- Email: [email protected]
Ectopic sinonasal craniopharyngioma arising from the anterior ethmoid sinus – Case report and literature review
Otolaryngology Case Reports 15 (2020) 100151
Contents lists available at ScienceDirect
Otolaryngology Case Reports journal homepage: www.elsevier.com/locate/xocr
Ectopic sinonasal craniopharyngioma arising from the anterior ethmoid sinus – Case report and literature review
T
Murray Smitha, Eugene H. Wonga,b,∗, Joyce Hoa, Richard Tjahjonoa, Hedley Colemanb,c, Narinder Singha,b, Niranjan Sritharana a
Department of Otolaryngology, Westmead Hospital, Westmead, NSW, 2145, Australia Sydney Medical School, University of Sydney, Camperdown, NSW, 2050, Australia c ICPMR and the Faculty of Dentistry, Westmead Hospital, Westmead, NSW, 2145, Australia b
A R T I C LE I N FO
A B S T R A C T
Keywords: Craniopharyngioma Ectopic Endoscopic Skull base surgery Sinus surgery
Objectives: We describe a rare case of an entirely ectopic adamantinomatous craniopharyngioma located in the anterior ethmoid sinus and the management approach undertaken at our institution. Case report: A 31-year old female presented with a history of chronic left sided nasal obstruction. Flexible nasendoscopy demonstrated a bony mass filling the entirety of the ostiomeatal complex on that side. High resolution computed tomography confirmed the presence of a heterogenous, bony mass which raised the possibility of an osteoma. Endoscopic sinus surgery was performed to achieve complete excision of the mass. Formal histopathological examination was consistent with an adamantinomatous craniopharyngioma. The patient underwent an uncomplicated post-operative course and continues to be disease-free six months post-operatively. Conclusions: This case represents one of only 23 reported infrasellar craniopharyngiomas described in the literature, and only the third that was completely ectopic from the sellar.
Introduction Craniopharyngiomas are rare, benign, epithelial neoplasms of the sellar region that account for approximately 1–10% of all intracranial tumours [1]. It is thought to be derived from Rathke's pouch epithelium, from which the craniopharyngeal canal develops. As a result, its usual presentation is that of a suprasellar mass (which accounts for approximately 50% of all suprasellar lesions in children), and therefore causes symptoms related to local intracranial mass effect, such as headache, bitemporal hemianopia or vomiting; or secondary to pituitary insufficiency. Infrasellar based craniopharyngiomas are extremely rare. To our knowledge, only 23 reported cases exist within the literature (Table 1), and of these only 3 are reported as lying beyond the sphenoid or nasopharynx. The two previously reported cases of craniopharyngioma within the ethmoid sinuses were both in children (aged 7 and 17 years). In contrast, the most common bony neoplasm arising within the sinonasal cavities is an osteoma, accounting for approximately 3% of all cases. Osteomas are slow-growing mesenchymal tumours most commonly presenting within the ethmoid sinuses, followed by the frontal sinuses [2]. Common symptoms from sinonasal osteomas relate to mass effect of the lesion to adjacent structures, and therefore may include ∗
nasal obstruction; facial pains; symptoms of sinusitis; or ocular signs and symptoms. This case report describes an extremely rare sinonasal presentation – the presence of a craniopharyngioma (a largely intracranial neoplasm) at a site distant to, and distinct from, the typical sellar region where it is normally seen. The case demonstrates the very early embryological origins of migration of Rathke's pouch epithelium. It also allows direct comparison with an osteoma as a far more common bony sinonasal lesion in the differential diagnosis. Case presentation A 31-year old female presented with a chronic, progressive history of unilateral left sided nasal obstruction, mouth breathing, and bruxism over several years. No specific symptoms of atopy or other rhinologic symptoms of chronic rhinosinusitis, such as facial pressure, nasal discharge or anosmia was reported. Flexible nasendoscopic examination in the outpatient setting revealed a right sided septal deviation, bilateral inferior turbinate hypertrophy, and a bony mass occluding the left ostiomeatal complex. The nasal mucosa surrounding the bony mass was noted to be unremarkable. The rest of the nasendoscopic examination to the level of the
Corresponding author. Department of Otolaryngology Westmead Hospital University of Sydney, Sydney, Australia. E-mail address: [email protected] (E.H. Wong).
https://doi.org/10.1016/j.xocr.2020.100151 Received 21 October 2019; Accepted 5 February 2020 Available online 07 February 2020 2468-5488/ © 2020 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Otolaryngology Case Reports 15 (2020) 100151
M. Smith, et al.
Discussion
Table 1 Cases of infrasellar craniopharyngioma in the literature. A list of all 22 previous described cases of infrasellar craniopharyngioma since 1977. Year
Author
Location
1977 1984 1988 1988 1989 1990 1996 1998 2001 2001 2002 2004 2005 2006 2007 2007 2009 2010 2011 2012 2014 2015 2017
Illum et al. [10] Mukada et al. [11] Hillman et al. [12] Benitez et al. [13] Akimura et al. [14] Byrne et al. [15] Cheddadi et al. [16] Jiang et al. [17] Falavigna et al. [18] Deutsch et al. [19] Fujimoto et al. [20] Ahsan et al. [21] Rakheja et al. [22] Koral et al. [23] Ragel et al. [24] Arndt et al. [25] Hwang et al. [26] Nourbakhsh et al. [27] Magill et al. [28] Chiun et al. [29] Kundu et al. [30] Zhang et al. [31] Preti et al. [32]
Nasopharynx Nasopharynx Not reported Nasopharynx Sphenoid and posterior ethmoid sinus Nasopharynx Nasopharynx Ethmoid sinus Sphenoid sinus Nasopharynx Sphenoid sinus Ostiomeatal complex and maxillary sinus Sphenoid sinus Sphenoid sinus Clivus Clivus Pterygopalatine fossa Sphenoid sinus Clivus Nasopharynx Nasopharynx Sphenoid sinus Ethmoid sinus
Craniopharyngiomas are rare, benign epithelial tumours that typically arise from sellar or suprasellar regions. The tumour development in these specific regions is secondary to its embryological origin from the craniopharyngeal canal, that runs from the roof of the nasopharynx to the adenohyophysis. This canal is of epithelial embryonic origin, from invagination of the stomodeal ectoderm), which explains the epithelium nature of the adenohypophysis. Craniopharyngiomas may arise at any point along the embryological craniopharyngeal canal, and development outside this tract is extremely rare. This case represents only the second reported case of an entirely ectopic tumour within the ethmoid sinus that is distinct from the classical description of the craniopharyngeal canal. The histogenesis and ectopic development of craniopharyngiomas remains therefore very unclear. One potential explanation is that the lesion may have arisen from misplaced odontogenic epithelium [1]. In fact, adamantinomatous craniopharyngiomas are histologically similar to ameloblastoma of the jaw and the keratinising and calcifying odontogenic cyst – a likeness so significant that in 1929, Soy proposed the term “ameloblastoma” in place of craniopharyngiomas [3]. Two other potential explanations for this ectopic location are ectopic migration of cell remnants of the obliterated craniopharyngeal canal destined to form tooth primordia; or metaplasia of the epithelium of the Schneiderian membrane (derived from stomodeal ectoderm) in a fashion similar to that which occurs with development of the craniopharyngeal canal [4]. In this study, the clinical presentation was most consistent with an isolated sinonasal osteoma, both on history, clinical examination and radiologically. However, radiological appearance of both lesions can carry considerable variability. Nevertheless, craniopharyngiomas typically demonstrate a lobulated contour due to multiple cystic lesions with peripheral calcifications [5], while sinonasal osteomas typically present as a well circumscribed mass of variable density – from a density similar to cortical bone to a ground-glass appearance [6]. However, craniopharyngiomas carry a much more unpredictable natural history. Suprasellar lesions carry a reported five-year survival rate of approximately 80% [7]. However, this figure may not necessarily translate to ectopic or infrasellar craniopharyngiomas, due to the difference in anatomical location, but also due the rarity of this subgroup. Nevertheless, craniopharyngiomas have been reported to recur multiple times, become locally aggressive [8], or undergo malignant transformation into carcinoma [9], and therefore complete surgical excision is typically recommended in these lesions.
larynx was also normal. Given these findings, a high resolution computed tomography (CT) scan was obtained (Fig. 1), which confirmed a well localised heterogenous, hyperdense calcified mass arising from the middle turbinate and extending into the anterior ethmoid sinus. There was no extension to the skull base or the sphenoid sinus. Given the radiologic appearance of the lesion, the primary diagnosis considered was that of a sinonasal osteoma. The patient then underwent an endoscopic septoplasty, bilateral turbinate reduction and complete excision of the left sinonasal mass. No specific intraoperative complications were noted, and the patient followed an uncomplicated postoperative course with regular saline douching. Interestingly, histopathological analysis demonstrated findings consistent with an ectopic adamantinomatous craniopharyngioma (Fig. 2). The bony trabeculae appeared thickened with a mosaic pattern with prominent basophilic resting lines, while a multifocal tumour was noted that comprised of ameloblastoma-like epithelium composed of follicles and a plexiform arrangement of cells. These follicles exhibited central stellate reticulum-like epithelium with peripherally palisaded columnar cells that demonstrate reverse nuclear polarisation and subnuclear vacuolation. Additionally, ghost cells were identified exhibiting keratinisation with karyolysis and dystrophic calcification. Immunohistochemistry with beta catenin was found to be focally positive with nuclear staining of the tumour cells.
Conclusion This case demonstrates only the third reported case in the literature of an entirely ectopic craniopharyngioma, and the first in an adult. Furthermore, to our knowledge it represents the most anterior (and distal from the sellar region) lesion reported. The case highlights the
Fig. 1. High resolution computed tomography images of the paranasal sinuses A) Coronal, B) axial and C) sagittal bone window slices demonstrating a well localised heterogenous, hyperdense calcified mass arising from the middle turbinate and extending into the anterior ethmoid sinus. 2
Otolaryngology Case Reports 15 (2020) 100151
M. Smith, et al.
Fig. 2. Histology slides of the ethmoid sinus lesion. Left slide demonstrates follicles exhibiting central stellate reticulum-like epithelium with peripherally palisaded columnar cells and right slide shows abundant ghost cells which exhibit keratinisation with karyolysis and dystrophic calcification.
breadth of differential diagnoses for isolated bony sinonasal lesions, and the importance of histopathological diagnosis in cases where potential morbidity is low.
[13] Benitez WI, Sartor KJ, Angtuaco EJ. Craniopharyngioma presenting as a nasopharyngeal mass: CT and MR findings. J Comput Assist Tomogr 1988;12(6):1068–72. [14] Akimura T, Kameda H, Abiko S, Aoki H, Kido T. Infrasellar craniopharyngioma. Neuroradiology 1989;31(2):180–3. [15] Byrne MN, Sessions DG. Nasopharyngeal craniopharyngioma. Case report and literature review. Ann Otol Rhinol Laryngol 1990;99(8):633–9. [16] Cheddadi D, Triki S, Gallet S, Marquet M, Muller J, Renaud H, et al. Neonatal rhinopharyngeal obstruction due to craniopharyngioma. Arch Pediatr 1996;3(4):348–51. [17] Jiang R-S, Wu C-Y, Jan Y-J, Hsu C-Y. Primary ethmoid sinus craniopharyngioma: a case report. J Laryngol Otol 1998;112:403–5. [18] Falagina A, Kraemer JL. Infrasellar craniopharyngioma: case report. Arq Neuro Psiquiatr 2001;11(2):121–8. [19] Deutsch H, Kothbauer K, Persky M, Epstein FJ, Jallo GI. Infrasellar craniopharyngiomas: case report and review of the literature. Skull Base 2001;11(2):121–8. [20] Fujimoto Y, Matsushita H, Valesco O, Rosemberg S, Please JP, Marino R. Craniopharyngioma involving the infrasellar region: case report and review of the literature. Pediatr Neurosurg 2002;37(4):210–6. [21] Ahsan F, Rashid F, Chapman A, Ah-See KW. Infrasellar craniopharyngioma presenting as epistaxis, excised via Denker's medial maxillectomy approach. J Laryngol Otol 2004;118(11):895–8. [22] Rakheja D, Meehan JJ, Gomez AM. Pathologic quiz case: sphenoid sinus mass in a 12-year-old girl. Infrasellar adamantinomatous craniopharyngioma. Arch Pathol Lab Med 2005;129(3):e73–4. [23] Koral K, Weprin B, Rollins NK. Sphenoid sinus craniopharyngioma simulating ucocoele. Acta Radiol 2006;47(5):494–6. [24] Ragel BT, Bishop FS, Couldwell WT. Recurrent infrasellar clival craniopharyngioma. Acta Neurochir (Wien) 2007;149(7):729–30. [25] Arndt S, Wiech T, Mader I, Maier W. Entire infrasellar craniopharyngioma simulating clival chordoma. Otolaryngol Head Neck Surg 2007;137(6):981–3. [26] Hwang KR, Lee JY, Byun JYU, Hong HS, Koh ES. Infrasellar craniopharyngioma originating from the pterygopalatine fossa with invasion to the maxillary sinus. Br J Oral Maxillofac Surg 2009;47(5):422–4. [27] Nourbakhsh A, Brown B, Vannemreddy P, Lian T, Nanda A, Guthikonda B. Extracranial infrasellar ectopic craniopharyngioma: a case report and review of the literature. Skull Base 2010;20(6):475–80. [28] Magill JC, Ferguson MS, Sandison A, Clarke PM. Nasal craniopharyngioma: case report and literature review. J Laryngol Otol 2011;125(5):517–9. [29] Chiun KC, Tang IP, Vikneswaran T, Nurshaline Pauline HK. Infrasellar craniopharyngioma of the posterior nasal septum: a rare entity. Med J Malaysia 2012;67(1):131–2. [30] Kundu S, Dewan K, Varshney H, Das S, Mukhopadhyay S, Ghosh A. An atypical rare case of extracranial craniopharyngioma. Indian J Otolaryngol Head Neck Surg 2014;66(1):122–5. [31] Zhang Z, Li M-Y, Liu Y-W, Wang Y-Z. An infrasellar craniopharyngioma involving the sphenoid sinus and clivus. Chin Med J (Engl) 2015;128(6):844–5. [32] Preti A, Karligkiotis A, Facco C, Ottini G, Volpi L, Castelnuovo P. Primary ectopic ethmoidal craniopharyngioma. J Craniofac Surg 2017;28(4):944–6.
Consent Consent was received by the patient. Ethical statement This study was performed in accordance with the Declaration of Helsinki. Declaration of competing interest None to declare. References [1] Barnes L. Barnes L, editor. Surgical pathology of the head and neck. third ed.Informa healthcare 2009. [2] Watkinson JC, Clarke RW, editors. Scott-Brown’s otorhinolaryngology and head and neck surgery. eighth ed.CRC Press; 2018. [3] Zeitlin H. Adamantinomas of the hypophyseal stalk and sphenoid bone. Am J Canc 1935;23(4):729–40. [4] Prabhu VC, Brown HG. The pathogenesis of craniopharyngiomas. Child’s Nerv Syst 2005;21(8–9):622–7. [5] Sartoeretti-Schefer S, Wichmann W, Aguzzi A, Valavanis A. MR differentiation of adamantinous and squamous-papillary craniopharyngiomas. Am J Neuroradiol 1997;18(1):77–87. [6] Lee DH, Jung SH, Yoon TM, Lee JK, Joo YE, Lim SC. Characteristics of paranasal sinus osteoma and treatment outcomes. Acta Otolaryngol 2015;135(6):602–7. [7] Bunin GR, Surawicz TS, Witman PA, et al. The descriptive epidemiology of craniopharyngioma. J Neurosurg 1998;89:547–51. [8] Gupta K, Kuhn MJ, Shevlin DW, Wacaser LE. Metastatic craniopharyngioma. Am J Neuroradiol 1999 Jun;20(6):1059–60. [9] Jeong TS, Yee GT, Kim NR. Malignant transformation of craniopharyngioma without radiation therapy: case report and review of the literature. J Kor Neurosurg Soc 2017 Jan;60(1):108–13. [10] Illum P, Elbrond O, Nehen AM. Surgical treatment of nasopharyngeal craniopharyngioma. Radical removal by the transpalatal approach. J Laryngol Otol 1977;91(3):227–33. [11] Mukada K, Mori S, Matsumara S, Uozumi T, Goishi J. Infrasellar craniopharyngioma. Surg Neurol 1984;21(6):565–71. [12] Hillman TH, Peyster RG, Hoover ED, Nair S, Finkelstein SD. Infrasellar craniopharyngioma: CT and MR studies. J Comput Assist Tomogr 1988;12(4):702–4.
3
Contents lists available at ScienceDirect
Otolaryngology Case Reports journal homepage: www.elsevier.com/locate/xocr
Ectopic sinonasal craniopharyngioma arising from the anterior ethmoid sinus – Case report and literature review
T
Murray Smitha, Eugene H. Wonga,b,∗, Joyce Hoa, Richard Tjahjonoa, Hedley Colemanb,c, Narinder Singha,b, Niranjan Sritharana a
Department of Otolaryngology, Westmead Hospital, Westmead, NSW, 2145, Australia Sydney Medical School, University of Sydney, Camperdown, NSW, 2050, Australia c ICPMR and the Faculty of Dentistry, Westmead Hospital, Westmead, NSW, 2145, Australia b
A R T I C LE I N FO
A B S T R A C T
Keywords: Craniopharyngioma Ectopic Endoscopic Skull base surgery Sinus surgery
Objectives: We describe a rare case of an entirely ectopic adamantinomatous craniopharyngioma located in the anterior ethmoid sinus and the management approach undertaken at our institution. Case report: A 31-year old female presented with a history of chronic left sided nasal obstruction. Flexible nasendoscopy demonstrated a bony mass filling the entirety of the ostiomeatal complex on that side. High resolution computed tomography confirmed the presence of a heterogenous, bony mass which raised the possibility of an osteoma. Endoscopic sinus surgery was performed to achieve complete excision of the mass. Formal histopathological examination was consistent with an adamantinomatous craniopharyngioma. The patient underwent an uncomplicated post-operative course and continues to be disease-free six months post-operatively. Conclusions: This case represents one of only 23 reported infrasellar craniopharyngiomas described in the literature, and only the third that was completely ectopic from the sellar.
Introduction Craniopharyngiomas are rare, benign, epithelial neoplasms of the sellar region that account for approximately 1–10% of all intracranial tumours [1]. It is thought to be derived from Rathke's pouch epithelium, from which the craniopharyngeal canal develops. As a result, its usual presentation is that of a suprasellar mass (which accounts for approximately 50% of all suprasellar lesions in children), and therefore causes symptoms related to local intracranial mass effect, such as headache, bitemporal hemianopia or vomiting; or secondary to pituitary insufficiency. Infrasellar based craniopharyngiomas are extremely rare. To our knowledge, only 23 reported cases exist within the literature (Table 1), and of these only 3 are reported as lying beyond the sphenoid or nasopharynx. The two previously reported cases of craniopharyngioma within the ethmoid sinuses were both in children (aged 7 and 17 years). In contrast, the most common bony neoplasm arising within the sinonasal cavities is an osteoma, accounting for approximately 3% of all cases. Osteomas are slow-growing mesenchymal tumours most commonly presenting within the ethmoid sinuses, followed by the frontal sinuses [2]. Common symptoms from sinonasal osteomas relate to mass effect of the lesion to adjacent structures, and therefore may include ∗
nasal obstruction; facial pains; symptoms of sinusitis; or ocular signs and symptoms. This case report describes an extremely rare sinonasal presentation – the presence of a craniopharyngioma (a largely intracranial neoplasm) at a site distant to, and distinct from, the typical sellar region where it is normally seen. The case demonstrates the very early embryological origins of migration of Rathke's pouch epithelium. It also allows direct comparison with an osteoma as a far more common bony sinonasal lesion in the differential diagnosis. Case presentation A 31-year old female presented with a chronic, progressive history of unilateral left sided nasal obstruction, mouth breathing, and bruxism over several years. No specific symptoms of atopy or other rhinologic symptoms of chronic rhinosinusitis, such as facial pressure, nasal discharge or anosmia was reported. Flexible nasendoscopic examination in the outpatient setting revealed a right sided septal deviation, bilateral inferior turbinate hypertrophy, and a bony mass occluding the left ostiomeatal complex. The nasal mucosa surrounding the bony mass was noted to be unremarkable. The rest of the nasendoscopic examination to the level of the
Corresponding author. Department of Otolaryngology Westmead Hospital University of Sydney, Sydney, Australia. E-mail address: [email protected] (E.H. Wong).
https://doi.org/10.1016/j.xocr.2020.100151 Received 21 October 2019; Accepted 5 February 2020 Available online 07 February 2020 2468-5488/ © 2020 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Otolaryngology Case Reports 15 (2020) 100151
M. Smith, et al.
Discussion
Table 1 Cases of infrasellar craniopharyngioma in the literature. A list of all 22 previous described cases of infrasellar craniopharyngioma since 1977. Year
Author
Location
1977 1984 1988 1988 1989 1990 1996 1998 2001 2001 2002 2004 2005 2006 2007 2007 2009 2010 2011 2012 2014 2015 2017
Illum et al. [10] Mukada et al. [11] Hillman et al. [12] Benitez et al. [13] Akimura et al. [14] Byrne et al. [15] Cheddadi et al. [16] Jiang et al. [17] Falavigna et al. [18] Deutsch et al. [19] Fujimoto et al. [20] Ahsan et al. [21] Rakheja et al. [22] Koral et al. [23] Ragel et al. [24] Arndt et al. [25] Hwang et al. [26] Nourbakhsh et al. [27] Magill et al. [28] Chiun et al. [29] Kundu et al. [30] Zhang et al. [31] Preti et al. [32]
Nasopharynx Nasopharynx Not reported Nasopharynx Sphenoid and posterior ethmoid sinus Nasopharynx Nasopharynx Ethmoid sinus Sphenoid sinus Nasopharynx Sphenoid sinus Ostiomeatal complex and maxillary sinus Sphenoid sinus Sphenoid sinus Clivus Clivus Pterygopalatine fossa Sphenoid sinus Clivus Nasopharynx Nasopharynx Sphenoid sinus Ethmoid sinus
Craniopharyngiomas are rare, benign epithelial tumours that typically arise from sellar or suprasellar regions. The tumour development in these specific regions is secondary to its embryological origin from the craniopharyngeal canal, that runs from the roof of the nasopharynx to the adenohyophysis. This canal is of epithelial embryonic origin, from invagination of the stomodeal ectoderm), which explains the epithelium nature of the adenohypophysis. Craniopharyngiomas may arise at any point along the embryological craniopharyngeal canal, and development outside this tract is extremely rare. This case represents only the second reported case of an entirely ectopic tumour within the ethmoid sinus that is distinct from the classical description of the craniopharyngeal canal. The histogenesis and ectopic development of craniopharyngiomas remains therefore very unclear. One potential explanation is that the lesion may have arisen from misplaced odontogenic epithelium [1]. In fact, adamantinomatous craniopharyngiomas are histologically similar to ameloblastoma of the jaw and the keratinising and calcifying odontogenic cyst – a likeness so significant that in 1929, Soy proposed the term “ameloblastoma” in place of craniopharyngiomas [3]. Two other potential explanations for this ectopic location are ectopic migration of cell remnants of the obliterated craniopharyngeal canal destined to form tooth primordia; or metaplasia of the epithelium of the Schneiderian membrane (derived from stomodeal ectoderm) in a fashion similar to that which occurs with development of the craniopharyngeal canal [4]. In this study, the clinical presentation was most consistent with an isolated sinonasal osteoma, both on history, clinical examination and radiologically. However, radiological appearance of both lesions can carry considerable variability. Nevertheless, craniopharyngiomas typically demonstrate a lobulated contour due to multiple cystic lesions with peripheral calcifications [5], while sinonasal osteomas typically present as a well circumscribed mass of variable density – from a density similar to cortical bone to a ground-glass appearance [6]. However, craniopharyngiomas carry a much more unpredictable natural history. Suprasellar lesions carry a reported five-year survival rate of approximately 80% [7]. However, this figure may not necessarily translate to ectopic or infrasellar craniopharyngiomas, due to the difference in anatomical location, but also due the rarity of this subgroup. Nevertheless, craniopharyngiomas have been reported to recur multiple times, become locally aggressive [8], or undergo malignant transformation into carcinoma [9], and therefore complete surgical excision is typically recommended in these lesions.
larynx was also normal. Given these findings, a high resolution computed tomography (CT) scan was obtained (Fig. 1), which confirmed a well localised heterogenous, hyperdense calcified mass arising from the middle turbinate and extending into the anterior ethmoid sinus. There was no extension to the skull base or the sphenoid sinus. Given the radiologic appearance of the lesion, the primary diagnosis considered was that of a sinonasal osteoma. The patient then underwent an endoscopic septoplasty, bilateral turbinate reduction and complete excision of the left sinonasal mass. No specific intraoperative complications were noted, and the patient followed an uncomplicated postoperative course with regular saline douching. Interestingly, histopathological analysis demonstrated findings consistent with an ectopic adamantinomatous craniopharyngioma (Fig. 2). The bony trabeculae appeared thickened with a mosaic pattern with prominent basophilic resting lines, while a multifocal tumour was noted that comprised of ameloblastoma-like epithelium composed of follicles and a plexiform arrangement of cells. These follicles exhibited central stellate reticulum-like epithelium with peripherally palisaded columnar cells that demonstrate reverse nuclear polarisation and subnuclear vacuolation. Additionally, ghost cells were identified exhibiting keratinisation with karyolysis and dystrophic calcification. Immunohistochemistry with beta catenin was found to be focally positive with nuclear staining of the tumour cells.
Conclusion This case demonstrates only the third reported case in the literature of an entirely ectopic craniopharyngioma, and the first in an adult. Furthermore, to our knowledge it represents the most anterior (and distal from the sellar region) lesion reported. The case highlights the
Fig. 1. High resolution computed tomography images of the paranasal sinuses A) Coronal, B) axial and C) sagittal bone window slices demonstrating a well localised heterogenous, hyperdense calcified mass arising from the middle turbinate and extending into the anterior ethmoid sinus. 2
Otolaryngology Case Reports 15 (2020) 100151
M. Smith, et al.
Fig. 2. Histology slides of the ethmoid sinus lesion. Left slide demonstrates follicles exhibiting central stellate reticulum-like epithelium with peripherally palisaded columnar cells and right slide shows abundant ghost cells which exhibit keratinisation with karyolysis and dystrophic calcification.
breadth of differential diagnoses for isolated bony sinonasal lesions, and the importance of histopathological diagnosis in cases where potential morbidity is low.
[13] Benitez WI, Sartor KJ, Angtuaco EJ. Craniopharyngioma presenting as a nasopharyngeal mass: CT and MR findings. J Comput Assist Tomogr 1988;12(6):1068–72. [14] Akimura T, Kameda H, Abiko S, Aoki H, Kido T. Infrasellar craniopharyngioma. Neuroradiology 1989;31(2):180–3. [15] Byrne MN, Sessions DG. Nasopharyngeal craniopharyngioma. Case report and literature review. Ann Otol Rhinol Laryngol 1990;99(8):633–9. [16] Cheddadi D, Triki S, Gallet S, Marquet M, Muller J, Renaud H, et al. Neonatal rhinopharyngeal obstruction due to craniopharyngioma. Arch Pediatr 1996;3(4):348–51. [17] Jiang R-S, Wu C-Y, Jan Y-J, Hsu C-Y. Primary ethmoid sinus craniopharyngioma: a case report. J Laryngol Otol 1998;112:403–5. [18] Falagina A, Kraemer JL. Infrasellar craniopharyngioma: case report. Arq Neuro Psiquiatr 2001;11(2):121–8. [19] Deutsch H, Kothbauer K, Persky M, Epstein FJ, Jallo GI. Infrasellar craniopharyngiomas: case report and review of the literature. Skull Base 2001;11(2):121–8. [20] Fujimoto Y, Matsushita H, Valesco O, Rosemberg S, Please JP, Marino R. Craniopharyngioma involving the infrasellar region: case report and review of the literature. Pediatr Neurosurg 2002;37(4):210–6. [21] Ahsan F, Rashid F, Chapman A, Ah-See KW. Infrasellar craniopharyngioma presenting as epistaxis, excised via Denker's medial maxillectomy approach. J Laryngol Otol 2004;118(11):895–8. [22] Rakheja D, Meehan JJ, Gomez AM. Pathologic quiz case: sphenoid sinus mass in a 12-year-old girl. Infrasellar adamantinomatous craniopharyngioma. Arch Pathol Lab Med 2005;129(3):e73–4. [23] Koral K, Weprin B, Rollins NK. Sphenoid sinus craniopharyngioma simulating ucocoele. Acta Radiol 2006;47(5):494–6. [24] Ragel BT, Bishop FS, Couldwell WT. Recurrent infrasellar clival craniopharyngioma. Acta Neurochir (Wien) 2007;149(7):729–30. [25] Arndt S, Wiech T, Mader I, Maier W. Entire infrasellar craniopharyngioma simulating clival chordoma. Otolaryngol Head Neck Surg 2007;137(6):981–3. [26] Hwang KR, Lee JY, Byun JYU, Hong HS, Koh ES. Infrasellar craniopharyngioma originating from the pterygopalatine fossa with invasion to the maxillary sinus. Br J Oral Maxillofac Surg 2009;47(5):422–4. [27] Nourbakhsh A, Brown B, Vannemreddy P, Lian T, Nanda A, Guthikonda B. Extracranial infrasellar ectopic craniopharyngioma: a case report and review of the literature. Skull Base 2010;20(6):475–80. [28] Magill JC, Ferguson MS, Sandison A, Clarke PM. Nasal craniopharyngioma: case report and literature review. J Laryngol Otol 2011;125(5):517–9. [29] Chiun KC, Tang IP, Vikneswaran T, Nurshaline Pauline HK. Infrasellar craniopharyngioma of the posterior nasal septum: a rare entity. Med J Malaysia 2012;67(1):131–2. [30] Kundu S, Dewan K, Varshney H, Das S, Mukhopadhyay S, Ghosh A. An atypical rare case of extracranial craniopharyngioma. Indian J Otolaryngol Head Neck Surg 2014;66(1):122–5. [31] Zhang Z, Li M-Y, Liu Y-W, Wang Y-Z. An infrasellar craniopharyngioma involving the sphenoid sinus and clivus. Chin Med J (Engl) 2015;128(6):844–5. [32] Preti A, Karligkiotis A, Facco C, Ottini G, Volpi L, Castelnuovo P. Primary ectopic ethmoidal craniopharyngioma. J Craniofac Surg 2017;28(4):944–6.
Consent Consent was received by the patient. Ethical statement This study was performed in accordance with the Declaration of Helsinki. Declaration of competing interest None to declare. References [1] Barnes L. Barnes L, editor. Surgical pathology of the head and neck. third ed.Informa healthcare 2009. [2] Watkinson JC, Clarke RW, editors. Scott-Brown’s otorhinolaryngology and head and neck surgery. eighth ed.CRC Press; 2018. [3] Zeitlin H. Adamantinomas of the hypophyseal stalk and sphenoid bone. Am J Canc 1935;23(4):729–40. [4] Prabhu VC, Brown HG. The pathogenesis of craniopharyngiomas. Child’s Nerv Syst 2005;21(8–9):622–7. [5] Sartoeretti-Schefer S, Wichmann W, Aguzzi A, Valavanis A. MR differentiation of adamantinous and squamous-papillary craniopharyngiomas. Am J Neuroradiol 1997;18(1):77–87. [6] Lee DH, Jung SH, Yoon TM, Lee JK, Joo YE, Lim SC. Characteristics of paranasal sinus osteoma and treatment outcomes. Acta Otolaryngol 2015;135(6):602–7. [7] Bunin GR, Surawicz TS, Witman PA, et al. The descriptive epidemiology of craniopharyngioma. J Neurosurg 1998;89:547–51. [8] Gupta K, Kuhn MJ, Shevlin DW, Wacaser LE. Metastatic craniopharyngioma. Am J Neuroradiol 1999 Jun;20(6):1059–60. [9] Jeong TS, Yee GT, Kim NR. Malignant transformation of craniopharyngioma without radiation therapy: case report and review of the literature. J Kor Neurosurg Soc 2017 Jan;60(1):108–13. [10] Illum P, Elbrond O, Nehen AM. Surgical treatment of nasopharyngeal craniopharyngioma. Radical removal by the transpalatal approach. J Laryngol Otol 1977;91(3):227–33. [11] Mukada K, Mori S, Matsumara S, Uozumi T, Goishi J. Infrasellar craniopharyngioma. Surg Neurol 1984;21(6):565–71. [12] Hillman TH, Peyster RG, Hoover ED, Nair S, Finkelstein SD. Infrasellar craniopharyngioma: CT and MR studies. J Comput Assist Tomogr 1988;12(4):702–4.
3