Presentation
This feature was in contrast to the previously described two cases, where mucinous adenocarcinoma as heterologous element was present as microscopic foci. [ncbi.nlm.nih.gov]
Figure 2B : Nests of Leydig cells present in an edematous background (H & E stain, 100x magnification ). [ispub.com]
Our case represented a diagnostic challenge, giving the unusual clinical presentation (lack of hormonal symptoms) and the late age of presentation. The gross appearance and unilaterality favored the diagnose of SLCT. [path.upmc.edu]
We here present a case report ofSertoli-Leydig Cell Tumor having the rarity of bilateral presentation and male karyotype in a 23-year-old phenotypically female patient. [medresearch.in]
Entire Body System
- Virilization
Androblastomas occur in the TESTIS or the OVARY causing precocious masculinization in the males, and defeminization, or virilization (VIRILISM) in the females. In some cases, the Sertoli cells produce ESTROGENS. [connects.catalyst.harvard.edu]
A 24-year-old woman was admitted to hospital because of hirsutism, virilism and amenorrhea, which had appeared 6 months earlier. [ncbi.nlm.nih.gov]
- Precocious Puberty
Twenty weeks following surgical removal, the patient presented again with central precocious puberty; inhibin B levels were raised on this occasion, a luteinizing releasing hormone stimulation test confirmed central precocious puberty. [ncbi.nlm.nih.gov]
Primary testicular abnormalities causing precocious puberty Leydig cell tumor, Leydig cell hyperplasia, and adrenal rest tumor. Ann Clin Lab Sci 1983 ;13: 315 - 320 23. Holland FJ. Gonadotropin-independent precocious puberty. [nejm.org]
Página 84 - Somatomedin-C in normal puberty and in true precocious puberty before and after treatment with a potent luteinizing hormone-releasing hormone agonist. Página 368 - HJ. [books.google.es]
Leydig and Sertoli cells are functionally active and patients with these tumors will have an excess of either testosterone or estrogen, which can result in precocious puberty. [lecturio.com]
Complications of Ovarian Sertoli-Leydig Cell Tumor may include: Precocious puberty (unusually early onset of puberty) will result in short stature (height) and early menarche (onset of menstruation) Deep-seated tumors may create problems for adjoining [dovemed.com]
- Fatigue
They had no voice and weight changes, fatigue or galactorrhea. Their modified Ferriman–Gallwey score were 19 and 36. Laboratory studies revealed normal complete blood counts, liver and kidney tests. [endocrine-abstracts.org]
[…] increased facial hair (hirsutism), which may be progressive in nature Irregularities of menstrual cycles or complete cessation of cycle Acne Enlarged clitoris Abdominal mass or pain Increased muscle tone Hairline recession Loss of appetite with weight loss Fatigue [dovemed.com]
- Fever
1841 - 1912) Norwegian physician Hartmann ハルトマン Hartmann's operation ハルトマン手術 Hartnup ハートナップ Hartnup disease ハートナップ病 Hashimoto 橋本 Hashimoto's disease 橋本病 橋本 策(はかる)(1881 - 1934) 九州大学の外科医 Hassall ハッサル Hassall's corpuscle ハッサル小体 Haverhill ヘーバヒル Haverhill fever [jams.med.or.jp]
Author:岡 賢二; 樋口 正太郎; 岸田 大; 矢崎 正英; 中村 昭則; 内川 順子; 山田 靖; 小原 久典; 鹿島 大靖; 宮本 強; 塩沢 丹里; Keywords:Familial Mediterranean fever; menstruation; endometriosis; dienogest; P3-57-1 悪性疾患の治療に伴う精子凍結保存 : 運動精子凍結に影響する因子(Group 122 癌と生殖,一般演題,公益社団法人日本産科婦人科学会第68回学術講演会) 日本産科婦人科學會雜誌 [soar-rd.shinshu-u.ac.jp]
Gastrointestinal
- Abdominal Mass
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. [ncbi.nlm.nih.gov]
Hormonally inactive tumors often have a nonspecific presentation such as an abdominal mass and/or pain. The majority of patients present with stage I disease. Most tumors are unilateral (>90% of cases) and confined to the ovaries. [radiopaedia.org]
Case report A 13-year-old female presented to the Shandong Provincial Hospital affiliated to Shandong University (Jinan, Shandong, China) with a 7-day history of abdominal distension, a 3-day history of abdominal pain and a pelvic mass. [spandidos-publications.com]
- Abdominal Distension
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. [ncbi.nlm.nih.gov]
Case report A 13-year-old female presented to the Shandong Provincial Hospital affiliated to Shandong University (Jinan, Shandong, China) with a 7-day history of abdominal distension, a 3-day history of abdominal pain and a pelvic mass. [spandidos-publications.com]
Skin
- Hirsutism
Prepregnancy findings included hirsutism, clitoromegaly, secondary amenorrhea, and elevated peripheral plasma testosterone (T; 5.7 ng/ml). [ncbi.nlm.nih.gov]
Treatment Rapid onset Hirsutism, virilization, adnexal mass Elevated testosterone Surgery Use of spironolactone disorders with excess androgen? Will help with? (But?) [brainscape.com]
Neurologic
- Confusion
Pan-keratins and AE1/AE3 may mark granulosa cell tumors and Sertoli cell tumors causing confusion with adenocarcinoma. EMA is a better marker to exclude an epithelial tumor as EMA is negative in sex cord-stromal tumors. [librepathology.org]
Occasional tumors have microcysts ( Figure 22 ) that we have seen cause confusion with yolk sac tumor. [nature.com]
Moreover, the acini of a carcinoid tumor may be confused with well-differentiated Sertoli tubules. [humpath.com]
Histopathology 2001 May;38(5):403-8 Abstract quote AIMS: Ovarian sex cord-stromal tumours are a heterogeneous group of neoplasms which may be confused morphologically with a wide variety of tumours. [thedoctorsdoctor.com]
Conclusion Poorly differentiated SLCT can be confused with many other tumors of the ovary. The diagnosis is especially important from therapeutic point of view. References 1. Young RH. [ccij-online.org]
Urogenital
- Clitoromegaly
Prepregnancy findings included hirsutism, clitoromegaly, secondary amenorrhea, and elevated peripheral plasma testosterone (T; 5.7 ng/ml). [ncbi.nlm.nih.gov]
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]
- Metrorrhagia
Around 50% of cases come to clinical attention because of progressive defeminization, as was seen in this patient who had a mild hirsutism(1).Other symptoms can be observed like menorhagia, metrorrhagia,amenorrhea ,abdominal pain. [webmedcentral.com]
Workup
Our goals are to review the workup of androgen-secreting tumors and discuss the clinical importance of the DICER1 mutation in the context of SLCT. [ncbi.nlm.nih.gov]
Laboratory tests showed elevated serum testosterone 27.6 ng/mL (normal range: 0.2-1.2 ng/mL) and dehydroepiandrosterone of 16.3 ng/mL (normal range: 0.8-3.2 ng/mL), and normal CA-125 of 22 U/mL (normal range The patient was referred to imaging workup [sjmms.net]
Treatment
The utility of serum alpha-fetoprotein in disease response to treatment and for surveillance of recurrence is also shown. [ncbi.nlm.nih.gov]
[…] to advances in diagnosis and treatment. [rarediseases.info.nih.gov]
Treatment Rapid onset Hirsutism, virilization, adnexal mass Elevated testosterone Surgery Use of spironolactone disorders with excess androgen? Will help with? (But?) [brainscape.com]
Prognosis
The prognosis of SLCTs is good, although poorly differentiated tumors may recur. [path.upmc.edu]
It seems that when rhabdomyosarcoma is a significant or predominant component of SLT, as occurred in this case, the prognosis is poor and is that of rhabdomyosarcoma in general. [ncbi.nlm.nih.gov]
Some authors opine that although overall SertoliLeydig cell tumors have a relatively good prognosis, intermediate tumors with retiform pattern and those containing heterologous elements have poorer prognosis. [ispub.com]
Etiology
When such multiple conditions involving one system occur in an individual, the possibility of single etiology arises, but beside some hypothesis, no definite link could be established in any of the cases reported. [ncbi.nlm.nih.gov]
Etiology and Pathophysiology of Sex Cord-Stromal Tumors of the Testis Image : “Adolescent with Gynecomastia.” by David Andrew Copeland, Dr. Mordcai Blau www.gynecomastia-md.com – Own work. [lecturio.com]
(Etiology) The exact cause of Ovarian Sertoli-Leydig Cell Tumor is not known Research studies seem to indicate that certain genetic mutations (in the DICER1 gene) may play a role in many cases What are the Signs and Symptoms of Ovarian Sertoli-Leydig [dovemed.com]
Etiology There appear to be several established clinical syndromes associated with several subtypes of sex cord-stromal tumours. 30% of patients with sex cord tumours with annular tubules have Peutz-Jeghers syndrome (PJS), an autosomal dominant disorder [atlasgeneticsoncology.org]
Epidemiology
Epidemiology of Sex Cord-Stromal Tumors of the Testis Sex cord-stromal tumors of the testis can be considered as the rarest form of testicular tumors. The most common form of testicular cancer is germ cell tumors. [lecturio.com]
Epidemiology The tumors typically occur in young females, sometimes children who typically present with sexual precocity, and occasional patients have Peutz-Jeghers syndrome. [humpath.com]
Epidemiology The frequency of sex cord-stromal tumours is similar throughout the world. There does not appear to a racial predisposition, in contrast to epithelial ovarian cancers. [atlasgeneticsoncology.org]
Pathophysiology
Corresponding Author Departments of Pathophysiological and Experimental Pathology and Katsuaki Sato, MD, Department of Pathophysiological and Experimental Pathology, Kanazawa Medical University, 1‐1 Daigaku, Uchinada, Kahoku‐gun, Ishikawa 920‐0293, Japan [onlinelibrary.wiley.com]
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]
Etiology and Pathophysiology of Sex Cord-Stromal Tumors of the Testis Image : “Adolescent with Gynecomastia.” by David Andrew Copeland, Dr. Mordcai Blau www.gynecomastia-md.com – Own work. [lecturio.com]
Prevention
There is at present no good evidence that postoperative adjuvant therapy is effective in preventing recurrence. [ncbi.nlm.nih.gov]
Currently, there are no effective measures to prevent the occurrence of Sertoli-Leydig Cell Tumor of Ovary. [dovemed.com]
Getting connected with these people is critical to getting preventative care for other risks. My younger daughter has not had cancer, but she tested positive for dicer 1 and is being monitored by the NIH. [cancercompass.com]