Endometrial stromal sarcoma

A tumour resembling endometrial stroma.

Clinical

Pulmonary metastases occur on average ten years after the diagnosis of the primary tumour8.

Histology

The classical appearance is of small cells with scant cytoplasm and round to oval nuclei. The accompanying vasculature resembles spiral arterioles, as well as staghorn vessels and anastomosing capillaries.

variants

Patients may have a primary with classical histology and metastases with variant (sometimes with other, classical, metastases) histology7.

Immunohistochemistry

SMA

1/121, 3/33, 2/24, 0/75, 8/138, 0/110

desmin

1/121, 12/242, 3/33, 2/24, 0/75, 7/138, 1/69, 0/110

h-caldesmon

0/121, 0/212, 0/69

HHF-35

3/33, 2/24

MyoD1

0/110

vimentin

3/33, 2/24, 7/75, 13/148, 1/110

cytokeratin

0/75

AE1 / AE3

6/138, 2/109

MNF116

0/110

Cam 5.2

1/138, 2/109, 0/110

BerEP4

0/110

CD10

17/196, 9/109

Calponin

7/109, 1/110

oestrogen receptors

2/24, 2/75, 13/138

progesterone receptors

3/33, 2/24, 14/148

calretinin

1/7

inhibin

1/138, 0/109

Chromogranin

1/138, 0/110

Synaptophysin

0/110

PGP9.5

1/110

NSE

0/110

S-100

0/110

HMB45

1/138

CD34

1/138, 0/109, 0/110

CD56

1/110

CD68

0/110

CD99

0/109

CD117

0/109

MIC2

0/110

   
   
   

Those cases with variant histology show a higher rate of positivity for muscle markers. There may be a CD10+ endometrial stromal component and a CD10- smooth muscle / myofibroblastic component7.

desmin

7/107, 4/49

h-caldesmon

6/77, 4/49

CD10

10/127

   
   

Differential diagnosis

Prognosis

The prognosis is excellent, even in the presence of pulmonary metastases8.

References

1Rush, D. S., Tan, J., Baergen, R. N., Soslow, R. A. h-Caldesmon, a novel smooth muscle-specific antibody, distinguishes between cellular leiomyoma and endometrial stromal sarcoma. Am J Surg Pathol 2001;25:253-258.

2Nucci, M. R., O'Connell, J. T., Huettner, P. C., Cviko, A., Sun, D., Quade, B. J. h-Caldesmon expression effectively distinguishes endometrial stromal tumors from uterine smooth muscle tumors. Am J Clin Pathol 2001;25:455-463.

3Fukunaga, M., Ishihara, A., Ushigome, S. Extrauterine low-grade endometrial stromal sarcoma: report of three cases. Pathology International 1998;48:297-302

4Fukunaga, M., Miyazawa, Y., Ushigome, S. Endometrial low-grade stromal sarcoma with ovarian sex cord-like differentiation: report of two cases with an immunohistochemical and flow cytometric study. Pathology International 1997;47:412-415

5Tosi, P., V. Sforza, et al. (1989). "Estrogen receptor content, immunohistochemically determined by monoclonal antibodies, in endometrial stromal sarcoma." Obstet Gynecol 73(1): 75-8.

6McCluggage, W. G., Sumathi, V. P., Maxwell, P. CD10 is a sensitive and diagnostically useful immunohistochemical marker of normal endometrial stroma and of endometrial stromal neoplasms. Histopathology 2001;39:273-278.

7Yilmaz, A., D. S. Rush, et al. (2002). "Endometrial stromal sarcomas with unusual histologic features: a report of 24 primary and metastatic tumors emphasizing fibroblastic and smooth muscle differentiation." Am J Surg Pathol 26(9): 1142-50.

8Aubry, M. C., J. L. Myers, et al. (2002). "Endometrial stromal sarcoma metastatic to the lung: a detailed analysis of 16 patients." Am J Surg Pathol 26(4): 440-9.

9Oliva, E., R. H. Young, et al. (2002). "An immunohistochemical analysis of endometrial stromal and smooth muscle tumors of the uterus: a study of 54 cases emphasizing the importance of using a panel because of overlap in immunoreactivity for individual antibodies." Am J Surg Pathol 26(4): 403-12.

10Khan, O. A., K. Rogers, et al. (2004). "Lung metastases as an initial presentation of endometrial stromal sarcoma: the utility of the CD10 antibody." Histopathology 45(5): 544-6.

This page last revised 25.12.2003.

©SMUHT/PW Bishop