PARAlTSTICuLAR TUMOURS

By GRANT WILLIAMS, M.Sc., F.R.C.S., and R. BANERJEE, M.R.C.P.

The Institute of Urology and The London Hospital, London, E.1

THE term “ paratesticular ” is used to distinguish those intrascrotal tumours which arise from the epididymis, spermatic cord and its coverings from the much larger group in which the testis is the primary site of the tumour.

Although some of these, the so-called adenomatoid tumours of the epididymis, arise from the epithelium of the Mullerian vestiges (Sundarasivarao, 1953), the majority are connective tissue tumours. They are all sufficiently rare for many to be recorded in the literature as single cases or small series from which it is difficult to appreciate their surgical pathology.

Cloquet (1819) is quoted by Brockow and Gummess (1951) as recording the first case of a tumour in the spermatic cord, but Walsham (1879) quoted three cases of John Hunter, three of Curling, one of Goodhart and one of Willet. These may antedate Cloquet’s case. Willet’s patient was a boy aged 5 months and we would now infer that the “ round and fustiform sarcoma ” which he diagnosed was probably a rhabdomyosarcoma. It is probable that Walsham’s ‘‘ myxosarcoma of cord ” was also a turnour of this type.

Rokitansky (1849) is quoted by Neuman (1892) as describing one of the early cases of rhabdornyosarcoma, and Fergusson (1856) recorded a patient aged 49 with a 10-year history of a paratesticular tumour which had recently enlarged and proved to be a fibroma which had undergone sarcomatous change.

Since then excellent reviews of the literature have been recorded by Tedenat and Martin (1908)’ Hinman and Gibson (1924)’ Thompson (1936).

This paper reports 26 cases from The London Hospital and reviews them together with 88 cases from the records of the Testicular Tumour Panel and Registry of Great Britain. The 26 cases from The London Hospital comprise 7 per cent. of all the intrascrotal tumours seen there between the years 1909 and 1966. One of these has been previously described by Cairns (1926) as a case of lymphosarcorna, a diagnosis with which we agree. He also describes five other cases without giving details, two lymphosarcomas, one fibromyosarcoma, one “ round and spindle-celled sarcoma ” and one angio-endothelioma. This paper by Cairns was based on The London Hospital material, but as we cannot be sure that all the tumours were paratesticular in situation we have excluded them from discussion in this paper.

One other case has also been previously reported, as a ‘‘ fibroliposarcoma ”, by Thompson (1939), but we are not aware of any other of The London Hospital cases being described elsewhere.

The Testicular Tumour Panel cases have already been reported (Collins and Pugh, 1964). Among them is one case of adenocarcinoma of the appendix testis previously recorded by Bailey, Willis and Wilson (1955).

The object of this paper is to examine the history and clinical findings recorded in these 114 patients, with a view to establishing some principles of diagnosis and treatment. Tables I and I1 show their distribution.

Diagnosis.-Although testicular tumours are commoner on the right than on the left in the ratio of 5 :4 (Blandy, 1966), the paratesticular tumours reviewed are equally distributed between the sides. None occurred bilaterally.

Trauma is always difficult to evaluate as an ztiological factor, but a history of a direct blow was obtained in three patients (one from The London Hospital and two from the Testicular Tumour Panel cases), all of whom had a malignant tumour.

332

P A R A T E S T I C U L A R T U M O U R S 333

A much more reliable and possibly more significant fact was that five patients had undergone previous herniorrhaphy on the same side as their tumour developed. Four of these five tumours were malignant.

Three patients had a history of undescended testicle on the side of their paratesticular tumour. These proved to be a lipoma of the cord, an adenomatoid tumour of the epididymis and a seminoma of the testis invading the epididymis.

In this the paratesticular tumours differ from testicular tumours where 5.9 per cent. of the patients have a history of maldescent of the testis (Collins and Pugh, 1964).

TABLE I Paratesticular Tumours

(The London Hospital Series, 1909-1966) Malignant

Rhabdomyosarcoma . . 3 Leiomyosarcoma . . 3 Lymphosarcoma . . 2 Fibrosarcoma . . . 1 Metastasis-distinct primary tumour . . 1 Direct spread of seminoma . . . 2

- 12 -

Benign Adenomatoid tumour of epididymis . . . 3 Leiomyoma . . . . . 2 Lipoma . . . . 4 Fibroma . . . . . 3 Angioma . . . . . 1 Myxoma of scrotum . . . . . 1

14 -

-

TABLE I1 Paratesticular Tumours

T.T.P.R. Series, 1958-1967)

Malignant Rhabdomyosarcoma . . Leiomyosarcoma . Malignant lymphoma . . Fibrosarcoma . . . . Myosarcoma . . Adeno-carcinoma of appendix testis Fibro-lipo-sarcoma . Metastases-distant primary . Direct spread of seminoma . Others . . . . .

. 16

. 10

. 3

. I

. 4

. 2

. 2

. 6 . . 4 . . 5

59 -

-

Benign Adenomatoid tuniour of epididymis . . . 20 Leiomyoma . . . . 2 Lipoma . . . . . . . 2 Fibroma . . . . , . . 4 Angioma . . . . . . . 1

Presentation.-Thirteen (8.7 per cent.) of these 114 tumours were found on routine examina-

The commonest presentation was the patient noticing a lump or a dull ache in the scrotum,

The six cases of lipoma were all found at the time of herniorrhaphy. Age of Patient.-This is a highly significant factor in the diagnosis. Though, as a generalisation, a patient under the age of 20 with a paratesticular tumour will

almost invariably have a rhabdomyosarcoma, a review of The London Hospital material has revealed a number of interesting exceptions. One patient was aged 14 years when in 1925 he was diagnosed as having a hzmorrhagic leiomyosarcoma of the epididymis. On reviewing the sections we believe the lesion to be an infarction of the testis secondary to torsion. We have therefore excluded this case from the 26 London Hospital patients detailed in Table I.

tion. Eight were benign, and five malignant.

but 13 patients complained of pain in the scrotum and of these, 11 had malignant tumours.

334 BRITISH JOURNAL OF UROLOGY

Another patient was aged 10 when he died in 1910 of generalised lymphosarcoma. We have been able to examine these sections and confim that it is also lymphosarcoma. Another boy of 10 presented with a chronic hydrocele, which at operation was found to be associated with a mesothelioma. This case has also been excluded because the tumour arose from tunica vaginalis.

Leaving aside the rhabdomyosarcomata, the malignant paratesticular tumours generally occurred in patients over the age of 40 years. Though most of the benign paratesticular tumours were also in patients over the age of 40 it is interesting that the majority of the cases occurring between the ages of 20 and 40 were benign, the exceptions being the two cases of fibro-lipo- sarcoma and one of the cases of carcinoma of the appendix testis.

Duration of History.-The duration of the history in the malignant paratesticular tumours has usually been less than a year, with the rhabdomyosarcomata having a history of weeks rather than of months.

In contrast, there are patients with fibrosarcoma where a lump may have been steadily increasing in size for up to five years with a more rapid increase in size in the latter months.

All the benign tumour patients had long histories, spanning up to five years.

Clinical Findings.-Many paratesticular swellings are epididymal cysts. We have found no paratesticular tumours associated with epididymal cysts which will not be considered further.

In this series all paratesticular tumours in which the tumour had been present for several years in the epididymis proved to be adenomatoid tumours.

We have not examined the precise incidence of tuberculous epididymitis between the years 1909 and 1966 but it is recorded much more frequently than an adenomatoid tumour in the records of The London Hospital Institute of Pathology. Although tuberculous epididymitis is unlikely to remain symptomless for as many years as an adenomatoid tumour, the possibility of this diagnosis must always be borne in mind.

One sinister clinical finding has been the presence of a hydrocele in association with a paratesticular swelling. This has been noted in our series in 12 cases, 10 of which had underlying malignant paratesticular tumours.

Rhubdomyosarcoma (19 patients).-This is the commonest malignant tumour in the cases under review. Only two of the 19 patients were over the age of 20 at the time of orchidectomy. The history was of swelling usually of a few weeks’ duration; pain was seldom a dominant feature. Twelve of these tumours were on the right and seven on the left. Figure 1 shows an example of this condition.

Of 16 patients who were treated by orchidectomy alone, three are lost to follow-up, and of the remaining 13, three survived for five years or more.

Two patients had combined orchidectomy and radiotherapy, one of whom is still alive seven years later. The one patient who had a local excision died within a year.

Of these 12 patients known to have died of their disease, only one survived for one year after operation, whilst none of the six patients noted to have metastases at the time of operation even survived one year.

Leiomyosarcoma (13 patients).-This has proved to be an older man’s disease in which there is a history of swelling and dull pain over a period of 6 to 12 months. Six of these tumours were on the left side and seven on the right.

Three of the patients reviewed had an associated hydrocele. All 13 were treated by orchidectomy (Fig. 2), two with post-operative radiotherapy. Three

patients were lost to follow-up, but of the remainder only two were known to have died of their tumour in a period of follow-up of between one and three years. Neither had received radio- therapy.

Fibrosarcoma (8 patients).-These were distributed between the ages of 50 and 80 years. Five were on the left side and three on the right. The duration of history varied between five

PARATESTICULAR TUMOURS

Fig. 1.-Rhabdomyosarcoma from a 16- year-old patient with a three-month history of swelling and dull pain in the testis treated unsuccessfully by orchidectomy and ex-

ternal irradiation.

Fig. 2.-Leiomyosarcoma of the epididymis from a 61-year-old patient with a three- month history of scrota1 swelling and hydrocele. Treated by orchidectomy and external irradiation. Alive five years later

Fig. 3.-Paratesticular fibrosarcoma from a 63-year-old patient with a five-year history of swelling in the scrotum which had recently and rapidly enlarged. There was also a hydrocele. Orchidectomy was performed. He was then lost to follow-up.

335

FIG. 1

FIG. 2

FIG. 3

336 BRITISH J O U R N A L OF UROLOGY

months and five years. Three patients had previously had herniorrhaphies, and one other had a hydrocele at the time of operation (Fig. 3).

Seven patients were treated by orchidectomy and one by local excision. None had post- operative radiotherapy. Three patients were lost to follow-up after one year. One died of his tumour two years after operation.

Lymphosarcoma and Malignant Lymphoma (5 patients).-Two of the patients, one aged 10 and the other 40, had paratesticular tissue involved as part of generalised lymphosarcomatous disease. Both had hydroceles and died of their disease within a year of diagnosis, in the days before the advent of modern radiotherapy.

The three patients with deposits of malignant lymphoma in their paratesticular tissues were over the age of 40. One had a hydrocele and only survived for six months after diagnosis but he also did not receive radiotherapy. One of the other two did receive post-operative radiotherapy and was alive without recurrence two years later, but was then lost to follow-up.

Myosarcoma (4 patients).-The term myosarcoma is used to describe those tumours of connective tissue origin where a clear diagnosis of rhabdomyosarcoma or leiomyosarcoma cannot confidently be assigned, but in which the predominant tumour tissue appears to be muscle.

In this series, these all arose in the epididymis and gave a short history of a few months’ tenderness and swelling. They were equalIy distributed between the two sides in late middle-aged patients, one of whom had a hydrocele.

Despite orchidectomy in three cases-two speedily recurred locally and all four died from their tumour, the longest survival being three years.

Adeno-carcinoma of Appendix Testis (2 patients).-One patient aged 24 years had a five-year history of a swelling in his right scrotum, which was treated by local excision (Bailey, Willis and Wilson, 1955). He was still alive after five years whereas the other patient aged 65 with a three- week history of a swelling in his right scrotum had an orchidectomy followed by radiotherapy and died from metastases in his lungs two years later.

Fibro-lipo-sarcoma (2 patients).-One patient was in his thirties when he first had his left paratesticular tumour removed by local excision. I t recurred despite radiotherapy on three occasions, but he was still alive six years later. The other patient aged 23 years had an orchid- ectomy and was still alive five years later.

Metastases (6 patients).-These patients were with one exception over the age of 60 and five of the six presented with symptoms due to the secondary deposits and not to the primary turnours. Three of these metastases were on the right and three on the left. It is interesting that two of these latter had left-sided renal-cell carcinoma.

Direct Spread of Seminoma (6 patients).-These cases are included as paratesticular tumours because the testis appeared to be clinically normal. One patient had previously had an operation for undescended testis. Two had hydroceles.

All six were treated by orchidectomy without post-operative irradiation. Two were alive without sign of recurrence four and six years later respectively. The other four are lost to follow-up.

Other Malignant Paratesticular Tumours (5 patients).-These included a pleiomorphic sarcoma in a 38-year-old mentally subnormal patient, who died within days of operation, and a carcinoid tumour in the epididymis in a 60-year-old patient who died one year later. He had metastases at the time of operation. The origins of these tumours and of three others could only be surmised, but they were probably metastases.

Benign Paratesticular Tumours. Adenomatoid Tumours (Fig. 4) (23 patients).-These were about equally distributed between the two sides. Sixteen occurred between the ages of 40 and 60 years. There was a history of slowly increasing swelling in the epididymis cver periods as long as 10 years. One had a previously undescended testicle on the same side and six were found at routine examination. Sixteen patients were treated by orchidectomy and the remaining seven by local excision.

PARATESTICULAR TUMOURS 337

Fibroma (7 patients).-Four of these occurred on the right side and three on the left (Fig. 5). The patients’ ages ranged from 25 years to 61 years. All had noticed a lump, increasing slowly in size over the years. Six were treated by orchidectomy.

FIG. 4 FIG. 5

Fig. 4.-Adenomatoid tumour of the left epididymis which had been present for four years. Fig. 5.-Paratesticular fibroma from a 25-year-old Pakistani which had been present for four years.

Treated by orchidectomy. Lost to follow-up.

Leiomyoma (4 patients).-Two had hydroceles and the tumours were found incidentally at operation. The oldest patient was 81 years old. Three were treated by orchidectomy.

Lipomata (6 patients).-All were found during the course of herniorrhaphy. They are much more frequent than these figures suggest, and are sufficiently commonplace that many are not examined histologically.

DISCUSSION

Paratesticular tumours are rare, and have not always been recognised as separate entities from the more common testicular tumour. Dew (191 1) thought that primary sarcomata accounted for 2 per cent. of all testicular tumours. It is possible that some of his “sarcomata” were seminoma.

In the unselected series from The London Hospital (Table I) we have shown that nine of these 26 cases were primary sarcomatous paratesticular tumours, and as such they comprise slightly less than 3 per cent. of all recorded intrascrotal tumours at The London Hospital during the period (1909-66).

3 F

338 BRITISH J O U R N A L OF UROLOGY

In the total of 114 patients, we are impressed by the significance of the patient’s age at the time of presentation and the duration of the symptoms.

It is clear that a patient under the age of 20 years, with a rapidly enlarging paratesticular swelling, will almost invariably have a rhabdomyosarcoma.

It is interesting to speculate about the case recorded by Louvet (1865) in a child aged 7 years diagnosed as a fibrosarcoma of the epididymis and cord. We would suggest that it was probably a rhabdomyosarcoma.

In the patients reviewed here rhabdomyosarcoma was a rapidly lethal condition. One-third of the patients already had metastases when they were first seen, and three-quarters of all patients with a rhabdomyosarcoma were dead within two years.

We are unable to assess the value of post-operative radiotherapy as it was so infrequently used in this condition. In two instances one patient survived to seven years but the other died rapidly.

An older man with a short history of a paratesticular tumour will often prove to have a leiomyosarcoma. Fifty-two of the patients we discuss were not in the rhabdomyosarcoma age- group, but had clinically malignant tumours. Of these 52 patients, seven had metastases from distant primary tumours.

The duration of the history is significant. A history in months is usually associated with a malignant tumour and a history in years is usually associated with a benign tumour.

The case of adeno-carcinoma of the appendix testis (previously recorded by Bailey, Willis and Wilson, 1955) had a five-year history of paratesticular tumour. As this patient also had intra-scrota1 metastases the tumour is classified as malignant, but this is an area where there is some pathological dispute, and where benign adenomatoid tumours have been classified as adeno-carcinomata (Hinman and Gibson, 1924; Thompson, 1936).

One of the patients accepted as a case of fibro-lipo-sarcoma had a long history of tumour with local recurrence and numerous local excisions-a well-known feature of this condition elsewhere in the body.

Neither of the two cases of fibro-lipo-sarcoma relates to the patient described by Thompson (1939). He was a 50-year-old patient who had had a scrota1 swelling for 15 years which had suddenly enlarged. The specimens from this patient have been re-examined and appear to be a rhabdomyosarcoma, but this is an exception to the general pattern of such cases as we have reviewed them.

Although fibromata of the spermatic cord may be multiple (Makins, 1912; Orlay, 1961), we did not observe this in either The London Hospital or Testicular Tumour Panel series.

One sinister clinical finding has been the presence of a hydrocele, In tumours of the testis this is a rare finding. John Hunter is quoted as having never seen a secondary hydrocele in association with a tumour of the testis. However, 12 of the 14 patients reviewed here had hydro- celes and of these 12, 10 had underlying malignant paratesticular tumours. This is therefore an indication for aspirating hydroceles so that the contents of the scrotum can be properly examined, taking care not to damage the underlying testis because of the risk of implantation along the needle track.

When the implications of the patient’s age, duration of history, and clinical findings are considered, the correct course of action will probably be to proceed to orchidectomy. However a patient between the ages of 40 and 60 years, with a long history of a paratesticular swelling which is slowly growing, and has no associated hydrocele, will almost invariably have an adenomatoid tumour of the epididymis and local excision of the lesion may be recommended,

We are unable to assess the value of post-operative radiotherapy in these cases, but there is a suggestion that it may be of use in cases of leiomyosarcoma.

P A R A T E S T I C U L A R TUMOURS 339

SUMMARY

One hundred and fourteen cases of paratesticular tumour are reviewed. The young patient with a rapidly growing tumour usually has a rhabdomyosarcoma, many

of which have already metastasised at the time of presentation. Of the other malignant paratesticular tumours approximately 25 per cent. are manifestations of generaiised malignant disease and are often metastases.

The presence of a hydrocele in association with a paratesticular tumour is usually an indication that the tumour is malignant.

In patients where the history and clinical findings are indicative of an adenomatoid tumour, there may well be a place for local excision of the tumour.

We would like to express our thanks to Professor I. Doniach and Dr R. C. B. Pugh for their help in the preparation of this paper and to the Chairman of the Testicular Tumour Panel and Registry for allowing us to consider this particular aspect of the Panel’s work.

REFERENCES

BAILEY, G. N., WILLIS, R. A., and WILSON, J. V. (1955). J. Path. Bact., 69, 326. BLANDY, J. P. (1966). Hosp. Med., 1, 133. BROCKOW, J. L., and GUMMESS, G. (1951). J. Urol., 65, 136. CAIRNS, H. W. B. (1926). Lancet, 1, 845. COLLINS, D. H., and PUGH, R. C. B. (1964). Br. J. Urol., 36, Suppl. DEW, H. (191 1). Surgery Gynec. Obstet., 46, 447. FERGUSSON, W. (1856). Lancet, 2, 11. HINMAN, F., and GIBSON, T. E. (1924). Archs Surg., Chicago, 8, 100. HINMAN, F., and SMITH, D. R. (1944). “ The Sexual Glands of the Male. ” London: Oxford

HUNTER, J. (1835). “ Works of John Hunter ”, ed. Palmer, J. F., Vol. 1, p. 469. London. LOUVET (1865). Bull. Soc. Anat., 10, 505. MAKINS, G. H. (1912). Proc. R. Soc. Med., 5, 155. NEUMAN (1892). ORLAY, G. (1961). Br. J. Surg., 49, 66. ROKITANSKY (1849). Quoted by Neuman (1892). SUNDARASNARAO, D. (1953). J. Path. Eact., 66, 417. T~DENAT, E., and MARTIN (1908). Archs gin. Chir., 2, 11 3. THOMPSON, G. J. (1936). Surgery Gynec. Obstef., 62, 712. THOMPSON, H. R. (1939). Br. J. Surg., 27, 169. WALSHAM, W. J. (1879). Trans. path. SOC. Lon& 31.

University Press.

Virchows Arch. path. Anat. Physiol., 130, 249.