PMC5733220_01
A mass in the left submandibular region.
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fig1
During a monitoring visit in March 2017, the patient noted the appearance of a dense painless mass in the left submandibular area, which rapidly increased in size within a month (Figure 1).
Submandibular salivary gland. (a) The architecture of the submandibular salivary gland is replaced by a diffuse small lymphocyte infiltrate H&E staining, x100. (b) The SLL component strongly expresses CD20, highlighting focal accumulations of CD20+ B cells, x100. (c) B cells express LEF1, x100. (d) Proliferative activity marker Ki-67 in the B-cell area is expressed by up to 20% of the cells, x100.
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Histologic examination revealed diffuse infiltration of the salivary gland tissue with small lymphocytes with atrophy of glandular parenchyma (Figure 2). Immunohistochemical staining showed that only a part of the infiltration, mainly the focal cluster, was composed of lymphocytes expressing CD20 (Figure 2), CD79a, PAX5, CD5, CD23, LEF1 (Figure 2), and CD43. Up to 20% cells of the focal cluster expressed the proliferative activity marker Ki-67 (Figure 2).
Submandibular salivary gland. (a) Immunohistochemistry for CD3 shows diffuse infiltration by CD3+ T cells, x50. (b) T cells express CD2, x50. (c) T cells express CD5, x50. (d) T cells express TIA1, x200. (e) Proliferative activity marker Ki-67 in the T-cell area is expressed by up to 70% of the cells, x100. (f) The neoplastic T cells coexpress CD79a, x50.
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TIA1, and granzyme B and coexpressing CD79a, with 70% Ki-67 positivity (Figure 3). The study of imprints of the submandibular salivary gland showed two types of lymphocytes: one corresponding to CLL/SLL lymphocytes and the other with irregular nuclei and cytoplasmic granules (Figure 4).
The cytology of the imprint of the submandibular salivary gland. Two lymphocyte populations can be detected. Some lymphocytes correspond to the characteristics of CLL/SLL lymphocytes. The others have an irregular shape of the nucleus, and some contain cytoplasmic granules. Romanowsky-Giemsa staining, x1000.
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TIA1, and granzyme B and coexpressing CD79a, with 70% Ki-67 positivity (Figure 3). The study of imprints of the submandibular salivary gland showed two types of lymphocytes: one corresponding to CLL/SLL lymphocytes and the other with irregular nuclei and cytoplasmic granules (Figure 4).
Molecular diagnostics of composite lymphoma. Clonality of the B cells and T cells was assessed for IGH and TCRbeta gene rearrangements by PCR. Clonal peaks of IGH rearrangement were detected with framework I-III primers. An incomplete rearrangement was revealed of beta chain of T-cell receptor: a clonal peak at 193 bp (panel C).
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Study of the DNA extracted from freshly prepared submandibular salivary gland tissue revealed clonal IGH and clonal TCRbeta gene rearrangements (Figure 5).
Positron emission tomography-computed tomography. Enlarged pericardial lymph node in the anterior mediastinum with SUVmax = 6.1.
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Positron emission tomography-computed tomography showed an increase in the size of the spleen, right submandibular, bilateral cervical, left supraclavicular and axillary, mediastinal, retrocrural, abdominal, retroperitoneal, and bilateral external iliac lymph nodes, with slightly increased accumulation of fluorine-18 fluorodeoxyglucose (SUV (standard uptake value) max = 3.7) as well as an enlarged pericardial lymph node in the anterior mediastinum (SUVmax = 6.1) (Figure 6).