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Familial benign cystic teratomata
Int. J. Gynaecol. Obstet., 1983, 21: 167-169 International Federation of Gynaecology & Obstetrics
FAMILIAL BENIGN CYSTIC TERATOMATA
STEVEN
H. BRENNER
Department (Received (Accepted
and ROBERT
C. WALLACH
of Obstetrics and Gynecology,
Beth Israel Medical Center, 10 Nathan D. Perlman Place, New York, NY 10003,
USA
May 20th, 1982) June 23rd, 1982)
Abstract
Case reports
Brenner S, Wallach RC (Department of Obstetrics and Gynecology, Beth Israel Medical Center, New York, NY, USA). Familial benign cystic teratomata. Int J Gynaecol Obstet 21: 167-169, 1983 A family with benign cystic teratomata in three successive generations is described. Other familial occurrences of these tumors have been reported; but not in three generations. Familial occurrence suggests an inherited characteristic for parthenogenic development and the occurrence of teratomata in twins and triplets supports the theory o.f a parthenogenie etiology. Benign cystic teratomata may, therefore, be tumors whose origin isgenetically controlled.
Case 1 H.P., a 19-year-old para 0000, who had undergone left salpingo-oophorectomy for a “dermoid cyst” at age 7 at another institution, was found to have an &cm right adnexal mass on routine examination. Teeth were seen on X-ray. A 7-cm right ovarian cyst was removed and found to be a benign cystic teratoma.
Key words: Ovarian teratomata; of ovarian tumors; occurrence occurrences; Genetically controlled.
Familial Multiple
Introduction Benign cystic teratomata are common among ovarian neoplasms but multiple occurrences in families are rarely reported. Surgery at this hospital for ovarian teratomata in three generations of one family is reported and no similar report has been found.
Case 3 A.R., a 78-yearold, para 2002, mother of J.P. and grandmother of H.P., was operated for adnexal mass and severe left lower quadrant pain at age 34. Left oophorectomy was performed for a benign cystic teratoma with incidental removal of a right ovarian follicle cyst and appendectomy. Discussion Pfannenstiel’
is quoted
as stating
that
al-
Int J Gynaecol Obstet 21
0020-7292/83/SO3.00
Q 1983 International Federation Printed and Published in Ireland
Case 2 J.P., a 45year-old para 3003, mother of H.P. had an asymptomatic 6-cm left adnexal mass found on routine examination. X-ray studies confirmed a mass with radiolucency suggestive of cystic teratoma. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed for myomata uteri and a left ovarian 5-cm benign teratoma.
of Gynaecology
& Obstetrics
168
Brenner and Wallach
though dermoid cysts can appear in sisters, the occurrence is rare [ 11. Reports of familial occurrence of dermoids have remained uncommon [ 2 I. This neoplasm has been reported in sisters [3,4], twins [Sl, triplets [6], mother and two daughters [2], and grandmother and granddaughter [ 71 . The authors of these case reports suggest a genetic predisposition to developing benign cystic teratomas. This report is unique in describing teratomata in a grandmother, daughter, and granddaughter. Cystic teratomata constitute about 10% of all ovarian tumors [8] and are reported to be bilateral in 15-l 8% of cases [ 91. Despite this high frequency, little attention is commonly paid to the possibility of an increased I familial incidence. The cystic teratomata are found in patients with a wide age distribution but with a peak incidence between 20 years and 40 years of age [ 101. Thirty-eight per cent of ovarian neoplasms in females under age 20 are reported to be teratomata [ 11 I. Common characteristics of inherited tumors are early onset and bilaterality, both common events with teratomata [7]. Discussing teratomas in three sisters, Sippel [ 31 in 1924, stated that although isolated reports of teratomata in siblings could not be used to prove a genetic basis for these tumors, their occurrence suggested some inherited factor from a mother or grandmother. Linder has demonstrated the parthenogenic origin of benign cystic teratomas from germ cells after the first meiotic division using chromosome banding and enzyme electrophoretic polymorphism studies [ 12- 141. Linder’s study [ 131 found five hosts to be heterozygous for 17 chromosome polymorphisms at or near the centromere while the teratomata in these patients were found to be homozygous. Biochemical data of enzyme polymorphisms showed six cases of host tissue heterozygosity and three cases in which the teratomata were heterozygous [131. Linder [ 131 states that teratomata would Int J Gynaecol Obstet 21
be homozygous for all chromosomal and gene markers if these tumors arose from a single mature ovum which doubled its chromosome number. Autofertilization of two mature ova would result in equal numbers of heterozygous and homozygous tumors. Teratomata resulting from suppression of the first meiotic division should be heterozygous for markers near the centromere if the host is heterozygous for those markers. Cytogenetic studies rule these two theories out [ 131. Heterozygosis is explained by crossing-over that may take place prior to the first meiotic division [ 131. Linder’s theory has become generally accepted and reports of familial benign cystic teratomata support this view [61. The theory of incomplete twinning or blastomere inclusions is apparently supported by the occurrence of teratomata in singleton sibs of non-tumor-bearing twins [ 41. No suggestion of an environmental causal agent has been made for this tumor which may represent an accident in development. From the current report, it may be inferred that the occurrence of benign cystic teratoma is due to an inherited trait. References 1 Pfannenstiel, quoted in Sippel A: Drei schwestern mit dermoid des ovars. Zentralbl Gynaekol48: 85,1924. 2 Plattner G, Oxorn H, Masterson J: Familial incidence of ovarian dermoid cysts. Can Med Assoc J 108: 892,1973. 3 Sippel A: lot. cit. [l] 48: 85, 1924. 4 Hollander H, Masterson J: Familial cystic teratoma of the ovary. Texas Rep Biol Med 23: 483, 1967. 5 Brown EH: Identical twins with twisted benign cystic teratoma of the ovary. Am J Obstet Gynecol 134: 879, 1979. 6 Feld D, Labes J, Nathanson M: Bilaterd ovarian dermoid cystsin triplets. Obstet Gynecol27: 525,1966. I Hecht F, McCaw BK, PatiI S: Ovarian teratomas and genetics of germ-cell formation (letter). Lancet 2: 7998: 1311,1976. 8 Ewing J: Neoplastic Diseases, A Treatise on Tumors, p. 672. W.B. Saunders Company, Philadelphia, PA, 1940. 9 Novack E, Woodruff J: Gynecologic and Obstetric Pathology, pp 485-490. W.B. Saunders Company, Philadelphia, PA, 1979. 10 Hertig AT, Gore H: Tumors of the ovary and fallopian tube, Section IX, fascicle 33, Tumors of the Female Sex Organs. Atlas of Tumor Pathology, p 59. Armed Forces Institute of Pathology, Washington, D.C., 1961.
Familial
11 Abell M, Johnson V, Holtz F: Ovarian neoplasms in childhood and adolescence. Am J Obstet Gynecol 92: 1059, 1965. 12 Lindner D, Rower J: Further evidence for post-meiotic origin of teratomata in the human female. Ann Hum Genet 34: 21,197O. 13 Lindner D, McCaw BK. Hecht F: Parthenogenic origin of benign ovarian teratomata. N Engl J Med 292: 63, 1975. 14 Gerald PS: Origin of teratomata. N Engl J Med 292: 3, 1975.
Teratomata
169
Address for reprints: Robert C. Wallach, M.D. Dept of Obstetrics and Gynecology Beth Israel Medical Center 10 Nathan D. Perlman Place New York, NY 10003 USA
Int J Gynaecol
Obstet -71
FAMILIAL BENIGN CYSTIC TERATOMATA
STEVEN
H. BRENNER
Department (Received (Accepted
and ROBERT
C. WALLACH
of Obstetrics and Gynecology,
Beth Israel Medical Center, 10 Nathan D. Perlman Place, New York, NY 10003,
USA
May 20th, 1982) June 23rd, 1982)
Abstract
Case reports
Brenner S, Wallach RC (Department of Obstetrics and Gynecology, Beth Israel Medical Center, New York, NY, USA). Familial benign cystic teratomata. Int J Gynaecol Obstet 21: 167-169, 1983 A family with benign cystic teratomata in three successive generations is described. Other familial occurrences of these tumors have been reported; but not in three generations. Familial occurrence suggests an inherited characteristic for parthenogenic development and the occurrence of teratomata in twins and triplets supports the theory o.f a parthenogenie etiology. Benign cystic teratomata may, therefore, be tumors whose origin isgenetically controlled.
Case 1 H.P., a 19-year-old para 0000, who had undergone left salpingo-oophorectomy for a “dermoid cyst” at age 7 at another institution, was found to have an &cm right adnexal mass on routine examination. Teeth were seen on X-ray. A 7-cm right ovarian cyst was removed and found to be a benign cystic teratoma.
Key words: Ovarian teratomata; of ovarian tumors; occurrence occurrences; Genetically controlled.
Familial Multiple
Introduction Benign cystic teratomata are common among ovarian neoplasms but multiple occurrences in families are rarely reported. Surgery at this hospital for ovarian teratomata in three generations of one family is reported and no similar report has been found.
Case 3 A.R., a 78-yearold, para 2002, mother of J.P. and grandmother of H.P., was operated for adnexal mass and severe left lower quadrant pain at age 34. Left oophorectomy was performed for a benign cystic teratoma with incidental removal of a right ovarian follicle cyst and appendectomy. Discussion Pfannenstiel’
is quoted
as stating
that
al-
Int J Gynaecol Obstet 21
0020-7292/83/SO3.00
Q 1983 International Federation Printed and Published in Ireland
Case 2 J.P., a 45year-old para 3003, mother of H.P. had an asymptomatic 6-cm left adnexal mass found on routine examination. X-ray studies confirmed a mass with radiolucency suggestive of cystic teratoma. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed for myomata uteri and a left ovarian 5-cm benign teratoma.
of Gynaecology
& Obstetrics
168
Brenner and Wallach
though dermoid cysts can appear in sisters, the occurrence is rare [ 11. Reports of familial occurrence of dermoids have remained uncommon [ 2 I. This neoplasm has been reported in sisters [3,4], twins [Sl, triplets [6], mother and two daughters [2], and grandmother and granddaughter [ 71 . The authors of these case reports suggest a genetic predisposition to developing benign cystic teratomas. This report is unique in describing teratomata in a grandmother, daughter, and granddaughter. Cystic teratomata constitute about 10% of all ovarian tumors [8] and are reported to be bilateral in 15-l 8% of cases [ 91. Despite this high frequency, little attention is commonly paid to the possibility of an increased I familial incidence. The cystic teratomata are found in patients with a wide age distribution but with a peak incidence between 20 years and 40 years of age [ 101. Thirty-eight per cent of ovarian neoplasms in females under age 20 are reported to be teratomata [ 11 I. Common characteristics of inherited tumors are early onset and bilaterality, both common events with teratomata [7]. Discussing teratomas in three sisters, Sippel [ 31 in 1924, stated that although isolated reports of teratomata in siblings could not be used to prove a genetic basis for these tumors, their occurrence suggested some inherited factor from a mother or grandmother. Linder has demonstrated the parthenogenic origin of benign cystic teratomas from germ cells after the first meiotic division using chromosome banding and enzyme electrophoretic polymorphism studies [ 12- 141. Linder’s study [ 131 found five hosts to be heterozygous for 17 chromosome polymorphisms at or near the centromere while the teratomata in these patients were found to be homozygous. Biochemical data of enzyme polymorphisms showed six cases of host tissue heterozygosity and three cases in which the teratomata were heterozygous [131. Linder [ 131 states that teratomata would Int J Gynaecol Obstet 21
be homozygous for all chromosomal and gene markers if these tumors arose from a single mature ovum which doubled its chromosome number. Autofertilization of two mature ova would result in equal numbers of heterozygous and homozygous tumors. Teratomata resulting from suppression of the first meiotic division should be heterozygous for markers near the centromere if the host is heterozygous for those markers. Cytogenetic studies rule these two theories out [ 131. Heterozygosis is explained by crossing-over that may take place prior to the first meiotic division [ 131. Linder’s theory has become generally accepted and reports of familial benign cystic teratomata support this view [61. The theory of incomplete twinning or blastomere inclusions is apparently supported by the occurrence of teratomata in singleton sibs of non-tumor-bearing twins [ 41. No suggestion of an environmental causal agent has been made for this tumor which may represent an accident in development. From the current report, it may be inferred that the occurrence of benign cystic teratoma is due to an inherited trait. References 1 Pfannenstiel, quoted in Sippel A: Drei schwestern mit dermoid des ovars. Zentralbl Gynaekol48: 85,1924. 2 Plattner G, Oxorn H, Masterson J: Familial incidence of ovarian dermoid cysts. Can Med Assoc J 108: 892,1973. 3 Sippel A: lot. cit. [l] 48: 85, 1924. 4 Hollander H, Masterson J: Familial cystic teratoma of the ovary. Texas Rep Biol Med 23: 483, 1967. 5 Brown EH: Identical twins with twisted benign cystic teratoma of the ovary. Am J Obstet Gynecol 134: 879, 1979. 6 Feld D, Labes J, Nathanson M: Bilaterd ovarian dermoid cystsin triplets. Obstet Gynecol27: 525,1966. I Hecht F, McCaw BK, PatiI S: Ovarian teratomas and genetics of germ-cell formation (letter). Lancet 2: 7998: 1311,1976. 8 Ewing J: Neoplastic Diseases, A Treatise on Tumors, p. 672. W.B. Saunders Company, Philadelphia, PA, 1940. 9 Novack E, Woodruff J: Gynecologic and Obstetric Pathology, pp 485-490. W.B. Saunders Company, Philadelphia, PA, 1979. 10 Hertig AT, Gore H: Tumors of the ovary and fallopian tube, Section IX, fascicle 33, Tumors of the Female Sex Organs. Atlas of Tumor Pathology, p 59. Armed Forces Institute of Pathology, Washington, D.C., 1961.
Familial
11 Abell M, Johnson V, Holtz F: Ovarian neoplasms in childhood and adolescence. Am J Obstet Gynecol 92: 1059, 1965. 12 Lindner D, Rower J: Further evidence for post-meiotic origin of teratomata in the human female. Ann Hum Genet 34: 21,197O. 13 Lindner D, McCaw BK. Hecht F: Parthenogenic origin of benign ovarian teratomata. N Engl J Med 292: 63, 1975. 14 Gerald PS: Origin of teratomata. N Engl J Med 292: 3, 1975.
Teratomata
169
Address for reprints: Robert C. Wallach, M.D. Dept of Obstetrics and Gynecology Beth Israel Medical Center 10 Nathan D. Perlman Place New York, NY 10003 USA
Int J Gynaecol
Obstet -71