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Early onset of puberty in an obese boy with Klinefelter syndrome.

Cho BW, Kwon SE, Kim SK, Lee T, Han JY, Lee JE - Ann Pediatr Endocrinol Metab (2016)

Bottom Line: Adolescents with KS are generally diagnosed as having the lack of pubertal progress.Early detection of KS can be difficult without awareness.We report an unusual case of early onset of puberty in obese boy with KS who presented with a unilateral non-hormone secreting testicular teratoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Inha University Hospital, Inha University Graduate School of Medicine, Incheon, Korea.

ABSTRACT
Klinefelter syndrome (KS) is one of the most common disease entities characterized by X-chromosomal aberration causing the primary hypogonadism in adult men. Patients with KS seem to be typically characterized by tall, slender bodies with delayed puberty and hypergonadotropic hypogonadism. However, it has been known that they have a broad spectrum of phenotype ranging from almost normal external appearances to typical phenotype. Only 25% KS Patients are ever diagnosed because KS remains unrecognized. Also, boys with KS have an onset of pubertal development within the normal range, not delayed onset of puberty. Adolescents with KS are generally diagnosed as having the lack of pubertal progress. Early detection of KS can be difficult without awareness. We report an unusual case of early onset of puberty in obese boy with KS who presented with a unilateral non-hormone secreting testicular teratoma.

No MeSH data available.


Related in: MedlinePlus

(A) Resected left testicular mass revealed a mature teratoma. The nodule in the testis showed mature squamous cell nests in the fibrotic stroma (H&E, ×200). (B) The testicular parenchyme showed atrophic seminiferous tubule without germ cells. The hyperplastic Leydig cells were noted (H&E, ×200).
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Figure 1: (A) Resected left testicular mass revealed a mature teratoma. The nodule in the testis showed mature squamous cell nests in the fibrotic stroma (H&E, ×200). (B) The testicular parenchyme showed atrophic seminiferous tubule without germ cells. The hyperplastic Leydig cells were noted (H&E, ×200).

Mentions: A 10.6-year-old boy was referred to the Department of Urology in Inha University Hospital for evaluation of different sizes in both testes. At a Doppler-ultrasound sonography, there were an ill-defined hyperechoic lesion with microcalcification and varicocele in his left testis. He had undergone left orchiectomy for a testicular tumor on the same side confirmed by frozen biopsy specimens. The histopathologic examinations indicated that the resected testicular mass was mature teratoma (0.30×0.3 cm-sized) with severe testicular atrophy and surrounding tissue had a marked decrease in the number of germ cells (Fig. 1). On karyotyping, the patient was diagnosed with a typical type of KS with 47, XXY. Then, he was referred to our clinic for evaluation of his abnormal karyotype.


Early onset of puberty in an obese boy with Klinefelter syndrome.

Cho BW, Kwon SE, Kim SK, Lee T, Han JY, Lee JE - Ann Pediatr Endocrinol Metab (2016)

(A) Resected left testicular mass revealed a mature teratoma. The nodule in the testis showed mature squamous cell nests in the fibrotic stroma (H&E, ×200). (B) The testicular parenchyme showed atrophic seminiferous tubule without germ cells. The hyperplastic Leydig cells were noted (H&E, ×200).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4835560&req=5

Figure 1: (A) Resected left testicular mass revealed a mature teratoma. The nodule in the testis showed mature squamous cell nests in the fibrotic stroma (H&E, ×200). (B) The testicular parenchyme showed atrophic seminiferous tubule without germ cells. The hyperplastic Leydig cells were noted (H&E, ×200).
Mentions: A 10.6-year-old boy was referred to the Department of Urology in Inha University Hospital for evaluation of different sizes in both testes. At a Doppler-ultrasound sonography, there were an ill-defined hyperechoic lesion with microcalcification and varicocele in his left testis. He had undergone left orchiectomy for a testicular tumor on the same side confirmed by frozen biopsy specimens. The histopathologic examinations indicated that the resected testicular mass was mature teratoma (0.30×0.3 cm-sized) with severe testicular atrophy and surrounding tissue had a marked decrease in the number of germ cells (Fig. 1). On karyotyping, the patient was diagnosed with a typical type of KS with 47, XXY. Then, he was referred to our clinic for evaluation of his abnormal karyotype.

Bottom Line: Adolescents with KS are generally diagnosed as having the lack of pubertal progress.Early detection of KS can be difficult without awareness.We report an unusual case of early onset of puberty in obese boy with KS who presented with a unilateral non-hormone secreting testicular teratoma.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Inha University Hospital, Inha University Graduate School of Medicine, Incheon, Korea.

ABSTRACT
Klinefelter syndrome (KS) is one of the most common disease entities characterized by X-chromosomal aberration causing the primary hypogonadism in adult men. Patients with KS seem to be typically characterized by tall, slender bodies with delayed puberty and hypergonadotropic hypogonadism. However, it has been known that they have a broad spectrum of phenotype ranging from almost normal external appearances to typical phenotype. Only 25% KS Patients are ever diagnosed because KS remains unrecognized. Also, boys with KS have an onset of pubertal development within the normal range, not delayed onset of puberty. Adolescents with KS are generally diagnosed as having the lack of pubertal progress. Early detection of KS can be difficult without awareness. We report an unusual case of early onset of puberty in obese boy with KS who presented with a unilateral non-hormone secreting testicular teratoma.

No MeSH data available.


Related in: MedlinePlus