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USMLE · Question #608

A 3-year-old male is brought to the physician due to an enlarging abdominal mass. Physical examination reveals a mass in the left abdomen that does not cross the midline. Ophthalmic examination is…

The correct answer is E. Wilms tumor. Wilms tumor is the most common renal malignancy of early childhood (ages 2-4) that can present as an abdominal mass with hypertension, nausea, hematuria, and intestinal obstruction. Histologically, it is a neoplasm of the embryonic renal tissue that consists of both neoplastic…

Abnormal Processes

Question

A 3-year-old male is brought to the physician due to an enlarging abdominal mass. Physical examination reveals a mass in the left abdomen that does not cross the midline. Ophthalmic examination is unremarkable. Microscopic examination of a biopsy specimen from the abdomen reveals dense immature islands of epithelial cells, ribbons of spindle fibroblastic stromal cells and poorly formed tubular structures. The most likely diagnosis:

Options

  • Aembryonal rhabdomyosarcoma
  • BEwing sarcoma
  • CHodgkin disease
  • Dneuroblastoma
  • EWilms tumor

How the community answered

(15 responses)
  • B
    7% (1)
  • C
    7% (1)
  • D
    13% (2)
  • E
    73% (11)

Explanation

Wilms tumor is the most common renal malignancy of early childhood (ages 2-4) that can present as an abdominal mass with hypertension, nausea, hematuria, and intestinal obstruction. Histologically, it is a neoplasm of the embryonic renal tissue that consists of both neoplastic epithelia and a stromal component. It is associated with mutations of the tumor suppressor genes WT1 or WT2 on chromosome 11. A close differential diagnosis for this child is a neuroblastoma but it is an extrarenal tumor which crosses the midline. Wilms tumor may also be associated with several syndromes such as the aniridia, genitourinary malformations and mental/motor retardation syndrome (WAGR complex), Denys-Drash syndrome and Beckwith-Wiedemann

Topics

#Wilms tumor#nephroblastoma#pediatric oncology#triphasic histology

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