Primary Intra-Abdominal Rhabdomyosarcoma in an Adult: an Unusual Presentation and Review of Literature

Abstract

Rhabdomyosarcomas, the commonest soft tissue sarcoma in paediatric age group, represent 5-10 % of all solid tumors in childhood. These tumors are rare in adult population. There are sporadic case reports of intra-abdominal rhabdomyosarcoma, but mostly in paediatric age group. Here we are reporting an unsual case of intra abdominal rhabdomyosarcoma in an elderly which presented with acute intestinal obstruction. CECT abdomen showed a solid mass in retroperitoneum compressing adjacent bowel loops. Upon emergency exploratory laparotomy a hard mass was found to be compressing the ascending colon and infiltrating into duodenum, pancreas and liver. No specific organ of origin could be identified. Biopsy was taken, as the mass was unresectable and ileostomy was done. Histopthological examination revealed embryonal rhabdomyosarcoma. Rarity of this disease and unusual presentation prompted us to report this case.

Adult omental alveolar rhabdomyosarcoma: An unusual site. A case report and literature review

“…Soft tissue sarcomas (STSs) are an unusual finding, mainly affecting the pediatric population [ 1 ]. Rhabdomyosarcomas account for over half of them [ 2 ]. This percentage drops significantly in adults where it doesn't exceed 2–5% [ 3 ].…”

“…Rhabdomyosarcoma is believed to originate from any immature mesenchymal cells, regardless of what it was going to become (a muscle, cartilage, bone, etc …) [ 2 ].…”

“…Stout et al, in 1946 were the first to classify Rhabdomyosarcoma tumors [ 6 ]. However, in 1956 Horn et al proposed the widely-used classification which comprises the embryonal, botryoid (a subtype of embryonal), alveolar (20% of cases and the worst prognosis [ 5 ]) and pleomorphic types [ 2 ]. WHO classifies this tumor into embryonal, alveolar, pleomorphic, and spindle cell/sclerosing RMS [ 1 ].…”

Primary alveolar rhabdomyosarcoma of retrorectal-presacral space in an adult patient

“…The most common primary site in adults is the extremities, and the head and neck parameningeal region is the most common site in children. [ 11 ] Available data on the extremely infrequent localisations of adulthood, such as larynx, [ 12 ] renal pelvis, [ 13 ] subcutaneous tissue, [ 8 ] uterine, [ 14 ] retroperitoneal space, [ 7 ] and so on, are insufficient and scarce, mostly presented as case reports or single institution experiences with small numbers of patients. In fact, our patient is the first single-case report of ARMS arising from the retrorectal-presacral space in an adult male, within our thorough investigation.…”

Embryonal rhabdomyosarcoma within abdomen and pelvis in an adult

“…The second case described that a 65–year-old male patient was diagnosed as intra-abdominal RMS with a clear margin and solid appearance. 5 Both cases were classified as embryonal RMS.…”