High-grade endometrial stromal sarcoma presenting with multiple brain metastases: a case report

Area of Science:

• Gynecologic Oncology
• Neuropathology
• Oncologic Imaging

Background:

• High-grade endometrial stromal sarcoma (HGESS) is a rare, aggressive uterine malignancy with a poor prognosis.
• Typical symptoms include abnormal vaginal bleeding and abdominal discomfort.
• Initial presentation with distant metastases, especially to the brain, is exceptionally uncommon.

Purpose of the Study:

• To report a unique case of HGESS presenting initially with neurological symptoms due to brain metastases.
• To highlight the diagnostic challenges and importance of considering HGESS in the differential diagnosis of brain metastases.

Main Methods:

• Detailed clinical case presentation of a 58-year-old woman with HGESS.
• Diagnostic workup included cranial CT, craniotomy, tumor resection, PET-CT, and CT-guided endometrial biopsy.
• Pathological and imaging findings were analyzed, including potential origin from adenomyosis.

Main Results:

• The patient presented with left hemiparesis and weight loss, found to have multiple brain tumors.
• Uterus identified as the primary site; HGESS confirmed via CT-guided biopsy after initial negative results.
• Rapid deterioration and death occurred within 4 months despite chemotherapy and radiosurgery.

Conclusions:

• Atypical presentation with neurological symptoms significantly delayed diagnosis.
• Clinicians must maintain a high index of suspicion for HGESS in patients with brain metastases, irrespective of gynecological symptoms.
• This case underscores the aggressive nature and poor prognosis of HGESS presenting with metastatic disease.