Imaging in gynecological disease (27): clinical and ultrasound characteristics of recurrent ovarian stromal cell tumors.

OBJECTIVE: To describe the clinical and ultrasound characteristics of recurrent granulosa cell and Sertoli-Leydig cell tumors. METHODS: This was a retrospective observational study performed at Fondazione Policlinico Universitario A. Gemelli, Istituto di Ricovero e Cura a Carattere Scientifico, IRCCS, Rome (Gemelli center), Italy. Patients with a histological diagnosis of recurrent granulosa cell tumor or Sertoli-Leydig cell tumor were identified from the database of the Department of Gynecological Oncology. Those who had undergone a preoperative ultrasound examination at the Gemelli center between 2012 and 2020 were included, and the data retrieved from the original ultrasound reports. In all of these reports, the recurrent tumors were described using International Ovarian Tumor Analysis (IOTA) terminology. If a patient had more than one episode of relapse, information from all episodes was collected. If there was more than one recurrent tumor at the same ultrasound examination, all tumors were included. One expert sonographer also reviewed all available ultrasound images to identify typical ultrasound patterns using pattern recognition. RESULTS: We identified 30 patients with a histological diagnosis of recurrent granulosa cell tumor (25 patients, 55 tumors) or Sertoli-Leydig cell tumor (five patients, seven tumors). All 30 had undergone at least one preoperative ultrasound examination at the Gemelli center and were included. These women had a total of 66 episodes of relapse, of which a preoperative ultrasound examination had been performed at the Gemelli center in 34, revealing 62 recurrent lesions: one in 22/34 (64.7%) episodes of relapse, two in 4/34 (11.8%) episodes and three or more in 8/34 (23.5%) episodes. Most recurrent granulosa cell tumors (38/55, 69.1%) and recurrent Sertoli-Leydig tumors (6/7, 85.7%) were classified as solid or multilocular-solid tumors, while 8/55 (14.5%) recurrent granulosa cell tumors and 1/7 (14.3%) recurrent Sertoli-Leydig cell tumors were unilocular cysts and 9/55 (16.4%) recurrent granulosa cell tumors were multilocular cysts. The nine unilocular cysts had contents that were anechoic (n = 2) or had low-level echogenicity (n = 7), had either smooth (n = 4) or irregular (n = 5) internal cyst walls, and ranged in largest diameter from 8 to 38 mm, with three being < 20 mm and five being 20-30 mm. On retrospective review of the images, two typical ultrasound patterns were described: small solid tumor measuring < 2 cm (15/62, 24.2%) and tumor with vascularized echogenic ground-glass-like content (12/62, 19.4%). CONCLUSIONS: Some granulosa cell and Sertoli-Leydig cell recurrences manifest one of two typical ultrasound patterns, while some appear as unilocular cysts. These are usually classified as benign, but in patients being followed up for a granulosa cell tumor or Sertoli-Leydig cell tumor, a unilocular cyst should be considered suspicious of recurrence. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Sertoli-Leydig cell tumor of ovary in children: A report of two cases, including retiform variant.

Sertoli-Leydig cell tumors (SLCTs) are rare and heterogeneous group of ovarian neoplasms which belong to the sex cord-stromal category of tumors. SLCTs are classified into well, intermediate, and poorly differentiated types. Retiform growth pattern and heterologous elements are commonly found in moderately and poorly differentiated tumors. SLCTs are usually encountered in the third decade of life and patients most often present with virilization. Here, we report two cases of SLCTs of the ovary, both in 2-year-old girls without any hormonal symptoms. The first case was a retiform variant of Sertoli-Leydig cell tumor and the second was a well-differentiated SLCT. Because of its wide spectrum of morphology, several tumors enter in the differential diagnosis and the presence of heterologous elements further complicates the diagnosis. Here, we have described the morphological characteristics of these tumors and discussed their differential diagnoses. SF-1, WT1, and α-inhibin are useful immunostains in establishing the diagnosis and differentiating these from the more the common ovarian germ cell tumors in children.

Tumor de células de Sertoli-Leydig: a propósito de un caso y revisión de la literatura reciente / Ovarian Sertoli-Leydig cell tumour: a clinical case and recent basic literature review

INTRODUCCIÓN: Los tumores de células de Sertoli-Leydig son neoplasias de ovario infrecuentes, lo que dificulta su diagnóstico y tratamiento. OBJETIVO: Revisar y sintetizar el manejo actual de los tumores de células de Sertoli-Leydig. MÉTODO: Se realizó una revisión de la literatura reciente sobre tumores de células de Sertoli-Leydig, a propósito de un caso en nuestro centro. RESULTADOS: Los tumores de las células de Sertoli-Leydig son infrecuentes, con mayor incidencia en edades tempranas. Ante una paciente joven con una lesión anexial unilateral y signos de virilización deberán considerarse estos tumores dentro del diagnóstico diferencial. En los estadios iniciales y en pacientes jóvenes podrá plantearse un tratamiento quirúrgico que preserve la fertilidad, y la asociación de tratamiento adyuvante dependerá de la diferenciación y del estadiaje del tumor.

INTRODUCTION: Sertoli-Leydig cell tumors are infrequent ovarian neoplasms, which difficults their diagnosis and treatment. Objective: To review and synthesize the current management of the Sertoli-Leydig cell tumor. METHOD: A review of the recent literature regarding the Sertoli-Leydig cell tumor was carried out, regarding a case in our center. RESULTS: Sertoli-Leydig cell tumors are an infrequent entity, with a higher incidence in early ages. In a young patient with a unilateral adnexal lesion and signs of virilization, these tumors should be considered within the differential diagnosis. In early stages and young patients, a surgical treatment that preserves fertility may be considered, and the association of adjuvant treatment will depend on the differentiation and staging of the tumor.

Imaging, clinical, and pathologic findings of Sertoli-leydig cell tumors.

To explore the clinical features, imaging findings, and pathological manifestations of ovarian Sertoli-Leydig cell tumors (SLCTs). The clinical and pathological manifestations, tumor location, size, morphology, vascularity, computed tomography (CT) density, magnetic resonance imaging (MRI) signal intensity, and contrast enhancement patterns in five cases with SLCTs were retrospectively reviewed. SLCTs most commonly occurred in young women. Virilization was observed in three cases (60%). All five tumors were unilateral and oval or round, with a clear boundary. The solid part of the tumor was isoattenuated on the conventional CT scan, and showed isoattenuation or slight hypoattenuation relative to adjacent myometrium on T1 weighted imaging (T1WI) and T2 weighted imaging (T2WI). On contrast-enhanced images, three tumors showed marked enhancement. DICER1 hotspot mutations were commonly seen in SLCTs. A highly vascularized mass with low signal intensity (SI) of the solid part on T2WI and androgen overproduction symptoms may suggest an SLCT.

Ovarian Sertoli-Leydig Cell Tumor With Heterologous Hepatocytes and a Hepatocellular Carcinomatous Element.

Sertoli-Leydig cell tumors are a group of tumors composed of variable proportions of Sertoli cells, Leydig cells, and sometimes heterologous elements. We describe the case of a 68-yr-old woman who presented with abdominal distention. A computed tomographic scan revealed a large right adnexal mass without evidence of intrahepatic tumors, and a complete cytoreductive surgery was performed. Pathologic examination revealed a moderately differentiated Sertoli-Leydig cell tumor with various heterologous elements, including gastrointestinal-type glands, insular carcinoid, and aggregations of hepatocytes without significant cytologic atypia. Moreover, adjacent to these hepatocytes, extensive overgrowth of highly atypical hepatocyte-like cells, providing a striking morphologic similarity to hepatocellular carcinoma of the liver, was identified. Both the heterologous hepatocytes and hepatocellular carcinomatous tumor cells were immunohistochemically positive for alpha-fetoprotein, hepatocyte paraffin 1, and arginase-1. Some Sertoli cells adjacent to the heterologous hepatocytes were also positive for alpha-fetoprotein and hepatocyte paraffin 1. The present case showed that a tumor morphologically and immunohistochemically analogous to hepatocellular carcinoma of the liver can arise in the ovary, in association with Sertoli-Leydig cell tumors.

Sertoli-Leydig cell tumors of ovary: A case series.

INTRODUCTION: The purpose of this study was to report the clinical features, computed tomography (CT) and magnetic resonance imaging (MRI) findings, clinical management, and prognoses of 7 patients with Sertoli-Leydig cell tumors (SLCT) of ovary, and to review the literature of this rare condition. METHODS: Seven patients with pathologically confirmed ovarian SLCT were included. Their clinical, CT and MRI characteristics (CT images obtained from 6 patients and MR images from 4 patients), clinical management, and prognoses of 7 patients were retrospectively analyzed. RESULTS: Patients symptoms included irregular menstruation (n = 3), infertile (n = 1), vaginal bleeding after 7 years of menopause (n = 1), a palpable abdominal mass (n = 1), and abdominal pain (n = 1). Three patients had elevated alpha-fetoprotein (AFP), 1 had elevated cancer antigen 125 (CA125), and 2 had elevated Testosterone (T). The 7 tumors of 7 patients were solid or mixed solid-cystic mass with clear boundaries. The solid components of the tumors showed iso-dense on CT. On MRI, the solid components showed iso- or slightly low signal intensity (SI) on T1-weighted imaging (T1WI), high or slightly high SI on T2WI, and high on diffusion-weighted imaging (DWI) with low apparent diffusion coefficient (ADC) value. On contrast-enhanced CT and MRI, 1 tumor exhibited heterogeneous enhancement consisting of multiple nodules with relatively marked homogeneous enhancement, and other 6 tumors showed moderate or marked and constantly heterogeneous enhancements. All patients were treated with surgical excision. Only 3 had received postoperative chemotherapy. With the exception of 1 patient lost to follow-up, the other 6 patients exhibited tumor-free survival with a median follow-up time of 13.5 months, the longest follow-up time being 24 months. CONCLUSION: The patients of SLCT can present with hormonal magnification and manifest high AFP, CA125, and T levels. SLCT is characterized by a solid or mixed solid-cystic mass on CT/MR scans, and shows marked or moderated heterogeneous and constantly enhancement upon postcontrast study. The clinical characteristics and imaging findings are features and appropriated imaging should be performed whenever an SLCT is suspected.

Sertoli - Leydig cell tumor with retiform areas and overgrowth of rhabdomyosarcomatous elements: case report and literature review.

BACKGROUND: Sertoli - Leydig cell tumors (SLCTs) are sex-cord stromal tumors that account less than 0.5 % of primary ovarian neoplasms. They are mostly benign and occur in reproductive age women. Variants with heterologous mesenchymal elements are exceptionaly rare. The usual presentation of SLCTs is with signs of androgen excess as majority of them produce androgens. CASE PRESENTATION: We present a case of a SLCT occurring in a 70 year old woman. Her presenting complaint was abdominal distension and pain. She had no signs of androgen or estrogen excess. Transvaginal ultrasound (TVUS) and CT scan showed a multilocular adnexal tumor and level of CA 125 was raised. A complete cytoreduction was achieved with surgical procedure. Histopathological examination revealed moderately differentiated SLCT with retiform areas and owergrowth of heterologous component in form of embrional rhabdomyosarcoma (RMS). She returned 7 months after the surgery with a large abdominal mass, ascites, right- sided hydronephrosis and massive pulmonary embolism. Due to the widespread disease and her poor general condition, she received only palliative care. She died 15 days after the admission. No autopsy was performed. CONCLUSIONS: Due to the rarity of SLCTs, especially those with retiform areas and heterologous elements, their management remains challenging. There is no firm evidence that adjuvant chemotherapy is effective in improving survival in SLCTs with malignant heterologous elements. Further studies with a higher number of cases and a longer follow-up are needed to better predicting the prognosis and determine the role of chemotherapy in such cases.

Recurrent Sertoli-Leydig cell tumor of ovary in 8-year-old girl.

Sertoli-Leydig cell tumors are rare sex cord-stromal neoplasms that account for <0.2% of ovarian tumors. These tumors with a retiform pattern pose difficult diagnostic problems, with the majority of being misinterpreted as serous papillary cystadenocarcinoma and endodermal sinus tumor. We report an 8-year-old female patient presented to our institution with a huge mass and pain in the lower abdomen and recurrence in the 10th months following the first operation. Only four cases of Sertoli-Leydig cell tumors have been reported under age of the eight years in the literature so far. It is difficult to define the stage and the morphology of Sertoli-Leydig cell tumors with retiform pattern in children and chemotherapy or radiotherapy administration is contraversial. However, fertility sparing surgeries should be considered as a first treatment choice on the time of the diagnosis and the recurrence.

DICER1 mutations in a patient with an ovarian Sertoli-Leydig tumor, well-differentiated fetal adenocarcinoma of the lung, and familial multinodular goiter.

DICER1 syndrome, a recently described tumor-predisposition syndrome, often involves multiple organs and is characterized by pleuropulmonary blastoma (PPB), cystic nephroma, ovarian Sertoli-Leydig tumors, familial multinodular goiter, etc. Germline DICER1 mutations have been identified in individuals with a variety of malignant conditions. However, in a review of the reported DICER1 syndrome cases that feature an unusual array of neoplastic and hyperplastic phenotypes, no mentions are made of these patients also presenting well-differentiated fetal adenocarcinoma of the lung. Here, we present a 16-year-old Chinese adolescent suffering from an ovarian Sertoli-Leydig cell tumor,well-differentiated fetal adenocarcinoma of the lung, and familial multinodular goiter with a nonsense mutation (c.3540C > A; p.Tyr1180*) in exon 21 of DICER1. This report presents the first case in which the clinical features of DICER1 syndrome appear in combination with well-differentiated fetal adenocarcinoma of the lung. We hypothesize that this case may suggest that well-differentiated fetal adenocarcinoma of the lung falls within the wide spectrum of manifestations of the DICER1 syndrome. Remarkably, this mutation is reported in a patient from The International PPB Registry.

Sertoli-Leydig cell tumors: current status of surgical management: literature review and proposal of treatment.

To identify the appropriate management we review the current literature on the diagnostic and different surgical procedures to which the patients affected by Sertoli-Leyding cell tumors (SLCTs) were submitted. Through the description of a case report we also propose an interdisciplinary diagnostic approach and a laparoscopic surgical staging, with a long-term follow-up. The analysis shows that pelvic ultrasound is primary diagnostic procedure, and only 36% of publications clearly describe to have performed more specific investigation. The hormone assessment is performed in the presence of specific endocrine symptoms. Laparoscopic approach is chosen by a few surgeon. Laparotomic surgery is preferred based in not recent recommendations for ovarian cancer treatment, although it is demonstrated the efficacy and safety of laparoscopy in the treatment of ovarian epithelial tumors. Different steps that are usually used for oncological ovarian cancer staging are not always performed. Conservative and fertility sparing surgery is commonly accepted, and even preferred due to the young age of patients. In the surgical treatment of SLCTs is necessary to adopt common guidelines, and evenly define the steps that the patient should be submitted. If are observed epithelial cancer oncological principles, laparoscopic surgery should be the approach of choice for these patients.

Incidental detection of Sertoli-Leydig cell tumor by FDG PET/CT imaging in a patient with androgen insensitivity syndrome.

A 29-year-old female patient who was being followed up for differentiated papillary thyroid carcinoma was referred to us for exploration of any possible metastasis since her serum thyroglobulin levels were high. The patient underwent an F-18 fluorodeoxyglucose positron emission tomography study, and a pathologically increased FDG uptake at the left lower abdomen was detected corresponding to a solid, cystic lesion on CT images. The patient had a history of primary amenorrhea and, together with the magnetic resonance imaging findings of absent uterus, short and blind end vagina, a diagnosis of androgen insensitivity syndrome was made. The patient underwent laparoscopic left pelvic mass resection, and the histopathology of the lesion revealed Sertoli-Leydig cell tumor.

[The use of ultrasonographic 3D power Doppler to describe an ovarian Sertoli-Leydig cell tumor]. / Description échographique d'une tumeur ovarienne de Sertoli-Leydig à l'aide du doppler énergie tridimensionnel.

We report one case of unilateral ovarian Sertoli-Leydig tumor with intermediate grade and heterologous element, in a young woman with clinical and biological typical presentation. We discuss 2D and 3D ultrasonographic and doppler features.

Scrotal ultrasonography: should it be used in routine evaluation of infertile men?

Several studies have suggested that male infertility and testicular cancer may have common aetiological factors. Scrotal ultrasonography (US) has an important role in the diagnosis of testicular tumours when not palpable by physical examination. In this study, we present two infertile men referred to our clinic. Patients were evaluated by a detailed physical examination, semen analyses and hormonal assessment. Both patients underwent scrotal US examination. Semen analysis of the patients revealed oligoasthenospermia in both patients. Scrotal US revealed hypoechoic masses in the left and right testes of both patients, which were nonpalpable by physical examination. Scrotal exploration and subsequent orchidectomy were performed. Histopathological examination revealed mixed germ cell tumour and Sertoli-Leydig cell tumour in case 1 and case 2 respectively. With these cases, we discussed the role of scrotal US in the routine diagnostic evaluation of infertile men.

Imaging of gynecological disease (2): clinical and ultrasound characteristics of Sertoli cell tumors, Sertoli-Leydig cell tumors and Leydig cell tumors.

OBJECTIVE: To describe the clinical history and ultrasound findings in women with ovarian Sertoli cell, Sertoli-Leydig cell and Leydig cell tumors. METHODS: Women with a histological diagnosis of Sertoli cell tumor, Sertoli-Leydig cell tumor or Leydig cell tumor who had undergone preoperative ultrasound examination were identified from the databases of each of three participating ultrasound centers. The tumors were characterized on the basis of ultrasound images, ultrasound reports and research protocols (when applicable) using the terms and definitions published by the International Ovarian Tumor Analysis (IOTA) group. In addition, all images were reviewed and described using pattern recognition. RESULTS: Of 22 patients identified, 15 had Sertoli-Leydig cell tumors, two had Sertoli cell tumors and five had Leydig cell tumors. Four patients were postmenopausal, one 48-year-old woman had undergone hysterectomy, 16 were of fertile age and one was a 4-year-old girl. Most patients (82%, 18/22) had endocrine symptoms, the most common being bleeding disturbance (64%, 14/22) and hirsutism (32%, 7/22). Twenty-two (96%) of 23 tumors (one woman had bilateral tumors) contained a solid component; 16 (70%) were purely solid. Pattern recognition showed that the Leydig cell tumors were small solid tumors (four of five had a largest diameter of 1-3 cm) and the two Sertoli cell tumors were somewhat larger solid tumors (4 cm and 7 cm); the Sertoli-Leydig cell tumors were either small (3-4 cm) or medium-sized (6-7 cm) solid tumors, or multilocular solid tumors of any size (3-18 cm) with purely solid areas mixed with areas of innumerable closely packed small cyst locules. CONCLUSIONS: On the basis of endocrine symptoms, the woman's age and ultrasound findings, it should be possible to suggest a correct preoperative diagnosis of Sertoli cell, Sertoli-Leydig cell or Leydig cell tumors in many cases.

[Ultrasound of the scrotum]. / Sonographie des Skrotums.

Ultrasound is an excellent tool for the diagnosis of benign or malignant intra- and extratesticular lesions depending on their structure and location in the scrotum. Besides the sonographic appearance, the clinical history and the location of a scrotal mass is crucial for the differential diagnosis between benign and malignant masses. In cases of acute scrotum or of vascular and inflammatory lesions, Doppler ultrasound is mandatory. This article gives a systematical survey of the most frequent scrotal lesions and their appearance in ultrasound.

[Androblastoma in a female patient with the signs of virility: a clinical-morphological and genetic study]. / Androblastoma u pacjentki z objawami wirylizacji--badanie kliniczo-morfologiczne i genetyczne.

We report a case of 24 year old patient with secondary amenorrhea, virilization and elevated serum testosterone concentration, in which cystic-solid tumor in right ovary was found. Surgical treatment was applied--adnexectomy. Histopathologic examination supported suspicion of androblastoma. After treatment regulation of menses and serum testosterone level was obtained.