CASE REPORT

Niger J Paed 2012; 39 (4):199 –201

Mava Y

Childhood ovarian juvenile

granulosa cell tumour: a case report

and review of literature

Chinda JY

Alhaji MA

Nggada HA

DOI:http://dx.doi.org/10.4314/njp.v39i4,11.

Accepted: 12th May 2012

Abstract Juvenile granulosa cell

tumour (JGCT) is very uncommon

gynecological malignancy that oc-

curs more commonly in under five

years old of age. We describe a case

of JGCT in a 4-years old girl. The

malignancy is assigned to Interna-

tional Federation of Gynecology

and Obstetric staging system (FIGO

stage I). Treated with complete ex-

cision only, the patient showed no

evidence of relapse one year after

surgery.

Findings in this case are discussed

and histological examination con-

firmed the diagnosis. The natural

history of JGCT, epidemiology,

histology, treatment and prognosis

are reviewed along with the case

presentation.

(

)

Mava Y

Alhaji MA

Department of Paediatrics

Chinda JY

Department of Paediatrics singun

Nggada HA

Department of Histopathology,

University of Maiduguri Teaching

Hospital, Maiduguri Nigeria.

Email: yakubumara@gmail.com

Tel: +2348036301748

Key words: Childhood, Juvenile

Granulosa Cell, Tumour, Ovary

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Introduction

adult male testes, though very rare. Majority of J₉GCT

present as localized disease confined to the ovary, and

usually behave in a benign manner desp₄_,i₉te having histo-

Ovaria₁n JGCT is extremely rare sex cord-stromal tu-

mour. It comprises only 5% of ovarian tumours of

pathological features of malignancy.

advanced JGCT and poorly differentiated sertolic cell

tumours are considered prognostically poor. Histopa-

thologically, ovarian follicles are irregular in size and

shape, leutenization occurs and the nuclei are immature,

atypical and have a high mitotic rate. Exner bodies are

class₁i₀c features. These are grooved, pale and round nu-

clei. A positive immunohistochemical stain for inhibin,

Conversely,

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childhood or adolescence. Mohammed et al from

Zaria Northern Nigeria reported only two cases of JGCT

among children 15 years and bellow in a 25 years re-

view of ovarian malignancies in childhood. Typically

they present as sexual precocity in prepubertal girls due

to excessive estrogen product₂ion. In some rare cases

androgens may be produced. JGCT is different from

adult granulosa cell tumour (AGCT) that is seen in older

females with respect to clinical and₄ pathological features

as well as biological behaviours. Common symptoms

include abdominal swelling, abnormal uterine bleeding,

appearance of acne, breast enlargement and occasional

facial hair appearance. There are no known risk factors

for this tumour but recently, associations with changes

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an ovarian glycoprotein is a key diagnostic feature.

Various modalities of treatment have been used ranging

from surgical removal of the tum₅o_,u₁₀r to chemotherapy,

radiotherapy and other treatments.

In adolescent girls

and adult females, fertility sparing surgery can be done.

Some have used platinum agent as standard treatment,

either combined with Vinblastine and₄_, B₅_,l₁e₀omycine or

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in certain chromosomes were suspected. Such changes

include Gas-activating mutations in hot spots position

Adriamycine and Cyclophosphomide.

Other au-

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exclusively localized in granulosa cell tumours.

01. Specifically, mutations R201C and R201₆H were

thors now recommend the use of Bleomycin, Eptoposide

and Platinum. Some studies suggested that patients aged

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toring for tumour recurrence only. Others have used

stem cell transplantation. Newer agents that block an-

giogenesis are being studied; two are being tried

currently; sunitinib and bevacizumab. Hormone based

9 years and below should have sur₄g_, e₁₀ry and close moni-

Juvenile granulosa cell tumour a subtype of ovarian stro-

mal cell tumours, has a favourable prognosis if diag-

nosed at early stage. Recurrences are uncommon and

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typically occur within the first year. The incidenc₃_,e₈ of

this group of tumour is same throughout the world.

treatments like paclitaxel and taxane in₀ combination

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with platinum are also being advocated. The use of

A more serious estrogen effects can occur in various end

organs such as endometrial hyperplasia, endometria₈l

adenocarcinomas and increased risk of breast cancers.

Granulosa cell tumours can occur in the juvenile and

radiotherapy has not been shown to confer any survival

benefit to JGCT at any stage. This case report is reported

in a four year old girl to highlight clinical presentation,

histologic characteristics of this rare tumor to remind

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clinicians the necessity to achieve correct diagnosis and

proper selection of treatment protocol.

encapsulated partly cystic mass measuring 14 X 14 X

9.5cm. The cut surface showed multilocular cyst con-

taining gelatinous materials as well as solid grey white

areas. Microscopically the solid section of the tumour

showed some neoplastic cells which were arranged in

trabecular, insular, diffuse and microfollicular (Call-

Exner bodies) patterns separated by thin fibrous septae.

The nuclei are relatively uniform with abundant cyto-

plasm. The tumor cells are lacking nuclei grooves

(coffee-bean appearance). (Fig 1b) These features are

consistent with JGCT. Patient did well postoperatively

and regular follow-up for one year showed weight gain,

regression of breast enlargement and no evidence of

relapse. Patient was not on cytotoxic drugs or any other

adjuvant therapy.

Case report

A four year old girl presented with a two month history

of progressive abdominal swelling, one month history of

fever and one week history of cough. There was associ-

ated progressive weight loss, no history suggestive of

tuberculosis. Painless breast enlargement preceded the

abdominal swelling; there was an associated abdominal

pains but no history of vaginal bleeding. Examination

revealed bilateral breast enlargement (Fig 1a) and galac-

torrhoea in either breasts but no adrenarche or signifi-

cant peripheral lymphadenopathy.

Fig 1a: Bilateral breast enlargement with galactorrhoea

in a four year old girl with JGCT

Fig 1b: Photomicrograph of Juvenile granulosa cell tu-

mour lacking the “nuclei grooves” (coffee-bean appear-

ance) in a four year old girl. H&E X180.

Sections from the ovarian mass show polygonal tumour cells

arranged in trabecular, insular and microfollicular (Call-Exner

bodies) patterns. The cells nuclei are relatively uniform with

abundant cytoplasm. The tumour cells are lacking nuclei

grooves (coffee-bean appearance).

The abdomen was uniformly distended with firm multi-

lobulated masses with cystic areas and well defined up-

per border. There was no ascitis and her blood pressure

was normal. A clinical diagnosis of Wilms’ tumour was

made with differential diagnoses of abdominal Burkitt

lymphoma and abdominal tuberculosis. Investigation

results showed a packed cell volume of 26% with pe-

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Discussion

ripheral leucocytosis of 15 X 10 /L and neutrophilia of

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4%. Peripheral blood film showed toxic granulations:

Granulosa cell tumours are rare sex cord stroma tum₁_,o₂r_, s₁₀;

incidence varies from 1-5% of all ovarian tumours.

A study in Zaria reported 2.1% prevalence of JGCT in

children less than 16 years. These tumors are divided

into JGCT and AGCT: JGCT often occurs within the

ESR was 50mm/hour and Mantoux test was negative.

Serum electrolytes were within normal limits. Abdomi-

nal radiography showed no calcification. Abdominal

ultrasound scan showed intraabdominal masses with

multiple solid and cystic components but liver, spleen

and kidneys were normal. Fine needle aspiration cytol-

ogy showed features suggestive of a round blue cell tu-

mour of childhood with possibilities of nephroblastoma,

neuroblastoma and embryonal rhadomyosarcoma. The

patient had exploratory laparatomy which showed huge

left tubo-ovarian mass, cystic, multilobulated with well

developed arterial supply from the surrounding caecum,

transverse colon and the small intestine. The peritoneum

was free: there was no ascites and no lymph node

enlargement. The liver and spleen were normal.

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first three years of life and cases_,₉have been reported

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even in a four months old baby. The index patient

presented at the age of four years which fal₈ls_,₉within the

age at which JGCT presents in children.

She pre-

sented with abdominal swelling, breast enlargement, and

abdominal pains these were c₁o_,n₁₀s_,i₁s₁tent with various

studies reported in the literature.

Dysfunctional uterine bleeding and menstrual irregular₁i-₀

ties are frequently seen in women of reproductive age.

Thus it is not surprising that despite breast enlargement

and galactorrhoea, the patient herein reported did not

present with uterine bleeding. Rare cases of JGCT have

The patient was offered excisional biopsy of the tumour

(

left oophrectomy). Histopathological report revealed an

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been reported to secrete androgen causing virilization.

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Also, other cases have been associated with Maffuccis

syndrome and enchondromatosis (Ollier’s disease).

of the disease. Nevertheless, a variety of chemother₃a_,₄-_,

peutic regimens have been suggested in the literature.

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, 10-12

The patient in our report had no evidence of virilization

or any other associated condition. Our patient was in

stage one disease as the tumor was well capsulated, re-

gional lymph₁₀_,n₁o₁ des were normal and there was no peri-

These include; Cisplatin-based multi drug regimen

effective in advanced stage of JGCT, Hormone based

treatment with Pacilitaxel and Taxane in combination

with Platinum based drugs. Some have used radiother-

apy as adjuvant treatment, but other authorit₄i_,e₁s₀ indicated

that this does not have any advantage at all.

toneal spill.

The histological report of this patient

was consistent with JGCT, though immunohistochemi-

cal staining for inhibin₁a_,n₁d₀ smooth muscle actin is posi-

tive in almost all cases.

These tests could not be done

Stage one tumours carry favourable prognosis with five-

year survival between 95-100%. Resection of stage one-

tumour is considered to be curative. Our patient has

been followed up for one year now, and so far she is

doing well₁.₀ Relapse in JGCT usually occurs within th₁e₀

first year. Some advocate follow-up to three years

but recent reports have shown late recurrence of JGCT

in our patient due to lack of reagents which could have

given more distinctive findings of JGCT and also helps

in monitoring for relapse. Computerized tomographic

scan and magnetic resonance imaging could have been

useful in diagnosis, but are very expensive.

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Some auth₁_,o₂r_,₄it_, i₉es advocate surgical therapy alone for

up to four years later. These suggest that long term sur-

stage one.

In fact others suggest that adjuvant che-

veillance is highly necessary for this patient. Tumour

markers such as inhibin if possible should be used to

assess recurrence in addition to other clinical and labora-

tory investigations for patients.

motherapy may not₁be necessary if the tumour can be

excised completely. This is the treatment strategy

adopted for our patient since she came in early stage one

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