One-Step Lower Leg Reconstruction with Vascularized Functional Vastus Lateralis Muscle Flap in the Treatment of Embryonal Rhabdomyosarcoma for a Six-Month-Old Boy: A Case Report.

Dzintars Ozols,Aigars Petersons,Marika Grutupa,Janis Krasts,Marisa Maija Berezovska

doi:10.3390/medicina56070362

Abstract

Rhabdomyosarcoma (RMS) is a common soft tissue sarcoma in childhood, however, it is very rare in the neonatal period (0.4–2% of cases). This case depicts a boy, who presented with RMS at two weeks of age, but officially diagnosed at the age of three months. MRI and scintigraphy determined a soft tissue tumor in the soleus muscle, while biopsy confirmed embryonal RMS with high mitotic activity (Ki67 (monoclonal antibodies) ~80%). CWS (Cooperative Weichteilsarkom Studiengruppe)-2012 with I2VA (ifosfamide, vincristine, actinomycin) chemotherapy regimen was administered per protocol. Surgical treatment was performed at age of six months and 18 days. The operation consisted of radical tumor resection and total triceps surae with partial fibula resection. Immediate reconstruction of triceps muscle was accomplished using a vascularized functional musculocutaneous vastus lateralis flap. Functional outcome was measured using the Lower Extremity Functional Scale (LEFS) and the Foot and Ankle Outcome Score (FAOS) with the results of 92.5% and 99% respectively.

Highlights

Pediatric soft tissue sarcomas are part of a heterogeneous group of tumors originating from embryonic mesodermal tissues during the process of differentiation into various mesenchymal tissue components of the human body
These tumors constitute 6% to 8% of all cancers in children less than 15 years of age [1], it is very rare in the neonatal period as its incidence is 0.4–2% of cases with male predisposition [2]
Treatment recommendations for limb RMS combine chemotherapy and surgical tumor removal with secondary functional reconstruction in comparison with previous recommendations consisting of radical excision, primary or secondary amputation combined with irradiation therapy [8]

Summary

Introduction

Pediatric soft tissue sarcomas are part of a heterogeneous group of tumors originating from embryonic mesodermal tissues during the process of differentiation into various mesenchymal tissue components of the human body. Operation started with a longitudinal incision of the lower leg to secure a wide opening and tibial artery were preserved, while two gastrocnemius motor branches were dissected for functional good visualization for the radical tumor resection. The MRC scale of posterior compartment muscle strength for the movement of the talocrural joint scored 4–5 points as strength was little bit were interpret as postoperative scaring tissues associate with postoperative hematoma. The patient received nine rounds of chemotherapy He completed treatment with port removal at the age of eight months and 23 days (327 days old). Sensation innervation at the peronealmuscles and tibial nerve zones of the of talocrural joint scored points and deep posterior compartment muscles scored toe foot were intact and the patient reacted to the pain using a two-point discriminatorypoints test. Riga Stradins University Ethics committee Nr.6-3/2/47 and ICF (informed consent form) was signed by his parents before treatment

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