FaCD Online Syndrome Fact Sheet
Last updated: 18 Apr 2008
Name: Langerhans Cell Histiocytosis
Synonym: Histiocytosis X, LCH
Mode of Inheritance: multifact?/ AD?
Tumor features (possible)ovarian germ cell tumor
pleuropulmonary blastoma
Non-tumor featureshistiocytosis
Comment
Langerhans cell histiocytosis is rare but is the commonest of the histiocytic disorders[1,2]. Familial cases are rare[3,4].
Mandel et al.[5] reported on a 3-generation family in which the paternal grandmother and 2 out of 4 granddaughters had been diagnosed with an ovarian germ cell tumor. Another granddaughter had been diagnosed with Langerhans' Histiocytosis at the age of 2 years. The four grandsons, as well as their parents, were healthy.
In a study of cancer in families of patients with pleuropulmonary blastoma, a range of tumors have been observed, including LCH[6].
References
[1] Weitzman S, Egeler RM. Langerhans cell histiocytosis: update for the pediatrician. Current opinion in pediatrics 2008; 20(1):23-9.
[2] Chang KL, Snyder DS. Langerhans cell histiocytosis. Cancer treatment and research 2008; 142():383-98.
[3] Aricò M, Nichols K, Whitlock JA, Arceci R, Haupt R, Mittler U, Kühne T, Lombardi A, Ishii E, Egeler RM, Danesino C. Familial clustering of Langerhans cell histiocytosis. British journal of haematology 1999; 107(4):883-8.
[4] Balikó Z, Schreiner M, Kishindy KK, Hegedüs G, Kosztolányi G. Different manifestations of langerhans cell histiocytosis affecting two members of a family. Respiration 2000; 67(5):583-5.
[5] Mandel M, Toren A, Kende G, Neuman Y, Kenet G, Rechavi G. Familial clustering of malignant germ cell tumors and Langerhans' histiocytosis. Cancer 1994; 73(7):1980-1983.
[6] Priest JR, Watterson J, Strong L, Huff V, Woods WG, Byrd RL, Friend SH, Newsham I, Amylon MD, Pappo A, Mahoney DH, Langston C, Heyn R, Kohut G, Freyer DR, Bostrom B, Richardson MS, Barredo J, Dehner LP. Pleuropulmonary blastoma: a marker for familial disease. The Journal of pediatrics 1996; 128(2):220-4.
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