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 Table of Contents  
Year : 2015  |  Volume : 5  |  Issue : 2  |  Page : 121-124

Mantle cell lymphoma of tongue masquerading as squamous cell carcinoma

1 Department of Laboratory Medicine, Basvatarakam Indo American Cancer Hospital and Research Institute, Hyderabad, Telangana, India
2 Department of Medical Oncology, Basvatarakam Indo American Cancer Hospital and Research Institute, Hyderabad, Telangana, India

Date of Web Publication10-Mar-2016

Correspondence Address:
Dr. Manasi Chetan Mundada
Department of Laboratory Medicine, Basvatarakam Indo American Cancer Hospital and Research Institute, Road No. 10, Banjara Hills, Hyderabad - 500 034, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2231-6027.178503

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The occurrence of a malignant lesion in oral cavity raises the possibility of most commonly squamous cell carcinoma. However, there are other malignant lesions that can arise in the tongue. Lymphomas in the tongue/Waldeyer's ring can pose a diagnostic challenge. Here, we present a rare case of Mantle cell lymphoma primarily presenting as a tongue lesion with a deceptive appearance that requires a high grade of suspicion and judicious use of immunohistochemistry (IHC) to reach the diagnosis. The recognition and differentiation of this entity are important because of its aggressive clinical behavior.

Keywords: Immunohistochemistry, mantle cell lymphoma, oral cavity

How to cite this article:
Mundada MC, Ahmed F, Murthy SS, Mohan MK. Mantle cell lymphoma of tongue masquerading as squamous cell carcinoma. Int J Oral Health Sci 2015;5:121-4

How to cite this URL:
Mundada MC, Ahmed F, Murthy SS, Mohan MK. Mantle cell lymphoma of tongue masquerading as squamous cell carcinoma. Int J Oral Health Sci [serial online] 2015 [cited 2023 Jun 1];5:121-4. Available from: https://www.ijohsjournal.org/text.asp?2015/5/2/121/178503

  Introduction Top

Non-Hodgkin's lymphoma represents the second leading malignancy in the oral cavity after squamous cell carcinoma.[1] Primary NHL of the oral cavity commonly arises from the lymphoid tissue of Waldeyer's ring or less commonly from extranodal site.[2] The favorite intraoral sites being the hard palate, the vestibule and gingiva, NHL of the tongue is a very rare diagnosis.[2] Mantle cell lymphoma is a distinct clinicopathologic subtype of B-cell NHL, typically present in elderly age group, predominantly in males with Stage IV disease.[3],[4],[5] Common extra-nodal sites of involvement being Waldeyer's ring, bone marrow, peripheral blood, gastrointestinal tract. Other uncommon sites reported in the literature are ocular adnexa, central nervous system, skin, and tongue.[5] Here we present a rare case of Mantle cell lymphoma involving the base of the tongue.

  Case Report Top

A 65-year-old male patient presented at medical oncology outdoor patient's department with chief complaints of difficulty in swallowing since 2 months. He did not complain of any B type symptoms. On examination, the oral cavity showed a non-ulcerated mass involving the posterior third of the tongue on the right side and another mass in the left tonsillar area. Clinically, there was no organomegaly or gross lymphadenopathy. Computed tomography showed a polypoidal lesion on the right side arising from the base of tongue and occupying the oropharyngeal cavity and left tonsillar mass [Figure 1].
Figure 1: Computed tomography scan show pendunculated lesion extending into the oropharyngeal space on the left side

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Incisional biopsy from the lesion revealed sheets of lymphoid cells underlying the squamous epithelium. These cells were of intermediate size, showing round to cleaved vesicular nucleus, small nucleolus and scanty cytoplasm [Figure 2]a and [Figure 2]b. IHC performed on deparaffininized sections using standard protocols showed positivity for CD20, CD79a, CD5, Bcl2 and CYCLIN D1 and lacked expression for CD23, CD10, and CD3. Ki-67 index was 45%. The morphology and the IHC profile were diagnostic of Mantle cell lymphoma [Figure 3]a,[Figure 3]b,[Figure 3]c. Laboratory findings include unremarkable peripheral blood examination, lactate dehydrogenase (LDH) levels-313 U/L, and uninvolved bone marrow. Positron emission tomography showed increased uptake at the base of the tongue, left tonsil, along with bilateral cervical, axillary, mediastinum, and left ileal lymph node. Mild FDG uptake was noted in bilateral inguinal lymph nodes. The MIPI score was 5-8(intermediate). The expected median survival is 51 months.
Figure 2: (a) Histopathology of the tongue lesion. H and E sections show overlying squamous epithelium with diffuse sheets of lymphoid cells underneath (×10 objective). (b) Histopathology of the tongue lesion. H and E photomicrograph demonstrates round to cleaved nuclei with eosinophillic cytoplasm (×40 objective)

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Figure 3: (a) The neoplastic cells express immunostains CD5 (×10 objective). (b) The neoplastic cells express immunostains CD20 (×10 objective). (c) The neoplastic cells express immunostains Cyclin D1 (×40 objective).

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The patient was started on rituximab, bendamustine, vincristine, and dexamethasone(R-BOP). Chemotherapy drug dosages were: Rituximab 375 mg/m 2 on day 1; bendamustine 90 mg/m 2 on day 1 and 2; vincristine 2 mg on day 1; oral prednisolone 100 mg/day from day1 to day 5 were given once every 21 days, no major complication were seen.

This regimen was followed in view of age and performance status of the patient.[6]

On follow-up after the first cycle of chemotherapy, the peripheral blood counts showed mild leucopenia and thrombocytopenia. After the second cycle of chemotherapy, patient had considerable symptomatic relief from dysphagia and clinically there was a significant decrease in the size of the lesion. During chemotherapy, there was leukopenia and thrombocytopenia. However the platelets remained in the safe range (spontaneous bleeding was not seen) hence platelet transfusions were not required. The hemoglobin of the patient remained in the range of 9–10.5 g/dl. After six cycles of chemotherapy, the complete blood examination of the patient is normal with a hemoglobin level of 12.7 gm/dl, white blood cell count and platelets are in normal range and is in clinical remission. The patient is being closely monitored.

  Discussion Top

Diagnosis of tongue neoplasm be it benign or malignant is challenging owing to the possibilities of a wide spectrum of varied lesions-fibroma, lipoma, squamous cell carcinoma, and rarely sarcomas or metastatic disease. The cases may present accidentally or with symptoms such as dysphagia, odynophagia or dysarthria. The current case presented with odynophagia of short duration. Examination revealed a nonulcerated submucosal lesion, different from an ulcerated growth associated with epithelial malignancy. Concurrent involvement of the left tonsil, a member of the Waldeyer's ring, leads to the clinical suspicion of a lymphoma. Evaluation of the biopsy revealed a lymphomatous process; the morphology of diffuse sheets of small cleaved cell type ruled out Diffuse large B-cell lymphoma (DLBCL) however, Low-grade follicular lymphoma (LGFL)/MCL/Small cell lymphocytic lymphoma (SLL) still remained the possibilities.[7] Immunohistochemistry is of immense help in differentiating the MCL from SLL (CD5+, CD10, CD23+), Follicular lymphoma (CD5, CD10+). The profile of CD5+, CD10, CD23 is characteristic of MCL. Positive reaction for cyclin D1 being the confirmatory test. Thus, immunohistochemistry substantiated the diagnosis of MCL. Therefore, it is important to use judiciously the IHC panel to recognize and differentiate this entity from other NHL because of variable prognosis.

MCL is rarely encountered neoplasm and constitutes 3-4% of the lymphomas arising in Waldeyer's ring [7] with incidence, in general, 2–10%.[8],[9],[10] Mantle cell lymphoma as a presentation in the oral cavity is rare. An extensive review of the literature showed many cases of MCL reported in the oral cavity. However, majority of them have been described in hard palate and waldeyer's ring.[11],[12] The tongue as a primary site of presentation is described in four cases, two cases at the base of the tongue and in two, the primary site was in tongue. However, the specific region in tongue was not mentioned.[8] Mantle cell lymphoma in majority occurs in lymph nodes, however, extranodal sites such as spleen, gastrointestinal tract, and waldeyer's ring have been described in the literature. The appearance of extra-nodal MCL is that of monomorphic sheets of lymphocytes disrupting the native tissue architecture. Other morphological features described are the presence of pink macrophages and perivascular hyalinization. The lymph nodes may show lymphoid cells arranged either in diffuse sheets or nodular pattern. The tumour in its initial stage cause mantle zone expansion followed by nodular pattern formation due to the involvement of germinal centres by the neoplastic cells and finally diffuse sheets.[9]

The morphologic spectrum of mantle cell lymphoma ranges from small cleaved cells to blastoid/pleomorphic types. The classic type consists of small size lymphoid cells having round to cleaved nucleus, small inconspicuous nucleoli and scanty cytoplasm. The blastoid morphology showed small to intermediate sized cells, having dispersed chromatin and scanty cytoplasm.[8] The Pleomorphic type shows the varying sized population of cells having round to oval nucleus and pale cytoplasm with at least some of the cells having prominent nucleoli.[10] Four cases reviewed in the literature revealed three cases showing classic morphology while one case showed blastoid cell pattern.[8] Bone marrow aspiration and biopsy are done as a part of the staging of disease. The most common pattern of involvement is nodular paratrabecular aggregates of lymphoid cells. In the present case bone marrow was free of involvement by lymphoma. The average period of survival was 3.6 years in cases with the classic pattern while the blastoid pattern showed the survival of 1 month duration hence substantiating the fact that blastoid morphology is associated with the aggressive clinical course. Further staging by ANN-ARBOR classification indicated the disease in the current case was in Stage III B with the possibility of an aggressive clinical course.

Although we have not assessed the cytogenetic abnormality, in this case, MCL is known to be associated with the characteristic chromosomal translocation t (11;14) (q13;q32) which results in the juxtaposition of bcl1 gene locus to the Ig heavy chain promoter. However, this is not universal.[8] This translocation results in expression of CCND1 protein which encodes for CyclinD1 which is overexpressed and deregulated by the translocation. Overexpression of Cyclin D1 both at mRNA and protein levels act as a driver for G1/S progression and mitotic growth factor signalling.[9]

Treatment which is in the form of polychemotherapy. Different regimens are followed like CHOP (cyclophosphamide, vincristine, doxorubicin, prednisolone), R-CHOP (addition of rituximab to CHOP), R-BOP, Hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, prednisone). The choice of regimen depends on the clinical presentation, proliferation index, clinical risk scores, and performance status.[6]

To conclude this is the 5th case being described in the literature with a primary lesion in the tongue a mantle cell lymphoma masquerading as squamous cell carcinoma. It is important to use judiciously the IHC panel to recognize and differentiate this entity from other NHL because of variable prognosis.

  Acknowledgment Top

Dr. Sandhya, Cytogeneticist, Mrs. Ajitha, Mr. Ravinder, (technical support).

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Epstein JB, Epstein JD, Le ND, Gorsky M. Characteristics of oral and paraoral malignant lymphoma: A population-based review of 361 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;92:519-25.  Back to cited text no. 1
Guastafierro S, Falcone U, Celentano M, Cappabianca S, Giudice A, Colella G. Primary mantle-cell non-Hodgkin's lymphoma of the tongue. Int J Hematol 2008;88:206-8.  Back to cited text no. 2
Swerdlow SH, Campo E, Seto M, Muller-Hermelink HK. Mantle cell lymphoma. In: Swedlow SH, editor. WHO Classification of Tumours of Haematopoetic and Lymphoid Tissue. 4th ed. Lyon: IARC; 2008. p. 229-32.  Back to cited text no. 3
Argatoff LH, Connors JM, Klasa RJ, Horsman DE, Gascoyne RD. Mantle cell lymphoma: A clinicopathologic study of 80 cases. Blood 1997;89:2067-78.  Back to cited text no. 4
Kyo C, Kawaoka Y, Kinoshita K, Ohno H. Mantle cell lymphoma presenting with a tumor of the hard palate. Intern Med 2010;49:1663-6.  Back to cited text no. 5
McKay P, Leach M, Jackson R, Cook G, Rule S; British Committee for Standards in Haematology. Guidelines for the investigation and management of mantle cell lymphoma. Br J Haematol 2012;159:405-26.  Back to cited text no. 6
Tan LH. Lymphomas involving Waldeyer's ring: Placement, paradigms, peculiarities, pitfalls, patterns and postulates. Ann Acad Med Singapore 2004;33 4 Suppl: 15-26.  Back to cited text no. 7
Guggisberg K, Jordan RC. Mantle cell lymphoma of the oral cavity: Case series and comprehensive review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:98-104.  Back to cited text no. 8
Pileri SA, Falini B. Mantle cell lymphoma. Haematologica 2009;94:1488-92.  Back to cited text no. 9
Gujral S, Agarwal A, Gota V, Nair R, Gupta S, Pai SK, et al. Aclinicopathologic study of mantle cell lymphoma in a single center study in India. Indian J Pathol Microbiol 2008;51:315-22.  Back to cited text no. 10
[PUBMED]  Medknow Journal  
Dereci Ö, Ay S, Açikalin MF, Karagülle M. Ill-fitting dentures as primary presentation of mantle cell lymphoma: A case report and literature review of the primary mantle cell lymphomas of the hard palate. J Oral Maxillofac Pathol 2015;19:255-9.  Back to cited text no. 11
[PUBMED]  Medknow Journal  
Scheller K, Becker S, Scheller C. Symmetric palatal swelling as the first clinical manifestation of a mantle cell non-Hodgkin's lymphoma: A case report and review of literature. J Oral Maxillofac Pathol 2011;15:311-5.  Back to cited text no. 12
[PUBMED]  Medknow Journal  


  [Figure 1], [Figure 2], [Figure 3]

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