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 Table of Contents  
Year : 2021  |  Volume : 8  |  Issue : 2  |  Page : 136-138

Hormone secreting juvenile granulosa cell tumor of the ovary in an infant presenting with massive ascites and precocious pseudopuberty

1 Department of Paediatric Surgery, B J Government Medical College, Pune, Maharashtra, India
2 Department of Pathology, B J Government Medical College, Pune, Maharashtra, India

Date of Submission27-Apr-2020
Date of Decision12-Jun-2020
Date of Acceptance21-Aug-2020
Date of Web Publication27-Oct-2021

Correspondence Address:
Minakshi Bhosale
G/101, Sudarshan Apartments, Behind Spencer's Daily, Karvenagar, Pune - 411 052, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/cjhr.cjhr_39_20

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Juvenile granulosa cell tumors (JGCTs), account for 4–5% of GCTs, the rare sex cord stromal tumors. Presentation of JGCTs in infants is even rarer. We present case of a 3-month-old female infant with abdominal distension, feeding intolerance and fever of 10 days' duration. The child had gross abdominal distension causing visible respiratory distress. She also had signs of isosexual precocious puberty. Radiological evaluation was suggestive of left ovarian tumor. Left oophorectomy with preservation of the fallopian tube was done after hemodynamic stabilization. Histopathology evaluation of the tumor was suggestive of JGCT, which was confirmed on immunohistochemical evaluation. On 1 year follow-up, the child is stable, has achieved age-appropriate milestones and is tumor-free. Parents have been explained importance of regular follow-up to detect tumor recurrence. This is probably the youngest case of JGCT presenting with precocious puberty reported in literature so far.

Keywords: Infant, inhibin, juvenile granulosa cell tumor, ovary, precocious pseudopuberty

How to cite this article:
Bhosale M, Kudrimoti J, Dangmali D. Hormone secreting juvenile granulosa cell tumor of the ovary in an infant presenting with massive ascites and precocious pseudopuberty. CHRISMED J Health Res 2021;8:136-8

How to cite this URL:
Bhosale M, Kudrimoti J, Dangmali D. Hormone secreting juvenile granulosa cell tumor of the ovary in an infant presenting with massive ascites and precocious pseudopuberty. CHRISMED J Health Res [serial online] 2021 [cited 2022 May 20];8:136-8. Available from: https://www.cjhr.org/text.asp?2021/8/2/136/329446

  Introduction Top

Granulosa cell tumors (GCTs) of ovary, i.e., sex-cord stromal tumors, comprising 2–5% of all ovarian neoplasms were first described in 1985 by Rokitansky.[1] They are known to have unusual clinical presentation, unpredictable biological behavior and are poorly understood tumors. They are known to have multifaceted clinical presentation and late relapses.[2]

  Case Report Top

Three-month-old girl was brought with abdominal distension of 10 days' duration. She also had high-grade fever, feeding intolerance, and whitish discharge per vaginum. She was a full-term normal delivery, second progeny of nonconsanguineous marriage. Antenatal period was uneventful. The mother had not received any hormonal agents during the antenatal period. The child was pale and irritable. She had tachypnea with respiratory rate of 50/min. Heart rate was 130/min. She had gross abdominal distension [Figure 1] (abdominal girth of 48 cm), but no lump was palpable. Vaginal mucosa appeared protruding [Figure 1]. Signs of precocious breast development (Tanner stage B2) were obvious.
Figure 1: Clinical photograph showing distended abdomen, precocious breast development, and protruding vaginal mucosa

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Her hemoglobin was 9 g%. Serum electrolyte, creatinine, alpha fetoprotein, and B-HCG levels were normal. CA-125 value was 160 units/ml and LDH was 1000 IU/L. Ultrasonography abdomen showed a large solid cystic left ovarian mass and gross-free fluid with multiple echoes. Computed tomography scan of abdomen and pelvis following administration of oral and intravenous contrast showed enhancing mass of 5.2 cm × 5.7 cm × 6.3 cm size with solid and cystic components [Figure 2]. There was free fluid within the abdomen and pelvis, but no lymphadenopathy. On exploratory laparotomy through Pfannenstiel incision, ~900cc blood tinged ascitic fluid with froth was drained. 7 cm × 6 cm × 6.5 cm sized solid-cystic left ovarian mass was found [Figure 3] with a small rent on the capsule causing seepage of fluid within the peritoneal cavity. The left fallopian tube was inflamed. Uterus and right ovary were normal. Liver, spleen, both kidneys, and omentum were normal. Left oophorectomy was done with preservation of the left fallopian tube. Abdomen was closed in layers using 3-0 polyglactin. Skin closure was done using 5-0 ethilon. Blood loss of ~50 cc was replaced on table. The excised tumor was a solid mass 6.5 cm × 6 cm ×5.5 cm with whitish, smooth external surface. On cut section, it was solid, multilobulated, grayish white in color with areas of cystic change. It was sent for histopathology evaluation.
Figure 2: Computed tomography scan suggestive of large tumor mass with solid and cystic components. Hollow arrow points out ascitic fluid. Solid arrow points to ovarian mass

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Figure 3: Intraoperative photograph showing large solid-cystic left ovarian tumor

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Oral feeding was started after 72 h and gradually advanced once tolerated. There were 1–2 episodes of vaginal bleeding 24 h postoperatively. There was no wound infection. Suture removal was done on postoperative day 10. Histopathology report showed tumor composed of round to oval cells arranged mainly in diffuse pattern and macrofollicular pattern at places. Cells had round to oval nuclei with prominent nucleoli and ill-defined eosinophilic cytoplasm. This picture is suggestive of juvenile granulosa cell tumor (JGCT) of the ovary [Figure 4]a. Immunohistochemistry report showed diffuse tumor immunoreactivity for inhibin and vimentin and focal positivity for calretinin, confirming the diagnosis [Figure 4]b, [Figure 4]c, [Figure 4]d.
Figure 4: (a) Light microscopy picture showing tumor composed of round to oval cells arranged in diffuse, macrofollicular pattern (H and E, stain). (b) Tumor cells showing diffuse immunoreactivity for Inhibin. (c) Tumor cells showing diffuse immunoreactivity for vimentin. (d) Tumor cells showing focal positivity for calretinin

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Presently, at follow-up of 1 year, the child is asymptomatic and thriving well. She has achieved age-appropriate milestones and is on regular follow-up to detect tumor recurrence.

  Discussion Top

Primary ovarian malignancies are classified into surface epithelium, germ cell, or sex cord tumors, based on cell type of origin. Sex cord tumors account for 1–2% of ovarian malignancies and 5% occur in prepubescent patients. Approximately 70% of sex cord tumors are GCTs. These tumors arise from granulose cells, the hormonally active stromal elements in close association with ovarian oocytes, which are responsible for the production of estradiol. Increased estrogen levels cause isosexual precocious puberty in prepubescent girls.[3] Juvenile GCT is the rare histologic subtype of GCTs. Most of the JGCTs in children present with clinical evidence of precocious pseudopuberty manifested by breast development, pubic and axillary hair growth, vaginal secretions, irregular uterine bleeding and other secondary sexual characteristics, because of increased estrogen levels. Surgery is the primary modality of therapy in the management of GCTs (including the juvenile subtype).[2] It enables tissue diagnosis, staging and debulking. Surgical tumor removal typically includes ipsilateral salpingo-oophorectomy. The role of chemotherapy or radiation therapy remains uncertain due to lack of prospective randomized trials supporting their roles as adjuvant agents because of overall rarity of these tumors.[1] Although they are thought to be benign, recurrences are documented. Prognosis depends on surgical stage at presentation and is favorable when the tumor presents during infancy, than in late childhood. Cases diagnosed at Stage I, enjoy a favorable prognosis; whereas patients diagnosed at Stages II through IV tend to have poor clinical outcomes with a higher rate of disease recurrence.[2] Our case can be categorized as stage IC as per FIGO staging criteria of carcinoma ovary, though the ascitic fluid did not show any malignant cells.[4]

Young et al. have described tumor rupture in 13 (11%) out of 125 JGCTs of the ovary. Rupture had occurred before or during operation and ascites was present in 11 cases (9%). Eighty-two percent of the prepubertal patients presented because of isosexual pseudoprecocity.[5] Hashemipour et al. have reported case of a 6-year-old girl with isosexual pseudoprecocity.[6] Our case, a 3-month-old female infant had features of isosexual pseudoprecocity. There was preoperative tumor rupture identified by rent on capsule of the ovary causing seepage of fluid and ascites, leading to gross abdominal distension and respiratory distress. Postoperative episodes of vaginal bleeding can be explained by withdrawal of hormone secretion after excision of ovarian tumor. Left oophorectomy with preservation of the left fallopian tube was carried out in our patient keeping in view her tender age and future prospects of (assisted) fertility. This approach is similar to the one employed by Kanthan et al. in a 7-day-old neonate with a large complex cystic mass and should be encouraged.[7]

Histological examination of the resected specimen requires a great deal of expertise since age of presentation, treatment modalities, fertility issues, and overall prognosis of this tumor differs from other ovarian tumors. Immunohistochemical (IHC) markers are specifically helpful in this rare condition since JGCTs express unique cell surface markers which can be detected by staining the sample tissue. Surface expression of inhibin appears to have the greatest pathologic diagnostic potential for this tumor type.[6] Our case was positive for inhibin and vimentin focal positivity for calretinin. This is probably the youngest child of JGCT presenting with precocious puberty reported in literature so far. This case is presented to create awareness about the entity, need of IHC evaluation for diagnosis and need of long-term follow-up to detect early tumor recurrence. With the small number of cases reported till now, it will be a valuable addition to existing database.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the parents have given their consent for her images and other clinical information to be reported in the journal. The parents understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Schumer ST, Cannistra SA. Granulosa cell tumor of the ovary. J Clin Oncol 2003;21:1180-89.  Back to cited text no. 1
Colombo N, Parma G, Zanagnolo V, Insinga A. Management of ovarian stromal cell tumours. J Clin Onco 2007;25:2944-51.  Back to cited text no. 2
Ali S, Gattuso P, Howard A, Mosunjac MB, Siddiqui MT. Adult granulosa cell tumor of the ovary: Fine-needle-aspiration cytology of 10 cases and review of literature. Diagn Cytopathol 2008;36:297-302.  Back to cited text no. 3
Odicino F, Pecorelli S, Zigliani L, Creasman WT. History of the FIGO cancer staging system. Int J Gynaecol Obstet 2008;101:205-10.  Back to cited text no. 4
Young RH, Dickersin GR, Scully RE. Juvenile granulosa cell tumor of the ovary. A clinicopathological analysis of 125 cases. Am J Surg Pathol 1984;8:575-96.  Back to cited text no. 5
Hashemipour M, Moddah MH, Nazem M, Mahzumi P, Salek M. Granulosa cell tumour in a six year old girl presented with precocious puberty. J Res Med Sci 2010;15:240-42.  Back to cited text no. 6
Kanthan R, Senger JL, Kanthan S. The multifaceted granulosa cell tumours-myths and realities: A review. ISRN Obstet Gynecol 2012;1-12. doi:10.5402/2012/878635.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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