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. Individuals with DICER1 syndrome are at increased risk for developing various tumors, including papillary thyroid cancer. This is due to the role of DICER1 gene in regulating cell growth and division. The other choices, B (medullary thyroid cancer), C (pheochromocytoma), and D (renal cell carcinoma), are not typically associated with DICER1 syndrome. Medullary thyroid cancer is commonly linked to RET gene mutations, pheochromocytoma to genes such as SDHB, SDHD, and VHL, and renal cell carcinoma to mutations in genes like VHL and MET.