Peritoneal Leiomyosarcoma in a Kidney Transplant Patient: A Case Report

Peritoneal Leiomyosarcoma in a Kidney Transplant Patient: A Case Report N. Cautero, S. De Luca, A. Vecchi, P. Garelli, D. Nicolini, G. Martorelli, G.M. Frascà, G. Gaffi, D. Taruscia, I. Bearzi, G. Adani, and A. Risaliti ABSTRACT Sarcomas are rare neoplasms, accounting for a 1.7% incidence among all transplanted patients presenting with de novo malignancies. Our present report focused on a 46-yearold woman who received immunosuppressive therapy based on cyclosporine and steroids for renal transplantation. Eight years after transplantations, she suffered lower abdominal pain and a mass involving peritoneal soft tissues was located near the right iliac vessels. Upon radical tumor excision, the histological examination revealed a high-grade leiomyosarcoma. Immunosuppression was reduced and cyclosporine switched to rapamycin. After 30 days, a computed tomography scan revealed two small pulmonary metastases, so the patient received adriamycin. Six months after the diagnosis, there was no intraabdominal relapse and the pulmonary metastasis remain stable. The function of the transplanted kidney was normal and the patient was listed for laparoscopic pulmonary resection. Sarcomas in solid organ transplant patients appear to have aggressive features with 62% being high grade and 40% metastatic at the time of primary diagnosis with a recurrence rate of 30% and a 5-year survival rate of 25%. Patients diagnosed with sarcoma should be treated with multimodality therapy. After aggressive surgery whenever possible, a combination of a traditional cytotoxic drug and a “signal” blocking agent like rapamycin may increase selectivity toward tumor cells.

S

ARCOMAS OF SOFT TISSUES are rare tumors accounting for approximately 1% of all adult malignancies. These neoplasms have rarely been reported in the transplant literature with an incidence of 1.7% among all de novo malignancies.1 Chronic immunosuppression is responsible for an increased incidence of de novo tumors and although not well understood, increases the relative risk of soft tissue sarcomas in allograft recipients. Our report focused on our experience of a case of leiomyosarcoma in a kidney transplant recipient. MATERIAL AND METHODS A 46-year-old woman received immunosuppressive therapy based on cyclosporine and steroids for renal transplantation. Eight years after transplantation, she was admitted with lower abdominal pain and had a palpable mass located near the transplanted kidney. Graft function was normal; a thoracoabdominal computed tomography (CT) scan demonstrated a solid tumor of 7 ⫻ 4.9 cm involving soft tissues near the right iliac vessels (Fig 1) without further lesions in other body districts. The patient underwent surgical exploration with radical excision of the mass connected to the mesenteric root of last ileal loop and

cecum. The tumor tissue was processed for routine histology and immunohistochemical stains. The histological features and staining profiles were consistent with a high-grade leiomyosarcoma. Serum and tissue specimens were negative for Epstein-Barr infection. The clinical course was uneventful and the immunosuppressive regimen modified by a switch to sirolimus.

RESULTS

Thirty days later, a CT scan revealed two small pulmonary metastases so the patient received adjuvant chemotherapy From the Chirurgia Epatobiliopancreatica e dei Trapianti di Fegato (N.C., S.D.L., A.V., P.G., D.N., G.M., A.R.), Rene e Pancreas; U.O. di Nefrologia e Dialisi (G.M.F., G.G., D.T.), Anatomia ed Istologia Patologica (I.B.), Azienda OspedalieroUniversitaria “Ospedali Riuniti,” Ancona, and Centro Trapianti Fegato (G.A.), Rene e Pancreas, Clinica Chirurgica, Università degli Studi di Udine, Udine, Italy. Address reprint requests to Nicola Cautero, MD, PhD, Chirurgia Epatobiliopancreatica e dei Trapianti di Fegato, Rene e Pancreas, Azienda Ospedaliero-Universitaria “Ospedali Riuniti” Ancona, Italy. E-mail: [email protected]

0041-1345/07/$–see front matter doi:10.1016/j.transproceed.2007.05.074

© 2007 by Elsevier Inc. All rights reserved. 360 Park Avenue South, New York, NY 10010-1710

2038

Transplantation Proceedings, 39, 2038 –2039 (2007)

PERITONEAL LEIOMYOSARCOMA

2039

DISCUSSION

Sarcomas in solid organ transplantation appear to have an aggressive pattern, with 62% being high grade and 40% metastatic at the time of a primary diagnosis. The recurrence rate is 30% and the 5-year survival rate, 25%. High histological grade is the strongest predictable; factor for 5-year survival and recurrence ratio.1,2 These findings suggest that transplant-related sarcomas are more aggressive than in nontransplanted patients and deserve increased attention. Patients with newly diagnosed sarcomas should be treated with multimodality therapy. The cornerstone of treatment is represented, whenever possible, by wide local excision followed by a combination of a traditional cytotoxic drug and a signal blocking agent like rapamycin that a increase selectivity toward tumor cells.3,4 REFERENCES Fig 1. Abdominal CT scan showing a mass located near right iliac vessels beside transplanted kidney.

with epirubicin (interrupted for general toxicity) followed by adriamycin. Six months after primary diagnosis, there was no intra-abdominal relapse and pulmonary metastases remain stable. The function of the transplanted kidney was normal and the patient was listed for laparoscopic pulmonary resection.

1. Husted TL, Buell JF, Hanaway MJ, et al: De novo sarcomas in solid organ transplant recipients. Transplant Proc 34: 1786, 2002 2. Penn I: Sarcomas in allograft recipients. Transplantation 60:1485, 1995 3. Merimsky O: Targeting metastatic leiomyosarcoma by rapamycin plus gemcitabine: an intriguing clinical observation. Int J Mol Med 14:931, 2004 4. Tahri A, Noel G, Figuerella-Branger D, et al: Epstein-Barr virus associated central nervous system leiomyosarcoma occurring after renal transplantation: case report and review of the literature. Cancer Radiother 7:308, 2003