Ovarian sex cord tumor with annular tubules: review of 74 cases including 27 with Peutz-Jeghers syndrome and four with adenoma malignum of the cervix

Author(s): Young RH, Welch WR, Dickersin GR, Scully RE

Abstract

The sex cord tumor with annular tubules (SCTAT) is a distinctive ovarian neoplasm the predominant component of which has morphologic features intermediate between those of the granulosa cell tumor and those of the Sertoli cell tumor; focal differentiation into either granulosa cell or Sertoli cell tumor may occur. Of the 74 cases that form the basis of this investigation 27 were associated with the Peutz-Jeghers syndrome; these tumors were all benign and were typically multifocal, bilateral, very small or even microscopical in size and calcified. Twelve of the 27 patients had symptoms suggestive of hyperestrinism attributable to the SCTAT; menstrual irregularity had occurred in eleven cases and postmenopausal bleeding in one. Four of the 27 patients had "adenoma malignum" of the cervix and two of them died of it. The 47 tumors from patients without evidence of the Peutz-Jeghers syndrome were unilateral and usually large. Twenty-five of them were accompanied by symptoms suggestive of hyperestrinism, such as menstrual irregularity, postmenopausal bleeding or sexual precocity; seven were malignant and four of these were fatal.

Similar Articles

Peutz-Jeghers syndrome: a systematic review and recommendations for management

Author(s): Beggs AD, Latchford AR, Vasen HF, Moslein G, Alonso A, et al.

A serine/threonine kinase gene defective in Peutz-Jeghers syndrome

Author(s): Hemminki A, Markie D, Tomlinson I, Avizienyte E, Roth S, et al.

Increased risk of cancer in Peutz-Jeghers Syndrome

Author(s): Giardiello FM, Welsh SB, Hamilton SR, Offerhaus GJ, Gittelsohn AM, et al.

Frequency and Spectrum of Cancers in the Peutz-Jeghers Syndrome

Author(s): Hearle N, Schumacher V, Menko FH, Olschwang S, Boardman LA, et al.

Increased risk for cancer in patients with the Peutz-Jeghers syndrome

Author(s): Boardman LA, Thibodeau SN, Schaid DJ, Lindor NM, McDonnell SK, et al.

Adenoma Malignum (minmal deviation adenocarcinoma of uterine cervix

Author(s): Gilks CB, Young RH, Aguirre P, DeLellis RA, Scully RE

A clinicocytopathologic study of adenoma malignum of the uterine cervix

Author(s): Hirai Y, Takashemi N, Haga A, Arai Y, Akiyama F, et al.

Mucious lesions of fallopian tube

Author(s): Seidamn JD

Multifocal tumorigenesis in the upper female genital tract--implications for staging and management

Author(s): Russell P, Bannatyne PM, Solomon HJ, Stoddard LD, Tattersall MH

Malignant ovarian sex cord tumor with annular tubules in a patient with Peutz-Jeghers syndrome: a case report

Author(s): Lele SM, Sawh RN, Zaharopoulus P, Adesokan A, Smith M, et al.

Peutz-Jeghers syndrome and cancer

Author(s): Rodu B, Martinez MG Jr

Sertoli Cell Tumor Causing Precocious Puberty in a Girl with Peutz-Jeghers Syndrome

Author(s): Zung A, Shoham Z, Open M, Altman Y, Dgani R, et al.

Very high risk of cancer in familial Peutz-Jeghers syndrome

Author(s): Giardiello FM, Brensinger JD, Tersmette AC, Goodman SN, Petersen GM, et al.

Peutz-Jeghers syndrome and management Recommendations

Author(s): Giardiello FM, Trimbath JD