Case A 76-year-old male with status post thyroidectomy in 2017 for compressive multi-nodular goiter. Pathology reported as benign thyroid tissue with follicular hyperplasia. In 2020, he presented with incidental finding of left neck mass on MRI. Ultrasound neck confirmed two extra-thyroidal masses left neck largest dimension of 2.9 cm. FNA reported as benign follicular cells favoring benign thyroid tissue. Surgical resection of the neck masses revealed benign thyroid tissue with focal oncocytic features and two benign lymph nodes. In 2021, he presented with left chest mass. Biopsy reported benign thyroid tissue, no evidence of papillary cancer. PET CT confirmed hypermetabolic chest mass, multiple lytic bone lesions and pulmonary nodules. Referred to Endocrinology for further evaluation, Thyroglobulin level elevated at >4500 ng/mL (Tg Ab <1.0 IU/mL). Molecular testing of chest wall mass was positive for TERT and HRAS mutations, loss of 22q and gain of 17q on microarray consistent with changes found in thyroid cancer. Surgical resection of the chest wall mass not feasible due to size and location. External beam radiation for cytoreduction of the sternal mass planned and radioactive iodine therapy if tumor RAI (radioactive iodine) avid. He is currently not a candidate for tyrosine kinase inhibitor (TKI) as he recently had acute coronary event. Zoledronic acid infusions initiated for bone involvement. Discussion Thyroid carcinomas that exhibit vascular invasion, or anaplastic dedifferentiation are readily and consistently diagnosed. However, the morphology of primary oncocytic thyroid tumors is similar to their non-oncocytic counterparts posing a huge challenge for the pathologist. This controversial and often confusing area of thyroid pathology requires careful evaluation for accurate diagnosis and management for patients with oncocytic thyroid lesions. Molecular and microarray testing should be considered when in doubt. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
Introduction Parathyroid cancer (PC) is a rare malignant tumor which represents <1% of patients with primary hyperparathyroidism (PHPT). With less than 1000 reported cases, PC is the rarest endocrine cancer. Complicating its rarity, is the known resistance to radiation and chemotherapy, and complete surgical resection is the only curative treatment available. Genomic sequencing targeted therapy has shown encouraging results. We hereby present a case where Pembrolizumab was used in treatment of metastatic PC. Case A 50 year old lady with a history of nephrolithiasis, hypercalcemia(HC) presented for evaluation of neck mass. She had a recent admission to outside hospital for HC, dehydration, bone pain, polyuria, constipation and workup was suggestive of PHPT with Calcium(Ca) 13.2mg/dL and PTH 1,500pg/ml. Neck ultrasound demonstrated a 3 cm nodule in the left thyroid and fine needle aspiration was suspicious for malignancy. She underwent left hemithyroidectomy, and left parathyroidectomy. PTH levels pre-, intra- and postoperatively were 680, 220, 20 pg/ml respectively with normalization of Serum Ca. Histopathology revealed high grade PC with lymphovascular and perineural invasion. Postoperatively she received adjuvant radiotherapy. Ten months later CT Neck surveillance revealed a 4cm Left prevertebral(PV) mass with recurrence of bone pain, PTH 110pg/ml, and Ca-11.2mg/dL. Parathyroid scan showed Left PV mass and Left level 2 lymph node. PET/CT later revealed fluorodeoxyglucose avidity of the recurrent mass, as well as bilateral lung, right ventricle, and pelvic lesions. Incisional biopsy confirmed carcinoma consistent with PC. Her neck lesions were deemed unresectable. Germline mutation testing confirmed Next Generation Sequencing: PDL1 5% by CPS, TMB 10 mut/Mb, IDH2, JAK1, KMT2C, NF1, PIK3RI. On the basis of TMB >10 mut/Mb Pembrolizumab was then initiated with good response, noting improvement in symptoms, and normalization of Ca 8.7mg/dL and PTH 35pg/ml. Within 4 months of therapy Surveillance CT showed an approximate 40% reduction in tumor burden noting decrease in size of neck mass and lesions in lungs, right ventricle, and pelvis. Discussion Approximately 10%-30% of PCs have metastasis at presentation, most commonly to the lung, bone, or liver. However they can often develop local recurrence and or distant metastases despite early intervention. Surgical resection remains the mainstay of treatment, and is often used to decrease tumor burden which has been shown to improve morbidity. However when surgical resection is not an option, newer approaches using gene sequence targeted therapy may be an attractive option in the future as was demonstrated in this patient. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.
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