Michigan AACE: Case Presentation

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1 Michigan AACE: Case Presentation Marco De Santis D.O. Endocrinology Fellow McLaren Medical Center Macomb Background O 83 year old female with fatigue and TSH of 0.13 O Medical history T2DM, Osteoporosis, Dyslipidemia, HTN, macular degeneration O Surgical History B/L cataract surgery and ankle surgery O Medications Repaglinide, Raloxifene, Simvastatin, Pioglitazone, Aspirin, Metformin, Vitamin D/Calcium O Allergies NKDA O Social History No tobacco/etoh/illicit drugs O Family History Negative for thyroid disease; mother deceased from T2DM O Physical Exam Slightly anxious appearing, mild B/L hand tremor and moderately enlarged thyroid with left lobe nodule 1

2 Workup O 7/23/2007 O I-123 scan/uptake 4 hour uptake 4.8% and 24 hour uptake of 10.8%; hot nodule lower pole of the left lobe with decreased activity of the upper pole of the left lobe O 8/1/2007 O Thyroid ultrasound right lobe 1.2 x 1.2 x 3.7 cm, left lobe 3.4 x 4.2 x 6.7 cm; subcentimeter hypoechoic nodules in right lobe and large heterogeneous nodule replacing most of left lobe measuring 5.0 x 3.5 x 4.7 cm. Workup O 9/28/2007 O Repeat TFTs reveal Free T4 0.79, Total T3 138 and TSH 0.19 O Thyroid antibodies and TSI - NEGATIVE O 11/6/2007 O FNA biopsy left dominant nodule NEGATIVE for malignant cells O honeycomb sheets of small sized thyroid follicular cells the nuclei are slightly variable in size but have compact chromatin O Patient s daughter refused further evaluation or follow up until. 2

3 FAST-FORWARD O Re-evaluated on 12/17/2010 O TFTs FT4 2.0, FT3 3.7, TSH 0.15 O Anti-TPO antibody and TSI NEGATIVE O 12/27/2010 O Thyroid Ultrasound Right lobe 3.2 x 0.9 x 0.8 cm, left lobe 7.4 x 4.6 x 5.3 cm with complex solid and cystic nodule occupying most of the lobe and measures 6.4 x 4.0 x 5.2 cm (previously 5.0 x 3.5 x 4.7 cm). Further workup O 1/11/2011 O Technetium thyroid scan with I-131 uptakes O 2 hour 4.7% O 24 hour 12.1% O gland appears multinodular with significantly enlarged left lobe O Started on Methimazole 5mg po daily 3

4 Further workup O 2/28/2011 O FNA Biopsy left nodule ABNORMAL/ATYPICAL; marginally cellular and contains follicular cells mostly in honeycomb sheets and follicles. These cells have small nuclei and compact chromatin in keeping with a nodular goiter. A single syncytial fragment of follicular cells and individual cells show enlarged and slightly pleomorphic nuclei, finely granular to coarse chromatin; some have prominent nucleoli. Although this may represent metaplastic or degenerative changes in a cystic nodular goiter, an underlying follicular lesion such as follicular neoplasm cannot be excluded from this limited sample. Further workup O 5/25/2011 O FNA ReRe-biopsy left nodule ABNORMAL/ATYPICAL; Some of the fragments are arranged in syncytial pattern. The cells have slightly pleomorphic nuclei, finely granular to coarse chromatin and some have micro-nucleoli. Rare nuclear grooves and inclusions are seen. The remaining fragments are arranged in honeycomb sheets and follicles. These follicular cells have small nuclei and compact chromatin. The background contains scant colloid histiocytes and squamous cells. Although these atypical follicular cells may represent metaplastic or degenerative changes in a cystic nodular goiter, an underlying follicular lesion such as a cystically degenerated papillary thyroid carcinoma is in the differential, as is a thyroglossal duct cyst. 4

5 Meanwhile O 5/16/2011 O Free T4 1.6 O TSH - <0.15 O Methimazole increased to 5mg bid O 6/16/2011 O Free T O TSH 0.03 O AST 38, 38 ALT - 34 O Methimazole increased to 10mg qam and 5mg qpm O 7/22/2011 O Free T4 2.4 O TSH - <0.15 O Free T3 3.1 O AST 25, ALT - 21 O Methimazole increased to 10mg bid Hospital Course O Surgical evaluation with decision to proceed with total thyroidectomy on 8/2/2011 O Post-operatively she developed atrial fibrillation with RVR as well as bilateral vocal cord paresis necessitating tracheostomy O Short ICU stay for acute respiratory failure with rapid wean off ventilator O Thyroid hormone replacement therapy was initiated 5

6 Pathology Pathology 6

7 Pathology Pathology 7

8 Pathology Report O Tumor type: Squamous cell carcinoma O Tumor laterality/focality: Left lobe, multifocal O Tumor size: Almost entire left lobe (6.0 x 4.5 x O O O O 3.5 cm) Tumor capsule: None Margin: Involved Extra thyroidal extension: Present, multifocal Stage O pt3 - >4 cm O pn0 6 examined O pm - NA Pathology Report O Immunocytochemistry O P53: POSITIVE O Ki 67: high proliferative index O Comment: Primary Squamous Cell Carcinoma is extremely rare. For this tumor to be a primary squamous cell tumor the following possibilities must be ruled out: metastatic carcinoma from another site, direct invasion from neighboring tumor such as laryngeal carcinoma, esophagus or trachea 8

9 Oncologic workup O Triple endoscopy was recommended O Laryngoscopy Bilateral vocal cord paresis; no evidence of laryngeal edema, masses or lesions of the naso, oro or hypopharynx. O EGD Patient refused O Bronchoscopy Patient refused Imaging O Chest XX-Ray (8/6/2011) Small left pleural effusion with likely atelectatic changes but difficult to exclude underlying infiltrate O CT Neck/Chest ( 8/8/2011) Post-surgical changes in the thyroid bed with fluid collections measuring 2.2 x 2.2 cm and 1.9 cm on the right. 2.2 x 1.9 cm mass lesion within the left lower lobe of the lung. lung Small satellite nodules are seen inferiorly. This is suspicious for primary or metastatic disease. 9

10 CT guided lung biopsy O 8/12/2011 O Benign alveolar and bronchial tissue present O No evidence of malignancy in sections examined 10

11 Follow up Imaging O 10/12/2011 O PET-CT O Low grade tracer uptake in pretracheal soft tissues in the region of the thyroid bed and prior tracheostomy. Asymmetric uptake adjacent to the trachea on the left with focal increased soft tissue activity measuring maximum SUV 3.8. Additional tumor focus cannot be ruled out in this area. There is no elevation of FDG avidity at a previously identified substernal mass with maximum SUV 1.3. Previously identified pulmonary parenchymal nodules are without evidence of elevated FDG avidity. 11

12 Lost to follow up O As of November 14, 2011, the patient refused any further diagnostic workup or imaging but is reported to be living and feeling relatively well 12

13 Squamous Cell Carcinoma of the Thyroid O Rare malignancy O 0.7%-3.4% of all malignant thyroid tumors O Has been reported in up to 40% of Papillary Carcinomas, especially the Tall Cell Variant O Aggressive O Presentation rapidly growing neck mass, dysphagia, hoarseness, and sometimes dyspnea O Poor prognosis Squamous Cell Carcinoma of the Thyroid O Etiological theories: O Metaplasia - resulting from inflammation (Hashimoto s) O Embryonic nest results from remnants of the thyroglossal duct, thymic epithelium and ultimobranchial body O De-differentiation from existing papillary, follicular, medullary or anaplastic carcinoma 13

14 Squamous Cell Carcinoma of the Thyroid O Diagnosis O Must exclude direct extension from adjacent sites or secondary metastases from squamous cell carcinoma in other distant organs O Treatment O No consensus O Total Thyroidectomy O Chemotherapy/XRT O Cisplatin, Paclitaxel, Doxorubicin, Cyclophophamide, 5-Fluorouracil O? Role for RAI therapy O If follicular origin Primary Cancers that Metastasize to the Thyroid O Most common: O Renal (33%) O Lung (16%) O Breast (16%) O Others reported including liver, colorectal, esophagus and uterus 14

15 Hot Nodules O O-4% reported prevalence of hot nodules harboring malignancy O 7.5% in one study O Hot nodules almost never represent clinically significant malignant lesions, whereas cold nodules have a reported malignant risk of about 5% to 8%. - AACE Thyroid Nodule Guidelines O When do we perform an FNA biopsy? References 1) 2) 3) 4) 5) 6) Daumerie C. Prevalence of thyroid cancer in hot nodules. Ann Chir 1998;52(5): Kleer C. Squamous Cell Carcinoma of the thyroid: An aggressive tumor associated with Tall Cell Variant of Papillary Thyroid Carcinoma. Mod Pathol 2000, 13 (7): Mercante G. Mixed squamous cell carcinoma and follicular carcinoma of the thyroid gland. Auris Nasus Larynx 2012, 39: Nakhjavani MK. Metastasis to the thyroid gland. A report of 43 cases. Cancer 1997 Feb 1;79(3):574-8 Tunio M. Primary squamous cell carcinoma of thyroid: a case report and review of literature. Head and Neck Oncology 2012, 4:8. Yves F. Combined therapy for thyroid squamous cell carcinoma. Head and Neck

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