Steroid cell tumor of the ovary in an infant 19
mo old
Strantzia-Michail C.1, Papathanasiou
A.2, Hatziathanasiou C.2,
Marinos Ev.3
1Pathology and 2Endocrinology
Department “P. & A. Kyriakou Children’s Hospital Athens, 3Laboratory
of Histology and Embryology Medical School, University of Athens
A 19-month-old female infant presented with progressive isosexual
precocious pseudopuberty. The clinical picture was associated
with marked elevation of serum dehydroepiandrostendione-sulfate,
oestradiole, testosterone and androstendione. A LHRH-test showed
basal and peak LH levels in normal prepubertal range. U/S disclosed
a right ovarian enlargement and exploratory laparotomy revealed
a right ovarian solid, yellow-brown circumscribed tumor 6 cm in
diameter. The clinical, morphological and immunohistochemical
findings classify it as "a steroid cell tumor not otherwise specified"
and differentiate it from the subtypes: stromal luteoma and Leydig-cell
tumor hilar or non-hilar type. Within five months after excision
of the tumor there is complete regression of characteristics of
precocious pseudopuberty. One and a half year later there is no
evidence of tumor relapse. Steroid cell tumors, which account
for only 0,1% of ovarian neoplasms in adults, are extremely rare
in childhood. (To our knowledge 23 cases in the literature considered
in investigating cases with precocious pseudopuberty, virilization
or Cushing’s syndrome).
Key words: Ovary, steroid-cell tumor, precocious puberty.
|
