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Presented by William Westra, M.D. and prepared by Ali Ansari-Lari, M.D.,Ph.D.
Case 3: Clinical history: 42 year-old man with an intranasal mass
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1. Question
Week 128: Case 3
Clinical history: 42 year-old man with an intranasal massimages/Bill3a.JPG
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images/Bill3e.JPGCorrect
Answer: Hemangiopericytoma
Histology: The submucosa is infiltrated by a sheet-like proliferation of tightly-packed tumor cells. The cells are oval to slightly spindled; and they have bland isomorphic nuclei. Mitotic figures are not present. The tumor is highly vascularized. A CD34 stain (not shown) highlights the presence of numerous thin-walled vascular channels that are compressed by the surrounding CD34 negative tumor cells.
Discussion: Hemangiopericytoma (HPC) of the sinonasal cavity is a tumor that is believed to arise from the pericyte. Usually considered a tumor of the deep soft tissues, but about 20% arise in the head and neck region. Of these, most occur in the sinonasal tract.
The differential diagnosis of sinonasal HPC is as listed above. Angiofibroma is easily excluded by the clinical history and morphologic findings. Specifically, angiofibromas typically involve the nasopharynx of young men (quite rare after the age of 25), and demonstrates a more fibrous rich stroma. Solitary fibrous tumor of the sinonasal cavity demonstrates significant morphologic overlap with HPC. Both tumors are characterized by tightly-packed spindled cells with a prominent “HPC-like” vascular pattern. In the present case, the absence of intervening collagen, the uniform cellularity, and negative CD34 staining of the tumor cells all support the diagnosis of HPC. Glomus tumors also may show considerable overlap with HPC both morphologically and immunohistochemically (both tumors may be actin positive). Careful attention to growth pattern may provide some helpful clues. In contrast to the more haphazard arrangement of tumor cells in HPCs, glomus tumors are characterized by a concentric organoid arrangement of tumor cells around blood vessels.
The outdated term “HPC-like” tumor of the sinonasal cavity draws attention to some notable differences between HPC of the sinonasal tract and its soft tissue counterpart. Most importantly, HPCs of the sinonasal tract have a more predictably benign clinical course. In the absence of a high mitotic rate, these tumors do not metastasize and local excision appears to be adequate treatment.
Incorrect
Answer: Hemangiopericytoma
Histology: The submucosa is infiltrated by a sheet-like proliferation of tightly-packed tumor cells. The cells are oval to slightly spindled; and they have bland isomorphic nuclei. Mitotic figures are not present. The tumor is highly vascularized. A CD34 stain (not shown) highlights the presence of numerous thin-walled vascular channels that are compressed by the surrounding CD34 negative tumor cells.
Discussion: Hemangiopericytoma (HPC) of the sinonasal cavity is a tumor that is believed to arise from the pericyte. Usually considered a tumor of the deep soft tissues, but about 20% arise in the head and neck region. Of these, most occur in the sinonasal tract.
The differential diagnosis of sinonasal HPC is as listed above. Angiofibroma is easily excluded by the clinical history and morphologic findings. Specifically, angiofibromas typically involve the nasopharynx of young men (quite rare after the age of 25), and demonstrates a more fibrous rich stroma. Solitary fibrous tumor of the sinonasal cavity demonstrates significant morphologic overlap with HPC. Both tumors are characterized by tightly-packed spindled cells with a prominent “HPC-like” vascular pattern. In the present case, the absence of intervening collagen, the uniform cellularity, and negative CD34 staining of the tumor cells all support the diagnosis of HPC. Glomus tumors also may show considerable overlap with HPC both morphologically and immunohistochemically (both tumors may be actin positive). Careful attention to growth pattern may provide some helpful clues. In contrast to the more haphazard arrangement of tumor cells in HPCs, glomus tumors are characterized by a concentric organoid arrangement of tumor cells around blood vessels.
The outdated term “HPC-like” tumor of the sinonasal cavity draws attention to some notable differences between HPC of the sinonasal tract and its soft tissue counterpart. Most importantly, HPCs of the sinonasal tract have a more predictably benign clinical course. In the absence of a high mitotic rate, these tumors do not metastasize and local excision appears to be adequate treatment.
