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Strahlentherapie und Onkologie

, Volume 193, Issue 2, pp 156–161 | Cite as

Vertically transmitted HPV-dependent squamous cell carcinoma of the external auditory canal

Case report of a child
  • Miroslaw Snietura
  • Liliana Chelmecka-Wiktorczyk
  • Slawomir Pakulo
  • Agnieszka Kopec
  • Wojciech Piglowski
  • Grazyna Drabik
  • Bogdan Kosowski
  • Lukasz Wyrobek
  • Agata Stanek-Widera
  • Walentyna Balwierz
Case Study

Abstract

Background

There is much evidence that high-risk human papillomavirus (HPV) plays a causative role in a subset of head and neck squamous cell cancer (HNSCC) in adults. HPV-positive tumors behave differently even in their response to treatment and are therefore a distinct subset. Both HPV-positive and HPV-negative tumors of the head and neck region are usually in the domain of adults and cases in children are rare; thus when a 2‑year-old child was diagnosed with this cancer in the external auditory canal, an in-depth assessment of the tumor was considered necessary.

Case report

A 2‑year-old girl was born to a HPV-positive mother who was diagnosed with cervical cancer during pregnancy. The child was delivered by caesarean section and the mother died of her cancer 7 months after delivery. After the diagnosis of locally invasive HPV-positive squamous cell cancer of the external auditory canal, the child was treated surgically, and with chemotherapy and radiotherapy. Full remission was obtained lasting up to 325 weeks since treatment was started, resulting in over 6 years of disease-free survival.

Conclusion

This is the first case of advanced, HPV-related HNSCC in a 2‑year-old child, in whom the tumor was located in the external auditory canal and who made a dramatic recovery after treatment with nonradical surgery, chemotherapy and radiotherapy. The child has currently been disease free for 6 years. This case supports the observation that HPV-related HNSCC tumors appear to respond favorably to treatment despite the patient’s young age and the clinically advanced stage of the tumor.

Keywords

Human papillomavirus Ear canal Vertical infection transmission Chemoradiotherapy Pediatrics 

HPV-abhängiges Plattenepithelkarzinom des äußeren Gehörgangs nach vertikaler Virusübertragung

Fallbericht eines Kindes

Zusammenfassung

Hintergrund

Es gibt reichlich Belege dafür, dass das menschliche Papillomavirus vom Hochrisikotyp (HR-HPV) in einigen Fällen von Plattenepithelkarzinomen des Kopf- und Halsbereichs (HNSCC) eine Schlüsselrolle spielt. HPV-positive Tumoren verhalten sich anders, auch bezüglich des Ansprechens auf die Behandlung. Deswegen stellen sie eine separate biologische Gruppe dar. Sowohl HPV-positive als auch HPV-negative Tumoren des Kopf- und Halsbereichs treten vor allem bei Erwachsenen auf und sind bei Kindern eine Seltenheit. Daher begründete die Diagnose eines Plattenepithelkarzinoms des äußeren Gehörgangs bei einem 2‑jährigen Kind die Notwendigkeit, die Krankheit eingehend zu untersuchen und zu beschreiben.

Fallbericht

Wir berichten über ein 2‑jähriges Mädchen, das von einer HPV-positiven Mutter geboren wurde, bei der während der Schwangerschaft Gebärmutterhalskrebs diagnostiziert worden war. Die Geburt erfolgte per Kaiserschnitt. Die Mutter erlag 7 Monate nach der Geburt ihrer Krebserkrankung. Das Kind wurde nach der Diagnose eines lokal fortgeschrittenen HPV-positiven Plattenepithelkarzinoms des äußeren Gehörgangs operativ und ergänzend mit Radio- und Chemotherapie behandelt. Erreicht wurde eine komplette Remission für mindestens 325 Wochen nach Beginn der Behandlung, sodass mehr als 6 Jahre keine Krankheitssymptome aufgetreten sind.

Schlussfolgerung

Dies ist die erste Fallbeschreibung eines stark fortgeschrittenen HR-HPV-positiven HNSCC des äußeren Gehörgangs bei einem 2‑jährigen Kind, bei dem nach Anwendung einer nichtradikalen chirurgischen Behandlung sowie einer Chemo- und Strahlentherapie eine spektakuläre Reaktion und ein aktuell 6‑jähriges krankheitsfreies Überleben erreicht wurden. Dieser Fall bestätigt die Beobachtungen, nach denen HNSCC-Tumoren im Zusammenhang mit HR-HPV günstig auf die Behandlung ansprechen – trotz des klinisch fortgeschrittenen Tumorstadiums und des jungen Patientenalters.

Schlüsselwörter

Humanes Papillomavirus Äußerer Gehörgang Vertikale Infektionsübertragung Chemoradiotherapie Pädiatrie 

Notes

Acknowledgements

We are grateful to Dr Donald Roberts, Associate Professor, (Sahlgrenska University Hospital, Gothenburg, Sweden) and Dr Tomasz Rutkowski, Associate Professor, (I Radiation and Clinical Oncology Department, Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, Gliwice Branch) for scientific comments and editorial support.

Compliance with ethical guidelines

Conflict of interest

M. Snietura, L. Chelmecka-Wiktorczyk, Sr. Pakulo, A. Kopec, W. Piglowski, G. Drabik, B. Kosowski, L. Wyrobek, A. Stanek-Widera and W. Balwierz state that they have no competing interest.

This article does not contain any studies with human participants or animals performed by any of the authors.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Miroslaw Snietura
    • 1
  • Liliana Chelmecka-Wiktorczyk
    • 2
  • Slawomir Pakulo
    • 1
  • Agnieszka Kopec
    • 1
  • Wojciech Piglowski
    • 1
  • Grazyna Drabik
    • 3
  • Bogdan Kosowski
    • 4
  • Lukasz Wyrobek
    • 5
  • Agata Stanek-Widera
    • 1
  • Walentyna Balwierz
    • 2
    • 6
  1. 1.Tumor Pathology DepartmentMaria Sklodowska-Curie Memorial Cancer Center and Institute of OncologyGliwicePoland
  2. 2.Department of Oncology and HematologyUniversity Children’s Hospital of CracowCracowPoland
  3. 3.Pathology DepartmentUniversity Children’s Hospital of CracowCracowPoland
  4. 4.NZOZ ProsmedCracowPoland
  5. 5.Department of RadiologyUniversity Children’s Hospital of CracowCracowPoland
  6. 6.Clinic of Oncology and Hematology, Polish-American Pediatric InstituteJagiellonian University in CracowCracowPoland

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