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Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen
Adult male with a paratesticular mass
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Correct: D
Histology: Sections show a proliferation of spindle cells with small nuclei and hyperchromatic chromatin and indistinct cell borders. These cells have an infiltrative growth and involve adipose tissue, extending in fibrous septae.
Discussion: Diffuse neurofibromas are more common in the head and neck region of children and young adults but can occur anywhere. A subset of them is associated with neurofibromatosis syndrome but can also occur sporadically. As its name implies, this neurofibroma is poorly defined and spreads extensively along connective tissue septae and between fat cells. It differs from conventional neurofibroma in that it has a uniform matrix of fine fibrillary collagen. Cellularity is usually low. The tumor shows occasional pseudomeissnerian body-like structures. Entrapped large vessels are characteristic. Extremely rarely, diffuse neurofibromas may progress to malignant peripheral nerve sheath tumor. By immunohistochemistry, the tumor is diffusely positive for S100, SOX10 and CD34 and negative for MUC4 and MDM2.
References:
- Schaefer IM and Fletcher CD. Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases. Am J Surg Pathol. 2015;39(9):1234-1241.
Incorrect
Correct: D
Histology: Sections show a proliferation of spindle cells with small nuclei and hyperchromatic chromatin and indistinct cell borders. These cells have an infiltrative growth and involve adipose tissue, extending in fibrous septae.
Discussion: Diffuse neurofibromas are more common in the head and neck region of children and young adults but can occur anywhere. A subset of them is associated with neurofibromatosis syndrome but can also occur sporadically. As its name implies, this neurofibroma is poorly defined and spreads extensively along connective tissue septae and between fat cells. It differs from conventional neurofibroma in that it has a uniform matrix of fine fibrillary collagen. Cellularity is usually low. The tumor shows occasional pseudomeissnerian body-like structures. Entrapped large vessels are characteristic. Extremely rarely, diffuse neurofibromas may progress to malignant peripheral nerve sheath tumor. By immunohistochemistry, the tumor is diffusely positive for S100, SOX10 and CD34 and negative for MUC4 and MDM2.
References:
- Schaefer IM and Fletcher CD. Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases. Am J Surg Pathol. 2015;39(9):1234-1241.