Anatomy of Hematologic System Related

Review Anatomy of Hematologic System related questions and content

A 10-year-old girl is a long-term survivor of type II pleuropulmonary blastoma (PPB). You suspect she has a cancer predisposition syndrome and perform genetic testing, which confirms she has DICER1 syndrome. Which other cancer is she predisposed to?

  • A. Papillary thyroid cancer
  • B. Medullary thyroid cancer
  • C. Pheochromocytoma
  • D. Renal cell carcinoma
Correct Answer: A

Rationale: The correct answer is A: Papillary thyroid cancer. DICER1 syndrome is associated with an increased risk of developing various tumors, including papillary thyroid cancer. This is due to the role of DICER1 gene mutation in dysregulating cell growth and division. Other choices like B: Medullary thyroid cancer, C: Pheochromocytoma, and D: Renal cell carcinoma are not typically associated with DICER1 syndrome. Medullary thyroid cancer is more commonly linked to RET gene mutations, pheochromocytoma to RET or SDHB/C/D gene mutations, and renal cell carcinoma to VHL gene mutations. Therefore, based on the genetic mechanism and known associations, papillary thyroid cancer is the most likely cancer this patient is predisposed to.