Mantle cell lymphoma: a rare cause for a swollen palate
- Ayah Mohamed ,
- Rachel Cowie and
- Dáire Shanahan
- Oral Medicine, University of Bristol Dental Hospital, Bristol, UK
- Correspondence to Dr Ayah Mohamed; ayah.mohamed@nhs.net
Abstract
We describe the case of a 68-year-old woman who was referred to the Oral Medicine Department in the Bristol Dental Hospital in November 2018 regarding a mass in the hard palate. The patient was previously diagnosed with stage IV mantle cell lymphoma of the upper tarsal conjunctiva in December 2014. Further investigations revealed lymphomatous deposits in other sites throughout the body. This patient underwent six cycles of chemotherapy, followed by consolidation LACE autograph and maintenance rituximab. While mantle cell lymphoma very rarely presents on the hard palate, knowledge of its clinical features and differential diagnoses is imperative in its accurate diagnosis and appropriate management.
Background
This article aims to raise awareness among clinicians about the oral presentation of mantle cell lymphoma (MCL). Although the oral cavity is a rare site for its presentation, knowledge of its clinical features and differential diagnoses is imperative in its accurate diagnosis and appropriate management. It is crucial that an oral examination is performed in all patients with suspected MCL to aid in its diagnosis and prevent unnecessary delays and investigations.
Case presentation
We present the case of a 68-year-old woman diagnosed with stage IV MCL of the upper tarsal conjunctiva bilaterally in December 2014 following presentation to the oculoplastic clinic complaining of unexplained epiphora for which she received radiotherapy. Three years later, she developed lymphomatous deposits in the perinephric cuff, bronchial tissues and bone marrow. She was referred to the Oral Medicine Department in the Bristol Dental Hospital regarding asymptomatic masses affecting the hard palate, which had been slowly growing since March 2018. On examination, hardened, non-tender, mobile swellings were noted bilaterally anterior to the parotids measuring 5–6 cm in diameter as well as lymphadenopathy of the left submandibular region. Symmetrical, spongy masses were noted on the hard palate extending from the canines to the second molars (figure 1).
Bilateral spongy tissue masses extending from the canines to the second molars were noted during initial assessment (published with the patient’s consent).
Investigations
Radiological imaging revealed no bony pathology associated with the palatal masses. A CT neck confirmed the presence of soft tissue swellings anterior to the parotids which had been noted as slowly enlarging since 2016 (figure 2).
Axial and coronal sections of CT scan taken prior to commencement of chemotherapy. Red arrows highlighting the soft tissue swellings anterior to the parotid glands (published with the patient’s consent).
Haematological investigations performed prior to initiation of chemotherapy revealed an elevated white cell, neutrophil, monocyte and lymphocyte count as well as raised creatinine levels (table 1).
Baseline haematological investigations performed prior to chemotherapy
| Patient's results | Normal range | |
| Calcium group | ||
| Albumin | 39 g/L | 35–50 |
| Calcium | 2.39 mmol/L | 2.20–2.60 |
| Adjusted calcium | 2.41 mmol/L | 2.20–2.60 |
| C reactive protein | 2 mg/L | <6.0 |
| Full blood count | ||
| White cell count | 32.02×109/L | 4.0-11 |
| Red blood cells | 3.94×1012/L | 3.80-5.30 |
| Haemoglobin | 120 g/L | 120-150 |
| Haematocrit | 0.372 L/L | 0.37-0.45 |
| MCV | 94.7 fL | 83-100 |
| MCH | 31.3 pg | 27.0-32.0 |
| MCHC | 331 g/L | 310-350 |
| Platelets | 207×109/L | 150-450 |
| Neutrophils | 10.31×109/L | 1.5-8.0 |
| Lymphocytes | 20.40×109/L | 1.0-4.0 |
| Monoctyes | 1.47×109/L | 0.2-1.0 |
| Eosinophils | 0.14×109/L | 0.0-0.5 |
| Basophils | 0.05×109/L | 0.0-0.2 |
| Magnesium | 0.92 mmol/L | 0.7–1.00 |
| Phosphate | 0.95 mmol/L | 0.80–1.50 |
| Lactate dehydrogenase | 365 U/L | 240–480 |
| Urea, creatinine, electrolytes | ||
| Sodium | 144 mmol/L | 133–146 |
| Potassium | 4.2 mmol/L | 3.5–5.3 |
| Urea | 6.8 mmol/L | 2.5–7.8 |
| Creatinine | 94 mmol/L | 45–84 |
| eGFR/1.73 m2 | 62 mL/min | |
| Liver function tests | ||
| Total bilirubin | 9 µmol/L | <21 |
| ALP | 112 U/L | 30–130 |
| ALT | 23 U/L | 10–50 |
| Total protein | 65 g/L | 60–80 |
| Globulin | 26 g/L | 22–36 |
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ALP, Alkaline phosphatase; ALT, Alanine transaminase; MCH, Mean corpuscular haemoglobin; MCHC, Mean corpuscular haemoglobin concentration; MCV, Mean Corpuscular Volume.
The decision was made to not biopsy the palatal swellings due to the urgency in commencing chemotherapy. Given her diagnosis of MCL, a tissue diagnosis at this stage would not have changed the management but would have posed additional risks, such as delayed healing and infection of the biopsy site.
Differential diagnosis
Provisional diagnoses of the palatal masses included salivary gland neoplasms such as mucoepidermoid carcinomas, adenoid cystic carcinomas and pleomorphic adenomas which commonly present as slowly enlarging, painless, rubbery soft tissue masses. Odontogenic cystic lesions were excluded due to the sound appearance of bone evident in the radiological imaging.
Oral lymphomas generally lack the systemic ‘B’ symptoms, such as night sweats, fevers and weight loss of most other lymphomas which was in keeping with the patient’s history and condition. As the patient had already been diagnosed with MCL, a provisional diagnosis of lymphomatous infiltration was made.
Outcome and follow-up
The patient underwent six cycles of chemotherapy, alternating between R-CHOP and R-DHAP followed by consolidation LACE autograph and maintenance subcutaneous rituximab therapy every 2–3 months to be continued for 3 years post initial treatment. She reported significant improvement of the palatal swelling 2 weeks following the first cycle of chemotherapy with the lesions exhibiting a complete response to therapy on review 8 months later, however long-term follow-up is planned (figure 3).
Complete resolution of hard palate lesions during follow-up following chemotherapy and LACE autograft (published with the patient’s consent).
Discussion
MCL is a distinct subtype of non-Hodgkin’s lymphoma with a historically aggressive clinical course and poor prognosis. It accounts for about 3%–10% of all non-Hodgkin lymphomas and typically occurs in middle-aged to older adults with a marked male predilection.1–3 In the head and neck region, the sites most commonly associated with MCL are the nasopharynx and Waldeyer’s tonsillar ring. The most common lymphoma in the palate is extra-nodal natural killer/T-cell lymphoma, which is frequently associated with Epstein-Barr virus.4 5
To the best of our knowledge, 14 cases of MCL affecting the palate have been described in the literature.4 6 Patients commonly present with lymphadenopathy, bone marrow involvement and gastrointestinal infiltration.7 Although MCL has rarely been noted in the oral cavity, the sites most implicated include the palate (65%), tongue (23%), gingivae and floor of the mouth (6%). Clinical presentations include a painless mass, upper airway obstruction, feeling of fullness in the tongue and ill-fitting denture.6
There are four histological variants of MCL: classical type, small cell type, pleomorphic type, and blastic type. The classic morphology usually consists of small-to medium-sized lymphocytes with irregular nuclear contours. MCLs have a distinct immunophenotype expressing pan B-cell markers but generally, the T-cell marker CD5 is also expressed, giving this tumour a characteristic profile. CD23, which differentiates chronic lymphocytic leukaemia from other B-cell neoplasms, are generally negative. The most sensitive and specific marker for MCL is cyclin D1, a growth-stimulating protein expressed after a translocation, of which almost all cases are moderate to strongly positive.6 8
Although recent progress has led to improvements in patient outcomes, MCL remains incurable, with a median overall survival of 4–5 years.8 With regards to treatment, several options exist dependent on factors such as the age and overall health of the patient. Treatment broadly consists of two components: cytarabine-containing induction chemotherapy followed by autologous stem cell transplantation (ASCT) as supported by the MCL Younger trial conducted by the European MCL Network.6 8 9 Prospective data conclude that rituximab maintenance provides a statistically significant overall survival advantage and is now considered a standard of care post-ASCT.10
In this particular case, as the diagnosis of MCL had already been established and the clinical presentation in the mouth was in keeping with this diagnosis, a biopsy was not performed as it could have potentially delayed chemotherapy had any complications occurred. Had the diagnosis been unclear or palatal masses not responded to initial therapy, a tissue diagnosis would have been sought urgently with incisional biopsy being the likely first choice investigation given the increased chance of retrieving a more representative sample.6
MCL rarely occurs in the oral cavity and shows a predisposition for the palate. Due to the poor overall prognosis, early identification is crucial in order to provide the best chance at any prolonged remission. An increased awareness of the various presentations of MCL among medical and dental clinicians is imperative to ensure an oral examination is performed in all patients with suspected MCL. This will aid in diagnosis and prevent unnecessary delays and investigations.
Patient’s perspective
When I first received the diagnosis of the mantle cell, it came as a bit of a shock. When I was initially seen, I was told by the ophthalmologist that it was cancer in my eyelids and that I would then be referred to the oncology people. By the time I met the oncologist, I had compiled a list of about 90 questions. At that stage, all they knew was that it was a type of non-Hodgkin’s lymphoma, but they wanted to narrow it down more. I had radiotherapy to my eyelids which was very quick and well organised. When I was told it was mantle cell lymphoma, the doctors I saw said they had never seen anything like it before which made me anxious. A few months later, I became aware of something in my mouth, in my palate. It was very spongy, almost as though it was full of liquid. I was worried that it was a gum boil and that it was going to burst. It was never painful but I did notice that when I had a fizzy drink, it would give me a strange sensation, it didn’t feel right. I’m not sure whether there just wasn’t enough room in my mouth for the fizz to go around because my mouth just felt so swollen. It affected my speech and made me quite lispy at the time. I noticed that whenever I put my tongue up to the roof of my mouth, I couldn’t make a clicking noise. I hadn’t put it down to the mantle cell at that stage, but I was hoping it wasn’t something else. I saw my dentist who said he had never seen anything like it before and so he referred me to the dental hospital. I felt like I was a bit of a walking experiment. When I eventually went to the dental hospital on the recommendation of the dentist, I was more worried that it wasn’t going to be connected to the mantle cell. I was extremely worried that they would have to do surgery to remove it. They took X-rays and told me that it wasn’t connected to my jaw and that it was more than likely the mantle cell in my mouth. I also had a CT because of the lumps in front of my ears. I noticed these lumps because when I wore my earrings, I couldn’t fully see them in the mirror because of the size of the lumps. By the time I got to the dental hospital the oncologists already had me down to starting treatment. I was just really glad that it was the mantle cell. I didn’t want to hear that it was another type of cancer. Again, I was told that they had never seen anything like this before. I was 100% relieved that it was nowhere else in my mouth and felt that there was a chance that it would be okay, that all the treatment I was having will knock out. Two weeks after the R-CHOP, things had improved and the swellings were completely gone. I felt that I could breathe better too. I was able to make the clicking noise with my tongue against the roof of my mouth again! I noticed the lumps on my face had gone down when I could see my earrings again. I still make that clicking sound with my tongue everyday to make sure it hasn’t come back. I realise how rare the mantle cell is because everyone I had seen kept telling me how they hadn’t seen anything like it before.
Learning points
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Although mantle cell lymphoma rarely presents in the palate, knowledge of its clinical appearance and possible differential diagnoses is crucial to ensure that no time is wasted on unnecessary investigations.
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Despite recent advances in the treatment of mantle cell lymphoma, it remains incurable with a poor long-term prognosis. As such, early identification is crucial to provide patients with the best chance at prolonged remission.
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It is imperative that an oral examination is performed in patients suspected of/diagnosed with mantle cell lymphoma and that regular dental follow-ups are arranged to allow for long-term monitoring of the oral cavity following initial treatment.
Footnotes
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Contributors All authors contributed to the drafting and approval of the manuscript.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Competing interests None declared.
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Patient consent for publication Obtained.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.
References
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