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Textbook of Head and Neck Pathology pp 127–165Cite as

Nasopharynx and Skull Base

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Abstract

How many times have the terms “sinonasal” and “nasopharyngeal” been incorrectly interchanged on requisition sheets? This chapter offers more examples that emphasize the importance of anatomic and clinicoradiographic correlation in arriving at a correct diagnosis. A “hairy polyp” may appear quite unimpressive until you realize it’s not a dermal lesion. A biopsy from an ectopic pituitary adenoma or macroadenoma may lead you down the incorrect path of sinonasal neuroendocrine carcinoma, a biopsy from a craniopharyngioma may have you thinking about an ameloblastoma, an ecchordosis physaliphora can mimic chordoma. In this cost conscious era where pathologists need be more time efficient, and use fewer immunohistochemical resources, less anatomic and clinical information is not an option.

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Authors and Affiliations

  1. Erie County Medical Center, State University of New York at Buffalo, Buffalo, NY, USA

    Margaret S. Brandwein

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  1. Margaret S. Brandwein
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Appendices

Self Study

  1. 1.

    Which statement regarding Fig. 5.27 is most likely to be correct?

    Fig. 5.27
    figure 27

    Self study

    Full size image
    1. (a)

      This is a nasopharyngeal biopsy from a young Chinese American woman. Skullbase CT and MR should be performed.

    2. (b)

      This is a nasopharyngeal biopsy from a young American Caucasian woman, status post lung transplant for cystic fibrosis. The next step would involve decreasing her immunosuppression.

    3. (c)

      This is a nasopharyngeal biopsy from a 15-year old African American male with obstructive sleep apnea.

    4. (d)

      This is a nasopharyngeal biopsy from a 15-year old Caucasian American female recently diagnosed with classical Hodgkin’s disease. Where is Thomas Hodgkin buried?

  2. 2.

    Which statement regarding Fig. 5.28 is most likely to be correct?

    Fig. 5.28
    figure 28

    Self study

    Full size image
    1. (a)

      This is a nasopharyngeal biopsy from a 15-year old Caucasian American male; IHC is for TTF-1.

    2. (b)

      This is a nasopharyngeal biopsy from a 15-year old male who complained of epistaxis and nasal obstruction. Holman-Miller sign is present radiographically.

    3. (c)

      This is a nasopharyngeal biopsy from a 50-year old male Caucasian American carpenter; serum EBV DNA is elevated.

    4. (d)

      This is a nasopharyngeal biopsy from a 70-year old Chinese male; IHC is for Cam 5.2

  3. 3.

    Which statement regarding Fig. 5.29 is most likely to be correct?

    Fig. 5.29
    figure 29

    Self study

    Full size image
    1. (a)

      CTNNB1 mutation and aberrant β-catenin expression are common.

    2. (b)

      “Wet keratin” and calcifications distinguished one variant, which commonly harbor the BRAF V600E mutation.

    3. (c)

      This tumor arises from Tornwaldt’s bursa.

    4. (d)

      Standard of care consists of primary radiotherapy.

Answers

  1. 1.

    Which statement regarding Fig. 5.27 is most likely to be correct?

    This is a nonkeratinizing nasopharyngeal carcinoma (NK-NPC) (left). The right image is of ISH for EBER.

    1. (a)

      This is a nasopharyngeal biopsy from a young Chinese American woman. Skullbase CT and MR should be performed. Yes. The carcinoma cells (left) have an immunoblast-like appearance. The relatively sharp demarcation from adjacent lymphocytes, on light microscopy and EBER ISH (right) speaks for their epithelial origin. The incidence of NPC in first generation Chinese Americans is less than in endemic regions, yet higher than Caucasian Americans.

    2. (b)

      This is a nasopharyngeal biopsy from a young American Caucasian woman, status post lung transplant for cystic fibrosis. The next step would involve decreasing her immunosuppression. No – A lymphoproliferative process is ruled out by the above features.

    3. (c)

      This is a nasopharyngeal biopsy from a 15-year old African American male with obstructive sleep apnea. No – Normal adenoidal crypt epithelial cells have smaller nuclei and are more mature. Any EBER signals, if present, would be confined to a few lymphocytes.

    4. (d)

      This is a nasopharyngeal biopsy from a 15-year old Caucasian American female recently diagnosed with classical Hodgkin’s disease. Where is Thomas Hodgkin buried? Thomas Hodgkin, a pre-eminent pathologist, was also the personal physician of Sir Moses Montefiore. He died of dysentery while accompanying Montefiore on one of his many trips to Israel, and is buried in Jaffa.

  2. 2.

    Which statement regarding Fig. 5.28 is most likely to be correct?

    This is another NK-NPC, and the IHC is for keratin.

    1. (a)

      This is a nasopharyngeal biopsy from a 15-year old Caucasian American male; IHC is for TTF-1. No–Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma form predominantly exophytic tumors. TTF-1 will be expressed in nuclei; you might be able to appreciate that this image demonstrates cytoplasmic staining.

    2. (b)

      This is a nasopharyngeal biopsy from a 15-year old male who complained of epistaxis and nasal obstruction. Holman-Miller sign is present radiographically. No – The Holman-Miller sign (bowing of the posterior antral wall anteriorly) is pathogneumonic for nasopharyngeal angiofibroma.

    3. (c)

      This is a nasopharyngeal biopsy from a 50-year old male Caucasian American carpenter; serum EBV DNA is elevated. No – NPC in US Caucasians is more likely to be keratinizing and unrelated to EBV.

    4. (d)

      This is a nasopharyngeal biopsy from a 70-year old Chinese male; IHC is for Cam 5.2.–Yes

  3. 3.

    Which statement regarding Fig. 5.29 is most likely to be correct?

     This is an adamantinomatous craniopharyngioma, which is usually associated aberrant activation of the WNT/β-catenin pathway

    1. (a)

      CTNNB1 mutation and aberrant β-catenin expression are common. Yes

    2. (b)

      “Wet keratin” and calcifications distinguished one variant, which commonly harbor the BRAF V600E mutation. No. Wet keratin” and calcifications distinguish the adamantinomatous variant from the papillary variant; the latter commonly harbors the BRAF V600E mutation.

    3. (c)

      This tumor arises from Tornwaldt’s bursa. No. It arises along the migrational path of Rathke’s pouch.

    4. (d)

      Standard of care consists of primary radiotherapy. No. Adamantinomatous craniopharyngioma is associated with a significant rate of local recurrence. Standard of care is primary surgery (either endoscopic endonasal or stereotactic microscopic) with adjuvant radiotherapy for inadequate resections.

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Brandwein, M.S. (2016). Nasopharynx and Skull Base. In: Textbook of Head and Neck Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-33323-6_5

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