JICDRO is a UGC approved journal (Journal no. 63927)

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CLINICAL SCIENCE AND TECHNIQUES/CASE REPORT WITH DISCUSSION
Year : 2022  |  Volume : 14  |  Issue : 1  |  Page : 74-78

Lymphocyte-rich classical hodgkin's lymphoma involving submandibular salivary gland: A case report


1 Department of Oral Medicine and Radiology, Bharati Vidyapeeth (Deemed to be University) Dental College and Hospital, Pune, Maharashtra, India
2 Department of Oral Pathology and Microbiology, Bharati Vidyapeeth (Deemed to be University) Dental College and Hospital, Pune, Maharashtra, India

Date of Submission09-May-2021
Date of Decision14-Aug-2021
Date of Acceptance26-Jan-2022
Date of Web Publication4-Jul-2022

Correspondence Address:
Dr. Isha Prasad Kale
Govind' 1621, Sadashiv Peth, Gopal Gayan Samaj Road, Pune-30, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jicdro.jicdro_26_21

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   Abstract 


Hodgkin's lymphoma (HL) is a malignant disease of the lymphoid tissue originating from the germinal center and postgerminal center B-cells comprising 10% of all lymphomas and 0.6% among all cancers. Here, we report a case of a 22-year-old male with a chief complaint of painless swelling in the right submandibular region for the past 6 months with no contributory medical history. Correlating the history and clinical examination, a clinical diagnosis of benign sialadenoma was made. Benign sialadenoma was confirmed on radiographic investigations such as ultrasonography and magnetic resonance imaging. Furthermore, excisional biopsy and immunohistochemistry performed revealed a case of classical HL (lymphocyte-rich type). The positron-emission tomography–computed tomography revealed metastasis to the inguinal and cervical lymph nodes.

Keywords: Classical Hodgkin's lymphoma, immunohistochemistry, magnetic resonance imaging, positron emission tomography–computed tomography, ultrasonography


How to cite this article:
Kale IP, Mhapuskar AA, Kulkarni VV, Hiremutt DR. Lymphocyte-rich classical hodgkin's lymphoma involving submandibular salivary gland: A case report. J Int Clin Dent Res Organ 2022;14:74-8

How to cite this URL:
Kale IP, Mhapuskar AA, Kulkarni VV, Hiremutt DR. Lymphocyte-rich classical hodgkin's lymphoma involving submandibular salivary gland: A case report. J Int Clin Dent Res Organ [serial online] 2022 [cited 2022 Aug 7];14:74-8. Available from: https://www.jicdro.org/text.asp?2022/14/1/74/349752




   Introduction Top


Classical Hodgkin's lymphomas (CHLs) account for approximately 85% of all Hodgkin's lymphoma cases. In literature, four different subtypes have been designated, which include nodular sclerosis CHL (NSCHL), mixed cellularity CHL (MCCHL), lymphocyte-depleted CHL (LDCHL), and lymphocyte-rich CHL (LRCHL).[1]

Most patients with CHL present with lymphadenopathy commonly involving cervical, mediastinal, supraclavicular, and axillary, with some variation in site preference among different subtypes.[1]

The classical Reed–Sternberg (RS) cells are those that originate from the germinal B centers and are identified in almost every subclass of CHL. They are seen as cells that have lost their genetic regulation for normal growth and development.

Here, we report a rare case of CHL affecting the maxillofacial region in a young patient that clinically manifested as a slow-growing benign mass with minimum or no symptoms.

Further investigations, however, revealed lymphocyte-rich type of CHL along with distant metastasis.

CHL affecting the maxillofacial region may thus misguide the clinician leading to improper diagnosis, delaying the treatment, and thus affecting the prognosis.


   Case Report Top


A 22-year-old male patient reported to the Department of Oral Medicine and Radiology with a chief complaint of swelling in the lower right back region of the jaw for the past 6 months. The swelling was found to have progressively increased over a period of 6 months with no associated pain or pus discharge.

The patient visited a physician for the same and took multivitamins 3 months ago for the same, after which the swelling was found to subside temporarily only to regain its size back within a week.

The patient had a habit of eating gutka 3–4 times a week and occasional drinking of alcohol and smoking over the period of 1 year. No abnormality was detected intraorally.

However, extraorally, the swelling was seen in the right submandibular region extending anteroposteriorly from the right parasymphyseal region to the right angle of mandible. Superoinferiorly, the swelling was seen extending from the inferior border of mandible to 5 cm below it. The swelling was approximately 5 cm × 5 cm in size [Figure 1]a and [Figure 1]b (original figure).
Figure 1: (a) and (b) extra oral swelling right submandibular region

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On palpation, an oval, painless, well-defined, rubber-like mass, nonfixated to superficial, and deep structures was felt. Orthopantomogram made to rule out any skeletal and dental involvement was found to be normal. The patient's blood investigations were also found to be within the normal limits.

On correlating the clinical findings, a provisional diagnosis of benign tumor of the right submandibular salivary gland was made.

Further to this the patient underwent a USG guided biopsy of the right submandibular region that revealed 2 enlarged submandibular lymph nodes with maintained fatty hilum as seen on level Ibon the right side suggestive of cervical lymphadenopathy [Figure 2]a and [Figure 2]b (Original figure).
Figure 2: (a) and (b) Enlarged lymph nodes as seen on level 1b

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The USG-guided biopsy done under ×10 and ×40 showed a monotonous population of lymphoid cells with few interspersed histiocytes and occasional lymphoid follicles. No metastatic deposits were seen, and the overall picture was suggestive of a low-grade lymphoproliferative disease [Figure 3]a and [Figure 3]b (Original figure).
Figure 3: (a) ×10 view shows connective tissue lymphocytic proliferation. (b) ×40 view shows chronic inflammatory infiltrate

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Magnetic resonance imaging (MRI) of the neck revealed a well-defined lobulated hypointense exophytic solid mass lesion arising from the right submandibular gland, approximately measuring 5.8 cm × 3.1 cm × 2.9 cm with no regional lymphadenopathy. Margins were regular without infiltration into the skin or surrounding soft-tissue structures and without any areas of necrosis. The overall picture was suggestive of right submandibular benign sialadenoma [Figure 4]a, [Figure 4]b, [Figure 4]c (Original figure).
Figure 4: (a) axial section (b) sagittal section. (c) axial section. (a-c) shows MRI scans of hypointense exophytic solitary mass with regular, well-defined margins

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On correlating the findings of USG-guided biopsy and MRI, a final diagnosis was given as benign sialadenoma of the right submandibular gland. The differential diagnosis was given as Warthin's tumor or pleomorphic adenoma involving the right submandibular gland.

Under general anesthesia with nasotracheal intubation, the patient was operated for total excision of the right submandibular gland along with the benign tumor. The excised mass contained both the lobes of the right submandibular salivary gland – superficial and deep along with the well-circumscribed ball-like mass surrounding the gland which was oval, lobulated, capsulated, soft, and firm [Figure 5] (original figure).
Figure 5: shows the excised mass of tissue with the gland

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Histopathological report of the specimen showed partially encapsulated lymph nodes with a monotonous population of small-to-medium-sized atypical lymphoid cells with occasional mitotic figures, showing salivary gland histology to be normal. This pointed to the diagnosis of non-Hodgkin's lymphoma (HL), which needed further investigation to confirm the diagnosis

Thus, immunohistochemistry (IHC) was carried out for the markers CD5, CD10, CD15, CD3, CD20, CD23, CD30, cyclin D1, PAX-5, Bcl-2, and Bcl-6.

Immunohistochemistry revealed that the lymph node architecture was replaced due to neoplastic lymphoid proliferation consisting of many mature lymphocytes, occasional eosinophils, and scattered large atypical binucleate as well as mononuclear Reed–Sternberg-like cells having convoluted, large nuclei and prominent pink nucleoli RS-like cells showed positive for CD30, CD15, CD20, CD45, CD3, and PAX-5 CD20 and Bcl-2 highlighted the involvement of B-cells. CD3 highlights background T-cells [Figure 6] (Original figure).
Figure 6: shows immunohistochemistry positive for CD3, CD20, CD45, and CD15

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Based on the IHC, the diagnosis was modified to CHL (lymphocyte-rich type).

The patient was further advised to undergo F18-Fludeoxyglucose (FDG) whole-body positron-emission tomography–computed tomography (PET-CT) Scan. The PET-CT scan showed hypermetabolic right level II cervical, left inguinal lymph nodes, and FDG-avid bilateral faucial tonsillar involvement [Figure 7]a, [Figure 7]b, [Figure 7]c respectively] (Original figure).
Figure 7: (a) shows hypermetabolic right level II cervical lymph nodes. (b) shows left inguinal lymph nodes. (c) shows bilateral faucial tonsillar involvement

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On correlating the clinical features, histopathology, IHC, and PET-CT, the final diagnosis of Stage III lymphocyte-rich CHL was given. The patient was then referred to a tertiary specialty hospital for further chemotherapy and treatment.


   Discussion Top


Hodgkin's lymphoma is classified into two largely distinct entities, namely Nodular lymphocyte predominant Hodgkin's lymphoma HL (NLPHL) and classical HL (CHL).

In literature, four different subtypes of CHL have been designated, which include NSCHL, MCCHL, LDCHL, and LRCHL.[1]

The subtypes of CHL differ in their manifestations, as shown in [Table 1].[2]
Table 1: Types of Classical Hodgkin's Lymphoma[2]

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Lymphocyte-rich CHL is a rare subtype of CHL, accounting for about 5% of all classical HLs that are observed in middle-aged adults (mean = 43 years). Even though both male and female genders are affected, this type of lymphoma affects males more (male-to-female ratio is 2:1). The condition is found worldwide, and no racial or ethnic predilection is observed in its occurrence.[3]

The risk factors for developing the will include a family history of the condition, being immunocompromised, and history of Epstein-Barr virus infection, but the exact cause of this type of cancer is unknown; in most cases, a mutation in B-lymphocytes is reported.[4]

A vast majority of the malignant cases are observed in the peripheral lymph nodes followed by the mediastinum (1 in 6 cases). Individuals usually experience nonspecific symptoms such as fever, fatigue, painless swollen lymph nodes, and unintended weight loss.[4]

Swollen or enlarged lymph nodes are often painless, and it is uncommon to find large tumor masses (bulky disease). In cases of mediastinal involvement, there could be associated cough, chest pain, and breathing difficulties.[4]

Our patient gave no such symptoms other than a painless swelling in the right submandibular region.

The Ann Arbor staging system for Hodgkin's lymphoma states–[5]

  • Stage I: Involvement of a single lymph node region or of a single extralymphatic organ or site
  • Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm or localized involvement of an extralymphatic organ or site
  • Stage III: Involvement of lymph node regions or structures on both sides of the diaphragm
  • Stage IV: diffuse or disseminated involvement of one or more extralymphatic organs, or either:


- isolated extralymphatic organ involvement without adjacent regional lymph node involvement, but with disease in distant sites

- involvement of the liver, bone marrow, pleura or cerebrospinal fluid

LRCHL usually presents with Stage I or II of the disease (~70%).[2]

In our case, no lymph nodes were palpable on clinical examination. The PET-CT, however, showed involvement of left inguinal, right level II cervical, and bilateral faucial tonsillar involvement indicating Stage III of the disease.

As the name implies, LRCHL presents with a rich background of normal lymphocytes, with the malignant cells found in a B-cell rich milieu in the mantle and marginal zones of reactive follicles. The neoplastic cells have the phenotype of classical Hodgkin's Reed–Stenberg cells but are smaller and, in H and E-stained sections, may resemble lymphocyte predominant cells. Thus, immunohistochemical studies are critical for correct diagnosis.[6]

The RS-like cells in LRCHL usually express CD30, CD15, CD20, and PAX-5 positivity.[3]

LRCHL bears histological resemblance to NLPHL, with both having a background of small B-lymphocytes with regressed germinal centers in the periphery. Due to the histological similarities, this subtype can easily be confused with NLPHL, and the demonstration of a classical Hodgkin's Reed Sternberg immunophenotype is essential in making the diagnosis.

NLPHL usually shows CD30 negativity in almost all cases.[7]

The tumour markers that are usually seen in different forms of Classical hodgkin's lymphoma as compared to Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) are seen in [Table 2].[3],[7],[8],[9],[10]
Table 2: The tumor markers seen in different forms of Classical hodgkin's lymphoma as compared to Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL)

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The prognosis of lymphocyte-rich CHL is good, with early diagnosis and prompt treatment. 95% of the affected individuals are noted to have complete remission.[4]

This subtype is a less aggressive form of CHL, and as such, its prognosis is better than other classical forms of Hodgkin's lymphoma.

However, 1 in 5 cases is known to recur. Progression to bone marrow failure is usually associated with a short survival period. In LRCHL, only 2% of the cases are known to involve the bone marrow.[4]


   Conclusion Top


Lymphocyte-rich CHL may occur in the head-and-neck region in young patients rarely. They tend to be slow growing, painless, and may mimic a benign pathology to start with. This leads to delay in treatment and affects the prognosis of the disease. As clinicians, we need to be aware of this possibility and include HL in the differential diagnosis of painless swellings in the maxillofacial region.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Wang HW, Balakrishna JP, Pittaluga S, Jaffe ES. Diagnosis of hodgkin lymphoma in the modern era. Br J Haematol 2019;184:45-59.  Back to cited text no. 1
    
2.
Jeffrey Medeiros L, Miranda RN. In: Diagnostic Pathology: Lymph Nodes and Extranodal Lymphomas, 2nd ed. Salt lake city, UT: Elsevier,2018. p. 274-83.  Back to cited text no. 2
    
3.
Luca DC. CHL Lymphocyte Rich. Available from: https://www.pathologyoutlines.com/topic/lymphomanonBLRHL.html. [Last accessed on 2021 Apr 27].  Back to cited text no. 3
    
4.
Lymphocyte-Rich Classical Hodgkin Lymphoma (dovemed.com). Available from: https://www.dovemed.com/diseases-conditions/lymphocyte-rich-classical-hodgkin-lymphoma/. [Last accessed on 2021 Apr 27].  Back to cited text no. 4
    
5.
Cheng J, Bell D. Ann Arbor staging system. Reference article, Radiopaedia.org. Available from: https://doi.org/10.53347/rID-63815. [Last Accessed on 2021 Apr 27].  Back to cited text no. 5
    
6.
Mani H, Jaffe ES. Hodgkin lymphoma: An update on its biology with new insights into classification. Clin Lymphoma Myeloma 2009;9:206-16.  Back to cited text no. 6
    
7.
Balakrishna J, Jaffe ES. Nodular Lymphocyte Predominant. Available from: https://www.pathologyoutlines.com/topic/lymphomanonBNLPHL.html. [Last accessed on 2021 Apr 27].  Back to cited text no. 7
    
8.
Luca DC, Smith L. CHL Lymphocyte Depleted. Available from: https://www.pathologyoutlines.com/topic/lymphomanonBlymphocytedepleted.html. [Last accessed on 2021 Apr 27].  Back to cited text no. 8
    
9.
Luca DC. Mixed Cellularity. Available from: https://www.pathologyoutlines.com/topic/lymphomanonBmixed.html. [Lastaccessed on 2021 Apr 27].  Back to cited text no. 9
    
10.
Luca DC. Nodular Sclerosis. Available from: https://www.pathologyoutlines.com/topic/lymphomanonBnshl.html. [Last accessed on 2021 Apr 27].  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
 
 
    Tables

  [Table 1], [Table 2]



 

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