Primary Pleomorphic Omental Rhabdomyosarcoma in an Adult: A Report of a Unique Case

Abstract

Rhabdomyosarcoma (RMS) is a very rare, highly malignant neoplasm thought to originate from the pluripotent mesenchymal tissue. Predominantly diagnosed among children and teenagers, however they can also be encountered in adults. There are a few risk factors associated with RMS like family history of malignancy and genetic syndromes like neurofibromatosis type 1, Li-Fraumeni syndrome, Noonan syndrome and Beckwith-Wiedemann syndrome; however, most cases of RMS are sporadic. Other factors like radiotherapy for other malignancy and pre-natal radiation exposure also are associated with increased risk of developing RMS. The most common reported sites for RMS are head, neck, trunk, pelvis and lower limbs. Omental involvement of primary RMS has been rarely reported in the literature. Principally, the survival of treated RMS cases has improved, primarily due to multidisciplinary management approaches. In this paper, we report a case of primary pleomorphic RMS in a 50-year-old female who presented with abdominal pain.

Giant right hydronephrosis with underlying double malignancy: a case report

“…In our case, the pleomorphic rhabdomyosarcoma expressed Desmin positivity, which is the commonest IHC marker positivity seen in any RMS. Other IHCs done were PanCK and SMA, which came out to be negative [ 13 ]. After nephrectomy, adjuvant chemotherapy with vincristine, dactinomycin, and cyclophosphamide should be started for rhabdomyosarcoma cases.…”