|Year : 2013 | Volume
| Issue : 3 | Page : 121-123
Pharyngeal fibromatosis presenting as adenoid hypertrophy: A case report and review of literature
Segun Segun-Busari1, Adekunle David Dunmade1, Biodun Sulyman Alabi1, Kayode Adebamiji Adeniji2, Olushola Abdulrahman Afolabi1, Habeeb Kayodele Omokanye1, Isaac Olarewaju Ajayi1
1 Department of Otorhinolaryngology, College of Health Sciences, Faculty of Clinical Sciences, University of Ilorin, Ilorin, Kwara State, Nigeria
2 Department of Histopathology, College of Health Sciences, Faculty of Clinical Sciences, University of Ilorin, Ilorin, Kwara State, Nigeria
|Date of Web Publication||22-Nov-2013|
Department of Otorhinolaryngology, College of Health Sciences, Faculty of Clinical Sciences, University of Ilorin, Ilorin, Kwara State
A 10-year-old girl presented with a noisy breathing noticed from birth and failure to thrive. Examination revealed a huge lobulated firm mass in the oropharynx (toward the left side). Plain radiography of the neck showed a huge circumscribed pharyngeal mass extending from the base of the tongue to the level of the fourth cervical vertebrae. The mass measuring 6 cm Χ 5 cm was excised through a left lateral pharyngotomy. Histological examination showed sections of highly cellular interlacing bundles of fibroblast with moderate hyalinization extending to the surrounding fibro-fatty tissue. The cells have spindle nuclei with tapering ends. Occasional mitosis and in some areas myxoid degeneration were seen. A diagnosis of pharyngeal fibroma was made. Patient had an uneventful post-operative recovery with complete resolution of symptoms for the past 6 years. This rare benign tumor has not previously been reported at this site and may thus give rise to difficulty in diagnosis or to a misdiagnosis.
Keywords: Fibromatosis, adenoid, pharynx
|How to cite this article:|
Segun-Busari S, Dunmade AD, Alabi BS, Adeniji KA, Afolabi OA, Omokanye HK, Ajayi IO. Pharyngeal fibromatosis presenting as adenoid hypertrophy: A case report and review of literature. Sahel Med J 2013;16:121-3
|How to cite this URL:|
Segun-Busari S, Dunmade AD, Alabi BS, Adeniji KA, Afolabi OA, Omokanye HK, Ajayi IO. Pharyngeal fibromatosis presenting as adenoid hypertrophy: A case report and review of literature. Sahel Med J [serial online] 2013 [cited 2021 Sep 27];16:121-3. Available from: https://www.smjonline.org/text.asp?2013/16/3/121/121923
| Introduction|| |
Cervicothoracic lesions are not uncommon in children.  The lesions could be congenital or acquired. The acquired ones could be traumatic, inflammatory or neoplastic. Lymphangioma is the most common cervicothoracic mass in children  while fibromatosis is one of the rarest. ,,, This uncommon spindle cell neoplasm is generally associated with serosal surfaces, especially the pleura. Very few cases of oropharyngeal fibromatosis has been reported in the literature. 
We present a 10-year-old girl with pharyngeal fibromatosis.
| Case Report|| |
A 10-year-old girl presented with noisy breathing noticed from birth; and failure to thrive. The noisy breathing was worse when patient was asleep. She had no nasal obstruction or discharge. There was no change in voice or otologic symptoms. There was no other significant medical history.
Examination revealed a huge, pink, lobulated, firm and non-pulsatile mass in the oropharynx (toward the left lateral side). It was separate from the uvula and soft palate; one could not get below or above it and the overlying mucosa was smooth.
Soft-tissue radiograph of the neck revealed a huge circumscribed mass in the pharynx extending from the base of the tongue to the level of the fourth cervical vertebrae [Figure 1]. Patient could not afford a computerized tomography scan. Nasoendoscopy was not available at the time of presentation.
|Figure 1: Soft-tissue X-ray of the neck showing a huge circumscribed mass in the pharynx (arrow points the mass in the pharynx)|
Click here to view
Examination under anesthesia revealed a firm mass extending from the base of the tongue to the left tonsillar fossa and then to the post-nasal space. There was no ulceration.
Complete excision of the mass (measuring 6 cm × 5 cm) was achieved via a left lateral pharyngotomy [Figure 2]. The mass was yellowish, multilobulated; solid in areas.
|Figure 2: Left lateral pharyngotomy incision. Excised mass measuring 6 cm in length|
Click here to view
Histopathology showed sections of highly cellular interlacing bundles of fibroblast, with moderate hyalinization, extending to the surrounding fibro-fatty tissue [Figure 3]. The cells have spindle-nuclei with tapering ends. Occasional mitosis and myxoid degeneration were seen in some areas. These features were consistent with a diagnosis of pharyngeal fibromatosis. Patient had an uneventful post-operative recovery with complete resolution of symptoms for the past 6 years.
|Figure 3: Fibromatosis section shows proliferating well-differentiated fi broblast with large amount of collagen between the cells. No cytologic features of malignancy (H and E, ×400)|
Click here to view
| Discussion|| |
Fibromatosis is a locally infiltrative fibrous tissue proliferation with a tendency to recurrence. It is of a rare entity in the head and neck. 
Pharyngeal fibroma is particularly rare. Being a slow growing tumor, growth may occur in a period of several years, prior to overt clinical manifestation. They are virtually always polypoidal and may arise from any site in the pharynx or upper esophagus. They tend to be dragged downward by the normal process of swallowing; thus, forming a large pedunculated mass hanging down the pharynx or esophagus. A typical presenting symptom is dysphagia. They may produce choking sensation and if the tumor is displaced upward and anteriorly it can fall into the larynx and cause dyspnea.
A high index of suspicion and thorough physical examination is essential especially in the early stages of tumor growth since clinical symptoms are few. Computerized tomography scan is required to confirm the diagnosis pre-operatively. The scan will show a mass with a characteristic low attenuation value.
The tumors have a very small pedicle and can often be removed endoscopically using a snare to divide the pedicle orthrough a lateral pharyngotomy.
Histology display highly cellular interlacing bundles of fibroblast that are moderately hyalinized and extending to the surrounding fibro-fatty tissue. The cells have spindle nuclei with tapering ends and occasional mitosis.
Preliminary investigations should include lateral neck X-ray examination and a barium swallow.  Specific pre-operative diagnosis can be achieved using computerized axial tomography, which not only correctly localize the site and extent of the lesion, but also reveals the nature of the lesion by virtue of its characteristically low attenuation value. , Magnetic resonance imaging is the method of choice for assessing the full extents of cervicothoracic lesions and their relationships to neurovascular structure.  Fibromyxoma may be asymptomatic or presents with dysphagia and dyspnea. , Since these neoplasms are benign, the treatment of choice is complete surgical extirpation, the approach being determined by the size and anatomical location of the individual lesion.  Malignant transformation is generally rare, although recurrence of the tumor may be encountered, depending on the extent of surgical excision. ,
From a large series of head and neck patients treated between 1977 and 1994 in our institute, nine adult patients were diagnosed with this disease. This illustrates the rarity of this head and neck tumor. 
Aggressive fibromatosis is an uncommon tumor that is locally aggressive, but not malignant. In the past, these lesions were misdiagnosed as low-grade fibrosarcomas. We have previously described two cases of aggressive fibromatosis of the parapharyngeal space. 
| References|| |
|1.||Castellote A, Vázquez E, Vera J, Piqueras J, Lucaya J, Garcia-Peña P, et al. Cervicothoracic lesions in infants and children. Radiographics 1999;19:583-600. |
|2.||Grand S, Lantuejoul S, Ferretti G, Reyt E, Le Bas JF. Fibromyxoma of the retropharyngeal space: MR appearance with pathologic correlation. AJNR Am J Neuroradiol 1998;19:1793-5. |
|3.||Gangopadhyay K, Taibah K, Manohar MB, Kfoury H. Solitary fibrous tumor of the parapharyngeal space: A case report and review of the literature. Ear Nose Throat J 1996;75:681-4. |
|4.||Porter MJ, Suen WM, John DG, van Hasselt CA. Fibromatosis of the parapharyngeal space. J Laryngol Otol 1994;108:1102-4. |
|5.||Al-Sinawi A, Johns AN. Parapharyngeal solitary fibrous tumour: An incidental finding at ENT examination. J Laryngol Otol 1994;108:344-7. |
|6.||Jesberg N. Fibrolipoma of the pyriform sinuses: Thirty-seven year follow-up. Laryngoscope 1982;92:1157-9. |
|7.||Som PM, Scherl MP, Rao VM, Biller HF. Rare presentations of ordinary lipomas of the head and neck: A review. AJNR Am J Neuroradiol 1986;7:657-64. |
|8.||D'Auria T, Santoro S, Gambardella A, D'Amico A, Di Salle F. Lipoma of the hypopharynx: Role of computerized tomography in the diagnosis protocol. Acta Otorhinolaryngol Ital 1990;10:87-92. |
|9.||Di Bartolomeo JR, Olsen AR. Pedunculated lipoma of the epiglottis. Second known case reported. Arch Otolaryngol 1973;98:55-7. |
|10.||Eagle WW. Lipoma of the epiglottis and lipoma of the hypopharynx in the same patient. Ann Otol Rhinol Laryngol 1965;74:851-62. |
|11.||Plaat BE, Balm AJ, Loftus BM, Gregor RT, Hilgers FJ, Keus RB. Fibromatosis of the head and neck. Clin Otolaryngol Allied Sci 1995;20:103-8. |
|12.||Sanders KW, Fowler MR, Milner J, Stucker FJ, Nathan CO. Aggressive fibromatosis of the parapharyngeal space: Two cases and treatment recommendations. Ear Nose Throat J 2004;83:262,264, 266. |
|13.||Sharma A, Ngan BY, Sándor GK, Campisi P, Forte V. Pediatric aggressive fibromatosis of the head and neck: A 20-year retrospective review. J Pediatr Surg 2008;43:1596-604. |
[Figure 1], [Figure 2], [Figure 3]