RESUMEN
Cells with cytologic and immunohistochemical features of Leydig cells are normally present in the ovary and the ovarian hilum, are testosterone-producing, and have been referred to as ovarian hilus cells. Rarely these cells form nests or nodules in extraovarian sites such as the mesovarium or mesosalpinx. Because they are so rare, these nodules can present a diagnostic challenge when first encountered. This report describes 2 such incidental nodules in the mesosalpinx associated with a small paratubal cyst and suggests that the term Leydig cell nodule be preferred over the nonspecific and confusing historical term ovarian hilus cell nest.
Asunto(s)
Tumor de Células de Leydig , Neoplasias Ováricas , Masculino , Femenino , Humanos , Células Intersticiales del Testículo , Neoplasias Ováricas/diagnóstico , Relevancia Clínica , Testosterona , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/complicacionesRESUMEN
Ovarian tumors are rare in children; however, their incidence increases with age. Of these ovarian tumors, Leydig cell tumors are some of the rarest, accounting for less than 0.1% of all ovarian tumors across all ages. Leydig cell tumors predominantly occur in postmenopausal women and are characterized by nodular proliferation of Leydig cells in the ovarian hilum with intracytoplasmic Reinke crystals. These tumors secrete androgens, which can disrupt ovarian function, clinically presenting with abnormal uterine bleeding and virilization. Although they are generally benign, current recommendations are for treatment with a unilateral salpingo-oophorectomy. In adolescents, hyperandrogenism is most commonly caused by polycystic ovarian syndrome (PCOS); however, the differential for hyperandrogenism is broad. We present a case of a 15-year-old girl with a history of primary amenorrhea who presented with a Leydig cell tumor associated with recurrent ovarian torsion and virilization. This case reviews the challenges with diagnosis, management, and future implications of a rare androgen-secreting tumor in young patients.
Asunto(s)
Hiperandrogenismo , Tumor de Células de Leydig , Neoplasias Ováricas , Niño , Humanos , Femenino , Adolescente , Tumor de Células de Leydig/complicaciones , Tumor de Células de Leydig/cirugía , Tumor de Células de Leydig/diagnóstico , Hiperandrogenismo/complicaciones , Virilismo/etiología , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/cirugía , AndrógenosRESUMEN
Ovarian Leydig cell tumor is a rare type of ovarian steroid cell neoplasms, presenting in only 0.1% of all ovarian tumor cases, and is generally androgen-secreting and unilateral. Although they are often malignant non-spreading tumors, which have excellent prognosis, benign ovarian Leydig cell tumors with low-risk malignancy can be also detected. Ovarian hyperthecosis is a rare non-neoplastic disorder, in most cases bilateral. Ovarian tumors and ovarian hyperthecosis are one of the main causes of hyperandrogenism in postmenopausal women, a condition strongly associated with both hormonal and metabolic changes. Here, we report a 65-year-old patient with complaints of excessive body hairiness and alopecia. The laboratory investigation showed increased levels of serum testosterone and dehydroepiandrosterone sulfate (DHEA-S). Imaging, including transvaginal ultrasound and pelvic MRI revealed the presence of two masses in the ovaries. The patient underwent a laparoscopic bilateral salpingo-oophorectomy due to the ovarian tumors unknown etiology, and histopathological examination revealed a unilateral benign left ovarian Leydig cell tumor with bilateral ovarian stromal hyperplasia and ovarian hyperthecosis. Making differential diagnosis between ovarian tumors and ovarian hyperthecosis is difficult. Bilateral salpingo-oophorectomy is the treatment of choice in postmenopausal women with benign Leydig cell ovarian tumor, as well as ovarian hyperthecosis, as it offers both a cure and diagnostic confirmation.
Asunto(s)
Hiperandrogenismo , Tumor de Células de Leydig , Neoplasias Ováricas , Síndrome del Ovario Poliquístico , Masculino , Humanos , Femenino , Anciano , Tumor de Células de Leydig/complicaciones , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/cirugía , Posmenopausia , Síndrome del Ovario Poliquístico/complicaciones , Hirsutismo/complicaciones , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/patología , TestosteronaRESUMEN
CONTEXT.: A recent study demonstrated that NKX3.1-positive staining can uncommonly be seen in testicular Sertoli cell tumors (1 of 4 cases). Also, it was reported that 2 of 3 Leydig cell tumors of the testis showed diffuse cytoplasmic staining for P501S, although it was unclear whether it was specific granular staining that defines true positivity. However, Sertoli cell tumors do not typically pose a diagnostic dilemma with metastatic prostate carcinoma to the testis. In contrast, malignant Leydig cell tumors, which are exceedingly rare, can closely resemble Gleason score 5 + 5 = 10 prostatic adenocarcinoma metastatic to the testis. OBJECTIVE.: To evaluate the expression of prostate markers in malignant Leydig cell tumors and steroidogenic factor 1 (SF-1) in high-grade prostate adenocarcinoma, as no data are currently published on these topics. DESIGN.: Fifteen cases of malignant Leydig cell tumor were collected from 2 large genitourinary pathology consult services in the United States from 1991 to 2019. RESULTS.: All 15 cases were negative immunohistochemically for NKX3.1, and all 9 with available additional material were negative for prostate-specific antigen and P501S and positive for SF-1. SF-1 was negative immunohistochemically in a tissue microarray with cases of high-grade prostatic adenocarcinoma. CONCLUSIONS.: The diagnosis of malignant Leydig cell tumor and its distinction from metastatic adenocarcinoma to the testis can be made immunohistochemically on the basis of SF-1 positivity and negativity for NKX3.1.
Asunto(s)
Adenocarcinoma , Tumor de Células de Leydig , Neoplasias de la Próstata , Neoplasias Testiculares , Humanos , Masculino , Adenocarcinoma/patología , Biomarcadores de Tumor , Tumor de Células de Leydig/diagnóstico , Antígeno Prostático Específico , Neoplasias de la Próstata/patología , Factor Esteroidogénico 1 , Factores de Transcripción , Neoplasias Testiculares/secundarioRESUMEN
A postmenopausal woman in her 60s was referred due to an elevated haemoglobin value found during her annual check-up. On physical examination, characteristic features of hyperandrogenism were observed which were not earlier mentioned. Laboratory investigations revealed polycythaemia accompanied by a normal erythropoietin and a negative analysis for JAK2-V617F mutation. A disproportionally and markedly elevated testosterone in combination with normal levels of adrenal androgens raised the suspicion of an ovarian source. CT scan showed nodular hyperdense lesions in both ovaries. A bilateral oophorectomy was performed and histological evaluation unfolded a Leydig cell ovarian tumour. Testosterone levels and haematological parameters normalised after surgery. Polycythaemia secondary to hyperandrogenism in postmenopausal women is an extremely rare condition and patients should be carefully analysed for the presence of androgen-secreting neoplasms. Diagnosis of the underlying pathology requires careful history, physical examination and comprehensive investigation. Treatment for this condition is surgery and resolves polycythaemia.
Asunto(s)
Hiperandrogenismo , Tumor de Células de Leydig , Neoplasias Ováricas , Policitemia , Andrógenos , Femenino , Humanos , Hiperandrogenismo/diagnóstico , Tumor de Células de Leydig/complicaciones , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/cirugía , Masculino , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/cirugía , Policitemia/complicaciones , TestosteronaAsunto(s)
Tumor de Células de Leydig , Neoplasias Ováricas , Tumor de Células de Sertoli-Leydig , Femenino , Humanos , Tumor de Células de Leydig/complicaciones , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/cirugía , Masculino , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/cirugía , Posmenopausia , Tumor de Células de Sertoli-Leydig/patología , Hemorragia Uterina/diagnóstico , Hemorragia Uterina/etiologíaRESUMEN
OBJECTIVES: An increasing number of incidental testicular tumors are diagnosed in patients during infertility workup. The aim of this study was to evaluate the accuracy of frozen section examination (FSE) for the management of these tumors. METHODS: We retrospectively studied a series of 46 testicular tumors diagnosed during exploration for infertility from 2000 to 2019 and submitted for FSE. RESULTS: A diagnosis of malignancy was made in 23 cases on both gross examination (yellow-white or cream-colored nodules for seminomas) and FSE, then confirmed on final diagnosis in 22 of the cases. One seminoma reported on FSE was revised as being a Leydig cell tumor. The 23 other lesions were diagnosed as benign on FSE, including 11 Leydig cell tumors (yellow-brown nodules), 2 Leydig cell hyperplasias, and 10 whitish fibrous lesions. All Leydig cell lesions were confirmed except 1, which was reclassified as a Sertoli cell tumor. Of the 10 cases of fibrous lesions, 6 were associated with malignancy. CONCLUSIONS: The high incidence of Leydig cell tumors and the accuracy of FSE for these lesions demonstrate the interest in FSE. In contrast, FSE is not reliable for fibrous lesions, and surgeons should be aware that a fibrosis result often corresponds with regressed tumors.
Asunto(s)
Infertilidad , Tumor de Células de Leydig , Seminoma , Neoplasias Testiculares , Humanos , Infertilidad/complicaciones , Tumor de Células de Leydig/complicaciones , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/patología , Masculino , Estudios Retrospectivos , Seminoma/complicaciones , Seminoma/diagnóstico , Seminoma/patología , Neoplasias Testiculares/patologíaRESUMEN
DICER1 syndrome is a rare paediatric autosomal dominant inherited disorder predisposing to various benign and malignant tumours. It is caused by a germline pathogenic variant in DICER1, and the second hit for tumour development is usually a missense hotspot pathogenic variant in the DICER1 ribonuclease IIIb domain. While DICER1 predisposing variants account for about 60% of ovarian Sertoli-Leydig cell tumours, no DICER1-related testicular stromal tumours have been described. Here we report the first two cases of testicular stromal tumours in children carrying a DICER1 germline pathogenic variant: a case of Sertoli cell tumour and a case of Leydig cell tumour diagnosed at 2 and 12 years of age, respectively. A somatic DICER1 hotspot pathogenic variant was detected in the Sertoli cell tumour. This report extends the spectrum of DICER1-related tumours to include testicular Sertoli cell tumour and potentially testicular Leydig cell tumour. Diagnosis of a testicular Sertoli cell tumour should prompt DICER1 genetic testing so that patients with a DICER1 germline pathogenic variant can benefit from established surveillance guidelines. DICER1 genetic evaluation may be considered for testicular Leydig cell tumour. Our findings suggest that miRNA dysregulation underlies the aetiology of some testicular stromal tumours.
Asunto(s)
Tumor de Células de Leydig , Síndromes Neoplásicos Hereditarios , Neoplasias Ováricas , Tumor de Células de Sertoli , Tumor de Células de Sertoli-Leydig , Neoplasias Testiculares , Niño , ARN Helicasas DEAD-box/genética , Femenino , Humanos , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/genética , Masculino , Neoplasias Ováricas/genética , Ribonucleasa III/genética , Tumor de Células de Sertoli/genética , Tumor de Células de Sertoli-Leydig/genética , Tumor de Células de Sertoli-Leydig/patología , Neoplasias Testiculares/genéticaRESUMEN
BACKGROUND Only 0.5% of all ovarian tumors are Leydig cell tumors and they are generally benign and unilateral. These androgen-secreting tumors lead to virilizing symptoms, most often in postmenopausal women. Because Leydig cell tumors are typically small, diagnosing them accurately can be challenging. CASE REPORT We report the case of a 77-year-old woman who was referred to our Endocrinology Clinic because of a 5-year history of hirsutism (Ferriman-Gallwey score of 11) with no discernible cause. The patient had high levels of serum testosterone and a normal level of dehydroepiandrosterone sulfate. Imaging, including transvaginal ultrasound and pelvic magnetic resonance, revealed a 16-mm uterine nodule, which was suspected to be a submucous leiomyoma, but no adrenal or ovarian lesions. Despite the lack of findings on imaging and because of the high suspicion for an androgen-secreting ovarian tumor, bilateral laparoscopic oophorectomy was performed. Histological examination of the specimen revealed a non-hilar Leydig cell tumor that measured 8 mm in its largest axis. After the surgery, the patient had significant clinical improvement and her laboratory test results normalized. Her sister had the same symptoms and laboratory findings at a similar age, which raised the suspicion of a possible familial genetic syndrome. No genetic testing was performed, however, because the patient's sister declined further diagnostic investigation. CONCLUSIONS Leydig cell tumors are rare, and even when they are small, they can cause symptoms related to androgen excess. As a result, diagnosing them often is challenging.
Asunto(s)
Tumor de Células de Leydig , Quistes Ováricos , Neoplasias Ováricas , Anciano , Femenino , Humanos , Tumor de Células de Leydig/complicaciones , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/cirugía , Masculino , Neoplasias Ováricas/diagnóstico , Posmenopausia , Virilismo/etiologíaRESUMEN
BACKGROUND: Tumors of the testes not originating from germinal epithelium are a rare entity and represent a diagnostic and therapeutic challenge. Leydig cell tumors (LCT) are rare stromal tumors of the testis. OBJECTIVES: To present current approaches in diagnostic and treatment of LCT. METHODS: A literature search in PubMed was performed and the currently available guidelines concerning LCT were evaluated. Articles and book chapters were selected based on relevance to daily practice. RESULTS: The low incidence of Leydig cell tumors not originating from the germinal epithelium, but from the stroma of the testis requires a standardized approach to determine relevant differential diagnosis and to optimize diagnosis and treatment depending on the current standard of knowledge and to determine whether it is benign or malignant. While more than 90% of LCT are benign and treatment is only restricted to the testis, malignant subtypes require radical surgical resection of the testicular and metastatic sites. CONCLUSION: A standardized diagnostic and therapeutic approach as well as a prospective registry of rare LCT could facilitate further detailed analysis to improve the understanding of tumor biology resulting in optimized therapeutic guidelines including follow-up strategies.
Asunto(s)
Tumor de Células de Leydig , Neoplasias Testiculares , Diagnóstico Diferencial , Humanos , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/terapia , Masculino , Neoplasias Testiculares/diagnóstico , Neoplasias Testiculares/terapiaRESUMEN
This postmenopausal patient developed hirsutism following a surgical procedure. Thorough lab work directed us to the unusual cause.
Asunto(s)
Hirsutismo/etiología , Tumor de Células de Leydig/diagnóstico , Neoplasias Ováricas/diagnóstico , Posmenopausia , Testosterona/sangre , Anciano , Femenino , Hirsutismo/sangre , Humanos , Tumor de Células de Leydig/metabolismo , Tumor de Células de Leydig/patología , Neoplasias Ováricas/metabolismo , Neoplasias Ováricas/patologíaRESUMEN
BACKGROUND: Pure Leydig cell tumors (LCTs) represent 0.1% of ovarian masses. Postmenopausal patients typically present with virilization. Although LCTs can be challenging to locate on conventional imaging, positron emission tomography (PET) has been demonstrated to be effective. CASE: A 64-year-old postmenopausal woman presented with alopecia, facial hirsutism, and clitoromegaly. Laboratory findings included elevated testosterone and androstenedione. Ultrasound, computed tomography, and magnetic resonance imaging showed no adnexal masses. PET did not demonstrate ovarian fludeoxyglucose-avidity. Histopathology after bilateral salpingo-oophorectomy revealed bilateral Leydig cell tumors. Her testosterone normalized 2 weeks postoperatively. CONCLUSION: We describe the occult, symptomatic, bilateral ovarian Leydig cell tumors, an occurrence that has not been described in the literature. Virilizing tumors must be considered in patients with evidence of hyperandrogenism, even without pelvic masses on imaging.
Asunto(s)
Tumor de Células de Leydig/patología , Neoplasias Primarias Múltiples/patología , Neoplasias Ováricas/patología , Virilismo/diagnóstico , Androstenodiona/metabolismo , Femenino , Humanos , Tumor de Células de Leydig/complicaciones , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/cirugía , Imagen por Resonancia Magnética , Persona de Mediana Edad , Neoplasias Primarias Múltiples/complicaciones , Neoplasias Primarias Múltiples/diagnóstico , Neoplasias Primarias Múltiples/cirugía , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/cirugía , Tomografía de Emisión de Positrones , Posmenopausia , Salpingooforectomía , Testosterona/metabolismo , Virilismo/etiología , Virilismo/metabolismoRESUMEN
Testicular Adrenal Rest Tumors, also known as Testicular Tumors of the Androgenital Syndrome, are benign tumors found in the testes of patients with congenital adrenal hyperplasia. While considered benign, they are significant in that they can proliferate within the rete testis and cause infertility. We present a patient who appeared to have findings consistent with testicular adrenal rest tumors and is in the process of malignancy rule out.
Asunto(s)
Tumor de Resto Suprarrenal/diagnóstico , Tumor de Células de Leydig/diagnóstico , Neoplasias Testiculares/diagnóstico , Adulto , Diagnóstico Diferencial , Humanos , MasculinoRESUMEN
A 71-year-old woman was referred to the endocrinology clinic to investigate postmenopausal hirsutism with 10 years of evolution. She had history of regular menses and menopause with 50 years old. Physical examination showed a male pattern facies, deepening of the voice, androgenic alopecia and hirsutism with a score of 23 according to the modified Ferriman-Gallwey scale. Testosterone and androstenedione were increased. Transvaginal ultrasound, abdominal and pelvic CT showed uterine fibroids with no pathological findings in the adrenals or ovaries. Since she had postmenopausal vaginal bleeding, uterine fibroids and suspicion of an ovarian source for her hyperandrogenism, total hysterectomy and bilateral oophorectomy were performed. Histopathological diagnosis was a Leydig cell tumour located in left ovary and endometrial carcinoma. Improvement of hirsutism was started to notice 1 month after the surgery and she was referred to the oncology clinic for adjuvant treatment.
Asunto(s)
Tumor de Células de Leydig , Neoplasias Ováricas , Anciano , Femenino , Hirsutismo/etiología , Humanos , Tumor de Células de Leydig/complicaciones , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/cirugía , Masculino , Persona de Mediana Edad , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/cirugía , Posmenopausia , Testosterona , VirilismoRESUMEN
AIMS: To investigate the morphological and molecular characteristics of Leydig cell tumours (LCTs) of the testis for the identification of cases that may metastasise. METHODS AND RESULTS: Six parameters for a predictive model of the metastatic risk were evaluated in 37 benign and 14 malignant LCTs of the testis [LCT Scaled Score (LeSS)]. The tumour size (benign LCTs, mean 13.3 mm; malignant LCTs, mean 44 mm) (P < 0.001) and five other parameters (infiltrative margins, necrosis, vascular invasion, mitotic count, and nuclear atypia) showed significant differences (Wilcoxon's test, P < 0.001). Eight metastatic LCTs and one benign LCT had infiltrative margins. Foci of coagulative necrosis occurred in 10 metastatic LCTs, whereas vascular invasion was identified in nine of 14 metastatic LCTs and none of 37 benign LCTs. Benign LCTs showed <2 mitoses/10 high-power fields (HPFs), whereas a high mitotic count (range, 3-50 mitoses/10 HPFs) was a feature of malignant LCTs. These parameters were selected by use of an inferential analysis based on univariate logistic regression models to develop a score. A LeSS of <4 correctly identified all histologically and clinically benign LCTs. A LeSS of ≥4 correctly identified all malignant LCTs. MDM2 and CDK4 immunostains were applied in all 51 cases: benign LCTs were negative; three of 11 malignant LCTs (27%) showed strong and diffuse immunopositivity and high levels of MDM2 and CDK4 amplification as determined with fluorescence in-situ hybridisation analysis and next-generation sequencing. CONCLUSION: We provide a new tool, the LeSS, for the prediction of malignant behaviour in LCTs.
Asunto(s)
Quinasa 4 Dependiente de la Ciclina , Tumor de Células de Leydig , Proteínas Proto-Oncogénicas c-mdm2 , Adulto , Anciano , Quinasa 4 Dependiente de la Ciclina/genética , Quinasa 4 Dependiente de la Ciclina/metabolismo , Secuenciación de Nucleótidos de Alto Rendimiento , Humanos , Inmunohistoquímica , Hibridación Fluorescente in Situ , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/patología , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Proteínas Proto-Oncogénicas c-mdm2/genética , Proteínas Proto-Oncogénicas c-mdm2/metabolismo , Neoplasias Testiculares/diagnóstico , Neoplasias Testiculares/patología , Testículo/patologíaRESUMEN
Androgen-producing tumors in women are rare neoplasms that can cause secondary virilizing characteristics. Of patients presenting with symptoms of hyperandrogenism, these tumors are found in â¼0.2% of cases. Androgen-producing tumors can arise from the ovary or the adrenal gland. Those arising from the ovary are rare, accounting for <5% of all ovarian tumors. This case presents a hilar Leydig cell tumor of the ovary, which resulted in secondary virilization of a 45-year-old female 2 months after cessation of combined oral contraceptives (COC). Laboratory findings showed markedly elevated total and free testosterone concentrations with normal dehydroepiandrosterone sulfate, however neither pelvic ultrasound nor magnetic resonance imaging demonstrated any masses. Venous sampling under fluoroscopy revealed supraphysiologic testosterone concentrations from the right ovarian vein suggesting the source. The patient underwent bilateral salpingo-oophorectomy revealing a 1.3 cm hilar cell tumor of the right ovary. This article reviews the clinical features, diagnosis, and treatment of hilar Leydig cell tumors and describes the long-term complications of supraphysiologic testosterone levels. As the tumor presented after cessation of COC, we also review the mechanisms by which COC might suppress supraphysiologic androgen levels and mask the secondary virilizing effects of androgen-producing tumors.
Asunto(s)
Hiperandrogenismo , Tumor de Células de Leydig , Neoplasias Ováricas , Femenino , Humanos , Hiperandrogenismo/etiología , Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/cirugía , Masculino , Persona de Mediana Edad , Neoplasias Ováricas/diagnóstico , Ovario/diagnóstico por imagen , Testosterona , Ultrasonografía , VirilismoRESUMEN
A 64-year-old postmenopausal female patient presented with approximately 5 years of intermittent spotting, progressive hirsutism and significantly increased libido and clitoral hypersensitivity with spontaneous orgasms multiple times a day beginning a few months prior. Initial hormone work-up revealed elevated total serum testosterone, androstenedione and 17-hydroxyprogesterone. Luteinising hormone, follicle stimulating hormone, estradiol, dehydroepiandrosterone-sulfate, thyroid stimulating hormone and prolactin were all within normal limits. Initial suspicions suggested an androgen-secreting tumour, likely in the ovary. The lesion was undetectable on transvaginal ultrasound and abdominal-pelvic CT scan. Laparoscopic bilateral salpingo-oophorectomy was performed to remove the likely source of excess androgens. Visible gross lesions were not observed intraoperatively; however, bilateral Leydig (hilus cell) tumours were confirmed by histopathology. Serum testosterone, androstenedione and 17-hydroxyprogesterone levels were normalised postoperatively within 2 weeks and 1 month, respectively.
Asunto(s)
Hirsutismo/etiología , Tumor de Células de Leydig/diagnóstico , Neoplasias Ováricas/diagnóstico , Ovario/patología , 17-alfa-Hidroxiprogesterona/sangre , Androstenodiona/sangre , Femenino , Humanos , Tumor de Células de Leydig/sangre , Tumor de Células de Leydig/complicaciones , Tumor de Células de Leydig/cirugía , Persona de Mediana Edad , Neoplasias Ováricas/sangre , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/cirugía , Ovario/cirugía , Posmenopausia , Salpingooforectomía , Testosterona/sangre , Resultado del TratamientoRESUMEN
PURPOSE: Leydig-cell tumours (LCT) of the testis are poorly understood clinically. The aim of this report is to analyse the clinical characteristics of LCT in a large patient sample and to compare these findings with corresponding data of germ-cell tumours (GCT). METHODS: In a sample of 208 patients treated during 1995-2017 in 33 institutions, the following characteristics were registered: age, presenting symptoms, primary tumour size, testis-sparing surgery (TSS) or orchiectomy, malignancy, laterality, medical history, and outcome. Data analysis included descriptive statistical methods and logistic regression analysis. RESULTS: The ratio LCT:GCT is 1:23 (4.4%). The findings are as follows: median age 41 years, undescended testis 8%, bilateral LCTs 3%, malignant LCT 2.5%, contralateral GCT 2.5%, incidental detection 28%, scrotal symptoms 43%, infertility 18%, elevated estradiol levels 29%. TSS was performed in 56% with no local relapse. Of the patients with malignant LCT, one was cured through surgery. CONCLUSION: LCT is rare, with a relative frequency (relative to GCT) of 1:23. Malignancy is found in 2.5%. LCT and GCT share a number of clinical features, e.g. bilaterality, history of undescended testis, and presenting age. TSS is safe in benign LCT. Surgery is the treatment of choice in malignant LCT.
Asunto(s)
Tumor de Células de Leydig/diagnóstico , Tumor de Células de Leydig/cirugía , Neoplasias de Células Germinales y Embrionarias/diagnóstico , Neoplasias de Células Germinales y Embrionarias/cirugía , Neoplasias Testiculares/diagnóstico , Neoplasias Testiculares/cirugía , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto JovenRESUMEN
PURPOSE: Leydig cell tumors are rare but they are the most common nongerm cell testicular tumors. Only limited evidence exists for reliably differentiating between benign and malignant Leydig cell tumors and for optimally managing the different types and stages of this rare disease. In this review we synthesize the available evidence on the clinical presentation and clinicopathological characteristics associated with Leydig cell tumor malignancy and management. MATERIALS AND METHODS: We analyzed published case series data on Leydig cell tumors. The association between clinicopathological variables and the presence of metastatic disease was assessed using regression analyses. RESULTS: We included 357 reports, reviewing available data from 1,375 patients (median age 34 years). Testis sparing surgery was performed in 463 patients. Local recurrence after testis sparing surgery occurred in 8 of 121 (7%) patients with available followup information. Metastases were found in 101 patients and were most often located in the retroperitoneal lymph nodes (60%), lungs (38%) and/or liver (29%). The multivariable models with or without multiple imputation predicting metastatic disease included older age, larger tumor size, presence of any adverse factor (larger tumor diameter, necrosis, angiolymphatic invasion, pleomorphism, high mitotic index, atypia) and any protective factor (Reinke crystals, lipofuscin pigments, gynecomastia) with model AUCs of 0.93. Durable remission after resection of metastases or use of platinum based chemotherapy was rarely seen. CONCLUSIONS: Our risk tables using clinicopathological parameters can help identify patients with malignant tumors. These patients should undergo disease staging and be followed or receive further treatment. In some patients with metastatic disease surgical and systemic treatment might result in disease control.