Medullary thyroid carcinoma with nodal metastases

Case contributed by Ammar Ashraf
Diagnosis certain

Presentation

Right neck swelling for the two months. No hearing or swallowing complaints. No fever or weight loss.

Patient Data

Age: 65 years
Gender: Male

Findings: An enlarged right upper cervical lymph node ( level II) showing significant peripheral enhancement and central necrosis, measuring 2.5 x 3.2 cm. Another necrotic lymph node measuring 12 x 13 mm is seen in the superior mediastinum, left paratracheal in location. No additional enlarged cervical lymph nodes. Average size heterogeneous thyroid gland with multiple thyroid nodules in both lobes. A few small nodular soft tissue densities are appreciable in both parotid glands which are likely small intraparotid lymph nodes.  A note is made of mildly tortuous and medialized right common and internal carotid arteries (retropharyngeal carotid artery). 

Impression: Enlarged heterogeneous right upper cervical and superior mediastinal lymph nodes which are likely metastatic in nature. Bilateral thyroid nodules. For further evaluation with thyroid ultrasound and FNAC.

Selected images of the thyroid gland from CT chest. Average size heterogeneous thyroid gland with multiple thyroid nodules in both lobes; some of these nodules are hypo and some are hyper-enhancing. No obvious calcifications are seen in the thyroid gland.

Selected images of the right thyroid lobe nodule and right cervical lymph node (level II) at the time of ultrasound guided fine needle aspiration cytology (FNAC). A tiny echogenic focus (likely a calcification) is seen in the hypoechoic thyroid lobe nodule, which is not appreciable on the CT scan.

Case Discussion

FNAC of right thyroid lobe nodule & right cervical lymph node: Medullary carcinoma (thyroid & lymph node). The immunostain done in both blocks (block 1=right thyroid lobe nodule, block 2=right cervical lymph node) show the following results: CD45 LCA:  Negative. Thyroglobulin:  Negative. TTF-1:  Positive. Calcitonin:  Positive. This immunophenotype is consistent with medullary carcinoma. The immunostain with the antibody anti-CEA, chromogranin A is strongly and diffusely positive in both blocks.  However, synaptophysin is positive in block 1 but negative in block 2.  This immunophenotype also is in favor of medullary carcinoma.

The patient underwent total thyroidectomy, central neck dissection and right-sided modified radical neck dissection (MRND).  Histopathology: Multifocal medullary carcinoma thyroid (both lobes) with lymph node metastases (level II & VI). 12/43 lymph nodes involved. Congo red special stain is positive and highlights the amyloid deposition. The immunostain with the antibody anti-calcitonin highlights the diffuse infiltration of thyroid tissue by the invasive medullary carcinoma with the features of nodular C-cell hyperplasia. Pathologic Stage Classification (pTNM, AJCC 8th Edition):  pT4, pN1b (level I and level VI).

Other laboratory investigations showed high calcitonin=3880 +++ pg/ml (normal up to 8.4), and chromogranin A=233+ ug/l (normal is <102) levels.

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