sex cord-stromal benign neoplasm

Sex Cord-Stromal Benign Neoplasms: An Overview

Sex cord-stromal benign neoplasms are a type of non-cancerous (benign) tumor that develops from the cells in the core of the ovary, surrounding the egg cells. These tumors are rare and account for only a small percentage of all ovarian tumors.

Types of Benign Neoplasms

There are two main types of benign sex cord-stromal neoplasms:

• Fibromas: These are solid, firm, and lobulated tumors that can vary in size. They are the most common type of benign sex cord-stromal tumor.
• Thecomas: These are soft and friable tumors that can also vary in size.

Characteristics

Benign sex cord-stromal neoplasms typically have the following characteristics:

• Unilateral: These tumors usually occur on one side of the ovary.
• Variable size: They can range from small to large, with some tumors being up to 15 cm in greatest dimension.
• Solid or soft texture: Fibromas are solid and firm, while thecomas are soft and friable.

Symptoms

Benign sex cord-stromal neoplasms may not cause any symptoms at all. However, if they do occur, symptoms can include:

• A painless lump in the scrotum (if a tumor is in the testicles)
• A lump in the belly, sometimes with pain
• Bloating

References

• [1] These tumors are typically unilateral, 10 to 15 cm in greatest dimension, and may vary from solid, firm, and lobulated to soft and friable, often with ... (Source: #2)
• [3] SCSTs develop from cells in the core of the ovary that surround the egg cells. (Source: #3)
• [8] Benign sex cord stromal tumors include fibromas and thecomas. These benign tumors can sometimes mimic cancerous tumors by producing ascites (abnormal fluid in ... (Source: #9)

Sex cord-stromal tumors, including benign neoplasms, can present with a variety of signs and symptoms depending on their location and hormonal activity.

Common symptoms:

• Abdominal pain and constipation, if the tumor is in the ovaries or belly [1]
• Shortness of breath or wheezing, if the tumor is in the chest
• Hormonal effects such as:
  • Hirsutism (excessive hair growth) [3]
  • Menstrual disorders due to hyperestrinism in women of reproductive age [6]
  • Irregular menstruation and vaginal bleeding in young girls with juvenile granulosa cell tumors [5]

Other symptoms:

• Ascites (fluid accumulation in the abdomen) with or without other abdominal symptoms [4]
• Sudden abdominal pain and swelling, which are frequent symptoms of nonfunctioning tumors [7]
• Elevated alpha-fetoprotein levels, which can be associated with certain types of sex cord-stromal tumors [8]

Types of benign neoplasms:

• Fibrosarcoma is an extremely rare and aggressive subtype of SCST [2]
• Juvenile granulosa cell tumors are a type of sex cord-stromal tumor that typically present in young girls with precocious pseudopuberty, vaginal bleeding, irregular menstruation, and other symptoms [5]

It's worth noting that the presentation of sex cord-stromal benign neoplasms can vary widely depending on the specific type of tumor and its location. A definitive diagnosis is usually made through a combination of clinical evaluation, imaging studies, and histopathological examination.

References: [1] KAP Schultz (2016) [2] [3] [4] DM Gershenson [5] R Al Harbi (2021) [6] M Horta (2015) [7] [8]

Diagnostic Tests for Sex Cord-Stromal Benign Neoplasms

Sex cord-stromal tumors are a rare type of ovarian tumor that can be challenging to diagnose. However, various diagnostic tests and markers can help in the diagnosis and management of these tumors.

• Immunohistochemical tests: These tests can be used to identify specific proteins expressed by sex cord-stromal tumors. The commonly available immunohistochemical tests for this type of tumor include vimentin, keratin, epithelial membrane antigen (EMA), inhibin, calretinin, CD56, SF-1, Melan-A, WT1, and FOXL2 [6][7].
• Serum tumor markers: Serum tumor markers such as inhibin can be used to assist in preoperative diagnosis and surveillance of sex cord-stromal tumors [3].
• Imaging studies: Imaging studies such as ultrasound, CT scans, and MRI can help in the differential diagnosis from more common epithelial tumors. Ultrasound is generally used to assist the diagnosis, and two-dimensional ultrasound has provided an early diagnosis for some cases [9][10].
• Molecular diagnostic testing: Molecular diagnostic testing can be useful in identifying specific genetic mutations associated with sex cord-stromal tumors, such as those observed in adult granulosa cell tumor, Sertoli-Leydig cell tumor, microcystic stromal tumor, and their mimics [5].

It's worth noting that the diagnosis of sex cord-stromal tumors often requires a combination of clinical, radiologic, and histopathological findings. A multidisciplinary approach involving gynecologists, radiologists, and pathologists is essential for accurate diagnosis and management.

References: [1] CD56 is a sensitive marker of ovarian sex cord-stromal tumors (Search result 1) [2] Transvaginal US with color Doppler and MRI are important tools for diagnosing these tumors (Search result 2) [3] Serum tumor markers may assist in preoperative diagnosis and surveillance (Search result 3) [4] Sex cord-stromal tumors of the ovary: Epidemiology, clinical features, and diagnosis in adults (Search result 4) [5] Practical roles for molecular diagnostic testing in ovarian adult granulosa cell tumour, Sertoli–Leydig cell tumour, microcystic stromal tumour and their mimics (Search result 5) [6] The commonly available immunohistochemical tests which can be used in the diagnosis of the neoplasm consists of vimentin, keratin, epithelial membrane antigen (EMA), inhibin, calretinin, CD56, SF-1, Melan-A, WT1, and FOXL2 (Search result 6) [7] A number of markers may be helpful in the diagnosis of a sex cord-stromal tumor, including inhibin (Search result 7)

Treatment Options for Sex Cord-Stromal Benign Neoplasms

Sex cord-stromal tumors are a type of ovarian tumor that can be either benign or malignant. The treatment options for sex cord-stromal benign neoplasms depend on several factors, including the type and stage of the tumor.

• Surgery: Surgery is often the primary treatment for sex cord-stromal benign neoplasms. The goal of surgery is to remove the tumor in its entirety, along with a margin of healthy tissue around it (1). This can be achieved through various surgical procedures, such as hysterectomy with or without bilateral salpingo-oophorectomy (BSO), or mass resection alone (10).
• Hormonal therapy: In some cases, hormonal therapy may be used to treat sex cord-stromal benign neoplasms. This can involve the use of medications that suppress hormone production, such as leuprolide (Lupron) and goserelin (Zoladex) (3).

Key Points

• Surgery is often the primary treatment for sex cord-stromal benign neoplasms.
• The goal of surgery is to remove the tumor in its entirety, along with a margin of healthy tissue around it.
• Hormonal therapy may be used in some cases to treat sex cord-stromal benign neoplasms.

References

(1) by KAP Schultz · 2016 · Cited by 131 (3) Apr 11, 2018 (10) by M Shibahara · Cited by 2

The differential diagnosis for sex cord-stromal benign neoplasms, such as sclerosing stromal tumors (SSTs), involves considering other types of ovarian tumors that may present with similar characteristics.

• Adult granulosa cell tumor: This is a type of sex cord-stromal tumor that can be difficult to distinguish from SSTs, particularly when they have a diffuse pattern. Nuclear grooves are characteristic of adult granulosa cell tumors (2).
• Thecoma: Thecoma is another type of sex cord-stromal tumor that may be considered in the differential diagnosis for SSTs.
• Steroid cell tumor: This type of tumor can mimic a microcystic stromal tumor, but unlike it, steroid cell tumors are inhibin and calretinin negative (9).

It's worth noting that immunostains can be definitive in differentiating between these types of tumors, as sex cord-stromal tumors are positive for inhibin, calretinin, and SF1 and negative for TTF-1 (6).

In terms of clinical presentation, sex cord-stromal tumors primarily treated surgically, and clinical and radiologic clues can be helpful in differential diagnosis from the more common epithelial tumors (10).

References: * [2] * [6] * [9] * [10]