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Basal Cell Nevus Syndrome

1966; American College of Physicians; Volume: 64; Issue: 2 Linguagem: Inglês

10.7326/0003-4819-64-2-403

1539-3704

Nathaniel I. Berlin,

Tumors and Oncological Cases

Clinical Staff Conference1 February 1966Basal Cell Nevus SyndromeCombined Clinical Staff Conference at the National Institutes of HealthNATHANIEL I. BERLIN, M.D., EUGENE J. VAN SCOTT, M.D., WILLIAM E. CLENDENNING, M.D., HOWELL O. ARCHARD, D.D.S., JEROME B. BLOCK, M.D., CARL J. WITKOP, D.D.S., HARLEY A. HAYNES, M.D.NATHANIEL I. BERLIN, M.D., EUGENE J. VAN SCOTT, M.D., WILLIAM E. CLENDENNING, M.D., HOWELL O. ARCHARD, D.D.S., JEROME B. BLOCK, M.D., CARL J. WITKOP, D.D.S., HARLEY A. HAYNES, M.D.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-64-2-403 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptDr. Nathaniel I. Berlin: This discussion of the basal cell nevus syndrome is presented by the National Cancer Institute.This syndrome was first described by Binkley in 1951, and since that time approximately 100 patients have been described. This rare disorder is characterized by multiple basal cell tumors of the skin, cysts of the jaw, abnormalities of the ribs and metacarpal bones, and several endocrine abnormalities. Response to intravenous administration of parathyroid extract is diminished. Considerable evidence indicates that this is a heritable disorder, and that it may be related to other neoplasms. It is a progressive disorder, and the...References1. POTT P: Chirurgical Observations Relative to the Cataract, the Polypus of the Nose, the Cancer of the Scrotum, the Different Kinds of Ruptures and the Mortification of the Toes and Feet. Hawes, Clarke & Collins, London, 1775. Google Scholar2. VAN SCOTT EJ: Definition of epidermal cancer, in The Epidermis, edited by MONTAGNA, W., LOBITZ, W. C., JR. Academic Press, Inc., New York, 1964, p. 573. CrossrefGoogle Scholar3. EPSTEINMAIBACH WLHI: Cell renewal in human epidermis. Arch. Derm. (Chicago) 92: 462, 1965. CrossrefMedlineGoogle Scholar4. VAN SCOTTEKELAUERBACH EJTMR: Determinants of rate and kinetics of cell division in scalp hair. J. Invest. Derm. 41: 269, 1963. MedlineGoogle Scholar5. LITTLE DC: Skin fragments in end-opening needles. Canad. Med. Ass. J. 72: 374, 1955. MedlineGoogle Scholar6. GIBSONNORRIS TW: Skin fragments removed by injection needles. Lancet 2: 983, 1958. CrossrefMedlineGoogle Scholar7. VAN SCOTTREINERTSON EJRP: The modulating influence of stromal environment on epithelial cells studied in human autotransplants. J. Invest. Derm. 36: 109, 1961. CrossrefMedlineGoogle Scholar8. ZELICKSON AS: Electron microscope study of the basal cell epithelioma. J. Invest. Derm. 39: 183, 1962. CrossrefMedlineGoogle Scholar9. ROTHBERGVAN SCOTT SEJ: Absence of normal epidermal protein in basal cell tumor J. Invest. Derm. 42: 141, 1964. CrossrefMedlineGoogle Scholar10. CLENDENNINGBLOCKRADDE WEJBIC: Basal cell nevus syndrome. Arch. Derm. (Chicago) 90: 38, 1964. CrossrefMedlineGoogle Scholar11. GORLINVICKERSKELLNWILLIAMSON RJRAEJJ: The multiple basal-cell nevi syndrome. An analysis of a syndrome consisting of multiple nevoid basal-cell carcinoma, jaw cysts, skeletal anomalies, medullo-blastoma, and hyporesponsiveness to parathormone. Cancer 18: 89, 1965. CrossrefMedlineGoogle Scholar12. HELWIGGRAHAMMASON EBJHJK: The pathology of the nevoid basal cell carcinoma syndrome. Arch. Path. (Chicago) 79: 401, 1965. MedlineGoogle Scholar13. POLLARDNEW JJPF: Hereditary cutaneomandibular polyoncosis. A syndrome of myriad basal-cell nevi of the skin, mandibular cysts, and inconstant skeletal anomalies. Radiology 82: 840, 1964. CrossrefMedlineGoogle Scholar14. BLOCKCLENDENNING JBWE: Parathyroid extract insensitivity in the syndrome of multiple basal cell nevi and bone defects. Clin. Res. 10: 222, 1962. Google Scholar15. BLOCKCLENDENNING JBWE: Parathyroid hormone hyporesponsiveness in patients with basal-cell nevi and bone defects. New Eng. J. Med. 268: 1157, 1963. CrossrefMedlineGoogle Scholar16. ANDERSONMCCLENDONHOWELL DEJLJB: Genetics and skin tumors with special reference to basal cell nevi, in Tumors of the Skin. Year Book Medical Publishers, Inc., Chicago, 1964, p. 91. Google Scholar17. DAVIDSON F: Multiple naevoid basal cell carcinomata and associated congenital abnormalities. Brit. J. Derm. 74: 439, 1962. CrossrefMedlineGoogle Scholar18. GORLINYUNISTUNA RJJJN: Multiple nevoid basal cell carcinoma, odontogenic keratocysts, and skeletal anomalies: a syndrome. Acta Dermatovener. (Stockholm) 43: 39, 1963. MedlineGoogle Scholar19. MADDOXWINKELMANNHARRISONDEVINEGIBILISCO WDRKEGKDJA: Multiple nevoid basal cell epitheliomas, jaw cysts, and skeletal defects—a clinical syndrome. JAMA 188: 106, 1964. CrossrefGoogle Scholar20. THOMA KH: Polycystoma. Oral Surg. 12: 484, 1959. CrossrefMedlineGoogle Scholar21. BINKLEYJOHNSON GWHH: Epithelioma adenoides cysticum: basal cell nevi, agenesis of the corpus callosum and dental cysts—a clinical and autopsy study. Arch. Derm. Syph. (Chicago) 63: 73, 1951. CrossrefGoogle Scholar22. HOWELLCARO JBMR: The basal-cell nevus: its relationship to multiple cutaneous cancers and associated anomalies of development. Arch. Derm. Syph. (Chicago) 79: 67, 1959. Google Scholar23. BERNIER JL: The Management of Oral Disease. C. V. Mosby Co., St. Louis, 1955. Google Scholar24. GORLINMESKINBRODEY RJLHR: Odontogenic tumors in man and animals: pathologic classification and clinical behavior—a review. Ann. N. Y. Acad. Sci. 108: 722, 1963. CrossrefMedlineGoogle Scholar25. PINDBORGCLAUSEN JJF: Classification of odontogenic tumors: a suggestion. Acta Odont. Scand. 16: 293, 1958. CrossrefGoogle Scholar26. PETERSONGORLIN WCRJ: Possible analogous cutaneous and odontogenic tumors. Arch. Derm. (Chicago) 90: 255, 1964. CrossrefMedlineGoogle Scholar27. ALBRIGHTBURNETTSMITHPARSON FCHPHW: Pseudohypoparathyroidism—an example of "Seabright-Bantam syndrome": report of three cases. Endocrinology 30: 922, 1942. Google Scholar28. LEVIN B: Gonadal dysgenesis: clinical and roentgenologic manifestations. Amer. J. Roentgen. 87: 1116, 1962. Google Scholar29. ALBRIGHTFORBESHENNEMAN FAPPH: Pseudopseudohypoparathyroidism. Trans. Ass. Amer. Physicians 65: 337, 1952. MedlineGoogle Scholar30. KOLBSTEINBACH FOHL: Pseudohypoparathyroidism with secondary hyperparathyroidism and osteitis fibrosa. J. Clin. Endocr. 22: 59, 1962. CrossrefGoogle Scholar31. BELLGERARDBARTTER NHESFC: Pseudohypoparathyroidism with osteitis fibrosa cystica and impaired absorption of calcium. J. Clin. Endocr. 23: 759, 1963. CrossrefGoogle Scholar32. DAVIDSONKEY FJJ: Multiple naevoid basal cell carcinomata and associated congenital abnormalities. Proc. Roy. Soc. Med. 57: 891, 1964. MedlineGoogle Scholar33. JONESDESPERWELTONFLINK JEPCWAEB: The nevoid basal cell carcinoma syndrome. Arch. Intern. Med. (Chicago) 115: 723, 1965. CrossrefMedlineGoogle Scholar34. BECKLATOUR RPJP: Review of 1019 benign ovarian neoplasms. Obstet. Gynec. 16: 479, 1960. Google Scholar35. HERMANSGROSFELDSPAAS EHJCJA: The fifth phacomatosis. Dermatologica (Basel) 130: 447, 1965. CrossrefGoogle Scholar36. BUTTERWORTHSTREAN TLP: Neurocutaneous syndromes, in Clinical Genodermatology. The Williams & Wilkins Co., Baltimore, 1962, p. 101. Google Scholar37. BELISARIO JC: Effects of sunlight on the incidence of carcinomas and malignant melanoblastomas in tropical and subtropical areas of Australia. Derm. Trop. 1: 127, 1962. MedlineGoogle Scholar38. WINKLER M: The sun factor in skin cancer in Rhode Island and nearby New England. Rhode Island Med. J. 46: 370, 1963. MedlineGoogle Scholar39. SCOTT OL: Diseases of the skin, in Clinical Genetics, edited by SORSBY, A. C. V. Mosby Co., St. Louis, 1953. Google Scholar40. PATNAIKKRESHNASWAMY RM: Keratosis palmaris et plantaris (tylosis) with carcinoma of the oesophagus. A case report. Indian J. Derm. 29: 225, 1963. Google Scholar41. GARDNER EJ: Follow-up study of a family group exhibiting dominant inheritance for a syndrome including intestinal polyps, osteomas, fibromas and epidermal cysts. Amer. J. Hum. Genet. 14: 376, 1962. MedlineGoogle Scholar42. CLARKE CA: Tylosis with esophageal cancer. Meeting, Royal Society Medical Section of Dermatology , London, Dec. 19, 1957. Google Scholar43. HOWEL-EVANSMCCONNELLCLARKESHEPPARD WRBCAPM: Carcinoma of the esophagus with tylosis. Quart. J. Med. 27: 413, 1958. MedlineGoogle Scholar44. GORLINPINDBORG RJJJ: Syndromes of the Head and Neck. McGraw-Hill Book Co., Inc., New York, 1964. Google Scholar45. WITKOP CJ: Epithelial intracellular bodies associated with hereditary dyskeratoses and cancer therapy, in Proceedings of the First International Congress of Exfoliative Cytology. J. B. Lippincott Co., Philadelphia, 1962, p. 259. Google Scholar46. WEARYLINTHICUMCAWLEYCOLEMANGRAHAM PEAEPCCGF: Gardner's syndrome. A family group study and review. Arch. Derm. (Chicago) 90: 20, 1964. CrossrefMedlineGoogle Scholar47. GROSS PP: Epithelioma adenoides cysticum with follicular cysts of the maxilla and mandible. J. Oral Surg. 11: 160, 1953. MedlineGoogle Scholar48. MCKELVEYALBRIGHTPRAZAK LECRG: Multiple hereditary familial epithelial cysts of the jaws with the associated anomaly of trichoepithelioma. Report of a case. Oral Surg. 13: 111, 1960. CrossrefGoogle Scholar49. RODIN HH: Neurofibromatosis. Arch. Derm. Syph. (Chicago) 63: 530, 1951. Google Scholar50. GRAHAMMASONGRAYHELWIG JHJKHREB: Differentiation of nevoid basal cell carcinoma from epithelioma adenoides cysticum. J. Invest. Derm. 44: 197, 1965. CrossrefMedlineGoogle Scholar51. NICHOLSHOLDSWORTHREINFRANK FLDERF: Familial hypocalcemia, latent tetany and calcification of the basal ganglia. Report of a kindred. Amer. J. Med. 30: 518, 1961. CrossrefMedlineGoogle Scholar52. CROFTWITKOPGLAS LKCJJ: Pseudohypoparathyroidism. Oral Surg. 21: In press. Google Scholar53. KLOTZKAHN HPF: Le soi-disant "pseudo-hypoparathyroidisme" et les tétanies neurogènes. Sem. Hôp. Paris 33: 3772, 1957. MedlineGoogle Scholar54. DICKSONMORITACOWSERTGRAVESMEYER LGYEJJJS: Neurological, electroencephalographic and heredofamilial aspects of pseudohypoparathyroidism and pseudo-pseudohypoparathyroidism. J. Neurol. Neurosurg. Pychiat. 23: 33, 1960. CrossrefGoogle Scholar55. GORLINGOLTZ RJRW: Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib. A syndrome. New Eng. J. Med. 262: 908, 1960. CrossrefMedlineGoogle Scholar56. COOK WA: A family pedigree—cancer, cysts, and oligodontia. Dent. Radiogr. Photogr. 37: 27, 1964. Google Scholar57. CUSMANOBAKERFINBY JVDHN: Pseudohypoparathyroidism. Radiology 67: 845, 1956. CrossrefMedlineGoogle Scholar58. FRAMECARTER BS: Pseudohypoparathyroidism. Clinical picture and relation to convulsive seizures. Neurology 5: 297, 1955. CrossrefMedlineGoogle Scholar59. FABRINIRAMORINANACCARATO AMLR: Microsoma adiposa osteodistrofica di Albright. Folia Endocr. 11: 545, 1958. Google Scholar60. COHENDONNELL MLGN: Pseudohypoparathyroidism with hypothyroidism. A case report and review of the literature. J. Pediat. 56: 369, 1960. CrossrefMedlineGoogle Scholar61. MOREAULEGRAINGUEDON RMJ: Pseudohypoparathyroidie et pseudo-pseudohypoparathyroidie. Deux nouveaux cas familiaux de la "dystrophia" d'Albright. Bull. Soc. Med. Hop. Paris 74: 543, 1958. MedlineGoogle Scholar62. MANNALTERMANHILLS JBSAG: Albright's hereditary osteodystrophy comprising pseudohypoparathyroidism and pseudo-pseudohypoparathyroichsm. With a report of two cases representing the complete syndrome occurring in successive generations. Ann. Intern. Med. 56: 315, 1962. LinkGoogle Scholar63. GERSHBERGWESELEY HAC: Pseudohypoparathyroidism and pregnancy. Is pseudo-pseudohypoparathyroidism a mild form of pseudohypoparathyroidism? J. Pediat. 56: 383, 1960. CrossrefMedlineGoogle Scholar64. BERGSTRANDEKENGRENFILIPSSONHUGGERT CGKRA: Pseudohypoparathyroidism. Familial incidence and comparison with idiopathic hypoparathyroidism. Acta Endocr. 29: 201, 1958. MedlineGoogle Scholar65. FREEMANKNOXHEATON RGJMCL: The treatment of skin cancer. A statistical study of 1,341 skin tumors comparing results obtained with irradiation, surgery and curettage followed by electrodesiccation. Cancer 17: 535, 1964. CrossrefMedlineGoogle Scholar66. MORGENSTERNLEEPER NLRW: Chemosurgical treatment of skin cancers in a general clinic. Cancer 17: 5, 1964. CrossrefMedlineGoogle Scholar67. VAN SCOTTSHAWCROUNSECONDIT EJRKRGPT: Effects of methotrexate on basal-cell carcinomas. Arch. Derm. (Chicago) 82: 762. 1960. CrossrefMedlineGoogle Scholar68. BELISARIO JC: Modern treatment of skin cancer. Aust. J. Derm. 6: 96, 1961. CrossrefMedlineGoogle Scholar69. BELISARIO JC: Topical cytotoxic therapy for cutaneous cancer and precancer. Arch. Derm. (Chicago) 92: 293, 1965. CrossrefGoogle Scholar70. KLEINMILGROMHELMAMBRUSTRAENKELSTOLL EHFJHLHS: Tumors of the skin: effects of local use of cytostatic agents. Skin 1: 81, 1962. MedlineGoogle Scholar71. DILLAHAJANSENHONEYCUTTHOLT CJGTWMGA: Further studies with topical 5-fluorouracil. Arch. Derm. (Chicago) 92: 410, 1965. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: NATHANIEL I. BERLIN, M.D.; EUGENE J. VAN SCOTT, M.D.; WILLIAM E. CLENDENNING, M.D.; HOWELL O. ARCHARD, D.D.S.; JEROME B. BLOCK, M.D.; CARL J. WITKOP, D.D.S.; HARLEY A. HAYNES, M.D.This is an edited transcription of a combined clinical staff conference at the Clinical Center, Bethesda, Md., by the National Cancer Institute, National Institutes of Health, Public Health Service, U. S. Department of Health, Education, and Welfare.Requests for reprints should be addressed to Nathaniel I. Berlin, M.D., Clinical Director, National Cancer Institute, Building 10, Room 4-B-17, National Institutes of Health, Bethesda, Md. 20014. 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