Testicular Stromal Tumors
Testicular Stromal Tumors
While only 5% of primary testicular tumors are Testicular Stromal Tumors (TSTs), they represent the 2nd most common testicular tumor in both pre- and post-pubertal males. Types of TSTs include Granulosa Cell Tumors, Leydig Cell Tumors, Sertoli Cell Tumors and Undifferentiated TSTs. TSTs originate from the cells in the testicle that surround and support the fertility function of the testicle and as part of their normal function may make hormones such as testosterone or estrogen.
Nearly all testis tumors present with a painless scrotal mass that is usually palpable and seen on ultrasound. However, some may present with signs of hormone production such as early-onset of puberty and/or enlarging breasts. Pre-pubertal testis tumors are rare and most behave in a benign fashion. Final diagnosis requires a pathologist to analyze the tumor under a microscope and so surgical removal of the tumor itself (Testicle Sparing Surgery, TSS) or the entire testicle (Orchiectomy) is needed. Pathologic findings can help assess the tumor’s malignant potential.
Infants with testicular juvenile granulosa cell tumor usually present within the first 6mo of life with a painless mass and no associated symptomsThese have been observed to behave in a benign fashion with no evidence for recurrence or metastases. Leydig cell tumors generally occur in children 5-10 years of age or in older men, and may produce testosterone or estrogen and thus patients may present with early-onset of puberty and/or enlarging breasts.
Testicular juvenile granulosa cell tumors may be seen in boys with otherwise normal genetic profiles or in those with abnormalities of the Y chromosome or ambiguous genitalia, thus chromosomal tests may be helpful. Testicular Sertoli cell may be seen with Peutz-Jager Syndrome. Carney syndrome may be associated with bilateral large cell calcifying Sertoli cell tumors or with Leydig cell tumors.
Clinical evaluation of patients suspected to have a TST should include a history and examination with careful attention to family history and signs of a genetic tumor predisposition syndrome. The patient’s pubertal status is critical as it may guide management decisions. Pre-pubertal boys with smaller tumors are considered for TSS as opposed to pubertal and post-pubertal adolescents or adults who are more routinely managed with Orchiectomy.
Principles of therapy
As mentioned above, surgical management of TSTs must account for the pubertal status of the patient as well as the pathologic diagnosis. In older children and adults TSTs may behave in a more malignant fashion. As indicated in the introduction, the pathology from the TSS or orchiectomy may guide clinical management. A full metastatic evaluation and careful follow-up should be undertaken. Generally speaking, localized TSTs with no evidence of metastases should be followed with periodic imaging and examination. Up to 10% of such lesions may recur and early recognition may allow for a better prognosis. The role of further therapy after TSS or Orchiectomy in patients with concerning pathology but no evidence of metastases on radiologic scans is unclear. If metastases are found whether at diagnosis or later recur, they likely require aggressive surgery and chemotherapy.
We are currently investigating this specific issue so please contact us if your case meets this description and wish to learn more.