WBR0725
| Author | [[PageAuthor::Yazan Daaboul, M.D. (Reviewed by Yazan Daaboul, M.D.)]] |
|---|---|
| Exam Type | ExamType::USMLE Step 1 |
| Main Category | MainCategory::Pathology |
| Sub Category | SubCategory::Genitourinary, SubCategory::Oncology |
| Prompt | [[Prompt::An 8-year-old girl is brought by her mother to the physician's office for progressive abdominal swelling and premature development of the breasts. On physical examination, the physician notes bilateral non-tender enlargement of the breasts. Abdominal inspection reveals moderate distention. Deep palpation of the abdomen is remarkable for a solid mass at the left lower quadrant. Following appropriate work-up, a large left ovarian mass is detected on CT scan. The patient is scheduled for surgical management. Resection of the tumor is performed, and the mass is sent to pathological analysis. Grossly, the mass is a solid white-gray tumor with a multilocular cystic appearance. Microscopic analysis shows Call-Exner bodies separated by thin fibrous septae. What is the most likely diagnosis of this patient's condition?]] |
| Answer A | AnswerA::Granulosa cell tumor |
| Answer A Explanation | AnswerAExp::Granulosa cell tumor is an ovarian non-germ cell tumor that often secretes estradiol. It has a gray-white multilocular appearance on gross pathology and characteristic Call-Exner bodies on microscopy. |
| Answer B | AnswerB::Teratoma |
| Answer B Explanation | AnswerBExp::Teratoma is the most common ovarian germ cell tumor. It is characterized by the presence of several germ layers. Call-Exner bodies are not typically observed in teratomas. |
| Answer C | AnswerC::Brenner tumor |
| Answer C Explanation | AnswerCExp::Brenner tumor is a benign ovarian non-germ cell tumor characterized by the presence of a pale yellow unilateral tumor on gross pathology and "coffee bean" nuclei on microscopy. |
| Answer D | AnswerD::Yolk sac tumor |
| Answer D Explanation | AnswerDExp::Yolk sac tumor is a malignant ovarian germ cell tumor. It is characterized by the presence of yellow friable mass on gross pathology and the presence of Schiller-Duval bodies that look like kidney glomeruli on microscopy. |
| Answer E | AnswerE::Dysgerminoma |
| Answer E Explanation | AnswerEExp::Dysgerminoma is a malignant ovarian germ cell cancer associated with Turner syndrome. Call-Exner bodies are not typically found in dysgerminoma. |
| Right Answer | RightAnswer::A |
| Explanation | [[Explanation::Granulosa cell tumor of the ovary is a rare estradiol-secreting sex cord stroma tumor. It may present among young children with a clinical presentation of precious puberty, or among adult women of reproductive age with a typical clinical presentation of menstrual irregularities, abnormal uterine bleeding (AUB), predisposition of endometrial hyperplasia, and endometrial cancer. Accordingly, granulosa cell tumors are classified into 2 subtypes: juvenile and adult. While most of granulosa cell tumors are unilateral, a bilateral presentation is observed in less than 5% of patients. The typical presentation includes signs of isosexual precocious pseudopuberty, such as premature breast development, presence of pubic hair, and vaginal bleeding, along with a palpable lower abdominal or pelvic mass on physical exam. Ruptured granulosa cell tumor is a complication of the disease, and patients classically present with acute abdominal pain and ascites.
Two important features of granulosa cell tumors include a long natural history of the tumor and a tendency to recur many years after the initial diagnosis. Diagnosis requires imaging, such as abdominopelvic CT scan. Management is first by surgery for resection, debulking, confirmation of histological diagnosis, and staging. Chemoradiation are also required in advanced disease. The stage of the disease is the most important prognostic factor of the disease, and is associated with the risk of recurrence. Theca cells are still important in the mechanism of estradiol-secretion in granulosa cell tumors. Theca cells are required for the production of the estradiol precursor, androstenedione. Because more than 30% of granulosa cell tumors lack theca cells, these tumors are non-secretory, making estradiol secretion a non-reliable element for either diagnosis or follow-up. Grossly, granulosa cell tumor appears as a white-gray solid mass with a possible hemorrhagic, fibrotic, multilocular, or cystic appearance. Five morphological patterns have been described: Microscopically, granulosa cell tumors may be moderately or well-differentiated. The histological appearance may be microfollicular, trabecular, solid tubular, diffuse, water-silk, or mixed. Call-Exner bodies, which are follicular structures containing acidophilic material, are characteristic. |
| Approved | Approved::Yes |
| Keyword | WBRKeyword::Granulosa, WBRKeyword::Cell, WBRKeyword::Tumor, WBRKeyword::Ovarian, WBRKeyword::Malignant, WBRKeyword::Call-exner, WBRKeyword::Call-Exner, WBRKeyword::Call, WBRKeyword::Exner, WBRKeyword::Precocious, WBRKeyword::Puberty, WBRKeyword::Endometrial cancer, WBRKeyword::Hyperplasia, WBRKeyword::Breast, WBRKeyword::Enlargement, WBRKeyword::Estrogen |
| Linked Question | Linked:: |
| Order in Linked Questions | LinkedOrder:: |