Baylor University Medical Center Proceedings October 2017 - 446

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

446

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

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