Clinical Outcome in Testicular Sex Cord Stromal Tumors: Testis Sparing vs Radical Orchiectomy and Management of Advanced Disease

Nicolai, Nicola; Necchi, Andrea; Raggi, Daniele; Biasoni, Davide; Catanzaro, Mario; Piva, Luigi; Stagni, Silvia; Maffezzini, Massimo; Torelli, Tullio; Farè, Elena; Giannatempo, Patrizia; Pizzocaro, Giorgio; Colecchia, Maurizio; Salvioni, Roberto

doi:10.1016/j.urology.2014.10.021

Cited by 47 publications

(37 citation statements)

References 23 publications

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“…In this study, it was emphasized that the vast majority of testicular MSCSTs present initially as stage I disease [9]. Multiple published studies have emphasized that the prognosis of MSCSTs is extremely favorable, with cure rates exceeding 90% for MSCSTs in general and for each of the tumor subtypes [5][6][7][8][9]. A series of clinical and pathological features have been cited for predicting the development of metastatic disease in testicular MSCSTs [5].…”

Section: Discussionmentioning

confidence: 99%

“…The majority of these tumors are clinically benign and will not progress to metastatic disease. However, 5-10% of testicular SCSTs are malignant (metastatic testicular SCSTs [MSCSTs]), and either present with metastatic lesions or relapse with metastases typically to retroperitoneal lymph nodes [5][6][7][8][9]. Curative therapeutic options in case of metastatic tumors are limited to the possibility to perform a radical surgical extirpation of the disease [5,7].…”

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confidence: 99%

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Genomic Features of Metastatic Testicular Sex Cord Stromal Tumors

Necchi

Bratslavsky

Shapiro

et al. 2019

European Urology Focus

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Background: Metastatic testicular sex cord stromal tumors of the testis (MSCSTs) comprise an extremely uncommon form of genitourinary malignancy. Objective: To perform comprehensive genomic profiling (CGP) to enable the search for potential therapy targets. Design, setting, and participants: Ten patients with testicular Leydig cell tumors (LCTs), six with Sertoli cell tumors (SCTs), and three with undifferentiated sex cord stromal tumors (USCSTs) and a comparison group of 366 patients with ovarian sex cord stromal tumors (SCSTs) underwent hybrid-capture-based CGP to evaluate all classes of genomic alterations (GAs). The tumor mutational burden (TMB) was determined on 1.1 Mbp of sequenced DNA, and microsatellite instability (MSI) was determined on 114 loci. Intervention: CGP on tumor samples. Outcome measurements and statistical analysis: Descriptive analyses and differences between histological subgroups were reported. Results and limitations: In these patients, all of whom had metastatic disease at the time of sequencing, the primary testis tumor was sequenced in six (32%) patients and a metastatic site in 13 (68%) patients. The overall frequencies of GAs were similar in LCTs, SCTs, and USCSTs, ranging from 3.0 to 3.5 GAs/tumor. The most frequent untargetable GAs included CTNNB1 and CDKN2A/B, both ranging from 20% to 33% of cases. Targetable GAs were uncommon in all MSCST subgroups, but several tumors showed potential for cell-cycle inhibitors (CDK4 in LCTs), mTOR inhibitors (RICTOR, NF2, and PTEN in all three tumor types), hedgehog inhibitors (PTCH1 in LCTs), and poly(ADP-ribose) polymerase inhibitors (BAP1 in SCTs). No MSI-high status was identified. The TMB was also low in all MSCST groups, and tumors featuring a TMB of !10 mutations/Mb were not identified. GA findings from ovarian SCSTs largely recapitulated those from MSCSTs. A lack of clinical outcome correlation is a limitation of the present analyses. Conclusions: Rare cases of testicular MSCSTs have GAs linked to potential targeted therapy benefits on CGP. In contrast, the lack of MSI-high status and an overall low TMB indicate a likely lack of benefit for immunotherapies. Patient summary: Genomic profiling can guide clinical research and disclose therapeutic opportunities for patients with rare testicular cancers for which standard therapies are lacking.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Genomic Features of Metastatic Testicular Sex Cord Stromal Tumors

Necchi

Bratslavsky

Shapiro

et al. 2019

European Urology Focus

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“…In adults, sex cord/stromal tumours of the testis are found in less than 5% of testicular neoplasms,10 whereas in children these tumours represent up to 40% of cases.…”

Section: Discussionmentioning

confidence: 99%

Adult testicular granulosa cell tumour: an extremely rare entity

et al. 2018

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Adult granulosa cell tumours are extremely rare and usually benign but sometimes can assume an aggressive behaviour. A 31-year-old man presented with a 45 mm testicular mass. Radical orchiectomy was performed. Histological examination showed elongated cells, with hyperchromatic nuclei and Call-Exner bodies. Immunohistochemical analysis revealed positivity to vimentin, inhibin and negative PLAP and AE1/AE3 staining. The patient did not receive any adjuvant therapy and remained asymptomatic during 10 years. There are few cases of adult granulosa cell tumours and there is no consensus regarding malignancy criteria, treatment and follow-up. The description of these entities is crucial to allow a better management of these patients.

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“…In the TSS cohort, authors observed no cases of metastasis or death, but did identify 1 patient with local recurrence, who required orchiectomy 26 months later. No specific difference was appreciated in the behavior of Leydig cell and Sertoli cell tumors; however, the sample size was small [41].…”

Section: Application To Adolescent Patients Extrapolating From the Amentioning

confidence: 97%

“…Most of these tumors exhibit favorable clinical behavior, and TSS for the treatment of TSCST has been reported with greater frequency in the last few decades [41]. In Giannarini's review, the 4 largest series of men treated by TSS for Leydig cell tumors reported no cases of local or distant recurrence during a 4 to 8 years follow-up.…”

Section: Application To Adolescent Patients Extrapolating From the Amentioning

confidence: 99%