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Leydig Cell Tumor

Interstitial Cell Tumor

Leydig cell tumor is a rare neoplasm arising from the Leydig cells of the testicular interstitium that produce testosterone. It can develop in children as a benign lesion, or in adults when 10% of cases undergo malignant transformation, in which case prompt recognition is necessary. A testicular mass is almost always present, and further imaging and histopathological findings are used to identify the exact tumor type.


Presentation

Leydig cell tumors belong to the group of sex cord-stromal tumors of the testes and constitute only 1-3% of all testicular neoplasms, making them a rare occurrence in clinical practice [1] [2] [3]. Leydig cells are located in the interstitium, and are responsible for the production of testosterone, but also estrogen to a lesser extent, which is why signs and symptoms are often related to the excess effects of androgen hormones caused by abnormal cellular proliferation [1] [3] [4]. The tumor is diagnosed in two age groups - in prepubertal boys, and more commonly, in adults between 30-60 years of age [1] [3] [5] [6]. In children, excess testosterone results in the development of precocious puberty - the early growth of pubic hair, the penis and the musculoskeletal system [1] [4]. On the other hand, increased estrogen production will induce feminization (gynecomastia and tenderness of the breasts, as well as feminine hair distribution) [1]. This clinical presentation is also seen in adults, but they are accompanied by loss of libido, impotence, erectile dysfunction, infertility, and most importantly, an enlarged testicle [1] [4]. Studies have confirmed the rare appearance of a bilateral testicular mass, but in the vast majority of patients, a unilateral and sometimes painful intratesticular mass is typical for Leydig cell tumors [1] [2] [4]. It is exclusively benign in infants, but the potential for malignant transformation in adults, seen in 10% of cases, implies that early recognition of the tumor is vital, as dissemination through the retroperitoneal lymph nodes into the liver, the lungs, and the skeletal system has been observed [1] [5] [6]. Thus, signs and symptoms that indicate a disbalance in androgen hormones should alert the physician to conduct a meticulous workup.

Precocious Puberty
  • We report two boys who developed true precocious puberty after surgical treatment of a Leydig cell tumor of the testis that presented peripheral precocious puberty.[frontiersin.org]
  • In children, excess testosterone results in the development of precocious puberty - the early growth of pubic hair, the penis and the musculoskeletal system.[symptoma.com]
  • Clinical presentation is testicular mass, pain and hormone alteration such as precocious puberty and gynecomastia. We present one case of LCT and his conservative management after an incidental finding. Literature is reviewed.[ncbi.nlm.nih.gov]
Recurrent Pulmonary Embolism
  • She had a history of recurrent pulmonary embolism and unexplained secondary erythrocytosis. Past hematological investigations had not revealed any evidence of malignancy or thrombophilia, and the JAK2 mutation was negative.[ncbi.nlm.nih.gov]
Inguinal Mass
  • A 53-year-old male presented with a right inguinal mass of one-year duration. The fine needle aspiration of the inguinal mass showed a highly cellular tumor composed of sheets and isolated, large round to polygonal cells with moderate pleomorphism.[ncbi.nlm.nih.gov]
Aspiration
  • We present a case of LCT with severe atypia diagnosed by fine-needle aspiration (FNA) in a 49-year-old man who presented with gynecomastia and right testis enlargement.[ncbi.nlm.nih.gov]
  • Google Scholar Handa U, Sood T, Punia RS: Testicular Leydig cell tumor diagnosed on fine needle aspiration. Diagn Cytopathol. 2010, 38: 682-684. 10.1002/dc.21306.[bmcresnotes.biomedcentral.com]
Cough
  • We report a 34-year-old man visited hospital because of coughing sputum mixed with blood. His chest CT showed bilateral patch clouding opacity. He was suspected with allergic alveolitis and treated with methylprednisolone.[ncbi.nlm.nih.gov]
  • The case of malignant LCT was seen in another 47 year old male who presented with cough. On examination, a testicular swelling was found, and after orchidectomy, he was diagnosed to have malignant LCT on histopathologic examination.[ijpmonline.org]
  • […] accounts for less than 0.2% of all testicular cancers as evidenced by metastatic spread and poor survival. [3] We report an interesting case of malignant LCT with mediastinal and lung metastasis in a 38-year-old man who presented with a complaint of dry cough[sjkdt.org]
Sputum
  • We report a 34-year-old man visited hospital because of coughing sputum mixed with blood. His chest CT showed bilateral patch clouding opacity. He was suspected with allergic alveolitis and treated with methylprednisolone.[ncbi.nlm.nih.gov]
Increased Hair Growth
  • A 60-year-old woman, presented with increased hair growth and androgenic alopecia and the hormonal laboratory examination showed that she had elevated serum testosterone level and normal dehydroepiandrosterone sulfate (DHEAS), androstenedione, 17- hydroxyprogesterone[ncbi.nlm.nih.gov]
Increased Libido
  • We report a case of a 58-year-old woman who complained of progressive hirsutism, nervousness, irritability, anxiousness and an increased libido. Examination showed an unusual redness of her head, décolleté, palms and soles of her feet.[ncbi.nlm.nih.gov]
  • The majority of the cases is diagnosed in the presence of a hyperandrogenic state with signs of virilization (hirsutism, acne, deep voice, mammary atrophy, increased libido, clitoromegalia) ( 2 - 5 ).[scielo.br]
Anxiety Disorder
  • The patient showed significant regression of clinical signs and symptoms, including the anxiety disorder.[ncbi.nlm.nih.gov]
Irritability
  • Both men suffered from severe restlessness, insomnia and irritability, which they described as intolerable and disrupting normal life activities.[ncbi.nlm.nih.gov]
Insomnia
  • Both men suffered from severe restlessness, insomnia and irritability, which they described as intolerable and disrupting normal life activities.[ncbi.nlm.nih.gov]
Testicular Mass
  • Hereby, we present the case of two brothers affected by 11-β hydroxylase deficiency and presenting with bilateral testicular masses.[ncbi.nlm.nih.gov]
  • A testicular mass is almost always present, and further imaging and histopathological findings are used to identify the exact tumor type.[symptoma.com]
Painless Testicular Mass
  • Each patient had a well circumscribed, painless testicular mass, increased serum testosterone (101 and 444 ng/dl [normal 0 to 25]), normal gonadotropins and negative alpha-fetoprotein levels.[ncbi.nlm.nih.gov]
  • Presents a painless testicular mass. Tumor cells may produce androgen, estrogens or both and presents with gynecomastia or precocious puberty; symptoms usually regress with excision.[auanet.org]
Testicular Pain
  • In the other patients diagnosis was made by ultrasound performed for testicular pain (4 patients, 16.6%) or the appearance of gynecomastia (3 patients, 12.5%). Frozen section examination was done in 20 of 24 patients (83%).[ncbi.nlm.nih.gov]
  • Indications for Leydig Cell Tumor of the Testis include: Loss of libido Overdevelopment of breasts in males (gynecomastia) Breast pain, especially if accompanied by enlargement Trouble breathing Headache Low blood pressure Anemia Testicular pain, lump[dovemed.com]
Testicular Disease
  • Schweyer , Tumoren des Gonadenstromas, Der Pathologe , 2014 , 35 , 3, 245 CrossRef 11 Giorgio Bozzini , Stefano Picozzi , Dario Ratti , Stefano Casellato , Luca Carmignani , Long-term follow up with sparing surgery for testicular diseases: A safe choice[onlinelibrary.wiley.com]
Scrotal Mass
  • The malignant forms are generally discovered in elder men presenting a scrotal mass with no hormonal changes [1-7]. We describe a small and rare benign Leydig cell tumor found incidentally in an old man.[smirg.org]

Workup

The initial diagnosis of a testicular tumor can be rather easily made with a proper physical examination that will palpate a mass and a detailed patient history that will assess the course and progression of symptoms [6]. However, a mass may not always be present, especially in the beginning of tumorigenesis. For this reason, a detailed laboratory workup is necessary. In all patients in whom signs of excess androgen production are present, serum values of luteinizing hormone (LH), follicle-stimulating hormone (FSH), estrogen, estradiol, progesterone, adrenocorticotropin hormone (ACTH) and cortisol should be evaluated [1] [6]. Testosterone levels will almost always be raised in the setting of precocious puberty, whereas increased estrogen and estradiol are seen in patients who suffer from feminization and gynecomastia [1] [6]. It should be noted that tumor markers for testicular tumors, including alpha-fetoprotein (AFP), beta-human chorionic gonadotropin (β- hCG), lactate dehydrogenase (LDH) and placental alkaline phosphatase (PALP) are negative, as they are only useful for germ cell testicular tumors [1] [3] [4] [6]. In addition to clinical and laboratory studies, imaging procedures, such as ultrasonography (US) of the testes, is the next step in confirming the diagnosis [2] [3] [6]. A homogeneous hypoechoic appearance on US is the hallmark of a benign Leydig cell tumor, but if the lesion is heterogeneous, malignant transformation should be suspected, in which case computed tomography (CT) and magnetic resonance imaging (MRI) of the pelvis and abdomen are recommended to exclude metastatic spread [1] [2] [3] [4] [6]. A definite diagnosis is made after a biopsy and subsequent histopathological examination with immunohistochemistry [1]. α-Inhibin, but also calretinin and Melan-A are reliable markers for differentiation between sex cord stromal tumors and germ cell tumors of the testes [1].

Treatment

  • We report on the--to our knowledge--first treatment experiment with imatinib in a patient with metastatic Leydig cell tumor. Unfortunately, the tumor progressed during treatment.[ncbi.nlm.nih.gov]
  • Testis-sparing surgery was the first-line treatment ( 9 , 13 , 15 , 17 , 18 , 20 – 22 ).[frontiersin.org]

Prognosis

  • Abstract Malignant Testicular Leydig Cell tumors (leydigomas) are extremely rare to occur and mostly carry a bad prognosis.[ncbi.nlm.nih.gov]
  • Typically, in a vast majority of cases, the prognosis is excellent, since it is usually a benign tumor In rare case of a malignancy, the prognosis may depend upon several factors including the stage of the tumor and health status of the individual Generally[dovemed.com]
  • Outlook (Prognosis) Prognosis of SLCT depends on the stage and degree of differentiation of the tumor. Since these tumors are typically benign and their growth is slow, outlook is generally very positive.[knowcancer.com]

Etiology

  • It is recommended that all endocrine, nutritional, neoplastic, chromosomal, and drug etiologies of gynecomastia be investigated before surgical resection of the enlarged breasts.[popline.org]
  • […] are malignant Sites Origin from testis is demonstrable in most cases Rarely originates from epididymis ( Andrologia 2013;45:430 ) Pathophysiology Produce testosterone, dehydroepiandrosterone and androstenedione, which cause symptoms described below Etiology[pathologyoutlines.com]
  • (Etiology) The cause of Leydig Cell Tumor of the Testis is unknown.[dovemed.com]
  • Malignant transformation is rare, and given the slow growth, prognosis is generally good. 2 The etiology of LCTs remains unknown.[pediatrics.aappublications.org]

Epidemiology

  • Epidemiology 1–3% of all testicular tumors. Most common in boys (5–10 years old) and in adults aged 30–60 years. Pathology of the Leydig cell tumor Small yellow-brown tumors, sharply circumscribed in the testicular tissue.[urology-textbook.com]
  • Leydig Cell Tumors Epidemiology & Pathology Leydig cell tumors are the most common non-germ cell tumors of the testis and account for 1-3% of all testicular tumors.[health.am]
  • Definition / general Most common sex cord stromal tumor; recapitulates normal development and evolution of Leydig cells Epidemiology 1 - 3% of testicular tumors No predilection based on ethnicity or race 10% are malignant Sites Origin from testis is demonstrable[pathologyoutlines.com]
  • Epidemiology Leydig cell tumours constitute 1-3% of all testicular tumours. Clinical features In children, they may present with isosexual pseudoprecocity. In adults, they present with a mass and/or gynaecomastia.[e-immunohistochemistry.info]
  • Summary Epidemiology Age related incidence rate was found to be 1 per 500,000 women. Sex cord stromal tumors constitute about 5% of ovarian malignancies.[orpha.net]
Sex distribution
Age distribution

Pathophysiology

  • Signs and Symptoms (Pathophysiology) Symptoms can include infrequent or no menstrual periods, the development of facial hair, a hoarse voice, increased muscle mass, acne, and clitoromegaly.[knowcancer.com]
  • […] development and evolution of Leydig cells Epidemiology 1 - 3% of testicular tumors No predilection based on ethnicity or race 10% are malignant Sites Origin from testis is demonstrable in most cases Rarely originates from epididymis ( Andrologia 2013;45:430 ) Pathophysiology[pathologyoutlines.com]
  • Background Pathophysiology A German anatomist, Franz Leydig, first described Leydig cells in 1870. [9] Leydig cells are located within the interstitium of the testis, between the seminiferous tubules, and produce testosterone in response to luteinizing[emedicine.medscape.com]
  • PMID 18382911. a b "Leydig Cell Tumors: Practice Essentials, Background, Pathophysiology". 2016-10-27. Biegel, L. B.; Liu, R. C.; Hurtt, M. E.; Cook, J. C. (1995).[en.wikipedia.org]

Prevention

  • In spite of extensive explanation and psychological counseling, cultural barriers prevented appropriate treatment. An ovarian Leydig cell tumor should always be considered for a woman in the reproductive age with symptoms of virilization.[ncbi.nlm.nih.gov]
  • […] the adrenogenital syndrome (which may contain fat) and may prevent misdiagnosis of a LCT with spindle cells as a sarcoma or unclassified sex cord-stromal tumor.[journals.lww.com]
  • Removal of the testis Debulking surgery to reduce tumor size, followed by chemotherapy Clotting the vessels in the tumor (embolization) Chemotherapy Radiotherapy Undertaking treatment of underlying conditions How can Leydig Cell Tumor of the Testis be Prevented[dovemed.com]

References

Article

  1. Al-Agha OM, Axiotis CA. An in-depth look at Leydig cell tumor of the testis. Arch Pathol Lab Med. 2007;131(2):311-317.
  2. Gheorghisan-Galateanu AA. Leydig cell tumors of the testis: a case report. BMC Res Notes. 2014;7:656.
  3. Suardi N, Strada E, Colombo R, et al. Leydig cell tumor of the testis: presentation, therapy, long-term follow-up and the role of organ-sparing surgery in a single-institution experience. BJU Int. 2009;103(2):197-200.
  4. Albers P, Albrecht W, Algaba F, et al. Guidelines on Testicular Cancer: 2015 Update. Eur Urol. 2015;68(6):1054-1068.
  5. Thambi R, Pothen L, Emmanuel KM, Vijayalakhmi A. Leydig cell tumor of testis with indeterminate features. Indian J Cancer. 2015;52(4):529-530.
  6. Sönmez N, Ton Ö, Arısan S, Kılınç F, Eken K, Güney S. Bilateral Leydig cell tumor of the testis: a case report. Contemp Oncol (Pozn). 2012;16(4):356-359.

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Last updated: 2019-06-28 11:45