hypertension due to renin secretion by neoplastic tissue was first reported by robertson et al in 1967 . subsequently , several cases of rennin - secreting tumors were presented with severe hypertension due to increased serum renin activity . based on several recent case reports , in the majority of patients , rennin - secreting tumors arise in the kidney as benign juxtaglomerular cell tumors [ 2 - 6 ] and more rarely as wilm s tumors , grawitz s tumors or renal hamartomas . extrarenal origin for renin - secreting tumors is rare ; however , some cases of adrenal and ovarian tumors or pelvic teratoma were reported [ 8 - 10 ] . the prognosis of these tumors is usually poor because the tumor is histologically aggressive and advanced at the time of diagnosis . severe hypertension due to increased serum rennin activity is usually the primary clinical manifestation of these tumors   and the term " primary reninism " has been applied for the clinical symptoms resulting from renin - secreting tumors . in this study , we report an infant with a rennin - producing hepatoblastoma that presented with severe hypertension and neurologic symptoms . to the best of our knowledge , a 22-month - old boy was admitted in the pediatric intensive care unit with recurrent partial seizures in the right side of his body in jun 2010 . the patient had had cough and intermittent fever since a month ago and had not recovered despite using oral antibiotics . the past history was remarkable for the presence of developmental delay , poor weight gain , and recurrent oral candidiasis since 4-month old . the studies for metabolic , immunologic , and endocrine disorders were inconclusive . from 2 weeks ago , the infant had developed right hemiparesis due to stroke , so he could not sit independently . he was the first offspring of consanguineous parents born in the thirty second week of gestational age with 2300gr birth body weight . at admission , physical examination revealed severe hypertension ( 160/100mmhg ) , marked hepatomegaly , and right hemiparesis . laboratory findings showed the following results : wbc count 9400/mm3 with 82% segmented cells , hb was 9.7 gr / dl and mcv 69 fl , platelets were normal . ldh level was 1056 iu / l , prothrombotic factors were done with normal results , and alfa - feto protein ( afp ) was 446.3 iu / ml ( normal : up to 5.5 iu / ml ) . abdominal sonography revealed multiple solid and isoechoic masses in both lobes of the liver which was confirmed with abdominal computed tomography ( ct scan ) ( figure 1 ) . magnetic resonance imaging of brain showed gliosis in medial portions of biparietal lobes and left occipital lobe due to previous vascular insult ( figure 2 ) . a markedly increased plasma renin activity was found ( 200 miclu / ml , normal level was up to 46.1 ) ; however , the level of plasma aldosterone was normal . subsequently , hypertension and seizures were controlled , but pneumonia has progressed despite different antibiotic and even antifungal therapy , the patient underwent assisted ventilation due to respiratory distress and depressed consciousness . histological examination of the tumor showed sheets of small , round , uniform cells with moderate amount of eosinophilic or clear cytoplasm , and distinct cytoplasmic membranes . the cells arranged into these trabeculae , usually two or three cells thick , with alternating light and dark areas and reported definitly as hepatoblastoma , epithelial type , fetal pattern ( figure 3 ) . the tumor was not resectable , and the patient expired finally at 12th day of the admission . paraneoplastic syndromes result from secretion of hormones , peptides or cytokines by tumor or immune cross - reactivity between malignant and normal tissues . the most common tumors are small cell lung cancer , breast , gynecologic , and hematologic cancers . the proper and timely diagnosis of paraneoplastic syndromes can result in finding an occult tumor at an early stage . these conditions are rare in children , but when the clinical presentation of a patient with a tumor is unusual , these syndromes should be emphasized like this infant with renin producing hepatoblastoma that presented with severe hypertension and neurologic symptoms . extensive work - up including radiological and biochemical studies did not demonstrate any renal parenchymal or renovascular origin . the laboratory facilities such as immunohistochemical study or polyclonal antibody for human renin activity were not available . therefore , we could not prove the secretion of renin just by tumor tissue cells , but this potentiality is very likely . hiroshi moritake et al have reported an 8-year - old boy with renin producing hepatoblastoma in 1999 . they confirmed the existence of renin messenger rna in the tumor tissue by reverse transcriptase polymerase chain reaction and immunohistochemical study . we also supposed that high plasma renin activity and severe hypertension resulted in cerebrovascular accident ( cva ) , seizure and encephalopathy in our patient . it is the most common primary and malignant tumor of the liver in children [ 13 , 14 ] and includes over 1% of pediatric cancers [ 12 , 14 ] . in children under the age of 15 , 79% of liver cancers male to female ratio is 1.6 - 3.3 [ 2 , 5 , 8 ] . the etiology of tumor is not well known , but it may be linked to several congenital anomalies such as beckwith - wideman syndrome , familial adenomatosis polyp , hemihypertrophy and low birth weight [ 12 , 14 ] . the incidence of hepatoblastoma is seemingly increased due to perinatal exposures and decreased premature infant mortality [ 12 , 15 ] . an association between premature birth and later development of hepatoblastoma has been suggested by several recent studies [ 16 - 18 ] . increased alfa - feto protein is a useful marker , but may also be found in hepatocelluar carcinoma . metastases are seen in 10 - 20% of patients at the time of the diagnosis and the lung is the most common site . therefore , chest radiography and ct scan are recommended at the time of diagnosis of hepatoblastoma . in our patient , pulmonary infiltrations in the form of pneumonia were seen by radiography , but chest ct scan did not reveal any metastatic foci . hepatoblastoma in abdominal sonography or ct scan appears as a focal or multifocal mass sometimes with calcification . recently , the role of chemotherapy has become increasingly important by enhancing the number of resectable tumors [ 4 , 22 ] . although liver transplantation is a useful treatment in children with unrespectable tumor with 90% survival rate , the outcome for such patients is poor [ 7 , 14 , 22 ] . while hepatoblastoma is the most common primary and malignant tumor of the liver in children , hypertension is a very rare and unusual presentation for it . when the clinical presentation of patients with a specific tumor is unusual , paraneoplastic syndromes should be emphasized ; like our case with renin producing hepatoblastoma that presented with severe hypertension and neurologic symptoms . several cases of rennin - secreting tumors were presented with severe hypertension due to increased serum renin activity . in the majority of these cases , the renin - producing tumor is located in the juxtaglomerular cells of the kidney and in some cases in other organs . as found in the literature , only in one other report the hepatoblastoma was the origin of renin secretion and the authors could prove the secretion of renin by tumor cells . although due to lack of specific laboratorytests we could not confirm the secretion of renin just by the tumor tissue cells in this patient , this potentiality is very likely . while hepatoblastoma is the most common primary and malignant tumor of the liver in children , hypertension is a very rare and unusual presentation for it . when the clinical presentation of patients with a specific tumor is unusual , paraneoplastic syndromes should be emphasized ; like our case with renin producing hepatoblastoma that presented with severe hypertension and neurologic symptoms . several cases of rennin - secreting tumors were presented with severe hypertension due to increased serum renin activity . in the majority of these cases , the renin - producing tumor is located in the juxtaglomerular cells of the kidney and in some cases in other organs . as found in the literature , only in one other report the hepatoblastoma was the origin of renin secretion and the authors could prove the secretion of renin by tumor cells . although due to lack of specific laboratorytests we could not confirm the secretion of renin just by the tumor tissue cells in this patient , this potentiality is very likely .
paraneoplastic syndromes result from secretion of hormones , peptides or cytokines by tumor or immune cross - reactivity between malignant and normal tissues . 
 these conditions are rare in children , but when the clinical presentation of patients with a tumor is unusual , these syndromes should be emphasized.extrarenal tumors with renin - secretion are rare in children . 
 they may be related to paraneoplastic syndromes . 
 we report a 22-month - old infant with hepatoblastoma presented with severe hypertension and related neurologic symptoms due to high plasma renin activity . to the best of our knowledge , 
 this is the second report of renin producing hepatoblastoma in the literature . 
 however , due to lack of laboratory facilities such as immunohistochemical study or polyclonal antibody for human renin activity , we could not prove the secretion of renin just by tumor tissue cells , but this potentiality is very likely . 
 other intensive investigations did not show any other origin for rennin secretion or hypertension in this patient .