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Presented by Dr. Ashley Cimino-Mmathews and prepared by Dr. Kevan Salimian
A 60 year-old female with a breast mass
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Question 1 of 1
1. Question
A 60 year-old female with a breast mass
Choose the correct diagnosis:
Correct
Diagnosis E. Myofibroblastoma
Histology:
The excisional biopsy reveals bland epithelioid cells within an associated dense, eosinophilic collagenous stroma with some admixed adipose tissue. The cells are variably nested or arranged as single cells in cords. The nuclei are hypochromatic with punctate nucleoli. The cells have indistinct cell borders and amphophilic cytoplasm. The lesional cells do not infiltrate benign terminal duct lobular units. The cells are positive for CD34 and ER, and are negative for: AE1/AE3, Cam5.2, CK903, S100 and SMADiscussion:
The morphology and immunoprofile are in keeping with a myofibroblastoma, which is a neoplastic proliferation of the breast stromal myofibroblasts. Classic-type myofibroblastomas are usually well-circumscribed, non-infiltrative nodules comprised of variable spindled to epithelioid cells with bland oval nuclei and amphophilic cytoplasm, arranged in nests with associated brightly eosinophilic collagen. There are multiple variant patterns, including epithelioid, myxoid, lipomatous, cellular, and collagenous/fibrous myofibroblastoma. Thus, the differential diagnosis varies with the histologic pattern, but in general the differential includes other spindle cell lesions such as spindle cell metaplastic carcinoma, phyllodes tumor, and fibromatosis. Notably, epithelioid myofibroblastomas can mimic infiltrating lobular carcinomas, which is especially treacherous since myofibroblastomas are typically strongly immunoreactive for ER. Hence, in this setting, a cytokeratin and CD34 immunostains should be included in the immunopanel to resolve the diagnosis. Mammary myofibroblastoma display similar genetic changes as spindle cell lipomas and mammary-type myofibroblastomas encountered elsewhere; specifically, they display recurrent loss or rearrangements of chromosomes 16q and 13q (encompassing the Rb gene).References
1. Maggiani F, Debiec-Rychter M, Verbeeck G, et al. Extramammary myofibroblastoma is genetically related to spindle cell lipoma. Virchows Arch. 2006;449:244–247.
2. Howitt BE, Fletcher CDM. Mammary-type Myofibroblastoma: Clinicopathologic Characterization in a Series of 143 Cases. Am. J. Surg. Pathol. 2016;40:361–367.
3. Charu V, Cimino-Mathews A. Spindle cell lesions of the breast. Am J Surg Pathol: Reviews & Reports. 2017 Mar-Apr;22(2):116-124.Incorrect
Diagnosis E. Myofibroblastoma
Histology:
The excisional biopsy reveals bland epithelioid cells within an associated dense, eosinophilic collagenous stroma with some admixed adipose tissue. The cells are variably nested or arranged as single cells in cords. The nuclei are hypochromatic with punctate nucleoli. The cells have indistinct cell borders and amphophilic cytoplasm. The lesional cells do not infiltrate benign terminal duct lobular units. The cells are positive for CD34 and ER, and are negative for: AE1/AE3, Cam5.2, CK903, S100 and SMADiscussion:
The morphology and immunoprofile are in keeping with a myofibroblastoma, which is a neoplastic proliferation of the breast stromal myofibroblasts. Classic-type myofibroblastomas are usually well-circumscribed, non-infiltrative nodules comprised of variable spindled to epithelioid cells with bland oval nuclei and amphophilic cytoplasm, arranged in nests with associated brightly eosinophilic collagen. There are multiple variant patterns, including epithelioid, myxoid, lipomatous, cellular, and collagenous/fibrous myofibroblastoma. Thus, the differential diagnosis varies with the histologic pattern, but in general the differential includes other spindle cell lesions such as spindle cell metaplastic carcinoma, phyllodes tumor, and fibromatosis. Notably, epithelioid myofibroblastomas can mimic infiltrating lobular carcinomas, which is especially treacherous since myofibroblastomas are typically strongly immunoreactive for ER. Hence, in this setting, a cytokeratin and CD34 immunostains should be included in the immunopanel to resolve the diagnosis. Mammary myofibroblastoma display similar genetic changes as spindle cell lipomas and mammary-type myofibroblastomas encountered elsewhere; specifically, they display recurrent loss or rearrangements of chromosomes 16q and 13q (encompassing the Rb gene).References
1. Maggiani F, Debiec-Rychter M, Verbeeck G, et al. Extramammary myofibroblastoma is genetically related to spindle cell lipoma. Virchows Arch. 2006;449:244–247.
2. Howitt BE, Fletcher CDM. Mammary-type Myofibroblastoma: Clinicopathologic Characterization in a Series of 143 Cases. Am. J. Surg. Pathol. 2016;40:361–367.
3. Charu V, Cimino-Mathews A. Spindle cell lesions of the breast. Am J Surg Pathol: Reviews & Reports. 2017 Mar-Apr;22(2):116-124.
