden or indolent pace, or poor functional status, this strategy may be employed.
  • High grade/ undifferentiated stromal sarcoma and adenosarcomas
    • Should be treated like Leiomyosarcoma

3. Follow Up

The objectives of follow-up visits are as follows:

  • To determine the patient’s immediate response to the treatment employed
  • Early recognition and prompt management of treatment related complications
  • Early detection of persistent or recurrent disease
  • Collection of meaningful data regarding the efficacy of existing treatment policies and their complications so that any appropriate modifications can be instituted.
Follow-up consists of full physical exam q 3m x 2 years then q6 – 12 m. Imaging with CT C/A/P q 6m x 3 years, then q6 -12m x 2 years.
SOURCE: Gynecological Sarcoma ( )
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