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Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Yembur Ahmad
This case talks about a 60-year-old female undergoing heart valve replacement.
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1. Question
Clinical history: A 60 year-old female undergoing heart valve replacement
Choose the correct diagnosis:
Correct
Diagnosis: D. Papillary fibroelastoma
Histology: The lesion has a papillary architecture characterized by delicate, arborizing fronds. The papillary fronds are hyalinized and acellular; stromal fibroblasts or capillaries are not apparent. The papillary fronds are lined by a single layer of bland spindle cells. There is no acute or chronic inflammation.
Discussion: This is a beautiful example of a papillary fibroelastoma, a benign lesion that arises on the cardiac valves. They are characterized by avascular, fibroelastic papillary fronds with delicate branching. The fronds are lined by bland endocardium. A subset of papillary fibroelastomas have mutations in KRAS, suggesting at least a subset are neoplastic. The differential diagnosis includes Lambl’s excrescences, which have the histologic similarity of avascular hyalinized-appearing fronds lined by endocardium; however, Lambl’s excrescences are not papillary and do not arborize. The primary clinical risk with papillary fibroelastoma is the risk of tumor emboli and stroke.
Reference:
Bois MC, Milosevic D, Kipp BR, Maleszewski JJ. KRAS Mutations in Papillary Fibroelastomas: A Study of 50 Cases With Etiologic and Diagnostic Implications. Am J Surg Pathol. 2020 May;44(5):626-632.Incorrect
Diagnosis: D. Papillary fibroelastoma
Histology: The lesion has a papillary architecture characterized by delicate, arborizing fronds. The papillary fronds are hyalinized and acellular; stromal fibroblasts or capillaries are not apparent. The papillary fronds are lined by a single layer of bland spindle cells. There is no acute or chronic inflammation.
Discussion: This is a beautiful example of a papillary fibroelastoma, a benign lesion that arises on the cardiac valves. They are characterized by avascular, fibroelastic papillary fronds with delicate branching. The fronds are lined by bland endocardium. A subset of papillary fibroelastomas have mutations in KRAS, suggesting at least a subset are neoplastic. The differential diagnosis includes Lambl’s excrescences, which have the histologic similarity of avascular hyalinized-appearing fronds lined by endocardium; however, Lambl’s excrescences are not papillary and do not arborize. The primary clinical risk with papillary fibroelastoma is the risk of tumor emboli and stroke.
Reference:
Bois MC, Milosevic D, Kipp BR, Maleszewski JJ. KRAS Mutations in Papillary Fibroelastomas: A Study of 50 Cases With Etiologic and Diagnostic Implications. Am J Surg Pathol. 2020 May;44(5):626-632.
