Long-term follow-up reports of low-grade
endometrial stromal sarcoma (LGESS) including its
clinical course and pathological data are rare. We previously reported the case of a Japanese woman diagnosed with LGESS, who was treated with multidisciplinary
therapy. She had been suffering from uterine cervical
tumor diagnosed as cervical
polyps, or
fibroid in statu nascendi, since 24 years old. The patient had survived for 25 years with the disease. This report presents her progress and pathological change since the previous report.
CASE PRESENTATION: At age 45, the patient experienced a relapse of the remnant LGESS
tumor between the right diaphragm and liver. Although
chemotherapy was not effective, the
tumor was eliminated by
proton therapy. At age 46 years, the remnant
tumors outside the irradiated field were resected. The disease was originally diagnosed as "
neuroendocrine carcinoma (NEC)" using the surgical specimen. Therefore,
cisplatin and
irinotecan combination chemotherapy were administered to treat the remnant dissemination. After 4 cycles of
chemotherapy, the liver
metastases had enlarged and were resected surgically. Consequently, no remnant
tumor was visible in the abdominal cavity at the end of the surgery. To determine the origin of NEC, we examined the previously resected specimens obtained from her ileum at age 40 years. A boundary between the LGESS and
neuroendocrine tumor grade 2 (NET G2)-like lesion was found in the
tumor, indicating that the origin of these
tumors was LGESS. After less than 2 years of
chemotherapy and undergoing surgery, a relapse of the
tumor in the liver induced biliary duct obstruction with
jaundice, which was treated with endoscopic retrograde biliary drainage. Although
pazopanib prolonged her life for 10 months, she died from
sepsis at age 49 years, which was caused by the
infection that spread to the liver metastatic
tumor via the stented biliary ducts. Autopsy revealed
adenocarcinoma-like differentiation of the
tumor.
CONCLUSION: This LGESS patient has survived for a long time owing to multidisciplinary treatment including
proton therapy. The LGESS
tumor differentiated to NET G2-like tissue and then further to
adenocarcinoma-like tissue during the long-term follow-up.