概述
Malignant fibrous histiocytoma (MFH), now more commonly called undifferentiated pleomorphic sarcoma (UPS), is a high-grade soft tissue sarcoma. It typically arises in the limbs or retroperitoneum of older adults and is known for aggressive behavior.
症状
- Painless, enlarging mass
- Swelling in arms, legs, or abdomen
- Pain if tumor compresses nerves or organs
- Fatigue or weight loss in advanced cases
病因与风险因素
- Prior radiation therapy
- Genetic predisposition (e.g., Li-Fraumeni syndrome)
- Chronic lymphedema
- Older age (>50 years)
诊断方法
- Physical exam and MRI/CT scan
- Core needle biopsy with histologic grading
- Chest imaging to check for lung metastases
- Pathology: pleomorphic spindle cells, no distinct line of differentiation
治疗方案
- Wide surgical excision (standard of care)
- Radiation therapy (pre- or post-op to improve local control)
- Chemotherapy (e.g., doxorubicin/ifosfamide for high-grade disease)
- Clinical trials for advanced/metastatic cases
预后情况
- Localized UPS 5-year survival: ~50–70%
- High recurrence risk if not completely excised
- Metastasis occurs in ~30–40%, usually to lungs
与此类癌症共处
- Post-surgical rehabilitation and mobility support
- Surveillance imaging every 3–6 months initially
- Pain and symptom management for metastatic disease
- Emotional and palliative support
预防与筛查
- No standard screening methods
- Avoid unnecessary radiation exposure
- Monitor irradiated fields and chronic lymphedema sites
FAQs
Q: Is MFH still used as a diagnosis?
A: MFH is now classified as undifferentiated pleomorphic sarcoma (UPS), a diagnosis of exclusion.
Q: Can it be cured?
A: Yes, especially if detected early and surgically removed with clear margins.
Q: Does it spread?
A: Yes. Common metastasis site is the lungs.
相关资源
- Sarcoma Alliance
- National Cancer Institute
- ClinicalTrials.gov
