Adenomyosis in a patient with mosaic Turner?s syndrome

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This case report describes a 31-year-old woman with mosaic Turner's Syndrome who presented with hypermenorrhea and abdominal pain, and was found to have both a uterine myoma and adenomyosis.

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This single-patient case report describes a 31-year-old woman with mosaic Turner’s syndrome (45,X/46,XX/47,XXX) who presented with severe iron deficiency anemia from hypermenorrhea and persistent lower abdominal pain for more than six months. After transabdominal ultrasound suggested a large posterior uterine wall mass consistent with a myoma, the mass was surgically removed, and histology confirmed leiomyoma with focal adenomyosis; the authors note that adenomyosis and leiomyomata are separate but both are primarily seen in reproductive-age women and are estrogen dependent. The explicit limitation is that this is a case report, so conclusions cannot extend beyond the individual presentation. This paper is centrally about endometriosis and/or adenomyosis—specifically adenomyosis in a patient with mosaic Turner’s syndrome.

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Abstract

BackgroundFew case reports describing endometriosis in patients with gonadal dysgenesis have been published, but none has reported the presence of adenomyosis in a patient with Turner's syndrome.CaseA 31-year-old woman with mosaic Turner's Syndrome (45,X/46,XX/47,XXX) was referred to us because of severe iron deficiency anaemia due to hypermenorrhea and persistent lower abdominal pain for more than six months. She presented normal secondary sex development, normal breast, normal pubic and axillary hair. The external genitalia were also normal. Laboratory examination showed normal gonadotropin, 17beta-estradiol, plasma androgens and cortisol levels. At transabdominal ultrasound a myoma (15 x 8.5 x 8 cm) arising from the posterior uterine wall was suspected. The mass was removed during laparotomy. Histologic examination confirmed the presence of the myoma and revealed the presence of focal adenomyosis.ConclusionAdenomyosis and leiomyomata are separate entities but they share a common pathology in that they develop primarily in women of reproductive age and their growth is oestrogen dependent. To our knowledge, this is the first case report in the literature of adenomyosis in a woman who had the Turner's syndrome.
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Abstract

Background Few case reports describing endometriosis in patients with gonadal dysgenesis have been published, but none has reported the presence of adenomyosis in a patient with Turner’s syndrome. Case A 31-year-old woman with mosaic Turner’s Syndrome (45,X/46,XX/47,XXX) was referred to us because of severe iron deficiency anaemia due to hypermenorrhea and persistent lower abdominal pain for more than six months. She presented normal secondary sex development, normal breast, normal pubic and axillary hair. The external genitalia were also normal. Laboratory examination showed normal gonadotropin, 17beta-estradiol, plasma androgens and cortisol levels. At transabdominal ultrasound a myoma (15×8.5×8 cm) arising from the posterior uterine wall was suspected. The mass was removed during laparotomy. Histologic examination confirmed the presence of the myoma and revealed the presence of focal adenomyosis.

Conclusion

Adenomyosis and leiomyomata are separate entities but they share a common pathology in that they develop primarily in women of reproductive age and their growth is oestrogen dependent. To our knowledge, this is the first case report in the literature of adenomyosis in a woman who had the Turner’s syndrome. Similar content being viewed by others

References

Binns BA, Banerjee R (1983) Endometriosis with Turner’s syndrome treated with cyclical oestrogen/progestogen. Case report. Br J Obstet Gynecol 90:581–582 Bösze P, Gaál M, Tóth A, László J (1987) Endometriosis and streak gonad syndrome. Arch Gynecol 240:253–254 Doty DW, Gruber JS, Wolf GC, Winslow RC (1980) 46, XY pure gonadal dysgenesis: report of 2 unusual cases. Obstet Gynecol 55:61S–65S Elsheikh M, Dunger DB, Conway GS, Wass JA (2002) Turner’s syndrome in adulthood. Endocr Rev 23:120–140 Ferenczy A (1988) Pathophysiology of adenomyosis. Hum Reprod Update 4:312–322 Meinen K, Crusius M, Schulz U (1989) Turner syndrome with peritoneal endometriosis and ascites—therapeutic effects of buserelin therapy. Arch Gynecol Obstet 245:943–945 Peress MR, Sosnowski JR, Mathur RS, Williamson HO (1982) Pelvic endometriosis and Turner’s syndrome. Am J Obstet Gynecol 144:474–476 Raju GC, Naraynsingh V, Woo J, Jankey N (1988) Adenomyosis uteri: a study of 416 cases. Aust N Z J Obstet Gynaecol 28:72–73 Tazuke SI, Milki AA (2002) Endometrioma of uterine serosa in a woman with mosaic Turner’s syndrome receiving hormone replacement therapy: case report. Hum Reprod 17:2977–2980 Vercellini P, Parazzini F, Oldani S, Panazza S, Bramante T, Crosignani PG (1995) Adenomyosis at hysterectomy: a study on frequency distribution and patient characteristics. Hum Reprod 10:1160–1162 Wang PH, Shyong WY, Lin CH, Chen YJ, Li YF, Chao HT, Yuan CC (2002) Analysis of genetic aberrations in uterine adenomyosis using comparative genomic hybridization. Anal Quant Cytol Histol 24:1–6 Yamamoto T, Noguchi T, Tamura T, Kitawaki J, Okada H (1993) Evidence for estrogen synthesis in adenomyotic tissues. Am J Obstet Gynecol 169:734–738 Author information Authors and Affiliations Corresponding author Rights and permissions About this article Cite this article Ferrero, S., Bentivoglio, G. Adenomyosis in a patient with mosaic Turner’s syndrome. Arch Gynecol Obstet 271, 249–250 (2005). https://doi.org/10.1007/s00404-004-0603-4 Received: Accepted: Published: Issue date: DOI: https://doi.org/10.1007/s00404-004-0603-4

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Condition tags

adenomyosis

MeSH descriptors

Adenomyoma Adenomyoma Turner Syndrome Uterine Neoplasms Uterine Neoplasms Adenomyoma Adenomyoma Adenomyoma Adult Amenorrhea Amenorrhea Female Humans Menorrhagia Menorrhagia Sex Characteristics Turner Syndrome Uterine Neoplasms Uterine Neoplasms Uterine Neoplasms

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