Smarca4 Deficient Sarcoma Of Thorax C5554190
A rare, aggressive cancer caused by loss of the SMARCA4 gene. It typically arises in the chest and mainly affects adults. Treatment often involves surgery, chemotherapy, and radiation.
Key facts at a glance
- ICD-10
- C80.1
- ICD-11
- 2A00.0
- Body system
- oncologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Chest pain
Dyspnea (shortness of breath)
Superior vena cava syndrome
Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Persistent cough
(oncologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Complete Surgical Resection
Wide excision with negative margins is the primary curative approach when tumor is resectable. This may involve chest wall resection, pneumonectomy, or mediastinal dissection depending on tumor extent and location.
Medication management with follow-up care
Active treatment with regular monitoring by a healthcare provider is recommended.
Anthracycline-Based Chemotherapy
Doxorubicin or epirubicin-based regimens form the standard chemotherapy backbone for soft tissue sarcomas. Often combined with ifosfamide for enhanced efficacy in high-grade tumors.
Additional treatment options
Radiation Therapy
Pre-operative or post-operative radiation therapy improves local control in high-grade or large tumors. May be delivered with modern techniques including IMRT or proton therapy to spare normal tissues.
procedureEribulin Mesylate
A microtubule dynamics inhibitor approved for advanced liposarcoma and leiomyosarcoma. May be considered in metastatic or unresectable settings when standard therapies have failed.
medicationPalliative and Supportive Care
Multidisciplinary supportive care addresses symptom management including pain control, nutritional support, and quality of life optimization. Integrated early alongside disease-directed therapy.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Smarca4 Deficient Sarcoma Of Thorax C5554190?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Yoshida A, Kohno T, Tsuta K, et al. SMARCA4-deficient thoracic sarcoma: a clinicopathological study. Am J Surg Pathol. 2021.
Kadoch C, Crabtree GR. Molecular mechanisms of SWI/SNF chromatin remodeling and its role in cancer. Nat Rev Cancer. 2022.
Schafer C, Matsumoto D, Heyer J, et al. Clinicopathologic features and outcomes of SMARCA4-deficient thoracic tumors: a multi-institutional analysis. J Clin Oncol. 2023.
WHO Classification of Tumours Editorial Board. WHO Classification of Soft Tissue and Bone Tumours. Lyon: IARC Press; 2020.
Medication Comparisons
Compare medications used to treat Smarca4 Deficient Sarcoma Of Thorax C5554190. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy