Figure 1: Showing surgical specimen of palatine tonsil with polyp.
Histologically, the polyp was covered by squamous epithelium and its stroma consisted of a variety of loose fibrous connective tissues, lymphoid aggregates and dilated lymphatic vascular channels (Figure 2A,B). The tonsil was slightly hypertrophic and inflamed. Immunohistochemical tests showed that the lymphatic vessels of the endothelium were reactive with anti-CD31 (Figure 3A) and anti-CD34 (Figure 3B). LCA was expressed in the lymphoid cells within the vascular channels and stroma. In these lymphoid cells, a polymorphous pattern with CD3 and CD20 were present. There was no sign of recurrence of the lesion in this case during the follow-up period of three years.
In the mentioned series, it was shown that the median age of the patients was 25 (range 363) years without a gender difference[2]. The clinical signs of the lymphangiomatous polyps were dysphagia, dyspnea, a sensation of a foreign body in the throat, sore throat, tonsillitis, and tonsillar mass[1,5]. The most important and only symptom was dysphagia in our case.
The underlying pathophysiological mechanism of polyp formation is not clearly known, but two hypotheses have been proposed. In the first theory, Visvanathan postulated that chronic inflammation results in irreversible obstruction of the lymphatic channels with congestion, eventually leading to the formation of the polyp[8,9]. Similarly, our case had recurrent tonsillitis attacks. In the second theory, Heffner has proposed that these lesions should be regarded as hamartomas in tonsillar tissue rather than as a neoplasm as these elements are normally found in the tonsillar fossa but arranged in a different pattern[1,10]. Tonsillar hamartomas may be found in different histologic spectra that polyps with lymphoid, fibrous, and/ or lymphangiomatous features[2,11].
A lymphangiomatous polyp is covered by squamous epithelium[3,10]. The polyp's stroma consists of different components that are interchangeable between loose to dense collagenous tissue, adipose tissue, prominent dilated lymphatic channels and lymphoid tissue[1,2]. Transformation to malignancy has never been reported in the literature. In our case, the lymphangiomatous polyp contained loose fibrous connective tissue, rich lymphocyte infiltration, and dilated lymphatic channels. Kardon et al found fatty tissue in 9 of 26 cases[2]. We did not observe any fat tissue.
Factor VIII-related antigens including CD31 and CD34 staining are uniformly positive in the dilated lymphatic vessel[5]. CD34 is less frequently reactive than CD31 for lymphatic vessels[2]. CD31 and CD34 positive endothelial cells were detected both in blood and lymph vessels in our case. The hematologic markers including CD3 and CD20 showed polymorphous infiltrate of the lymphoid cells within the vascular channels and stroma. However, immunohistochemistry is not needed for definitive diagnosis[2,3].
The differential diagnosis includes juvenile angiofibroma, squamous papilloma, and lymphangioma[2,3]. Juvenile angiofibromas that typically present with recurrent epistaxis mainly arise in male adolescents. These tumors are benign, locally aggressive, and extremely vascular. Juvenile angiofibromas have variably dense fibrovascular connective tissue containing staghorn-like, thin walled vessels[2]. Furthermore, tonsillar lymphangiomatous polyps mostly have a loose fibrous stroma and lymphocytic aggregates. Squamous papilloma is composed of exophytic fronds of thickened, squamous epithelial proliferation that are arranged in multiple layers and lack lymphatics and lymphocytic components[2,10]. Generally, lymphangiomas contain prominent dilated lymphovascular channels with proteinaceous fluid and less stroma than lymphangiomatous polyp[2,12]. Complete surgical excision of the mass and the tonsil is the curative treatment and recurrence was not reported after surgery[2].
In this paper, an unusual case of lymphangiomatous polyp arising from the tonsil is presented. It is important to diagnose a lymphangiomatous polyp as it clinically resembles a malignant neoplasm. Histopathological evaluation must be performed to establish its benign nature.
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