ENDOMETRIAL STROMAL SARCOMA

Endometrial Stromal Sarcoma Grade III/IV; High Grade Endometrial Stromal Sarcoma; ESS; Undifferentiated Uterine Sarcoma; UUS

Endometrial Stromal Sarcoma (ESS) is the rarest type of uterine cancer that accounts for less than 1% of cancers of the female reproductive organs. These tumors tend to occur more often in premenopausal women between 40-50 years of age. This is younger than the average for uterine cancer in general (early 60s). African American women are two times more likely to get these rare uterine cancers.

Diagnostic testing:

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  Examination of endometrial cells collected during a dilation and curettage (D&C) procedure;

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  Endometrial biopsy;

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  Hysteroscopy; and

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  MRI or CT scans of the pelvis.

Physical findings:

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  Vaginal bleeding and/or spotting in postmenopausal women;

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  Abnormal uterine bleeding;

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  Anemia caused by chronic loss of blood;

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  Enlarged uterus;

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  Lower abdominal pain or pelvic cramping; or

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  Vaginal discharge.

ICD-9: 182.0

The prognosis for ESS is poor as it is a high grade malignancy that grows and metastasizes quickly. By the time this sarcoma is diagnosed, it has often spread outside of the uterus. Once treated, it also has a high propensity for recurrence.

Most ESS are treated with complete surgical resection including total abdominal hysterectomy (removal of the uterus) and bilateral salpingo-oophrorectomy (removal of the fallopian tube and ovary), plus chemotherapy and radiation therapy.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Pathology report of a biopsy specimen of the stroma of the endometrium; and

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  Surgery reports and/or imaging that document spread of cancer.

REMARKS

Listing level severity must be documented.