T-cell lymphoblastic lymphoma of the lower jaw in a young

child
A case report

E p p o B. W o l v i u s , D D S , a Paul van der Valk, M D , PhD, b Jacques A. Baart, D D S , c

N e t t e k e Y.N. S c h o u t e n - v a n M e e t e r e n , M D , d and Isa~ic van d e r W a a l , D D S , PhD, e
Amsterdam, The Netherlands
FREE UNIVERSITY HOSPITAL

Among non-Hodgkin's lymphomas occurring in childhood two major histologic subgroups can be identified:
(1) Burkitt's lymphoma and (2)T-cell lymphoblastic lymphoma, an uncommon high-grade malignant non-Hodgkin's
lymphoma. Although Burkitt's lymphoma with maxillofacial involvement is a well-documented disease, T-cell
lymphoblastic lymphoma in the perioral region is rare. An unusual case of T-cell lymphoblastic lymphoma with initial
oral manifestation in an 1 8-month-old child is presented. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod
1996;82:434-6)

T h e n o n - H o d g k i n ' s l y m p h o m a s ( N H L s ) are a heter-

o g e n o u s g r o u p o f m a l i g n a n t conditions arising f r o m
the w i d e l y d i v e r s e cells o f the i m m u n e system.
A l t h o u g h the initial lesions often d e v e l o p in the
l y m p h nodes, e x t r a n o d a l sites such as the gastrointes-
tinal tract, the skin, the oral c a v i t y and pharynx, small
intestine, and central n e r v o u s s y s t e m m a y b e i n v o l v e d
as well. M o s t types o f N H L occur in the sixth and
seventh d e c a d e o f life. In contrast, N H L s a m o n g
y o u n g children p r e d o m i n a n t l y fall into t w o m a j o r
subgroups: (1) B u r k i t t ' s l y m p h o m a , w h i c h occurs
m a i n l y b e t w e e n the third and sixth y e a r o f life, and
(2) T-cell l y m p h o b l a s t i c l y m p h o m a , w h i c h occurs
p r e d o m i n a n t l y in the first and second d e c a d e o f life. 1
Fig. 1. Large blue-red mass in mandible spreading to-
A l t h o u g h in B u r k i t t ' s l y m p h o m a m a x i l l o f a c i a l in- wards floor of mouth and labial sulcus. Primary teeth are
v o l v e m e n t is well d o c u m e n t e d , 2 oral manifestations clearly displaced.
o f T-cell l y m p h o b l a s t i c l y m p h o m a have r a r e l y b e e n
d e s c r i b e d ; patients u s u a l l y have a m e d i a s t i n a l m a s s
CASE REPORT
and w i d e l y d i s s e m i n a t e d disease. 3 A case o f T-cell In July 1995 an 18-month-old girl had been referred by
l y m p h o b l a s t i c t y p e o f N H L o f the l o w e r j a w in a the dentist because of a rapidly growing swelling in the
y o u n g child is presented. midline of the lower jaw. Intraoral examination revealed a
large, ulcerated, blue-red, rubbery-firm tumor mass in the
region of the anterior mandibular deciduous teeth (Fig. 1).
aDepartment of Oral and Maxillofacial Surgery and Oral Pathol- The teeth were clearly displaced and mobile. On radio-
ogy. graphic evaluation there was diffuse loss of bone, giving the
bDepartment of Pathology. impression of teeth "floating in air" (Fig. 2). The buds of
CDepartment of Oral and Maxillofacial Surgery and Oral Pathol- the permanent teeth seemed to be undamaged. A tentative
ogy. diagnosis of Burkitt's-type lymphoma was made.
dDepartment of Pediatric Hematology, Free University Hospital, With the patient under general anesthesia, an incisional
Amsterdam, The Netherlands.
biopsy was performed, and the primary teeth were re-
eDepartment of Oral and Maxillofacial Surgery and Oral Pathol-
ogy. moved. Histologic examination of the tumor revealed a
Received for publication Jan. 6, 1996; accepted for publication diffuse lymphoid population of medium-sized cells (Fig.
March 3, 1996. 3). The cytoplasm of these cells was moderately basophilic.
Copyright 9 1996 by Mosby-Year Book, Inc. High mitotic activity was seen. Immunohistochemical
1079-2104/96/$5.00 + 0 7/14/73826 stainings for leukocyte (CD3) (Fig. 3), OKT11 (CD2) (both

434
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY
Volume 82, Number 4
markers from Becton Dickinson, Erembodegem, Belgium),
and RIV4 (CD8) (from Sanbio, Uden, Holland) (all t-cell
markers) were positive. Stainings for CD34 (from Becton
Dickinson), L26 (from DAKO, Copenhagen, Denmark),
and leukocyte 14 (from Becton Dickinson) (pan B-cell
markers) were negative. Staining for latent membrane pro-
tein-1 (LMP-1) (S12, from Organon, Teknika, Boxtel,
Holland) was negative. A final diagnosis of T-cell lym-
pboblastic lymphoma was made,
The patient was referred to the pediatric oncologic
department for staging and treatment. Staging procedures
included a medical history and physical examination,
complete blood count with differential, radiographs of the
chest, abdominal ultrasonography, computed tomography
(CT) and magnetic resonance imaging (MRI) of the head,
radionuclide bone scanning, a biochemical profile includ-
ing renal and liver function studies, bone marrow morpho-
logic characteristics, and cytologic evaluation of cere-
brospinal fluid (CSF). The CSF showed lymphoblastic
cells, also staining positive for T-cell markers. Besides the
CNS involvement, no other positive sites were found in
these staging procedures. Thus with CNS involvement the
disease was in stage IV.
The patient was treated with a chemotherapeutic regimen
according to the NHL-94 protocol of the Dutch Leukemia
Study Group for patients with stage III and IV non B-cell
NHL. According to this scheme the patient receives intra-
venous and intrathecal multiagent chemotherapy in the first
6 months followed by 1~ years of oral maintenance. Be-
cause of the very young age, craniospinal irradiation was
omitted.
Rapid disappearance of the oral lesion within 3 weeks
was noticed, and CSF cytologic evaluation demonstrated
complete remission of the disease.
DISCUSSION
In a study of 441 malignant lymphomas involving
children younger than 15 years of age, 30% of the
cases were Hodgkin's lymphoma, and 70% were
NHL. 4 Childhood NHLs are predominantly high-
grade tumors, with Burkitt's lymphoma (40%.) and
T-cell lymphoblastic l y m p h o m a (25%) being the
most frequent t y p e s ) In the study by Lennert and
Feller, 1 the youngest patient was 11 months.
Oral NHLs are predominantly B-cell tumors, How-
ever, in the Japanese population a relatively high in-
cidence of T-cell lymphoma has been reported. 5 This
might be related to the high prevalence of seroposi-
tivity for human T-cell leukemia/lymphoma virus
type I in that population. This virus seems to play no
role, however, in the pathogenesis of T-cell lympho-
blastic lymphoma.
Lymphoblastic lymphoma of T-cell type is derived
from the precursor cells of peripheral T-lymphocytes.
According to their immunohistochemical marker
profile they can originate in the bone marrow ("pre-
thymic t y p e " ) or the thymus ( " e a r l y " and "late thy-
Wolvius et al. 435
Fig. 2. Radiograph of anterior mandible showing exten-
sive diffuse loss of bone in region of primary teeth, giving
impression of teeth "floating in air." Tooth-buds of per-
manent teeth seemed to be undamaged.
mus cortex type"). Histologic distinction between T-
cell lymphoblastic lymphoma and acute lymphoblas-
tic leukemia of T-cell type is made by assessing the
percentage of bone marrow blasts (<25% being char-
acteristic of T-cell lymphoblastic lymphoma). How-
ever, it has been recognized that this percentage is
somewhat arbitrary, because both conditions respond
equally well to continuous and sustained antileuke-
mic therapy.
Clinically, the disease begins in 80% of the cases
with a mediastinal mass, generally considered to
originate from the thymus. In addition, lymph nodes
in the supraclavicular region or elsewhere may be
enlarged. In 80% of the patients the bone marrow is
involved by the tumor process, and as a result a leu-
kemic blood picture appears. Possibly, the initial oral
lesion of this case originated from the bone marrow
of the mandible.
On histologic evaluation lymphoblastic lymphoma
displays a monotonous picture of diffuse lymphoid
cells that are relatively small- or medium-sized. The
cytoplasm of T-lymphoblasts is scanty and only
moderately basophilic. There is generally high mi-
totic activity. The cells are occasionally interspersed
with eosinophilic cells. T-cell and B-cell lympho-
blastic lymphoma must be differentiated by means of
immunohistochemistry, because B-cell neoplasms
require different therapeutic approaches.
Children with advanced T-cell lymphomas had a
poor prognosis until sustained multiagent chemother-
apy protocols were introduced. 6 Series examining
Burkitt's lymphoma and T-cell lymphoblastic lym-
p h o m a have shown that patients with localized (stage
I or II) lymphoma are responsive to chemotherapy. 7
In contrast, children with advanced (stage III or
436 Wolvius et al.
Fig. 3. A, Diffuse and m o n o t o n o u s picture of lymphoblasts is observed t h r o u g h o u t specimen. (hematox-
ylin-eosin-stained section, original magnification x330). B, L y m p h o b l a s t s stain positive for leukocyte (CD3),
a T-cell marker. (Original magnification x330).
IV) disease have a poor prognosis, and demonstrable
bone marrow or CSF involvement seems to be
invariably associated with short survival. For those
patients who have a relapse, this is especially true,
because drug resistance results in rapidly progressive
disease.
In conclusion, a rapidly growing tumor mass in the
maxillofacial area of a young Child is an unusual
finding. Apart from the well known Burkitt's lym-
phoma, other lymphoproliferative malignancies in-
cluding T-cell lymphoblastic lymphoma or leukemia
should be suspected. Fresh tissue should be provided
for adequate histologic and immunohistochemical
evaluation as well as for cytogenetic analysis and im-
munophenotyping by flow cytometry. The patient
should be referred to the pediatric oncologist for
staging and therapy in an early phase.
REFERENCES
1. Lennert K, Feller AC. Histopathology of non-Hodgkin's
lymphomas (based on the updated Kiel classification). Berlin:
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2. Anave Y, Kaplinsky C, Calderon S, Zaizov R. Head, lneck,
and maxillofacial childhood Burkitt's lymphoma: a retro-
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October 1996
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Reprint requests:
Paul van der Valk, MD, PhD
Department of Oral and Maxillofacial Surgery and Oral Pathology
Free University Hospital
Postbox 7057
1007 MB Amsterdam
The Netherlands