• Reprinted from NCI Cancer Bulletin for February 8, 2011

    The spread of cancer cells from their original location to other sites in the body, known as metastasis, has long been thought of as a one-way journey. But some researchers also believe that metastatic cancer cells can fuel primary tumor growth, with potentially important implications for the timing and nature of cancer treatment.

    The concept of tumor self-metastasis, or tumor “self-seeding,” originated at Memorial Sloan-Kettering Cancer Center, based on a series of studies led by Drs. Joan Massagué, head of the Metastasis Research Center, and Larry Norton, deputy physician-in-chief of the center’s breast cancer programs.

    In studies of mice, Dr. Massagué observed that breast tumors expressing genes associated with metastasis were growing faster than tumors that didn’t express these genes, even though the genes had no apparent role in increased cell division or decreased cell death. “Moreover, the fraction of dividing cells was not higher in fast-growing tumors versus tumors that are slower growing,” explained Dr. Norton.

  • Introduction

    Preoperative differentiation of endometrial stromal sarcoma (ESS) from myoma is challenged by the similarities in their clinical presentations and the unreliability of imaging studies. Often, ESS is diagnosed postoperatively after laparoscopic power morcellation of a presumed myoma (Amant et al., 2014 and Park et al., 2011). In approximately 80% of ESS cases diagnosed postoperatively after morcellation, preoperative diagnosis was either leiomyoma or adenomyosis (Park et al., 2011). Laparoscopic morcellation of ESS with an electromechanical morcellator can result in the dissemination of the tumor (Park et al., 2011 and Badia and Karini, 2010), but there has been no report of the spread of ESS after morcellation with a single incision on the uterus using a harmonic scalpel. Furthermore, if the ovaries are preserved at the time of the initial surgery, bilateral salpingo-oophorectomy (BSO) for recurrence is recommended (Amant et al., 2014 and Yoon et al., 2014), but the effect of BSO for recurrent ESS has not been reported. Here, we present a case where morcellation with a simple incision in total laparoscopic hysterectomy for a presumed myoma led to the peritoneal dissemination of ESS; and complete remission was achieved and laparoscopically confirmed after BSO for recurrent ESS.

    Link to full article: http://www.sciencedirect.com/science/article/pii/S235257891400037X

  • From the 2009 ASCO Annual Meeting

    Abstract:

     

    Background:Endometrial stromal sarcomas (ESS) traditionally have been classified as low grade or high grade based on mitotic activity and histologic appearance. High-grade tumors are currently referred to as undifferentiated uterine sarcomas and are not included in this series. ESS are known to have high expression of estrogen and progesterone receptors. This is a retrospective study of patients with metastatic ESS treated with hormonal therapy. Methods: Following approval by the institutional review board all patients diagnosed with ESS from 1987-2007 were identified. Clinical and demographic information were abstracted from the charts and all histologic materials were re-reviewed. Estrogen and progesterone receptor testing was performed utilizing mouse monoclonal antibodies and the Cell Analysis Systems 200 Image analyzer. Survival was calculated using the Kaplan-Meier method and comparisons utilized the log rank test. Results: Thirteen patients with ESS were identified during this period. Seven had disease confined to the uterus. Six had extrauterine disease; 5 patients presented with metastases and 1 patient presented with a pelvic recurrence 20 years following a hysterectomy. All underwent surgical resection except for 1 patient that declined surgery. All 6 patients with metastases had tumors that tested positive for estrogen and progesterone receptors; all were treated with megestrol acetate initially for a period of 1-4 years. Two patients were then changed to maintenance with medroxyprogesterone acetate. Three patients with persistent disease were changed to aromatase inhibitors; 1 to letrozole and 2 to anastrazole. One of these patients has had a complete response and 2 have had stable disease. One patient has been lost to follow-up. Follow-up for the 6 patients was 2-22 years; no known patients died of their disease. Actuarial 2- and 5-year survivals were 80% and 65%, respectively. There was no significant difference in survival between patients with metastases and without metastases. Conclusions: ESS tumors are relatively uncommon and there is an absence of studies to guide treatment of patients with metastases. This experience indicates that these tumors respond well to hormonal manipulation. Treatment with progestins or aromatase inhibitors may result in remission or stable disease.

    Link: http://www.abstract.asco.org/AbstView_65_30210.html

  • From PubMed

    BACKGROUND: Approximately 50% of patients with low-grade endometrial stromal sarcoma (ESS) develop recurrent disease, mainly in lung or pelvis. Bone metastasis of low-grade ESS is an extremely rare phenomenon. CASE: A 63-year-old Japanese female developed multiple bone and lung metastases 18 years after initial treatment for low-grade ESS. Bone scintigram showed a high uptake area at thoracic spine (Th6, Th8-9 and Th12), right 9th rib, iliac bone, and sacrum. Radiation therapy with Liniac of 4500cGy to the Th6 vertebra and Liniac of 4200cGy to sacrum was performed for the palliation of the pain. Radiotherapy was effective for the pain relief, although the size of recurrent tumor was unchanged. CONCLUSION: This is the first detailed reported case of multiple bone recurrence in a patient with low-grade ESS. The long-term follow-up after treatment is recommended.

    Click here for link to article

  • BACKGROUND: Endometrial stromal sarcoma (ESS) represents 0.2% of all uterine malignancies. Based on the mitotic activity, a distinction is made between low and high-grade ESS. Although the overall five-year survival rate for low-grade ESS exceeds 80%, about 50% of the patients show tumor recurrence, mostly after a long latency period. Tumor invasion of the great vessels is extremely rare. We describe a patient with advanced low-grade ESS with tumor invasion of the infrarenal aorta and the inferior vena cava. The patient presented with a large tumor thrombus extending from the inferior vena cava into the right atrium. METHODS: Review of literature and identification of 19 patients, including our own case report, with advanced low-grade ESS with invasion of the great vessels and formation of an inferior vena cava tumor thrombus. RESULTS: All 19 patients presented with an abdominal tumor mass and a tumor thrombus protruding into the inferior vena cava. The tumor thrombus extended into the right heart cavities in nine patients reaching the right atrium in four, the right ventricle in three and the pulmonary artery in two patients. There were 5 patients with an advanced primary tumor and 14 patients with an advanced recurrent tumor. Seven patients presented with synchronous metastatic disease and six patients with a pelvic tumor infiltrating the bladder, the rectosigmoid colon or the infrarenal aorta. Mean age at surgery was 45.9+/-12.3 years (median 47, range 25-65 years). Tumor thrombectomy was accomplished by cavatomy or by right atriotomy after installation of a cardiopulmonary bypass. There was no peri-operative mortality and a very low morbidity. Radical tumor resections were achieved in 10 patients. The follow-up for these 10 patients was 2+/-1.3 years (median 2, range 0.3-4.5 years). Nine patients remained recurrence free whereas one patient suffered an asymptomatic local recurrence.

  • Biotech Histochem. 2011 Aug 15. [Epub ahead of print]

    Proper evaluation of lung nodules is a difficult issue for clinical management of patients. Discriminating metastatic endometrial stromal sarcoma (ESS) from other primary spindle cell neoplasms of the lung using histological analysis can be challenging. This is particularly true when an adequate clinical history is lacking, because ESS metastasis can be delayed by a couple of decades. To emphasize the importance of the correlation of pathological findings with clinical history and imaging studies, we investigated 11 cases of ESS (seven low grade and four high grade) metastatic to the lung. All cases presented with one to multiple unilateral or bilateral lung nodules that were detected by chest computed tomography. Primary ESS was diagnosed from hysterectomy specimens except for one by endometrial biopsy, 0.5 to 23 years prior to metastasis. Immunohistochemical studies showed that all ESS cases were moderately to strongly positive for Bcl-2 and CD10 with > 50% of tumor cells stained, except for one high grade ESS that was negative for CD10. Eight (72.7%) and seven (63.6) of the 11 cases showed positive estrogen and progesterone receptors, respectively, with a majority of positive cases showing diffuse and moderate to strong staining. Strong but patchy staining for CD34 was detected in one (9.1%) case with smooth muscle differentiation. CK7 and TTF-1 were negative in all cases. Two (18.2%) cases exhibited patchy and strong positivity for caldesmon. Two (18.2%) low grade ESS cases showed moderate to strong AE1/AE3 positivity in > 50% of tumor cells, one of which also showed moderate CK19 and Cam 5.2 staining in >30% of tumor cells. One should be cautious when assessing spindle cell neoplasms of the lung in women with a history of hysterectomy. Correlation of clinical history and imaging studies with histological and immunohistochemical findings is essential to diagnosis of metastatic ESS to the lung.

    Link to abstract:
    http://www.ncbi.nlm.nih.gov/pubmed/21838613

  • Reprinted from Human Pathology,November 2017, Volume 10, Pages 98–101

    Abstract

    Endometrial stromal sarcoma (ESS) is a rare malignant tumor of the endometrium, usually seen in perimenopausal females. We report a case of ESS in a 24 year old woman, presenting as rapid enlargement of a uterine fibroid associated with irregular and excessive vaginal bleeding along with a lung nodule. Hysterectomy was performed. Histopathological examination and immunohistochemistry confirmed ESS. A proper preoperative diagnosis is difficult and in most cases the diagnosis is confirmed after hysterectomy for a presumed benign disease. Simultaneous pulmonary metastasis of ESS is an extremely rare event. As the tumor is rarely encountered, management protocols are still questionable. Although rare, ESS should be considered in the differential diagnosis of all women who present with a rapid enlargement of a uterine leiomyoma. This report is aimed to present a case of endometrial stromal tumor in a young female with simultaneous lung metastasis because of its rare existence and difficulties in establishing histological diagnosis.