Ann Pediatr Endocrinol Metab.  2016 Mar;21(1):51-55. 10.6065/apem.2016.21.1.51.

Male patients presenting with rapidly progressive puberty associated with malignant tumors

• Kim SJ
• Ko AR
• Jung MK
• Kim KE
• Chae HW
• Kim DH
• Kim HS
• Kwon AR

• KMID: 2161308
• DOI: http://doi.org/10.6065/apem.2016.21.1.51

Abstract

In males, precocious puberty (PP) is defined as the development of secondary sexual characteristics before age 9 years. PP is usually idiopathic; though, organic abnormalities including tumors are more frequently found in male patients with PP. However, advanced puberty in male also can be an important clinical manifestation in tumors. We report 2 cases of rapidly progressive puberty in males, each associated with a germ-cell tumor. First, an 11-year-old boy presented with mild fever and weight loss for 1 month. Physical examination revealed a pubertal stage of G3P3 with 10-mL testes. Investigations revealed advanced bone age (16 years) with elevated basal luteinizing hormone and testosterone levels. An anterior mediastinal tumor was identified by chest radiography and computed tomography, and elevated α-fetoprotein (AFP) and β-human chorionic gonadotropin (β-hCG) levels were noted. Histopathologic analysis confirmed a yolk-sac tumor. Second, a 12-year-old boy presented with diplopia, polydipsia, and polyuria for 4 months. Physical examination revealed a pubertal stage of G3P3 with 8-mL testes. Bone age was advanced (16 years) and laboratory tests indicated panhypopituitarism with elevated testosterone level. A mixed germ-cell tumor was diagnosed with elevated AFP and β-hCG levels. Of course, these patients also have other symptoms of suspecting tumors, however, rapidly progressive puberty can be the more earlier screening sign of tumors. Therefore, in male patients with accelerated or advanced puberty, malignancy should be considered, with evaluation of tumor markers. In addition, advanced puberty in male should be recognized more widely as a unique sign of neoplasm.

Keyword

Puberty; Neoplasms; Germ-cell tumor

MeSH Terms

Adolescent
Child
Chorionic Gonadotropin
Diplopia
Fever
Humans
Luteinizing Hormone
Male*
Mass Screening
Physical Examination
Polydipsia
Polyuria
Puberty*
Puberty, Precocious
Radiography
Testis
Testosterone
Thorax
Tumor Markers, Biological
Weight Loss
Chorionic Gonadotropin
Luteinizing Hormone
Testosterone
Tumor Markers, Biological

Figure

• 

  Fig. 1 (A) Chest radiograph showing multiple lung and pleural nodules and masses with right pleural effusion. (B) Left hand bone age radiograph at diagnosis. The arrow indicates lung and pleural nodules and masses.

• 

  Fig. 2 (A) Brain magnetic resonance imaging revealing mass lesions at pineal gland and suprasellar area. (B) Left hand bone age radiograph at diagnosis. The arrow indicates pineal gland and suprasellar area masses.

Reference

1. Berberoglu M. Precocious puberty and normal variant puberty: definition, etiology, diagnosis and current management. J Clin Res Pediatr Endocrinol. 2009; 1:164–174. PMID: 21274291.

2. Low LC, Wang Q. Gonadotropin independent precocious puberty. J Pediatr Endocrinol Metab. 1998; 11:497–507. PMID: 9777570.

3. Lazar L, Pertzelan A, Weintrob N, Phillip M, Kauli R. Sexual precocity in boys: accelerated versus slowly progressive puberty gonadotropin-suppressive therapy and final height. J Clin Endocrinol Metab. 2001; 86:4127–4132. PMID: 11549638.

4. Wendt S, Shelso J, Wright K, Furman W. Neoplastic causes of abnormal puberty. Pediatr Blood Cancer. 2014; 61:664–671. PMID: 24155044.

5. Schwabe J, Calaminus G, Vorhoff W, Engelbrecht V, Hauffa BP, Gobel U. Sexual precocity and recurrent beta-human chorionic gonadotropin upsurges preceding the diagnosis of a malignant mediastinal germ-cell tumor in a 9-year-old boy. Ann Oncol. 2002; 13:975–977. PMID: 12123344.

6. Lin CM, Lee CT, Tung YC, Wu MZ, Tsai WY, Yang YL, et al. Endocrine dysfunction in Taiwanese children with human chorionic gonadotropin-secreting germ cell tumors. J Formos Med Assoc. 2014; 113:102–105. PMID: 24530243.

7. Crawford JR, Santi MR, Vezina G, Myseros JS, Keating RF, LaFond DA, et al. CNS germ cell tumor (CNSGCT) of childhood: presentation and delayed diagnosis. Neurology. 2007; 68:1668–1673. PMID: 17502547.

8. Schneider DT, Calaminus G, Gobel U. Diagnostic value of alpha 1-fetoprotein and beta-human chorionic gonadotropin in infancy and childhood. Pediatr Hematol Oncol. 2001; 18:11–26. PMID: 11205836.

9. Schneider DT, Calaminus G, Koch S, Teske C, Schmidt P, Haas RJ, et al. Epidemiologic analysis of 1,442 children and adolescents registered in the German germ cell tumor protocols. Pediatr Blood Cancer. 2004; 42:169–175. PMID: 14752882.

10. Odagiri K, Omura M, Hata M, Aida N, Niwa T, Ogino I, et al. Treatment outcomes, growth height, and neuroendocrine functions in patients with intracranial germ cell tumors treated with chemoradiation therapy. Int J Radiat Oncol Biol Phys. 2012; 84:632–638. PMID: 22420962.