Pathophysiology
Summary
Ovarian tumors display a wide range of pathologies and can be primarily sorted into germ cell tumors, sex cord-stromal tumors, and benign cysts. Germ cell tumors originate from primordial germ cells and include cystic teratomas, dysgerminomas, and yolk sac tumors.
Cystic teratomas are usually benign and predominantly occur in individuals aged 10-30 years. They contain cells from all three germ layers: ectoderm, mesoderm, & endoderm. The most abundant tissue type is ectodermal, often manifesting as hair, sebaceous glands, and teeth. On imaging, calcification from teeth and bone may appear as bright spots. Clinical presentation may include ovarian torsion, leading to severe abdominal pain. Struma ovarii is a subtype of cystic teratomas containing functioning thyroid tissue that can cause hyperthyroidism. Histologically, they display thyroid follicles filled with eosinophilic material and demonstrate uptake of radioactive iodine-123. Immature teratomas are a malignant variant containing poorly differentiated tissues.
Dysgerminomas are malignant germ cell tumors usually occurring in adolescents, and are similar histologically to testicular seminomas in males. Tumor markers include B-hCG and lactate dehydrogenase (LDH). The characteristic histologic feature is the presence of ‘fried egg cells’—large central nuclei with clear cytoplasm.
Yolk sac tumors are often malignant and predominantly occur in girls and young women. These tumors are marked by elevated levels of α-fetoprotein (AFP). Histologically, they feature Schiller-Duval bodies, which consist of a papillary structure with a central blood vessel.
Sex cord-stromal tumors encompass thecomas-fibromas, granulosa-theca tumors, and sertoli-leydig tumors.
Thecoma-fibromas are generally benign commonly present in perimenopausal women. These tumors appear yellow on gross pathology and are associated with Meigs syndrome, characterized by ascites and pleural effusion.
Granulosa-theca tumors are low-grade malignant neoplasms that can occur at any age but are most common during perimenopause. These tumors produce estrogen, which leads to clinical manifestations such as precocious puberty in younger patients and abnormal uterine bleeding or postmenopausal bleeding in older individuals. The estrogenic activity also increases the risk of endometrial hyperplasia and endometrial cancer. Inhibin serves as the specific tumor marker for these tumors. Histologically, they are characterized by Call-Exner bodies, which consist of cuboidal granulosa cells arranged in a rosette pattern around an eosinophilic center, often displaying ‘coffee bean’ nuclei. Upon gross inspection, the yellow color of these tumors is attributed to the lipid content in the theca cells.
Sertoli-Leydig tumors can be either benign or malignant and frequently occur in young adults. These tumors produce androgens, resulting in androgenic symptoms such as hirsutism, clitoromegaly, and voice changes. Histologically, they exhibit tubular structures lined by round Sertoli cells.
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FAQs
Cystic teratomas are a type of ovarian germ cell tumor originating from primordial germ cells. They usually contain cells from all three germ layers: ectoderm, mesoderm, and endoderm. These tumors are most commonly found in individuals between the ages of 10 and 30 and are generally benign. The ectodermal tissue is the most prominent component, featuring elements like hair, sebaceous glands, and teeth. On imaging studies, bright spots may appear due to calcification from teeth and bone, and the tumors can sometimes cause severe abdominal pain if ovarian torsion occurs.
Struma ovarii is a specialized subtype of cystic teratoma characterized by the presence of functioning thyroid tissue. This germ cell tumor can secrete thyroid hormones, potentially leading to hyperthyroidism. On histological examination, struma ovarii shows thyroid follicles filled with eosinophilic material. Additionally, these tumors can demonstrate uptake of radioactive iodine-123, aiding in their identification.
Dysgerminomas are malignant ovarian germ cell tumors most commonly found in adolescents. Histologically, they resemble testicular seminomas in males. These tumors often manifest as palpable adnexal masses and can cause severe abdominal pain if ovarian torsion occurs. Lactate dehydrogenase (LDH) and B-hCG serve as tumor markers for dysgerminomas. The histology often reveals "fried egg cells," which are characterized by large central nuclei surrounded by clear cytoplasm.
Thecoma-fibromas and granulosa-theca tumors are both types of ovarian sex-cord stromal tumors but differ in several key aspects. Thecoma-fibromas are usually benign and most commonly occur in perimenopausal women. They can cause Meigs syndrome, which is characterized by ascites and pleural effusion. Granulosa-theca tumors, however, are low-grade malignant tumors that can appear at any age but are most common in perimenopausal women. These tumors produce estrogen, leading to symptoms like precocious puberty or abnormal uterine bleeding, and are associated with the tumor marker inhibin.
Sertoli-Leydig tumors are a subtype of ovarian sex-cord stromal tumors that can be either benign or malignant. They primarily occur in young adults and produce androgens, leading to symptoms such as hirsutism, clitoromegaly, and voice changes. On histological examination, these tumors display tubular structures lined by round Sertoli cells, aiding in their identification.
