Primary Mantle Cell Lymphoma of the Tonsil: A Case Series

Background: Mantle cell lymphoma (MCL) is a mature B cell non- Hodgkin lymphoma (NHL) characterized by the t (11; 14) (q13; q32) translocation, which results in over-expression of cyclin D1. It comprises about 7% of NHL in the United States and Europe and its overall incidence appears to be increasing in the United States. While most patients present with advanced stage and generalized lymphadenopathy, 25% of patients with MCL present with extra-nodal disease. The most common site of involvement being gastrointestinal tract, liver and bone marrow. Isolated involvement of waldeyer's ring is rare. To date, only a few cases have been reported in the literature. Here we describe three patients with primary MCL of the tonsil, summarizing their presentation and clinical course. Method: We present a case series of MCL of the tonsils diagnosed and followed at a single center from August 2019-May 2020. Data regarding demographics, morphology, IHC stains, clinical presentation, radiographic findings, management and outcome are reviewed. Results: Three patients were diagnosed with tonsillar MCL. Clinical findings are summarized in table 1. All were men with a mean age of 63 years at diagnosis. None had B symptoms. They presented initially with swallowing difficulty and underwent tonsillectomy. They were found to have low risk pathology and clinical features suggesting the indolent form of MCL. Case 1: A 73 year-old with history of MEN-2 disorder presented with swallowing difficulty. A left tonsil mass was noted on examination and he underwent left tonsillectomy. Pathology confirmed diagnosis of MCL of the tonsil, with positive IHC stain for CD5, PAX5, cyclin D1 and SOX11, negative for CD10. The Ki-67 proliferative index was approximately 10-15%. PET-CT did not show any evidence of disease except for few non-specific level 1b and III left cervical nodes. Patient declined further staging work up, including a bone marrow biopsy and biopsy of a small left cervical lymph node to exclude other sites of involvement. He remains symptom-free on observation. Case 2: A 61 year-old presented with intermittent chocking sensation and difficulty swallowing. An enlarged right tonsil was observed on examination. He underwent right tonsillectomy and pathology was consistent with MCL, with positive IHC stain for CD5, cyclin D1 and SOX11, negative for CD 10. Ki-67 proliferative index was 30% and TP53 mutation was negative. Bone marrow evaluation showed 30% involvement with MCL, however, his peripheral cell counts were normal. PET-CT did not show any convincing evidence for other sites of involvement. We recommended watchful waiting and patient remains symptom free on close follow up. Case 3: A 55 year-old presented with dysphagia and sensation of a lump in his throat. He was noted to have bilateral tonsil enlargement. He underwent bilateral tonsillectomy. Pathology showed a population of B-cells with kappa light chain restriction and co-expression of CD5 and SOX 11 on the left tonsil only, consistent with diagnosis of MCL of left tonsil. The Ki-67 proliferative index was 20%. Bone marrow evaluation showed low level of involvement ~1% by MCL. PET-CT did not show any convincing evidence of disease elsewhere. We recommended watchful waiting and patient remains symptom free on close follow up, Conclusion: Primary MCL of the tonsils is an uncommon diagnosis. To the best of our knowledge this is the largest case series of this rare disease reported by a single institution. The demographics, histology and molecular features of our patients are similar to published data on MCL. Mainstay of therapy for MCL remains combination chemo immunotherapy which is not considered curative. Some studies have showed a prolonged median survival of 7 years in MCL patients treated nonaggressively, suggesting that a subset of patients may have an indolent course. Identification of this unique group of patients is challenging as there is no validated prognostic tool yet. In addition to MIPI, the Ki-67 score seems to be a powerful predictor of survival, with scores less than 30% showing prolonged survival. Our three patients presented with low Ki-67 score and low burden disease. They did not have B symptoms and were asymptomatic after surgery. Further studies are needed to assess whether MCL of the tonsil, without other dominant systemic disease, represents a unique clinical entity that predicts the indolent form of the disease which could be managed conservatively. Disclosures No relevant conflicts of interest to declare.

Measles Lymphadenopathy in a Child With PFAPA Syndrome

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a common cause of periodic fever in children. The pathogenesis of PFAPA is unknown but likely involves immune system dysregulation and may be initiated by an environmental trigger. Tonsillectomy resolves or improves symptoms in some patients, but the reason for this is unknown; moreover, specific abnormalities in tonsillectomy specimens from PFAPA patients have not been described. Here, we report measles virus in tonsil from a child with PFAPA. Measles-type viral cytopathic effect was discovered on histological examination of tonsillar tissue after therapeutic tonsillectomy for PFAPA. Molecular testing showed the left tonsil was positive for measles RNA by reverse transcription polymerase chain reaction (RT-PCR) while the right tonsil was inconclusive (weakly positive). Real-time RT-PCR specific for measles vaccine strain RNA (genotype A) was weakly reactive in the left tonsil tissue when tested in 3 independent replicates, but this result could not be confirmed with conventional genotyping by sequencing. The relationship and clinical significance between measles virus and PFAPA in this case is unclear but may be related to PFAPA-associated immune dysregulation. Additional investigation of measles virus in PFAPA patients would be helpful in further exploring this potential association.

Giant Tonsil Teratoma

The case presented is one extremely rare of giant tonsil teratoma with oropharynx localization, with palatine left tonsil origin, inducing respiratory failure, swallowing and phonetic disorders. This paper is based in a study case of a giant tonsil pediculous teratoma with slow evolution, with tolerancy, due to some diagnosis errors, developing respiratory failure and swallowing difficulties.

Lymphangiomatous Polyp Presenting as Tonsillar Mass

A 19-year-old female presented to ENT OPD of Dhulikhel Hospital, Kathmandu University Hospital, with history of foreign body sensation in throat for 2 weeks and mass in left tonsil for 1 week. There is no history of difficulty swallowing or recurrent throat infection. Physical examination revealed a pedunculated mass arising from upper pole of left tonsil. Bilateral tonsillectomy was done under general anaesthesia. Grossly, 2.5 × 1.5 × 1 cm polypoidal mass, soft in consistency, was found to be attached to left tonsil. Histopathology report was consistent with lymphangiomatous polyp of tonsil. Postoperative period was uneventful and she was normal during her follow-up at 1 month with bilateral healthy tonsillar fossa.

Lymphangiomatous Polyp of the Tonsil: An Unusual Cause of Snoring

ABSTRACT Benign vascular tumors and hamartomatous malformations of the palatine tonsils are less common than malignancies and are infrequently encountered. Lymphangiomatous polyp (LAP) is a rare hamartomatous lesion, arising from the surface of tonsil in children and young adults. They usually present with sore throat and dysphagia. Tonsillectomy with excision of polyp is the curative treatment. Histopathological examination will aid in the final diagnosis. We report a case of an adult male with LAP of left tonsil, who presented with snoring, sore throat, and change of voice. How to cite this article Bhat SP, P Kamath SD, Rent NH, Shetty KC. Lymphangiomatous Polyp of the Tonsil: An Unusual Cause of Snoring. Int J Otorhinolaryngol Clin 2015;7(2):88-90.

Fibroepithelial polyp of the tonsil: case report of a rare, benign tonsillar lesion

Objective:We report the case of a man presenting with a tonsillar mass causing difficult breathing, snoring and disturbed sleep.Method:Case report.Results:The patient was found to have a fibroepithelial polyp of the left tonsil.Conclusion:To our knowledge, this is the first case of its type to be presented in the English-language literature. The case is presented and the tumour and its management are discussed.

Unusual complication of tonsillectomy: taste disturbance and the lingual branch of the glossopharyngeal nerve

Taste disturbance is an unusual complication of tonsillectomy of which there are very few reports in the literature. The possible causes of this rare complication are: (1) direct or indirect damage to the glossopharyngeal nerve or its lingual branch (LBGN), (2) lack of dietary zinc, and (3) habitual drug intake.We report a 41-year-old man, who complained of taste disturbance following tonsillectomy that was performed for chronic tonsillitis and unilateral (left) tonsillar hypertrophy. During surgery, hypertrophic tonsils were found to be sited deeply into the tonsillar bed, especially at the lower pole of the left tonsil. Pathologic examination following tonsillectomy revealed a keratinous cyst and chronic infection at the left tonsil, and lymphoid hyperplasia and chronic infection at the right tonsil. Although his complaint had been getting better, qualitative examination of his taste function revealed bilateral impairment of the sense of sweet taste on the base of his tongue two months after the surgery, and a taste disturbance of sweet taste on the left side persisted the 10th month after the surgery. His serum zinc value was normal, and he did not take any drug that could affect his sense of taste. Depending on the literature data, possible indirect damage to the LBGN was suspected as the cause of the taste disturbance. This symptom may be reversible within two years after tonsillectomy, but it can also be irreversible. Therefore, tonsillectomy should be performed with minimal trauma to the tonsillar bed, especially when there is an additional pathology extending into the lower pole, and such a patient should be informed of the risk of post-operative taste disturbance after tonsillectomy as being one of the rare complications of this surgery.

Patient with primary tonsillar and gastric syphilis

A male patient with syphilitic lesions in the tonsil and stomach is presented. The patient was infected while practising oral sex with heterosexual friends. He complained of nausea and snoring; his left tonsil was enlarged. Spirochetes were detected in a smear preparation from the left tonsil. As a gastric lesion, initially believed to be cancer, appeared to result from spirochete ingestion, the case is considered to represent primary syphilis. After antibiotic therapy with ampicillin, the left tonsil returned to normal size and gastric changes were no longer evident endoscopically. Gastroscopy should be considered if syphilis of the tonsil is observed, particularly when gastrointestinal symptoms are present. Both the oral and the gastric lesion can be mistaken for malignant neoplasm.