- Pediatric primary extra-ovarian Sertoli Leydig cell tumor of the retroperitoneum. [Journal Article]Indian J Pathol Microbiol. 2023 Jan-Mar; 66(1):145-147.IJ
- Ovarian Sertoli Leydig cell tumors (SLCT) accounts for less than 0.5% of all ovarian malignancies. The incidence of primary extra-ovarian SLCT is extremely rare with reported cases occurring in young adult women till now. We report case of primary retroperitoneal extra-ovarian SLCT in a seven-year girl child without any hormonal manifestation. She presented with complaint of left side abdominal s…
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- Ultrasound examination assisted clinical diagnosis of Leydig cell tumor of ovary: An extremely rare case report. [Case Reports]
- CONCLUSIONS: This case suggests that when women of childbearing age have abruptly decreased menstrual flow with increased testosterone, clinicians should pay attention to intra-ovarian occupying lesions and consider the possibility of LCT. In such cases, ultrasound examination can determine the presence, location, shape and size of occupying ovarian lesions and play an important role in the diagnosis of condition.
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- Well-differentiated Sertoli-Leydig Cell Tumors (SLCTs) Are Not Associated With DICER1 Pathogenic Variants and Represent a Different Tumor Type to Moderately and Poorly Differentiated SLCTs. [Journal Article]Am J Surg Pathol. 2022 Dec 30 [Online ahead of print]AJ
- Sertoli-Leydig cell tumors (SLCTs) are uncommon ovarian sex cord-stromal neoplasms which are currently classified into well, moderately, and poorly differentiated and retiform types. Well-differentiated SLCT is the least common and typically occurs in pure form, whereas moderately and poorly differentiated and retiform types often comprise a morphologic spectrum with an admixture of all 3. DICER1…
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- A 14-Year-Old Saudi Boy with Gynecomastia, Cushing Syndrome, Large-Cell Calcifying Sertoli Cell Tumor of the Testis, and Carney Complex. [Case Reports]
- BACKGROUND Carney complex (CNC) is a rare multiple neoplasia syndrome with autosomal dominant inheritance. CNC is frequently misdiagnosed owing to its diverse clinical characteristics. We reported the case of a 14-year-old Saudi boy with a history of gynecomastia, Cushing syndrome, large-cell calcifying Sertoli cell tumor of the testis, and CNC. CASE REPORT The patient was referred to the pediatr…
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- The value of contrast-enhanced ultrasonography in the diagnosis of primary testicular non-neoplastic and neoplastic lesions in adults. [Journal Article]
- CONCLUSIONS: CEUS could accurately distinguish between benign and malignant testicular tumors, as well as differentiate specific pathological types (testicular focal infarction, epidermoid cysts, spermatocytic tumors, SLTC and lymphoma). Accurate preoperative diagnosis is critical for guiding the selection of appropriate treatment plans for different pathological types of testicular tumors.
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- Key changes in WHO classification 2022 of testicular tumors. [Journal Article]Cesk Patol. 2022 Fall; 67(4):198-204.CP
- Compared to the WHO classification of the male genital tumors in 2016, minimal changes were introduced in the current WHO 2022. Classification of germ cell tumors remains the same as in the previous edition, dividing germ cell tumors into those derived from germ cell neoplasia in situ (GCNIS) and those independent of GCNIS. The group of GCNIS derived germ cell tumors is essentially unchanged. Mos…
- Ovarian Sertoli Cell Tumor with Immature Prepubertal-like Sertoli Cell Component: A Case Report and Literature Review. [Case Reports]
- The Sertoli cell tumor of the ovary is a rare ovarian tumor with non-specific symptoms. According to the literature, endocrine manifestations occur in two-thirds of patients, but testosterone production is extremely rare. Typically, it is a unilateral benign tumor of the ovary that most commonly presents in adolescents and young women of childbearing potential. We report a 29-year-old patient, pr…
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- DICER1 Mutation in Recurrent Ovarian Sertoli-Leydig Cell Tumor: A Case Report. [Case Reports]J Pediatr Adolesc Gynecol. 2022 Nov 17 [Online ahead of print]JP
- CONCLUSIONS: This report adds to the small body of evidence about this rare but unexpectedly highly aggressive tumor, especially in the recurrent setting, and reminds the reader of the importance of cancer diagnosis in this population.
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- Laparoscopic resection of ectopic Sertoli cell tumor with torsion in an adolescent girl. [Journal Article]Pediatr Int. 2023 01; 65(1):e15414.PI
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- Severe intrahepatic cholestasis of pregnancy due to a Sertoli-Leydig cell tumour in a woman with polycystic ovary syndrome: a case report. [Case Reports]
- CONCLUSIONS: This unusual case highlights the concurrence of original hyperandrogenism and subsequent hyperestrogenism during pregnancy and the resultant confounding manifestations. Obstetricians should be aware of the potential association between androgen excess and ICP via placental aromatization.
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- cAMP-specific phosphodiesterase 8A and 8B isoforms are differentially expressed in human testis and Leydig cell tumor. [Journal Article]
- Cyclic adenosine monophosphate/Protein kinase A (cAMP/PKA) signaling pathway is the master regulator of endocrine tissue function. The level, compartmentalization and amplitude of cAMP response are finely regulated by phosphodiesterases (PDEs). PDE8 is responsible of cAMP hydrolysis and its expression has been characterized in all steroidogenic cell types in rodents including adrenal and Leydig c…
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- 11-Oxygenated androgens are not secreted by the human ovary: in-vivo data from four different cases of hyperandrogenism. [Journal Article]
- CONCLUSIONS: This study confirms that 11-OHA4 and 11-KT are not biosynthesized by the ovary. We propose that the testosterone/11-KT ratio as well as 11-OHA4 could help identify predominant adrenal androgen excess and distinguish neoplastic and non-neoplastic ovarian androgen source.
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- Efficacy of aromatase inhibitor therapy in a case with large cell calcifying Sertoli cell tumour-associated prepubertal gynaecomastia. [Case Reports]J Pediatr Endocrinol Metab. 2022 Dec 16; 35(12):1565-1570.JP
- CONCLUSIONS: Testicular tumour should be investigated in a patient with PJS who presents with prepubertal gynaecomastia. When findings are consistent with LCCSCT, aromatase inhibitors may be preferred in the treatment.
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- Bilateral Ovarian Sertoli-Leydig Cell Tumors Harboring DICER1 Germline and Distinct Somatic Mutations: Case Report and Literature Review. [Journal Article]Fetal Pediatr Pathol. 2022 Sep 19 [Online ahead of print]FP
- Background: DICER1 tumor predisposition syndrome is characterized by an increased risk for development of pleuropulmonary blastoma, pituitary blastoma, multinodular thyroid goiter, thyroid carcinoma, sex cord stromal tumor, cystic nephroma, embryonal rhabdomyosarcoma, and tumors of the CNS, amongst others. Of this list, only pituitary blastoma is recognized as pathognomonic for the syndrome. Case…
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- Chlorinated organophosphorus flame retardants-induced mitochondrial abnormalities and the correlation with progesterone production in mLTC-1 cells. [Journal Article]Food Chem Toxicol. 2022 Nov; 169:113432.FC
- Environmental monitoring data have indicated that three chlorinated organophosphorus flame retardants (Cl-OPFRs), including tris(2-chloroethyl)-phosphate (TCEP), tris(2-chloropropyl)-phosphate (TCPP), and tris(1,3-dichloro-2-propyl)-phosphate (TDCPP) are the predominant chemicals in various environmental matrices and exhibit reproductive endocrine disrupting activities. Currently, mitochondrial a…
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- Is testis sparing surgery safe in patients with incidental small testicular lesions referring to a fertility center? A retrospective analysis reporting factors correlated to malignancy and long-term oncological outcomes. [Journal Article]
- CONCLUSIONS: Ultrasonographic characteristics and intraoperative appearance of the lesion can predict the malignant nature of small testicular lesions, guiding their surgical management in patients referring to a fertility center. Based on our experience, clinicians may safely perform TSS in carefully selected patients.
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- Histidine decarboxylase inhibitors: a novel therapeutic option for the treatment of leydigioma. [Journal Article]J Endocrinol. 2022 12 01; 255(3):103-116.JE
- Recent reports indicate an increase in Leydig cell tumor (LCT) incidence. Radical orchiectomy is the standard therapy in children and adults, although it entails physical and psychosocial side effects. Testis-sparing surgery can be a consideration for benign LCT of 2.5 cm or less in size. Malignant LCTs respond poorly to conventional chemotherapy, so new treatment modalities are needed. In this s…
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- Ovarian Sertoli-Leydig cell tumor: A case report. [Letter]Asian J Surg. 2022 Sep 03 [Online ahead of print]AJ
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- DICER1-Altered Extraovarian Moderately Differentiated Sertoli-Leydig Cell Tumor: Report of a Rare Case. [Journal Article]Int J Gynecol Pathol. 2022 Aug 03 [Online ahead of print]IJ
- We report an unusual case of a pelvic extraovarian moderately differentiated Sertoli-Leydig cell tumor arising in a 4-yr-old female. The tumor contained a DICER1 pathogenic variant which was absent in the germline ruling out DICER1 syndrome. In reporting this case, we discuss the differential diagnosis and possible histogenesis and review reported cases of extraovarian Sertoli-Leydig cell tumor.
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- Sertoli-Leydig cell ovarian tumour: a rare cause of virilisation and androgenic alopecia. [Case Reports]BMJ Case Rep. 2022 Aug 25; 15(8)BC
- Sertoli-Leydig cell tumours (SLCTs) represent a rare cause of hyperandrogenic state. SLCTs are sex cord ovarian neoplasms, accounting for <0.2% of all ovarian tumours. Most of the sex cord-stromal tumours have a benign clinical course, with 10%-20% of them at risk of aggressive course. We report a case of a woman in her 30s who presented with androgenic alopecia, virilisation and secondary amenor…
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- Embryonal Rhabdomyosarcoma of the Uterine Cervix: A Clinicopathologic Study of 94 Cases Emphasizing Issues in Differential Diagnosis Staging, and Prognostic Factors. [Journal Article]Am J Surg Pathol. 2022 11 01; 46(11):1477-1489.AJ
- Embryonal rhabdomyosarcoma of the uterine cervix (cERMS) is rare and frequently associated with DICER1 mutations. We report 94 tumors that arose in patients aged 7 to 59 (median=23) years and presented with vaginal bleeding (52), protruding vaginal mass (17), cervical polyp (8), or expelled tumor fragments per vagina (5). Nine had DICER1 syndrome, 8 of whom had other syndromic manifestations incl…
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- Complete androgen insensitivity syndrome - rare case of malignancy of dysgenetic gonads. [Case Reports]Ceska Gynekol. 2022; 87(3):184-187.CG
- CONCLUSIONS: Complete androgen insensitivity syndrome is a rare genetic disease characterized by varying degrees of feminization in individuals with a male karyotype. It should not be neglected, especially in the differential diagnostic work-up of primary amenorrhea. Genetic testing of the karyotype should be performed whenever uterine agenesis is suspected.
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- Multiparametric ultrasound for the diagnosis of Leydig cell tumours in non-palpable testicular lesions. [Journal Article]Andrology. 2022 10; 10(7):1387-1397.A
- The widespread use of ultrasonography has led to an increased number of incidentally detected small non-palpable lesions, with Leydig cell tumours representing the majority of them.
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- Hyperandrogenism due to ovarian Leydig cell tumour presenting with polycythaemia. [Case Reports]BMJ Case Rep. 2022 Jul 15; 15(7)BC
- 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 mar…
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- Collision Tumor of the Ovary Involving Sertoli-Leydig Cell Tumor and High-Grade Serous Carcinoma-Report of the First Case. [Journal Article]
- We report a collision tumor in the ovary of a 60-yr-old woman composed of high-grade serous carcinoma and Sertoli-Leydig cell tumor. Collision tumors in the ovary are rare and to the best of our knowledge, combination of ovarian high-grade serous carcinoma and Sertoli-Leydig cell tumor has not been described before.
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- Pleuropulmonary blastoma (PPB) and other DICER1-associated high-grade malignancies are morphologically, genetically and epigenetically related - A comparative study of 4 PPBs and 6 sarcomas. [Journal Article]Ann Diagn Pathol. 2022 Oct; 60:152002.AD
- DICER1-related tumors occur hereditary or sporadically, with high-grade malignancies sharing clinicopathological and (epi)genetic features. We compared 4 pleuropulmonary blastomas (PPBs) and 6 sarcomas by mutation analysis, whole transcriptome sequencing and methylation profiling. 9/10 patients were female. PPB patients were 0-4 years. 3/4 were alive; 2 without disease. One patient died of metast…
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- DICER1-associated Tumors in the Female Genital Tract: Molecular Basis, Clinicopathologic Features, and Differential Diagnosis. [Review]Adv Anat Pathol. 2022 Sep 01; 29(5):297-308.AA
- DICER1 syndrome is a tumor predisposition syndrome in which patients are at an increased risk of developing a wide variety of benign and malignant neoplasms with a hallmark constellation of pediatric pleuropulmonary blastoma, cystic nephroma, and thyroid lesions. DICER1 encodes an RNA endoribonuclease that is crucial to the processing of microRNA and may play a role in the maturation of Müllerian…
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- Sex cord stromal tumors and tumors of the paratestis: new and old entities in a landscape of rare tumors. [Review]Curr Opin Urol. 2022 07 01; 32(4):339-343.CO
- The 5th edition of WHO classification incorporates the most relevant new data available in the literature regarding tumors of the male genitourinary tract. In this review, the authors summarize and critically discuss the most relevant new information regarding tumors occurring in the stromal testis and in the paratestis that will be reported in the new edition of WHO classification of tumors of t…
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- Spontaneous neoplasms in harbour porpoises Phocoena phocoena. [Journal Article]Dis Aquat Organ. 2022 Jun 23; 149:145-154.DA
- Harbour porpoises are widely distributed in the North Atlantic and represent the most abundant cetacean species in the North and Baltic Seas. Spontaneous neoplasms are relatively rarely reported in cetaceans, and only little is known about neoplasia in harbour porpoises. Thus, archival material was reviewed for spontaneous neoplasms in harbour porpoises recorded during post-mortem examinations be…
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- Update on Ovarian Sex Cord-Stromal Tumors. [Review]Surg Pathol Clin. 2022 Jun; 15(2):235-258.SP
- This article focuses on the recent advances in ovarian sex cord-stromal tumors, predominantly in the setting of their molecular underpinnings. The integration of genetic information with morphologic and immunohistochemical findings in this rare subset of tumors is of clinical significance from refining the diagnostic and prognostic stratifications to genetic counseling.
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