Kaposi ' s sarcoma after heart transplantation : a case report

Introduction: Kaposi's sarcomas have been associated with different conditions of immunosuppression and are also known to be a typical complication of solid organ transplantations. Case presentation: We report the case of a 65-year-old Turkish man with a history of heart transplantation 10 months ago who presented for clarification of his dyspnea. The patient had a known history of chronic obstructive pulmonary disease and a smoking history of 40 pack years. Radiologically, three progressively growing intra-pulmonary nodules were detected. The histology was diagnostic for a Kaposi's sarcoma. Visceral and especially primary intra-pulmonary Kaposi's sarcomas are very rare and have been described to have a rather unfavorable prognosis. Conclusions: Even with a history suggestive for conventional lung cancer, Kaposi's sarcomas should be considered in patients after transplantation of solid organs. It should be noted that in a minority of cases this tumor exists in the absence of the typical cutaneous lesions. Introduction Kaposi's sarcomas are infrequent malignant tumors in the USA derived from vasoformative mesenchyme with a higher regional prevalence (endemic form) in the Mediterranean basin, the Middle East and Eastern Europe and in immunocompromised patients [1]. The latter encompasses mainly patients with acquired immunodeficiency syndrome (AIDS) and to a lesser extent individuals with other causes of compromised immunity, for example after solid organ transplantation (so called iatrogenic forms). It was estimated that the frequency of Kaposi's sarcoma in AIDS patients is still several hundred times higher than that in immunocompromised patients of other causes [1]. Classic (cutaneous) Kaposi's sarcoma typically manifests as bluish-red, well demarcated, painless dermal maculae, plaques or nodules in the distal lower extremities, which can become pedunculated and may ulcerate. Advanced lesions might display a brown color and hyper-keratotic surfaces. In addition to mucosal involvements visceral tumors (lymph nodes, gastrointestinal tract, lung, spleen) have been described for advanced cases and rarely as primary manifestations [1]. Most Kaposi's sarcomas were found in individuals older than 50 years with a clear male predominance. The course of these tumors is generally prolonged, although short courses have been described [1]. In the 1990 s the connection between the human herpes virus 8 (HHV8) and Kaposi's sarcomas was established. Virtually all Kaposi's sarcomas are thought to harbor this virus which can be detected by immunohistochemical and molecular-pathological techniques [1]. In this case report we describe the rare case of a primary intra-pulmonary Kaposi's sarcoma in a human immunodeficiency virus (HIV)-negative 65-year-old man with a history of heart transplantation 10 months previously. Case presentation A 65-year-old Turkish man presented to the hospital for clarification of dyspnea. Computed tomography (CT) showed a small tumor in the lower lobe of the left lung. Subsequent bronchial lavage was non-contributive concerning possible viral, bacterial (including mycobacteria) and fungal causes or malignancy. It was decided to perform a follow-up CT in three months. Our patient had a medical history of heart transplantation 10 months previously due to biventricular heart insufficiency, chronic atrial fibrillation and multi-vessel * Correspondence: florian.fritzsche@usz.ch 1 Institute of Surgical Pathology, Universit228;tsSpital Zürich, Schmelzbergstr. 12, 8091 Zurich, Switzerland Full list of author information is available at the end of the article © 2010 Fritzsche et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Fritzsche et al. Journal of Medical Case Reports 2010, 4:206 http://www.jmedicalcasereports.com/content/4/1/206 Page 2 of 4 coronary heart disease. Additionally, he had known chronic obstructive pulmonary disease (COPD; smoking history of 40 pack years), hyper-cholesterinemia, substituted hypothyroidism, articular gout, normocytic normochromic anemia, steroid induced myopathia and obesity, hypertrichosis (following therapy with cyclosporine) and a recently polymerase chain reaction (PCR)-confirmed cytomegaly virus infection. Follow-up CT after three and four months displayed three progressively growing tumors in the upper and lower lobe of the left lung (up to 1.6 cm) and in the lower right lobe (Figure 1A, B). Physical examination revealed no relevant findings. There were no palpable lymph nodes, no signs of tumors on the extremities and the skin. Since the above described lesions were not thoracoscopically detectable, an open thoracotomy was performed. The tumors in the left upper and lower lobe were surgically removed by wedge resection. One of the tumors was sent for intra-operative frozen section. Macroscopically, the tumor was red-brown and well demarcated from the surrounding parenchyma. Histologically, the frozen section revealed an intra-pulmonary spindlecell tumor. Intra-operatively the dignity of the tumor could not be definitely determined. On paraffin sections, the hematoxylin and eosin staining in all obtained specimens displayed rather monomorphic spindle-cell tumors (Figure 2A) with slit-like vascular clefts, entrapped erythrocytes and evidence of older hemorrhages. Tumor cells were positive for vascular molecular markers (CD 31, Figure 2B), moderately proliferating, and showed a fine granular nuclear positivity for HHV8 (LNA-1, Figure 2C). These findings were diagnostic for Kaposi's sarcoma. In the post-operative course our patient was transferred to the intensive care unit for two days for short-term intubation owing to pulmonary edema and reduced diuresis. He recovered well and was transferred back to the normal ward. Heart and lung function were normal. Our patient was able to leave hospital on the tenth postoperative day. Immunosuppressive therapy was changed from cyclosporine to everolimus and a follow-up CT in three months was arranged. In the meantime the immunosuppressive therapy might be further adapted to a possible chemotherapy. Discussion Iatrogenic (post-transplantation) Kaposi's sarcomas are most often detected in patients with a previous history of Figure 1 Lung imaging. Computed tomography displaying the progressively growing tumors (arrows) in the left (A) and right (B) lung. A