A case of a primary smooth muscle tumor arising in a liver allograft is presented. This complication has not been previously reported in the literature. The tumor was diagnosed while still asymptomatic, and the patient, a young child, has done well following a left lateral segmentectomy

Doyle, H

Starzl, TE

Tzakis, AG

Yunis, E

PubMed

Name not available

Smooth muscle tumor arising de novo in a liver allograft: A case report

D-Scholarship@Pitt

\. AFSENDT 2 9 OKT. 1pD~:g A 31680/90-35 Smooth muscle tumor arising de liver allograft: A case report novo 111 a I kS I ( Doyle H, Tzakis AG, Yunis E, Starzl TE. Smooth muscle tumor arising de novo in a liver allograft. A case report. Howard Dovle, Andreas G. Tzalds, Eduardo Yunls and Thomas E. Starzl Departments 01 Surgery and Pathology. UrWer-sity Health Center 01 Pittsburgh. University 01 Pittsburgh. and the Veterans Administration Medical Center. Pittsburgh, Pennsylvania. USA Clin Transplantation 1990: 4: 000-000. Introduction The development of de novo malignancies is a well-recognized complication of the immunosuppressed state that follows organ transplantation (1-6). In the cyclosporine era, lymphomas, skin cancer and Kaposi's sarcoma account for more than half of the reported cases (6). Other tumors of mesenchymal origin are uncommon. We describe a case of a primary smooth muscle tumor arising in a liver allograft. The tumor was of donor origin. To our knowledge this compli-cation has not been seen before. Case Rellort An 18-month old white female underwent orthoto-pic liver transplantation for biliary atresia in April of 1986. Immunosuppression was with cyclospor-ine and prednisone. She had previously undergone a Kasai procedure at the age of 2 months. The donor was a healthy 22-month-old black male who died of a closed head injury. A complete autopsy was subsequently performed, which revealed only bilateral subdural hematomas. The kidneys were also harvested, and transplanted en bloc into a 53-year-old female. The patient's post-operative course was initially complicated by steroid-resistant rejection, which was successfully treated with OKT3. She later de-Supported by Research Grants from the Veterans Adminis-tration and Project Grant No. DK 29961 from the National Institutes of Health, Bethesd~ Maryland. Key words: 11111 Thomas E. Starzl, M.n. Ph.D. Departmen1 01 Sur-gery. 3601 Fifth Avenue. University of Pittsburgh. Pittsburgh. Pennsylvania, 15213. USA Accepted lor publication 13 September 1990 veloped cortical blindness of unknown etiology, foHowing an episode of grand mal seizure, but this resolved gradually over the next several months. Abdominal ullrasounds were performed over the next several weeks (4, 12, 21, and 29 days post-transplant). None of these showed a hepatic pai'ft'"1 fJ("tMh, '" cbymal lesion. An abdominal CT scan performed on post-op d 25 also showed a normal liver. She was discharged on cyclosporine and prednisone (60 mgfkg and 0.75 mgfkg respectively), with normal liver function. In May of 1989 a routine follow-up abdominal ultrasound revealed a single, hypoecho-ic lesion in the left lobe of the liver measuring 3 x 2 em. A CT scan of the abdomen confirmed this finding, but was otherwise unremarkable. At the time she was completely asymptomatic, and her physical examination was also unremarkable. Total bilirubin was 0.2 mgldl, alkaline phospha-tase was 267 ull, GOTP 53 ull, SOOT 31 ufl, and SOPT 22 ufl. Prothrombin time was 12.2 seconds. Alpha fetoprotein was < 2 ng/ml and CEA 1.2 ngl ml. An ultrasound-guided biopsy showed fibroadi-pose tissue, plus a moderately cellular minute por-tion of tissue composed of wavy, thin, elongated cells that stained OJ positively with muscle-specific actin. The patient underwent a left lateral segmentecto-my. Thorough exploration of the abdominal cavity failed to reveal any additional pathology. The resection specimen measured 10 x 7 x 3.5 cm and contained a 3 cm tumor with clean lines of resec-tion. The tumor was oval and encapsulated except for two small satellite nodules adjacent to it, each Doyle et a1. measuring 0.7 cm in diameter. It was composed of interlacing bundles of spindle cells (Fig. 1) tightly packed in some areas with some cellular pleomor-phism focally. The nuclei were long, blunt-ended and sometimes wavy. It had focal areas of necrosis, possibly corresponding to a previous biopsy site. Mitoses were less than 1/10 HPF. Tumor cells stained with desmin focally and with muscle-specific actin, but not withS 100 protein, suggesting a muscle origin. This was proven by electron microscopy (Fig. 2). The tumor cells had a well-defined base-ment membrane, masses of cytoplasmic filaments with dense bodies, scattered electron-dense conden-sations along the plasma membrane and numerous pinocytotic vesicles as well as abundant glycogen. The liver and tumor tissues, as well as positive and negative controls, were studied with a probe comple-mentary to a repeated sequence on the Y -chromo-some labeled with biotinylated d-UTP by the ran-dom priming method. The tumor showed a male pattern indicative of its donor origin. The patient made an uneventful recovery. Post-operatively a CT scan of the chest was obtained that showed only left lower lobe atelectasis, which was attributed to her recent surgery. A bone scan was negative. She was discharged with normal liver function. She is currently doing well 8 months fol-lowing the resection. DlsCDssi •• Any state of immunodeficiency, whether congenital acquired or iatrogenic, is complicated by an in-creased incidence of certain neoplasms (1-6). Since the early days of renal transplantation malignant disease has been recognized as a significant hazard, rig. 1. Interlacing bundles of elongated cells showing some nuclear variation, perinuclear clearing and distinct cell borders. H&E stain. 2 Fig. 2. Electron micrograph illustrating tumor cells with fea-tures of smooth muscle: basement membrane, pinocytoxic ves-icles, masses of cytoplasmic filaments with scattered electron densities and glycogen. with an incidence ranging from 1 % to 16%, and an average of 6.1 % (6). Although the inadvertent transplantation of ma-lignant tumors continues to occur sporadically de-spite careful screening of donors (7), most of these tumors arise de novo. In the cyclosporine era, im-munosuppression therapy is complicated by a rela-tively high incidence of non-Hodgkin's lymphoma, skin cancer, and Kaposi's sarcoma (6). In the particular case of liver transplantation, the Cambridge group reported a series of 122 pa-tients who survived for 3 months or more, among whom 7 de novo tumors were found (8). These included a patient described as having a reticulum cell sarcoma of host origin that developed in the grafted liver (8, 9). The remaining 6 patients had extrahepatic neoplasms, including a case of small bowel leiomyoma. To the best of our knowledge our case represents the first instance of a primary liver tumor, that is, of donor origin, to be reported in a liver transplant patient. Primary smooth muscle tumors of the liver are rare entities and we were only "able to find 21 re-ported cases in the literature (10-13). Four were lei-omyomas and the rest were primary hepatic lei-omyosarcomas. Clinical information was provided in 20 of these cases. There were 13 females and 7 males, with an age range of]2 to 86 yr. Most pre-sented with an abdominal mass or hepatomegaly (10,11). Smooth muscle tumors seem to represent a bio-logical continuum in which one is forced to draw certain arbitrary lines in designating some as "be-nign" and others as "malignant". A number of features such as size, cellularity, atypia, and necros-is correlate to some extent with malignancy, with mitotic activity being the most accurate and repro-ducible. Even this last criterion, however, is subor-dinate to the organ of origin (14). Because of their rarity it is very difficult to set forth criteria that will distinguish benign from malignant variants in cases of primary hepatic smooth muscle tumors. Leiomyosarcomas are described as having cells with hyperchromatic nuclei, with considerable variation in size, and frequent mitoses (one or more per high-power field) (15). In the 2 reported cases of leiomyoma that have addressed this issue, mito-ses were rare (less than 1/10 high-power fields) (16). By analogy with other gastrointestinal smooth muscle tumors it is to be expected that, although a combined assessment of size, cellularity, and cellular atypia conveys some impression of the ag-gressive potential of these neoplasms, absolute cri-teria for the diagnosis of malignancy cannot be established. The case we present, with its low mitotic rate, focally highly cellulax., and areas of necrosis, falls into an indeterminate category. Treatment consists of resection with a margin of normal liver. The unpredictability of these tumors mandates a careful and life-long follow-up. References 1. PENN I, HAMMOND W, BRETTSCHNEIDER L. STARZL TE. Ma-lignant lymphomas in transplantation patients. Transplant Proc 1969: I: 1 O6-1J 2. 2. STARn TE, NALESNIK MA, PORlER KA, et aJ. Reversibility of lymphomas and Iympboproliferative lesions developing under cyclosporine-steroid therapy. Lancet 1984: 1: 583-587. De nOI'(; smooth muscle tumor in liver Tx 3. NALESNtK MA. MAKOWKA L, STARZL TE. The diagnosis and treatment of post transplant Iymphoproliferative dis· orders. Curr Prob Surg 1988: 25: 365-472. 4. PENN 1. Malignancies associated with immunosuppressive or cytotoxic therapy. Surgery 1978: 83: 492-502. 5. PENN 1. The price of immunotheraP.r Curr~mrob~D" Surg..,.;)!p U E ! 5 h-1981: I ~: V ~ 2 - 1-5 I 6. PENN I, BRUNSOI' ME. Cancers after cycJosporine therapy. Transplant Proc 1988: 20: ~upplK 3):885-892. 7. MARSH JW JR., EsQUIVEL CO, MAKOWKA L, et a1. Acciden-tal transplantation of malignant tumor from a donor to multiple recipients. Transplantation 1987: 44: 449-450. 8. POLSON RJ, NEUBERGER J, FORMAN 0, CALN[ R, WtLLlAMS R. De Novo malignancies after liver transplantation. Tmns-plant Proc 1988: 20: • ' % a 94-97. 9. PORTMANI' B, SCHINDLER AM, MURRAy-Lyo/\ 1M, WILLI-AMS R. Histological sexing of i:I reticulum cell sarcoma arising after liver transplantation. Gastroenterology 1976: 70: 82-84. 10. MAKI HS, HUBERT BC, SAJJAD SM. KIRCHNER JP, KUEII"ER ME. Primary hepatic leiomyosarcoma. Arch Surg 1987: 122: 1193-1196. a II. HAWKINS EP, JORDAN GL. MCGAVRAK MH. Prim)ry lei· omyoma of the liver. Successful lrei:llmenl by lobectomy and presentation of criteria for diagnosis. Am J Surg Path 1980: 4: 301-304. 12. !WATSUKI S, TODO S, STARZL TE. Right triscgmentectomy with a synthetic vena cava graft. Arch Surg 1988: 123: 1021-1022. 13. RUMMENY E, WElSSLEDER R. STARK DD. c, al. Primary liver tumors: diagnosis by MR imaging. AJR 1989: 152: 63-72. 14. BROOK 11. Disorders of 50ft tissues. In: Diagnostic Surgical Pat!:::bgy (ed. Sternberg S) Raven Press ','0i. i. Chdp. 5, pp. 166-168, 1989. IS. lSHACK KG. Mesenchymal tumors of the liver. In: OKUDE K, PETERS RL (eds.) Hepatocellular Carcinoma. New York, John Wiley and Sons, Inc., pp. 268-275. 1976. 16. HAWKINS EP, JORDAN GL, McGAVRAN MH. Primary leiomyoma of the liver. Am .1 Surg Pat hoi 1980: 4: 301-304. 3 

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Smooth muscle tumor arising de novo in a liver allograft: A case report

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