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. Author manuscript; available in PMC: 2020 Dec 1.
Published in final edited form as: J Pediatr Adolesc Gynecol. 2019 Jun 21;32(6):605–611. doi: 10.1016/j.jpag.2019.06.005

Table 1.

Included Studies

Reference Clinical Diagnosis(es) n Age at Gonadectomy AR Sequencing Performed Germ Cells Present Precursor Lesions Malignant Tumors
Morris7 Testicular feminization 80 12-51 Years * * * 6/80: Seminoma, alveolar carcinoma, malignant teratoma, pelvic sarcoma, and malignant arrhenoblastoma
Morris and Mahesh26, Testicular feminization 181 Postpubertal * * * 11/50: Seminoma
Dewhurst et al27 Androgen insensitivity (AIS) 82 Before puberty to older than 30 years * * * 0/82
Dewhurst et al15 AIS, PAIS 66 (44 CAIS; 22 PAIS) Younger than 15 to older than 30 years * * * 0/44 CAIS; 1/12 PAIS: seminoma
Manuel et al19 Testicular feminization 23 * * * * 0/23
Muller21 CAIS, IAIS 12 (4 CAIS; 8 IAIS) 2 Months to 19 years * 50% 3/12 Carcinoma in situ
Hurt et al22 AIS 1 14 Years * * * Seminoma
Rutgers and Scully23 CAIS, IAIS 43 (40 CAIS; 3 IAIS) 16-83 Years CAIS; 14-47 years PAIS * 28% CAIS; 0% PAIS * 3/40 CAIS: seminomas and sex cord tumor; 0/3 PAIS
Ahmed et al28 CAIS, PAIS 278 (173 PAIS; 105 CAIS) 14 Years (0.1-18)§ Yes * * 0/278
Hannema et al24 CAIS 44 5.5 Years (1-13) Yes 95% 2/44 Carcinoma in situ
Purves et al25 CAIS 16 18.2 Years (2-21) * * * 0/16
Kravarusic et al14 CAIS, PAIS 11 (6 CAIS; 5 PAIS) 3-8 Years * * * 0/6 CAIS; 2/5 PAIS: bilateral seminoma, gonadoblastoma
Liu et al16 Gonadal dysgenesis, CAIS, PAIS 102 (30 CAIS; 18 PAIS) 20.7 Years (16-34) * * 0/48 9/30 CAIS: gonadoblastoma; 3/18 PAIS: gonadoblastoma
Kaprova-Pleskacova et al18 CAIS 19 (36 Gonads) 3 Months to 18.5 years Yes 84% 6/13 Precursor lesion types not reported; 1/13: intratubular germ cell neoplasia
Cools et al3 CAIS, PAIS 52 (42 CAIS; 10 PAIS) 17.5 Years (14-54) Yes 64% CAIS 88% PAIS 7/52 Precursor lesions types not reported; 0/52 in situ tumors

AIS, androgen insensitivity syndrome; IAIS, incomplete androgen insensitivity syndrome; AR, androgen receptor; CAIS, complete androgen insensitivity syndrome; PAIS, partial androgen insensitivity syndrome.

*

Not reported or unknown.

Literature review of cases.

At clinical presentation.

§

Sixty-six percent of CAIS cases had gonadectomy before puberty, 29% postpubertal, 5% unknown age; 39% of PAIS cases had gonadectomy before puberty, 9% postpubertal, and 52% unknown age.

In a subset of cases; 51 CAIS, 114 PAIS.

Median.