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Severe menorrhagia, unilateral ovarian mass, elevated inhibin levels, and severe hypothyroidism: An unusual presentation of Van Wyk and Grumbach syndrome

      Abstract

      A 10 year old female presented with a 3 week history of persistent vaginal bleeding, lower abdominal pain, and generalized fatigue. Further work-up determined that she had severe autoimmune hypothyroidism, precocious puberty, and a large left cystic ovarian mass, consistent with Van Wyk and Grumbach syndrome. In addition, the patient had elevated inhibin A and B levels, which may be elevated in granulosa cell tumor. The patient was managed with thyroid hormone replacement therapy and careful observation. Complete resolution of the ovarian mass and interval normalization of the inhibin levels were achieved. In females with severe primary hypothyroidism, ovarian masses, and elevated tumor markers, operative resection may be avoided with appropriate thyroid replacement therapy and close observation.

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      References

        • Brown L.P.
        • Boswell H.B.
        • Crotty E.J.
        • et al.
        Van Wyk and Grumbach syndrome revisited: imaging and clinical findings in pre- and postpubertal girls.
        Pediatr Radiol. 2008; 38: 538-542
        • Chattopadhyay A.
        • Kumar V.
        • Kamataka M.
        Polycystic ovaries, precocious puberty, and acquired hypothyroidism: the Van Wyk and Grumbach syndrome.
        J Pediatr Surg. 2003; 39: 1390-1392
        • Durbin K.L.
        • Diaz-Montes T.
        • Loveless M.B.
        Van Wyk and Grumbach syndrome: an unusual case and review of the literature.
        J Pediatr Adolesc Gynecol. 2011; 24: e93-e96
        • Mohsin F.
        • Nahar N.
        • Azad K.
        • et al.
        Vaginal bleeding with multicystic ovaries and a pituitary mass in a child with severe hypothyroidism.
        Memensing Med J. 2007; 16: S60
        • Hunold A.
        • Alzen G.
        • Wudy S.A.
        • et al.
        Ovarian tumor in a 12-year old female with severe hypothyroidism: a case of Van Wyk and Grumbach syndrome.
        Pediatr Blood Cancer. 2009; 52: 677
        • Panico A.
        • Lupoli G.A.
        • Fonderico F.
        • et al.
        Multiple ovarian cysts in a young girl with severe hypothyroidism.
        Thyroid. 2007; 17: 1289
        • Kubota K.
        • Itho M.
        • Kishi H.
        • et al.
        Primary hypothyroidism presenting as multiple ovarian cysts in an adult woman: a case report.
        Gynecol Endocrinol. 2008; 24: 586
        • Sanjeevaiah A.R.
        • Sanjay S.
        • Deepak T.
        • et al.
        Precocious puberty and large multicystic ovaries in young girls with primary hypothyroidism.
        Endocr Pract. 2007; 13: 652
        • Van Wyk J.J.
        • Grumbach M.M.
        Syndrome of precious menstruation and galactorrhea in juvenile hypothyroidism: an example of hormonal overlap in pituitary feedback.
        J Pediatr. 1960; 57: 416-435
        • Cass D.L.
        • Hawkins E.
        • Brandt M.L.
        • et al.
        Surgery for ovarian masses in infants, children, and adolescents: 102 consecutive patients treated in a 15-year period.
        J Pediatr Surg. 2001; 36: 693-699
        • Anasti J.N.
        • Flack M.R.
        • Froelich J.
        • et al.
        A potential novel mechanism for precocious puberty in juvenile hypothyroidism.
        J Clin Endocrinol Metab. 1995; 80: 276-279
        • Lappohn R.
        • Burger H.
        • Bouma J.
        • et al.
        Inhibin as a marker for granulosa-cell tumors.
        N Engl J Med. 1989; 32: 790-793
        • Torok K.S.
        • Arkachaisri T.
        Autoimmune thyroiditis in antinuclear antibody positive children without rheumatologic disease.
        Pediatr Rheumatol Online J. 2010; 8: 15
        • Cappa M.
        • Bizzarri C.
        • Crea F.
        Autoimmune thyroid disease in children.
        J Thyroid Res. 2010; 2011: 675703
        • Eiland L.
        • Oyesiku N.M.
        • Ritchie J.C.
        • et al.
        Pathogenesis of marked pituitary enlargement and increased serum thyroid-stimulating hormone in primary hypothyroidism.
        Thyroid. 2012; 22: 101-102