Question 63 · LDEK · Benign tumors
Which of the following clinical pictures may be diagnosed as Gorlin-Goltz syndrome?
1) a 19-year-old woman with a focal skin lesion of basal cell carcinoma, the average degree of hypertelorism, ovary fibroma;
2) a 23-year-old man, whose sister was diagnosed with Gorlin-Goltz syndrome - medulloblastoma, Sprengel’s deformity;
3) a 50-year-old woman presenting with three depressions on the plantar surface of the left foot, calcification of one of the laminae of the cerebral falx, syndactylia;
4) a 12-year-old boy with fused ribs, histologically confirmed presence of two keratinizing cystic tumors of the jaws, cephalomegaly;
5) a 32-year-old woman with fibromas of the ovary, accentuation of the frontal bone, cheiloschisis.
The correct answer is:
2) a 23-year-old man, whose sister was diagnosed with Gorlin-Goltz syndrome - medulloblastoma, Sprengel’s deformity;
3) a 50-year-old woman presenting with three depressions on the plantar surface of the left foot, calcification of one of the laminae of the cerebral falx, syndactylia;
4) a 12-year-old boy with fused ribs, histologically confirmed presence of two keratinizing cystic tumors of the jaws, cephalomegaly;
5) a 32-year-old woman with fibromas of the ovary, accentuation of the frontal bone, cheiloschisis.
The correct answer is:
- A. 2,5.
- B. 3,5.
- C. 1,4.
- D. 2,4.
- E. 1,2.
Hint
Apply the major and minor criteria (two major, or one major plus two minor) to each case and pick the cases that clearly reach the threshold.
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