Intravascular Large B-Cell Lymphoma Presenting As Thyroid, Adrenal And Mesenteric Nodules

• Published in: AACE clinical case reports 2017-01, Vol.3 (2), p.e106-e110
• Main Authors: Dedhia, Priya H., Lopez, Kerri, Reddy, Anupama, McHugh, Jonathan B., Langer, Eric, Miller, Barbra S.
• Format: Article
• Language: English
• ISSN: 2376-0605; 2376-0605
• DOI: 10.4158/EP161228.CR

Objective: Identification of multiple endocrine abnormalities can result from endocrine or nonendocrine malignancies, genetic syndromes, or systemic diseases. Here, we evaluate an unusual presentation of several endocrine pathologies.Methods: We present a female with multiple endocrine abnormalities who was ultimately diagnosed with intravascular large B-cell lymphoma (IVLBCL). We also review the literature on IVLBCL, including presentation, management, treatment, and outcomes.Results: A 52-year-old woman with multinodular goiter presented with an enlarging thyroid nodule. Fine needle aspiration revealed a high-grade neoplasm of unclear etiology. Due to a history of nephrolithiasis, the patient was also evaluated for and found to have probable primary hyperparathyroidism. Magnetic resonance imaging performed for lumbar pain identified an incidental indeterminate 2.7-cm nonfunctioning adrenal nodule. [18F]-fluorodeoxyglucose (FDG) positron emission tomography–computed tomography (PET-CT) demonstrated an FDG-avid adrenal nodule and an additional mesenteric nodule with standardized uptake values of 12.8 and 11.9, respectively. The thyroid gland showed no clear focus of FDG uptake. She underwent total thyroidectomy, limited central neck dissection, and excision of an enlarged hypercellular parathyroid gland. Final pathology revealed the rare diagnosis of diffuse IVLBCL coursing throughout the vasculature of the thyroid, including an otherwise benign thyroid nodule. The patient received rituximab-cyclophosphamide-doxorubicin-vincristine-prednisone chemotherapy with intrathecal methotrexate postoperatively. Post-chemotherapy PET-CT documented resolution of the adrenal and mesenteric nodules. This is a rare case of IVLBCL presenting in the background of an enlarging thyroid nodule and a multinodular goiter.Conclusion: Pathology involving multiple endocrine organs necessitates careful evaluation and management with consideration for syndromal or other systemic disorders.Abbreviations: CNS = central nervous system; FDG = [18F]-fluorodeoxyglucose; IVLBCL = intravascular large B-cell lymphoma; PET-CT = positron emission tomography–computed tomography; R-CHOP = rituximab-cyclophosphamide-doxorubicin-vincristineprednisone