Lymphoblastic lymphoma of the palate Rakul Nambiar, MD, Geetha Narayanan, MD, DM, Lali V. Soman, MBBS, and Nandini Devi, MD

Lymphoblastic lymphomas are neoplasms of immature or precursor lymphoid cells with no or limited bone marrow involvement, whose clinical presentation varies according to the immunophenotype. While mediastinal involvement is predominant in T-lymphoblastic lymphomas, B-lymphoblastic lymphomas frequently involve nodal sites. Extranodal presentation of B-lymphoblastic lymphomas is extremely rare. We present the case of a 49-year-old man with B-lymphoblastic lymphoma of the hard palate. The patient was treated with the R-Hyper CVAD regimen and is on maintenance chemotherapy. This is the first reported case of B-lymphoblastic lymphoma arising in the hard palate.

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ymphoblastic lymphoma (LBL) is a rare aggressive neoplasm of T-cell or B-cell precursors resembling acute lymphoblastic leukemia, with no or limited bone marrow involvement, that develops more frequently in children and young adults. Lymphoblastic lymphoma of the B-cell type is uncommon, and extranodal presentation is even rarer. We report what is, to the best of our knowledge, the first reported case of B-lymphoblastic lymphoma (B-LBL) of the hard palate. CASE REPORT A 49-year-old man presented with pain and swelling in the hard palate for 3 months. Examination of the oral cavity showed diffuse, soft, nontender swelling in the hard palate (Figure 1a). There was no pallor, lymphadenopathy, or organomegaly. Computed tomography scan of the head revealed irregular rarefaction of the anterior aspect of the hard palate. An incisional biopsy of the lesion disclosed subepithelium diffusely infiltrated with a monotonous population of medium to large cells with vesicular nuclei, prominent nucleoli, and scanty cytoplasm (Figure 2). On immunohistochemistry, the cells were positive for CD20, CD34, Bcl2, and Tdt with an MIB labeling index of about 90%. The picture was diagnostic of B-LBL. His hemoglobin was 15 g/dL; platelets, 3.5 lakhs/mm3; and total leucocyte count, 7300/mm3. Lactate dehydrogenase was 540 U/L. His cerebrospinal fluid and bone marrow studies were normal. Computed tomography scans of the neck, thorax, abdomen, and pelvis were normal, and he was staged as stage 1. The patient was started on rituximab, cyclophosphamide, vincristine, doxorubicin, and dexamethasone (R-Hyper CVAD protocol). After completion Proc (Bayl Univ Med Cent) 2017;30(4):445–446

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Figure 1. (a) Diffuse swelling in the hard palate. (b) Regression of the lesion after one cycle of chemotherapy.

Figure 2. Monotonous population of medium to large cells with vesicular nuclei and scanty cytoplasm (hematoxylin and eosin, 100×).

From Regional Cancer Centre, Trivandrum, India. Corresponding author: Geetha Narayanan, MD, DM, Professor and Head, Department of Medical Oncology, Regional Cancer Centre, Trivandrum 695011, India (e-mail: [email protected]). 445

of the first cycle of chemotherapy, his symptoms subsided and the lesions showed significant clinical and radiological regression (Figure 1b). At present, he is on maintenance chemotherapy. DISCUSSION LBL is a highly aggressive neoplasm of lymphoblasts that may be of either T-cell origin (T-LBL) or B-cell origin. Lymphoblastic lymphoma accounts for approximately 2% of all non-Hodgkin lymphomas, out of which T-LBL constitutes around 90% of cases (1). LBLs are grouped together with acute lymphoblastic leukemia in the 2008 World Health Organization classification of hematopoietic malignancies (2). These two entities are biologically very close but not identical; in LBL, the bone marrow is not involved or is only partially involved, with less than 20% infiltrating blast cells. LBL occurs commonly in children, mostly males. T-LBL usually presents with a mediastinal mass, central nervous system involvement, and pleural and pericardial effusion, whereas B-LBL presentation is more limited than that of T-LBL and the localized disease usually involves single nodal or extranodal sites such as skin, bone, and soft tissue (3, 4). Lymphoid lesions of the palate can be either lymphomatous or benign lymphoid hyperplasia. Oral lymphomas are relatively rare and constitute about 4% of all oral malignancies (5). The oral cavity is the primary site of approximately 2% of all extranodal lymphomas (6). Lymphomas can affect both bony and soft tissue of the oral cavity, with the most frequent localization being the tonsil. The most common type is diffuse large B-cell lymphoma, but mantle cell lymphoma, marginal zone B-cell lymphoma, Burkitt’s lymphoma, lymphomablastic lymphoma, peripheral T-cell lymphoma, and anaplastic large cell lymphoma have also been reported in the oral cavity (7). However, B-LBL arising from the hard palate has not been reported previously. Clinical manifestations depend on the location of the lesion. The most common clinical appearance of non-Hodgkin lymphoma in the mouth is a nonhealing, painless ulceration (8). Patients may complain of localized or diffuse soft tissue swelling, pain, mucosal discoloration, paresthesias, anesthesia, and loosening of teeth (9). Lymphoblastic lymphomas are treated similar to acute lymphoblastic leukemia with combination chemotherapy protocols

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consisting of intensive remission-induction chemotherapy, central nervous system prophylaxis, a consolidation chemotherapy, and subsequent maintenance therapy. Treatment of LBL with protocols derived from acute lymphoblastic leukemia therapy are effective, with an 82% event-free survival and an 85% overall survival at 5 years (10). 1.

The Non-Hodgkin’s Lymphoma Classification Project. A clinical evaluation of the international lymphoma classification of non-Hodgkin’s lymphoma. Blood 1997;89(11):3909–3918. 2. Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood 2011;117(19):5019– 5032. 3. Metzgeroth G, Walz C, Score J, Siebert R, Schnittger S, Haferlach C, Popp H, Haferlach T, Erben P, Mix J, Müller MC, Beneke H, Müller L, Del Valle F, Aulitzky WE, Wittkowsky G, Schmitz N, Schulte C, Müller-Hermelink K, Hodges E, Whittaker SJ, Diecker F, Döhner H, Schuld P, Hehlmann R, Hochhaus A, Cross NC, Reiter A. Recurrent finding of the FIP1L1-PDGFRA fusion gene in eosinophilia-associated acute myeloid leukemia and lymphoblastic T-cell lymphoma. Leukemia 2007;21(6):1183–1188. 4. Salloum E, Henry-Amar M, Caillou B, Friedman S, Pico JL, Bayle C, Hayat M. Lymphoblastic lymphoma in adults: a clinicopathological study of 34 cases treated at the Institut Gustave-Roussy. Eur J Cancer Clin Oncol 1988;24(10):1609–1616. 5. 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(5):519– 525. 6. Ferry JA, Harris NL. Lymphomas and lymphoid hyperplasia in head and neck sites. In Pilch BZ, ed. Head and Neck Surgical Pathology. Philadelphia: Lippincott Williams and Wilkins, 2001:476–533. 7. Hicks MJ, Flaitz CM. External root resorption of a primary molar: “incidental” histopathological finding of clinical significance. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;92(1):4–8. 8. Richards A, Costelloe MA, Eveson JW, Scully C, Irvine GH, Rooney N. Oral mucosal non-Hodgkin’s lymphoma—a dangerous mimic. Oral Oncol 2000;36(6):556–558. 9. Parrington SJ, Punnia-Moorthy A. Primary non-Hodgkin’s lymphoma of the mandible presenting following tooth extraction. Br Dent J 1999;187(9):468–470. 10. Ducassou S, Ferlay C, Bergeron C, Girard S, Laureys G, Pacquement H, Plantaz D, Lutz P, Vannier JP, Uyttebroeck A, Bertrand Y. Clinical presentation, evolution, and prognosis of precursor B-cell lymphoblastic lymphoma in trials LMT96, EORTC 58881, and EORTC 58951. Br J Haematol 2011;152(4):441–451.

Baylor University Medical Center Proceedings

Volume 30, Number 4

Lymphoblastic lymphoma of the palate.

Lymphoblastic lymphomas are neoplasms of immature or precursor lymphoid cells with no or limited bone marrow involvement, whose clinical presentation ...
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