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Gynecological Sarcoma

Gynecological Sarcomas

​​Revised: Mar 2000

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

These uncommon tumours usually arise in the uterus but can occasionally originate in the ovary or broad ligament. Histologically they are subdivided as follows: 

  1. Malignant mullerian mixed tumors. These tumours are composed of adenocarcinoma and sarcoma which may either be homologous (representing tissue normally seen in the uterus) or heterologous (resembling tissue not normally found in the uterus)    
  2. Leiomyosarcoma    
  3. Endometrial stromal sarcoma    
  4. Rhabdomyosarcoma    
  5. Others

WHO/ISGP Classification of Mixed Epithelial-Mesenchymal Tumours

LMS

Endometrial Stromal Tumours 

  1. Endometrial stromal nodule    
  2. Low-grade endometrial stromal sarcoma    
  3. High-grade endometrial stromal sarcoma

Smooth Muscle Tumors

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

2. Management

​Operable Cases

A total abdominal hysterectomy and bilateral salpingo-oophorectomy is recommended for all histological subtypes.

Patients with malignant mullerian mixed tumours and endometrial stromal sarcomas received pelvic radiotherapy identical to high risk Stage I endometrial adenocarcinoma.

The chemotherapy of rhabdomyosarcoma is individualized and can be curative.

The completed BCCA trial of adjuvant chemotherapy for leiomyosarcomas and malignant mullerian mixed tumours has failed to demonstrate any advantage. Adjuvant chemotherapy has shown no survival effect and is therefore not used.

Patients with fully resected leiomyosarcomas usually receive no further adjuvant therapy but this may be offered in investigative protocols.

Low Grade Endometrial Stromal Sarcomas

The distinction between high and low grade stromal sarcomas is important because of prognostic and therapeutic considerations. Pelvic and abdominal recurrence develop in one-third to one-half of patients. The interval to recurrence can vary with a mean of three years. Distant metastases are uncommon.

In many cases recurrent low grade sarcomas can be successfully treated with surgical excision, radiation, or progestin therapy. Prognosis for this group is generally good with 90 - 100% of patients surviving 10 years from diagnosis.

Patients with low-grade stromal sarcomas should be referred post diagnosis to BCCA for staging and an opinion regarding adjuvant treatment.

Inoperable Cases (Recurrent or Metastatic Disease)

About one-third of patients with metastatic gynecological sarcomas may derive some palliative benefit from chemotherapy. Such patients with metastatic leiomyosarcoma may be candidates for experimental chemotherapy.

Malignant mullerian mixed tumours receive adriamycin 50 mg/m² and cisplatin 50 mg/m² q3wks to maximal response (maximum nine chemotherapy treatments). 

Radiotherapy may be added on an individualized basis to patients with metastatic sarcoma.

On occasion patients with a long tumor-free interval may benefit from surgery.

Chemotherapy Protocols

Estrogen Replacement Therapy - Site Specific Information

Some gynecologic tumours are considered to be hormone responsive (i.e., some low grade uterine sarcomas). Estrogen replacement in this group of patients should be for symptomatic control. A thorough discussion with the patient of the potential risks and anticipated benefits of such treatment should take place.

Recommendation: continuous estrogen plus progestogen.

Radiotherapy - General Information

3. Follow Up

Patients should be reminded that it is their responsibility to keep their recommended follow-up appointments. The objectives of the follow-up visits are as follows: 

  1. To determine the patient's immediate response to the treatment employed    
  2. Early recognition and prompt management of treatment related complications    
  3. Early detection of persistent or recurrent disease    
  4. Collection of meaningful data regarding the efficacy of existing treatment policies and their complications so that any appropriate modifications can be instituted

These objectives are best met by having the initial follow-up examination performed by the Agency medical staff. When appropriate, arrangements will be made for follow-up by the referring physician.

Year 1every 3 months
Year 2every 4 months
Year 3-5every 6 months
Years 5+annually
SOURCE: Gynecological Sarcoma ( )
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