Perfect Prognosis of a Boy with Alveolar Rhabdomyosarcoma of the Nasal Wing Treated with Brachytherapy and Chemotherapy
Alveolar rhabdomyosarcoma (ARMS) is a rare and aggressive form of soft tissue sarcoma that predominantly affects children and adolescents. It is characterized by the presence of small round blue cells and is often associated with distant metastasis at the time of diagnosis, which significantly worsens the prognosis. The nasal wing is an exceedingly rare primary site for ARMS, and achieving complete surgical excision in this location is particularly challenging due to functional and cosmetic constraints. This case report presents the successful multidisciplinary management of a 29-month-old boy with ARMS of the left nasal wing accompanied by bone metastasis, treated with systemic chemotherapy and 125I interstitial brachytherapy, resulting in a perfect prognosis with minimal long-term side effects.
The patient, a 29-month-old boy, presented with a 10-month history of a gradually increasing mass in the left nasal wing. The mass was palpable, measuring approximately 1 cm in diameter, and had a hard texture. There were no associated symptoms such as rhinorrhea or nasal congestion, and the localized skin showed no signs of inflammation. Enhanced magnetic resonance imaging (MRI) of the paranasal sinuses revealed a soft tissue density shadow in the left nasal wing, extending from the subcutaneous left nasal root to the left nasal wing, measuring 12 x 17 mm. A whole-body bone scan indicated metastasis to the ninth lumbar vertebra. A biopsy of the left nasal alar tumor confirmed the diagnosis of ARMS, supported by positive immunohistochemical staining for desmin and myogenin, and hematoxylin-eosin staining revealed small round blue cells. Fluorescence in situ hybridization (FISH) confirmed the presence of the PAX3-FOXO1 fusion gene, a hallmark of ARMS.
Given the primary site and the presence of bone metastasis, a treatment strategy combining systemic chemotherapy and 125I interstitial brachytherapy was proposed. The patient initially received one cycle of chemotherapy, followed by the implantation of six 125I seeds into the local mass. Each seed had an activity of 0.70 mCi, and the total particle dose was 12000.0 cGy. Radioactivity detection indicated that the maximum dose from the six seeds was 62761.5 cGy. Seven days after the brachytherapy, five additional cycles of chemotherapy were administered. The chemotherapy regimens alternated between two schemes: the first included pirarubicin (25 mg/m2, days 2 and 9), vincristine (1.5 mg/m2, days 1 and 8), cyclophosphamide (300 mg/m2, days 1-3), and cisplatin (90 mg/m2, day 1); the second consisted of ifosfamide (1.5 g/m2, days 1-5), actinomycin-D (300 mg/m2, days 1-5), etoposide (100 mg/m2, days 1-3), and vincristine (1.5 mg/m2, day 1).
The multidisciplinary treatment approach yielded remarkable results. Post-treatment imaging showed no evidence of the mass in the left nasal wing, and the metastasis to the ninth lumbar vertebra had completely resolved. At the five-year follow-up, enhanced MRI of the nasal sinuses confirmed local control of the left nasal wing, and a whole-body bone scan revealed no signs of metastasis in the ninth lumbar vertebra. The patient’s visual acuity, cornea, lens, fundus, visual electrophysiology, and tear function tests were all within normal limits. Nasal acoustic reflection and nasal resistance tests indicated slightly higher resistance in the left nasal cavity compared to the right, but the face and nasolabial folds were symmetrical. As of September 2019, the boy had been in complete remission for 8 years and 9 months, with normal development of the teeth and alveolar bone. The left nasal wing was slightly smaller than the right, but there was no scar formation or pigmentation. The patient was attending primary school and had an excellent quality of life.
ARMS is a highly aggressive subtype of rhabdomyosarcoma, accounting for approximately 25% of all cases. It is characterized by the presence of the PAX3-FOXO1 or PAX7-FOXO1 fusion genes, which are detected in 75% of ARMS cases and are associated with a poorer prognosis. Distant metastasis at the time of diagnosis is a significant risk factor, and ARMS commonly spreads to the lungs, bones, bone marrow, and distal lymph nodes. The treatment of rhabdomyosarcoma typically involves a multimodal approach, including surgery, radiotherapy, and chemotherapy. However, in cases of head and neck rhabdomyosarcoma (HNRMS), complete surgical excision is often not feasible due to the risk of functional and cosmetic impairments. Radiotherapy, including external beam radiotherapy and brachytherapy, plays a crucial role in local control, but external radiotherapy can cause long-term toxic side effects, such as facial development abnormalities, visual impairments, and alveolar bone development issues. Brachytherapy, on the other hand, has been shown to improve prognosis and quality of life with fewer side effects, making it a valuable option for select patients.
Chemotherapy is a cornerstone of rhabdomyosarcoma treatment, with vincristine, actinomycin D, and alkylating agents (cyclophosphamide or ifosfamide) being the most commonly used drugs. Recent advancements have introduced newer chemotherapeutic agents, such as etoposide, carboplatin, and epirubicin, which have shown promise in treating distant metastasis cases. Intensive multidrug therapy is often necessary for patients with metastatic disease.
The successful outcome in this case highlights the importance of a multidisciplinary approach in the management of ARMS, particularly in challenging locations such as the nasal wing. The combination of 125I interstitial brachytherapy and systemic chemotherapy not only achieved complete remission but also minimized long-term side effects, preserving the patient’s quality of life. This case underscores the potential of brachytherapy as an effective treatment modality for HNRMS, especially in pediatric patients, where preserving form and function is paramount.
In conclusion, the management of ARMS of the nasal wing with distant metastasis requires a tailored, multidisciplinary approach. The use of 125I interstitial brachytherapy in conjunction with systemic chemotherapy can result in a perfect prognosis with minimal long-term side effects, as demonstrated in this case. Further research and clinical trials are needed to explore the potential of brachytherapy in the treatment of pediatric rhabdomyosarcoma and to optimize treatment protocols for improved outcomes.
doi.org/10.1097/CM9.0000000000001188
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