BRIEF REPORT Pulmonary inflammatory myofibroblastic tumor associated with nephrotic syndrome Divna Kruščić & Amira Peco-Antić & Brankica Spasojević-Dimitrijeva & Mirjana Stanić & Gordana Miloševski-Lomić & Mirjana Kostić & Petar Ivanovski & Ivan Boričić Received: 26 November 2006 / Revised: 17 April 2007 / Accepted: 20 April 2007 / Published online: 17 July 2007 # IPNA 2007 Abstract Inflammatory myofibroblastic tumor (IMT) of the lung is a benign, non-metastasizing tumor with the possibil- ity of local infiltration, recurrence or persistent local growth. This kind of tumor arises due to an unregulated growth of inflammatory cells. To our knowledge, IMT associated with nephrotic syndrome has not yet been recognized. Therefore, we present the case of a 14-year-old girl with lung IMT associated with secondary nephrotic syndrome (NS), which was cured after tumor removal. Keywords Inflammatory myofibroblastic tumor . Inflammatory pseudotumor . Primary lung mass . Children . Secondary nephrotic syndrome Introduction According to the literature, only malignant tumors can be associated with paraneoplastic nephrotic syndrome [1]. As the association of nephrotic syndrome and inflammatory myofibroblastic tumor (IMT) has not been reported in the literature until now, we present details of such a case in a female patient. Case report A 14-year-old girl was referred from a local hospital due to inflammatory syndrome, associated with hypoproteinemia, mild hypoalbuminemia, hypercholesterolemia and protein- uria (1,440 mg/24 h). Glomerular filtration rate (GFR) was normal (Table 1). Her past history was unremarkable except for recurrent obstructive bronchitis. Her family history was positive (her maternal grandfather had pulmonary carci- noma). On admission she had mild persistent edema; normal blood pressure, as well as other clinical findings. Inflammatory and nephrotic syndromes were confirmed (Table 1). She had no hematuria. Serum complement was normal. Chest X-ray exhibited a limited circular shadow, 35 mm in diameter, para-hilary right. Bronchoscopy finding was normal. CT scan confirmed radiological finding (Fig. 1). Her parents refused to allow her to undergo kidney biopsy. A subtotal right lobectomy was performed. A fibrous histiocytic pattern, the most common patholog- ical form of IMT, was seen on pathohistological and immunohistochemical examinations. Seven days after her operation the edema had disappeared and the proteinuria had significantly decreased. One month after her operation, the proteinuria had completely disappeared and remained absent during a further 1-year follow-up. Discussion We have presented details of a patient with nephrotic syndrome and IMT. Kidney biopsy was not done. If Pediatr Nephrol (2007) 22:1785–1786 DOI 10.1007/s00467-007-0517-z D. Kruščić : A. Peco-Antić : B. Spasojević-Dimitrijeva (*) : M. Stanić : G. Miloševski-Lomić : M. Kostić Nephrology Department, University Children’s Hospital, Tiršova 10, 11000 Belgrade, Serbia e-mail: brankicaspasojevic@yahoo.com P. Ivanovski Hematology Department, University Children’s Hospital, Belgrade, Serbia I. Boričić Medical School of Belgrade, Institute for Pathology, Belgrade, Serbia