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Dysgerminoma S Postgrad Med J: first published as 10.1136/pgmj.43.500.400 on 1 June 1967. Downloaded from Postgrad. med. J. (June 1967) 43, 400-405. Dysgerminoma S. A. SELIGMAN M.D., F.R.C.S., M.R.C.O.G. Luton and Dunstable Hospital IT IS generally accepted that the dysgerminoma is The cytoplasm is clear except where retracted by a tumour arising from the ovarian germ cells. It fixation. There is a striking picture of uniformity is identical with its male counterpart, the semi- of the cells which are arranged in an alveolar noma, and with tumours found in other areas pattern, being separated by fibrous septa infiltrated where germ cells may be present-retroperitoneally by lymphocytes. In some areas a columnar and in the thymus and pineal. The origin of this arrangement may predominate. There may be areas type of tumour from the spermatogonia of of necrosis and symplastic giant cell formation. atrophic tubules has been demonstrated in dogs The clinical features of dysgerminoma have (Scully & Coffin, 1952) although these tumours, been studied in a series from the Tumour Registry whilst similar in some aspects to the classical of the Royal College of Obstetricians and Gynae- human seminoma, in others resemble the human cologists and compared with previous important spermatocytic seminoma (Scully, 1961). Histo- collections of cases (Novak & Gray, 1938; Seegar, logical relations of the dysgerminoma indicate an 1938; Sjovall, 1943; Santesson, 1947; Mueller, origin from oocytes of the normal female germinal Topkins & Lapp, 1950; Pedowitz, Felmus & lineage (Hughesdon, 1959). Grayzel, 1955; Thoeny et al., 1961; Koller & The typical histological pattern is easily recog- Gjonnaess, 1964; Pece, 1964). copyright. nizable, with large nucleii distorted from their The series, up to the end of 1965, contained basic spherical shape, with one or more nucleoli. twenty-nine cases. One of these was combined with http://pmj.bmj.com/ on September 28, 2021 by guest. Protected FIG. 1. Dysgerminoma. Postgrad Med J: first published as 10.1136/pgmj.43.500.400 on 1 June 1967. Downloaded from Dysgerminoma 401 a teratoma, the patient dying of leukaemia Some of the women are admitted in an emer- (Chalmers, 1950). This tumour behaved differently gency, either with abdominal pain due to accidents from the rest, the teratomatous element pre- to the tumour, or, as two of the Tumour Registry dominating, and has been excluded from this cases were, because of acute retention of urine. series. It is of interest that one of the patients with There can also be difficulty with micturition of a combined teratoma and seminoma studied by less dramatic onset. the Testicular Tumour Panel and Registry also Other less frequent symptoms were: upset bowel died of leukaemia (Collins & Pugh, 1964). habit, either constipation or diarrhoea, dysmenorr- hoea, general malaise. In two patients the tumour Clinical features was found antenatally (Bigby, 1961). Age incidence (Table 1) The tumour is principally one of young adults. TABLE 3 In about 70% of cases the age of onset is between Symptoms 10 and 30. It is rare after the menopause and in 210 cases from Twenty-six cases girls in whom it occurs before the reproductive age Symptoms literature from Tumour is often accompanied by hormonal changes. (%) Registry (%) Lump 35 58 TABLE 1 Pain 36 46 Age incidence Lump + pain - 27 Menstrual upset - 19 Twenty-eight cases Emergency 14 15 Age (years) 500 cases from from Tumour Urinary upset - 12 literature (%) Registry (%) 0-10 7 4 Ovary involved (Table 4) 10-20 37 25 The two ovaries are not involved to an equal 20-30 34 46 extent, the and left ovaries affected in right being copyright. 30-40 12 21 the ratio of 5 : 4. This is the same 40-50 5 4 as for involve- 50+ 5 0 ment of the testes with seminoma and is evidence against some of the bizarre theories advanced to explain this discrepancy in the male. It is probably Duration of symptoms (Table 2) of more relevance that in most normal female This is a rapidly growing tumour, usually bring- birds the right gonad is a rudimentary structure as ing the patient to hospital within weeks of the a result of an organ specific inhibitory substance onset of symptoms. It may, however, be asympto- in the left produced gonad (Gardner, Wood & http://pmj.bmj.com/ matic, being found on routine antenatal examina- Taber, 1964). tion or during the investigation of primary In some cases bilateral ovarian involvement is amenorrhoea. found at operation or on histological examination. TABLE 2 In only one case in the series was the apparently Duration uninvolved ovary later the seat of a dysgerminoma, of symptoms after an interval of 2 years. An ovarian biopsy Duration of Sixty-nine cases Twenty-six cases 6 months after the original operation had not symptoms from literature from Tumour revealed any evidence of involvement at that time. on September 28, 2021 by guest. Protected (%) Registry (%) Acute 15 8 TABLE 4 <3 months 54 57 Ovary affected 3-6 months 7 19 6-12 months 10 8 500 cases from Twenty-seven cases > 12 months 7 0 Side literature from Tumour Registry Asymptomatic 7 8 (%) (%) Right 50 44 Symptoms (Table 3) Left 36 37 The commonest presenting symptoms are an Bilateral 14 19 abdominal mass, abdominal pain, or both. Although the tumour is generally regarded as being Mortality (Table 5) hormonally inactive, about 20% of patients had In most patients who die from dysgerminoma, some menstrual upset. Precocious puberty or the the progress of the disease is rapid and they suc- onset of vaginal bleeding in patients with primary cumb within 1 year, after which the death rate amenorrhoea may be the presenting symptom. subsides, but even a 5-year interval of freedom Postgrad Med J: first published as 10.1136/pgmj.43.500.400 on 1 June 1967. Downloaded from 402 S. A. Seligman does not guarantee cure. One patient in the series to be involved at operation in three cases in the developed metastases and died 6 years after the series and the Fallopian tube once, indicating that original operation. In Table 5, percentages are of if conservative surgery is not contemplated the cases surviving. uterus should be removed with both tubes and ovaries. In one patient death was associated with TABLE 5 rupture of an infiltrated bladder. Post-operative survival rates The sites of metastatic spread are difficult to 237 cases from Cases from Tumour define owing to the paucity of post-mortem reports Years literature (%) Registry (%) in the literature and the position with regard to seminoma in the male is little better. Clinical 1 55-5 71 (from 23 cases) reports cannot usually give the precise origin of 5 41 59 (from 17 cases) deeply sited masses. Apart from the patient with the combined teratoma and dysgerminoma, only Survival is markedly related to the extent of the one post-mortem was performed in the series. disease at operation (Table 6). Where this is limited Common sites of metastasis appear to be along to one ovary, the outlook is good, but where the the aortic chain of lymph nodes and in the supra- tumour has spread, and this includes bilateral clavicular nodes. Any of the abdominal viscera ovarian involvement, the outlook is poor. may be involved, particularly the liver and kid- neys. Lungs, pleura and mediastinum are the sites TABLE 6 of frequent metastasis, whilst the vertebrae in all Effect of extent of disease on 5-year survival rate regions of the spine seem particularly prone to 215 cases from Seventeen cases from involvement with large malignant masses and Extent of literature Tumour Registry consequent paralysis. disease (%) (%) Limited 64 90 Tumours of dysgenetic gonads Spread 23 14 Robert Meyer (1931) who first suggested the copyright. name disgerminoma (spelled dis-) found the The presence of bloody ascites at operation is of tumour to occur in twenty-seven intersex patients grave import, clear ascites not necessarily so but only twenty-one apparently normal females. (Table 7). This is overemphasized in the Tumour This ratio has not been confirmed. In the Tumour Registry series due to the small numbers involved. Registry series four patients had intersex states. One had a mixed gonadal dysgenesis, the chromo- TABLE 7 somal complement of patient and tumour being Effect of ascites on 5-year survival rate of the normal male type (Seligman, 1967). The http://pmj.bmj.com/ precise nature of the intersex condition in the other Thirty-one cases Six cases from three is not known, but testicular tissue was Type of from literature Tumour Registry present ascites in one and typical dysgenetic gonads with calcifi- (%) (%) cation in another. Clear 33 100 In 1953, Scully described a gonadal tumour, Bloody 12-5 0 which he named a gonadoblastoma, in part dys- germinoma but elsewhere composed of Sertoli or on September 28, 2021 by guest. Protected The operative findings give a good indication granulosa cells and Leydig or theca cells, and of the prognosis. Those patients who at operation associated with masculinization. He refers to these are found to have growths which have spread cells as respectively of sex-cord and mesenchymal beyond one ovary, with haemorrhagic ascites, origin although the embryological derivation which adhesion or rupture of the tumour, are killed by he gives is not universally accepted (Richardson, their disease usually within weeks of operation and 1966) and the female cells morphologically corre- almost invariably within 3 years, and in this group sponding to the Leydig cells are the ovarian hilus of patients the tumours do not readily respond to cells. radiotherapy. In patients with a solitary encap- The gonadoblastoma probably always arises in sulated tumour, not adherent and with no ascites, tissues of testicular origin containing the Y there is a good prognosis, most recurrences being chromosome (Teter et al., 1964b), the gonads being extremely radiosensitive and radiocurable.
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