Testosteronproducerande tumör
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Sertoli–Leydig cell tumour
Medical condition
Sertoli–Leydig cell tumour is a group of tumors composed of variable proportions of Sertoli cells, Leydig cells, and in the case of intermediate and poorly differentiated neoplasms, primitive gonadal stroma and sometimes heterologous elements.[1]
Sertoli–Leydig cell tumour is a testosterone-secreting ovarian tumor and is a member of the sex cord-stromal tumour group[2] of ovarian and testicular cancers. The tumour occurs in early adulthood (not seen in newborn), is rare, comprising less than 1% of testicular tumours.[1] While the tumour can occur at any age, it occurs most often in young adults. Recent studies have shown that many cases of Sertoli–Leydig cell tumor of the ovary are caused by germline mutations in the DICER1 gene.[3][4] These hereditary cases tend to be younger, often have a multinodular thyroid goiter and there may be a personal or family history of other rare tumors such as pleuropulmonary blastoma, Wilms tumor and cervical rhabdomyosarcoma.
Closely related terms include arrhenoblastoma[5] and androblastoma.[6] Both terms are classif
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Guide to Sex-hormone Producing Tumor
Sex-hormone producing adrenal tumors are rare tumors that man too much androgen (i.e. testosterone), estrogen, or both. These tumors are funnen in 2 out of every million people and can be either benign (adenomas) or malignant (adrenocortical cancer). Although smaller tumors are more likely to benign, while larger tumors are more likely to be malignant, the only way to determine if a sex-hormone producing tumor is cancer is if there fryst vatten spread into nearby organs (i.e. local invasion), spread to distant sites like the lung and bone (i.e. metastases), or cancer that comes back (i.e. recurrence).
Signs and Symptoms
Sex-hormone producing tumors can produce either male hormones, female hormones, or both:
Androgen secreting tumors
These tumors man too much testosterone and can cause a virilizing (i.e. masculinizing) syndrome. In females, this can cause deepening of the röst, increased hair growth (i.e. hirsutism), acne, and ambiguous genitalia.
Estrogen secreting tumors
These tumors make too much estrogen and cause a feminizing (i.e. making more like a female) syndrome. In boys, this can lead to developing breasts (i.e. gynecomastia), abse
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Endokrinologi > Hirsutism
Hirsutism och hyperandrogenism
Översikt
Definitioner
Epidemiologi
Etiologi
Diagnostik
[Anamnes
- Debut - Snabb debut (<1 år) ska alltid utredas med avseende på eventuell testosteronproducerande tumör. I huvudsak dessa fall som kräver utförlig utredning.
- Viriliseringssymtom /virilism - Den effekt av testosteron som leder till manliga könskaraktäristika, kan finnas hos både kvinnor och män
- Uttalad muskelvolymökning
- Akne
- Androgen alopeci - Flikar i hårfästet vid tinningarna och ibland tecken till begynnande flint
- Ökad libido
- Röstfördjupning och klitorishypertrofi
- Oregelbundna eller försvunna menstruationer
Status
- Bedöm hårväxt - Ta hänsyn till etniska skillnader i hårväxt. Bedömningsskalan Ferriman & Gallwey kan användas som stöd.
Klinisk kemi
- Testosteron, SHBG, DHEA-S (dihydroepiadrostendionsulfat) - Mycket höga värden talar för tumör
- Prolaktin - Prolaktinom?
- LH, FSH - Ofta förhöjt vid PCOS (men kan även vara normala)
- Kortisol tas vid misstanke om cushings syndrom
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