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Questions and Answers
What is the most common gene associated with Lynch syndrome?
What is the most common gene associated with Lynch syndrome?
BRCA2 is associated with a higher risk of ovarian cancer compared to BRCA1.
BRCA2 is associated with a higher risk of ovarian cancer compared to BRCA1.
False
What is the recommended prevention strategy for women with BRCA1 mutations after completing family?
What is the recommended prevention strategy for women with BRCA1 mutations after completing family?
TAH + BSO
Ovarian cancer constitutes ____% of all hereditary ovarian cancers.
Ovarian cancer constitutes ____% of all hereditary ovarian cancers.
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Match the following ovarian tumor types with their characteristics:
Match the following ovarian tumor types with their characteristics:
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Which type of tumor is classified as an estrogen-producing tumor?
Which type of tumor is classified as an estrogen-producing tumor?
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The Sertoli-Leydig cell tumor is a type of estrogen-producing tumor.
The Sertoli-Leydig cell tumor is a type of estrogen-producing tumor.
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What is the most common demographic affected by sex cord stromal tumors?
What is the most common demographic affected by sex cord stromal tumors?
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The granulosa cell tumor produces _____ and inhibin.
The granulosa cell tumor produces _____ and inhibin.
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Match the sex cord stromal tumors with their characteristics:
Match the sex cord stromal tumors with their characteristics:
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Which type of epithelial ovarian tumor is most commonly associated with BRCA-1 and BRCA-2 mutations?
Which type of epithelial ovarian tumor is most commonly associated with BRCA-1 and BRCA-2 mutations?
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Mucinous tumors are more frequently bilateral compared to serous tumors.
Mucinous tumors are more frequently bilateral compared to serous tumors.
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Which of the following tumors is the most common in women of childbearing age?
Which of the following tumors is the most common in women of childbearing age?
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What is the most common type of epithelial ovarian tumor?
What is the most common type of epithelial ovarian tumor?
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The gross finding of mucinous tumors typically displays a ______ appearance.
The gross finding of mucinous tumors typically displays a ______ appearance.
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Dysgerminomas are entirely resistant to radiation therapy.
Dysgerminomas are entirely resistant to radiation therapy.
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Match the following tumor types with their characteristics:
Match the following tumor types with their characteristics:
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What is the characteristic histological feature of a Yolk Sac Tumor?
What is the characteristic histological feature of a Yolk Sac Tumor?
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The most malignant germ cell tumor with the worst prognosis is called a _____
The most malignant germ cell tumor with the worst prognosis is called a _____
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Match the tumor type with its corresponding feature:
Match the tumor type with its corresponding feature:
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What is the most common benign ovarian tumor cyst that may undergo torsion?
What is the most common benign ovarian tumor cyst that may undergo torsion?
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In the second trimester, management includes removal of cysts irrespective of gestational age if malignant features are identified on ultrasound.
In the second trimester, management includes removal of cysts irrespective of gestational age if malignant features are identified on ultrasound.
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What is a known protective factor related to ovulation?
What is a known protective factor related to ovulation?
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Risk factors for ovarian cancer include excessive estrogen, late menopause, and _______.
Risk factors for ovarian cancer include excessive estrogen, late menopause, and _______.
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Match the following protective factors with their related categories:
Match the following protective factors with their related categories:
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Which of the following tumor markers is associated with Choriocarcinoma?
Which of the following tumor markers is associated with Choriocarcinoma?
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The most common germ cell cancer is Dysgerminoma.
The most common germ cell cancer is Dysgerminoma.
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What is the likely malignancy associated with a Dermoid cyst?
What is the likely malignancy associated with a Dermoid cyst?
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The most common ovarian tumor seen in dysgenetic gonads is __________.
The most common ovarian tumor seen in dysgenetic gonads is __________.
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Match the ovarian tumors to their characteristics:
Match the ovarian tumors to their characteristics:
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Which type of ovarian cyst is most common and often resolves spontaneously?
Which type of ovarian cyst is most common and often resolves spontaneously?
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Malignant adnexal masses are more commonly unilateral than benign adnexal masses.
Malignant adnexal masses are more commonly unilateral than benign adnexal masses.
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What are some common vague symptoms associated with ovarian cancer?
What are some common vague symptoms associated with ovarian cancer?
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A functional ovarian cyst occurs due to __________ disturbances.
A functional ovarian cyst occurs due to __________ disturbances.
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Match the ultrasound features with their corresponding characterization:
Match the ultrasound features with their corresponding characterization:
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Which of the following is the most common cause of Pseudomyxoma Peritonei?
Which of the following is the most common cause of Pseudomyxoma Peritonei?
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Clear cell tumors are primarily associated with endometriosis and in-utero DES exposure.
Clear cell tumors are primarily associated with endometriosis and in-utero DES exposure.
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What type of tumor is characterized by Walthard cell nests and coffee bean nuclei on histopathological examination?
What type of tumor is characterized by Walthard cell nests and coffee bean nuclei on histopathological examination?
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The most common type of germ cell tumor observed in young girls is _________.
The most common type of germ cell tumor observed in young girls is _________.
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Match the following germ cell tumors with their characteristics:
Match the following germ cell tumors with their characteristics:
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What is the defining characteristic of Call Exner bodies?
What is the defining characteristic of Call Exner bodies?
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Meig's syndrome can be associated with benign tumors.
Meig's syndrome can be associated with benign tumors.
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Name three primary metastatic tumors that can affect the ovary.
Name three primary metastatic tumors that can affect the ovary.
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The _____ tumor is characterized by signet ring cells filled with mucin in the metastatic ovarian tumor.
The _____ tumor is characterized by signet ring cells filled with mucin in the metastatic ovarian tumor.
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Match the following syndromes or tumors with their descriptions:
Match the following syndromes or tumors with their descriptions:
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What is the management strategy for ovarian cysts sized between 5-7 cm?
What is the management strategy for ovarian cysts sized between 5-7 cm?
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CA-125 has significant value in the reproductive age group for diagnosing ovarian cysts.
CA-125 has significant value in the reproductive age group for diagnosing ovarian cysts.
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What percentage likelihood of malignancy is associated with post-menopausal ovarian masses?
What percentage likelihood of malignancy is associated with post-menopausal ovarian masses?
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If a pre-pubertal patient exhibits any elevated markers such as AFP, HCG, or LDH, the recommended management strategy is __________ after investigations.
If a pre-pubertal patient exhibits any elevated markers such as AFP, HCG, or LDH, the recommended management strategy is __________ after investigations.
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Match the reproductive age groups with their corresponding management strategies for ovarian cysts:
Match the reproductive age groups with their corresponding management strategies for ovarian cysts:
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Study Notes
Sex Cord Stromal Tumors
- Sex cord tumors can produce estrogen (feminizing) or androgen (virilizing) hormones.
- Granulosa cell tumors are estrogen-producing.
- Sertoli-Leydig cell tumors, Leydig cell tumors, and Hilus cell tumors produce androgens.
- Reineke's crystals are a characteristic histological feature.
- Stromal tumors include fibroma, thecoma, and fibrothecoma.
- Sex cord stromal tumors have a better prognosis than germ cell tumors and epithelial cell tumors.
Granulosa Cell Tumors
- Originate from granulosa cells.
- Produce estrogen and inhibin.
- Antimullerian hormone is a tumor marker.
- Present with excessive bleeding (atypical uterine bleeding, postmenopausal bleeding, precocious puberty).
- Ovarian cancers associated with endometrial cancer include endometrioid tumors and granulosa cell tumors.
- Endometrial sampling is mandatory in all cases with granulosa cell tumors.
Genetic Syndromes
- Lynch syndrome is associated with a high risk of colorectal cancer and endometrial cancer, with a 20% risk of ovarian cancer.
- BRCA1 mutation carries a maximum risk of 40% for ovarian cancer and an increased risk of endometrial cancer.
- BRCA2 mutation carries a 15% risk of ovarian cancer.
Hereditary vs Sporadic Ovarian Cancer
- Hereditary ovarian cancer affects younger women (under 50 years old) and accounts for about 5-10% of all ovarian cancers.
- Sporadic ovarian cancer is most common in women aged 60-70 years old.
Screening
- Women with a first-degree relative with hereditary ovarian cancer or a BRCA mutation need to be assessed for sporadic ovarian cancer.
- Annual screening with transvaginal sonography (TVS) and CA 125 is recommended for women aged 35-40 years old.
WHO Classification & Epithelial Ovarian Tumors
- Epithelial ovarian tumors are the most common (90%), followed by germ cell tumors (5-8%), then sex cord stromal tumors (3-5%).
- Serous cystadenoma is the most common overall, while dermoid cysts are most common in reproductive age.
- Serous cystadenocarcinoma is the most common type of ovarian cancer.
Types of Epithelial Ovarian Tumors
- Serous tumors are the most common.
- Other types include mucinous, Brenner's, endometrioid, and clear cell tumors.
- Most common age for epithelial ovarian cancer is 60 years old.
- Most commonly bilateral.
- Prognosis is poor due to non-specific symptoms.
- Psammoma bodies are a characteristic histological feature.
- Mutations include KRAS, PTEN, and p53.
Serous vs. Mucinous Tumors
- Serous tumors are more commonly benign (60% are serous cystadenoma) and often bilateral.
- Mucinous tumors are more commonly benign (80% are mucinous cystadenoma) and mostly unilateral.
- Serous tumors are associated with BRCA1, BRCA2, and p53 mutations.
- Mucinous tumors are associated with KRAS mutation.
- Mucinous tumors are linked to smoking, while serous tumors are not.
- Serous cystadenocarcinoma is the malignant counterpart of serous cystadenoma.
- Mucinous cystadenocarcinoma is the malignant counterpart of mucinous cystadenoma.
- CA 125 is the tumor marker for serous tumors.
- CEA and CA 19-9 are the tumor markers for mucinous tumors.
- Psammoma bodies are present in serous tumors.
- Mucinous tumors present with a honeycomb appearance on gross examination due to multiloculated cysts filled with mucinous material.
Dysgerminoma
- The second most common germ cell tumor (GCT).
- Most common in women of childbearing age.
- 15-20% are bilateral, with the highest risk of bilaterality among GCTs.
- Typically appears fleshy, solid, lobulated, and tan-colored.
- Has the best prognosis among GCTs.
- Only radiosensitive ovarian tumor.
- Histologically characterized by nests of cells separated by fibrous septa with lymphocyte infiltration.
- USG features include tip of iceberg sign and dot & dash appearance.
- Management includes cystectomy or oophorectomy depending on family plans.
Yolk Sac Tumor
- The most malignant GCT with the worst prognosis due to rapid progression.
- Histologically characterized by Schiller Duval bodies (glomeruloid bodies).
- Can present with acute abdomen.
- Typically unilateral (100%).
- Histologically shows central capillaries surrounded by tumor cells within cystic spaces.
Ovarian Pathologies in Pregnancy
-
Management:
- Benign ovarian tumor cysts are most likely to undergo torsion, especially at the end of the first trimester or during the puerperium.
- Dysgerminoma is the most common ovarian cancer in pregnancy.
-
1st Trimester:
- Corpus luteum is the most common type of ovarian cyst and usually resolves spontaneously.
- Removal of cysts in the first trimester could lead to decreased progesterone and abortion.
-
2nd Trimester:
- Symptomatic cysts (rupture or torsion) or cysts with malignancy features on USG (>10 cm) should be removed regardless of gestational age.
Factors Related to Ovarian Cancer
-
Proven Risk Factors:
- Excessive estrogen
- Early menarche
- Late menopause
- Genetic syndromes (Lynch syndrome, BRCA1, BRCA2)
- Exposure to carcinogens
- Nulliparity
- Asbestos
-
Controversial Risk Factors:
- Polycystic Ovarian Syndrome (PCOS)
- Hormone Replacement Therapy (HRT)
- Obesity
- Endometriosis
- Infertility and ovulation-inducing drugs
- Talc
- Smoking (mucinous adenocarcinoma)
Protective Factors
-
Related to Estrogen:
- Physical exercise
- Oral contraceptive pills (OCP)
- Breastfeeding
-
Related to Ovulation:
- Anovulation
- Multiparity
- OCP
-
Surgeries:
- Hysterectomy
- Tubal ligation
- Salpingectomy
Tumor Markers
- Used to diagnose and monitor GCTs.
- GCTs: LDH, HCG, PLAP (Placental alkaline phosphatase)
- Dysgerminoma: LDH, HCG
- Yolk Sac Tumor: AFP, LDH
- Embryonal Cancer: AFP, HCG
- Choriocarcinoma: HCG
- Teratoma: No tumor marker
Germ Cell Tumors (GCT)
- Most common in young girls (10-30 years old).
- Typically occur unilaterally.
- Types include teratoma (mature cystic teratoma/dermoid cyst, immature teratoma, struma ovarii), dysgerminoma, yolk sac tumor, embryonal cancer, choriocarcinoma, and mixed types.
- Have a better prognosis than other ovarian cancers due to early diagnosis.
258
- Histological feature of granulosa cell tumor, characterized by central acidophilic material surrounded by follicle-like cells with coffee bean nuclei.
Fibroma
- Unilateral, benign, solid tumor.
- Histologically characterized by well-differentiated fibroblasts.
- Associated with Meig's syndrome.
Meig's vs. Pseudo-Meig's Syndrome
- Meig's syndrome is characterized by a fibroma/thecoma/Brenner's tumor/granulosa cell tumor, ascites, and pleural effusion.
- Pseudo-Meig's syndrome is characterized by any other ovarian tumor (mucinous cystadenoma) or any other condition (fibroid), ascites, and pleural effusion.
- Removing the tumor in Meig's syndrome resolves ascites and pleural effusion.
Metastatic Tumors of the Ovary
- Most commonly originate from the stomach.
- Other common primary sources include gastrointestinal tract and breast carcinoma.
- Krukenberg tumors are metastatic tumors from stomach cancer (pyloric end), which spread through retrograde lymphatics to the ovaries.
- Bilateral (80%) and symmetrical enlargement of ovaries.
- The shape and capsule of the ovary are retained.
- Mobile and waxy consistency.
- Histologically characterized by signet ring cells with mucin-filled cytoplasm pushing the nucleus to one side.
Ovarian Cysts & Ovarian Cancer
- Functional ovarian cysts are the most common type, occurring due to hormonal disturbances.
- They are usually temporary and resolve spontaneously, requiring no specific treatment.
- Types include follicular cyst, corpus luteal cyst, and theca lutein cyst.
- Follicular cysts are the most common type and are more likely to rupture.
- Theca lutein cysts are associated with increased HCG levels and are seen in cases like molar pregnancy, twin pregnancy, and fertility treatments with clomiphene and HMG.
Features of Ovarian Cancers
- Rare, therefore screening is not routinely performed.
- Present with vague symptoms like nausea, vomiting, loss of appetite, and adnexal masses.
- Imaging of the ovaries (IOC) with a transvaginal sonography (TVS) is crucial for diagnosis.
Benign vs. Malignant Adnexal Masses
-
Benign Adnexal Masses:
- More common in reproductive age.
- Pain is usually present due to inflammation.
- No specific history.
- Unilateral.
- Cystic consistency.
- Tenderness.
-
Malignant Adnexal Masses:
- More common in extreme age groups (pre-pubertal/menopausal).
- Often no pain.
- Rapid progression and weight loss are common.
- Bilateral.
- Solid consistency.
- No tenderness.
Ultrasound Features
-
Benign:
- Unilateral
- Anechoic
- Unilocular
-
Malignant:
- Bilateral
- Solid components >10 cm
- Variable consistency (Solid components)
- Thick septa with papillary outgrowths (Excrescenes).
- Highly vascular.
- Evidence of ascites, enlarged lymph nodes, and matted bowel loops.
Management of Ovarian Cysts
-
Reproductive Age Group:*
- 3-5 cm: Wait and Watch.
- 5-7 cm: Follow up with serial ultrasounds.
-
7 cm: Surgery for torsion or rupture.
-
Extremes of Age:*
- Post-menopausal (CA125 ≥ 35 IU): Further investigations and surgery.
- Pre-pubertal: If elevated markers (AFP, HCG, LDH) are present, further investigations and surgery.
Features Suggestive of Ovarian Malignancy on Ultrasound (USG)
- Papillary excrescences
- Thick >3mm septations
- Mural nodularity
- Papillary projections
- Frank solid components
Notes
- CA-125 has limited value in the reproductive age group.
- Important: This document is a summary. Seek medical advice for diagnosis and treatment.
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Description
This quiz covers the essential aspects of sex cord stromal tumors, including their hormone production, types, and prognosis. It also delves into granulosa cell tumors, their characteristics, symptoms, and associations with other cancers. Additionally, it examines genetic syndromes that increase cancer risk, particularly Lynch syndrome.
