A patient with Fanconi's anemia is at increased risk for developing a malignancy.

Pathogenesis: inability to repair DNA interstrand cross-links (defect in DNA repair)


General features:

(1) onset at an earlier age than usual

(2) suboptimal response to chemotherapy


Malignancies associated with Fanconi's anemia include:

(1) myelodysplastic syndrome and acute myelogenous leukemia (AML)

(2) head and neck squamous cell carcinoma, especially in the oral cavity

(3) nonmelanoma skin cancer

(4) cancer of the genitourinary tract

(5) liver tumors

(6) esophageal cancer

(7) pancreatic cancer

(8) breast cancer

(9) lung cancer

(10) gastric cancer

(11) anal carcinoma

(12) brain tumor

(13) other (retinoblastoma, osteogenic sarcoma, Wilms tumor, malignant lymphoma, etc)


Factors associated with an increased risk of malignancy:

(1) hematopoietic stem cell transplant (HSCT)

(2) androgen therapy for bone marrow failure (liver tumor)

(3) exposure to HPV

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