ASYMMETRIC TONSIL HYPERTROPHY: CASE REPORT OF LYMPHANGIOMA OF THE TONSIL
Lymphangiomas are benign lymphatic tumours typically composed of dilated lymphatic channels, which often occur subcutaneously in the head and neck region. Tonsillar lymphangiomas have been reported rarely in the literature and on clinical examination are indistinguishable from malignant tonsillar tumours. Histological confirmation is essential for diagnosis. We report a case of lymphangioma of the tonsil in an otherwise well 22 year old man and review the clinical and histological features of this rare tumour.
A 22 year old man presented with a 2 month history of recurrent odynophagia, associated with left-sided otalgia and intermittent low grade fevers. He had been treated with several courses of oral antibiotics prior to his referral with no improvement. He reported constant left sided pharyngeal discomfort and described several episodes in the previous week of spitting out fresh blood in his saliva. He was otherwise in excellent health, being a professional sportsman, with no other symptoms to report. He was a nonsmoker with less than 10g of alcohol consumed per week.
Examination of his oropharynx revealed marked unilateral hypertrophy of his left tonsil, which was firm on palpation with an area of shallow surface ulceration ~2cm in diameter with a sloughy, friable base. There was no associated trismus. The right tonsil was much smaller in size with a normal appearance. There was no cervical lymphadenopathy palpable and full head & neck examination detected no other abnormal findings.
FBE and CXR were normal. An examination under anaesthesia and bilateral tonsillectomy was performed. No other sites of pathology were detected. He made an uncomplicated postoperative recovery and is clinically well 18 months after removal of the lesion. Histopathological examination of the specimen revealed a lymphangioma of the left tonsil, which was completely excised with no evidence of spread beyond the tonsillar capsule. Microscopy showed a large polypoidal mass containing multiple vascular spaces lined by endothelial cells and filled with lymph (Figures 1 & 2). The surrounding stroma was infiltrated by plasma cells and scattered aggregates of lymphocytes, forming follicles with germinal centres. The overlying mucosa was partially ulcerated but otherwise normal appearing stratified squamous epithelium. There were no atypical features or evidence of malignancy and the mass was surrounded by normal tonsillar tissue.
Lymphangiomas are benign tumours which have been reported to occur rarely in the tonsil, with only 31 cases documented in the English literature (Medlinc 1966-2003), with 26 of these in a single scries from the Armed Forces Institute of Pathology (AFIP), in the U.S.1-5 Lymphangiomas occur in the head & neck region in ~90% of cases, being located primarily in the skin or subcutaneous tissue. They classically occur in association with congenital malformations of the lymphatics, presenting in childhood as cystic masses (cystic hygroma). They may also occur in adults in varied locations including the oral cavity, tongue, larynx or parotid/’ The majority of previously reported cases involving the palatine tonsils were pedunculated or polypoidal lesions with histological features consistent with those noted in this case. Surgical excision via tonsillectomy provides definitive management for these lesions, with patients undergoing an uncomplicated postoperative recovery. There have been no reported instances of disease recurrence after complete excision.
The differential diagnosis of unilateral tonsillar hypertrophy includes acute or chronic inflammation (including granulomatous change); malignant neoplasms, most commonly lymphoma or squamous cell carcinoma; benign lesions including haemangiomas, papillomas or lymphangiomas or parapharyngeal masses which may give rise to apparent tonsillar enlargement due to medial displacement of the tonsil. This case was clinically suggestive of a tonsillar malignancy and accurate diagnosis of a lymphangioma is only possible with histological confirmation. Although histological diagnosis was straight forward in this case, these lesions may be difficult to differentiate from papilloma, fibroepithelial polyps or juvenile angiofibroma depending on the degree of proliferation of vascular channels, fibrous stroma or lipomatous tissue.1
The true incidence of tonsillar lymphangiomas is difficult to determine from reviewing the literature with several authours suggesting these lesions may occur more commonly than reported, possible accounting for 1.9% of all tonsillar tumours.1 Historically these tumours may have been reported as fibroangiomas or llbrolipomas depending on the histological components seen. This particular case demonstrated prominent lymphatics with luminal proteinaccous fluid and lymphocytes within normal tonsillar tissue with no lipomatous tissue and a paucity of fibrous tissue.
The pathogenesis of tonsillar lymphangioma is uncertain. It has been proposed that these lesions develop as a result of chronic inflammation interfering with lymphatic drainage- but this does seem unlikely given the rarity of these tumours in comparison to the frequency of recurrent tonsillitis in the general population. Another possibility is that these lesions represent harmartomatous proliferation of histological elements seen in normal tonsillar tissue which seems more probable.3
Tonsillar lymphangioma is an unusual pathological entity which has been reported rarely although its incidence may be higher than previously thought. These lesions present with unilateral, polypoidal enlargement of the tonsil and can not be differentiated clinically from malignant tonsillar tumours. It is essential that these lesion be examined histologically after surgical excision to establish the diagnosis and thus exclude tonsillar malignancy, which would require more extensive treatment. Tonsillectomy is curative for tonsillar lymphangioma with no episodes of recurrence having been reported.
1. KARDON D.E., WENIG B.M., HEFFNER D.K., THOMPSON L.D. Tonsillar Lymphangiomatous Polyps: A Clinicopathologic Series of 26 cases. Mod Pathol 2000; 10:1128-1133
2. VISVANATHAN P.G. A pedunculated tonsillar lymphangioma J Laryngol Otol 1971;85:93-96
3. AL SAMARRAE S.M., AMR S.S., HYAMS V.J. Polypoid lymphangioma of the tonsil J Laryngol Otol 1985; 99:819-823
4. ABU SHARA K., AL-MUHANA A., AL-SHENAWY M. Harmartomatous tonsillar polyp J Laryngol Otol 1991; 105:1089-1090
5. ROTH M. Lymphangiomatous polyp of the palatine tonsil Otolaryngol Head Neck 1996; 1 15:172-3
6. STAL S., HAMILTON S., SPIRA M. Haemangiomas, lymphangiomas and vascular mal formations of the head and neck. Otolaryngol Clin North Am 1986: 19:769-796
S. BOARDMAN, J. CUROTTA and M.J. DA CRUZ
Department of Otolaryngology, Westmead Hospital, Sydney, Australia
M.J. Da Cruz
Department of Otolaryngology, Westmead Hospital, University of Sydney
M.J. da Cruz,
C/o Department of Surgery,
Westmead, New South Wales 2145
Copyright Australian Society of Otolaryngology Head & Neck Surgery Ltd. Jun 2004
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