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, weight loss, and low grade fever. On physical examination, the physician notes bilateral non-tender enlargement of the breasts with induced galactorrhea. Abdominal inspection reveals moderate distention. Deep palpation of the abdomen is remarkable for a multi-lobulated mass at the left lower quadrant. Following appropriate work-up, a large left ovarian mass is detected; and the patient is scheduled for resection. Grossly, the mass 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 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 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 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 is a rare estrogen-secreting sex cord stroma tumor that 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 abnormal uterine bleeding (AUB), predisposition of endometrial hyperplasia, and endometrial cancer. Grossly, granulosa cell tumor appears as a multilocular mass in the ovaries. 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 among young females and the presence of Call-Exner bodies on microscopy. References: 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.]]
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
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