Indian Journal of Pathology and Microbiology
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Year : 2014  |  Volume : 57  |  Issue : 1  |  Page : 151-152
Papillary variant of medullary carcinoma thyroid

Department of Pathology, Dr. Ram ManoharLohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

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Date of Web Publication17-Apr-2014

How to cite this article:
Shukla S, Awasthi NP, Husain N. Papillary variant of medullary carcinoma thyroid. Indian J Pathol Microbiol 2014;57:151-2

How to cite this URL:
Shukla S, Awasthi NP, Husain N. Papillary variant of medullary carcinoma thyroid. Indian J Pathol Microbiol [serial online] 2014 [cited 2022 Aug 16];57:151-2. Available from: https://www.ijpmonline.org/text.asp?2014/57/1/151/130933


Medullary thyroid cancer (MTC) is a calcitonin-producing neuroendocrine tumor that accounts for less than 10% of all thyroid carcinomas and arises from the parafollicular C cells.It is characterized by peculiar morphological, functional and behavioral features.Majority of MTC are sporadic and about 10-20% are familial.We describe a rare case of papillary variant of medullary carcinoma of the thyroid gland that poses a diagnostic challenge for the pathologist.

A 45-year-old female presented with painless enlargement in the left side of the neck for 4 years. On examination, the swelling moved with deglutition and was more prominent on the left side. A computed tomography (CT) scan revealed an encapsulated lesion involving the left lobe of the thyroid. The hormone profile was euthyroid. A total thyroidectomy with bilateral neck dissection was performed.

On gross evaluation, the external surface of the gland was smooth. The cut-surface of the left lobe showed a solid white growth partly encapsulated measuring 3.5 cm × 3 cm × 1.5 cm with a normal thyroid parenchyma at the periphery. Capsular break was not evident. Cut-surface of the right lobe and isthmus were unremarkable.

On microscopic evaluation, the left lobe of the thyroid showed malignant epithelial tumor disposed in sheets, lobules and focal papillary configuration. The tumor cells had regular nuclei with condensed chromatin and finely granular cytoplasm. Majority of the tumor showed cells with optically clear, ground glass nuclei having nuclear pseudoinclusions and nuclear groves. Foci of hyalinization and fibrosis were also evident.The right lobe of the thyroid and the isthmus showed normal histology. Metastatic tumor deposits were evident in one of 36 upper cervical lymph nodes.

A diagnosis of papillary carcinoma with dedifferentiation was considered. Subsequently, the biochemical tests of the patient revealed elevated levels of serum calcitonin and normal alkaline phosphatase levels. Congo red stain highlighted the red amyloid, which showed agreen bi-refringence in polarizing light [Figure 1]. Immunohistochemistry in tumor sections showed a diffuse calcitonin positivity in the tumor and thyroglobulin was negative. A final diagnosis of papillary variant of medullary carcinoma of thyroid was confirmed.
Figure 1: (a) Gross evaluation of the thyroid showing a solid neoplasm in the left lobe of thyroid. (b) Microphotograph showing tumor cells in sheets with partial papillary configuration (hematoxylin and eosin [H&E], 100 digital magnification). (c) clear nuclei with prominent nucleoli (H&E, 400 digital magnification). (d) Foci of amyloid in the tumor showing (Congo red, 100 digital magnification). (e) Green birefringence in polarizer (Congo red, 100 digital magnification). (f) calcitonin expression in tumor cells (3,3' Diaminobenzidine) 100 digital magnification)

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The papillary variant of medullary carcinoma is a rare distinct entity that has very few cases reported in the literature. [1],[2] In most cases reported, about 20-25% of the tumor cells demonstrated the characteristic clear nuclei of papillary carcinoma. Our case presented with focal papillae but diffuse orphan annie nuclei. The presence of amyloid aided in diagnosis. Metastasis to lymph nodes is a rare finding in MTC. In our case also, only one lymph node showed a small metastatic deposit while papillary thyroid cancer (PTC) usually tends to have early and extensive lymphatic spread. The tumor encapsulation seen is also not common in PTC.

Histologically, MTC consists of solid masses of rounded, polygonal and spindled neoplastic cells of various sizes often mixed with amyloid. Variants of MTC described in the literature include papillary, pseudopapillary, follicular, clear cell, oxyphil, small cell,squamous and melanotic variants. [2] Classically,insular and trabecular patterns are common. Rosettes, pseudo-rosettes, glandular, tubular and follicular structures are also seen in some cases. [3] A mixed medullary-papillary carcinoma of the thyroid has been described with an immunoreactivity in 20-30% cells with features of PTC for thyroglobulin and calcitonin in the remaining cells with an MTC morphology. Calcitonin immunoreactivity appears to be poor in the small cell variant. [4] Medullary carcinoma has a poorer prognosis as compared with other thyroid neoplasms and may be associated with MEN 2A syndrome, which is characterized by the occurrence of MTC, pheochromocytomas and parathyroid hyperplasia or adenomas. Rone et al. have described a patient with asingle parathyroid adenoma and non-medullary PTC, with unexplained calictonin rise suggesting an occult MTC, leaving open the possibility of an MEN 2 variant with the rare association of PTC. [5]

In view of the considerable morphological overlap and the need for definitive tumor diagnosis with varying treatment options, we conclude that thyroid carcinomas should be classified according to immunoreactivity pattern in a profile comprising of calcitonin, carcinoembryonic antigen, chromogranin, cytokeratin, leukocyte common antigen and thyroglobulin.

   References Top

1.Ozkara SK, Gürbüz Y, Müezzinoðlu B, YumbalZ.Encapsulated cystic papillary variant of medullary carcinoma of thyroid gland. Endocr Pathol 2002;13:167-71.  Back to cited text no. 1
2.Raý¢khlin NT, Smirnova EA, SatylganovI Zh. Histological variants of thyroid medullary carcinoma. Arkh Patol 2001;63:10-4.  Back to cited text no. 2
3.Desai SS, Sarkar S, Borges AM. A study of histopathological features of medullary carcinoma of the thyroid: Cases from a single institute in India. Indian J Cancer 2005;42:25-9.  Back to cited text no. 3
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4.Yerly S, Triponez F, Meyer P, Kumar N, Bongiovanni M. Medullary thyroid carcinoma, small cell variant, as a diagnostic challenge on fine needle aspiration: Acasereport. Acta Cytol 2010;54(Suppl 5):911-7.  Back to cited text no. 4
5.Rone JK, Lane AG, Grinkemeyer MD. Papillary thyroid carcinoma, parathyroid adenoma, and unexplained hypercalcitoninemia: An unusual presentation of multiple endocrine neoplasia type 2A? Thyroid 1998;8:781-5.  Back to cited text no. 5

Correspondence Address:
Nuzhat Husain
Department of Pathology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow - 226 010, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.130933

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