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.
A 70-year-old male presented a rapidly expanding mass of the neck associated with history of airway compression symptoms; progressive dyspnea and dysphonia lasting for 4 weeks, in a context of apyrexia and impairment of general condition. The patient was admitted to the hospital because of increasing dyspnea and urgently received a tracheostomy.\nA biopsy of the cervical mass was carried out and the histological examination showed diffuse infiltration of the thyroid gland by a monotonous population of atypical intermediate-sized lymphoid cells (Fig. ). These last possess scanty amphophilic to basophilic cytoplasm with centrally located nuclei of irregular shape, displaying dispersed basophilic chromatin, and frequent apoptotic figures (Fig. ). Scattered tingible body type macrophages were also present. Little residual thyroid follicles and some areas of necrosis was observed.\nImmunohistochemical staining was then performed and the tumour cells were positive for CD20, CD10 and BCL6. Ki-67 showed proliferation index approaching 100 %. CD3 and CD5 stained the background T cells (Fig. ). Immunoreactivity for Epstein-Barr virus (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. Bone marrow examination was normal.\nThe patient was transferred to the Clinical Haematology department. On physical examination, he was apyretic and hemodynamically stable with a cervical armouring by a huge mass of hard consistency. Neurological examination shows no sensorimotor deficits.\nOther systemic examinations were normal, without any palpable lymphadenopathy or organomegaly.\nThe computerized tomography (CT) scan showed a heterogeneous process of the thyroid gland measuring 10.5 × 8.2 × 6.5 cm in size, extending up towards the laryngeal region, infiltrating the right vocal cord and reducing the laryngeal lumen (Fig. ).\nThe thoraco-abdominal CT scan showed no other localization.\nThe examination of the cephalorachidian liquid showed no central nervous system involvement. The patient was diagnosed with thyroidal Burkitt lymphoma, stage I of Murphy [].\nAfter cardiac, renal and liver functions assessment, the patient received chemotherapy according to the LMBA02 protocol [], group A (Age > 60 years, no CNS nor bone marrow involvement), with a first course of COP (Cyclophosphamide, Vincristine and Prednisone) and intrathecal Methotrexate, followed a week after by a course of R-COPADEM (Rituximab at day 0 and day 6, Cyclophosphamide day 2,3 and 4, Prednisone from day 1 to 5, Doxorubicineat day 2, high dose Methotrexate at day 1 with folic acid rescue from day 2 to day 6 and intrathecal chemotherapy at day 2 and day 6).\nAt day 15 of chemotherapy, the patient developed febrile neutropenia, refractory to broad-spectrum antibiotics and antifungals. The evolution was marked by the installation of a septic shock with acute respiratory distress syndrome that led to his transfer to intensive care unit where he was intubated and ventilated. The patient died 2 days after.