MEN2 Syndrome-Related Medullary Thyroid Carcinoma with Focal Tyrosine Hydroxylase Expression: Does It Represent a Hybrid
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MEN2 Syndrome-Related Medullary Thyroid Carcinoma with Focal Tyrosine Hydroxylase Expression: Does It Represent a Hybrid Cellular Phenotype or Functional State of Tumor Cells? Ozgur Mete 1,2,3 & Ahmed Essa 4 & Anil Bramdev 4 & Navendren Govender 5 & Runjan Chetty 1,2
# Springer Science+Business Media New York 2017
Case History A 38-year-old woman presented with abdominal pain, rightsided large thyroid mass with dysphagia, lower backache, and weight gain. During the clinical examination, she was pale and was found to have hypertension. The initial laboratory testing showed a normal thyroid function along with low hemoglobin (7.6 g/dl, normal: 12–15 g/dl). Ultrasound identified a large thyroid mass occupying almost the entire right lobe. No cervical lymphadenopathy was detected. A fine needleaspiration biopsy of the thyroid mass was scheduled; however, the patient opted for total thyroidectomy given her elevated calcitonin (19930 ng/l; normal: 0–5 ng/l) and CEA (1425 ng/ ml; normal: 0–5 ng/ml) levels.
What is your Diagnosis? Figures 1, 2, and 3. Clinicopathological Diagnosis: MEN2 syndrome-related medullary thyroid carcinoma with focal tyrosine hydroxylase expression. * Ozgur Mete [email protected]
1
Department of Pathology, University Health Network, 200 Elizabeth Street, 11th floor, Toronto, Ontario M5G 2C4, Canada
2
Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada
3
Endocrine Oncology Site Group, The Princess Margaret Cancer Centre, Toronto, Canada
4
Lancet Laboratories, Durban, South Africa
5
Department of Surgery, Alberlito Hospital, Ballito, South Africa
The thyroidectomy specimen showed a dominant 5.0 cm well-differentiated neuroendocrine tumor consisting of spindle to epithelioid cells. The tumor displayed a predominant nested growth pattern resembling meningothelial whorls or a zellballen pattern of paragangliomas (Fig. 1). Microcystic change was seen in areas. No amyloid deposition was identified in sections examined. A panel of immunochemical stains was ordered by taking into consideration the differential diagnoses of medullary thyroid carcinoma and paraganglioma of the thyroid gland, as well as a remote possibility of metastatic neuroendocrine neoplasm originating from various sites. The tumor cells were diffusely positive for chromogranin-A and synaptophysin. Calcitonin (Fig. 2 a, b), and monoclonal CEA (mCEA) (Fig. 2 c, d) stained around 90% of the tumor cells. Staining for keratins were variable with focal reactivity for cytokeratin 7 (Fig. 2e) and patchy reactivity for low molecular weight cytokeratin (CAM5.2) (Fig. 2f). Interestingly, focal tyrosine hydroxylase reactivity was identified in up to 10% of the tumor cells (Fig. 3 a, d); however, TTF-1 remained positive in almost all tumors cells including those with focal tyrosine hydroxylase expression (Fig. 3 b, e). No immunoreactivity for GATA-3 (Fig. 3c), CDX-2, and epithelial membrane antigen was seen. Isolated tumor cells revealed positivity for serotonin. In addition to a very few sustentac
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