Poorly differentiated thyroid carcinoma (PDTC) is an uncommon form of thyroid cancer, accounting for less than 5 % of all cases. It tends to be more clinically aggressive than differentiated thyroid cancers. While thyroidectomy is the mainstay of treatment, radioactive iodine plays an adjunctive role as well as TSH suppression with thyroid hormone. Some patients with advanced disease, may benefit from external beam radiation and/or systemic therapy. Case Description: A 94-year-old man presents with a left neck mass. His past medical history is significant for controlled hypertension. He notices a slowly growing mass while shaving over few months. He has no dysphagia, hoarseness, or shortness of breath. He has no history of radiation exposure and no family history of thyroid disease. A CT of the neck with contrast shows multiple pathologic appearing lymph nodes on the left side of the neck. The largest measures 5.4 x 4 x 3.9 cm, displacing the carotid space, and indenting the left internal jugular vein. The left lobe of the thyroid is heterogeneously enlarged compared to the right lobe with multiple irregular hypo-enhancing nodules. A fine-needle aspiration of the left cervical lymph node is positive for malignant large cells in sheets and clusters. Some clusters have a vague papillary/trabecular type arrangement. A total thyroidectomy and left level II-IV neck dissection is performed. Pathology shows multifocal poorly differentiated papillary thyroid carcinoma measuring 2.2 cm in greatest dimension with extensive necrosis. There is a background of lymphocytic thyroiditis. There is extensive vascular invasion (>4 foci) and extra-thyroidal extension invading only the strap muscles. There is metastasis to 7 of 12 examined lymph nodes. The largest metastatic deposit at level III measures 2.8 cm with extra-nodal extension. Tumor is negative for BRAF-V600, NRAS and TERT. His postoperative labs are as follows: TSH 346 (post-rhTSH) uiu/ml (0.5-5.0), free T4 0.87 ng/dl (0.6-1.15),Tg 1.0 ng/ml and anti-Tg <0.9 iu/ml. Adjuvant therapy with 125 mCi of I-131 is performed as well as thyroid replacement with levothyroxine 100 mcg daily. A whole-body scan shows residual uptake in the thyroid bed and increased uptake to the left of the thyroid bed. FDG PET scan shows 2 left parapharyngeal nodes with SUV of 6.94 (0.8 cm), SUV of 3.73 (1.8 cm). There is a focus of increased activity at lateral left thyroid bed SUV of 4.59. There is a right mediastinal paratracheal lymph node with SUV of 9.79 measures 1.5 x 1.2 cm. Considering patient age, external beam radiation is deferred with patient agreement as the risk outweighs the benefit. Patient will be followed by imaging closely. Conclusion: PDTC is an uncommon form of thyroid cancer. These tumors generally do not produce thyroglobulin and close monitoring with imaging is essential to detect early metastatic disease. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstra...
Introduction: POEMS syndrome is a rare disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. The cause of POEMS syndrome is unknown, although chronic overproduction of proinflammatory and other cytokines is thought to be a contributing factor. Diagnosis requires thorough evaluation as well as high clinical suspicion. Case Presentation : A 44 year old man presents to emergency department due to abdominal swelling and dyspnea for one week. He does endorse diffuse joint pain, fatigue and peripheral neuropathy. He denies constipation, diarrhea, fever and chills. His vital signs on presentation are significant for hypotension with BP of 94/63 mmHg and tachycardia at 108 bpm, otherwise unremarkable. His physical exam is significant for a chronically ill appearing male, with a distended abdomen, non-tender to palpation, with decreased bowel sounds throughout, and splenomegaly but no fluid wave. He has bilateral pitting edema to the ankles. His presenting labs are significant for WBCs 12.7 10^3/uL Hemoglobin 12.3 gm/dL, hematocrit 36.3%, platelets 816 10^3/uL, Na 139, K 4.1, Co2 of 21, Cr. 1.19, glucose 74 mg/dl. CT of the abdomen and pelvis shows large volume ascites, hepatosplenomegaly, lymphadenopathy and an osteosclerotic lesion of T10. Paracentesis is consistent with portal hypertension with no evidence of infection. Echocardiogram is consistent with pulmonary hypertension. Patient has continued multidisciplinary evaluation for his ascites, thrombocytosis, weight loss, lymphadenopathy, organomegaly, and osteosclerotic lesion. Vascular endothelial growth factor is elevated at 264 pg/mL. Kappa and lamba light chains are both elevated at 99.5 mg/L and 92.6 mg/L, respectively. Bone marrow biopsy demonstrates increased plasma cells that rim lymphoid aggregates with lambda predominance suggestive of POEMS syndrome. TSH is found to be 12.33 ulU/mL (0.5-5.0), FT4 0.52 ng/dL (0.6-1.15), free T3 of 1.1 (2.4-4.2). Cosyntropin stimulation test reveals a baseline cortisol of 7.2 mg/dl and inadequate peak cortisol of 15.1 mcg/dL. Patient is started on steroids followed by thyroid replacement therapy. Further evaluation of endocrinopathy reveals LH 14.3 miu/ml (2-12.0), FSH 20.2 miu/ml (1.5-15), testosterone 376 ng/dl (300-890), SHBG 29 nmol/l (11-80), free testosterone 74 pg/ml (47-244), prolactin 14 ng/ml (1-18). Brain MRI is normal. He is then started on chemotherapy followed by bone marrow transplant. Discussion: Endocrinopathies are central feature of POEMS. Degree resolution of endocrinopathies following successful therapy of the disease is unknown. Patients on hormonal replacement therapy who receive systemic treatment of the plasma cell disorder, should receive ongoing evaluation of endocrine abnormalities to prevent drug overdoses.
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