Eur J Pediatr Surg 2010; 20(6): 423-425
DOI: 10.1055/s-0030-1254144
Case Gallery
© Georg Thieme Verlag KG Stuttgart · New York

Endobronchial, Pulmonary and Liver Leiomyomata in a Child with Primary Immune Deficiency

I. R. User1 , D. Dogru2 , B. Talim3 , D. Orhan3 , I. Karnak1
  • 1Hacettepe University, Department of Pediatric Surgery, Ankara, Turkey
  • 2Hacettepe University, Department of Pediatric Pulmonology, Ankara, Turkey
  • 3Hacettepe University, Department of Pediatric Pathology, Ankara, Turkey
Weitere Informationen


07. Juni 2010 (online)


Leiomyoma of the lung is a rare benign tumor in children [8]. It is associated with immune suppression after organ transplantation and viral infections such as Eppstein-Barr virus (EBV) and HIV [4]. It is of mesodermal origin and it has been hypothesized that leiomyoma of the lung arises from the smooth muscles of the bronchial wall or artery [2]. Half of these tumors are parenchymal and the rest of the lesions are endobronchial or tracheal [5]. Symptoms include bronchial obstruction and atelectasis, and the lesions can be the cause of recurrent pulmonary infections, but they are otherwise coincidentally found [3] [7]. We present the first case of a child with a primary immune deficiency and EBV-negative serology and endobronchial, pulmonary and liver leiomyomata to emphasize the importance of planning before surgery and the necessity of investigating for associated leiomyomata.


  • 1 Alessi G, Lemmerling M, Vereecken L. et al . Benign metastasizing leiomyoma to skull base and spine: a report of two cases.  Clin Neurol and Neurosurg. 2003;  105 170-174
  • 2 Allan JS. Rare solitary benign tumors of lung.  Semin Thorac Cardiovasc Surg. 2003;  15 315-322
  • 3 Bilgin S, Yılmaz A, Okur E. et al . Primary endobronchial leiomyoma: a case report.  Tüberküloz ve Toraks Dergisi. 2004;  52 272-274
  • 4 Hatano M, Takada H, Nomura A. et al . Epstein-Barr virus-associated bronchial leiomyoma in a boy with cellular immunodeficiency.  Pediatr Pulmonol. 2006;  41 371-373
  • 5 Karnak I, Akcoren Z, Senocak ME. Endobronchial leiomyoma in children.  Eur J Pediatr Surg. 2000;  10 136-139
  • 6 Kishore R, Richards AP, Evans N. Benign metastasizing leiomyoma.  Clinic Radiol Extra. 2004;  59 29-31
  • 7 McClian Kl. Epstein-Barr virus and HIV–AIDS-associated diseases.  Biomed Pharmacother. 2001;  55 348-352
  • 8 Ozcelik U, Kotiloglu E, Gocmen A. et al . Endobronchial leiomyoma: a case report.  Thorax. 1995;  50 101-102
  • 9 Reyes C, Abuzaitoun O, Jong A. et al . Epstein-Barr virus-associated smooth muscle tumors in ataxia telangiectasia: a case report and review.  Hum Pathol. 2002;  33 133-136
  • 10 Tietze L, Günther K, Hörbe A. et al . Benign metastasizing leiomyoma: a cytogenetically balanced but clonal disease.  Hum Pathol. 2000;  31 126-129
  • 11 Wilson R, Kirejczyk W. Pathologic and radiologic correlation of endobronchial neoplasms: Part 1, benign tumors.  Ann Diagn Pathol. 1997;  1 31-46


Dr. Ibrahim Karnak

Hacettepe University

Department of Pediatric Surgery

06100 Ankara


Telefon: + 90 312 311 5541

Fax: + 90 312 311 5541