© Borgis - New Medicine 4/2004, s. 94-96
Paweł Krawczyk1, Beata Gryglicka1, Robert Kieszko1, Elżbieta Korobowicz2, Sebastian Sojczuk1, Janusz Milanowski1
Malignant neoplasms and pulmonary sarcoidosis – A case report
1Department of Pneumonology, Oncology and Allergology, Medical University of Lublin, Poland
Head: Prof. Janusz Milanowski MD, PhD
2Department of Pathomorphology, Medical University of Lublin, Poland
Head: Prof. Elżbieta Korobowicz MD, PhD
Most researchers believe that patients with pulmonary sarcoidosis are predisposed to develop malignancies, but others presume that malignancies lead to sarcoidosis. The case of a 52-year-old female patient with histopathologically diagnosed pulmonary sarcoidosis is reported. Computed tomography revealed an abnormal mass in the liver hilus. Histopathological examination indicated mucocellular carcinoma of unknown cause. An increased level of CA 125 (3641 U/ml) and normal values of CEA and CA 19-9 were suggestive of the onset of gynaecological neoplasia. The case report presented confirms that an association between sarcoidosis and malignancy is highly probable.
Little is known about cancer risk following sarcoidosis; some authors even hypothesise that malignancy gives rise to sarcoidosis (1). In 1974, Brincker et al. found out that lymphoma and pulmonary carcinoma occurred eleven and three times more frequently in patients with than those without sarcoidosis (2). The problem is further complicated when a sarcoid-like reaction in the course of neoplasia is considered in the lymph nodes, spleen or liver. The reaction may not indicate metastatic disease and does not necessarily suggest sarcoidosis, but may signify a local response or resistance to cancer cells. Therefore, some malignancies may be initially misdiagnosed as sarcoidosis and reversely, which in both situations require biopsy (3, 4).
The cause of a high risk of malignant neoplasms in patients with pulmonary sarcoidosis remains obscure. However, an increased incidence of lymphoma may result from immune defects often found in patients with sarcoidosis. Alterations in the function of cells con-trolling inflammatory response give rise to sarcoidosis. An increase in Th1-lymphocyte proliferation with abnormal cytokine secretion can be found in the airway and local lymph nodes. Cytokines, chemotactic agents and growth factors promote granuloma formation followed by destruction and fibrosis of the lung parenchyma with its permanent disorganization, which may produce the so-called "scar cancer” of the lung. Immunomodulatory activity also causes immunological abnormalities in the peripheral blood leading not only to an increased risk of lymphoma but also to a dec-reased anti-cancer response efficacy. Moreover, genetic alterations determined at the microsatellite DNA level (microsatellite instability) and a loss of heterozygosity have been detected in patients with sarcoidosis, and this may influence the co-existence of sarcoidosis and malignancies (5, 6, 7).
In October 2002, a 52-year-old female patient with histopathologically confirmed sarcoidosis originating from the mediastinal lymph node was admitted to the Pulmonary Department, Medical University of Lublin, in order to diagnose the cause of mediastinal and abdominal lymph node enlargement. Chest X-ray and abdominal ultrasonography revealed the presence of lesions but the patient complained only of weakness, thoracalgia and abdominal pain. In May 2002, the patient was admitted to hospital for pericarditis, and in June 2002, for gastric ulceration confirmed histo-pathologically following a gastroscopy.
Results of routine serum biochemical and haema-tological tests were within the normal range. The immunological parameters demonstrated a high level of gammaglobulins (28.7% of total proteins; reference ranges: 9-22%) resulting from an increased IgG concentration (3022 mg/dl; norm: 800-1700 mg/dL). Cytometry of peripheral blood lymphocytes showed the prevalence of cellular response; 80.4% of all lymphocytes were of a T-cell phenotype, and the CD4:CD8 ratio value was 1.05. The surface IL-2 (CD25 antigen) receptor was found on 33.5% of T-helper cells.
Bronchoscopy demonstrated a vascular network in the bronchial mucosa. 45% of the bronchoalveolar lavage fluid (BALF) cells were T-cell phenotype lymphocytes. The CD4:CD8 ratio was 4.7; 72.9% of T-lymphocytes had the HLA DR antigen on the surface and 18.6% of T-helper cells were CD25-positive. Histopathology of endobronchial biopsy specimens revealed the presence of giant and epithelial cell granulomas, which confirmed the diagnosis of pulmonary sarcoidosis.
The patient´s condition progressively deteriorated. Serum biochemistry showed an increased level of bilirubin of 5.2 mg/dL with direct-reacting bilirubin of 3.9 mg/dL. The serum levels of AsPAT, AlAT, diastase, lipase, alkaline phosphatase and GGTP were signi-ficantly increased. Abdominal CT revealed an abnormal mass of 6 to 7 cm in diameter with packets of lymph nodes in the liver hilus, close to the head of the pancreas and in the pyloric region of the stomach. The presence of lesions resulted in an occluded bile tract, disturbed venous circulation and deformed duodenum.
Abdominal abnormalities required histopathological examination. Laparoscopy revealed mucocellular car-cinoma but of an unknown origin. The malignant cells demonstrated anti-cytokeratin and anti-mucus reactivity. An increased level of CA 125 (3641 U/ml) and normal values of CEA and CA 19-9 might have been suggestive of the onset of gynaecological neoplasia.
In view of a high bilirubin level (17.3 mg/dL), transcutaneous drainage of the bile tract was performed. The patient was treated with Endoxan and received two courses of chemotherapy with Cisplatin, Adriamycine and Vincristine. Since the therapy was ineffective, palliative treatment was instituted.
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