Question:medium
Primary amenorrhea with absent uterus, normal breasts and scant pubic hair is seen in:
Primary amenorrhea with absent uterus, normal breasts and scant pubic hair is seen in:
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Normal breasts but scant pubic hair is the androgen insensitivity giveaway.
Updated On: Jun 24, 2026
- Mayer Rokitansky Kuster Hauser syndrome
- Turner's syndrome
- Androgen insensitivity syndrome
- Noonan syndrome
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The Correct Option is C
Solution and Explanation
Use the pubic hair as the decisive feature, since two options share the absent uterus plus normal breasts picture.
An absent uterus tells you the Mullerian ducts regressed, which occurs either in Mullerian agenesis or when AMH-secreting testes are present. Normal breasts tell you estrogen is working on the breast tissue. Those two clues alone fit both Mullerian agenesis and androgen insensitivity, so they cannot settle the answer by themselves.
The tie breaker is sexual hair. Pubic and axillary hair are androgen dependent. In androgen insensitivity, the receptor cannot read testosterone, so this hair is scanty or missing even though estrogen keeps the breasts normal. That single mismatch, good breasts but poor hair, is the textbook signature of androgen insensitivity.
In Mullerian agenesis the patient has functioning ovaries and intact androgen response, so pubic hair is normal, which the stem specifically denies. Turner syndrome keeps the uterus but gives streak gonads, short stature and poor breasts, and Noonan syndrome does not produce this triad at all.
$Absent\ uterus + normal\ breasts + scant\ hair \Rightarrow blocked\ androgen\ action$
\[\boxed{Androgen\ insensitivity\ syndrome}\]
An absent uterus tells you the Mullerian ducts regressed, which occurs either in Mullerian agenesis or when AMH-secreting testes are present. Normal breasts tell you estrogen is working on the breast tissue. Those two clues alone fit both Mullerian agenesis and androgen insensitivity, so they cannot settle the answer by themselves.
The tie breaker is sexual hair. Pubic and axillary hair are androgen dependent. In androgen insensitivity, the receptor cannot read testosterone, so this hair is scanty or missing even though estrogen keeps the breasts normal. That single mismatch, good breasts but poor hair, is the textbook signature of androgen insensitivity.
In Mullerian agenesis the patient has functioning ovaries and intact androgen response, so pubic hair is normal, which the stem specifically denies. Turner syndrome keeps the uterus but gives streak gonads, short stature and poor breasts, and Noonan syndrome does not produce this triad at all.
$Absent\ uterus + normal\ breasts + scant\ hair \Rightarrow blocked\ androgen\ action$
\[\boxed{Androgen\ insensitivity\ syndrome}\]
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