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Sertoli cell tumor

Description

A Sertoli cell tumor, also known as a Sertoli-Leydig cell tumor, is a rare type of sex cord-stromal tumor that can occur in the ovaries or testes.

Characteristics:

  • These tumors are composed of cells resembling embryonal, prepubertal, and adult Sertoli cells.
  • They can produce male sex hormones, such as testosterone, which can cause symptoms like facial hair and a deep voice in females.
  • Sertoli cell tumors tend to grow in an expansile fashion, forming nodular, homogeneous or multi-cystic masses.

Types:

  • There are several subtypes of Sertoli cell tumors, including general, large-cell calcifying, and sclerosing Sertoli cell tumors.
  • Some Sertoli-Leydig cell tumors can also contain Leydig cells, which release male sex hormones.

Incidence:

  • Sertoli cell tumors account for less than 1% of all testicular tumors.
  • They are the second most common sex cord-stromal tumor after granulosa cell tumors.

Symptoms:

  • Symptoms may include hormone-related changes, such as facial hair and a deep voice in females.
  • In rare cases, Sertoli-Leydig cell tumors can produce estrogen, leading to symptoms like endometrial hyperplasia or cancer.

Treatment:

  • Treatment for Sertoli cell tumors typically involves surgical removal of the tumor.
  • In some cases, hormone replacement therapy may be necessary to manage symptoms related to hormone production.

Overall, Sertoli cell tumors are rare and complex tumors that require careful diagnosis and treatment.

Additional Characteristics

  • hormone replacement therapy
  • Sertoli-Leydig cell tumor
  • sex cord-stromal tumor
  • ovaries or testes
  • male sex hormones (testosterone)
  • facial hair and deep voice in females
  • nodular, homogeneous or multi-cystic masses
  • large-cell calcifying subtype
  • sclerosing Sertoli cell tumor
  • Leydig cells releasing male sex hormones
  • endometrial hyperplasia or cancer

Signs and Symptoms

Common Signs and Symptoms of Sertoli Cell Tumor

Sertoli cell tumors, also known as Sertoli-Leydig cell tumors, are rare ovarian tumors that can cause a range of symptoms in women. Here are some common signs and symptoms associated with this condition:

  • Painless lump or swelling in the ovary: A palpable mass may be felt during a pelvic exam.
  • Abdominal pain and swelling: The tumor can cause discomfort, bloating, and swelling in the abdominal area.
  • Hormonal changes: The tumor produces male sex hormones, leading to symptoms such as:
    • Facial hair and deepening voice: Women may experience virilization, including facial hair growth and a deeper voice.
    • Early puberty in males: Boys may exhibit early signs of puberty, including rapid growth and development.
  • Other physical changes: Some women may experience other physical symptoms, such as:
    • Alopecia (hair loss)
    • Single mammary gland enlargement
    • Testicular asymmetry

These symptoms can vary in severity and may not be present in all cases. If you are experiencing any of these symptoms or have concerns about your health, it is essential to consult a healthcare provider for proper evaluation and diagnosis.

References:

  • [1] A painless lump in the testicle, which is the most common sign; Swelling of the testicle, with or without pain; A feeling of weight in the testicles; A dull ... (Search Result 4)
  • [2] by HY Zhang · 2014 · Cited by 51 — Patients with Sertoli-Leydig cell tumor often present with an abdominal pain and a series symptoms of defeminization and progressive virilization. (Search Result 10)
  • [3] Sertoli-Leydig cell tumor is a rare ovarian tumor composed of sex cord (Sertoli cells) and stromal (Leydig cells) elements, accounting for < 0.5% of all ovarian neoplasms ... Clinical presentation may include pelvic pain or a pelvic mass, rarely with ascites and tumor rupture Androgenic hormonal symptoms (virilization) are common and present in ... (Search Result 11)
  • [4] Sertoli cell tumor is a pure sex cord neoplasm of the ovary composed of Sertoli cells most commonly arranged in a tubular pattern ... This type of tumor is sometimes called arrhenoblastoma of the ovary or a stromal tumor. (Search Result 12)

Additional Symptoms

  • Abdominal pain and swelling
  • Painless lump or swelling in the ovary

Diagnostic Tests

Diagnostic Tests for Sertoli Cell Tumor

Sertoli cell tumors are rare testicular neoplasms that can be challenging to diagnose. However, various diagnostic tests can help identify this condition.

  • Imaging tests: An ultrasound or CT scan is often performed to locate the tumor and determine its size and shape [5]. These tests use x-rays, magnetic fields, sound waves, or radioactive substances to create images of the internal structures.
  • Serum alpha-fetoprotein (AFP) tumor marker test: Elevated levels of AFP can indicate a Sertoli cell tumor [4].
  • Immunohistochemistry: This test uses antibodies to detect specific proteins in tissue samples. AMH has been shown to be a useful marker for immature and neoplastic Sertoli cells in dogs, but its application in humans is not well established [7].
  • Histopathology: A biopsy or surgical removal of the tumor is often necessary to confirm the diagnosis through histopathological examination [8]. This involves examining tissue samples under a microscope to identify characteristic features of Sertoli cell tumors.
  • Genetic testing: In some cases, genetic testing may be recommended to identify potential underlying genetic mutations that could contribute to the development of Sertoli cell tumors [6].

It's essential to note that these diagnostic tests are not exhaustive and may vary depending on individual circumstances. A comprehensive evaluation by a healthcare professional is necessary for an accurate diagnosis.

References: [4] - Diagnostic studies showed an isolated elevation of the serum alpha-fetoprotein tumor marker. [5] - An ultrasound or CT scan will likely be done to find out where the tumor is and its size and shape. [6] - Diagnosis of a testicular Sertoli cell tumor should prompt DICER1 genetic testing so that patients with a DICER1 germline pathogenic variant can benefit ... [7] - Using immunohistochemistry, AMH has been shown to be a useful marker of immature and neoplastic Sertoli cells in dogs. [8] - These tumors are commonly diagnosed via histopathology at the Texas A&M Veterinary Medical Diagnostic Laboratory (TVMDL).

Treatment

Treatment Options for Sertoli Cell Tumor

Sertoli cell tumors, whether benign or malignant, can be treated with various methods. The primary goal is to remove the tumor and prevent its recurrence.

  • Benign Sertoli Cell Tumors: For benign Sertoli cell tumors, the standard treatment is surgical removal of the affected testis, also known as orchiectomy (Treatment for benign Sertoli cell tumor is orchiectomy. Malignant Sertoli cell tumors may be treated additionally with RPLND.[5]). This approach is usually curative and prevents further complications.
  • Malignant Sertoli Cell Tumors: In cases of malignant Sertoli cell tumors, additional treatment may be necessary to address potential metastasis or recurrence. Chemotherapy can be used in conjunction with surgery (If so, chemotherapy (chemo) may not be needed. If your child needs chemotherapy, they may get the drugs cisplatin, etoposide, and bleomycin.[9]). The specific chemotherapy regimen may vary depending on individual circumstances.

Chemotherapy for Sertoli Cell Tumors

While chemotherapy is an option for malignant Sertoli cell tumors, its use can depend on various factors such as tumor size, grade, and the presence of metastasis. Chemotherapy regimens like cisplatin, etoposide, and bleomycin may be considered (If so, chemotherapy (chemo) may not be needed. If your child needs chemotherapy, they may get the drugs cisplatin, etoposide, and bleomycin.[9]).

References

  • [5] Malignant Sertoli cell tumors may be treated additionally with RPLND.
  • [9] If so, chemotherapy (chemo) may not be needed. If your child needs chemotherapy, they may get the drugs cisplatin, etoposide, and bleomycin.

Note: The information provided is based on the context given and might not reflect the most up-to-date or comprehensive treatment guidelines for Sertoli cell tumors.

Recommended Medications

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Differential Diagnosis of Sertoli Cell Tumor

Sertoli cell tumors are rare ovarian neoplasms that can be challenging to diagnose due to their similarity in appearance with other sex cord-stromal tumors. The differential diagnosis for Sertoli cell tumors includes:

  • Intratubular large cell hyalinizing Sertoli cell neoplasia: This is a rare variant of Sertoli cell tumor that is characterized by the presence of large, hyalinized cells within the tubules.
  • Sertoli cell adenoma: This is a benign tumor composed of Sertoli cells that are arranged in a tubular or gland-like pattern.
  • Tubular hamartoma: This is a rare, benign tumor that consists of disorganized tubules and stroma.
  • Leydig cell tumors: These are sex cord-stromal tumors that produce hormones, particularly androgens. They can be difficult to distinguish from Sertoli cell tumors based on morphology alone.
  • Granulosa cell tumors: These are sex cord-stromal tumors that are composed of granulosa cells and can produce estrogen.
  • Endometrioid carcinoma: This is a type of ovarian cancer that can mimic the appearance of Sertoli cell tumor.

Key Features for Differential Diagnosis

To differentiate Sertoli cell tumors from other sex cord-stromal tumors, several key features should be considered:

  • Immunohistochemistry: The use of immunohistochemical markers such as inhibin and SF1 can help to confirm the diagnosis of Sertoli cell tumor.
  • Histological appearance: The presence of tubules or gland-like structures composed of Sertoli cells is characteristic of Sertoli cell tumors.
  • Clinical presentation: Sertoli cell tumors are often associated with hormone production, particularly androgens.

References

[4] Most Sertoli cell tumors are stage I, unilateral, cytologically bland, and lack significant atypia. [10] Immunohistochemistry can be an important part of the diagnosis of Sertoli cell tumor of the ovary, including distinction from non-sex cord-stromal tumors such as the sertoliform variant of endometrioid carcinoma and carcinoid.

Additional Differential Diagnoses

Additional Information

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It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.