American Journal of Neuroradiology 27:1649-1650, September 2006
© 2006 American Society of Neuroradiology
Case Report
HEAD & NECK
Palatal Enlargement in Chronic Lymphocytic Leukemia
P. Vibhutea,
E. Carneiroc,
E. Gendena,b and
P.M. Somb
a Department of Radiology, The Mount Sinai School of Medicine of New York University, New York, New York
b Department of Otolaryngology, The Mount Sinai School of Medicine of New York University, New York, New York
c Department of Neuroradiology, São João Hospital, Porto, Portugal
Address correspondence to Peter M. Som, MD, Department of Radiology, Mount Sinai Hospital, One Gustave Levy Pl, New York, NY 10029; e-mail: Peter.Som{at}MSSM.Edu
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Abstract
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SUMMARY: Palatal involvement in chronic lymphocytic leukemia
(CLL) is rare and has only been reported 3 previous times in
the non-radiology literature. To our knowledge this is the first
imaging description of this entity. Based on our experience,
when smoothly lobulated, homogeneous masses are identified on
the oral surface of the hard palate, the diagnosis of CLL should
be considered.
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Introduction
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Chronic lymphocytic leukemia (CLL) is a B-lineage neoplasm of
prefollicular center cells that is usually associated with circulating
neoplastic small lymphocytes. From a morphologic and immunophenotypic
perspective, the malignant cells of CLL are identical to those
of nodal-based small lymphocytic lymphoma (SLL), and these 2
malignancies are thought to represent different manifestations
of the same disease.
1 CLL is the most common type of leukemia
in the Western hemisphere; its prevalence in Europe and America
ranges from 29% to 38% of all leukemias. Ninety percent of patients
with CLL are older than 50 years of age.
2 The most common oral
manifestations of the leukemias are gingival hypertrophy, petechial
hemorrhage and ecchymosis, infection, ulceration, and necrosis.
Less commonly, as a result of leukemic infiltrates, multiple
localized tumorlike growths can occur in the gingiva.
3 Similar
localized leukemic infiltrations occurring elsewhere in the
oral cavity have only rarely been reported in the literature.
3–5 In this article, we report on a patient with CLL who presented
with bilateral masses on the oral surface of the hard palate.
This represents only the fourth such reported case and the first
documented with imaging.
3–5
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Case Report
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A 62-year-old man presented with a 3-week history of bilateral
neck fullness and a mass in his palate. He had no pertinent
prior medical history. Physical examination showed bilateral
multiple nontender lymphadenopathy and a large firm palatal
mass with a midline ridge. Blood work revealed leukocytosis
and he was diagnosed as having chronic lymphocytic leukemia,
stage 1. MR imaging showed smoothly lobulated soft tissue masses
confined to the oral surface of the hard palate on either side
of the midline. Compared with the skeletal muscle, the masses
had overall intermediate T1-weighted (
Fig. 1) and moderately
high T2-weighted (
Fig 2) signal intensity and enhanced uniformly
(
Fig 3). The patient also had diffuse homogeneous discrete lymphadenopathy.
At this time, he is being clinically watched for any progression
of disease, which would signal the initiation of appropriate
therapy.
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Fig 1. Axial T1-weighted MR image shows nodular soft tissue masses (arrows) of low-intermediate signal intensity along the oral surface at the level of hard palate.
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Fig 2. Axial T2-weighted MR image shows that the masses have an intermediate signal intensity that is higher than in Fig. 1. The smoothly lobulated nature of the masses with a midline cleft can be well appreciated.
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Fig 3. Coronal contrast enhanced T1-weighted MR image shows uniform enhancement of the masses confined to the oral surface of the hard palate with an incomplete midline furrow.
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Discussion
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The leukemias are divided into acute and chronic forms based
on their clinical course, and the cell line involved further
classifies them into myelocytic and lymphocytic types. The immunologically
incompetent small neoplastic lymphocytes replace the bone marrow
and infiltrate lymph nodes and various organs. The marrow replacement
leads to a gradual loss of other cell lines, which manifests
clinically as malaise (anemia), hemorrhage (thrombocytopenia),
and infection (loss of competent white blood cells). The recirculating
neoplastic lymphocytes, which make up more than 90% of the peripheral
blood white cell count, infiltrate and enlarge the lymph nodes
and visceral organs.
4
The most common oral manifestations in leukemia, in order of frequency, include gingival hemorrhage, mucosal ulcers, infections, and gingival hyperplasia in tooth-bearing areas.6 Tooth loosening can occur from loss of the periodontal membrane. Leukemic infiltration of the tonsils and salivary glands can lead to their enlargement.4 In the head and neck, CLL most commonly presents as diffuse lymphadenopathy, often with quite large nodes. The oral manifestations of the chronic leukemias are less common than those associated with the acute leukemias.3, 5 In turn, the oral manifestations associated with CLL are the least common of all the chronic leukemias.
Palatal enlargement secondary to leukemic infiltration is rare. To our knowledge, there are only 3 prior, similar cases in the English language literature; none had CT or MR imaging studies.3–5 In these 3 prior patients, as with our patient, the palatal swelling was a painless, soft to firm, lobular mass with an overlying intact mucosa and an incomplete midline furrow. The relatively symmetric configuration of this process makes other palatal masses such as squamous carcinoma and minor salivary gland tumors quite unlikely.
Benign follicular lymphoid hyperplasia (FLH) affecting the oral surface of the hard palate is morphologically identical and, therefore, confusing in appearance and presentation. FLH in this location may present as a slowly growing, smoothly lobulated submucosal mass. Associated multicentric oral lesions and lymphadenopathy may also be present. Because isolated palatal enlargement can be the presenting feature of both CLL and FLH, and because FLH can recur after excision, it is important to distinguish these entities through pathologic examination.4, 7 Unlike CLL, which is a monoclonal leukemic infiltrate, FLH is characterized by an attenuated follicular lymphoid infiltrate within the lamina propria, replete with germinal centers.7, 8 These patients with either CLL or FLH palatal disease are typically asymptomatic for the palatal enlargement, except for ill-fitting upper dentures.4, 8,9
Other diagnostic considerations should include lymphomas of all grades, whether primary nodal or extranodal.4, 7, 9 Even though palatal involvement by lymphomas can present as soft, nontender, bilateral palatal swellings, in many cases, the palatal disease is unilateral, hard, painful, and accompanied by bone destruction.7 The final diagnostic differentiation between CLL, FHL, and the lymphomas can only be accomplished by pathologic examination.
Although a variety of other lesions (such as minor salivary gland neoplasms, torus palatinus, and infections and abscesses) can affect the hard palate, they are easily distinguished clinically and histologically from CLL.7
No prior description of the imaging findings of palatal involvement in CLL is available in literature. In our case, the MR imaging showed smoothly lobulated, uniformly enhancing soft tissue masses on either side of the midline confined to the oral cavity surface of the hard palate. There was overall intermediate T1-weighted and moderately high T2-weighted signal intensity (compared with skeletal muscle). These signal intensities are nonspecific with regard to the cell type that composes the mass. However, they suggest that this is not an inflammation-related process, because these latter conditions usually have higher T2-weighted signal intensities. In addition, the patient had the typical diffuse lymphadenopathy associated with CLL.10 The imaging findings correlated well with the clinical description of this lesion found in the literature.3–5
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Summary
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Palatal involvement in CLL is rare and has only been reported
3 previous times in the nonradiology literature. To our knowledge
this is the first imaging description of this entity. Based
on our experience, when smoothly lobulated, homogeneous masses
are identified on the oral surface of the hard palate, especially
in a patient who has a diffuse lymphadenopathy, the diagnosis
of CLL should be considered.
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References
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- Arber DA, Medeiros LJ. Hematopoietic lesions. In: Diagnostic Surgical Pathology of the Head and Neck. Gnepp DR, ed. Philadelphia: WB Saunders; 2001:725–76
- Ioachim HL. Chronic lymphocytic leukemia. In: Lymph Node Pathology. 2nd ed. Philadelphia: JB Lippincott; 1994:531–538
- Chaudhry AP, Sabes WR, Gorlin RJ. Unusual oral manifestations of chronic lymphatic leukemia. Report of a case. Oral Surg Oral Med Oral Pathol 1962;15:446–49[Medline]
- Henefer EP, Nelson JF, Beaupre EM. Palatal enlargement in chronic lymphocytic leukemia: report of case. J Oral Surg 1970;28:371–75[Medline]
- Porter SR, Matthews RW, Scully C. Chronic lymphocytic leukemia with gingival and palatal deposits. J Clin Periodontol 1994;21:559–61[Medline]
- Lynch MA, Ship II. Oral manifestations of leukemia: a postdiagnostic study. J Am Dent Assoc 1967;75:1139–44[Medline]
- Harsany DL, Ross J, Fee WE. Follicular lymphoid hyperplasia of the hard palate simulating lymphoma. Otolaryngol Head Neck Surg 1980;88:349–56[Medline]
- Menasce LP, Shanks JH, Banerjee SS, et al. Follicular lymphoid hyperplasia of the hard palate and oral mucosa: report of three cases and a review of the literature. Histopathology 2001;39:353–58[Medline]
- Nelson MA Jr, Stiff RH. An unusual manifestation of lymphoma. Oral Surg Oral Med Oral Pathol 1967;23:351–53[Medline]
- Som PM, Brandwein MS. Lymph nodes. In: Som PM, Curtin HD, eds. Head and Neck Imaging. 4th ed. St. Louis: Mosby; 2003:1865–934
Received April 14, 2005;
accepted after revision September 30, 2005.
TOP
Abstract
Introduction