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Sertoli Leydig Cell Tumor

Sertoli-Leydig Cell Tumor


Presentation

  • The usual presentation of SLCTs is with signs of androgen excess as majority of them produce androgens. We present a case of a SLCT occurring in a 70 year old woman. Her presenting complaint was abdominal distension and pain.[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]
  • 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]
  • 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]
  • Excessive estrogen production may result in precocious puberty, abnormal vaginal bleeding, menstrual irregularities, generalized edema, weight gain, endometrial hyperplasia, endometrial polyps and endometrial carcinoma. 2 , 5 Features of masculinization[pagepress.org]
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]
Ascitic Fluid
  • The authors present a case of a poorly differentiated Sertoli-Leydig cell tumor in which ascitic fluid was found to have a high concentration of androgenic hormones and precursors from the delta steroid pathway (17-OH progesterone, testosterone, and androstenedione[ncbi.nlm.nih.gov]
  • There was no ascitic fluid; hence peritoneal washings were taken for malignant cells.[npplweb.com]
Nail Abnormality
  • Following surgery, the patient's unique nail abnormalities also resolved gradually. The IHC evaluation also confirmed the presence of AR in nail bed tissues of females.[ncbi.nlm.nih.gov]
Increased Abdominal Girth
  • A 15-year-old girl presented to a primary care clinic with secondary amenorrhea, masculinization, and rapidly increasing abdominal girth.[ncbi.nlm.nih.gov]
  • ., CTR Vice President of Cancer Registry 78 y/o female admitted for vague abdominal pain and increasing abdominal girth. Initial imaging work-up revealed bilateral adnexal masses, right larger than left. No significant pelvic adenopathy.[mrahis.com]
Hypertension
Hirsutism
  • The patient was alive and well with improved hirsutism 4 years after the operation. The duration of symptoms and T level in our patient were among the highest values ever recorded in the literature.[ncbi.nlm.nih.gov]
Sparse to No Axillary Hair
  • The complete androgen insensitivity (testicular feminization) syndrome was described in phenotypic females with 46XY karyotype, presenting with primary amenorrhea, adequate breast development, and absent or sparse pubic or axillary hair.[ncbi.nlm.nih.gov]
Secondary Amenorrhea
  • A 15-year-old girl presented to a primary care clinic with secondary amenorrhea, masculinization, and rapidly increasing abdominal girth.[ncbi.nlm.nih.gov]
Primary Amenorrhea
  • The complete androgen insensitivity (testicular feminization) syndrome was described in phenotypic females with 46XY karyotype, presenting with primary amenorrhea, adequate breast development, and absent or sparse pubic or axillary hair.[ncbi.nlm.nih.gov]
  • Case Report A 23-year-old unmarried woman presented to the Gynecology OPD with primary amenorrhea and a history of intermittent pain over left lower abdomen for 8 months. Physical examination revealed hirsutismand normal stature.[medresearch.in]
Urinary Retention
  • A 13-year-old girl presented with acute urinary retention, excessive hair growth and deepening of the voice. A mass the size of a 28-week gravid uterus was arising from the pelvis. Serum testosterone level was raised to 145.2 ng/dl.[ncbi.nlm.nih.gov]

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]
Ovarian Mass
  • The sections from her ovarian mass for which she was operated a year back displayed features of a poorly differentiated SLCT with heterologous elements, including focal rhabdomyoblastic differentiation.[ncbi.nlm.nih.gov]
Multilocular Cyst
  • The tumor had a heterologous element of gastrointestinal type epithelium, retiform pattern, and multilocular cysts, many of which were lined by Sertoli-like cells.[ncbi.nlm.nih.gov]

Treatment

  • Surgical treatment was conservative with preservation of the contralateral adnexa and uterus. No adjuvant treatment was given. At 4 years control post surgery, the patient was without evidence of disease.[ncbi.nlm.nih.gov]

Prognosis

Etiology

  • We discuss the complex aspects of etiology and pathogenesis, the clinical and hormonal settings, the role of immunocytochemical markers in diagnosis, as well as the therapy and the prognostic features of this ovarian tumor.[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]
Sex distribution
Age distribution

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]

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