Zusammenfassung
Ein 87-jähriger Patient berichtete über eine knotige, größenprogrediente Struktur des anterioren Nasenseptums mit partieller Verlegung der Naseneingänge. In der Bildgebung sowie intraoperativ zeigte sich die Raumforderung lokal begrenzt, ohne Anzeichen der Infiltration von Subkutis, Knochen sowie der angrenzenden Sinus paranasales. Histologisch zeigte sich ein chondroider Tumor mit lobulärem Wachstum sowie physaliphorem Zytoplasma. Durch die immunhistochemische Untersuchung konnte bei Positivität für Brachyury und Negativität für ein EWSR1(Ewing-Sarcoma-Breakpoint-Region)-Rearrangement die Diagnose eines chondroiden Chordoms gestellt werden. Der vorliegende Fall belegt die differenzialdiagnostischen Überlegungen zu diesem Tumor, der per se selten ist, aber auch an untypischen extraaxialen Stellen lokalisiert sein kann. Nach Literaturrecherche wurde eine Übersicht aller von uns identifizierten 34 primären extraossären Chordome der Nase, des Nasopharynx und der paranasalen Sinus erstellt und der vorgestellte Fall in diese Übersicht eingeordnet.
Abstract
An 87-year-old patient reported a nodular, progressively enlarging mass of the anterior nasal septum leading to partial obstruction of the nostrils. The tumor showed no infiltration of the subcutis, bone, or paranasal sinuses in imaging or intraoperatively. Histological examination revealed a chondroid tumor with lobular growth and physaliferous cell morphology. Immunohistochemistry revealed a brachyury-positive tumor without EWSR1 rearrangement, leading to the diagnosis of a chondroid chordoma. The reported case demonstrates the differential diagnostic considerations pertaining to this rare tumor, which can also have an untypical and very rare extra-axial location. Review of the literature identified 34 primary extraosseous chordomas of the nose, nasopharynx, and paranasal sinuses, and allowed the nasal chordoma presented herein to be included in this group of extra-axial chordomas.
Literatur
Bergh P, Kindblom LG, Gunterberg B et al (2000) Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer 88:2122–2134
Bohman LE, Koch M, Bailey RL et al (2014) Skull base chordoma and chondrosarcoma: influence of clinical and demographic factors on prognosis: a SEER analysis. World Neurosurg 82:806–814
Bompas E, Le Cesne A, Tresch-Bruneel E et al (2015) Sorafenib in patients with locally advanced and metastatic chordomas: a phase II trial of the French sarcoma group (GSF/GETO). Ann Oncol 26:2168–2173
Casali PG, Bielack S, Abecassis N et al (2018) Bone sarcomas: ESMO-PaedCan-EURACAN clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 29:iv79–iv95
Chen H, Garbutt CC, Spentzos D et al (2017) Expression and therapeutic potential of SOX9 in Chordoma. Clin Cancer Res 23:5176–5186
Chugh R, Tawbi H, Lucas DR et al (2007) Chordoma: the nonsarcoma primary bone tumor. The Oncol 12:1344–1350
Czerniak B (2015) Chordoma and related lesions. In: Czerniak B (Hrsg) Bone tumors. Elsevier, Philadelphia, S 1174–1216
Delaney TF, Liebsch NJ, Pedlow FX et al (2014) Long-term results of phase II study of high dose photon/proton radiotherapy in the management of spine chordomas, chondrosarcomas, and other sarcomas. J Surg Oncol 110:115–122
Dwianingsih EK, Snak Y, Rinonce HT et al (2019) Primary Chordoma of the Nasopharynx: a rare case report and review of the literatures. Case Rep Pathol. Article ID: 3826521
Gröschel S, Hübschmann D, Raimondi F et al (2019) Defective homologous recombination DNA repair as therapeutic target in advanced chordoma. Nat Commun 10:1635
Lauer SR, Edgar MA, Gardner JM et al (2013) Soft tissue chordomas: a clinicopathologic analysis of 11 cases. Am J Surg Pathol 37:719–726
Lebellec L, Aubert S, Zaïri F et al (2015) Molecular targeted therapies in advanced or metastatic chordoma patients: facts and hypotheses. Crit Rev Oncol Hematol 95:125–131
Lv G‑H, Zou M‑X, Liu F‑S et al (2019) Clinicopathological and prognostic characteristics in extra-axial Chordomas: an integrative analysis of 86 cases and comparison with axial Chordomas. Neurosurgery 85:E527–E542
Magnaghi P, Salom B, Cozzi L et al (2018) Afatinib is a new therapeutic approach in chordoma with a unique ability to target EGFR and brachyury. Mol Cancer Ther 17:603–613
Mcmaster ML, Goldstein AM, Bromley CM et al (2001) Chordoma: incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control 12:1–11
Meis JM, Giraldo AA (1988) Chordoma. An immunohistochemical study of 20 cases. Arch Pathol Lab Med 112:553–556
Mirra Jm DRC, Sd N, Mertens F (2002) Chordoma. In: Fletcher Mertens CDKUF (Hrsg) Pathology and genetics of tumours of soft tissue and bone. IARC Press, Lyon, France, S 316–317
Morris LGT, Rihani J, Lebowitz RA et al (2009) Chondromyxoid fibroma of sphenoid sinus with unusual calcifications: case report with literature review. Head and Neck Pathol 3:169–173
Neumann J, Gersing AS, Barth TF et al (2019) Intra-articular extra-axial chordoma of the wrist: a case report with review of the current literature. Skelet Radiol 48:2015–2020
Nguyen RP, Salzman KL, Stambuk HE et al (2009) Extraosseous Chordoma of the Nasopharynx. AJNR Am J Neuroradiol 30:803
Oakley GJ, Fuhrer K, Seethala RR (2008) Brachyury, SOX‑9, and podoplanin, new markers in the skull base chordoma vs chondrosarcoma differential: a tissue microarray-based comparative analysis. Mod Pathol 21:1461–1469
Presneau N, Shalaby A, Ye H et al (2011) Role of the transcription factor T (brachyury) in the pathogenesis of sporadic chordoma: a genetic and functional-based study. J Pathol 223:327–335
Rekhi B, Sable M, Jambhekar NA (2012) Histopathological, immunohistochemical and molecular spectrum of myoepithelial tumours of soft tissues. Virchows Arch 461:687–697
Salisbury JR, Isaacson PG (1985) Demonstration of cytokeratins and an epithelial membrane antigen in chordomas and human fetal notochord. Am J Surg Pathol 9:791–797
Stacchiotti S, Marrari A, Tamborini E et al (2009) Response to imatinib plus sirolimus in advanced chordoma. Ann Oncol 20:1886–1894
Stacchiotti S, Morosi C, Lo Vullo S et al (2018) Imatinib and everolimus in patients with progressing advanced chordoma: a phase 2 clinical study. Cancer 124:4056–4063
Stacchiotti S, Sommer J (2015) Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncol 16:e71–83
Stacchiotti S, Tamborini E, Lo Vullo S et al (2013) Phase II study on lapatinib in advanced EGFR-positive chordoma. Ann Oncol 24:1931–1936
Von Witzleben A, Goerttler LT, Lennerz J et al (2016) In chordoma, metastasis, recurrences, Ki-67 index, and a matrix-poor phenotype are associated with patients’ shorter overall survival. Eur Spine J 25:4016–4024
Von Witzleben A, Goerttler LT, Marienfeld R et al (2015) Preclinical characterization of novel Chordoma cell systems and their targeting by pharmocological inhibitors of the CDK4/6 cell-cycle pathway. Cancer Res 75:3823–3831
Vujovic S, Henderson S, Presneau N et al (2006) Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas. J Pathol 209:157–165
WHO (Hrsg) (2020) WHO classification of tumours editorial board. Soft tissue and bone tumours. WHO classification of tumour series, 5th ed.; col.3. International Agency for research on Cancer, Lyon
Zuckerman SL, Bilsky MH, Laufer I (2018) Chordomas of the skull base, mobile spine, and sacrum: an epidemiologic investigation of presentation, treatment, and survival. World Neurosurg 113:e618–e627
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Interessenkonflikt
C. Holley, T. Breining, M. Scheithauer, P. Möller und T.F.E. Barth geben an, dass kein Interessenkonflikt besteht.
Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien. Für Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts, über die Patienten zu identifizieren sind, liegt von ihnen und/oder ihren gesetzlichen Vertretern eine schriftliche Einwilligung vor.
Caption Electronic Supplementary Material
Rights and permissions
About this article
Cite this article
Holley, C., Breining, T., Scheithauer, M. et al. Primäres extraaxiales chondroides Chordom des anterioren Nasenseptums: Fallbericht eines seltenen Chordoms mit Literaturübersicht. HNO 69, 221–228 (2021). https://doi.org/10.1007/s00106-020-00957-3
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00106-020-00957-3