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 language | English |
|---|---|
| Pages (from-to) | 553-560 |
| Number of pages | 8 |
| Journal | British Journal of Cancer |
| Volume | 44 |
| Issue number | 4 |
| DOIs | |
| State | Published - 1981 |
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All Science Journal Classification (ASJC) codes
- Cancer Research
- Oncology
Cite this
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 journal › Article
}
TY - JOUR
T1 - Tumour spectrum in the FAMMM syndrome
AU - Lynch, Henry T.
AU - Fusaro, R. M.
AU - Pester, J.
AU - Oosterhuis, J. A.
AU - Went, L. N.
AU - Rumke, P.
AU - Neering, H.
AU - Lynch, J. F.
PY - 1981
Y1 - 1981
N2 - 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.
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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U2 - 10.1038/bjc.1981.225
DO - 10.1038/bjc.1981.225
M3 - Article
C2 - 7295511
AN - SCOPUS:0019785626
VL - 44
SP - 553
EP - 560
JO - British Journal of Cancer
JF - British Journal of Cancer
SN - 0007-0920
IS - 4
ER -