Primary Thyroid Lymphoma Presenting in Stridor

Abstract

Primary thyroid lymphoma is an uncommon disorder of the thyroid gland. It is an aggressive yet potentially curable malignancy when diagnosed early and treated appropriately. We present three cases who presented with rapidly enlarging neck swelling of approximately one month duration and stridor. Contrast enhanced computed tomography of neck showed enlarged thyroid gland compressing trachea and displacing major vessels. All three patients underwent isthmusectomy and tracheostomy under general anaesthesia. Histopathology showed features of diffuse large B cell lymphoma. They were planned to receive chemo-radiation. Two became seriously ill and one was lost to follow-up. Therefore, early consideration should be given to the possibility of primary thyroid lymphoma in patients presenting with rapidly enlarging neck swelling and stridor. Contrast enhanced computed tomography provides quick useful information to plan for surgical management of airway and immunohistochemistry of tissue provides key information in making the diagnosis. Prognosis is grave when the patient presents late.

Primary Thyroid Lymphoma: How Molecular Biology and Ancillary Techniques Can Help the Cytopathologist Overcome This Diagnostic Challenge

“…Figure 4 illustrates schematically the actual flow of management of these patients, from the clinical presentation to the final assessment of PTL. PTL usually mimics poorly differentiated and anaplastic thyroid carcinoma [7][8][9][16][17][18]. Differentiating these two entities is very important, as the treatment for PTL (and for DLBCL in particular), The results of the analysis indicated the presence of an insertion in exon 20 of ERBB2 gene c.2313_2324dupATACGTGATGGC (p.Ala775_GLy776insTyrValMetAla) with a VAF of 37.84% and classified as likely pathogenic according to the ACMG guidelines (ClinVar ID 13875; dbSNP rs397516975).…”

“…Figure 4 illustrates schematically the actual flow of management of these patients, from the clinical presentation to the final assessment of PTL. PTL usually mimics poorly differentiated and anaplastic thyroid carcinoma [ 7 , 8 , 9 , 16 , 17 , 18 ]. Differentiating these two entities is very important, as the treatment for PTL (and for DLBCL in particular), usually implicates only chemotherapy, while surgical resection is necessary for anaplastic carcinoma.…”

“…In this regard, a newly published study revealed the effectiveness of trastuzumab deruxtecan, an antibody–drug combination, on a variety of HER2-mutated hard-to-treat advanced solid tumors, indicating its potential as an agnostic tumor treatment for these different types of cancer [ 16 ]. In fact, the "agnostic" approach to cancer therapy requires that the most effective cure is chosen based on the presence of a specific molecular mutation in cancer, regardless of the tissue or histology of the tumor [ 22 , 23 , 24 , 25 ], and could potentially also be applied to PTL.…”