Published case study – Patient with an Intravascular Large B-Cell Lymphoma diagnosed late and with thyroid and cerebellum involvement

On 25^th August 2017, in Diagnostic Pathology, Bo Luo (Tongji Medical College, Wuhan City, Hubei Province, People’s Republic of China) and Jia-mei Chen (Renmin Hospital of Wuhan University, Whan City, Hubei Province, People’s Republic of China) et al. published a case report of 68-year-old male patient with an Intravascular Large B-Cell Lymphoma (IVLBCL) involving the thyroid and accompanied by bilateral nodular goiter.

Key Highlights:

Clinical History:

• History of hypertension (~20 years) and chronic bronchitis (~10 years)
• Admitted to hospital due to dyspnea and intermittent headaches for approx. 1 month
• Physical examination identified a 7x6cm non-tender mass in the neck
• No signs of hyperthyroidism or hoarseness; no family history of thyroid disease; levels of serum thyroid hormones and anti-thyroid autoantibodies were normal preoperatively
• CT found:
  • A slightly high density nodule (1.5 cm) in the cerebellum, which caused a slight space-occupying effect
  • Enlarged left lobar thyroid, occupied by a low-density mass (5.8x4.7x8.4cm), which protruded down to chest entrance level and pushed trachea to the right becoming narrow
  • A higher density mass of round nodules (1.3x1.2cm) without calcification or significant enhancement in the right lobar thyroid
• Main manifestations of left lobar mass: scattered calcified lesions, flaky necrosis areas of low density, and inhomogeneous enhancement
• Thyroid mass resection was carried out to establish a diagnosis and relieve symptoms
• Frozen section examination revealed nodular goiter of bilateral and focal atypical hyperplasia in the left lobe
• Patient underwent total thyroidectomy with central neck area lymph node dissection

Histological Features:

• Tumor cells located in interstitium of thyroid follicular cells without forming a clear boundary
• Low magnification identified invasion by lymphocytes: intravascular growth pattern, atypical nuclei, larger size compared to mature small lymphocytes
• Capillaries of lesion filled by atypical lymphoma cells
• Higher magnification identified large-sized tumor cells with minimal cytoplasm, thick nuclear membrane, irregular nuclear contours, prominent nucleoli, and dispersed chromatin

Immunohistochemistry:

• Large-sized cells positive for CD20, PAX-5, MUM-1, and BCL2; negative for CD3, CD5, CD43, CD10, CD23, Cyclin D1, CD138, CD30, ALK, CD56, MPO, S-100, TTF-1, thyroglobulin, and calcitonin
• KI-67 index = 85%

Treatment and Outcome:

• Diagnosed as “Classical” IVLBCL non-germinal center B-cell type
• Recommended patient undergo BCL6/BCL2 and MYC gene re-arrangement test and six cycles of R-CHOP
• Patient declined chemotherapy and passed away 5 months after resection operation

To the author’s knowledge, only two cases of IVLBCL involving the thyroid had been published in the literature previously. They note that their case was diagnosed late with both thyroid and cerebellum involvement. The group emphasize that “clinicians and pathologists should be aware of the existence of thyroid IVLBCL.” Lastly, they conclude that diagnosing IVLBCL earlier via biopsy and treatment with R-CHOP could have improved the patient’s survival.

Abstract:

BACKGROUND: Intravascular large B-cell lymphoma (IVLBCL) is a subtype of diffuse large B-cell lymphoma (DLBCL) that is rare and highly aggressive and that may progressively involve many organs. CNS (central nervous system), BM (bone marrow) and skin are the most common systems involved. To date, only 2 cases of IVLBCL involving the thyroid have been reported.

CASE PRESENTATION: Here, we report a case of IVLBCL involving the thyroid and accompanied by bilateral nodular goiter. In this case, a thyroid mass was identified in a physical examination of a 68-year-old male who initially presented with dyspnea accompanied by intermittent headache for approximately 1 month. Computed tomography scans revealed that the left lobar thyroid was occupied by a large, slightly lower density mass (5.8 × 4.7 × 8.4 cm). However, the patient had no hyperthyroidism or hoarseness. Levels of thyroid hormones and anti-thyroid autoantibodies in the serum were normal preoperatively. Thyroid mass resection was performed to establish a diagnosis and to relieve symptoms.

CONCLUSIONS: Pathological results of the surgical specimen revealed that large atypical lymphoma cells filled the capillaries in the lesion area. Immunohistochemical staining revealed that the large-sized tumor cells were positive for CD20, PAX-5, MUM-1 and BCL-2, and were negative for CD3, CD5, CD43, CD10, CD23, CyclinD1, CD138, CD30, ALK, CD56, MPO, S-100, TTF-1, TG (thyroglobulin) and CT (calcitonin). The Ki-67 index was estimated to be approximately 85%. The patient was subsequently diagnosed as "Classical" IVLBCL non-germinal center B-cell type. The patient declined chemotherapy and died in the fifth month after operation.