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Intensity-modulated radiotherapy for localized nasopharyngeal amyloidosis

Case report and literature review

Intensitätsmodulierte Strahlentherapie bei lokalisierter nasopharyngealer Amyloidose

Kasuistik und Review der Literatur

  • Case Study
  • Published:
Strahlentherapie und Onkologie Aims and scope Submit manuscript

Abstract

Background

Primary localized amyloidosis is characterized by the deposition of amyloid proteins restricted to one organ, without systemic involvement. Primary nasopharyngeal amyloidosis is an exceedingly rare condition, for which the standard treatment remains unknown. Because of its challenging anatomical position, surgery alone hardly results in complete resection of the localized amyloidosis. Therefore, an interdisciplinary planning board to design optimal treatment is of particular importance.

Patient and methods

A 39-year-old man presented with a several-week history of nasal obstruction and epistaxis. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed the presence of a retro-odontoid nonenhancing soft tissue mass.

Results

The endoscopic biopsy demonstrated that the mass was amyloid in nature. An extensive systemic workup revealed an absence of inflammatory process, systemic amyloidosis, or plasma cell dyscrasia. The patient was treated with a combination of surgery and radiotherapy, showing no evidence of recurrence or progression at his 1‑year follow-up.

Conclusion

Primary solitary amyloidosis is a rare form of amyloidosis. To the best of our knowledge, this is the first report of a nasopharyngeal amyloidosis case treated with excision and radiation leading to complete remission. Because of the difficulty for surgeons to achieve radical resection with such lesions, radiotherapy proved to be an excellent adjuvant treatment in this case.

Zusammenfassung

Hintergrund

Die primäre lokalisierte Amyloidose ist durch die Ablagerung von Amyloidproteinen gekennzeichnet, die sich auf ein Organ beschränkt, also nicht systemisch ist. Eine primäre Amyloidose im Nasen-Rachen-Raum ist außerordentlich selten, bisher gibt es keine Standardtherapie. Ihre anatomische Position bedeutet eine Herausforderung, nur selten resultiert eine chirurgische Intervention in einer vollständigen Resektion der lokalisierten Amyloidose. Daher ist die Beteiligung mehrerer Disziplinen für eine optimale Behandlung von besonderer Bedeutung.

Patient und Methoden

Bei einem 39-jährigen Patienten mit seit einigen Wochen bestehenden Symptomen nasaler Obstruktion und Nasenbluten zeigten Computer- (CT) und Magnetresonanztomographie (MRT) einen hinter dem Dens liegenden Weichteiltumor ohne Enhancement.

Ergebnisse

Die endoskopisch gewonnene Biopsie zeigte, dass es sich bei dem Tumor um eine Amyloidose handelte. Eine umfassende Diagnostik ergab keine entzündlichen Prozesse, keine systematische Amyloidose und keine Plasmazell-Dyskrasie. Der Patient wurde kombiniert chirurgisch und strahlentherapeutisch behandelt. In der 1‑Jahr-Follow-up-Untersuchung fand sich kein Hinweis für ein Rezidiv bzw. einen Progress.

Fazit

Die primäre solitäre Amyloidose ist eine seltene Amyloidoseform. Unseres Wissens ist dies der erste Bericht eines Patienten mit nasopharnygealer Amyloidose, der kombiniert chirurgisch und strahlentherapeutisch behandelt wurde und bei dem eine vollständige Remission erzielt wurde. Wegen der schwierig zu erreichenden radikalen Resektion bei Läsionen in diesem Bereich hat sich die Strahlentherapie für den vorgestellten Patienten als ausgezeichnete adjuvante Behandlungsoption erwiesen.

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Authors and Affiliations

  1. Department of Clinical Oncology, Taihe Hospital, Hubei University of Medicine, 442000, Shiyan, Hubei, China

    Ming Luo

  2. Cancer Center, Union Hospital, Tongji Medical College, HuaZhong University of Science and Technology, 430022, Wuhan, China

    Gang Peng, Liangliang Shi, Zhenyu Li, Shijiang Fei, Qian Ding & Jing Cheng M.D. Ph.D.

  3. Department of infection control and prevention, Taihe Hospital, Hubei University of Medicine, 442000, Shiyan, Hubei, China

    Xing Ming

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  1. Ming Luo
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Correspondence to Jing Cheng M.D. Ph.D..

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Conflict of interest

M. Luo, G. Peng, L. Shi, X. Ming, Z. Li, S. Fei, Q. Ding, and J. Jing state that there are no conflicts of interest.

The accompanying manuscript does not include studies on humans or animals.

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Ming Luo and Gang Peng contributed equally to this work and should be considered co-first authors

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Luo, M., Peng, G., Shi, L. et al. Intensity-modulated radiotherapy for localized nasopharyngeal amyloidosis. Strahlenther Onkol 192, 944–950 (2016). https://doi.org/10.1007/s00066-016-0996-6

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