Aust. N.Z.J. Obstet. Gynaec. (1979) 19: 52
A Case of Dysgerminoma-Gonadoblastoma in Pure Gonadal Dysgenesis J. Foulkesl" and P. E. Nicholls2 Department of Obstetrics and Gymecology and Histopdhology, Westminster Hospital, London
Summary: A case is described in which the diagnosis of dysgerminomagonadoblastoma in an 18-year-old phenotypical female with primary amenorrhoea was initiated by biopsy of an ovary which appeared normal at laparoscopy.
Gonadoblastoma is a rare tumour, which almost invariably arises in an abnormal gonad. In about one-third of the cases they are bilateral. In appearance, they are usually rounded with a smooth or bossed surface and vary in size from microscopic up to 10 cm in diameter. Larger tumours usually contain another element such as a dysgerminoma. The incidence is difficult to define since many of the reported cases have been described in insufficient detail to permit their inclusion in this category of tumour. Histologically, the gonadoblastoma contains germ cells with smaller cells of epithelial type which resemble immature Sertoli and granulosa cells. In two-thirds of the cases examined by Scully (1970) elements indistinguishable from Leydig or luteumtype cells were also present. CASE REPORT
The patient was aged 18 years and presented with primary amenorrhoea. Investigation elsewhere
at the age of 16 years had revealed a normal but infantile uterus, and gonadotrophin levels were shown to be low. At this stage a diagnosis of delayed menarche had been made, and a course of clomiphene citrate had been given without effect. On examination, the patient was a phenotypic female of normal weight, height and span, with normal secondary sexual development which had occurred in the usual sequence and time, with the exception of menstruation. There was no evidence of virilization. There were no siblings or relevant family history. The endocrine profile showed raised gonadotrophins and low oestrogen excretion (table 1). Examination under anaesthesia and laparoscopy were performed. The uterus and Fallopian tubes were unremarkable; the ovaries were normal in site and size but had smooth surfaces and looked inactive (figure 1). A punch biopsy was taken from the left ovary, and muscle and blood were taken for karyotyping. The biopsy showed the typical
Table 1. Pre and Post Gonadectomy Endocrine Profiles
Serum
L.H.
Serum F.S.H.
Serum prolactin
G/1)
0.1 1)
P6/1
29 40
48 40
Urinary oestrogen nmo1/24 hr
Plasma oestradiol pmol/l
Plasma testosterone nmol/l
Plasma cortisol nmol/l
32
1.4
140 240
~
Before operation 10 days after oueration
11 8
8
1. Senior Registrar. rp 2. Lecturer. * Present address: Plymouth General Hospital, Devon.
15
70
A Case of Dysgerminoma-Gonadoblastoma in Pure Gonadal Dysgenesis J. Foulkesl" and P. E. Nicholls2 Department of Obstetrics and Gymecology and Histopdhology, Westminster Hospital, London
Summary: A case is described in which the diagnosis of dysgerminomagonadoblastoma in an 18-year-old phenotypical female with primary amenorrhoea was initiated by biopsy of an ovary which appeared normal at laparoscopy.
Gonadoblastoma is a rare tumour, which almost invariably arises in an abnormal gonad. In about one-third of the cases they are bilateral. In appearance, they are usually rounded with a smooth or bossed surface and vary in size from microscopic up to 10 cm in diameter. Larger tumours usually contain another element such as a dysgerminoma. The incidence is difficult to define since many of the reported cases have been described in insufficient detail to permit their inclusion in this category of tumour. Histologically, the gonadoblastoma contains germ cells with smaller cells of epithelial type which resemble immature Sertoli and granulosa cells. In two-thirds of the cases examined by Scully (1970) elements indistinguishable from Leydig or luteumtype cells were also present. CASE REPORT
The patient was aged 18 years and presented with primary amenorrhoea. Investigation elsewhere
at the age of 16 years had revealed a normal but infantile uterus, and gonadotrophin levels were shown to be low. At this stage a diagnosis of delayed menarche had been made, and a course of clomiphene citrate had been given without effect. On examination, the patient was a phenotypic female of normal weight, height and span, with normal secondary sexual development which had occurred in the usual sequence and time, with the exception of menstruation. There was no evidence of virilization. There were no siblings or relevant family history. The endocrine profile showed raised gonadotrophins and low oestrogen excretion (table 1). Examination under anaesthesia and laparoscopy were performed. The uterus and Fallopian tubes were unremarkable; the ovaries were normal in site and size but had smooth surfaces and looked inactive (figure 1). A punch biopsy was taken from the left ovary, and muscle and blood were taken for karyotyping. The biopsy showed the typical
Table 1. Pre and Post Gonadectomy Endocrine Profiles
Serum
L.H.
Serum F.S.H.
Serum prolactin
G/1)
0.1 1)
P6/1
29 40
48 40
Urinary oestrogen nmo1/24 hr
Plasma oestradiol pmol/l
Plasma testosterone nmol/l
Plasma cortisol nmol/l
32
1.4
140 240
~
Before operation 10 days after oueration
11 8
8
1. Senior Registrar. rp 2. Lecturer. * Present address: Plymouth General Hospital, Devon.
15
70
