King Edward Memorial Hospital - Department : Obstetrics and Gynaecology

Obstetrics and Gynaecology

Interesting Cases

Case of the Month : July 1999.

A 16-year- old unmarried girl, presented with a lump in the abdomen and low grade fever since one month. the patient had not yet attained menarche.

Physical Examination :

Revealed temporal baldness and a 16 weeks size mass palpable per abdomen. There was clitoromegaly (Fig 1) and a one finger-per vaginum/per rectal examination showed a 6 week size mass with restricted motility. The uterus could not be made out separately.


Fig 1	Fig 2

The hemoglobin was 10 gm% and the ESR was 112 mm at the end of the first hour. The Mantoux test was negative.

The chest radiograph and the ECG were normal

Pelvic Ultrasonography :

Revealed a smooth ,well defined, heterogenous pelvic mass 12.5 x 12 x 9.5 cm, in midline (suprapubic region), with calcification. The kidneys showed dilated pelvi-calyceal system with mild to moderate dilatation of upper ureters.

The CT Scan :

Showed a 9.5 x 10 x 13 cm mixed density mass in midline indenting the superior and left lateral surfaces of the bladder. The prevesical and ischiorectal fat showed evidence of multiple nodules suggestive of infiltration / lymphadenopathy. Mild hydroureters with hydronephrosis was present. The left renal function was more compromised than the right.

Exploratory Laparotomy :

Showed a highly vascular mass 12 x 12 x 6 cm, twisted around its connection to the round ligament. The left tube was lying attached to the mass. The uterus and the right tube were hypoplastic with a streak ovary . A left sided ovariotomy with right sided oophorectomy with partial omentectomy was done.

Histopathology Revealed :

Left gonadal mass showing focal clumps of coarse calcification representing "burnt out" gonadoblastoma. Also elements of dysgerminoma and immature teratoma are present.

Right sided streak gonad seen as cellular nests with coarse calcification representing a gonadoblastoma, amidst fibrous ovarian stroma.

Omentum negative for tumour.

On "fluorescent in situ hybridisation", the left sided gonad and buccal mucosa was positive for"y"chromosome.

The patient was then referred for chemotherapy.

First described by Scully in 1953, gonadoblastomas are a variety of germ cell tumours of ovary. They are almost always associated with gonadal dysgenesis .A Y chromosome or Y chromosomal fragment is invaiably present. The tumor is composed of germ cells with sex cord derivatives. There is a malignant potential ,half being seen in association with dysgerminomas.

Clinical Features :

Primary / secondary amenorrhea

Lack of secondary sexual characteristics

Infantile internal genitalia

Atrophic vaginal mucosa.

Prompt bilateral gonadectomy.

Etoposide with cisplatin or bleomycin.

References:

1. A case of gonadoblastoma in 52 cases of ovarian tumours in premenarchal girls over 16 years. Quint WH, et al. J Pediatr Adoles Gynecol 1999, 12, 27 - 29.

2. Four cases of gonadoblastoma in 78 cases of non-teratomatous germ cell tumours in children over 52 years .Bethel et al. J Pediatr Surg. 1998, 33, 1122 - 27.

3. A case of gonadoblastoma in ovarian tumours in children over 15 years Fabbro MA, et al. J Pediatr Med. 1996, 18, 151 - 154.

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