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Presented by Ann E. Smith Sehdev, M.D. and prepared by Bahram R. Oliai, M.D.
Case 1: A 56 year old female with a hysterectomy performed for “fibroid” uterus.
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Question 1 of 1
1. Question
Week 58: Case 1
A 56 year old female with a hysterectomy performed for fibroid uterus./images/01-21021a.jpg
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/images/01-21021e.jpgCorrect
Answer: Endometrial stromal sarcoma
Histology: This tumor is composed of cells that resemble the stromal cells of proliferative phase endometrium. The cells are uniform with ill-defined cell borders, ovoid nuclei, and inconspicuous nucleoli. Mitotic figures are infrequent (<3/10 HPF). The tumor invades the myometrium in a characteristic pattern, which is evident in this case, and can also invade lymphatics including those of the mesosalpinx and mesovarium.
Discussion: Low grade endometrial stromal sarcomas (LGESS) occur in younger women than does carcinosarcoma, leiomyosarcoma, or endometrial carcinoma. Patients typically present with abnormal vaginal bleeding, pelvic pain, and a clinical impression of uterine leiomyomata. The uterus is typically enlarged and has an irregular contour. The stage of the tumor and the type of surgical procedure determine the risk of recurrence. Adnexal spread is not always appreciated during the operation and pelvic recurrence is more frequent when the ovaries are conserved. Women with stage I LGESS have >80% survival, while the survival is less favorable (~50%) when there is advanced stage disease. Although progestin therapy may be effective in those cases with progesterone receptors, chemotherapy is usually ineffective. Arterioles that resemble the endometrial spiral arterioles are uniformly distributed throughout LGESS and can lead to the mistaken diagnosis of hemangiopericytoma. Distinguishing LGESS from cellular leiomyomas can be difficult. Recently a new immunohistochemical marker, CD 10, has been introduced to help distinguish leiomyomas from LGESS. In a recently published study, CD 10 was shown to be positively expressed in 10 of 10 LGESS and in only 3 of 9 smooth muscle tumors, (Appl Immunohistochem Molecul Morphol. 2001 Jun;9(2):164-9). At our institution we commonly use an immuno panel including desmin, actin, and CD 10 to distinguish smooth muscle neoplasms from LGESS. Although LGESS typically express estrogen and progesterone receptors, they should not be confused with the much more malignant mixed mesodermal tumor which show marked pleomorphism and a high mitotic index.
Incorrect
Answer: Endometrial stromal sarcoma
Histology: This tumor is composed of cells that resemble the stromal cells of proliferative phase endometrium. The cells are uniform with ill-defined cell borders, ovoid nuclei, and inconspicuous nucleoli. Mitotic figures are infrequent (<3/10 HPF). The tumor invades the myometrium in a characteristic pattern, which is evident in this case, and can also invade lymphatics including those of the mesosalpinx and mesovarium.
Discussion: Low grade endometrial stromal sarcomas (LGESS) occur in younger women than does carcinosarcoma, leiomyosarcoma, or endometrial carcinoma. Patients typically present with abnormal vaginal bleeding, pelvic pain, and a clinical impression of uterine leiomyomata. The uterus is typically enlarged and has an irregular contour. The stage of the tumor and the type of surgical procedure determine the risk of recurrence. Adnexal spread is not always appreciated during the operation and pelvic recurrence is more frequent when the ovaries are conserved. Women with stage I LGESS have >80% survival, while the survival is less favorable (~50%) when there is advanced stage disease. Although progestin therapy may be effective in those cases with progesterone receptors, chemotherapy is usually ineffective. Arterioles that resemble the endometrial spiral arterioles are uniformly distributed throughout LGESS and can lead to the mistaken diagnosis of hemangiopericytoma. Distinguishing LGESS from cellular leiomyomas can be difficult. Recently a new immunohistochemical marker, CD 10, has been introduced to help distinguish leiomyomas from LGESS. In a recently published study, CD 10 was shown to be positively expressed in 10 of 10 LGESS and in only 3 of 9 smooth muscle tumors, (Appl Immunohistochem Molecul Morphol. 2001 Jun;9(2):164-9). At our institution we commonly use an immuno panel including desmin, actin, and CD 10 to distinguish smooth muscle neoplasms from LGESS. Although LGESS typically express estrogen and progesterone receptors, they should not be confused with the much more malignant mixed mesodermal tumor which show marked pleomorphism and a high mitotic index.
