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Objective: To report three patients with persistent Müllerian duct syndrome (PMDS) associated with a unilateral testicular tumour., Patients and Methods: Three adults with PMDS and an associated testicular malignancy were evaluated using physical examination, imaging, measurement of tumour markers, surgical exploration and chromosome analysis., Results: The position of the uterus and fallopian tubes differed in all patients: one was in the scrotal sac, another in the abdomen and the third in the left inguinal canal. Two of the patients were cousins and their pedigree showed that they were probably in a sex-limited group. Both also had transverse testicular ectopia; fertility was documented in the younger patient. In all cases, the karyotype was proved to be 46, XY., Conclusion: We recommend that the diagnosis of PMDS is made radiologically and that the detection of Müllerian inhibiting factor is mandatory. As malignancy does not occur in the retained Müllerian ducts, hysterectomy should not be performed at abdominal exploration, although orchidectomy should be, because orchidopexy offers only limited protection against future malignancy if performed after 2 years of age. It is not necessary to perform testicular biopsy to detect tumour in the scrotal testis in this syndrome, because an impalpable tumour can be localized by ultrasonography.

The records of 273 patients with germ cell tumours of the testis referred between 1970 and July 1991 were reviewed. There were 25 (9%) black, 40 (14%) mixed race and 214 (77%) white patients. Histology showed seminoma in 53% and non-seminomatous and germ cell tumours in 47% of patients. Maldescent of the testis (MDT) was found in 30 patients--an incidence of 11% overall. MDT was present in 8 of 25 (32%) black, 7 of 40 (18%) mixed race and 15 of 214 (7%) white patients with testicular cancer. The incidence of MDT was statistically significantly higher in both black and mixed race patients compared with white patients. None of the black patients had undergone orchiopexy but 71% of mixed race and 87% of white patients had done so. This resulted in a different pattern of presentation in black compared with mixed race and white patients with MDT and testicular cancer. The mean age was 40 years for black, 32 years for mixed race and 33 years for white patients. Black patients presented with abdominal or inguinal tumours rather than scrotal tumours and they had an increased tendency to present with seminomas.

We report an analysis of 68 of 1112 couples who attended an infertility clinic where the man had past or present evidence of testis maldescent. The age at orchiopexy for these 68 men was later than would be expected, taking into account surgical practice in Scotland for the relevant time period and this supports the view that late orchiopexy is a risk factor for subsequent infertility. There was no evidence that antisperm antibodies are responsible for the damage to spermatogenesis in unilateral cases; however, measurement of the serum hormones showed elevations of LH and FSH which cannot be entirely explained as secondary to the damaged spermatogenesis. There was weak but nevertheless significant evidence that testis maldescent may be a poor prognostic factor as far as subsequent fertility is concerned and that this is independent of the length of time the couple have been trying and the man's motile sperm density. In two men there was intra-abdominal undescent and both were found to have testis tumours; we would re-emphasise that abdominal exploration is mandatory in such cases.

Twenty-six of approximately 1300 patients (1.9%) with unilateral testicular cancer developed a second primary germ cell cancer (seminoma 18; non-seminoma 8). Patients with previous seminoma had a significantly higher risk of developing a new seminoma than those with a previous non-seminoma. The diagnosis of second primary was made within 3 years of the first diagnosis in only 50% of the patients. In patients with a history of undescended and/or atrophic testes the interval significantly decreased between the diagnosis of the two testicular cancers. The prognosis of bilateral testicular cancer is generally good. Patients in whom the second testicular cancer is at clinical stage I (no metastases) at diagnosis can safely be observed without further treatment after orchiectomy. A patient with unilateral testicular cancer should be informed of the increased risk of developing a second primary germ cell tumour and should be encouraged to perform regular examination of the remaining testis. The need for life-long follow-up visits for patients with testicular cancer is questionable.

Two patients with Leydig cell tumours of the testis are described. Both presented with gynaecomastia, but on initial examination had apparently normal testes. The causative tumour did not become obvious for some months, despite being carefully sought. Review of the literature suggests that gynaecomastia preceding a readily palpable tumour is a recognised problem. The detection and management of Leydig cell tumours of the testis are discussed.

This study concerns 2043 male partners of infertile marriages who underwent testicular biopsy in the 28 years from 1955 to 1982. In a review of the pathological material carcinoma in situ (CIS) was diagnosed in 8 men (0.39%). Six of these men were later found to have invasive germ cell tumours; one has remained tumour-free and one is lost to follow-up. The implication of these findings in relation to the selection of patients for biopsy and the treatment of CIS when diagnosed is discussed.