Granulosa Cell Tumors Quiz
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Questions and Answers

What is the most common age range for diagnosis of adult granulosa cell tumors?

  • Before puberty
  • During childbearing age (correct)
  • After menopause
  • All of the above

    • What is the most common gross appearance of adult granulosa cell tumors?

  • Smooth, lobulated outline with a predominantly solid cut surface (correct)
  • Firm, rubbery texture with a fibrous appearance
  • Soft, fleshy texture with a hemorrhagic appearance
  • Irregular, nodular surface with a cystic appearance

    • Which of the following is a characteristic microscopic feature of granulosa cell tumors?

  • Presence of folds or grooves in the nuclei, resulting in a "coffee-bean" appearance (correct)
  • Presence of large, multinucleated giant cells
  • Presence of spindle-shaped cells arranged in sheets
  • Presence of epithelial cells with prominent nucleoli

    • What is the most likely reason for the development of metrorrhagia in adults with granulosa cell tumor?

    <p>Excessive production of estrogen (C)</p> Signup and view all the answers

    What is the most likely reason for the development of isosexual precocious puberty in children with granulosa cell tumor?

    <p>Excessive production of estrogen (C)</p> Signup and view all the answers

    Which of the following patterns of growth is NOT observed in granulosa cell tumors?

    <p>Tubular (D)</p> Signup and view all the answers

    Which of the following immunohistochemical markers is NOT consistently associated with granulosa cell tumors?

    <p>ERBB2 (A)</p> Signup and view all the answers

    Which of the following statements about androgenic granulosa cell tumors is TRUE?

    <p>They are often associated with a cystic appearance (D)</p> Signup and view all the answers

    What is the significance of the presence of luteinization in granulosa cell tumors?

    <p>It is often associated with pregnancy (B)</p> Signup and view all the answers

    Which of the following statements about granulosa cell tumors is FALSE?

    <p>They are always hormonally active (A)</p> Signup and view all the answers

    What type of protein, commonly associated with Ewing sarcoma, is frequently found in granulosa cell tumors?

    <p>CD99 (B)</p> Signup and view all the answers

    What is the primary diagnostic marker for adult granulosa cell tumors, appearing in almost all cases?

    <p>FOXL2 gene mutation (A)</p> Signup and view all the answers

    What is the common clinical presentation for juvenile granulosa cell tumors, occurring in nearly 80% of cases?

    <p>Isosexual precocity (A)</p> Signup and view all the answers

    What feature is typically absent in juvenile granulosa cell tumors, compared to their adult counterpart?

    <p>Nuclear grooves (A)</p> Signup and view all the answers

    What is characteristic of granulosa cell tumors that makes them diagnostically challenging?

    <p>They often show weak or absent inhibin expression. (C)</p> Signup and view all the answers

    Which of the following is NOT a characteristic feature of juvenile granulosa cell tumors?

    <p>Low mitotic activity (B)</p> Signup and view all the answers

    Which of these factors is most likely to influence the prognosis of an adult granulosa cell tumor?

    <p>Clinical stage and resectability of the tumor (C)</p> Signup and view all the answers

    In a difficult case, what test could help to distinguish between adult granulosa cell tumor and a poorly differentiated carcinoma?

    <p>FOXL2 mutation testing (C)</p> Signup and view all the answers

    What is a potential reason for the poor prognosis reported in some early studies of granulosa cell tumors?

    <p>Inclusion of ovarian carcinomas in the study group (D)</p> Signup and view all the answers

    What is a key factor distinguishing granulosa cell tumors from ovarian granulosa cell proliferations of pregnancy?

    <p>Size and number of lesions (C)</p> Signup and view all the answers

    Flashcards

    Granulosa Cell Tumor

    A type of ovarian neoplasm with differentiation towards follicular granulosa cells.

    Types of Granulosa Cell Tumor

    Includes adult and juvenile types, differing in age presentation.

    Adult Granulosa Cell Tumor Diagnosis

    Often diagnosed during childbearing age but can appear at any age.

    Hyperestrinism

    Excessive production of estrogens, associated with many granulosa cell tumors.

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    Clinical Features

    Symptoms may include isosexual precocious puberty in children and metrorrhagia in adults.

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    Gross Appearance

    Typically presents as a smooth, lobulated tumor with gray or yellow areas.

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    Microscopic Appearance

    Variable types of growth including microfollicular and macrofollicular patterns.

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    Coffee-Bean Nuclei

    Key diagnostic feature of granulosa cell tumors, showing folds or grooves.

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    Immunohistochemical Markers

    Consistent markers include vimentin, FOXL2, and SF-1 for diagnosing these tumors.

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    Cystic Patterns

    Granulosa cell tumors can present as large cystic lesions, either unilocular or multilocular.

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    Inhibin expression in tumors

    Inhibin expression may be weak or absent in tumors with scant cytoplasm, leading to diagnostic confusion.

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    Keratin expression patterns

    Keratin expression is present in 1/3 to 1/2 of cases, typically with a dot-like distribution, mainly CK8 and CK18 types.

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    S-100 protein reactivity

    Approximately 50% of cases show reactivity for S-100 protein; none are immunoreactive for EMA.

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    FOXL2 gene mutation

    Almost all adult granulosa cell tumors show somatic mutations in the FOXL2 gene, which is a useful diagnostic marker.

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    Juvenile granulosa cell tumors

    Diagnosed in nearly 80% of cases during the first two decades of life, often presenting with isosexual precocity.

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    Differential diagnosis of granulosa tumors

    Includes poorly differentiated carcinoma of surface epithelial origin, carcinoid tumor, and endometrial stromal tumors.

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    Prognosis of adult granulosa cell tumors

    Mostly indolent with prognosis similar to age-matched controls; metastases may occur years later.

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    Trisomy for chromosome 12

    Juvenile granulosa cell tumors consistently show trisomy for chromosome 12.

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    Pelvic metastases causes

    Clinical relapses may occur 10-20 years post-surgery, especially with intraoperative tumor rupture.

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    Ovarian granulosa cell proliferations

    Differential diagnosis includes microscopic changes often linked with atretic follicles during pregnancy.

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    Study Notes

    Granulosa Cell Tumor Overview

    • Granulosa cell tumor is an ovarian neoplasm originating from sex cord stromal cells, differentiating into follicular granulosa cells.
    • Two types exist: adult and juvenile.

    Adult Granulosa Cell Tumor

    • Typically diagnosed during childbearing years, but can occur before puberty or after menopause.
    • Most (75%) cases display hyperestrinism (excessive estrogen production). This can result in:
      • Isosexual precocious puberty in children.
      • Metorrhagia (irregular uterine bleeding) in adults, including postmenopausal patients.
    • Some cases are hormonally inactive, and a few are androgenic.
    • Gross appearance: Smooth, lobulated outline; predominantly solid cut surface; gray color, possibly yellow in areas with luteinization; cysts filled with straw-colored or mucoid fluid may be present, sometimes simulating a cystadenoma. Androgenic tumors tend to be large and cystic (unilocular or multilocular).
    • Microscopic appearance: Extremely variable, presenting with various growth patterns (e.g., microfollicular, macrofollicular, trabecular, etc.), potentially including a theca cell component, focal luteinization, and the characteristic "coffee bean"-shaped nuclei.
    • Diagnostic markers:
      • Immunohistochemistry: Vimentin, FOXL2, SF-1 are consistent markers. Inhibin may be weak or absent, sometimes causing confusion. Keratin, mainly CK8 and CK18, is present in about half of cases, with a dot-like distribution; S-100 protein in about 50% of cases; EMA is never positive. Estrogen and progesterone receptors are typically expressed.
      • Serum levels: Peptide hormone inhibin and follicle regulatory proteins are often elevated.
      • Genetic marker: FOXL2 gene mutation (402C→G), present in nearly all cases. This is a highly specific marker. Mutation testing is crucial in difficult cases.

    Juvenile Granulosa Cell Tumor

    • Diagnosed predominantly in the first two decades of life, with most cases presenting with isosexual precocity.
    • Slightly more common in the first two decades of life.
    • Morphological features:
      • Diffuse or macrofollicular growth patterns (diffuse more frequent); intrafollicular secretion sometimes mucin-positive; larger tumor cells; extensive luteinization; fewer nuclear grooves; theca cell component; nuclear atypia; variable, but often high mitotic activity. May show pseudopapillary features.
    • Other features: Consistent with trisomy 12.
    • Potentially associated with enchondromatosis (Ollier disease) or Maffucci syndrome.

    Differential Diagnosis

    • Adult tumours need differentiation from poorly differentiated surface epithelial carcinomas, carcinoid tumors and rare endometrial stromal tumours, especially focusing on nuclear features. Tumours of epithelial origin can be easily misdiagnosed in particular if strong keratin positivity (especially diffuse cytoplasmic positivity) is present.
    • Ovarian granulosa cell proliferations of pregnancy should be considered. This is a very important diagnostic difference.

    Prognosis

    • Adult: Indolent, with a prognosis similar to age-matched controls. Metastases (pelvic or upper abdominal) can occur, particularly with intraoperative tumour rupture. Recurrence can occur more than 10-20 years after initial surgery.
    • Juvenile: Favourable prognosis, especially when confined to the ovary at diagnosis.

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    Description

    Test your knowledge on granulosa cell tumors with this quiz. It covers diagnostic age ranges, gross appearances, microscopic features, and associated conditions. Perfect for students and professionals in the field of oncology and pathology.

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