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Metastatic ESS

Metastatic endometrial stromal sarcoma of the lung: importance of immunohistochemical staining, clinical history and imaging studies

Written by Mentrikoski M, Zhao C, Zhang J, Wang H, McMahon L, Yang Q, Li F, Xu H. | 15 August 2011

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

Low-grade endometrial stromal sarcoma with inferior vena cava tumor thrombus and intracardiac extension

Written by Renzulli F, Weimann R, Barras JP, Carrel TP, Candinas D | 11 July 2010

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.

Hormonal treatment of metastatic endometrial stromal sarcoma

Written by K. Y. Terada, J. Davis, J. Kitayama, D. Shimizu; University of Hawaii, Honolulu, HI; Cancer Research | 09 June 2009

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

Long-term survival of patients given hormonal therapy for metastatic endometrial stromal sarcoma

Written by Suzy Johnson | 28 September 2008

J. -P. Spano, J. C. Soria, M. Kambouchner, S. Piperno-Neuman, F. Morin, J. -F. Morere, A. Martin and J. -L Breau

(1)	Department of Oncology, Avicenne Hospital, Bobigny, France

(2)	Department of Oncology, Institut Gustave Roussy, Willejuif, France

(3)	Department of Anatomopathology, Avicenne Hospital, Bobigny, France

(4)	Department of Oncology, Institut Curie, Paris, France

(5)	Service d’Oncologie Médicale, Hôpital Avicenne, 125 rue de Stalingrad, 93 009 Bobigny, Cedex, France

Received: 29 May 2002 Accepted: 30 September 2002

Abstract Endometrial stromal sarcoma (ESS) is a rare neoplasm, mainly observed in premenopausal women. We describe two women 44 and 34 years old at the time ESS diagnosis, who developed lung metastases 3 and 6 years, respectively, after initial treatment: hysterectomy without (case 1) or with oophorectomy (case 2), followed by hormone replacement therapy (HRT) for the latter. Their estrogen (ER) and progesterone receptors (PR) were analyzed biochemically in metastatic lung tissue, yielding respective concentrations of ER 242 and 184, and PR 910 and 100 fmol/mg of cytosol protein. Both patients started treatment with the aromatase inhibitor aminoglutethimide (500 mg qid) after surgery for the first patient and after stopping HRT for the second. Under aromatase-inhibitor therapy, both patients achieved a complete response, patient 1 remains disease-free with 14+ years of follow-up, and patient 2 with 7+ years. Our data suggest that an aromatase inhibitor may be an effective treatment for ESS. Furthermore, routine ER and PR analyses could be useful to predict the response to hormonal therapy in ESS.

Link to abstract

LG ESS recurring with multiple bone and lung metastases

Written by Suzy Johnson | 16 October 2007

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.

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