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Mantle B cell lymphoma

Case contributed by Bruno Di Muzio
Diagnosis certain

Presentation

Gum bleeding and spontaneous loss of the second inferior premolar tooth on the left. The patient has been referred after a focal gum/mandibular biopsy.

Patient Data

Age: 70
Gender: Female

Panoramic radiograph (OPG)

x-ray

Panoramic radiograph showing bone lucency within the expected dental alveolus of the inferior left second premolar tooth representing a biopsy tract, but otherwise with unremarkable appearances.

MRI Supra-hyoid Neck

mri

There is an enhancing soft tissue mass within the gun at the anterior left mandibular region. The bone biopsy tract is noted. 

CT Neck Chest A/P staging

ct

Ill-defined soft-tissue again noted with infiltration into the left mandibular body, slightly smaller in size when compared to the previous MRI. Bone biopsy defect noted. A few rounded left submandibular enlarged lymph nodes are now present, the largest measuring 18 x 13 mm in level IIA. A few enlarged nodes also noted in the upper left cervical chain and supraclavicular regions, measuring up to 22 x 15 mm in the level V. Multinodular thyroid gland. 

Enlarged mediastinal lymph nodes: pre-tracheal measuring 18 x 16 mm and infra carinal measuring 21 x 18 mm. Enlarged nodes also in the left axilla measuring up to 28 x 17 mm.

Abdomen and pelvis Ill-defined and infiltrative enhancing mass involving the fat planes of the right renal hilum and tracking down along the retroperitoneum to the right iliac fossa, where it partially encases the iliac vessels and also extends around the perirectal fascia to the presacral space. The infiltrative mass also crosses the midline retroperitoneum at the level of the 3rd part of duodenum involving enlarged paraaortic lymph nodes. There is also a separate, ill-defined soft tissue deposit inferolateral to the left adrenal. Multiple enlarged lymph nodes scattered through the mesentery root. Bilateral extraperitoneal enlarged pelvic lymph nodes, measuring up to 44 x 25mm on the right. No focal liver lesions. The spleen, pancreas, kidneys and adrenal glands are normal. No abnormality of the bowel is identified.

Case Discussion

Enlarged neck, chest, and abdominal lymph nodes, as well as an ill-defined retroperitoneal mass, in keeping with lymphoproliferative disease. 

The mandibular biopsy has shown:

MICROSCOPIC DESCRIPTION: The biopsy shows fragments of trabecular bone and soft tissue infiltrated by atypical lymphoid cells. Several fragments are crushed, limiting assessment. In the better-preserved areas, There are large atypical cells, which are occasionally the predominant cell type. There are abundant mitotic figures. The atypical cells are expressing B cell phenotype (CD20 and CD79a positive). PAX5, MUM1 and c-Myc are positive. BCL2, BCL6 and CD10 are difficult to interpret due to crushing artefact. CyclinD1, CD138, AE1/3 and EBER-CISH are negative. Ki67 is >90% in the atypical large cells.

DIAGNOSIS: Soft tissue left maxilla: Atypical lymphoid proliferation with features favoring Diffuse Large B-cell Lymphoma. 

Further nodal biopsy has confirmed the diagnosis. 

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