# Case report: A left forearm mass with eccentric intramedullary ulnar destruction diagnosed as alveolar rhabdomyosarcoma and treated by wide resection and free vascularized fibular graft

**Authors:** Chenyu Yang, Xinjia Wang, Huaitai Lin, Jinhao Zhu, Zijian Xu, Weidong Wang

PMC · DOI: 10.3389/fonc.2024.1395233 · Frontiers in Oncology · 2024-05-10

## TL;DR

A 17-year-old with a rare aggressive tumor in his forearm was successfully treated with surgery and chemotherapy, preserving function and preventing recurrence.

## Contribution

The paper presents a novel surgical approach using a vascularized fibular graft for bone reconstruction in alveolar rhabdomyosarcoma with bone invasion.

## Key findings

- Wide resection and vascularized fibular graft preserved forearm function without tumor recurrence.
- Multidisciplinary treatment combining chemotherapy and surgery is effective for aggressive bone-invasive tumors.
- Careful surgical planning is critical for managing tumor boundaries and musculoskeletal reconstruction.

## Abstract

Alveolar Rhabdomyosarcoma is a profoundly malignant soft-tissue sarcoma that predominantly affects children and adolescents. However, the medical field lacks consensus regarding the optimal surgical approach to be undertaken in cases where this tumor causes local bone destruction in the upper limb.

A 17-year-old male presented a mass in his left forearm and CT and MRI indicated that the mass had penetrated the ulnar cortex and infiltrating the medulla, resulting in the formation of an eccentric trans-ventricular tumor focus. The sizable tumor affected the volar muscles of the forearm as well as the ulnar bone marrow, exerting pressure on the ulnar artery and vein. It was confirmed by needle biopsy that the mass is alveolar rhabdomyosarcoma. Following two courses of neoadjuvant chemotherapy, the tumor was widely excised en bloc. Autologous fibula with a vascular pedicle was utilized for reconstruction during the procedure. In the postoperative follow-up, no local recurrence of the tumor was observed. Furthermore, the patient retained satisfactory wrist flexion and pronation function in the left forearm.

Alveolar rhabdomyosarcoma is an uncommon and highly aggressive form of soft tissue sarcoma. Scientific management necessitates a multidisciplinary approach, combining chemotherapy with surgery. In cases where the tumor invaded into compartment of the bone, careful consideration should be given to the boundaries of tumor resection, the extent of osteotomy, and the approach to musculoskeletal reconstruction when designing the surgical plan. Through reporting our own case and thoroughly reviewing previous clinical experiences, we aim to provide valuable insights for the treatment of this particular disease.

## Linked entities

- **Diseases:** Alveolar rhabdomyosarcoma (MONDO:0009994)

## Full-text entities

- **Diseases:** bone destruction (MESH:D001847), tumor (MESH:D009369), Alveolar Rhabdomyosarcoma (MESH:D018232), ventricular tumor (MESH:D002551), soft tissue sarcoma (MESH:D012509), forearm mass (MESH:C536030)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11116586/full.md

## References

18 references — full list in the complete paper: https://tomesphere.com/paper/PMC11116586/full.md

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Source: https://tomesphere.com/paper/PMC11116586