Tumour spectrum in the FAMMM syndrome

Henry T. Lynch, R. M. Fusaro, J. Pester, J. A. Oosterhuis, L. N. Went, P. Rumke, H. Neering, J. F. Lynch

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Abstract

The Familial Atypical Multiple Mole-Melanoma Syndrome (FAMMM) is characterized by an autosomal dominantly inherited susceptibility to multiple atypical naevi. Patients with this hereditary phenotype show a strong susceptibility to cutaneous malignant melanoma (CMM). Our investigation of an extended Dutch kindred showing the FAMMM phenotype revealed a proband with bilateral intraocular malignant melanoma (IOM) and multiple CMM. The family revealed an array of tumours which included carcinoma of the lung, skin, larynx, and breast in addition to CMM and IOM, which were transmitted vertically through 3 generations. There was male-to-male transmission, and the number of affected males and females was about the same, which was consistent with an autosomal dominant inheritance. Thus the FAMMM syndrome not only indicates a potential for CMM, but a susceptibility to other systemic cancers as well. These observations, though limited to a single kindred, merit a painstaking evaluation of cancer of all anatomical sites in other kindreds showing the FAMMM syndrome. Such studies could yield clues to cancer aetiology, pathogenesis, and control.

Original languageEnglish
Pages (from-to)553-560
Number of pages8
JournalBritish Journal of Cancer
Volume44
Issue number4
DOIs
StatePublished - 1981

Fingerprint

Dysplastic Nevus Syndrome
Neoplasms
Melanoma
Phenotype
Nevus
Larynx
Breast
Carcinoma
Lung
Skin
Cutaneous Malignant Melanoma

All Science Journal Classification (ASJC) codes

  • Cancer Research
  • Oncology

Cite this

Lynch, H. T., Fusaro, R. M., Pester, J., Oosterhuis, J. A., Went, L. N., Rumke, P., ... Lynch, J. F. (1981). Tumour spectrum in the FAMMM syndrome. British Journal of Cancer, 44(4), 553-560. https://doi.org/10.1038/bjc.1981.225

Tumour spectrum in the FAMMM syndrome. / Lynch, Henry T.; Fusaro, R. M.; Pester, J.; Oosterhuis, J. A.; Went, L. N.; Rumke, P.; Neering, H.; Lynch, J. F.

In: British Journal of Cancer, Vol. 44, No. 4, 1981, p. 553-560.

Research output: Contribution to journalArticle

Lynch, HT, Fusaro, RM, Pester, J, Oosterhuis, JA, Went, LN, Rumke, P, Neering, H & Lynch, JF 1981, 'Tumour spectrum in the FAMMM syndrome', British Journal of Cancer, vol. 44, no. 4, pp. 553-560. https://doi.org/10.1038/bjc.1981.225
Lynch HT, Fusaro RM, Pester J, Oosterhuis JA, Went LN, Rumke P et al. Tumour spectrum in the FAMMM syndrome. British Journal of Cancer. 1981;44(4):553-560. https://doi.org/10.1038/bjc.1981.225
Lynch, Henry T. ; Fusaro, R. M. ; Pester, J. ; Oosterhuis, J. A. ; Went, L. N. ; Rumke, P. ; Neering, H. ; Lynch, J. F. / Tumour spectrum in the FAMMM syndrome. In: British Journal of Cancer. 1981 ; Vol. 44, No. 4. pp. 553-560.
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AB - The Familial Atypical Multiple Mole-Melanoma Syndrome (FAMMM) is characterized by an autosomal dominantly inherited susceptibility to multiple atypical naevi. Patients with this hereditary phenotype show a strong susceptibility to cutaneous malignant melanoma (CMM). Our investigation of an extended Dutch kindred showing the FAMMM phenotype revealed a proband with bilateral intraocular malignant melanoma (IOM) and multiple CMM. The family revealed an array of tumours which included carcinoma of the lung, skin, larynx, and breast in addition to CMM and IOM, which were transmitted vertically through 3 generations. There was male-to-male transmission, and the number of affected males and females was about the same, which was consistent with an autosomal dominant inheritance. Thus the FAMMM syndrome not only indicates a potential for CMM, but a susceptibility to other systemic cancers as well. These observations, though limited to a single kindred, merit a painstaking evaluation of cancer of all anatomical sites in other kindreds showing the FAMMM syndrome. Such studies could yield clues to cancer aetiology, pathogenesis, and control.

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