Multimodal therapy of an embryonal rhabdomyosarcoma of the paranasal sinuses

Introduction:

The embryonal rhabdomyosarcoma is one of the most common mesenchymal malignancies in children. Often affected are the head, neck as well as the urogenital system. The tumor entity is quite rare in adults.

There are various therapeutic modalities available such as chemotherapy, surgery and radiotherapy.

Depending on the size and location of the primary tumor as well as the histological category and patient age an individual treatment concept is chosen.

Case presentation:

A 28-year-old female patient presented with anosmia and ageusia due to nasal obstruction. Clinically, obstruction of the right nasal cavity was indicated caused by suspicious, tumorous tissue. The clinical examination also revealed a right-sided exopthalmus and a deforming swelling of the right cheek.

Imaging showed a mass in the right paranasal sinuses infiltrating adjacent orbital and intracerebral structures. The pathological result of the biopsy showed an embryonal rhabdomyosarcoma.

According to the CWS-2002-protocol, the patient was classified into the high-risk group. Therefore, a neoadjuvant chemotherapy following the VIAA-III-regimen using ifosfamide, vincristine and adriamycin was initiated. After three cycles of chemotherapy the residual tumor was resected performing an orbital exenteration via lateral rhinotomy. Consolidating radiotherapy and chemotherapy followed. Finally, an epithesis was adapted.

One year after the end of therapy clinical examination as well as imaging showed no recurrence.

Discussion:

Embryonal rhabdomyosarcomas are rare malignancies in adults. Due to the poor prognosis in the case presented, an aggressive multimodal therapy with neoadjuvant chemotherapy, radical resection of the residual tumor and consolidation therapy was performed.

Publication History

Publication Date:
23 April 2019 (online)