Testicular and paratesticular tumors and tumor-like lesions in the first 2 decades
Introduction
Testicular and paratesticular tumors and tumor-like lesions differ in their frequency, and in some instances behavior, in children and teenagers in comparison to adults. This is partly illustrated by the experience at a university-based practice, where the relative frequency of testicular tumors in patients less than 15 years of age compared to those 15 years and older was 1:19,1 and in the findings of the British Testicular Tumour Panel (BTTP), where among 952 cases of testicular tumors, 2% were in boys 0–14 years old.2 As a consequence many pathologists are not familiar with the morphologic spectrum and clinical behavior of these neoplasms. For purposes of this essay we restricted our analysis to those under 20 years of age. Although puberty could have been used as a breakpoint as it marks a significant milestone for the germ cell tumors, it is only as males age beyond 20 years that they acquire the highest risk for this group of tumors. If the age of 30 years is the breakpoint in females, beyond which the “usual” ovarian tumors occur with appreciable frequency, we believe 20 years represents the comparable cutoff in the males. Accordingly, this discussion will be confined to patients less than 20 years of age, with recognition that this is an arbitrary line. This essay reviews the common as well as uncommon tumors and tumor-like conditions that occur in and adjacent to the testis of young patients in an effort to call attention to features, if any, unique to this era and differences that may exist between the two gonads. Also, some lesions of the young tend to receive limited consideration when the entire field of testicular tumors and tumor-like lesions is considered, and this article offers an opportunity to give these lesions particular attention.
Before considering each neoplasm and tumor-like lesion in turn, we will first consider the broad differences in frequencies of the various categories in the first 2 decades as compared to all males. Further differences within the first 2 decades will be considered when individual entities are discussed.
Section snippets
The first decade
The most overt difference when only children in the first decade are considered is the decreased proportion of germ cell tumors compared to males as a group (Table 1). For practical purposes the only germ cell tumors that are seen in this decade are the yolk sac tumor and teratoma, with cases in the old literature designated as “infantile embryonal carcinoma,” all now being regarded as yolk sac tumors. An interesting aspect of the germ cell tumors in this group is their stable incidence,3
Germ cell tumors
Germ cell tumors of the testis are less common than those of the ovary and sacrococcygeal region in the 0–14-year age group, representing 18% of the total.6 They are, however, the most common form of testicular tumor during this period of life. Yolk sac tumor and teratoma accounted for 85% of the cases in a prepubertal testicular tumor registry of 395 neoplasms (Table 1).4 The earliest age, to the best of our knowledge, at which other germ cell tumors occurred in patients who did not have a
Sex cord–stromal tumors
Sex cord–stromal tumors represent the second most common category of testicular tumors in children. They represented 11% of the tumors in children younger than 12 years of age in the registry of 395 cases noted earlier,4 with unclassified sex cord–stromal tumors being slightly more common (4%) than juvenile granulosa cell tumors (3%) (Table 1). Brosman,104 in a literature review, found they were 15% of prepubertal testicular tumors, contrasting with the roughly 5% frequency in postpubertal
Lymphoma
Lymphomas may be either primary or secondary in the testis of children, with a proclivity for the secondary tumors to be Burkitt-type and the primary cases follicular.174, 175, 176, 177, 178, 179, 180, 181, 182, 183 In 23 cases of childhood B-cell non-Hodgkin׳s lymphoma that involved the testis, 78% occurred in the first decade from ages 2 to 9.5 years and 22% in the second, from 10 to 14 years. Overall, the mean age was 8.1 years.174
The most common lymphoma to involve the testis of children is
Hemangioma
Hemangiomas are rarely encountered in infants and older children.197, 198, 199, 200 Based on a review of reported cases, they occur with about equal frequency in patients within the first 2 decades and those beyond 20 years of age. There is also an approximately equal distribution of cases between the first decade and the second. In their series of 8, Kryvenko and Epstein201 found one in a 9-year old and a second in a 17-year old. Other reports documented cases in a 17-week-gestation fetus202
Metastatic tumors to the testis
Metastases secondary to neuroblastoma or Wilms׳ tumor occur in occasional cases.161, 193, 215, 216, 217, 218, 219, 220 Patients with testicular metastases of neuroblastoma may present with scrotal masses or the testicular involvement may be identified in the face of known disseminated disease. Most are under 1 year of age and show the typical morphological features. Based on the literature, Wilms׳ tumor appears to be a less common metastatic tumor in the testis than neuroblastoma. Since both
Testicular “tumor” of the adrenogenital syndrome
The testicular “tumor” of the adrenogenital syndrome is a non-neoplastic hyperplasia of steroid cells that characteristically develops near the testicular hilum but may also produce large intratesticular and paratesticular masses. These lesions occur in boys and young men with either C-21- or C-11-hydroxylase deficiency, resulting in secondary elevation of adrenocorticotropin that induces the hyperplasia. They occur from early childhood to adult years, with a mean age at diagnosis of 22.5
Adenomatoid tumor
The most common tumor of the paratestis,268 the adenomatoid tumor, a benign neoplasm of mesothelial origin, is not encountered in the first decade and is rare in the second. In the AFIP series, the youngest patient was 18 years old.269 The same features are seen in the young as in older patients.270, 271 There is a potential for infarction within adenomatoid tumors to cause a pseudomalignant appearance and diagnostic confusion.272
Mesothelioma
For a detailed discussion of the spectrum of the varied
Meconium periorchitis
Meconium periorchitis is the consequence of an intrauterine intestinal perforation with leakage of intestinal contents into the peritoneal cavity and subsequent passage through a patent processus vaginalis to localize adjacent to the testis.368, 369, 370 Most patients do not have cystic fibrosis, and the perforation typically is clinically silent. In their review, Jeanty et al.371 found 9% of 32 patients with meconium periorchitis tested positive for cystic fibrosis. This contrasted with a 25%
Summary
In this article, we have attempted to provide a comprehensive survey of the neoplastic and non-neoplastic masses that affect the testis and adjacent structures of patients less than 20 years old. One of the crucial lessons that becomes evident during the study of this group is that careful attention to the clinical history (always an important component of a proper pathologic analysis), in conjunction with the gross features and awareness of the proclivity of various lesions to preferentially
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