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Primary alveolar rhabdomyosarcoma: a horrendous presentation with miraculous remission
  1. Anil Kumar Dhull
  1. Department of Radiation Oncology, Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India
  1. Correspondence to Dr Anil Kumar Dhull, anildhull{at}sify.com

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Description

Alveolar rhabdomyosarcoma constitutes approximately 1% of all paediatric tumours. Primary alveolar rhabdomyosarcoma of lip is a very rare pathological entity.1 Here is presented a case of primary alveolar rhabdomyosarcoma of upper lip treated with palliative external radiation therapy followed by combination chemotherapy.

A 5-year-old girl presented with painful swelling of upper lip of 3 months duration, which progressed rapidly. There was no history of other swelling. There was no history of any malignancy in the family. Local examination revealed an ulcero-proliferative growth of 10×8 cm size present over the upper lip involving bilateral nostrils and was locally advanced, causing difficulty in swallowing and respiration (figures 1 and 2). Histopathological examination confirmed the diagnosis of alveolar rhabdomyosarcoma.

Figure 1

Anterior view showing a locally advanced growth over the upper lip involving bilateral nostrils.

Figure 2

Lateral view of the growth.

The poor general condition of the patient as well as obstructive symptoms prompted us to treat the patient with palliative intention. The patient received palliative external radiation therapy and showed partial regression of the swelling after radiotherapy. The patient was further planned for intravenous combination chemotherapy with vincristine 1 mg, actinomycin 0.5 mg and cyclophosphamide 400 mg 3 weekly for six cycles and after 6 months of the initial presentation, the patient was in complete remission as is evident from the serial photographs (figures 35). Malignancy at this rare site is uncommon and clinical management presents considerable challenges.1 Intensive multimodality approach should be tested, incorporating surgery, dose-intensive combination chemotherapy and radiation therapy.2 Future challenges include the development of less toxic therapy for patient with localised disease and new approaches for patients with metastatic disease.

Figure 3

Clinical photograph at the end of palliative radiotherapy.

Figure 4

Post-treatment photograph after 2 months of completion of radiotherapy.

Figure 5

Post-treatment photograph showing complete remission after 6 months of completion of radiotherapy and six cycles of chemotherapy.

Learning points

  • Primary alveolar rhabdomyosarcoma of the lip is a rare pathological entity and usually present in the aggressive form.

  • External radiotherapy and adjuvant combination chemotherapy with vincristine, actinomycin and cyclophosphamide is very effective treatment protocol.

  • Intensive multi-modality approach for treatment is recommended as the results are miraculous and complete remission of the disease is possible.

References

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Footnotes

  • Competing interests None.

  • Patient consent Obtained.

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