- Email: [email protected]
Nasopharyngeal hairy polyp with recurrence in the middle ear
International Journal of Pediatric Otorhinolaryngology (2008) 72, 261—264
www.elsevier.com/locate/ijporl
CASE REPORT
Nasopharyngeal hairy polyp with recurrence in the middle ear Steven S. Chang a, Marc Halushka b, Joseph Vander Meer a, Martin Goins IIIa, Howard W. Francis a,* a
Department Otolaryngology-Head and Neck Surgery, Johns Hopkins University, Baltimore, MD, United States b Department Pathology, Johns Hopkins University, Baltimore, MD, United States Received 12 June 2007; received in revised form 28 September 2007; accepted 1 October 2007 Available online 19 November 2007
KEYWORDS Nasopharyngeal hairy polyp; Middle ear choristoma; Dermoid; Teratoma; Recurrence
Summary A 6-year-old girl was referred to The Johns Hopkins Hospital for a suspected congenital cholesteatoma. The lesion was determined to be a recurrence of a nasopharyngeal hairy polyp removed in the postnatal period. Simple excision is normally regarded as curative: malignant transformation and local recurrence do not occur. Our case is exceptional in that incomplete excision of the nasopharyngeal polyp was followed by re-growth and extension into the middle ear over a 6-year period. Thus, not all hairy polyps are static lesions. Indeed, this case documents progressive growth of a hairy polyp during physical development of a child, and indicates a potential for local recurrence following incomplete excision. A review of possible etiologies is conducted. # 2007 Elsevier Ireland Ltd. All rights reserved.
A 6-year-old girl was referred to The Johns Hopkins Hospital for a suspected congenital cholesteatoma. The lesion was discovered after a routine hearing screen revealed a 40 dB unilateral conductive hearing loss. She was the product of an uncomplicated full term pregnancy, but exhibited marked inspiratory stridor and respiratory distress soon after birth prompting emergent endotracheal intubation. An obstructive nasopharyngeal mass
* Corresponding author at: Department Otolaryngology-Head and Neck Surgery, 601 N. Caroline Street, Johns Hopkins Outpatient Center 6th Floor, Baltimore, MD 21287. Tel.: +1 410 955 1640; fax: +1 410 614 8610. E-mail address: [email protected] (H.W. Francis).
was seen on intubation, which appeared on CAT scan to be composed of adipose tissue (Fig. 1, arrow). Also noted on the CT scan was the normal middle ear status of the patient bilaterally. On pharyngoscopic examination under general anesthesia the mass was found to arise from the left lateral nasopharyngeal wall. Following a partial resection of the mass, dysphagia to solids and frequent episodes of otitis media in the left ear prompted further resection 9 months later. She remained free of any further otologic symptoms until the diagnosis of left conductive hearing loss and middle ear mass 5 years later. On physical examination a white bulging mass was found medial to an intact left tympanic membrane.
0165-5876/$ — see front matter # 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2007.10.003
262
S.S. Chang et al.
Fig. 1 Axial CAT scans of head and neck performed within hours of birth. A. Soft tissue mass filling the nasopharynx. B. Middle ears are aerated.
She had normal cranial nerve function including normal facial movement. Audiometry revealed normal bone conduction thresholds and an air-bone gap of 40 dB in the left ear. The remainder of the head and neck examination was normal. CAT scans of the temporal bone revealed a soft tissue density filling the left middle ear (Fig. 2, asterisk), which was not present in the neonatal study. There was no apparent erosion of the ossicular chain. The middle ear lesion appeared to be contiguous with soft tissue in the infratemporal fossa via a defect in the tympanic bone (Fig. 2). Using a post-auricular approach, a tympanomeatal flap was elevated revealing a protruding white mass surrounded by a dense fibrous capsule. The mass did not adhere to either the ossicular chain or the horizontal segment of the facial nerve. Dysplasia of neither the middle ear ossicles nor facial nerve was observed. The mass arose from the hypotympanum, which lacked a bony floor, as suggested by the preoperative CAT scans (Fig. 2). A combination of blunt and sharp dissection was used to mobilize the mass from the bony walls of the middle ear. A canalplasty was performed on the inferior aspect of the bony canal to better access the hypotympanum. A vascular pedicle originating from the hypotympanum was coagulated with bipolar cautery, and the mass was removed. It became evident however that the mass might extended beyond the bony confines of the middle ear into the infratemporal region, from which removal would require a separate surgical approach. Due to the benign histology no further resection was undertaken.
Medium power light micrographs of representative sections through the nasopharyngeal (Fig. 3A) and middle ear components of this lesion are presented (Fig. 3B, C). Keratinizing squamous epithelium forms the surface covering of the nasopharyngeal component deep to which is seen hair follicles (HF), sebaceous glands (SG) and sweat glands in both locations (Fig. 3A—C). Histopathologic diagnosis: Hairy polyp of nasopharynx with recurrence in the middle ear. Hairy polyp is the most common congenital mass in the nasopharynx. It is six times more common in females than males [1]. Embryologically, hairy polyp is composed of a central core of mesodermal structures and an outer zone of ectodermal structures [2]. They can measure up to 6 cm in diameter, and they are often associated with feeding difficulty or respiratory compromise in the neonatal period. Other presentations include cough, nasal discharge, epistaxis, and Eustachian tube dysfunction complicated by recurrent otitis media [3]. Ten percent present, with a concomitant cleft palate due to a mechanical impediment to normal closure [4]. Middle ear involvement is exceedingly rare and almost always involves direct extension through the Eustachian tube. Niklaus et al. reported eight cases of hairy polyps originating from the Eustachian tube, but the number extending to the middle ear was not stated [3]. As demonstrated in the nasopharyngeal lesion and its middle ear component (Fig. 3), hairy polyps are characterized by a surface of keratinized squamous epithelium under which lies collections of
Nasopharyngeal hairy polyp
Fig. 2 Sequence of serial coronal CAT sections performed at 6 years of age. Note: mesotympanic mass (asterisk) extending up to the level of the tensor tympani muscle. This soft tissue appears to be continuous with infratemporal region through a partial dehiscence of the hypotympanic floor (ICA, internal carotid artery).
sweat glands, sebaceous glands (SG) and hair follicles (HF). The stroma is composed of adipose tissue (AT) between fibrous bands, collagen, smooth muscle and skeletal muscle. Some hairy polyps also contain cartilage, bone, nerve fibers and minor salivary glands [5]. In this case, the nasopharyngeal component of the polyp was composed of mature adipose tissue covered by keratinized squamous epithelium with hair follicles (Fig. 3). The recurrent polyp in the middle ear was comprised mostly of mature fat, scattered minor salivary gland lobules
263 (MS) and hair follicles. Except for the presence of hair follicles it had the histopathologic appearance of a choristoma. Simoni et al. describes the only other case in the English literature of a simultaneous hairy polyp of the pharynx and choristoma of the middle ear. The gross anatomic features of the middle ear mass and its relationship with the middle ear anatomy are nearly identical to that described in this report, but differ in the predominance of salivary tissue [6]. The precise nature of the hairy polyp has been a matter of considerable confusion. Some regard hairy polyps as a type of germ cell neoplasm, and others regard them as embryologically displaced non-neoplastic tissue. The teratomatous theory includes hairy polyp among a spectrum of congenital nasopharyngeal neoplasms encompassing epignathi, tertomas, teratoid and dermoids [7]. The teratomatous theory, however, is not easily defended. First, hairy polyps are comprised of only two germ layers (ectoderm and mesoderm). Unlike true teratomas, they do not harbor elements of endodermal derivation. Second, hairy polyps demonstrate a clear female predominance in contrast to true teratomas, which affect both sexes equally. The developmental theory states that hairy polyps are the result of errors during embryogenesis, because they occur in regions of the head and neck with disparate developmental origins. Branchial arch malformations, for example, are implicated in hairy polyps that extend to the middle ear [1]. Heffner et al. proposed that hairy polyps represent displaced tissues derived from the first or second arch tissue originally destined to form the auricle and tragus, and referred to them as pharyngeal accessory auricles [8]. However, not all hairy polyps have cartilage, and not all are within the distribution of the first or second branchial arches. Lesions of the nasopharynx may result from anomalous fusion of the epiblast of the stomodeum with the anterior foregut and regression of the nasopharyngeal membrane [9]. Given the anatomic and histologic diversity of hairy polyps, it is difficult to accept a single unifying embryologic mechanism. Simple excision is regarded as curative: malignant transformation and local recurrence do not occur. Our case is exceptional in that incomplete excision of the nasopharyngeal polyp was followed by re-growth and extension into the middle ear over a 6-year period. Thus, not all hairy polyps are static lesions. Indeed, this case documents progressive growth of a hairy polyp during physical development of a child, and indicates a potential for local recurrence following incomplete excision.
264
S.S. Chang et al.
Fig. 3 Medium power light micrographs of representative sections through the nasopharyngeal and middle ear components of this lesion. HF hair follicle, SG sebaceous gland, AT adipose tissue.
References [1] B.V. Burns, P.R. Axon, A. Pahade, Hairy polyp of the pharynx in association with an ipsilateral branchial sinus: evidence that the ‘‘hairy polyp’’ is a second branchial arch malformation, J. Laryngol. Otol. 115 (2001) 145—148. [2] S.J. Jarvis, P.D. Bull, Hairy polyps of the nasopharynx, J. Laryngol. Otol. 116 (6) (2002) 467—469. [3] P. Niklaus, V. Forte, P. Thorner, Hairy polyp of the eustachian tube, J. Otolaryngol. 20 (1991) 254—257. [4] J. Haddad Jr., C.W. Senders, C.S. Leach, S.E. Stool, Congenital hairy polyp of the nasopharynx associated with cleft palate: report of two cases, Int. J. Pediatr. Otorhinolaryngol. 20 (1990) 127—135.
[5] S.E. Mills, M.J. Gaffey, H.F. Frierson, Tumors of the upper aerodigestive tract and ear, third series, Armed Forces Inst. Pathol. 26 (2000) 365. [6] P. Simoni, B.J. Wiatrak, D.R. Kelly, Choristomatous polyps of the aural and pharyngeal regions: first simultaneous case, Int. J. Pediatr. Otorhinolaryngol. 67 (2003) 195—199. [7] J. Arnold, Ein Fall von congenitalem zusammengestztem Lipom der Zunge under Pharynx mit perforation in die Schaedelhoehle, Virchows Arch fur Pathol Anat Physiol. 50 (1870) 482—516. [8] D.K. Heffner, L.D.R. Thompson, D.G. Schall, V. Anderson, Pharyngeal dermoids (‘‘hairy polyps’’) as accessory auricles, Ann. Otol. Rhinol. Laryngol. 105 (1996) 819—824. [9] R. Badrawy, S.A. Fahmy, A.M. Taha, Tertoid tumours of the nasopharynx, J. Laryngol. Otol. 87 (1973) 795—799.
Available online at www.sciencedirect.com
www.elsevier.com/locate/ijporl
CASE REPORT
Nasopharyngeal hairy polyp with recurrence in the middle ear Steven S. Chang a, Marc Halushka b, Joseph Vander Meer a, Martin Goins IIIa, Howard W. Francis a,* a
Department Otolaryngology-Head and Neck Surgery, Johns Hopkins University, Baltimore, MD, United States b Department Pathology, Johns Hopkins University, Baltimore, MD, United States Received 12 June 2007; received in revised form 28 September 2007; accepted 1 October 2007 Available online 19 November 2007
KEYWORDS Nasopharyngeal hairy polyp; Middle ear choristoma; Dermoid; Teratoma; Recurrence
Summary A 6-year-old girl was referred to The Johns Hopkins Hospital for a suspected congenital cholesteatoma. The lesion was determined to be a recurrence of a nasopharyngeal hairy polyp removed in the postnatal period. Simple excision is normally regarded as curative: malignant transformation and local recurrence do not occur. Our case is exceptional in that incomplete excision of the nasopharyngeal polyp was followed by re-growth and extension into the middle ear over a 6-year period. Thus, not all hairy polyps are static lesions. Indeed, this case documents progressive growth of a hairy polyp during physical development of a child, and indicates a potential for local recurrence following incomplete excision. A review of possible etiologies is conducted. # 2007 Elsevier Ireland Ltd. All rights reserved.
A 6-year-old girl was referred to The Johns Hopkins Hospital for a suspected congenital cholesteatoma. The lesion was discovered after a routine hearing screen revealed a 40 dB unilateral conductive hearing loss. She was the product of an uncomplicated full term pregnancy, but exhibited marked inspiratory stridor and respiratory distress soon after birth prompting emergent endotracheal intubation. An obstructive nasopharyngeal mass
* Corresponding author at: Department Otolaryngology-Head and Neck Surgery, 601 N. Caroline Street, Johns Hopkins Outpatient Center 6th Floor, Baltimore, MD 21287. Tel.: +1 410 955 1640; fax: +1 410 614 8610. E-mail address: [email protected] (H.W. Francis).
was seen on intubation, which appeared on CAT scan to be composed of adipose tissue (Fig. 1, arrow). Also noted on the CT scan was the normal middle ear status of the patient bilaterally. On pharyngoscopic examination under general anesthesia the mass was found to arise from the left lateral nasopharyngeal wall. Following a partial resection of the mass, dysphagia to solids and frequent episodes of otitis media in the left ear prompted further resection 9 months later. She remained free of any further otologic symptoms until the diagnosis of left conductive hearing loss and middle ear mass 5 years later. On physical examination a white bulging mass was found medial to an intact left tympanic membrane.
0165-5876/$ — see front matter # 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2007.10.003
262
S.S. Chang et al.
Fig. 1 Axial CAT scans of head and neck performed within hours of birth. A. Soft tissue mass filling the nasopharynx. B. Middle ears are aerated.
She had normal cranial nerve function including normal facial movement. Audiometry revealed normal bone conduction thresholds and an air-bone gap of 40 dB in the left ear. The remainder of the head and neck examination was normal. CAT scans of the temporal bone revealed a soft tissue density filling the left middle ear (Fig. 2, asterisk), which was not present in the neonatal study. There was no apparent erosion of the ossicular chain. The middle ear lesion appeared to be contiguous with soft tissue in the infratemporal fossa via a defect in the tympanic bone (Fig. 2). Using a post-auricular approach, a tympanomeatal flap was elevated revealing a protruding white mass surrounded by a dense fibrous capsule. The mass did not adhere to either the ossicular chain or the horizontal segment of the facial nerve. Dysplasia of neither the middle ear ossicles nor facial nerve was observed. The mass arose from the hypotympanum, which lacked a bony floor, as suggested by the preoperative CAT scans (Fig. 2). A combination of blunt and sharp dissection was used to mobilize the mass from the bony walls of the middle ear. A canalplasty was performed on the inferior aspect of the bony canal to better access the hypotympanum. A vascular pedicle originating from the hypotympanum was coagulated with bipolar cautery, and the mass was removed. It became evident however that the mass might extended beyond the bony confines of the middle ear into the infratemporal region, from which removal would require a separate surgical approach. Due to the benign histology no further resection was undertaken.
Medium power light micrographs of representative sections through the nasopharyngeal (Fig. 3A) and middle ear components of this lesion are presented (Fig. 3B, C). Keratinizing squamous epithelium forms the surface covering of the nasopharyngeal component deep to which is seen hair follicles (HF), sebaceous glands (SG) and sweat glands in both locations (Fig. 3A—C). Histopathologic diagnosis: Hairy polyp of nasopharynx with recurrence in the middle ear. Hairy polyp is the most common congenital mass in the nasopharynx. It is six times more common in females than males [1]. Embryologically, hairy polyp is composed of a central core of mesodermal structures and an outer zone of ectodermal structures [2]. They can measure up to 6 cm in diameter, and they are often associated with feeding difficulty or respiratory compromise in the neonatal period. Other presentations include cough, nasal discharge, epistaxis, and Eustachian tube dysfunction complicated by recurrent otitis media [3]. Ten percent present, with a concomitant cleft palate due to a mechanical impediment to normal closure [4]. Middle ear involvement is exceedingly rare and almost always involves direct extension through the Eustachian tube. Niklaus et al. reported eight cases of hairy polyps originating from the Eustachian tube, but the number extending to the middle ear was not stated [3]. As demonstrated in the nasopharyngeal lesion and its middle ear component (Fig. 3), hairy polyps are characterized by a surface of keratinized squamous epithelium under which lies collections of
Nasopharyngeal hairy polyp
Fig. 2 Sequence of serial coronal CAT sections performed at 6 years of age. Note: mesotympanic mass (asterisk) extending up to the level of the tensor tympani muscle. This soft tissue appears to be continuous with infratemporal region through a partial dehiscence of the hypotympanic floor (ICA, internal carotid artery).
sweat glands, sebaceous glands (SG) and hair follicles (HF). The stroma is composed of adipose tissue (AT) between fibrous bands, collagen, smooth muscle and skeletal muscle. Some hairy polyps also contain cartilage, bone, nerve fibers and minor salivary glands [5]. In this case, the nasopharyngeal component of the polyp was composed of mature adipose tissue covered by keratinized squamous epithelium with hair follicles (Fig. 3). The recurrent polyp in the middle ear was comprised mostly of mature fat, scattered minor salivary gland lobules
263 (MS) and hair follicles. Except for the presence of hair follicles it had the histopathologic appearance of a choristoma. Simoni et al. describes the only other case in the English literature of a simultaneous hairy polyp of the pharynx and choristoma of the middle ear. The gross anatomic features of the middle ear mass and its relationship with the middle ear anatomy are nearly identical to that described in this report, but differ in the predominance of salivary tissue [6]. The precise nature of the hairy polyp has been a matter of considerable confusion. Some regard hairy polyps as a type of germ cell neoplasm, and others regard them as embryologically displaced non-neoplastic tissue. The teratomatous theory includes hairy polyp among a spectrum of congenital nasopharyngeal neoplasms encompassing epignathi, tertomas, teratoid and dermoids [7]. The teratomatous theory, however, is not easily defended. First, hairy polyps are comprised of only two germ layers (ectoderm and mesoderm). Unlike true teratomas, they do not harbor elements of endodermal derivation. Second, hairy polyps demonstrate a clear female predominance in contrast to true teratomas, which affect both sexes equally. The developmental theory states that hairy polyps are the result of errors during embryogenesis, because they occur in regions of the head and neck with disparate developmental origins. Branchial arch malformations, for example, are implicated in hairy polyps that extend to the middle ear [1]. Heffner et al. proposed that hairy polyps represent displaced tissues derived from the first or second arch tissue originally destined to form the auricle and tragus, and referred to them as pharyngeal accessory auricles [8]. However, not all hairy polyps have cartilage, and not all are within the distribution of the first or second branchial arches. Lesions of the nasopharynx may result from anomalous fusion of the epiblast of the stomodeum with the anterior foregut and regression of the nasopharyngeal membrane [9]. Given the anatomic and histologic diversity of hairy polyps, it is difficult to accept a single unifying embryologic mechanism. Simple excision is regarded as curative: malignant transformation and local recurrence do not occur. Our case is exceptional in that incomplete excision of the nasopharyngeal polyp was followed by re-growth and extension into the middle ear over a 6-year period. Thus, not all hairy polyps are static lesions. Indeed, this case documents progressive growth of a hairy polyp during physical development of a child, and indicates a potential for local recurrence following incomplete excision.
264
S.S. Chang et al.
Fig. 3 Medium power light micrographs of representative sections through the nasopharyngeal and middle ear components of this lesion. HF hair follicle, SG sebaceous gland, AT adipose tissue.
References [1] B.V. Burns, P.R. Axon, A. Pahade, Hairy polyp of the pharynx in association with an ipsilateral branchial sinus: evidence that the ‘‘hairy polyp’’ is a second branchial arch malformation, J. Laryngol. Otol. 115 (2001) 145—148. [2] S.J. Jarvis, P.D. Bull, Hairy polyps of the nasopharynx, J. Laryngol. Otol. 116 (6) (2002) 467—469. [3] P. Niklaus, V. Forte, P. Thorner, Hairy polyp of the eustachian tube, J. Otolaryngol. 20 (1991) 254—257. [4] J. Haddad Jr., C.W. Senders, C.S. Leach, S.E. Stool, Congenital hairy polyp of the nasopharynx associated with cleft palate: report of two cases, Int. J. Pediatr. Otorhinolaryngol. 20 (1990) 127—135.
[5] S.E. Mills, M.J. Gaffey, H.F. Frierson, Tumors of the upper aerodigestive tract and ear, third series, Armed Forces Inst. Pathol. 26 (2000) 365. [6] P. Simoni, B.J. Wiatrak, D.R. Kelly, Choristomatous polyps of the aural and pharyngeal regions: first simultaneous case, Int. J. Pediatr. Otorhinolaryngol. 67 (2003) 195—199. [7] J. Arnold, Ein Fall von congenitalem zusammengestztem Lipom der Zunge under Pharynx mit perforation in die Schaedelhoehle, Virchows Arch fur Pathol Anat Physiol. 50 (1870) 482—516. [8] D.K. Heffner, L.D.R. Thompson, D.G. Schall, V. Anderson, Pharyngeal dermoids (‘‘hairy polyps’’) as accessory auricles, Ann. Otol. Rhinol. Laryngol. 105 (1996) 819—824. [9] R. Badrawy, S.A. Fahmy, A.M. Taha, Tertoid tumours of the nasopharynx, J. Laryngol. Otol. 87 (1973) 795—799.
Available online at www.sciencedirect.com