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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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 www.erciyesmedj.com
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) Scrotum 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, - multiseptated, hypoechoic, intratesticular solid mass - 25x25x20 mm - Multicystic, intratesticular, haterogeneous solid mass 25x16x25 mm 2 - Fetal (36th week) - USG: Multicystic testicular mass Postnatal USG: encapsulated, complex cystic mass 15.7x13.3 mm 3 XXY(triploidy) ? ? ambiguous genitalia 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 - heterogeneous mass 22x16x15 mm 7 ? ? ? 8 - Cystic mass (cystic intestinal duplication?) 9 - Multiple septic - cystic mass (Swiss cheese appearance) _ 6x6x4 cm Multiple septic cystic mass (Swiss cheese appearance) 5x6x3 cm (Prenatal mass) 10 Chromosome 4 ? ? abnormality, polymicrogyria 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 agenesis lung and adrenal hypoplasia - 14 - - - - Multicystic mass - 3x3x1.5 cm 15 Testicular ? ? torsion 16 - Multicystic mass - Swiss cheese appearance (Teratoma?) Follow- Case up No Operation Pathology Met time 1 HSO JGCT - - HSO JGCT - - Frozen biopsy: EST/ JGCT 2 HSO JGCT - 5 years 3 ? JGCT ? ? 4 - JGCT - - 5 HSO JGCT - 6 HSO JGCT - - (Frozen: GCT) 7 ? JGCT ? ? 8 Laparotomy JGCT - - 9x9x7 cm 9 HSO JGCT - 2 years HSO JGCT _ 9 months 10 Mass JGCT - - resection 11 ? JGCT - 6 months 12 Laparotomy, JGCT - - hormone replacement therapy 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
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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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