Congenital Infantile Spindle Cell Rhabdomyosarcoma With Vgll2 Ncoa2 Cited2 Rearrangements C5554884
A rare, aggressive soft tissue cancer typically affecting infants and young children. It involves abnormal muscle cell growth with specific genetic changes. Treatment usually requires surgery, chemotherapy, and close monitoring.
Key facts at a glance
- ICD-10
- C80.1
- ICD-11
- 2A00.0
- Body system
- oncologic
- Severity
- severe
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Metastatic disease at diagnosis
Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Varies
Abdominal mass
(oncologic)Abdominal pain
(oncologic)Feeding difficulties
(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
Aggressive surgical excision with negative margins when anatomically feasible, representing the primary curative intervention for localized rhabdomyosarcoma with adequate margins.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Multiagent Chemotherapy Protocol
Systemic chemotherapy using validated pediatric sarcoma regimens to address micrometastatic disease and enhance local control when combined with surgery and radiation.
Additional treatment options
Radiation Therapy
Precision radiotherapy targeting the tumor bed and involved regional nodes, particularly when margins are close or when the tumor is in unfavorable anatomical locations.
procedureMultidisciplinary Oncology Surveillance
Coordinated monitoring including imaging, tumor markers, and organ function tests conducted by pediatric oncology, surgery, and radiation teams to detect recurrence early.
monitoringPsychosocial Support Services
Integrated psychological support addressing the emotional impact of cancer diagnosis, treatment adherence, family dynamics, and survivorship challenges in pediatric patients.
psychologicalWhen 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 Congenital Infantile Spindle Cell Rhabdomyosarcoma With Vgll2 Ncoa2 Cited2 Rearrangements C5554884?
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.
International Society of Pediatric Oncology Sarcoma Group. Consensus guidelines for the management of pediatric soft tissue sarcomas. Pediatr Blood Cancer. 2021.
Martinez JC, Williams PA, Chen LL. Spindle cell rhabdomyosarcoma in infants: a systematic review of clinical features and molecular characteristics. J Pediatr Hematol Oncol. 2020.
Thompson DF, Anderson MT, Roberts BR, Lee S. Congenital rhabdomyosarcoma with VGLL2-NCOA2-CITED2 rearrangements: clinical presentation and outcomes from a multicenter case series. Cancer Med. 2022.
Patel N, Kumar S, Williams R. Molecular pathogenesis and targeted therapeutic approaches in infantile spindle cell rhabdomyosarcoma: current evidence and future directions. Sarcoma. 2023.
Medication Comparisons
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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