Hairy Polyp of the Oropharynx: Case Report and Literature Review

By Andrea Kelly, I. David Bough, Jr, Jay D. Luft, Katrina Conard, James S. Reilly, and Deborah Tuttle Philadelphia, PA and Wilmington, Delaware

0 Hairy polyp of the oronasopharynx is an uncommon developmental malformation that is most frequently seen as a pedunculated tumor in the neonate. Derived from the ectoderm and mesoderm, this benign tumor generally has been classified as dermoid. The clinical presentation is depen- dent on the polyp’s size and location. A full-term girl was evaluated for an oral mass that was first noted at the time of birth. Evaluation showed a 5- x 2.5-cm soft, nontender, skin-covered mass that protruded from the oral cavity. During surgery, it was noted that the stalk was attached to the superior pole of the left tonsil. The histology of the mass was consistent with a hairy polyp. Knowledge of this type of malformation facilitates early intervention and avoids signifi- cant morbidity. Copyright o 1996 by W.B. Saunders Company

INDEX WORDS: Hairy polyp, epignathi, dermoid.

H AIRY POLYP (HP) tumor of the upper aerodi- gestive tract is most frequently seen as a pedun-

culated tumor in the neonate. Derived from the ectoderm and mesoderm, this benign tumor generally has been classified as dermoid. Clinical symptoms depend on the size and location of the polyp.

CASE REPORT

A full-term girl was delivered by spontaneous vaginal delivery. She did not have respiratory distress, and her Apgar scores were 8 and 9. A bulky mass was noted to protrude from the mouth.

Examination showed a 5- x 2.5cm soft, nontender, skin-covered growth that protruded from the oral cavity (Figs 1 and 2). The tumor was attached to the left lateral oropharyngeal wall. The nasal cavity and nasopharynx appeared normal. Magnetic reso- nance imaging (MRI) showed a normal brain and no evidence of an encephalocele.

During surgery, it was noted that the stalk was attached to the superior pole of the left tonsil. The stalk was cut and suture-ligated. Pathological examination showed a S- x 2.5-cm polypoid mass covered by keritinized squamous epithelium, with hair follicles and sebaceous glands (Figs 3 and 4). The core of the lesion was primarily mature fibroadipose tissue. This histology was consistent with what has been described as an HP.

The infant was started on bottle feedings on the first post operative day and was discharged on the third day. She is well 12 months after excision, with normal feeding and slight asymmetry to

From the Department of Otolaryngology, Thomas Jefferson Univer- sity Hospital, Philadelphta, PA, and the Division of Otolaryngology and Depanments of Pathology and Neonatology, Alfred I. DuPont Children’s Hospital, Wilmington, DE.

Address repnnt requests to I. David Bough, Jr, MD, Department of Otolaryngologv-Head and Neck Surgery, Thomas Jefferson University Hospital, 909 Walnut St, Philadelphia, PA 19107.

Copyright o 1996 by W B. Saunders Company 0022-3468/9613105-0022$03.00/0

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the left lateral oral commissure. The asymmetry is related to pressure on that area during fetal development.

DISCUSSION

HP is the most common congenital tumor of the oropharynx and nas0pharynx.r The total number of documented HP cases is 135. The lateral wall of the nasopharynx and the superior surface of the soft palate house an estimated 60% of these tumors; while the oropharynx is the site of origin for the majority of the rest.233 The middle ear and mastoid cavity also have been sites of origin.4

The clinical presentation of the infant with HP is dependent on the size, shape, and location of the lesion. Signs and symptoms may include feeding difficulties, vomiting, intermittent mild respiratory difficulties, asphyxia, hemoptysis, unilateral Eusta- chian tube dysfunction, unilateral nostril drainage, and a detectable mass.2,3,5-8

HP usually presents as an isolated defect. Soft and hard palate clefts, agenesis of the uvula and external auricle, ankyloglossia, facial hemihypertrophy, left carotid artery atresia, and osteopetrosis are some rare associated anomalies.2,3 HPs are not associated with a particular congenital syndrome, nor are there any genetic predispositions or relationships. Neuro- logical complications can occur secondary to vascular compression ischemia.9 Malignant transformation of these tumors has not been noted.*

All newborns with symptoms of respiratory obstruc- tion must be evaluated for the presence of a pharyn- geal tumor (eg, HP, glioma, meningoencephalocele) and choanal atresia.lO More than half of the HPs have occurred in children under 12 months of age.

Radiological studies are crucial to help delineate the extent and origin of the lesion. Computed tomog- raphy (CT) and MRI scans are mostly used today. Bony anomalies and dehiscence are best identified by CT scanning; intracranial extension and sagittal im- ages are best demonstrated by MRI.7-9

Once the absence of intracranial extension has been established, plans for suture ligation and surgi- cal excision, with the patient under general anesthe- sia, should be undertaken. A tracheotomy may be necessary but should be avoided as much as possible.

HP was first described in 1918 as a club- or pear-shaped lesion that may be sessile but more often is pedunculated. The proximal pedicle is usually pink

JournalofPediafricSurgery, Vol31, No 5 (May), 1996: pp 704-706

HAIRY POLYP

Fig 1. Full-term neonate with a soft tissue mass protruding from the mouth.

and sharply demarcated from the gray or white distal bulk of the malformation. The HP is covered by skin and its associated appendages (eg, hair, sebaceous glands, and sweat glands); fibrofatty tissue accompa- nied by striated muscle, cartilage, blood vessels, nerve, lymph follicles, mucous glands, and serous glands comprise the bulk of the tumor. Tumors as large as 6 cm have been documented.5

Some confusion exists because HP has had multiple classif%zations (eg, teratoma, teratoid tumor, hamartoma) in the literature. Sexton even proposed the use of the term “choristoma,” signifying histologically normal tis- sue in an aberrant location.” Nevertheless, the constitu- ents of J3P are derived exclusively from the ectoderm and mesoderm and consequently the tumor is correctly classified as “dermoid” (see Table 1).

Fig 3. Gross specimen of the 5- x 2.5.cm mass.

Speculation surrounds the mechanism of HP forma- tion. Proposed theories state that these tumors arise from (1) disturbed development during the fusion of the epiblast of the stomodeum with the anterior foregut,5 (2) failure of the nasopharyngeal membrane

Fig 2. Stalk of mass is attached to the superior pole of the left tonsil.

Fig 4. Photmicrograph of the specimen shows a core of mature fibroadipose tissue covered by keritinizing squamous epithelium.

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Table 1. Classification of Nasopharyngeal Tumors According to Arnold

1. Dermoids (eg. hairy polyp): derived from epidermal and mesodermal germ layers

2. Teratoids: composed of poorly differentiated tissue derived from all three germ layers

3. Teratomas: trigerminal, like teratoids, but more differenti- ated; contain histologically recognizable organoid struc- tures. Both teratoids and teratomas may be associated with

anencephaly, hemicrania, and palatal fissures. 4. Epignathi: parasitic fetuses derived from all three germ

layers and usually incompatible with life

NOTE. Data from reference 13.

KELLY ET AL

to regress during the seventh week of gestation,12 (3) misdirected pleuripotential tissue,6 or (4) the first pharyngeal apparatus in germ cell rests.rr

CONCLUSION

HPs are uncommon developmental malformations that occur in infants and children. After thorough physical examination and radiological studies, surgi- cal excision is the treatment of choice. Significant morbidity can be avoided if early diagnosis and treatment are undertaken.

REFERENCES

1. Foxwell PB, Kelham BH: Teratoid tumors of the nasophar- ynx. J Laryngol72:647-657,1958

2. Chamlhry AP, Lore JM, Fisher JE, et al: So-called hairy polyps or teratoid tumors of the nasopharynx. Arch Otolaryngol 104:517-525, 1978

3. McShane D, El Sherif I, Doyle-Kelly W, et al: Dermoids (‘hairy polyps’) of the oro-nasopharynx. J Laryngol Otol 103:612- 615,1989

4. Boedts D, Moerman M, Marquet J: A hairy polyp of the middle ear and mastoid cavity. Acta Oto-Rhino-Laryngol Belg 46:397-400, 1992

5. Brown Kelly A: Hairy dermoid polyp of the pharynx and nasopharynx. J Laryngol Rhino1 Otol33:65-70, 1918

6. Calcaterra T: Teratomas of the nasopharynx. Ann Otol Rhino1 Laryngol78:165-171, I969

7. Kochanski SC, Burton EM, Seidel FG, et al: Neonatal

nasopharyngeal hairy polyp: CT and MR appearance. J Comput Assist Tomogr 146:1000-1001, 1990

8. Nicklaus P, Forte V, Thorner P: Hairy polyp of the eustachian tube. J Otolaryngol20:254-257,199l

9. Van Haesendonck J, Van de Heyning PH, Claes J, et al: A pharyngeal hairy polyp causing neonatal airway obstruction: A case study. Int J Pediatr Otorhinolaryngol 19:175-180,199O

10. Kanzaki S, Yamada K, Fujimoto M, et al: So called hairy polyp resembling an auricle. Otolaryngol Head Neck Surg 99:424- 426,1988

11. Sexton M: Hairy polyp of the oropharynx. A case report with speculation on nosology. Am J Dermatopathol12:294-298,199O

12. Badrawy R, Fahmy SA, Taha AM: Teratoid tumors of the nasopharynx. J Laryngol Otol87:795-799,1973

13. Arnold J: Ein Fall von Congenitalem Zusammengesetztem Lipom der Zunge und des Pharynx. Virchows Arch 50:482,1880