Pediatric Annals

Testicular Tumors in Infancy and Childhood

Mitchell Levy, MD; Norbert Platt, MD; Martin W Abrams, MD; Burton Bronsther, MD

Abstract

Abell. M. R.. and Holtz. F. Testicular neoplasms in infants and children. I: Tumors of germ cell origin. Cancer 76 (1963). 965.

Alexander, F. Pure testicular rhabdomyosarcoma. Br. J. Cancer 22 (1968), 498.

Allen, B., Jr., and Skust, L. Testicular tumors in infants and children: A case report. J. Urol. 86 (1961), 795.

Anderson. R. E.. and Huston, C. Tumors of the testicle in childhood. Am. J. Surg. 95 (1958), 445.

Arean, F., and Kraeger. J. Paratesticular rhabdomyosarcoma. Am. J. Clin. Pathol. 43 (1965), 418.

Arlen, M., Grabstald. H., and Whitmore. W. Malignant tumors of the spermatic cord. Cancer 23 (1969), 525.

Assini. M., Otero, G., and Canton, J. Rhabdomyosarcoma of the vaginal tunic. J. Urol. 101 (1969), 732.

Bachmann, K. D., and von Grawert, H. Tumoren des Hodens im Kindesalter: Bericht über 545 Fälle. Monatsschr. Kinderheilkd. 120 (1972), 40.

Bamett, W. A. Paratesticular interstitial cell tumor in a boy aged 11. J. Urol. 78 (1957). 158.

Bhansau, S. Rhabdomyosarcoma of spermatic cord. Br. J. Surg. 50 (1963). 883.

Bhargava, M. K.. and Reddy, DG. Tumors of the testis. II: Tumors of the testis in children. Cancer 19 (1966), 1655.

Bishop, P. M., et al. Interstitial cell tumour of the testis in a child: Report of a case and review of the literature. Br. Med. J. 5168 (1960), 238.

Burrington. J. Rhabdomyosarcoma of the paratesticular tissues in children: A report of 8 cases. J. Pediatr. Surg. 4 (1969), 503.

Cannon, E., Altheide, J., and Allen, H. Malignant tumors of the spermatic cord and testicular tunics. South. Med. J. 49 (1956), 17.

Collins. J. D., and Schoenenberger, A. P. An unusual germinal tumor of the testis in a nine-year-old boy: A variant of a seminoma. J. Urol. 87 (1962), 710.

Collins, J. 0., and Schoenenberger, A. P. A ten year survey of testicular tumors in a general hospital. Wis. Med. J. 61 (1962), 517.

Culp, D. A., Frazier, R. G., and Butler, J. J. Sertoli cell tumor in an infant. J. Urol. 76 (1956). 162.

Davis. A. Rhabdomyosarcoma of the testicle. J. Urol. 87 (1962), 148.

Dutta-Chaudhuri, R. Occurrence of carcinoma of the rete-testis in an infant. Indian J. Pediatr. 23 (1956), 1.

Fox. T.. and Collier, R. Rhabdomyosarcomas of the spermatic cord: A review and case presentation. Am. J. Surg. 33 (1967). 483.

Friedman. N. B.. and Moore. R. A. Tumors of the testis. Mil. Surgeon 99 (1946), 573.

Gangai, M. P. Testicular neoplasms in an infant. Cancer 22 (1968), 658.

Giebink, S. G.. and Ruymann, F. B. Testicular tumors in childhood: Review and report of 3 cases. Am. J. Dis. Child. 127 (1974), 433.

Gilbert, J. B., and Hamilton, J. B. Studies in malignant testes tumors. Ill: Incidence and nature of tumors in ectopic testes. Surg. Gynecol. Obstet. 71 (1940), 731.

Gilbert, J. B. In Matasserin, F. W. Embryonal adenocarcinoma of the testicle in an infant: Case report. J. Urol. 52 (1944), 575.

Gould, S. E., et al. Lesions of testis. Am. J. Clin. Pathol. 38 (1962), 533.

Gowing, J., and Morgan, A. Paratesticular tumours of the connective tissue and muscle. Br. J. Urol. 36 (1964), 78.

Gray, C. and Biorn. C Rhabdomyosarcoma of spermatic cord. J. Urol. 74 (1955), 402.

Grosfeld, J. L., Smith, J. P., and Clatworthy, H. W., Jr. Pelvic rhabdomyosarcoma in infants and children. J. Urol. 107 (1972), 673.

Gross, R. W. The Surgery of Infancy and Childhood: Its Principles and Techniques. Philadelphia: W. B. Saunders Company. 1953. p. 486.

Grotts. B. F. "Adult" testicular teratoma in a child: A case report. J. Indiana State Med. Assoc. 45 (1952), 888.

Hoffman, W.. and Baird. S.…

Testicular and paratesticular tumors occur very rarely in children. In 1957 a total of 464 cases had been collected in the world literature. By 1965 a further report increased the number to 599. It is reasonable to assume that within the past 10 years an additional 20 cases per year would have brought the total to between 750 and 800 cases. This represents a rare grouping.

Because the reported cases are sporadic and there are no large series in the experience of any one surgeon or group, it is important to place general concepts of the tumor (diagnosis and treatment) before those who are to care for these children. Since pediatricians are primary physidans to whom the family turns for advice and help, it is necessary that the general considerations be available to them.

EMBRYOLOGY OF THE TESTIS

Before the seventh week of fetal life, the male testicular cells are in different cellular structures that cannot be isolated from their female counterparts; thus the term "gonad" is applied. Three cell types give rise to this gonad:

1. Primary germ cells, which migrate to the genital ridge from the yolk sac.

2. Mesenchymal cells, from the mesonephros near the mesenteric root.

3. Coelomic cells, epithelially derived, overlying the mesenchyme.

Gradually the genital ridge develops by proliferation and elongates into the coelomic cavity connecting with the mesonephros, bound by a thick mesenteric attachment. By the end of the sixth week, the epithelial cells migrate and cover the gonad, insinuating themselves into the mesenchymal tissue and germ cells as cords. These, then, are the primary sex cords; by the eighth week, they have differentiated into beginning seminiferous tubules (Sertoli's cells) and rete testis.

They remain solid cords until the fifth or sixth month, when they begin to hollow out. Other surface epithelial cells become the tunica albuginea and the connective-tissue septa. Portions forming the rete tubules join the persistent mesonephric tubules to become associated in the efferent ductules. The mesenchymal cells undergo marked proliferation between the third and sixth months, rapidly decreasing thereafter by cellular degeneration to become the permanent interstitial cells. Leydig's cells can be identified anywhere from the third month of fetal life. It is quite conceivable that during this process primordial undifferentiated cellular rests remain to form a nidus of a tumor, under proper stimulus.

TESTICULAR DESCENT

Gubernacular development begins before the seventh week, when the structure is recognizable. The processus vaginalis appears from the caudal end of the abdominal cavity. It enters the scrotal swellings by piercing the abdominal wall, forming the inguinal canal. The processus forms a herniation that is completely covered by all layers of the abdominal wall. Within this canal, reaching up to the testis, is the gubernaculum, a thick mesenchymal proliferation from the abdominal waU. This begins to shorten at about the seventh fetal month into the bottom of the scrotum, and its lower two-thirds disappears.

This descent, it is conjectured, is mediated by hormonal influences-chorionic gonadotropin, testoster-one, and maternal source hormones being variously hypothesized as playing a role. Perhaps this is so. Certainly, it is generally accepted that malignant testicular tumors occur up to 50 times more frequently in those with undescended testicles than in the general population. Perhaps the hormonal imbalances leading to the failure of descent also cause abnormal stimuli to rests of totipotential cells, which then become malignant.

The work of Friedman and Moore has provided us with a satisfactory histologic classification of tumors of the testis. The more recent classification of testicular tumors by Price et al. embodies the works of previous authors. These authors recognize two groups of testicular tumors, the germinal and the nongerminal (Table 1).

HISTOGENESIS

The histogenesis of tumors of the testis has been debated by many authors. There is general agreement that seminoma originates from germ cells. The only dissenter is Masson, who in 1946 suggested that the spermatocytic seminoma was the only tumor that originated from germ cells.

As for the cell origin of nonseminomatous primary testicular tumors, a number of theories have been put forth. These can be summarized as follows:

1. Teratogenesis by metamorphosis of undifferentiated cells (Askanazy, 1907).

2. Parthenogenesis (rejected by Askanazy and Willis).

3. Teratomas originate from cells that were released from the normal developmental control at the primitive stalk stage.

4. Origin of teratomas from displaced "blastomeres" in early embryonic development after having escaped the influence of organizers (Collins and Pugh).

Currently, most workers in this field adhere to a holistic approach, which maintains that all noninterstitial tumors of the testis originate from germ cells. This is supported by ample documentation based on studies of spontaneous testicular tumors in the mouse, coupled with transplantation and electron-microscopic studies.

Nothing is known about the mechanism of origin of infantile testicular teratoma, a tumor that has an entirely different behavior in the adult.

EMBRYONAL CARCINOMA

Embryonal carcinoma, a testicular tumor derived from germ cells, is anaplastic, with a striking tubuloacinar papillary histologic appearance. These tumors often show solid areas having an embryonal mesenchymal appearance.

Of the three types described by Mostofi and Price, the highly anaplastic type is seen only in adults. It is an aggressive tumor that is radioresistant and spreads mainly via the lymphatics and, less frequently, via the bloodstream. Most of the embryonal carcinomas of the testis are pure. Occasional cases show trophoblastic or somatic differentiation or both. The finding of foci of choriocarcinoma seems to worsen the prognosis.

Table

TABLE 1PATHOLOGIC CLASSIFICATION OF TESTICULAR TUMORS

TABLE 1

PATHOLOGIC CLASSIFICATION OF TESTICULAR TUMORS

Embryonal cell carcinoma spreads to periaortic and iliac lymph nodes in 96 per cent of cases, with lungs, liver, pleura, bones, and gastrointestinal tract being affected in order of decreasing frequency. The five-year mortality is about 65 per cent.

The second type of embryonal carcinoma of the adult testis is the rare polyembryoma. It consists of embryoid bodies resembling embryos of one to two weeks' gestation. These are often seen in adult embryonal carcinomas and teratomas but never in children.

The third type, the infantile embryonal carcinoma (orchioblastoma or yolk-sac tumor) occurs almost exclusively in infants and children and has a lower level of aggressiveness. When they occur in the male adult, they too have a low degree of malignancy.

The average age at diagnosis in a series of 134 patients seen by Bachmann and von Grawert was three years. Four tumors were discovered at birth. Fifteen children under two years of age died following orchiectomy. Nineteen of 28 patients over two years of age died of the disease.

Abell and Holtz also pointed out a much better prognosis in children treated by orchiectomy before the age of three years.

Gangai reported on 11 patients treated with orchiectomy and retroperitoneal node dissection. The lymph nodes were not affected, raising the question as to the validity of lymph node dissection as a primary operative approach.

TERATOMA

This is a complex tumor bearing some similarity to the ovarian cystic and solid tumors. Histologically, these tumors show elements derived from one or more of the three germ layers of the embryo. Generally, they form a complicated mosaic of recognizable tissues and organ formation. Occasionally, dermoid and epidermoid cysts originate and are thought to represent the one-sided development of a teratoma. Mostofi and Price subdivide testicular teratomas into two types, immature and mature. The immature variant is composed of primitive neuroectodermal, entodermal, and mesodermal elements; they are often associated with mature tissues or organoid elements.

Mature testicular teratomas are composed of well-differentiated tissue elements representing the three germ cell layers. Mature teratomas rarely undergo malignant changes in children.

The mature type of teratoma in children has an incidence of 26.8 per cent. The average age at diagnosis, according to Bachmann and von Grawert, is 3.6 years. In 26 per cent of these, the tumor was found at or shortly after birth. Woolley et al. demonstrated the presence of calcium by x-rays in all their four cases.

There has been little malignant transformation of testicular teratomas or metastatic spread in the 87 cases reported. Thirty-three patients were observed for two or more years. Only one died of metastases, postoperatively. Of 26 patients compiled by Giebink et al., 14 were followed for at least six months postoperatively. Eleven of these showed no evidence of metastases two or more years after orchiectomy.

Testicular teratomas require adequate histopathologic study. Those containing well-differentiated tissue do not metastasize. Of the few cases in which retroperitoneal dissection was carried out, none showed evidence of metastases.

Julien reported good results in four children with adult teratomas of the testis who were treated by simple enucleation of the tumor.

A review of reported cases of mature testicular teratoma occurring in infants and children points to the benign character of this tumor. We agree that orchiectomy, with high ligation of the spermatic cord, is curative.

TERATOCARCINOMA

Teratocardnomas of the testis in infants and children are prognostically related to age. Eighty-six patients with testicular teratocarcinoma were studied by Bachmann and von Grawert. Forty-six of these were observed for two or more years: 71 per cent of the children under two survived without metastases, while 60 per cent of those over two survived without metastatic disease at time of diagnosis.

Retroperitoneal node dissection is recommended, especially in children over two years of age. This procedure may increase survival, since seven of nine patients over two years of age at diagnosis died with metastases.

INTERSTITIAL CELL TUMORS

These are tumors of specialized gonadal stroma. Although there is no full agreement that Leydig, Sertoli, granulosa, and theca cells have a common origin, their frequent occurrence in tumors showing a combination of these cell types is explainable on the basis of a common origin from the primitive gonadal stroma.

These tumors are extremely rare, particularly in patients under 14 years of age. A survey of the literature by Bachmann et al. included 545 children with testicular neoplasms. Five per cent of these were made up of Sertoli cell tumors.

Leydig cell tumors are occasionally functional, producing hormonal changes characterized by precocious pseudopuberty or gynecomastia. These changes are usually reversed by orchiectomy. They are occasionally bilateral and rarely malignant. No malignant cases have been reported in children.

SERTOU CELL TUMORS

These are extremely rare in all age groups, especially under 14 years. Weitzner et al. reviewed 23 patients with Sertoli cell tumors in childhood. Sixteen patients were under the age of one year. Two patients had bilateral tumors. In one patient, the tumor was located in an undescended testis. Precocious pseudopuberty was noted in one boy. Gynecomastia was noted in three boys. However, this abnormality regressed in two following orchiectomy. Metastatic spread to the retroperitoneal lymph nodes occurred only in one boy, who was alive and free of tumor two years after orchiectomy, bilateral retroperitoneal lymph node dissection, and postoperative cobalt radiation and vincristine sulfate therapy.

It is extremely difficult to predict the course and functional character of these tumors on the basis of the morphologic appearance.

There is a group of Sertoli cell lesions that occur in undescended testes, many of which are also dysgenetic. These lesions consist of hyperplasia of Sertoli cells or Sertoli cell adenomas. The latter were often seen in patients with the testicular feminization syndrome.

GONADOBLASTOMA

Scully used the designation "gonadoblastoma" for tumors composed of germ cells and immature cells of Sertoli or granulosa type. Cells resembling Leydig and lutein cells were also present. Calcification, which was common, was occasionally revealed on roentgenograms of the pelvis. All patients were sexually abnormal. The gonad bearing the tumor was a dysgenetic cryptorchid testis or a streak. Eighty per cent were phenotypic females; the rest were phenotypic males, who almost always had associated cryptorchidism, hypospadias, and female internal sex organs. Scully regarded the gonadoblastoma as in situ cancer from which germinomas and other types of invasive germ cell tumors can develop.

Patients with sexual abnormalities, including patients with a cryptorchidism, should be thoroughly investigated from the standpoint of potentially malignant tumors.

According to Siebert and Hamilton, a cryptorchid testis is 30 to 50 times more likely to develop a malignant tumor than the normally descended companion.

MESENCHYMAL TUMORS

Benign mesenchymal tumors of the testis - such as lipoma, fibroma, and hemangioma - are exceedingly rare.

Sarcomas of various types often affect the paratesticular structures. The testis becomes involved secondarily.

Rhabdomyosarcomas are highly malignant tumors; 15 per cent of them occur in the genital tract. The sites of predilection for the occurrence of this tumor are the bladder trigone urethra, vagina, and paratesticular tissues.

Giebink and Ruymann compiled a total of 86 cases of testicular mesenchymal tumors occurring in children and adolescents. Twenty-six cases (30 per cent) occurred in children less than five years of age. The youngest was three months old.

Postoperative survival figures were available for 80 patients. Thirty-five per cent survived at least one year, and 59 per cent died. The mean length of survival was 13 months. Children less than five years of age had the best prognosis, with 71 per cent surviving for at least one year. Twenty-five per cent died, with a mean postoperative survival of 11 months.

The highest case fatality rate was noted in 13 children whose diagnosis was made during puberty. Eightyfive per cent died, and only one was well without disease 15 months after orchiectomy.

ETIOLOGY

The cause of testicular neoplasms remains unclear. Nevertheless, the role of trauma and cryptorchidism has been emphasized as a possible predisposing factor.

In a review of 113 testicular malignancies, there was a history of trauma before onset of the mass in 28, representing 24.7 per cent of the series.

A 20-year study of 505 cases of cryptorchidism was reviewed by CuIp. The incidence of malignancy was 0.79 per cent. During the same period there were 179,899 male admissions to the hospital. In this group, 109 cases of testicular tumors were discovered, representing a real incidence of 0.06 per cent. Therefore, malignant changes occurred 13 times more frequently in the undescended testicle than in the normally descended one.

CLINICAL ASPECTS

Patients with testicular tumors usually have either painless swelling of the testicle (frequently after a trauma) or symptoms referable to metastases from the primary lesion. The most common metastatic sites are retroperitoneal, pulmonary, cervical lymph nodes, mediastinal nodes, and spine. Less common sites include pelvic bone, peritoneum, ribs, liver, brain, meninges, adrenals, scapula, penis, intraocular areas, and diaphragm.

As stated above, most patients have a painless testicular swelling. Less frequently, the child may complain of pain in the testicle or in the abdomen, back, or groin. He may also have a cough with fever, weight loss, and anorexia; occasional cervical swelling may call attention away from the true site of the disease. Jaundice is an infrequent occurrence, but has been reported in cases of massive hepatic metastasis.

CLINICAL WORKUP

Careful routine and special studies should be made in all malignancy workups, and this is applicable to testicular swellings. They should include complete blood count, urine examination, hematologic studies as prechemotherapeutic base lines, serum evaluations (particularly of liver chemistries), adrenal function studies, and, in those rare instances where sexual precocity appears, hormonal assays. Survey x-rays should include chest, spine, long bone, and skull. Bone scans are indicated when the evidence suggests bone involvement.

Scrotal x-rays are important, since calcification may appear, leading one to consider a diagnosis of dermoid cyst and a more conservative surgical approach. Bone marrow biopsies as pretherapeutic or diagnostic evaluations are occasionally valuable. In general, intravenous pyelograms are not rewarding studies unless there is an abdominal mass as a metastatic complication. Inferior vena cavagrams may reveal nodal compression. Lymphangiography has some value, but it should not be used in small infants. There may be validity in delaying postoperative lymphangiography in order to evaluate radical lymph node dissection as a second surgical procedure. It is important to stress that needle aspiration biopsy of the tumor should not be utilized. The danger of spreading to the scrotal wall or surrounding area is too great. Occasionally, the superficial lymphatics may be seeded in this manner.

TREATMENT

The plan of treatment of testicular tumors in children is dependent on the particular pathologic history of the lesion. The basic surgical operation is unquestioned. Orchiectomy and high ligation of the cord are mandatory. Occasional "shelling out" procedures have been reported in an attempt to find those few benign lesions that occur (i.e., dermoid); in general, however, the literature decries these as possibly leading to spread of misdiagnosed lesions. Partial excision of the scrotum and inguinal canal has been carried out successfully, but not as a routine procedure. The surgical endeavor ceases at this point for all nongerminal interstitial cell tumors (e.g., Leydig and Sertoli).

Some advocate transscrotal excision of these tumors. It is more practical, however, not to leave a useless spermatic cord, for two reasons. First, there may be a mistake in diagnosis, and the lesion will be a more highly malignant type that may be spread by cutting across the tissue's plane. Second, there may be a small hernia at the internal inguinal ring area that can be treated effectively by the high ligation of the cord. One of us (Dr. Abrams) recently had such a case in a lesion that appeared in the midcord region associated with an asymptomatic hernia, which was treated by high ligation. It is considerably less clear, in relation to germinal cell tumors and rhabdomyosarcoma, whether there is a role for lymph node dissection.

Metastasis from testicular tumors in childhood is most commonly by the lymphatics and rarely by the bloodstream. Notably, a combination of the two routes is found in the extra testicular rhabdomyosarcoma. There is not yet a conclusive amount of detailed evidence to emphatically advocate the routine use of regional node dissection in all types of germinal cell-based tumors. It would appear that in most series the results of primary regional (retroperitoneal) node dissection are disappointing in the overall long-term results. There are some series that tend to support their efficacy in embryonal carcinoma and teratocarcinoma. The role of delayed nodal dissection after lymphangiography has not been established. Many of these tumors are highly radioresistant. For this reason, preoperative radiation is certainly not indicated.

Recent work suggests that metastatic lesions can respond especially in combination with chemotherapy. Routine postoperative irradiation is generally not encouraging. Indeed, there is speculation that the combination of x-ray therapy and chemotherapy may so weaken the host's responses to the tumor that new growth may occur more easily. Certain centers treat germinal cell lesions by a routine combination of surgery and lymph node dissection (both sides of the lower aortoiliac chain), followed by x-ray and chemotherapy. Recently the chemotherapeutic agents have been utilized with more encouraging results, especially for embryonal carcinomas, teratocarcinomas, and rhabdomyosarcomas. Certainly they are the treatment of choice for widespread metastases, usually in combination with radiotherapy. Drugs now in popular and experimental usage are:

Cytotoxic agents - the alkylating agents, especially cyclophosphamide (Cytoxan®), which act to inhibit the formation of nucleic acids.

Antibiotics - actinomycin D, mithramycin, and bleomycin. Actinomycin is the most effective and least toxic of this group and is the drug of choice.

Vinca alkyloids - vincristine. This is especially effective for rhabdomyosarcoma.

VAC therapy (Vincristine-Actinomycin D-Cytoxan) - a combination of the above three drugs that is especially effective in treating rhabdomysarcoma and diffuse metastatic disease of all types of germinal cell tumors.

Other agents being variously employed are the antimetabolites, such as methotrexate; furan derivatives, such as nitrofurazone; and hormones, such as medroxyprogesterone (Provera®), testosterone, and prednisone. The last group is relatively untested, and limited in effectiveness thus far. These drugs are given in cyclical dosages anywhere from one to two years following initial usage. All drug protocols must be monitored closely because of their marked toxic effects, with or without concurrent usage of radiation. Hematopoietic, neural, gastrointestinal, renal, and integumentary toxicities accompany their employment.

SUMMARY

The rarity of testicular and paratesticular tumors in children should not deter from the need to be alert to their existence. These lesions are readily accessible to the examiner's hand and eye. Early recognition and treatment offer a very encouraging prognosis in a great many instances.

The definitive therapy after orchiectomy and high ligation of the spermatic cord depends a great deal on the pathologic determination under the microscope. A close working understanding among surgeon, chemotherapist, radiotherapist, and pathologist is essential to successful management. One must bear in mind that these tumors generally do not metastasize as readily in infants and children as they do in the adult. Moreover, the young patient's responses in the host-tumor milieu are different from those of the adult. Therefore, the prognosis may not be so grave as under many similar circumstances in adults. As with other tumors in children, age of onset is of particular importance, since children under the age of two seem to have a better prognosis.

Advances in chemotherapy in the past decade have had a markedly beneficial effect on the treatment of these lesions - especially paratesticular rhabdomyosarcoma, for which recent application of combined VAC therapy and radiation is producing encouraging results.

There is general agreement that orchiectomy and spermatic cord ligation should be performed for all testicular tumors. "Shelling out" procedures and local excisions are not warranted. Preoperative irradiation is contraindicated on the basis of:

1. General uncertainty of diagnosis.

2. Possible contralateral testicular damage.

3. Questionable results of radiation for testicular tumors.

The value of postoperative radiation is still to be elucidated. Certainly the seminoma group exhibit a high degree of rathosensitivity, but these are virtually nonexistent in childhood. Ultimately, radiation may be reserved for rhabdomyosarcoma, embryonal carcinoma, and malignant teratoma.

The role of node dissection is still under scrutiny. Perhaps it will be of value in embryonal carcinoma, but this is only assumptive. Malignant teratomas may be a stronger indication for retroperitoneal and regional node dissection, since they metastasize early to these areas. Likewise, since rhabdomyosarcomas metastasize via the bloodstream and lymphatics, the indication there is questionable. Perhaps delayed lymphangiography will give a better indication as to which patients should be candidates for these procedures.

It is important for the pediatrician as primary physician to recognize that, in all cases of precocious puberty, examination of the testicle can be diagnostic of interstitial cell tumors, which represent the largest number of nongerminal tumors. The general benign nature of Sertoli and Leydig cell tumors renders them amenable to orchiectomy and high cord ligation with no other therapy. In contrast to these, the so-called streak gonads that accompany certain intersex problems are associated with a high degree of malignancy. For that reason, cases of sexual ambiguity should be approached with this association in mind.

One must remember that, because mese lesions are so few in number, treatment is still highly individualized. Experience is limited from center to center. The importance of collation of these unusual tumors into the larger referral centers is indicated in order to garner long-term experience and spot trends that will improve long-term results.

ADDENDUM

Occasionally a testicular or paratesticular tumor may present as a sudden swelling, noticed by a mother while she is bathing or routinely dressing a young child. The subject of sudden swellings will be dealt with in another article in a future issue. For the sake of completeness, however, it should be mentioned here.

A sudden swelling of the cord or testicular area may or may not be painful. This can be categorized with relative ease. Among the painless swellings, the most common is an acute hydrocele. It is generally of the cord per se but may involve the testicular area. Bowel incarceration is rarely manifested as a painless swelling. Bowel is almost always reducible; hydroceles are not. Hernias occasionally transilluminate; hydroceles usually do. Therefore, this becomes an unreliable test. Tumors are harder to the touch than hydroceles or hernias. They may or may not be painful. X-rays, especially trans-table lateral examination, are very valuable in this regard. Air in the scrotum or outside the peritoneal line, along the inguinal canal, is diagnostic of bowel incarceration. Calcification implies that some tumefaction is present.

Among the painful acute swellings, in addition, one must consider torsion of the testicle or appendix testis as well as incarcerated bowel in a hernia. Torsions are very sudden in onset. The swelling is prompt and more painful than any other type of involvement. The testicle is swollen and tender - often excruciatingly so. Early on, the cord may not be enlarged. It becomes larger as the venous and arterial supplies become compromised. Appendix testis torsion presents early as a pinpoint swelling and tenderness at the testicular or epididymal area. Careful examination will often reveal a small nodule of tenderness that is diagnostic. Later, the entire scrotum may be red and edematous on one side, just as in testicular torsion, and make it more difficult to differentiate one from the other. Testicular swelling occurs very late, if ever, in the case of torsion of the appendix testis.

Orchitis, orchioepididymitis, and epididymitis do occur in children. The onset is less acute, the swelling gradual. Quite often pain precedes the swelling by some time. A history of mumps or a swelling over the parotid may be helpful in diagnosis. Again a viral syndrome may be present preceding or accompanying the testicular pain and swelling.

GENERAL REFERENCES

Abell. M. R.. and Holtz. F. Testicular neoplasms in infants and children. I: Tumors of germ cell origin. Cancer 76 (1963). 965.

Alexander, F. Pure testicular rhabdomyosarcoma. Br. J. Cancer 22 (1968), 498.

Allen, B., Jr., and Skust, L. Testicular tumors in infants and children: A case report. J. Urol. 86 (1961), 795.

Anderson. R. E.. and Huston, C. Tumors of the testicle in childhood. Am. J. Surg. 95 (1958), 445.

Arean, F., and Kraeger. J. Paratesticular rhabdomyosarcoma. Am. J. Clin. Pathol. 43 (1965), 418.

Arlen, M., Grabstald. H., and Whitmore. W. Malignant tumors of the spermatic cord. Cancer 23 (1969), 525.

Assini. M., Otero, G., and Canton, J. Rhabdomyosarcoma of the vaginal tunic. J. Urol. 101 (1969), 732.

Bachmann, K. D., and von Grawert, H. Tumoren des Hodens im Kindesalter: Bericht über 545 Fälle. Monatsschr. Kinderheilkd. 120 (1972), 40.

Bamett, W. A. Paratesticular interstitial cell tumor in a boy aged 11. J. Urol. 78 (1957). 158.

Bhansau, S. Rhabdomyosarcoma of spermatic cord. Br. J. Surg. 50 (1963). 883.

Bhargava, M. K.. and Reddy, DG. Tumors of the testis. II: Tumors of the testis in children. Cancer 19 (1966), 1655.

Bishop, P. M., et al. Interstitial cell tumour of the testis in a child: Report of a case and review of the literature. Br. Med. J. 5168 (1960), 238.

Burrington. J. Rhabdomyosarcoma of the paratesticular tissues in children: A report of 8 cases. J. Pediatr. Surg. 4 (1969), 503.

Cannon, E., Altheide, J., and Allen, H. Malignant tumors of the spermatic cord and testicular tunics. South. Med. J. 49 (1956), 17.

Collins. J. D., and Schoenenberger, A. P. An unusual germinal tumor of the testis in a nine-year-old boy: A variant of a seminoma. J. Urol. 87 (1962), 710.

Collins, J. 0., and Schoenenberger, A. P. A ten year survey of testicular tumors in a general hospital. Wis. Med. J. 61 (1962), 517.

Culp, D. A., Frazier, R. G., and Butler, J. J. Sertoli cell tumor in an infant. J. Urol. 76 (1956). 162.

Davis. A. Rhabdomyosarcoma of the testicle. J. Urol. 87 (1962), 148.

Dutta-Chaudhuri, R. Occurrence of carcinoma of the rete-testis in an infant. Indian J. Pediatr. 23 (1956), 1.

Fox. T.. and Collier, R. Rhabdomyosarcomas of the spermatic cord: A review and case presentation. Am. J. Surg. 33 (1967). 483.

Friedman. N. B.. and Moore. R. A. Tumors of the testis. Mil. Surgeon 99 (1946), 573.

Gangai, M. P. Testicular neoplasms in an infant. Cancer 22 (1968), 658.

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TABLE 1

PATHOLOGIC CLASSIFICATION OF TESTICULAR TUMORS

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