Abstract
BackgroundCongenital alveolar rhabdomyosarcoma is an extremely uncommon and invariably fatal tumor with the current therapy. Less than 25% of patients present with evidence of cutaneous metastasis.Case presentationWe report a case of congenital alveolar rhabdomyosarcoma in an 18-month-old male who presented with a progressively increasing mass lesion in the left post-auricular region since birth. Radiological examination did not show any intracranial involvement of the mass lesion. Upon resecting the mass that was 10-cm in largest dimension, the gross, microscopic, and ultrastructural findings were consistent with congenital alveolar rhabdomyosarcoma.ConclusionThe suspicion of alveolar subtype on histological grounds and proper evaluation of this tumor by immunostain and ultrastuctural examination is necessary. In the Medline literature search, there is no report of large congenital alveolar rhabdomyosarcoma in the post-auricle region.
Highlights
ConclusionThe suspicion of alveolar subtype on histological grounds and proper evaluation of this tumor by immunostain and ultrastuctural examination is necessary
Congenital alveolar rhabdomyosarcoma is an extremely uncommon and invariably fatal tumor with the current therapy
In the Medline literature search, there is no report of large congenital alveolar rhabdomyosarcoma in the post-auricle region
Summary
Our case exemplifies the rare sub-type of congenital RMS occurring in the post-auricular area. This is a highly malignant tumor with no record of long-term survivors. In this setting, clinical outcome and radiological findings must be accounted for by both pathologists and clinicians who must be aware of such an unusual presentation of congenital rhabdomyosarcoma, whose diagnosis requires representative tissue sampling and whose treatment may require an adjustment of drug dosage
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