Perfect prognosis of a boy with alveolar rhabdomyosarcoma of the nasal wing treated with brachytherapy and chemotherapy
Rhabdomyosarcoma is the most common soft tissue sarcoma in children and the third most common extracranial solid tumor—affecting about six kids per million each year. For children with the alveolar subtype (ARMS) that has spread to other parts of the body, the prognosis is often grim, especially when the cancer starts in a rare, hard-to-treat site like the nasal wing. But a 2021 study from Beijing Tongren Hospital shows that a combination of targeted radiation and chemotherapy can lead to a near-perfect outcome, even for these high-risk patients.
In 2011, a 29-month-old boy came to doctors with a 10-month history of a growing mass on his left nasal wing. The lump was about 1 cm in diameter—hard, but without redness or pain. He had no nasal congestion, runny nose, or other symptoms. Tests revealed a stark reality: an enhanced MRI showed a soft tissue mass in his left nasal wing, and a bone scan found metastasis to his ninth lumbar vertebra (L9). A biopsy confirmed alveolar rhabdomyosarcoma: tiny, round blue cells on hematoxylin-eosin (H&E) staining, and positive results for desmin and myogenin (proteins that mark muscle tumors). Most critically, a fluorescence in situ hybridization (FISH) test detected the PAX-FOXO1 fusion gene—a genetic hallmark of ARMS present in 75% of cases. There was no spread to lymph nodes, lungs, or bone marrow. The patient’s guardians provided written consent for the use of his clinical information and images in the study.
Doctors faced a dilemma: the nasal wing is too small and cosmetically sensitive for complete surgery, and external beam radiation could damage his developing face or eyes. Instead, they chose a multimodal approach: first, one cycle of chemotherapy to shrink the tumor. Then, they implanted six radioactive 125I seeds directly into the mass—delivering a total dose of 12,000 cGy (each seed had 0.70 mCi of activity). Seven days later, the boy started five more cycles of chemotherapy, alternating between two regimens: one with pirarubicin, vincristine, cyclophosphamide, and cisplatin; another with ifosfamide, actinomycin-D, etoposide, and vincristine.
The results were remarkable. Five years later, an MRI showed no sign of the original tumor, and a whole-body bone scan confirmed the L9 metastasis was gone. The boy’s vision—tested for acuity, cornea health, and retinal function—was normal. His left nasal wing was slightly smaller than the right, but there were no scars or skin darkening. As of September 2019, he’d been in complete remission for over eight years. Today, he’s a happy primary school student with a symmetrical face and no long-term health issues from treatment.
Rhabdomyosarcoma’s rarity and aggression make cases like this stand out. ARMS is the more dangerous subtype: it grows faster, spreads earlier, and is linked to the PAX-FOXO1 fusion gene—a marker for poor survival. About 25% of RMS patients have ARMS, and many already have metastasis when diagnosed (most commonly to the lungs, bones, bone marrow, or lymph nodes). For head and neck RMS (HNRMS), surgery is often off the table because of the risk to appearance or function (like breathing or speaking). External radiation, while effective, can cause lifelong problems: stunted facial growth, vision loss, or abnormal tooth development. Brachytherapy—implanting radioactive seeds directly into the tumor—offers a solution: it delivers high doses to the cancer while sparing nearby healthy tissue, reducing long-term side effects.
Chemotherapy is the backbone of RMS care. Standard drugs include vincristine (a plant alkaloid), actinomycin D (an antibiotic), and alkylating agents like cyclophosphamide or ifosfamide. Newer options, such as etoposide, help tackle high-risk cases with metastasis. For HNRMS, doctors prioritize a “multimodal” approach—combining chemo, radiation, and sometimes limited surgery—to save lives while preserving quality of life. This case is unique because the nasal wing is an extremely rare primary site for ARMS, and metastasis to the spine made it even higher risk. But the combination of 125I brachytherapy (for precise local control) and intensive chemotherapy (to kill systemic cancer) worked.
This boy’s story offers hope for families facing ARMS in hard-to-treat locations. While more research is needed to confirm these results in larger groups, the case shows that targeted radiation (like 125I brachytherapy) paired with chemotherapy can lead to long-term remission—even for kids with metastasis. Most importantly, it highlights the value of personalized, multidisciplinary care: balancing cancer control with the need to protect a child’s growth and well-being.
Original study: Hu HM, Zhang WL, Huang DS, Luo S, Sarang A, Chen XH. Perfect prognosis of a boy with alveolar rhabdomyosarcoma of the nasal wing treated with brachytherapy and chemotherapy. Chinese Medical Journal 2021;134(3). doi: doi.org/10.1097/CM9.0000000000001188
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