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U n ive rs ity Jou rna l o f D en ta l S c ien ces , An O ffic ia l P ub lica tion o f A liga rh M us lim U n ive rs ity, A liga rh . Ind ia 88 U n ive rs ity J D en t S c ie 20 15 ; N o . 1, Vo l. 3 Abstract : Non Hodgkin's lymphoma (NHL) belongs to a group of lymphoid neoplasm that is diverse in manner of presentation, response to therapy & prognosis. NHL cases might occur extra- nodally and in 3% of these cases the initial presentation may be in the oral cavity. Extra-nodal NHL of the oral cavity is rare but patients with oral lesions of NHL commonly present at the dental clinic in the first instance. Thus a careful clinical and histopathological examination along with other laboratory investigations will help in diagnosing the disease at an early stage, resulting in better prognosis. Usually the oral manifestation of NHL is secondary to a more widespread involvement throughout the body. Other times it may represent a primary extra nodal disease confined to oral cavity or jaws. This case report presents a painless swelling in the mandibular left posterior gingival region which unusually came out to be NHL on histopathological & immunohistochemical 1 Aparna Dave Professor, Department of Oral & Maxillofacial Pathology, SGT Dental College & 2 Hospital, Gurgaon. India, V.P. Shetty Prof& HOD, Department of Oral & Maxillofacial Pathology. 3 SGT Dental College & Hospital, Gurgaon. India, Pulin Saluja Senior Lecturer, Department of Oral 4 & Maxillofacial Pathology, SGT Dental College & Hospital, Gurgaon. India, Manpreet Arora Reader, Department of Oral & Maxillofacial Pathology. SGT Dental College & Hospital, Gurgaon. India INTRODUCTION Lymphomas are heterogeneous group of clonal malignant disease with a spectrum of behavior ranging from relatively indolent to highly aggressive and potentially fatal. They share the single characteristic of arising as the result of somatic mutation in a lymphocytic progenitor1. Lymphomas unlike leukemia are solid tumors. A broad division of the group into Hodgkin Lymphoma and Non Hodgkin Lymphoma (NHL) is widely accepted. Extranodal presentation of Hodgkin lymphoma is rare but NHL is relatively common group of neoplasm2 that presents up to 40% of time at an extra nodal site. 2% to 3% of these extra nodal cases may primarily arise in oral cavity and jaws. Waldeyers ring is more frequently involved in head and neck region. Orbit, salivary glands are the other sites of involvement3. Oral NHL can involve the paranasal sinuses- but can also arise from within the soft tissue or bone, floor of the mouth and cheek. However the involvement of the gingiva is relatively uncommon. Owing to scarcity of reported cases of oral lymphomas, the interpretation of the biological- behavior and treatment options involving this disease entity becomes difficult. A thorough clinical, histopathological, and immunohistochemical evaluation is therefore essential for the diagnosis and management of oral NHL. Case report A 65 year old male patients reported to the OPD with complain of painless growth and mobility in relation to 33, 34 and 35. The growth was of 3 months duration. On examination, the patient was afebrile, gave no history of weight loss and did not exhibit any palpable lymph nodes. On intraoral examination, an ill defined gingival growth, pink in color, irregular in shape, soft to firm in consistency was seen extending from mandibular left lateral incisor to mandibular left premolar region and also involved the lingual vestibule (Figure 1). Figure 1: Intraoral picture showing gingival swelling wrt left mandibular canine premolar region. PRIMARY EXTRA-NODAL B - CELL TYPE OF NON HODGKIN'S LYMPHOMA MANIFESTING AS GINGIVAL SWELLING IN POSTERIOR MANDIBLE : A RARE CASE REPORT. Journal of Dental Sciences University Key words : Non Hodgkin Lymphoma, extra nodal, neoplasm lymphocytic progenitor Source of support : Nil Conflict of Interest : None Case Report
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Page 1: 19 PRIMARY EXTRA-NODAL B - Aligarh Muslim …Figure 2: Panoramic radiograph showing ill defined radiolucency with moth eaten appearance with respect to edentulous region of 36 37 and

University Journal of Dental Sciences, An Official Publication of Aligarh Muslim University, Aligarh. India 88

University J Dent Scie 2015; No. 1, Vol. 3

Abstract : Non Hodgkin's lymphoma (NHL) belongs to a group of lymphoid neoplasm that is diverse in manner of presentation, response to therapy & prognosis. NHL cases might occur extra-nodally and in 3% of these cases the initial presentation may be in the oral cavity. Extra-nodal NHL of the oral cavity is rare but patients with oral lesions of NHL commonly present at the dental clinic in the first instance. Thus a careful clinical and histopathological examination along with other laboratory investigations will help in diagnosing the disease at an early stage, resulting in better prognosis. Usually the oral manifestation of NHL is secondary to a more widespread involvement throughout the body. Other times it may represent a primary extra nodal disease confined to oral cavity or jaws. This case report presents a painless swelling in the mandibular left posterior gingival region which unusually came out to be NHL on histopathological & immunohistochemical

1Aparna Dave Professor, Department of Oral & Maxillofacial Pathology, SGT Dental College & 2Hospital, Gurgaon. India, V.P. Shetty Prof& HOD, Department of Oral & Maxillofacial Pathology.

3SGT Dental College & Hospital, Gurgaon. India, Pulin Saluja Senior Lecturer, Department of Oral 4& Maxillofacial Pathology, SGT Dental College & Hospital, Gurgaon. India, Manpreet Arora

Reader, Department of Oral & Maxillofacial Pathology. SGT Dental College & Hospital, Gurgaon. India

INTRODUCTION Lymphomas are heterogeneous group of

clonal malignant disease with a spectrum of behavior ranging

from relatively indolent to highly aggressive and potentially

fatal. They share the single characteristic of arising as the

result of somatic mutation in a lymphocytic progenitor1.

Lymphomas unlike leukemia are solid tumors.

A broad division of the group into Hodgkin Lymphoma and

Non Hodgkin Lymphoma (NHL) is widely accepted.

Extranodal presentation of Hodgkin lymphoma is rare but

NHL is relatively common group of neoplasm2 that presents

up to 40% of time at an extra nodal site. 2% to 3% of these

extra nodal cases may primarily arise in oral cavity and jaws.

Waldeyers ring is more frequently involved in head and neck

region. Orbit, salivary glands are the other sites of

involvement3. Oral NHL can involve the paranasal sinuses-

but can also arise from within the soft tissue or bone, floor of

the mouth and cheek. However the involvement of the gingiva

is relatively uncommon. Owing to scarcity of reported cases

of oral lymphomas, the interpretation of the biological-

behavior and treatment options involving this disease entity

becomes difficult. A thorough clinical, histopathological, and

immunohistochemical evaluation is therefore essential for the

diagnosis and management of oral NHL.

Case report

A 65 year old male patients reported to the OPD with

complain of painless growth and mobility in relation to 33, 34

and 35. The growth was of 3 months duration. On

examination, the patient was afebrile, gave no history of

weight loss and did not exhibit any palpable lymph nodes.

On intraoral examination, an ill defined gingival growth, pink

in color, irregular in shape, soft to firm in consistency was

seen extending from mandibular left lateral incisor to

mandibular left premolar region and also involved the lingual

vestibule (Figure 1).

Figure 1: Intraoral picture showing gingival swelling wrt left

mandibular canine premolar region.

PRIMARY EXTRA-NODAL B - CELL TYPE OF NON HODGKIN'S LYMPHOMA MANIFESTING AS GINGIVAL SWELLING IN POSTERIOR MANDIBLE : A RARE CASE REPORT.

Journal of Dental Sciences

University

Key words : Non Hodgkin Lymphoma, extra nodal, neoplasmlymphocytic progenitor

Source of support : NilConflict of Interest : None

Case Report

Page 2: 19 PRIMARY EXTRA-NODAL B - Aligarh Muslim …Figure 2: Panoramic radiograph showing ill defined radiolucency with moth eaten appearance with respect to edentulous region of 36 37 and

Figure 2: Panoramic radiograph showing ill defined

radiolucency with moth eaten appearance with respect to

edentulous region of 36 37 and floating tooth wrt 38

Radiographically ill defined radiolucency in edentulous

region wrt 36,37 was seen with moth eaten appearance along

the superior region, floating tooth appearance wrt 38,loss of

trabecular pattern within the lesion suggestive of malignant

changes wrt to area of interest (Figure 2). Clinical diagnosis

of squamous cell carcinoma of the gingiva was given.

Incisional biopsy was done under local anesthesia and the

tissue was sent for histopathological examination.

Microscopic examination revealed presence of a highly

cellular connective tissue stroma with a stratified squamous

surface epithelium. A diffuse infiltration of atypical lymphoid

cells exhibiting pleomorphism, hyperchromatism and

abnormal mitosis was noted. A provisional diagnosis of Non

Hodgkin Lymphoma was given (Figure 3, 4)

.Immunohistochemistry showed CD20 positivity and CD3

negativity favoring the diagnosis of B cell type NHL (Figure

5). Examination of peripheral blood did not show any

significant finding. No other foci of lymphomatous process

could be noted.

Figure 3: Photomicrograph showing atypical lymphocytes

arranged in sheets (H&E 10X)

Figure 4 : Photomicrograph showing Rounded

hyperchromatic atypical lymphocytes (H&E 40X)

Figure 5: Immuno histochemical analysis of the specimen

showing cells diffusely positive for CD20 (20X).

Discussion : Lymphomas characterized by diffuse or nodular

sheets of lymphocytes and lymphoblasts without the presence

of Reed Sternberg cells are classified as Non Hodgkin

Lymphoma4. NHL has great tendency to affect organs and

tissue that do not ordinarily contain lymphoid cells. 20 - 30%

of NHL arises from extra nodal sites. Tonsils, nasopharynx,

base of tongue, paranasal sinuses, orbits and salivary glands,

maxilla5 are the sites involved in the head and neck region.

Jaw involvement is not so common however maxilla is more

frequently involved than mandible6. Mandible accounts for

0.6% of isolated NHL7 and it is rare to find extranodal NHLs

in the gingiva8.

The most common presenting symptoms are local swelling,

pain or discomfort or at times ulcer9. Generalized symptoms

such as fever, weight loss, night sweats, fatigue and pruritus

are more frequent in HL than NHL10.Determining the origin

of these lesions is difficult if there is both hard and soft tissue

involvement.

The oral NHL may mimic more commonly benign oral and

dental pathological conditions. Diagnosis of NHL is often

difficult because of clinical features that can mimic other

pathological conditions (eg fibroma, periodontal disease,

pyogenic granuloma etc)11. Thus the lesion may easily be

misdiagnosed without any particular identifiable features.

Also there is a lot of variation in racial incidence, histology

and immunological subtypes of lymphomas making the

diagnosis more difficult. The prevalence of NHL is increasing

day by day specially among the immunocompromised

individuals. There is increased evidence of NHL in patients

suffering from AIDS and oral lesions of NHL have been the

first manifestation of AIDS in most of the cases12. Therefore

it is important to remember that lymphoma should be the part

of the differential diagnosis in any oral lesions.

NHL at histological level can be broadly grouped into two

major subcategories - diffuse and nodular4. The histological

University Journal of Dental Sciences, An Official Publication of Aligarh Muslim University, Aligarh. India 89

University J Dent Scie 2015; No. 1, Vol. 3

Page 3: 19 PRIMARY EXTRA-NODAL B - Aligarh Muslim …Figure 2: Panoramic radiograph showing ill defined radiolucency with moth eaten appearance with respect to edentulous region of 36 37 and

subtyping is based on differentiation of lymphoid precursor

cell. The more primitive anaplastic tumors are high grade and

the more differentiated tumors which bear a greater

resemblance to mature lymphocytes are low grade. Early

Rapports classification was used and it was based on

morphology pattern, small cells were lymphocytic and large

cells were histiocytes. Lukes Collins classification of 1974

was immunologic based classification with morphologic

consideration. Keels classification is also immunologic based

but includes grades of malignancy. The working formulation

was devised to divide tumors into low, intermediate and high

grade tumors at the same time maintain the distinction

between nodular (follicular) and diffuse type as well as the

concept of cell size. Revised European American Lymphoma

(REAL) classification superseded working classification. In

most recent WHO classification of neoplastic disease of

hemopoetic & lymphoid tissue adopted this REAL

classification of lymphoid neoplasm with some

modification13. In our case, the patient reported with gingival

swelling without any other systemic complaints of weight

loss or long standing fever, or lymph node involvement which

suggest atypical presentation of this uncommon lesion.

As the occurrence of NHL in oral mucosa is uncommon and

the clinical presentation varies the only reliable method to

distinguish these lesions is by employing both biopsy and

immunological techniques. Therefore, apart from H & E

staining different immune histochemical markers are useful

to confirm the lineage of the neoplastic cell as B cell or T

lymphocytes. CD20 is often used as a B cell marker whereas

CD3, CD45 are frequently used as a marker of T cell lineage.

In the present case immunohistochemical expression of CD20

was seen variable staining which is indicative of B cell type

NHL.

The overall prognosis of NHL depends on the stage of the

tumor and aggressiveness of the malignant cell. A proper

clinical evaluation is very important as well as is the

histopathologic evaluation. Many a times these lesions

present a very benign appearance resulting which we may

miss the diagnosis. So, it is important to have an

understanding of tumors that could mimic an ordinary

swelling. Lymphomas should be considered in differential

diagnosis of oral lesions as patients with oral lesions of NHL

might present at the dental clinic in the first instance. Hence,

dentist can play an important role in early detection of the

disease, thus improving the prognosis.

Bibliography

1. van der Waal R, Huijgens P. C, van der Valk P, .van der

Waal I. Characterstic of 40 primary extranodal non

Hodgkin lymphoma of oral cavity in perspective of new

WHO classification and International Prognostic Index.

Int J Oral maxillofac Surg, 2005; 34: 391-395

2 . Regezi, Sciubba, Jordan. Oral Pathology: Pathologic

correlations, Fifth edition, Saunders, Elsevier: 220-225

3. Epstein J.B, Epsteinb J, Le N, Gorsky M.

Characteristic of Oral and Paraoral malignant

lymphoma. A population based review of 361 cases. Oral

Surg, Oral Med, Oral Pathol, Oral Radiol Endol, 2001;

92:519-25

4. Sapp J, Eversole L, George Wysocki .Contemporary

Oral and Maxillofacial Pathology, second edition,

Elsevier: 409-412,

5. Sankarnarayanan S, Chandrasekar T, Srinivas Rao,

Rooban T, Ranganath K. Maxillary Non Hodgkins

Lymphoma. J Oral MaxillofacPathol, 2005; 9: 1: 34-36

6. Parihar S, Garg RK, Narain P. Primary extranodal non

–Hodgkins lymphoma of gingival: A diagnostic

dilemma. J Oral Maxilllofac Pathol 2013;17: 320

7. Tina JE, Narayanswamy M, sundaresh K., Malikarjun K.

Primary extranodal variant of non-Hodgkins lymphoma

BMJ Case Rep 2014 doi;10 1136/bcr-2013-010132

8. Kobler P, Borcic J, Filipovic I, Nola M, Sertic D.

Primary non Hodgkin lymphoma of the oral cavity. Oral

Oncology Extra 2005; 41: 1: 12-14

9. Maheshwari GK, Baboo H A , Shah NM , Patel M, Shah

R. Primary nonhodgkin lymphoma of oral tongue.

Turkish Journal Of cancer, 2001; 31:.3 :121-124

10. Zapater E, Bagan JV, Carbonell F, Basterra J. Maxillary

lymphoma of head and neck. Oral Disease, 2010; 16:

119-128

11. Martinelli-Klay C.P , Martinelli C R, Martinelli C Dias

JB, Cheade TC, Lombardi T. Primary extra nodal non

Hodgkin lymphoma of gingiva initially misdiagnosed as

dental abscess. Quintesence International, Nov-Dec

2009;40:10: 805-808

12. Manjunatha BS, Gowramma R, Nagarajappa D, Tanveer

A. Extranodal non-Hodgkin's lymphoma presenting as

gingival mass. J Indian Soc Periodontol 2011; 15: 418-

420.

13. Marx RE, Stern D, Ross: Oral and Maxillofacial

Pathology. A Rationale for diagnosis and treatment.

Quintessence Publishing Co, Inc

University Journal of Dental Sciences, An Official Publication of Aligarh Muslim University, Aligarh. India 90

University J Dent Scie 2015; No. 1, Vol. 3

Page 4: 19 PRIMARY EXTRA-NODAL B - Aligarh Muslim …Figure 2: Panoramic radiograph showing ill defined radiolucency with moth eaten appearance with respect to edentulous region of 36 37 and

Legends

Figure 1: Intraoral picture showing gingival swelling wrt left

mandibular canine premolar region.

Figure 2: Panoramic radiograph showing ill defined

radiolucency with moth eaten appearance with respect to

edentulous region of 36 37 and floating tooth wrt 38

Figure 3: Photomicrograph showing atypical lymphocytes

arranged in sheets (H&E 10X)

Figure 4 : Photomicrograph showing Rounded

hyperchromatic atypical lymphocytes (H&E 40X)

Figure 5: Immuno histochemical analysis of the specimen

showing cells diffusely positive for CD20 (20X).

CORRESPONDING AUTHOR :

Dr. Pulin Saluja

T2 ,604, Sushant Estate ,sector 52,

Gurgaon, Haryana

E-mail: [email protected]

Phone No: 9910791584

University Journal of Dental Sciences, An Official Publication of Aligarh Muslim University, Aligarh. India 91

University J Dent Scie 2015; No. 1, Vol. 3


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