Case Report Published on June 28, 2023

 

Diffuse Large B Cell Lymphoma Mimicking Ulceroproliferative Lesion

Anila Melody Thomas1, J Kiranjith1, Krishnan K1

1.  Department of ENT, Sree Gokulam Medical College and Hospital, Venjarammoodu, Kerala*

 

ABSTRACT

Introduction: Oropharynx contains abundance of lymphoid tissue, particularly in the palatine and lingual tonsils which forms the anterolateral components of Waldeyers ring. Lymphomas arising in the oropharynx are almost exclusively Non Hodgkins lymphoma and arise in lymphoid tissue.

Methods: A case of 69 year old male with Non Hodgkins Lymphoma. Oropharynx, discussing clinical features, radiological and histopathological findings and management.

Discussion: Majority of the patients with Non Hodgkins Lymphoma presents with neck swellings, even though infections and inflammatory conditions can also lead to lymphadenopathy, with available modalities of investigations along with clinical examination, early diagnosis and treatment can be helpful to save life.

Results: A case of diffuse large B Cell Lymphoma with presenting complaints of neck swelling, odynophagia and dysphagia, mimicking oropharyngeal squamous cell carcinoma on clinical examination. Biopsy taken from the lesion showed diffuse large B Cell lymphoma. Patient was started on chemotherapy, but was unable to save life due to poor prognosis.

Conclusion: Non Hodgkins Lymphoma of Oropharynx can mimic Squamous Cell Carcinoma. Better patient awareness and early detection might have reduced the mortality. DLBCL has a poor prognosis.

 

Keywords:  Non Hodgkins Lymphoma, Diffuse Large B Cell Lymphoma

 

INTRODUCTION

Oropharynx  contains  abundance  of  lymphoid  tissue, particularly  in  the palatine  and  lingual  tonsils  which  forms  the  anterolateral  components of  Waldeyers  ring. Lymphomas  arising  in  the  oropharynx  are  almost exclusively  Non  Hodgkins  lymphoma  and  arise  in  lymphoid  tissue.

They  present  in  younger  patients (45-55 yrs), diagnosed  by  biopsy  and thereafter  treated  by  haematooncologists.

CASE PRESENTATION

69  old  Male  presented  with  complaints  of  swelling  of  5  x  5cms over  left  side  of  neck  of 1 month  duration, which  was  gradually progressing  in  size,  associated  with  occasional  pain. He  also  complained  of  dysphagia  &  odynophagia  since  10 days. There  was history  of  foreign  body  sensation  of  throat  and  change  in  voice  since  6  days. History  of  snoring  &  halitosis  was  present. He  also  had  left  ear  pain. Bilateral level 1b  and Left level V lymphnodes  were palpable. On Oral  cavity  examination, An  ulcer proliferative  lesion  involving   base  of  tongue  on  left  side  extending  laterally  to  the  left  tonsillar  fossa  and  anterior  pillar, continuing  into  the  soft  palate. Medially  it  was just  crossing  the  midline. It  was  hard  in  consistency with  induration,  non  tender,  slough  was  present  over  it. It  didn’t bleed  on  touch (Figure 1)

Figure 1 (a &b). Oral Cavity Examination (a), Confirmed by Videolaryngoscopy (b)

CECT Neck Axial cut showed heterogenously enhancing lesion occupying left parapharyngeal space, medially pushing the airway and laterally reaching till parotid and posteriorly till retropharyngeal space. Anteriorly it extended till retromolar trigone, involving left vallecula and   inferiorly it reaches just below hyoid bone (Figure 2)

Figure 2. CECT Neck Axial Cut showing lesion occupying Parapharyngeal Space

Peripheral  smear  showed  Neutrophilic  leucocytosis  with relative eosinophilia.

FNAC  from  Left  submandibular  lymph  node  showed  reactive lymphadenitis,  negative  for  atypical  or  malignant  cells.  

Figure 3. Biopsy from the lesion showing large cells with moderate to clear eosinophilic cytoplasm and enlarged pleomorphic vesicular nuclei with prominent nucleoli. Mitosis 3-4/HPF.
Necrosis present. Overlying epithelium was hyperplastic.

Punch  biopsy  taken  from  base  of  tongue  revealed  lymphoid  tissue lined  by  stratified  squamous  epithelium  with  underlying  neoplasm composed  of  diffuse  sheets  of  atypical  lymphoid  cells (Figure 3).

So was Started on chemotherapy with R -CHOP regimen, after immunohistochemistry  confirmation,  but  unfortunately  couldn’t  save the patient.

DISCUSSION

NHL is a group of neoplasms with lymphoreticular systems origin.

Corresponds to 0.2-2% of all the lymphomas. More  common  in males than females. Extranodal site of occurrence corresponds to 40%. Only  less  than  1% of all head and neck cancers. Occurance more in Tonsil > Nasopharynx > Base of tongue.

Risk  factors  include  Immunodeficiency, EBV,  Autoimmune  disease, Hepatitis  C,  H-pylori  infection. Prognostic factors  In addition to the REAL classification, a robust prognostic scoring system has been developed. It has long been appreciated that within the same lymphoma subtype there are factors which help predict patients with a poor outcome, for example the presence of ‘B’ symptoms (weight loss, night sweats and fevers). The International Prognostic Index (IPI) divides patients into four specific risk groups at diagnosis: high, high-intermediate, low-intermediate and low. The factors which make up the index  are:  age, performance status, stage of disease, number of extranodal sites of disease, serum lactate dehydrogenase.1

Diagnosis

The majority of patients present with painless lymphadenopathy. Although infections and inflammatory conditions can lead to lymphadenopathy, persistently enlarged nodes require surgical excision. Fine needle aspiration is not adequate if lymphoma is suspected, for several reasons:  Tumour cells do not always uniformly invade lymph nodes, so normal cytology does not exclude disease.  Reactive and malignant lymphocytes can be morphologically similar. It is increasingly important to subclassify NHL, for which fresh intact nodal tissue is ideal. Lymphomas may present in many other ways to the general physician, with an increasing number presenting in extranodal sites such as the gastrointestinal tract.

Many lymphoma subtypes are associated with specific cytogenetic abnormalities. For example:  (14;18) in follicular NHL  (8;14) in Burkitt’s NHL  (11;14) in mantle cell NHL2

With increasing application of molecular testing, particularly poly-merase chain reaction, and more recently fluorescence in situ hybridisation, it has become relatively easy to detect these translocations. This can be helpful to differentiate between reactive and malignant lymph nodes where the histology is inconclusive, but it can also be applied following therapy to detect evidence of minimal residual disease. The newly evolving technique of microarray analysis, with which multiple known translocations can be looked for simultaneously, may in future not only be used for NHL diagnosis but may lead to reclassification based on molecular abnormalities rather than on histological features.3

Treatment

Initial treatment has changed little over the last three decades for the majority of patients with NHL. Where disease is localised, radiotherapy is often employed irrespective of disease type. For aggressive lymphomas, the drug combination cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP) remains the gold standard. A number of  five- and six-agent regimens briefly appeared superior, but this was not confirmed in large phase III studies.5 Following relapse, salvage HDT, consolidated by an autologous transplant, is evidence-based standard therapy, but applicable only to younger patients. Treatment in indolent lymphoma is effectively palliative but, with median survivals of ten years in the commonest subtypes, the fact that the disease is not ‘curable’ may be irrelevant. Many drugs will induce remissions for these lymphomas. Increasingly, purine analogue drugs, particularly fludarabine, are being used in the indolent lymphomas, usually in the relapsed setting and often in combination with other chemotherapeutic agents. Purine analogues lead to a profound lymphocytopenia, which can last for many months post-therapy and predisposes these patients to opportunistic infections, thus making Pneumocystis carinii pneumonia prophylaxis essential.4

New approaches

Two potential advances in the management of NHL are becoming established in clinical practice. Monoclonal antibody therapy Lymphoma provides a good target for monoclonal antibody therapy as the malignant cells have abundant membrane-bound antigens confined to the tumour and renewable tissue. Antibodies against CD20, a membrane bound antigen found on many forms of B cell lymphoma, have been developed and are now licensed. This antibody binds to the cell surface and kills tumour cells through a number of mechanisms including cell-mediated and complement-mediated cytotoxicity and the induction of apoptosis. Treatment is given by weekly infusion, and is generally well tolerated although immediate hypersensitivity reactions can occur. However, it does not have the side effects commonly associated with chemotherapy, namely hair loss, nausea, vomiting and suppression of the bone marrow. Response rates of up to 60%have been seen in follicular lymphomas  when used as a single agent, but the exciting prospect is its combination with standard chemotherapy where response rates appear significantly better than with chemotherapy alone . In newer agents the anti-CD20 antibody is conjugated with a radioactive moiety which effectively delivers local radiotherapy directly into the tumour, often with impressive clinical result.5

Bone marrow (stem cell) transplantation

The other evolving field is bone marrow (stem cell) transplantation. Autologous transplantation is established in the management of relapsed aggressive NHL, and also in selected younger patients with more indolent diseases.

CONCLUSION

As  with  all  forms of  cancer,  it  is  important  for  patients  with  NHL to be referred to the  appropriate  local  clinic.  Patients  should  be  offered  a  multidisciplinary  approach  to  both  diagnosis  and  therapy. The latter may require  a  combination  of  treatments  including  chemotherapy, radiotherapy,  transplantation  and,  in  future,  immune-based  therapies.

End Note

Author Information

  1. Dr. Anila Melody Thomas,
    Post Graduate Resident,
    Department of ENT, Sree Gokulam Medical College and Hospital, Venjarammoodu, Kerala
  2. Dr J Kiranjith, Professor, Department of ENT,
    Sree Gokulam Medical College and Hospital, Venjarammoodu, Kerala
  3. Dr Krishnan K, Assistant Professor,
    Department of Ent, Sree Gokulam Medical College and Hospital, Venjarammoodu, Kerala

Conflict of Interest: None declared. 

REFERENCES

  1. Flint PW, Haughey BH, Lund VJ, Niparko JK, Robbins KT, Thomas JR, et al. Cummings Otolaryngology - Head and Neck Surgery E-Book. 6th edition. Saunders; 2014. 16273 p.
    [Source]
  2. Robbins cotran pathological basis of disease- 8 th edition pg no 145 to 170,chapter Neoplasia
  3. Scottbrown- 8 th edition,volume 3,pg no-207 to 237,chapter oropharyngeal malignancies,Barnes L,Eveson J W,Reichart P
  4. Liu C, Delaney S, Brown TN. An Unusual Presentation of B-Cell Lymphoma as a Large Isolated Epiglottic Mass: Case Report and Literature Review. Case Rep Otolaryngol. 2016;2016:9787432.
    [Crossref] | [Pubmed]
  5. Rule S. Non-Hodgkin’s lymphoma. Clin Med (Lond). 2001;1(5):362–4.
    [Pubmed] | [Crossref]