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Juvenile Granulosa Cell Tumor of the Testis in a Newborn with Swelling in the Scrotum: A Rare Case and Literature Review.


Juvenile granulosa cell tumors (JGCTs) of the testis in newborns are very rarely seen benign tumors. They occur in association with sex chromosome disorders and ambiguous genitalia. Alpha-fetoprotein levels have a poor diagnostic value for teratomas in infants <6 months of age. Most of JGCTs are confused with a teratoma based on histopathological analysis results. Inguinal orchiectomy yields satisfactory treatment results. We present the case of our patient diagnosed with a JGCT of the testis and review the relevant literature.

Keywords: Juvenile granulosa cell tumor, testis, newborn, orchiectomy


Testicular tumors comprise 1% of all pediatric tumors and are rarely seen in newborns. Granulosa cell tumors, which are a sex cord-stromal tumor group, can be divided into adult granulosa cell tumor and juvenile granulosa cell tumor (JGCT) subtypes (1). In contrast to adult granulosa cell tumors, the juvenile subtype has benign characteristics. A physical examination usually reveals a painless mass, and inguinal orchiectomy is sufficient for treatment (2). This study aimed to present the rare case of a patient diagnosed as having JGCT and to review the relevant literature.


A 12-day-old male infant was brought to our clinic with swelling in the left scrotum that was noticed by the family 1 week prior. A physical examination revealed a palpable, but painless, mobile, and diffuse, 2x2 cm mass with a smooth surface. The right testis had dimensions of 7x6 mm, and penile and other system examinations were unremarkable. There were no changes in the color of the scrotum (Figure 1). A complicated cystic lesion, the contours of which could not be discriminated from the left testicular parenchyma, contained 15x10 mm septations, and occasional areas of vascular flow were detected on performing scrotal ultrasound (US; Philips, Epiq 5, USA) (Figure 2a, b). A 2 mm simple cyst was observed in the right epididymis. Pelvic magnetic resonance imaging (MRI; Philips, Ingenia 1,5T, USA) revealed a septated mass lesion in the left testis with regular contours, which could not be differentiated from the left testicular parenchyma, containing a 15x11 mm cystic area and millimeter-sized cysts in the head of the right epididymis (Figures 3a, b). The levels of human chorionic gonadotropin (hCG; <2 mlU/mL) and alpha-fetoprotein (AFP; 33.118 ng/mL on day 7 and 2.209 ng/mL on day 25) were also measured. Chromosomal analysis revealed a 46 XY genotype. The infant underwent high scrotal orchiectomy through a left inguinal incision. The orchiectomized testis measured 2x1.5 cm. Three cysts with regular contours were detected in sections, and these were surrounded with a dirty white fibrous capsule containing yellow serous fluid; the largest cyst was 1 cm in diameter (Figure 4a, b). A histopathological examination of the mass revealed a JGCT. The postoperative AFP level was 450 ng/mL. No complications were encountered during the follow-up period.


Granulosa cell tumors belong to the sex cord-stromal tumor group and are divided into adult granulosa cell tumor and JGCT subtypes (1). Adult granulosa cell tumors are malignant and generally originate from the ovaries, whereas JGCTs are benign and originate from testicular tissue (3).

Although JGCTs are rare testicular tumors, they are the most frequently seen testicular tumor in infants <6 months of age (1). The most commonly seen clinical finding is unilateral, painless scrotal swelling noticed by the family. In our case, scrotal swelling was detected by the family. A concomitant Y-chromosomal disorder and ambiguous genitalia have been reported in 20% of patients, but we found no Y-chromosome disorder and/or genital anomaly in the present case (4, 5).

Ultrasound can detect an intratesticular multicystic mass, and the normal testicular parenchyma can be evaluated when the clinical diagnosis suggests a cystic teratoma (1, 5-6). In the present case report, we described a complicated cyst with intratesticular septa and indistinct parenchymal contours. This mass lesion was evaluated as a teratoma. As shown in the present case, the MRI signs of this tumor include a multiseptated scrotal mass (1). Although hCG and AFP levels are used as tumor markers, particularly within the first 6 months after disease onset, AFP level do not help in diagnosing a teratoma (7). Bellini et al. (8) demonstrated changes in AFP levels based on body weight and gestational age (Figures 5A and 5B). In such cases, although the AFP level had no diagnostic value during the neonatal period, an increase from a previously low AFP level during the postoperative follow-up period is positively associated with the likelihood of recurrence (9). In our case, the preoperative AFP level was extremely higher than normal, suggesting the diagnosis of a teratoma, but it failed to define the condition. The histopathological examination established the diagnosis of JGCT.

Clinical information about the diagnosis, treatment, and followup of JGCTs is primarily in the form of case reports. To date, we found only 24 cases where patients were diagnosed as having JGCTs in the English medical literature (1, 2, 4-6, 11, 12). In total, 6 (25%) of these 24 cases were intra-abdominal, including 3 (50%) bilateral cases. Triploidy (4, 5, 13, 15, 17, 18) a chromosome 4 deletion, and multiple anomalies were reported in the abdominal cases (n=5; 83%). We accessed the data of 12 JGCT cases with the JGCT located in the scrotum; however, no preoperatively diagnosed case of JGCT was found. JGCTs were confused with teratomas, and the diagnosis in all these cases was established based on the histopathology. These were not recurrent cases, and no medical or oncological treatment was applied (Table 1). Therefore, we only performed high scrotal orchiectomy. Organ-sparing surgery has been recommended for small, histopathologically benign tumors with distinct margins. No recurrence or testicular atrophy was reported during the long-term follow-up of these patients (10). We performed orchiectomy because the tumor tissue could not be macroscopically differentiated from the testicular tissue during the surgery.


Juvenile granulosa cell tumors are rarely seen, painless, benign masses, particularly in newborns. The preoperative diagnosis of a JGCT is difficult, and it is usually initially diagnosed as a teratoma. Inguinal orchiectomy is a satisfactory treatment modality.

Ethics Committee Approval: Authors declared that the research was conducted according to the principles of the World Medical Association Declaration of Helsinki "Ethical Principles for Medical Research Involving Human Subjects", (amended in October 2013).

Informed Consent: Written informed consent was obtained from patient who participated in this study.

Peer-review: Externally peer-reviewed.

Author Contributions: Conceived and designed the experiments or case: TT., UB. Performed the experiments or case: TT., [??]C. Analyzed the data: MS. Wrote the paper: TT. All authors have read and approved the final manuscript.

Conflict of Interest: No conflict of interest was declared by the authors.

Financial Disclosure: The authors declared that this study has received no financial support.


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(2.) Bulotta AL, Molinaro F, Angotti R, Ferrara F, Maggio GD, Bindi E, et al. Juvenile granulosa cell tumor of the testis: prenatal diagnosisandprescrotalapproach. Ital J Pediatr 2012; 38: 67. [CrossRef]

(3.) Young RH. Sexcord-stromal tumors of theovaryand testis: their similarities and differences with consideration of selected problems. Mod Pathol 2005; 18: 81-98. [CrossRef]

(4.) Raju U, Fine G, Warrier R, Kini R, Weiss L. Congenital testicular juvenile granulosa cell tumor in a neonatal with X/XY mosaicism. Am J Surg Pathol 1986; 10(8): 577-83. [CrossRef]

(5.) Leylek AM, Kane RA. Juvenile granulosa cell tumor of the testis. Ultrasound Quarterly 2014; 30(3): 219-20. [CrossRef]

(6.) Peterson C, Skoog S. Prenatal diagnosis of juvenile granulosa cell tumor of the testis. J Pediatr Urol 2008; 4(6): 472-4. [CrossRef]

(7.) Grady RW, Ross JH, Kay R. Epidemiological features of testicular teratoma in a prepubertal population. J Urol 1997; 158(3 Pt 2): 1191-2. [CrossRef]

(8.) Bellini C, Bonacci W, Parodi E, Serra G. Serum ct-fetoprotein in newborns. Clin Chem 1998; 44(12): 2548-50.

(9.) Kaygusuz EI, Cetiner H, Celayir AC, Mora[??][??][u S, Bosnal[??] O. Testicular yolk sac tumor simulating acute scrotum in an infant. J Turkish Assoc Pediatr Surg 2013; 27(2): 81-4. [CrossRef]

(10.) Sugita Y, Clarnette TD, Cooke Yarbrough C, Chroww CW, Waters K. Hutson JM. Testicular and paratesticular tumors in children: 30 years experience. Aust NZJ Surg 1999; 69(7): 505: 8.

(11.) Alexiev BA, Alaish SM, Sun CO Testicular juvenile granulosa cell tumor in a newborn: Case report and review of the literature. International J Surg Pathol 2007; 15(3): 321-5. [CrossRef]

(12.) Levy DA, Kay R, Elder JS. Neonatal testis tumors: a review of the prepubertal testis tumor registry. J Urol 1994; 151(3): 715-7. [CrossRef]

(13.) Partalis N, Tzardi M, Barbagadakis S, Sakellaris G. Juvenile granulosa cell tumor arising from intra-abdominal testis in newborn: case report and review of the literature. J Urol 2012; 79(5): 1152-4. [CrossRef]

(14.) Zugor V, Labanaris AP, Witt J, Seidler A, Weingartner K, Schott GE. Congenital juvenile granulosa cell tumor of the testis in newborn. Anticancer Research 2010; 30(5): 1731-4.

(15.) Yu DC, Pathak B, Vargas SO, Javid PJ, Hisama FM, Wilson JM, et al. Congenital intra-abdominal bilateral juvenile granulosa cell tumor of the testis associated with constitutional loss of material from chromosome 4. Pediatr Dev Pathol 2011; 14(3): 224-7. [CrossRef]

(16.) Dudani R, Giordano L, Sultania P, Jha K, Horens A, Joseph T. Juvenile granulosa cell tumor of testis: case report and review of literature. Am J Perinatol 2008; 25(4): 229-1. [CrossRef]

(17.) Y[??][??]maz A, Lee EY MRI findings of bilateral juvenile granulosa cell tumor of the testis in a newborn presenting as intraabdominal masses. Pediatr Radiol 2007; 37(10): 1031-4. [CrossRef]

(18.) Kos M, Nogales FF, Kos M, Stipoljev F, Kunjko K. Congenital juvenile granulosa cell tumor of the testis in a fetus showing full 69, XXY triploidy. International J Surg Pathol 2005; 13(2): 219-21. [CrossRef]

(19.) Chan YF, Restall P, Kimble R. Juvenile granulosa cell tumor of the testis: report of two cases in newborns. J Pediatr Surg 1997; 32(5): 752-3. [CrossRef]

(20.) Nistal M, Redondo E, Paniagua R. Juvenile granulosa cell tumor of the testis. Arch Pathol Lab Med 1988; 112(11): 1129-32.

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Department of Pediatric Surgery, Firat University Faculty of Medicine, Elazig, Turkey






Tugay Tartar, Department of Pediatric Surgery, Firat University Faculty of Medicine, Elazig, Turkey

Phone:+90 424 233 35 55


Cite this article as:

Tartar T, Sarac M, Bakal U, Canpolat S, Kazez A. Juvenile Granulosa Cell Tumor of The Testis in a Newborn with Swelling in the Scrotum: A Rare case and Literature Review. Erciyes Med J 2017; 39(2): 78-82.

[c]Copyright 2017 by Erciyes University Faculty of Medicine - Available online at
Table 1. Cases of juvenile granulosa cell tumor from the literature

                                                 AFP        HCG
Case                    Right/                 level,    level, mL
 No       Ref.      n    Left      Location     ng/mL      U/mL

 1        Giin      2   I. Left    Scrotum        N          N
       et al. (1)

                       II. Right   Scrotum      521.1        N

 2      Bulotta     1    Left      Scrotum      6679         8
       et al. (2)                               IU/mL

 3        Raju      1      ?        Intra-        ?          ?
       et al. (4)                 abdominal

 4       Leylek     1    Bil.       Intra-        ?          ?
       et al. (5)                 abdominal

 5      Peterson    1    Left      Scrotum     55.141        4
       et al. (6)

 6      Borislav    1    Right     Scrotum      7068        <2
      et al. (11)

 7        Levy      6      ?       Scrotum    23-61.700      ?
      et al. (12)
 8      Partalis    1    Left       Intra-        N          N
      et al. (13)                 abdominal

 9       Zugor      2  I. Right    Scrotum      35350        N
      Gt al. (14)

                       II. Left    Scrotum      9038         N

 10        Yu       1    Bil.       Intra         ?          ?
      Gt al. (15)                 abdominal

 11      Dudani     1      ?       Scrotum        ?          ?
      Gt al. (16)
 12   Y[??][??]maz  1    Bil.       Intra-        -          -
      Gt al. (17)                 abdominal

 13       Kos       1    Right      Intra-        -          -
      Gt al. (18)                 abdominal

 14       Chan      2  I. Right    Scrotum        -          -
      Gt al. (19)                 (3x3x2 cm)
                       II. Left   18x15x1 mm    27100        -

 15      Nistal     1      ?          ?           ?          ?
      Gt al. (20)
 16     Uehling     1    Right     Scrotum       450         -
      Gt al. (21)

Case    Additional            US
 No     pathology          and size              MRI

 1          -              Complex,               -
                          solid mass
            -             25x25x20 mm             -
                      haterogeneous solid
                       mass 25x16x25 mm
 2          -          Fetal (36th week)          -
                       USG: Multicystic
                        mass Postnatal
                          cystic mass
                         15.7x13.3 mm
 3    XXY(triploidy)           ?                  ?
 4      Chromosome        Very large          Bilateral
       4 deletion,       multicystic,           large
        hypotonia,       multiseptated      multiseptated
      micrognathia,         intra-             cystic
      low-set ears,        abdominal          abdominal
        downward-            mass              masses
       slanted eyes
 5          -               Complex               -
                        cystic mass in
                       the left testis-
 6          -          Cystic, solid and          -
                          22x16x15 mm
 7          ?                  ?                  ?

 8          -             Cystic mass
                      (cystic intestinal
 9          -           Multiple septic           -
                          cystic mass
                         (Swiss cheese
            _              6x6x4 cm
                        Multiple septic
                          cystic mass
                         (Swiss cheese
                           5x6x3 cm
                        (Prenatal mass)
 10    Chromosome 4            ?                  ?
       renal cysts
 11         -                  -                  -

 12     Cerebellar         Prenatal         MRI: multipe
        dysplasia        (18th week):        septated 2
      polymicrogyria        Normal           cystic mass
                          Postnatal:           Right:
                             large           8x5x5.5 cm
                         multiseptated          Left:
                         cystic intra-      10.5x7.5x9 cm
                        abdominal mass
 13   Cordocentesis:       Prenatal             USG:?
          69 XXY         (26th week):        multicystic
       (Triploidy)         multiple         serous fluid
          Inv(9)         malformations       containing
         (p11q13)       (spina bifida,          cysts
                         microcephaly,     (5.5x4.1x 5 cm)
                       abdominal tumor)
                        corpus callosum
                       lung and adrenal
                          hypoplasia              -
 14         -                  -                  -

            -          Multicystic mass           -
                          3x3x1.5 cm
 15     Testicular             ?                  ?
 16         -          Multicystic mass           -
                         Swiss cheese

Case                                  up
 No    Operation   Pathology  Met    time

 1        HSO        JGCT      -      -

          HSO        JGCT      -      -
 2        HSO        JGCT      -   5 years

 3         ?         JGCT      ?      ?

 4         -         JGCT      -      -

 5        HSO        JGCT      -

 6        HSO        JGCT      -      -

 7         ?         JGCT      ?      ?

 8    Laparotomy     JGCT      -      -
       9x9x7 cm

 9        HSO        JGCT      -   2 years

          HSO        JGCT      _   9 months

 10      Mass        JGCT      -      -

 11        ?         JGCT      -   6 months

 12   Laparotomy,    JGCT      -      -

 13        ?         JGCT      -      -

 14       HSO        JGCT      -      -

          HSO        JGCT      -      -

 15        ?         JGCT      ?      ?

 16       HSO        JGCT      -      -

US: ultrasound; MRI: magnetic resonance image; Bil: bilateral; EST:
endodermal sinus tumor; GCT: germ cell tumor; HSO: high scrotal
orchiectomy; JGCT: juvenile granulosa cell tumor; Met: metastasis; N:
normal; Ref: reference; (-): not specified in article patient details;
(?): unreachable patient details
No portion of this article can be reproduced without the express written permission from the copyright holder.
Copyright 2017 Gale, Cengage Learning. All rights reserved.

Article Details
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Title Annotation:CASE REPORT
Author:Tartar, Tugay; Sarac, Mehmet; Bakal, Unal; Canpolat, Senay; Kazez, Ahmet
Publication:Erciyes Medical Journal
Article Type:Case study
Date:Jun 1, 2017
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