GYNECOLOGIC

ONCOLOGY

8,

92-96 (1979)

CASE REPORT

Stage III Uterine Sarcoma: Case Report and Literature Review ROBERTO

YAZIGI, M.D., M. STEVEN PIVER, M.D., AND JOSEPH J. BARLOW, M.D., FACOG

FACOG,

Department of Obstetrics and Gynecology, Roswell Park Memorial Institute, New York State Department of Health, Buffalo, New York 14263 Received May 23, 1978 A case of Stage III endometrial stromal sarcoma treated by combination of surgery, Adriamycin, and radiation therapy with survival of more than 5 years is presented. A literature review of Stage III uterine sarcomas reveals a survival rate of 7.1% (16/224). These patients are analyzed by extension of disease and treatment. This report confirms the poor prognosis of this disease and suggests that the best treatment may be aggressive combined management.

Uterine sarcomas are considered to be the most lethal of tumors arising in this organ. The poor prognosis of this disease, even in its early stages, is widely recognized and has not improved in the last two decades despite different treatment modalities. Most oncologists agree that surgery has a definite role, but there has been debate concerning the benefits of radiation therapy and/or chemotherapy in women with Stage III (pelvic extension) uterine sarcomas. In a previous report from Roswell Park Memorial Institute on clinical Stage I (confined to fundus) and II (cervical involvement) uterine sarcomas, seven women were found to have surgical Stage III; three of the seven survived 5 years, one by surgery alone and two by the addition of pelvic irradiation. These patients had ovarian, broad ligament, and obturator node metastases, respectively. We have treated a patient with clinical Stage III endometrial stromal sarcoma successfully with chemotherapy, surgery, and radiation therapy. This case has encouraged us to review the American literature in regard to treatment and survival of Stage III uterine sarcomas. CASE REPORT

B.B. is a 36-year-old white female who in February 1972 was found to have a pelvic mass. She underwent exploratory laparotomy and was found to have a uterine tumor which extended to the right broad ligament and parametrium. The 92 0090-8258/79/040092-05$01.00/O Copyright All rights

@ 1979 by Academic Press, Inc. of reproduction in any form reserved

STAGE

111

UTERINE

SARCOMA

93

tumor was partially resected, and pathology demonstrated endometrial stromal sarcoma, with a mitotic rate of greater than 10 mitoses per 10 high-power fields. She was referred to Roswell Park Memorial Institute with a residual 14 x 10 x IO-cm pelvic mass. A urogram demonstrated that there was no function of the right kidney. She was treated with Adriamycin, 30 mg/m’, daily for 3 days and the treatment was repeated every 4 weeks. A good objective response was observed after five courses of chemotherapy with diminution of size of the pelvic mass to 8 x 8 cm and complete recovery of right kidney function on repeat urogram. In August 1972 she underwent exploratory laparotomy with a view toward resecting the residual mass. The tumor extended from the right parametrium to the pelvic wall. A total abdominal hysterectomy, bilateral salpingo-oophorectomy, and partial resection of pelvic tumor was performed. However, attempt at complete tumor removal had to be abandoned because of her unstable cardiovascular status during the procedure. Pathology demonstrated stromal sarcoma of the uterus with extension to the right ovary and right parametrium, and at this time the mitotic rate was found to be less than 10 per 10 high-power fields. Postoperatively, she was started on pelvic radiotherapy, delivering 5600 rad in 6 weeks. She remained asymptomatic and with no evidence of disease until May 1977 when she developed an enterocutaneous and enterovesicovaginal fistula, demonstrated by cystogram, fistulogram, and small bowel series. She underwent ileotransverse enterocolostomy and isolation of fistula in September 1977. At the time of exploratiom, there was no evidence of recurrence. She is now alive without recurrence 6 years after diagnosis. MATERIAL

AND

METHODS

On reviewing published articles on uterine sarcomas, our first attempt was to determine survival rates on those cases clearly stated to be Stage III, having excluded all of those series which did not clearly define the stage of disease. Only 20 series were found in which these data could be obtained, allowing analysis of 224 cases evaluable for S-year survival. We have divided these cases into endometrial stromal sarcomas, leiomyosarcomas, mixed miillerian tumors, and unclassified sarcomas. Included in the term mixed miillerian tumors are those with either homologous or heterologous elements. The term carcinosarcoma has not been used according to Kempson and Bari’s [ 11 classification of uterine sarcomas. We have excluded analysis of endolymphatic stromal myosis which is considered a disease of entirely different biologic behavior in terms of recurrence, metastatic potential, and prognosis. An attempt was also made to correlate survival with treatment modalities: surgery alone: surgery plus radiation therapy: or radiotherapy alone. However, of this group of 224 cases, only 70 women had their treatment clearly specified and are available for comparison. RESULTS

F&e-year survivul. Table 1 lists the 20 authors who have presented series of Stage III uterine sarcomas. There were 85 cases of mixed miillerian tumors, 64

94

YAZIGI,

PIVER, AND BARLOW

TABLE 1 STAGE III UTERINE SARCOMAS: ~-YEAR SURVIVAL Total Author

Year

Finn [2] Crawford and Tucker [3] Ober and Tovell [4] Edwards et al. [5] Montague et al. [6] Aaro et al. [7] Mendel et al. [83 Ruffolo et al. [9] Badib et al. [lo] Chuang et a/. [ 1I] Kempson and Bari [l] Giarratano and Slate [ 121 Saksela et a/. [ 131 Mortel et al. [14] Hayes [ 151 Gilbert ef al. [ 161 Belgrad et al. [ 171 Sakamoto and Sugano [IS] Vongtama ef a/. [I91 Yoonesi and Hart [20]

1950 1955 1959 1963 1965 1966 1967 1969 1969 1970 1970 1971 1974 1974 1974 1975 1975 1976 1976 1977

Total

No.

Survivors

8 2 1 3 3 36 4 3 24 22 26 4 18 7 17 30 5 2 7 2

0 0 1 0 0 3 0 0 1 0 0 0 1 2 0 2 2 0 3 0

2.24

16 (7.1%)

endometrial stromal sarcomas, 58 leiomyosarcomas, and 17 unclassified sarcomas. Of these 224 cases, only 7.1% (16) of the patients survived 5 years or more. Table 2 outlines the treatment of the 16 women surviving 5 or more years. Of this group, 7 were treated with surgery plus radiation therapy, 3 with surgery alone, 2 with radiotherapy alone, and in four cases, treatment was not specified. Although 16 patients survived over 5 years, 3 subsequently died of their disease 7, 9, and 11 years after treatment; one was alive at 12 years with cancer and only 12 were apparently alive without evidence of disease. Correlation of treatment-survival by histology. Of the 70 patients which can be analyzed for treatment, 20 were treated by surgery alone and these had a median survival of 13 months. Thirty-three were treated with surgery plus radiation therapy with a median survival of 16 months, and 17 were treated with radiation therapy alone with a median survival of 4 months (Table 3). DISCUSSION

The prognosis of patients with advanced uterine sarcomas continues to be poor. We have collected 224 cases from the literature with Stage III uterine sarcoma and have found a 5-year survival rate of only 7. I%, with an equally poor prognosis for the different histologic types. A few patients with Stage III uterine sarcoma are successfully treated by

STAGE

111 UTERINE

95

SARCOMA

TABLE 2 STAGE III UTERINE SARCOMA ANALYSIS OF FIVE-YEAR SURVIVAL Case 1

4 5 6 7 8

13 14 15 16

Author Ober and Tovell [4] Aaro ct (il. ]7] Aaro et al. [7] Aaro et trl. [7] Badib Yf al. [IO] Kempson and Bari [I] Saksela 81 ul. [ 131 Mortel (‘I ul. [ 141 Mortel Cl ul. [ 141 Gilbert et ui. [ 161 Gilbert er ul. [ 161 Belgrad C? 4. [ 171 Belgrad (‘I cl/. [I71 Vongtama t’t cd. [ 191 Vongtama (‘I ul. [I91 Vongtama (‘I UI. [ 191

a RT, radiation rectomy.

Treatment”

Extension

Outcome

Broad ligament infiltrated Right broad ligament Not specified

Supravaginal hysterectomy. pelvic RT TAH. BSO, resection of lig. nodule Not specified

11 years, died of disease 9 years, died of disease >5 years

Not specified

Not specified

>5 years

Not specified

Surgery plus RT

>5 years

Parametrium

Not specified

Beyond uterus

Not specified

7 years, died of disease >5 years

Ovarian

TAH. BSO

Ovarian Not specified

TAH, BSO, P32, RT Surgery plus RT

Not specified

Surgery plus RT

6 years NED

Vagina and pelvis

RT

8 years NED

Pelvis

RT

Ovarian

Surgery plus RT

12 years with cancer >5 years

Obturator nodes

Surgery plus RT

>S years

Right broad ligament

Surgery

>S years

therapy: TAH,

total abdominal

hysterectomy:

6 years, 8 months, NED (‘?) 18 years NED (?) 14 years NED

BSO, bilateral

salpingo-oopho-

surgery alone or surgery plus pelvic irradiation. However, this number is too small to recommend this as standard therapy. We have achieved a 6-year survival without recurrence in a patient with Stage III uterine sarcoma by the use of Adriamycin, surgery, and radiation therapy. Possibly only by the use of multimodality therapy with chemotherapy, surgery, and radiation will we improve upon the very poor prognosis of women with Stage III uterine sarcomas. REFERENCES 1. Kempson, R. L., and Bari, W. Uterine sarcomas. Classification, diagnosis and prognosis, Hum. Pathol. 1, 331-349 (1970). 2. Finn. W. F. Sarcoma of the uterus, Amer. J. Obuet. G~etd. 60, 1254-1262 (1950).

YAZIGI,

96

PIVER,

AND BARLOW

TABLE 3 STAGE III UTERINE SARCOMA SUMMARY-TREATMENT-SURVIVAL

Histology Endometrial stromal sarcoma Mixed mesodermal tumor Leiomyosarcoma Unclassified or unknown Total

No.

RT

Surgery + RTa

Surgery

Survivors

No.

Survivors

No.

Total

Survivors

No. Survivors

4

0

5

2

5

1

14

3

8 7

1 I

18 8

1 2

8 4

0 1

34 19

2 4

I

I

2

2

0

0

3

3

3

33

7

17

2

70

20 15%

21.2%

11.7%

12 17%

a RT, radiation therapy. 3. Crawford, E. J., and Tucker, R. Sarcoma of the uterus, Amer. J. Obstet. Gynecol. 77, 286-291 (1959). 4. Ober, W. B., and Tovell, H. M. Mesenchymal sarcomas of the uterus, Amer. J. Obster. Gynecol. 77, 246-269 (1959). 5. Edwards, D. L., Sterling, L. N., Keller, R. M., and Nolan, J. F. Mixed heterologous mesenchymal sarcomas (mixed meiodermal sarcomas) of the uterus, Amer. J. Obsret. Gynecol. 85, 1007-1011 (1963). 6. Montague, A. C., Swartz, D. P., and Woodruff, J. D. Sarcoma arising in a leiomyoma of the uterus, Amer. J. Obster. Gynecol. 92, 421-427 (1965). 7. Aaro, L. A., Symmonds, R. E., and Dockerty, M. B. Sarcoma of the uterus, Amer. J. Obster. Gynecol. 94, 101-109 (1966). 8. Mendel, E. B., Lucas, F., and Panopio, I. Malignant mixed Mullerian tumors of the uterus, Obsret. Gynecol. 30, 66-69 (1967). 9. Ruffolo, E. H., Metts, N. B., and Sanders, H. L. Malignant mixed Mullerian tumors of the uterus, Obstet. Gynecol. 33, 544-552 (1969). 10. Badib, A. D., Vongtama, V., Kurohara, S. S., and Webster, J. H. Radiotherapy in the treatment of sarcomas of the corpus uteri, Cancer 24, 724-729 (1969). il. Chuang, J. T., Van Velden, D. J. J., and Graham, J. B. Carcinosarcoma and mixed mesodermal tumor of the uterus corpus, Obster. Gynecol. 35, 769-780 (1970). 12. Giarratano, R. C., and Slate, T. A. Sarcomas of the uterus, Obsret. Gynecol. 38,472-477 (1971). 13. Saksela, E., Lampinen, V., and Procope, B. J. Malignant mesenchymal tumors of the uterine corpus, Amer. J. Obstet. Gynecol. 120, 452-460 (1974). 14. Mortel, R., Koss, L. G., Lewis, J. L., and D’Urso, J. R. Mesodermal mixed tumors of the uterine corpus, Obster. Gynecol. 43, 248-252 (1974). 15. Hayes, D. Mixed Mullerian tumors of the corpus uteri, J. Obster. Gynaecol. &it. Commonw. 81, 160-164 (1974). 16. Gilbert, H. A., Kagan, A. R., Lagasse, L., Jacobs, M. R., and Tawa, K. The value of radiation therapy in uterine sarcoma, Obstet. Gynecol. 45, 84-88 (1975). 17. Belgrad, R., Elbadowi, N., and Rubin, P. Uterine sarcoma, Radiology 114, 181-188 (1975). 18. Sakamoto, A., and Sugano, H. Mixed mesodermal tumor of the uterine body: Relationship between histology and survival, Gann 67, 263-273 (1976). 19. Vongtama, V., Karlen, J. R., Piver, M. S., Tsukada, Y., and Moore, R. E. Treatment, results and prognostic factors in stage I & II sarcomas of the corpus uteri, Amer. J. Roentgenol. Radium Ther. Nucl. Med. 126, 139-147 (1976). 20. Yoonesi, M., and Hart, W. R. Endometrial stromal sarcomas, Cancer 40, 898-906 (1977).

Stage III uterine sarcoma: case report and literature review.

GYNECOLOGIC ONCOLOGY 8, 92-96 (1979) CASE REPORT Stage III Uterine Sarcoma: Case Report and Literature Review ROBERTO YAZIGI, M.D., M. STEVEN PI...
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