Leydig Cell Tumors Treatment & Management

Updated: Dec 18, 2019
  • Author: Thomas Calvert, MD, MPH; Chief Editor: Edward David Kim, MD, FACS  more...
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Treatment

Medical Care

Medical therapy plays little role in the management of Leydig cell tumor, as follows:

  • Chemotherapy with the bleomycin-etoposide-platinum regimen used for germ cell malignancies has limited efficacy in managing malignant Leydig cell tumors [20]
  • The tyrosine kinase inhibitor imatinib has shown some chemotherapeutic activity in animal models, [10, 21]
  • No known role exists for radiation therapy in malignant Leydig cell tumors [22]
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Surgical Care

Leydig cell tumors have been primarily managed with surgical extirpation using radical inguinal orchiectomy. Inguinal orchiectomy should be performed with early control of the spermatic cord and without violation of the scrotal skin.

However, testis-sparing surgery with enucleation of the mass, to maintain fertility, has been increasingly reported in children and younger adults. [23, 24]  Typically, this testis-sparing approach is performed through an inguinal or scrotal incision, and intraoperative ultrasound guidance has been used to locate nonpalpable tumors. 

The rationale for testis-sparing surgery is that testicular sex cord stromal tumors differ from germ cell tumors as they are not multifocal, are not associated with precancerous lesions (ie, testicular intratubular neoplasia) that could progress to invasive cancer, and have shown a low rate of local recurrence. These aspects seem to be appropriate premises to justify testis-sparing surgery in the case of testicular sex cord stromal tumors. [8, 25]

In testisi-sparing surgery, the mass is enucleated with a small surrounding edge of testicular parenchyma and immediately sent for frozen section analysis. Frozen section examination successfully discriminated between benign and malignant neoplastic lesions in a study of 86 patients with testicular nodules, including five patients with Leydig cell tumors and six patients with Leydig cell hyperplasia. [26]

Additional frozen sections of the tumor bed can be assessed and/or a radical inguinal orchiectomy can be performed if malignancy is subsequently suspected. If the tumor appears malignant, retroperitoneal lymph node dissection (RPLND) is also recommended.

In a retrospective study of 25 patients with testicular tumors who underwent testis-sparing surgery, including four patients with Leydig tumors, overall survival was 100% at a mean follow-up of 42.7 months. Three patients experienced local recurrence. Frozen-section examinations were performed in tumors from 14 patients; results matched the final pathological analysis in 11. None of the patient with a preserved testicle needed androgen therapy. [27]

Retroperitoneal lymph node dissection

Limited data are available regarding the role of RPLND for Leydig cell tumors. Moshara et al reported on 4 patients with clinical stage II who underwent RPLND; all of them had documented disease, but 1 patient remained alive and disease free. [28]   Silberstein et al reported on 2 patients with clinical stage IIa who underwent RPLND; disease was confirmed in one and both remained alive and disease free. [29]

Nicolai et al described successful treatment with RPLND alone in four of five patients with retroperitoneal metastasis, which had been identified at presentation in three patients and at follow-up in one patient who had a delayed intervention for a small retroperitoneal recurrence.  Three of the five patients undergoing immediate RPLND had nodal metastasis; all three, and another node-negative patient remained alive and disease free, whereas one patient with negative nodes developed distant metastasis and ultimately died of disease.  All three patients with at least three risk factors actually had metastasis. [27]

 

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Long-Term Monitoring

Observation is sufficient in patients with a benign Leydig cell tumortreated with radical inguinal orchiectomy.

Patients with malignant tumors require regular follow-up imaging, including CT scanning of the chest and abdomen. Metastases most frequently involve the retroperitoneal lymph nodes. Other reported metastatic sites include the liver (45%), lungs (40%), and bone (25%).

The ideal frequency of subsequent abdominal CT scanning and chest imaging is poorly defined. However, a reasonable follow-up protocol includes a chest imaging study and abdominal CT scanning every 4 months during the first year, followed by similar imaging at 6-month intervals during the second year and yearly examinations thereafter. [8]

Late onset of metastasis have been reported as long as 8 and 17 years after orchiectomy. [12] This supports the recommendation of long-term tumor surveillance, continuing for 10-15 years after surgery.

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