Key result
Aromatase inhibitors treat rare large-cell calcifying Sertoli cell tumors linked to Peutz-Jeghers or Carney complex.
LCCSCTs in children are typically benign and can often be managed conservatively or with aromatase inhibitors rather than orchiectomy, while prompting evaluation for underlying genetic syndromes.
May support aromatase inhibitors and syndrome screening over orchiectomy in pediatric LCCSCT; leaves open prospective trials to guide practice.
PURPOSE OF REVIEW: The aim of this review is to describe the clinical, biochemical, radiographic, histological, and functional characteristics of large-cell calcifying Sertoli cell tumors of the testes (LCCSCTs). We describe the two main syndromes associated with these tumors: Peutz-Jeghers syndrome (PJS) caused mainly by mutations in the STK11 (aka LKB1) gene, which encodes a serine-threonine kinase, and Carney complex (CNC), which is most often caused by PRKAR1A mutations, the gene encoding regulatory subunit type 1 of protein kinase A. RECENT FINDINGS: Relatively few patients have been reported in the literature with LCCSCTs. In children they often present as prepubertal and/or peripubertal gynecomastia. Although these tumors are very rare, they occur with higher frequency among patients with PJS and CNC. Orchiectomy was often performed in the past; however, these tumors are overwhelmingly benign and, unless there are significant hormonal changes or pain and/or mass effects, there is no need for surgery. Tumors that lead to hyperestrogenemia may be treated efficiently with aromatase inhibitors; any change in appearance should prompt evaluation for malignancy. SUMMARY: The detection of LCCSCTs may point to an underlying genetic multiple neoplasia syndrome such as PJS or CNC. Surgery is rarely indicated and aromatase inhibitors constitute an effective treatment for those cases that are associated with gynecomastia and/or advanced skeletal age.
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Gourgari et al. (2012) conducted a review in Large-cell calcifying Sertoli cell tumors of the testes (LCCSCTs). Large-cell calcifying Sertoli cell tumors of the testes are rare, benign tumors often associated with Peutz-Jeghers syndrome or Carney complex, and can be treated with aromatase inhibitors.
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