Pediatric Germ Cell and Human Chorionic

Gonadotropin\p=n-\ProducingTumors Clinical and Anna T.

Englund, MD; Barbara M.

Laboratory Features

Mitchell E.

Lippe, MD;

\s=b\ Germ cell tumors may cause various aberrations in pubertal development. In prepubertal boys, these tumors may secrete human chorionic gonadotropin, resulting in

precocious puberty. Human chorionic gonadotropin and \g=a\-fetoproteinare both useful as germ cell tumor markers in the diagnosis and detection of recurrence. Pregnancyspecific \g=b\1-glycoprotein, another oncoplacental antigen,

has been used as a tumor marker for trophoblastic neobut not previously for human chorionic gonadotropin\p=n-\producingtumors associated with precocious puberty. Patients with germ cell tumors may also have abnormal karyotypes. Herein, we describe six male pediatric patients with germ cell tumors and pubertal derangements seen during an 8-year period. We confirm the high incidence of associated sexual precocity, the usefulness of \g=a\-fetoprotein, human chorionic gonadotropin, and pregnancy-specific \g=b\1-glycoprotein as tumor markers in the diagnosis and follow-up of these patients, and the occurrence of sex chromosomal abnormalities.

plasms,

(AJDC. 1991;145:1294-1297) occur

cause

on

anatomic

of human chorionic gonadotropin

or

(hCG).

to

in secretion

The most com¬

hCG-secreting tumor causing precocious puberty is hepatoblastoma; however, germ cell tumors in various locations have also been reported to cause sexual precocity.1"6 Human chorionic gonadotropin and a-fetoprotein (AFP) are two tumor markers that have been used in the diagnosis and treatment of these patients. Pregnancy-specific ßrglycoprotein (SP-1), like hCG, is an oncoplacental protein that has been used as a marker for trophoblastic disease and various other neoplasms.7,8 mon

Accepted for publication June 3, 1991. From the Department of Pediatrics, UCLA Medical Center (Drs Englund, Geffner, Nagel, and Lippe) and the Department of Med-

icine, Cedars-Sinai Medical Center, Los Angeles, Calif (Dr Braunstein). Reprint requests to Department of Pediatrics, UCLA Medical Center, MDCC 22-315,10833 Le Conte, Los Angeles, CA 90024 (Dr

Englund).

However, there are no reports concerning use of SP-1

as

tumor marker in patients with precocious puberty sec¬ ondary to hCG-producing tumors. We describe six pedi¬ atrie patients with germ cell tumors seen in our clinic

a

during an 8-year period, in whom the usefulness of these tumor markers was investigated. Because of an associa¬ tion between germ cell tumors and Klinefelter's syndrome,4-911 chromosomal analyses were also under¬ taken.

PATIENTS AND METHODS

Patients Between 1983 and 1990, five patients (all male) were referred to both the pediatrie endocrinology and hematology services at UCLA Medical Center for evaluation of germ cell tumors with abnormalities in pubertal development. A sixth male patient is included who presented with precocious puberty associated with a hepatoblastoma. The clinical data of these patients are summarized in Table 1 and their laboratory data in Table 2.

Methods

cell rarely in children, but when Germ abnormalities present, they may puberty their based location due the tumors

Geffner, MD; Ronald A. Nagel, MD; Glenn D. Braunstein, MD

Serum luteinizing hormone (LH) and follicle-stimulating hor¬ mone (FSH) concentrations were measured with radioimmunoassay (Amersham Corp, Arlington Heights, 1). Testosterone was measured with standard radioimmunoassay. ot-Fetoprotein was measured using an enzymatic immunoassay (Abbott Labo¬ ratories, Abbott Park, 1); hCG, with an immunoradiometric as¬ say (Hybritech Ine, San Diego, Calif); and SP-1, with radioim¬ munoassay as previously described.12 Chromosomal karyotype was determined from peripheral lymphocytes, skin fibroblasts, and bone marrow using trypsin-Giemsa staining. A karyotype test was also performed on tumor tissue from patient 3. Bone age was determined from the standards of Greulich and Pyle.13

RESULTS Patients 2, 3, and 6 clearly had precocious puberty and patient 5 had delayed puberty. While receiving therapy for growth hormone deficiency, patient 1 unexpectedly developed early pubertal changes, prompting an investi¬ gation that led to the discovery of his tumor.3 The testicular volumes were small with respect to the degree of pu¬ bertal development in all patients except patient 2. Patients 2, 3, and 4 had germ cell tumors in the anterior mediastinum; patient 5, in the testicle; patient 1, in the

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Table 1.—Clinical Data* Patient No.

Age, y

1

11.4

Suprasellar

2

10.3

Anterior mediastinal teratoma/choriocarcinoma

3

11.3

Anterior mediastinal

4

15.9

Anterior mediastinal

5

18.5

L testicular seminoma

Age,y

Ba, y

8.8

10

4-5

11

16

15

13

Ht

Diagnosis teratoma

yolk sac tumor yolk sac tumor

Test

Vol,

16-17

8-10

18+

ND

5-6

15

16

mL

R4;L8-10

Puberty Precocious; Tanner II Precocious; Tanner V Precocious; Tanner V

Appropriate; Tanner V Delayed; Tanner III

1.7 4 1.5 Precocious; Tanner II Hepatoblastoma *Ht Age indicates the age at which the patient's height was at the 50th percentile; BA, bone age; Test Vol, testicular volume; R, right; L, left; ND, not done; and, Tanner, Tanner stage of genital/pubic hair development (II, early puberty; III, midpuberty; and V, adult). 6

1.5

Table

2.—Laboratory Data* Patient No.

hCG, IU/L

Preoperative Postoperative AFP, µg/L Preoperative Postoperative SP-1, |Ag/L Preoperative Postoperative

33

1935

171

Pediatric germ cell and human chorionic gonadotropin-producing tumors. Clinical and laboratory features.

Germ cell tumors may cause various aberrations in pubertal development. In prepubertal boys, these tumors may secrete human chorionic gonadotropin, re...
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