Long-term survival in low-grade endometrial stromal sarcoma with childbirth and multidisciplinary treatment: a case report.
BACKGROUND
Low-grade endometrial stromal sarcoma is very rare and difficult to diagnose in the early stage. A standard treatment has not been established. In this case report of a patient with long-term survival, we describe an effective treatment for advanced low-grade endometrial stromal sarcoma.


METHODS
A 24-year-old Japanese woman who presented with prolonged menstruation was diagnosed with leiomyoma on the basis of a specimen resected transvaginally. She underwent ten resections in 10 years without a malignancy diagnosis. During this period, she gave birth. At age 34 years, she visited our hospital, complaining of lower abdominal pain. A 10cm tumor was detected behind her uterus. The disease was diagnosed as an advanced malignant ovarian tumor before surgery. A laparotomy was performed, with many remnants left in the abdominal cavity. The final diagnosis was advanced low-grade endometrial stromal sarcoma. After 12 cycles of gemcitabine and docetaxel combination chemotherapy, the tumor disappeared completely. A retrospective pathological review of the specimens resected transvaginally showed that the tumors included low-grade endometrial stromal sarcoma elements. When the patient was age 42 years, the sarcoma recurred. It was detected around the right diaphragm and liver. Despite administration of gemcitabine and docetaxel, ascites and pleural effusion accumulated. Administration of medroxyprogesterone acetate, leuprorelin acetate, and anastrozole gradually reduced the ascites and pleural effusion. In addition to the three hormone drugs, 18 cycles of paclitaxel and carboplatin were administered. The patient recovered from her critically ill state and is currently alive with reduced tumor at age 45 years.


CONCLUSIONS
Our patient with low-grade endometrial stromal sarcoma whose disease began in her youth gave birth and experienced long-term survival with surgery, chemotherapy, and hormone therapy.
Department of Obstetrics and Gynecology, Meijo Hospital, Sannomaru 1-3-1, Naka-ku, Nagoya, 460-0001, Japan. osamumaeda@med.nagoya-u.ac.jp.
Osamu
O
Maeda
Department of Pathology, National Hospital Organization, Nagoya Medical Center, Nagoya, Japan. moritanis@aol.com.
Suzuko
S
Moritani
Department of Pathology, National Hospital Organization, Nagoya Medical Center, Nagoya, Japan. shu-kkr@umin.ac.jp.
Shu
S
Ichihara
Kishokai Medical Corporation, Inazawa, Aichi, Japan. tinoue-kishokai-iph@i.softbank.jp.
Takami
T
Inoue
Department of Gynecology, Inazawa Municipal Hospital, Inazawa, Aichi, Japan. ishi-y@sannet.ne.jp.
Yutaka
Y
Ishihara
Department of Obstetrics and Gynecology, Kariya-Toyota General Hospital, Kariya, Aichi, Japan. ktgh.sanfujinka@toyota-kai.or.jp.
Shinichi
S
Yamamoto
Department of Pathology and Laboratory Medicine, Kariya-Toyota General Hospital, Kariya, Aichi, Japan. makoto.ito@toyota-kai.or.jp.
Makoto
M
Ito
Department of Obstetrics and Gynecology, Meijo Hospital, Sannomaru 1-3-1, Naka-ku, Nagoya, 460-0001, Japan. y-matsumura@meijohosp.com.
Yoko
Y
Matsumura
Department of Obstetrics and Gynecology, Meijo Hospital, Sannomaru 1-3-1, Naka-ku, Nagoya, 460-0001, Japan. k-sugiyama@meijohosp.com.
Kumiko
K
Sugiyama
Department of Obstetrics and Gynecology, Meijo Hospital, Sannomaru 1-3-1, Naka-ku, Nagoya, 460-0001, Japan. m-horio@meijohosp.com.
Maiko
M
Horio
Department of Obstetrics and Gynecology, Meijo Hospital, Sannomaru 1-3-1, Naka-ku, Nagoya, 460-0001, Japan. 19kondo@meijohosp.com.
Ikuyo
I
Kondo
D000328:Adult; D000077384:Anastrozole; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D001706:Biopsy; D016889:Endometrial Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000601:surgery*; D009570:Nitriles / Q000627:therapeutic use; D018203:Sarcoma, Endometrial Stromal / Q000175:diagnosis* / Q000628:therapy*; D017741:Survivors; D014057:Tomography, X-Ray Computed; D014230:Triazoles / Q000627:therapeutic use; D055815:Young Adult
D018931:Antineoplastic Agents, Hormonal; D009570:Nitriles; D014230:Triazoles; D000077384:Anastrozole