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Presented by Pedram Argani, M.D. and prepared by Doreen Nguyen, M.D.
Case 2: This is a 35 year-old female with a large infiltrative ethmoid mass.
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Question 1 of 1
1. Question
Week 605: Case 2
This is a 35 year-old female with a large infiltrative ethmoid mass.
Correct
Answer: NUT midline carcinoma
Histology: This is highly cellular malignant small round blue cell tumor. Mitotic figures are readily evident. Focally, one sees the abrupt squamous differentiation with basaloid cells rapidly transforming into fairly well differentiated keratinized epithelium. These are the typical features of NUT midline carcinoma. The lesion was diffusely immunoreactive for cytokeratin and p63, and demonstrated diffuse nuclear labeling for NUT protein by immunohistochemistry.
Discussion: High grade olfactory neuroblastoma is essentially a high grade malignant small round cell tumor. It would not be expected to demonstrate squamous differentiation as the current lesion does. It would also label for neuroendocrine markers. Melanoma of the sinonasal tract may have a small cell appearance; however, it should not demonstrate focally squamous differentiation and would label for melanocytic markers, not epithelial markers. HPV-associated squamous cell carcinomas are frequently basaloid and demonstrate abrupt keratinization similar to that seen in the current case. However, the ethmoid is not a typical area for HPV-associated squamous cell carcinoma. The latter is excluded by absence of HPV DNA sequences by in situ hybridization, and by the labeling for NUT protein.
NUT midline carcinomas are highly aggressive lesions, with a mean survival in the range of 1 year. These tumors are all defined by chromosome translocation resulting in gene fusions involving the nuclear protein of testis (NUT) gene, which is located at chromosome 15q24.
Reference(s)
– Stelow E. Head and Neck Pathology 2011; 5:31-35.Incorrect
Answer: NUT midline carcinoma
Histology: This is highly cellular malignant small round blue cell tumor. Mitotic figures are readily evident. Focally, one sees the abrupt squamous differentiation with basaloid cells rapidly transforming into fairly well differentiated keratinized epithelium. These are the typical features of NUT midline carcinoma. The lesion was diffusely immunoreactive for cytokeratin and p63, and demonstrated diffuse nuclear labeling for NUT protein by immunohistochemistry.
Discussion: High grade olfactory neuroblastoma is essentially a high grade malignant small round cell tumor. It would not be expected to demonstrate squamous differentiation as the current lesion does. It would also label for neuroendocrine markers. Melanoma of the sinonasal tract may have a small cell appearance; however, it should not demonstrate focally squamous differentiation and would label for melanocytic markers, not epithelial markers. HPV-associated squamous cell carcinomas are frequently basaloid and demonstrate abrupt keratinization similar to that seen in the current case. However, the ethmoid is not a typical area for HPV-associated squamous cell carcinoma. The latter is excluded by absence of HPV DNA sequences by in situ hybridization, and by the labeling for NUT protein.
NUT midline carcinomas are highly aggressive lesions, with a mean survival in the range of 1 year. These tumors are all defined by chromosome translocation resulting in gene fusions involving the nuclear protein of testis (NUT) gene, which is located at chromosome 15q24.
Reference(s)
– Stelow E. Head and Neck Pathology 2011; 5:31-35.
