Embryonal rhabdomyosarcoma of the orbit in a liver transplant recipient

Abstract

Although an increased incidence of de novo malignancies is reported in transplant recipients, rhabdomyosarcoma, an aggressive mesenchymal tumor typical of childhood, is not considered a neoplasm commonly related to immunosuppression. A 21-year-old woman presented with unilateral diplopia and proptosis 16 months after liver transplantation for fulminant hepatic failure. A tumoral mass originating from the medial rectus muscle was partially removed and diagnosed as being an embryonal rhabdomyosarcoma. Since the patient refused complete orbital excision, one course of radiotherapy and six courses of chemotherapy were administered, while immunosuppression was re-modulated, without interruption of the administration of cyclosporine. Complete control of tumor growth was achieved, while no alterations of graft function were observed throughout the treatment period.

Sarcomas other than Kaposi sarcoma occurring in immunodeficiency

“…Three of these rhabdomyosarcoma cases occurred in children, but both cases in transplant recipients were adults, one a 21 year-old female liver recipient [125] and the other a 67 year-old male kidney recipient [126]. Only a limited number of other sarcomas were described in the case literature (Table 1) [127–152].…”

Rhabdomyosarcoma in a child with nephrotic syndrome treated with cyclosporine: a case report with literature review

“…A 15-year-old boy, treated with CsA to prevent kidney transplant rejection, developed RMS of the nasopharynx [13]. The tumors of our patient and the patient of Cescon et al [11] were of the embryonal histology, which is the most common subtype. Mycophenolate mofetil (MMF), an ester prodrug of mycophenolic acid (MPA), is known as an FDA-approved immunosuppression agent and exists the function of anti-tumor activity, which mainly acts on inosine monophosphate dehydrogenase (IMDPH) to treat with immune-related adverse events effectively [15,16].…”

“…It is reported effective and safe in treatment of that condition to avoid the toxicity of corticosteroids [10]. Nevertheless, because of cyclosporine's risk in a wide variety of cancers, including RMS [6,7,[11][12][13], even an isolated case of RMS in a child with nephrotic syndrome should prompt caution about its use in that population.…”

“…The present study expanded this finding and further showed that CsA-related RMS has been described only in case reports ( Table 1). Cescon et al [11] reported a 23-yearold girl who received CsA for liver transplantation immunosuppression and later developed orbital embryonal RMS. A 47-year-old Japanese man, who received CsA for treatment of Behçet's disease, developed a malignant rhabdoid tumor in his posterior femoral region 3 years later [12].…”