European Journal of Pediatrics

, Volume 146, Issue 1, pp 70–71 | Cite as

Clitoromegaly and abdominal tumour as leading signs of a mediastinal non-Hodgkin lymphoma

  • R. Ludwig
  • U. Heinrich
  • W. E. Brandeis
Short Communications

Abstract

A 2-year-old girl was admitted with clitoromegaly, mediastinal tumour and enlarged kidneys. A seventh cranial nerve palsy was associated with an increased cell count in cerebrospinal fluid. The diagnosis of a lymphoblastic lymphoma was confirmed by a cervical lymph-node biopsy.

Six years after diagnosis and treatment according to the BFM protocol the girl remains in first complete remission.

Key words

Mediastinal non-Hodgkin lymphoma Clitoromegaly 

References

  1. 1.
    Appelt H, Gerken H, Lenz W (1966) Tetraphokomelie, Lippen-Kiefer-Gaumenspalte und Clitorishypertrophie — ein Syndrom. Pädiatr Pädol 2:119–124Google Scholar
  2. 2.
    Carpentier PS, Potter EL (1958) Nuclear sex and genital malformation in 48 cases of renal agenesis with specific reference to nonspecific female pseudohermaphroditism. Am J Obstet Gynecol 78:235–258Google Scholar
  3. 3.
    Danks DM, Tippett P, Adams C, Campbell P (1975) Cerebro-hepato-renal syndrome of Zellweger. J Pediatr 86:382–387Google Scholar
  4. 4.
    Davidoff F, Federman DD (1973) Mixed gonadal dysgenesis. Pediatrics 52:725–742Google Scholar
  5. 5.
    Ein SH, Darte SMM, Stephan CA (1970) Cystic and solid ovarian tumors in children: A 44 year review. J Pediatr Surg 5: 148–156Google Scholar
  6. 6.
    Evans PR (1955) Leprechaunism. Arch Dis Child 30:479–483Google Scholar
  7. 7.
    Gordon LS, Murillo-Gucci G, Mulholle P (1974) Progressive idiopathic clitoral hypertrophy in a child. Birth Defects (VII):201–204Google Scholar
  8. 8.
    Haddad HH, Jones HW (1960) Clitoral enlargement simulating pseudohermaphroditism. Am J Dis Child 99:282–286Google Scholar
  9. 9.
    Jones HW (1981) Nonadrenal female pseudohermaphroditism. In: Josso N (ed) The intersex child. Pediatr adolesc Endocr 8:65–79Google Scholar
  10. 10.
    Kenny FM, Fetterman GH, Preeyasombat C (1966) Neurofibromata simulating a penis and labio-scrotal gonads in a girl with von Recklinghausen's disease. Pediatrics 37:456–459Google Scholar
  11. 11.
    Kenny FM, Hashida Y, Askavi A, sieber WH, Fetterman GH (1968) Virilizing tumours of the adrenal cortex. Am J Dis Child 115:445–458Google Scholar
  12. 12.
    Levitt LJ, Aisenberg AC, Harris NL, Linggood RM, Poppema S (1982) Primary non-Hodgkin's lymphoma of the mediastinum. Cancer 50:2486–2492Google Scholar
  13. 13.
    Lozano GB, Castaneda PF (1981) Priapism of the clitoris. Br J Urol 53:390Google Scholar
  14. 14.
    Maes M, Lee PA, Jeffs AD, Sultan C, Migeon CJ (1980) Phenotypic variation in a family with partial androgen insensitivity syndrome. Am J Dis Child 134:470–473Google Scholar
  15. 15.
    Müller-Weihrich ST, Beck J, Henze G, Jobke A, Kornhuber B, Lampert F, Ludwig R, Prindull G, Schellong G, Spaar HJ, Stollmann B, Treuner J, Wahlen W, Weinel P, Riehm H (1984) BFM-Studie 1981/83 zur Behandlung hochmaligner Non-Hodgkin-Lymphome bei Kindern: Ergebnisse einer nach histologisch-immunologischem Typ und Ausbreitungs-stadium stratefizierten Therapie. Klin Pädiatr 196:135–142Google Scholar
  16. 16.
    Murphy SB, Frizzera G, Evans AE (1975) A study of childhood non-Hodgkin's lymphoma. Cancer 36:2121–2131Google Scholar
  17. 17.
    New M, Levine LS (1984) Steroid 21-hydroxylase deficiency. In: New MI (ed) Adrenal disease in childhood. Pediatr adolesc Endocr 13:1–46Google Scholar
  18. 18.
    Niekerk van WA (1981) True hermaphroditism. In: Josso N (ed) The intersex child. Pediatr adolesc Endocr 8:80–89Google Scholar
  19. 19.
    Seip M (1959) Lipodystrophy and gigantism with associated endocrine manifestation: a new diencephalic syndrome? Acta Paediatr (Uppsala) 48:555–574Google Scholar
  20. 20.
    Steinhardt GF, Steinhardt E (1981) Priapism in children with leukemia. Urology 18:604–606Google Scholar
  21. 21.
    Stögmann W, Beitzke A, Falk W (1972) Typische und seltene Erscheinungsbilder der Neurofibromatosis von Reckling-hausen im Kindesalter. Klin Pädiatr 184: 420–427Google Scholar
  22. 22.
    Sullivan MP, Butler JJ (1977) Non-Hodgkin's lymphoma of childhood. In: Sutow W, Vietti T, Fernbach R (eds) Clinical pediatric oncology. Mosby, St Louis, pp 444–466Google Scholar
  23. 23.
    Vadakan VV, Ortega J (1972) Priapism in acute lymphoblastic leukemia. Cancer 30: 373–375Google Scholar
  24. 24.
    Wiedemann HR (1969) Das EMG-Syndrom: Exophtalmos, Makroglossie, Gigantismus und Kohlenhydratstoffwechsel-Störung. Z Kinderheilkd 106:171–185Google Scholar
  25. 25.
    Williams DL, Bell BA, Ragab AH (1985) Clitorism at presentation of acute nonlymphocytic leukemia. J Pediatr 107:754–755Google Scholar
  26. 26.
    Wilson JD, Harrod MJ, Goldstein JL, Hemsell DL, McDonald PC (1974) Familial incomplete male pseudohermaphroditism typ I: evidence for androgen resistence and variable clinical manifestations in a family with the Reifenstein syndrome. N Engl J Med 290:1097–1103Google Scholar

Copyright information

© Springer-Verlag 1987

Authors and Affiliations

  • R. Ludwig
    • 1
  • U. Heinrich
    • 1
  • W. E. Brandeis
    • 1
  1. 1.University Children's HospitalHeidelbergFederal Republic of Germany

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