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J Pediatr Genet 2017; 06(04): 244-246
DOI: 10.1055/s-0037-1602789
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Plexiform Neurofibroma of Clitoris

Dhanya Yesodharan
1   Department of Pediatric Genetics, Amrita Institute of Medical Sciences & Research Center, Cochin, Kerala, India
,
Bindu Sudarsanan
2   Department of Pediatric Surgery, Amrita Institute of Medical Sciences and Research Center, Cochin, Kerala, India
,
Annie Jojo
3   Department of Clinical Pathology, Amrita Institute of Medical Sciences & Research Center, Cochin, Kerala, India
,
Mohan Abraham
2   Department of Pediatric Surgery, Amrita Institute of Medical Sciences and Research Center, Cochin, Kerala, India
,
Nisha Bhavani
4   Department of Endocrinology, Amrita Institute of Medical Sciences & Research Center, Cochin, Kerala, India
,
Hima Mathews
5   Department of Pediatrics, Amrita Institute of Medical Sciences & Research Center, Cochin, Kerala, India
,
Sheela Nampoothiri
1   Department of Pediatric Genetics, Amrita Institute of Medical Sciences & Research Center, Cochin, Kerala, India
› Institutsangaben
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Publikationsverlauf

04. Januar 2017

03. April 2017

Publikationsdatum:
02. Mai 2017 (online)

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Abstract

The most frequent genital presentation of neurofibromatosis in females is clitoromegaly. We report a case of a 5-year-old girl with neurofibromatosis type 1 with clitoral plexiform neurofibromatosis. Clitoroplasty was done, and the histopathology confirmed the diagnosis. Though rare, plexiform neurofibroma of clitoris should always be considered as a differential diagnosis in children with clitoromegaly before embarking on detailed investigations.

Keywords

plexiform neurofibroma - clitoromegaly - neurofibromatosis - NF1 - café au lait spots

Funding

None.


 

  • References

  • 1 Ferner RE, Huson SM, Thomas N. , et al. Guidelines for the diagnosis and management of individuals with neurofibromatosis 1. J Med Genet 2007; 44 (02) 81-88
    CrossrefPubMedGoogle Scholar
  • 2 Gordon MD, Weilert M, Ireland K. Plexiform neurofibromatosis involving the uterine cervix, endometrium, myometrium, and ovary. Obstet Gynecol 1996; 88 (4 Pt 2): 699-701
    CrossrefPubMedGoogle Scholar
  • 3 Haddad HM, Jones Jr HW. Clitoral enlargement simulating pseudohermaphroditism. AMA J Dis Child 1960; 99: 282-287
    PubMedGoogle Scholar
  • 4 Labardini MM, Kallet HA, Cerny JC. Urogenital neurofibromatosis simulating an intersex problem. J Urol 1967; 98 (05) 627-632
    CrossrefPubMedGoogle Scholar
  • 5 Pascual-Castroviejo I, Lopez-Pereira P, Savasta S, Lopez-Gutierrez JC, Lago CM, Cisternino M. Neurofibromatosis type 1 with external genitalia involvement presentation of 4 patients. J Pediatr Surg 2008; 43 (11) 1998-2003
    CrossrefPubMedGoogle Scholar
  • 6 Sutphen R, Galán-Goméz E, Kousseff BG. Clitoromegaly in neurofibromatosis. Am J Med Genet 1995; 55 (03) 325-330
    CrossrefPubMedGoogle Scholar
  • 7 Cost NG, Sanchez FS, Weinberg AG, Koral K, Baker LA. Neurofibromatosis presenting as painless clitoromegaly. Urol J 2009; 6 (03) 220-222
    PubMedGoogle Scholar
  • 8 Cheng L, Scheithauer BW, Leibovich BC, Ramnani DM, Cheville JC, Bostwick DG. Neurofibroma of the urinary bladder. Cancer 1999; 86 (03) 505-513
    CrossrefPubMedGoogle Scholar
  • 9 Thomas WJ, Bevan HE, Hooper DG, Downey EJ. Malignant schwannoma of the clitoris in a 1-year-old child. Cancer 1989; 63 (11) 2216-2219
    CrossrefPubMedGoogle Scholar
  • 10 Bilommi R, Drajat D. Case report: Clitoroplasty in neurofibromatosis presenting as clitoromegaly. J Pediatr Surg Case Rep 2015; 3 (05) 201-203
    PubMedGoogle Scholar