A 14-year-old white British Caucasian boy presented with a 3-month history of malaise, lethargy, and weight loss. There was no history of fever or any other thyroid related symptoms. Past and family history were noncontributory and there was no recent foreign travel. On examination he was found to have a large predominantly left-sided firm thyroid swelling. Clinical examination was otherwise unremarkable with no lymphadenopathy or hepatosplenomegaly or signs of hypo/hyperthyroidism. Baseline haematological and biochemical investigations on admission were within normal limits (Hb 136 g/L, WCC 9.1 109/L, CRP <4 mg/dL). Levels of thyroxine, TSH, and calcitonin were normal. Lactase dehydrogenase (LDH) was slightly elevated at 352 U/L. Thyroperoxidase was elevated (1277 IU/mL) and thyroid stimulating hormone receptor antibodies (TRAb) of <5 U/L. CSF analysis showed no tumour cells. EBV PCR was negative as was HIV-I and HIV-II.\nA fine needle aspiration biopsy was carried out and was suspicious of NHL. The diagnosis of Burkitt type NHL (BL) was made following an open wedge biopsy. On microscopic examination, the tumour was composed of diffuse sheets of lymphoid cells () having round nuclei with coarse chromatin and conspicuous single to multiple nucleoli and scanty amphophilic cytoplasm. Numerous mitotic figures, apoptosis, and scattered tingible body type macrophages were also present. Tumour cells were positive for CD79A, CD20, and CD10 and negative for TdT and T cell markers (CD3, CD4, and CD8). Ki 67 showed proliferation index approaching 100%. Immunohistochemistry for EBV was negative. The diagnosis of BL was confirmed on fluorescence in situ hybridisation that showed tumour cell positivity for the t(8; 14) translocation.\nStaging CT () showed a large (6.7 cm × 5.8 cm × 6.1 cm) heterogeneous mass in the left lobe and isthmus of the thyroid which extended craniocaudally from level of C4/5 to T1/2, with some evidence of intratumoural necrosis. There was significant mass effect with displacement and compression of the trachea along with compression of the left carotid artery and lateral displacement of the left jugular vein. There were two small indeterminate lung nodules. Abdominally there were two focal cortical abnormalities in the right kidney and an ill-defined attenuation in the pancreatic head. There was no evidence of bone marrow involvement.\nHe was treated according to the Children's Cancer and Leukaemia Group guidelines for BL—Group B protocol. This consisted of initial induction chemotherapy with COP (cyclophosphamide [300 mg/m2], vincristine [1 mg/m2], and prednisolone [60 mg/m2—7 days]) followed by 2 courses of COPADM (doxorubicin 60 mg/m2, methotrexate 3 g/m2, cyclophosphamide 500 mg/m2/day—5 days, vincristine [2 mg/m2], and prednisolone [60 mg/m2—5 days]) and two courses of CYM (cytarabine 100 mg/m2/day—5 days and methotrexate 3 g/m2) chemotherapy. This was accompanied by intrathecal chemotherapy (the CSF contained no tumour cells at diagnosis). The patient had a good response to treatment with a rapid decrease in the size of the thyroid mass and resolution of the lung, kidney, and pancreas metastases. Reassessment after the first course of CYM showed a residual thyroid mass of 15 mm × 30 mm but with resolution of the lung, kidney, and pancreas metastases.\nBecause of residual radiological abnormality, and in accordance with the CCLG NHL protocol, a hemithyroidectomy was performed with the residual lesion showing no evidence of tumour cells on histological examination. Chemotherapy was then completed and he is now disease-free 3 years after end of treatment.
A 52-year-old woman presented to our Endocrinology Unit with a growing thyroid mass, which had enlarged so rapidly she had become unable to wear her motorcycle helmet in the weeks prior to her visit. She suffered from Hashimoto’s thyroiditis for which she was taking levothyroxine. There was no history of neck irradiation or family history of thyroid cancer. On examination, there was a large, firm thyroid nodule on the right side of the neck, without palpable cervical lymphadenopathy. TSH was 4.79 μU/mL with FT3 and FT4 within the reference range. Otherwise, there was only a mild thrombocytopenia. Thyroid ultrasonography showed a solid hypoechoic nodule in the right lobe of the gland, with significant internal vascularity and absence of calcifications (Figure ). FNA cytology with rapid on-site evaluation of the material adequacy showed that there were only atypical lymphoid cells with no thyrocytes and the specimens were considered suggestive of a lymphoproliferative disorder but insufficient to make a diagnosis, such that a CNB was scheduled for the following day.\nAfter checking the blood coagulation profile, the patient underwent a CNB, which allowed histological/morphological tissue analysis. This showed that normal thyrocytes were virtually all replaced by homogeneous medium-sized lymphocytes with scanty blue cytoplasm, round nuclei, coarse chromatin, and multiple small nucleoli. There were frequent mitotic figures and scattered macrophages ingesting apoptotic cells, giving to the tissue section the so-called ‘starry sky’ appearance (Fig. ). Overall, these features were consistent with the presence of a thyroid Burkitt’s lymphoma, and further investigations were ordered to confirm the diagnosis and evaluate the disease extent. A CT of chest and abdomen showed the 44x43x87 mm thyroid nodule with left tracheal deviation (Figure ) without other visible masses or lymph nodes. Bone marrow biopsy showed almost 100% lymphoid infiltration, consisting of a population of intermediate-sized blast-like cells, with prominent nucleoli, which were replacing all normal cells. These cells expressed CD10, CD20, and were negative for Bcl2, CD34, and TdT. Altogether these results led us to the final diagnosis of stage IV Burkitt’s lymphoma [].\nThe patient was admitted to our hospital’s Haematology Unit and was successfully treated with 3 cycles of Hyper-CVAD chemotherapy (cyclophosphamide, vincristine, doxorubicin and dexamethasone) completed in five months. The thyroid mass disappeared (Fig. ) and the platelets returned to baseline levels. At 60 months after diagnosis the patient is alive, and remains disease-free at regular follow-up.