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.
