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Amphicrine Medullary Thyroid Carcinoma — a Case-Based Review Expanding on Its MUC Expression Profile

Endocrine Pathology volume 33, pages 378–387 (2022)Cite this article

Abstract

Amphicrine phenotype in medullary thyroid carcinoma (MTC) is a rare phenomenon characterized by tumor cells that show both endocrine differentiation (calcitonin secretion) and exocrine differentiation (mucin production and secretion). Not much is known about the pathobiology of amphicrine MTCs. This report undertook a case-based review approach by discussing the cytological, histopathological, and ultrastructural features of this rare enigmatic entity, expanding on the radiological and novel MUC immunohistochemistry findings from a 28-year-old MEN2B syndrome patient with C cell hyperplasia and multifocal MTC with amphicrine features. The patient had widespread hematogenous metastases at presentation. MUC immunoexpression analysis revealed evidence of micro-lumina formation, and unique to-date unreported expression patterns of MUC1, MUC5AC, and MUC6 in an amphicrine subtype of MTC. Review of the literature identified five other MTC cases with well-documented amphicrine features. Of these six cases, two were associated with MEN2B syndrome, and four had metastatic disease. Follow-up was available in three patients, and two died of disease. Recognition of this rare subtype of MTC may be of clinical interest given their frequent link to MEN2B syndrome and biological aggressiveness.

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Authors and Affiliations

  1. Department of Pathology, First Floor, Teaching Block, All India Institute of Medical Sciences, New Delhi, 110029, India

    Hena Khandakar, Shipra Agarwal & Mehar Chand Sharma

  2. Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India

    Devasenathipathy Kandasamy

  3. Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India

    Chandrasekhar Bal

  4. Department of Surgical Disciplines, All India Institute of Medical Sciences, New Delhi, India

    Yashvant Rathode & Rijuta Aphale

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  1. Hena Khandakar
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Contributions

HK and SA wrote the main manuscript text, and prepared Figs. 2, 3, and 4; DK, CB, YR, and RA prepared the images in Fig. 1, while MCS prepared the images in Fig. 3C–E. All the authors reviewed the manuscript.

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Correspondence to Shipra Agarwal.

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Ethical approval was waived by the institutional review board in view of the retrospective nature of the study and all the procedures being performed were part of the routine care.

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Khandakar, H., Agarwal, S., Sharma, M.C. et al. Amphicrine Medullary Thyroid Carcinoma — a Case-Based Review Expanding on Its MUC Expression Profile. Endocr Pathol 33, 378–387 (2022). https://doi.org/10.1007/s12022-022-09725-1

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Keywords

  • Amphicrine carcinoma
  • Amphicrine subtype medullary thyroid carcinoma
  • Medullary thyroid carcinoma
  • MiNEN
  • MEN2B syndrome
  • RET
  • MUC1
  • MUC5AC
  • MUC6
  • Radiology