Oral Lymphoma

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Enhanced CPD DO C OralMedicine

Olive FL Allsobrook

Issam Bakri, Paula M Farthing, Nick J Morley and Anne M Hegarty

Oral Lymphoma: A Case Series


Abstract: Oral presentation of lymphoma is rare but highlights the need for dentists in primary care to consider it for inclusion when
determining differential diagnoses for patients who present with oral lesions. Oral lymphoma manifests in both intra- and extra-osseous
sites within the oral cavity with a variety of clinical appearances which may mimic benign or innocuous processes. There may be non-
specific signs which may be misleading to the clinician or present mimicking other oral malignancies, such as a non-healing ulcer,
rapidly growing swelling with sudden onset or dental pathological processes. This case series highlights the varied presentations of oral
lymphoma and the role of general dental practitioners in the early diagnosis, timely referral and management of these patients.
CPD/Clinical Relevance: This article highlights the need for primary care clinicians to consider oral lymphoma in patients who present in
the general dental setting with oral lesions.
Dent Update 2018; 45: 641–644

This case series highlights the important malignant salivary gland tumours and develops in patients with a history of
role general dental practitioners play in oral lymphoma may present in the oral CLL, and Follicular Lymphoma (FL). These
the general screening and management cavity, and should raise clinical suspicion.1 patients presented to the Oral Medicine
pathways for rare oral malignancies outside Lymphomas encompass a broad range Department between 2012 and 2016
the routinely diagnosed common oral of cancers derived from lymphocytes with a variety of oral presentations, with
conditions, which may present as innocuous (immune cells). These can occur both varying degrees of systemic involvement
swellings, ulcers or bony lesions, without within lymph nodes and also extra-nodally and dissemination. All cases were
any systemic signs to raise suspicion. By within soft tissue and bone, including the ultimately managed by the Haematology
oral cavity. Lymphomas can be divided Department.
far the most common oral malignancy is
broadly into Hodgkin’s and Non-Hodgkin’s
squamous cell carcinoma however, rarely,
type based on cell morphology and the
presence of Reed-Sternberg cells (large Case reports (Table 1)
binucleated cells with a distinct histological From 2012 to 2016, five
Olive FL Allsobrook, BDS, DClinDent, appearance). Hodgkin’s lymphoma patients with oral lymphoma presented
Oral Pathology, Oral Medicine and develops in the lymphatics, within lymph to the Oral Medicine Department at
Surgery, Issam Bakri, DMD, MFDS, nodes, whereas Non-Hodgkin’s lymphoma Charles Clifford Dental Hospital. The
RCS(Eng) MSurgDent RCS(Eng), FDS (NHL) can present intra- or extra-nodally, patients were of an elderly age group,
RCPS, PhD, Oral Medicine and Surgery, including in the oral cavity.2 Both Hodgkin’s in their seventh and eighth decades,
Paula M Farthing, BSc, BDS, FDS and Non-Hodgkin’s lymphoma can be four female and one male, and all
RCS, PhD, FRCPath, FHEA, Oral and divided into further subtypes according had a variety of oral presentations of
Maxillofacial Pathology, Nick J Morley, to histological morphology and lineage. lymphoma (Figures 1−4). Three patients
BA, MBBS, MRCP, FRCPath, Haematology The majority of adult presentations of presented with DLBCL, one Richter’s
Department and Anne M Hegarty, Non-Hodgkin’s lymphomas are Diffuse transformation of CLL and one oral FL in
BDentSc, MSc(OM), MBBS, MFD, RCSI, Large B Cell Lymphoma (DLBCL),3 where various oral locations with varying clinical
FDS(OM) RCS, Oral Medicine and Surgery, the cell population is dominated by large sign and symptoms. Three patients had
The School of Clinical Dentistry, Sheffield neoplastic B cells. Five cases of extra- gingival swellings, one a history of a
and Charles Clifford Dental Hospital, nodal oral NHL are described, including painful palatal ulcer and one with an
Sheffield Teaching Hospitals Foundation DLBCL, Richter’s transformation of Chronic asymptomatic swelling of the upper lip.
Trust, UK. Lymphocytic Leukaemia (CLL), where DLBCL All five cases of oral lymphoma were
July/August 2018 DentalUpdate 641
OralMedicine

Case Sex Age Site of Referrer Presenting Symptoms Type of Outcome


lesion Lymphoma

1 F 70 Maxillary Oral Medicine 3 month history of right- DLBCL Received chemotherapy


alveolus sided headaches and facial Ongoing remission at
pain 2 years

2 M 64 Palate Rheumatology 4 week history of a painful DLBCL Received chemotherapy


palatal ulcer and a recently Ongoing remission at
loose-fitting upper denture 2½ years

3 F 68 Palate Haematology Extensive gingival swelling Richter’s Received palliative


of four months' duration transformation radiotherapy
of CLL Progressed with CNS
disease

4 M 65 Maxillary Primary care Gingival lesion on the left DLBCL Received chemotherapy
gingivae maxilla

5 F 63 Upper lip Primary care Asymptomatic lesion in the Follicular Monitoring


upper lip of eight months' lymphoma
duration

Table 1. Five patients with oral lymphoma presented to the Oral Medicine Department at Charles Clifford Dental Hospital from 2012 to 2016.

Figure 1. Case 1: Swelling right alveolus. Figure 2. Case 2: Right palatal ulcer. Figure 3. Case 3: Gingival swelling left palate.

initially diagnosed by oral histopathologists undergo malignant transformation or the soft palate and oropharynx), the
and confirmed by a specialist pathology manifest systemic signs of disseminated palate, maxilla and mandible.5 The most
lymphoma team using both morphological disease. NHL make up the majority of important factors affecting prognosis
and immunohistochemical (IHC) analysis of NHL oral cavity lymphomas which are of oral lymphoma are the histological
the tissue samples. The histological features mostly DLBCL,2 as was the case for the classification subtype and clinical stage
of lymphoma are highly variable. patients in this case study. Studies have at presentation.6 For oral lymphoma,
reported 58% of oral lymphomas are intra- or extra-bony presentation does not
DLBCL, have a mean age at presentation appear to have an effect on the prognostic
Discussion of 62−71 years and have an aggressive outcome.7 Oral lymphoma may present
Lymphoma makes up a small phenotype with a poor long-term as a solitary focus, but more commonly
but not insignficant proportion of oral prognosis.2,4 The most common oral site it may be a presenting manifestation of
malignancies. The oral cavity contains a is Waldeyer’s ring (an arch of lymphoid systemic and widespread disease, as was
large volume of lymphoid tissue which may tissue at the posterior junction of seen in some of the patients in this study,
642 DentalUpdate July/August 2018
OralMedicine

facial pain or numbness may be present in part of disseminated systemic disease, is


the absence of any organic signs, which will paramount for patients due to the poor
be useful in aiding clinicians to consider overall survival rates and hence the need
systemic disease or a malignant process.9 for prompt referral from primary care where
Imaging is critical and may reveal bone these patients may first present. Even with
lesions or oral presentation of metastatic continued monitoring, oral lymphoma is
disease, which should be included in the an aggressive disease process and many
differential diagnoses.9 patients require extensive long-term follow-
Treatment of DLBCL and FL up from multiple specialties to allow for
includes chemotherapy or radiotherapy, early diagnosis of disease progression.
with variable success; 70−80% of patients
with DLBCL achieve complete remission.7
In this case series the patients all presented Conclusion
in the seventh and eighth decades. The Oral lymphoma may rarely
majority of cases were diagnosed as DLBCL, present in the oral cavity either as a new
the most common oral presentation diagnosis or on a background of lymphoma
of lymphoma.10 NHLs show varied and in other body sites. Any unusual lesions
characteristic histopathological features manifesting in the oral cavity warrant
with distinctive responses to treatment expedited investigation, as malignant
therapies.11 DLBCL can be divided into two processes do not always cause the typical
groups with varying prognosis. Those with clinical systemic signs or symptoms. These
Figure 4. Case 4: Gingival lesion.
germinal centre B cell-like formation (GCB) cases highlight the need for a cautionary
tend to show a better clinical outcome than approach and high level of suspicion from
those that do not form germinal centres. primary care practitioners for prompt
Studies have shown oral lymphomas tend referral for the diagnosis and management
which would raise clinical suspicion to be non-germinal, centre-forming (non- of rapidly growing lesions. Despite the
for dentists treating these patients in GCB) and hence have a poorer prognosis.12 aggressive nature of DLBCL, there is a
primary care.6 Lymphoma is often a The revised World Health Organization good 5-year survival rate. This case series
relapsing remitting disease and many (WHO) classification of lymphoid neoplasms highlights to primary care clinicians the
patients with a history of lymphoma may has further classified these types according possible oral presentations of lymphoma,
be treated in the primary care setting to their prognostic outcome and is based and the important role that dentists can
on gene rearrangements diagnosed by play in identifying unusual presentations
after discharge from tertiary care whilst
gene expression profiling.13 and referring patients to a tertiary
in remission. These patients require
Richter’s syndrome can care centre for prompt diagnosis and
ongoing regular screening and a low
develop in patients with a previous CLL management of oral lymphoma.
clinical threshold for clinical suspicion
or small lymphocytic lymphoma (SLL).
of unusual or fast-growing lesions or
These patients undergo transformation of
swellings with no obvious dental cause.
malignant CLL cells to a high-grade NHL References
The patients in this case
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lymphoma can manifest with varied
CLL.14 Transformation has been reported cell lymphoma of the oral cavity with
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innocuous causes. Symptoms of atypical oral lymphoma, whether de novo or as a M. Characteristics of oral and paraoral
July/August 2018 DentalUpdate 643
OralMedicine

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Book Review and materials are discussed, such as of materials. Unfortunately, there is very
Craig’s Restorative Dental digital impression taking, CAD/CAM and little in the way of clinical application of
Materials 14th edn. Sakaguchi R, Ferracane orofacial implants. the materials themselves demonstrated
J and Powers J. Oxford: Mosby Elsevier, The text has been updated by a paucity of clinical photographs, one
2019 (340pp; £84.99, p/b). ISBN: 978-0-323- and revised to take account of the newer of which features a resin composite core
47821-2. materials and technologies, in particular retained by four dentine pins!! Instead,
First published in 1960, biomaterials and tissue engineering. It images of products are presented but
Sakaguchi et al’s textbook on restorative is a little surprising what subject matter with a distinct and perhaps unconscious
dental materials is now on its 14th edition, has been included and omitted. The bias towards 3M products, which may
which is a measure of the success of section on dental amalgam has rightly be explained by the fact that one of the
previous editions. It is also a reflection of been reduced to reflect the phase down contributors has an association with
the need to keep abreast of developments of this material in many countries across that particular company. There is a lot of
in this ever and quickly changing subject. the globe. However, quartz-tungsten- written prose and therefore information
The book is comprehensive, halogen curing lights are discussed in which is extensively referenced at the end
featuring sections on the full range of depth, whereas more emphasis could of each chapter. The text is appropriately
restorative dental materials, and begins have been placed on the now more illustrated with many quality diagrams and
by putting their role into context. commonplace LED units. There was also photomicrographs.
Useful and helpful sections follow on a lack of information on the tricalcium In summary, Craig’s Restorative
the fundamentals of materials science, silicate cements such as MTA. These Dental Materials would be a good addition
materials testing and biomechanics, products have revolutionized endodontics to the library of any practising dental
which are very readable and easy to since their introduction and their usage clinician, postgraduate student or keen
understand. There is a chapter which continues to increase apace. This may undergraduate student who has an interest
deals with biocompatibility and tissue illustrate the point that textbooks are out
in dental materials or dental materials
reaction to biomaterials before the authors of date before they are published!
science.
go systematically through all classes of It is fair to say that this
restorative dental materials used in both tome is really a dental material’s science Steve Bonsor
GDP, Aberdeen and Hon Senior
the clinic and laboratory. In the last three textbook with its emphasis very much Clinical Lecturer, University of
chapters, more contemporary techniques on the physics, chemistry and mechanics Aberdeen

644 DentalUpdate July/August 2018

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