WBR0725

Author	[[PageAuthor::Rim Halaby, M.D. [1]]]
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, weight loss, and low grade fever. On physical examination, the physician notes bilateral nontender enlargement of the breasts with induced galactorrhea. The abdomen is distended and multi-lobulated masses are palpated. Fine-needle aspiration followed by excisional biopsy of an ovarian tumor are performed. Grossly, the tumor is a solid white-gray tumor with multilocular cystic appearance. Microscopic analysis shows Call-Exner bodies separated by thin fibrous septae. What is the most likely diagnosis in this patient?]]
Answer A	AnswerA::Granulosa cell tumor
Answer A Explanation	AnswerAExp::Granulosa cell tumor is an ovarian non-germ cell tumor that often secretes estrogen. It has a grey-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 seen in teratomas.
Answer C	AnswerC::Brenner tumor
Answer C Explanation	AnswerCExp::Brenner tumor is an benign ovarian non-germ cell tumor characterized by the presence of 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 is a rare estrogen-secreting sex cord stroma tumor that may present in young children with a clinical presentation of precious puberty or in adult female patients of reproductive age with a typical clinical presentation of abnormal uterine bleeding and predisposition of endometrial hyperplasia and endometrial cancer. Grossly, granulosa cell tumor appears as a multilocular mass in the ovaries and microscopically, Call-Exner bodies, which are follicular structures containing acidophilic material, are characteristic. Educational Objective: Granulosa cell tumor is an estrogen-secreting sex cord stroma tumor characterized by the clinical presentation of precocious puberty in young females the presence of Call-Exner bodies on microscopy. Reference: Mava Y, Chinda JY, Alhaji MA, Naggada HA. Childhood ovarian juvenile granulosa cell tumor: a case report and review of literature. Niger J Paed. 2012; 39(4):199-201. Educational Objective: References: ]]
Approved	Approved::No
Keyword	WBRKeyword::granulosa, WBRKeyword::cell, WBRKeyword::tumor, WBRKeyword::ovarian, WBRKeyword::germ, WBRKeyword::malignant, WBRKeyword::call-exner, WBRKeyword::Call-Exner, WBRKeyword::Call, WBRKeyword::Exner, WBRKeyword::call, WBRKeyword::exner, WBRKeyword::bodies, WBRKeyword::precocious, WBRKeyword::puberty, WBRKeyword::endometrial cancer, WBRKeyword::hyperplasia, WBRKeyword::breast, WBRKeyword::enlargement, WBRKeyword::estrogen
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