Ann Hepatobiliary Pancreat Surg.  2020 May;24(2):182-187. 10.14701/ahbps.2020.24.2.182.

Pathologic Doppelgänger: Thyroid–like intrahepatic cholangiocarcinoma with synchronous primary thyroid carcinoma in a young woman

• Mittal N
• Bal M
• Ramadwar M
• Patkar S
• Goel M

• KMID: 2500803
• DOI: http://doi.org/10.14701/ahbps.2020.24.2.182

Abstract

Intrahepatic cholangiocarcinoma (ICC) accounts for 8-10% of all malignant liver tumors. Preponderance for elderly males and occurrence of varied morphological patterns in ICC is well known. Recent reports have described a newly recognized variant of thyroid-like cholangiocarcinoma. Herein, we present a hitherto unreported synchronous occurrence of an intrahepatic thyroid-like cholangiocarcinoma and a separate thyroid carcinoma in a 23-year-old post-partum woman. Both tumors displayed striking resemblance to follicular variant of papillary thyroid carcinoma (FVPTC) however exhibited disparate immunohistochemical profiles: the intrahepatic tumor was positive for CK7 and CK19, and negative for TTF-1, PAX-8 and thyroglobulin whereas, the thyroid tumor was positive for TTF-1, thyroglobulin and PAX-8. Young age, female proclivity, large mass at presentation and unique histology in thyroid-like ICC hint towards a distinctive subset of ICC. Awareness and recognition of this rare entity is essential, not only for accurate diagnosis, but also for gathering information on its biology and clinical behavior. Synchronous occurrence with a FVPTC is a challenging scenario that can simulate metastatic disease and mislead subsequent patient management. Whether morphologic similarity points to an underlying linkage between the two different tumors needs exploration.

Keyword

Liver; Thyroid-like cholangiocarcinoma; Synchronous; Follicular variant of papillary thyroid carcinoma; Clinicopathologic features

Figure

• 

  Fig. 1 Thyroid-like cholangiocarcinoma (A-C, upper panel): (A) Tumor displays a conspicuous macrofollicular pattern at low magnification. (B) Eosinophilic colloid-like secretions in the neoplastic follicles. (C) Microfollicular architecture with prominence of “Orphan Annie eye” nuclei. Follicular variant of papillary thyroid carcinoma (D-F, lower panel): (D) Tumor nodule separated from surrounding thyroid follicles by a thin capsule. (E) Macrofollicular pattern of the thyroid tumor with eosinophilic secretions in the lumen, similar to areas seen in the liver tumor, (F) Subtle nuclear grooving and focal clearing in the thyroid tumor, characteristic of Follicular variant of papillary thyroid carcinoma.

• 

  Fig. 2 Immunohistochemical profile of thyroid-like cholangiocarcinoma (A-D, upper panel) vis a vis papillary thyroid microcarcinoma (E-H, lower panel). (A) Hematoxylin & Eosin stained section of liver tumour showing microfollicular pattern. On immunohistochemistry, tumor showed strong positivity for CK7 (B) and focal for CK19 (not shown here). Intrahepatic cholangiocarcinoma is negative for thyroid specific markers, i.e. TTF-1 (C), and Thyroglobulin (D). Immunohistochemical profile of thyroid carcinoma (E-H, lower panel). (E) Thyroid tumor is encapsulated and has a microfollicular pattern. The tumor is diffusely positive for thyroid related markers, namely PAX8 (F), TTF-1 (G), and thyroglobulin (H).

Reference

1. Hapke MR, Dehner LP. 1979; The optically clear nucleus. A reliable sign of papillary carcinoma of the thyroid? Am J Surg Pathol. 3:31–38. DOI: 10.1097/00000478-197902000-00004. PMID: 534382.

2. DeLellis RA. 1993; Orphan Annie eye nuclei: a historical note. Am J Surg Pathol. 17:1067–1068. DOI: 10.1097/00000478-199310000-00014. PMID: 8372945.

3. Michal M, Kacerovska D, Kazakov DV. 2013; Cribriform adenocarcinoma of the tongue and minor salivary glands: a review. Head Neck Pathol. 7 Suppl 1(Suppl 1):S3–S11. DOI: 10.1007/s12105-013-0457-9. PMID: 23821209. PMCID: PMC3712093.

4. Amin MB, Gupta R, Ondrej H, McKenney JK, Michal M, Young AN, et al. 2009; Primary thyroid-like follicular carcinoma of the kidney: report of 6 cases of a histologically distinctive adult renal epithelial neoplasm. Am J Surg Pathol. 33:393–400. DOI: 10.1097/PAS.0b013e31818cb8f5. PMID: 19047894.

5. Chang SY, Fleiszer DM, Mesurolle B, El Khoury M, Omeroglu A. 2009; Breast tumor resembling the tall cell variant of papillary thyroid carcinoma. Breast J. 15:531–535. DOI: 10.1111/j.1524-4741.2009.00773.x. PMID: 19594763.

6. Fornelli A, Bondi A, Jovine E, Eusebi V. 2010; Intrahepatic cholangiocarcinoma resembling a thyroid follicular neoplasm. Virchows Arch. 456:339–342. DOI: 10.1007/s00428-009-0874-z. PMID: 20082203.

7. Chablé-Montero F, Shah A, Angeles- Ángeles A, Henson DE, Albores-Saavedra J. , Montante-Montes de Oca D. 2012; Thyroid-like cholangiocarcinoma of the liver: an unusual morphologic variant with follicular, trabecular and insular patterns. Ann Hepatol. 11:961–965. DOI: 10.1016/S1665-2681(19)31427-9. PMID: 23109464.

8. Foucar E, Kaplan LR, Gold JH, Kiang DT, Sibley RK, Bosl G. 1979; Well-differentiated peripheral cholangiocarcinoma with an unusual clinical course. Gastroenterology. 77:347–353. DOI: 10.1016/0016-5085(79)90291-9. PMID: 221305.

9. Barrera JR, Manalo LA, Ang FL. 2012; Papillary thyroid-type carcinoma arising from struma ovarii. BMJ Case Rep. 2012:bcr0320126145. DOI: 10.1136/bcr.03.2012.6145. PMID: 22787184. PMCID: PMC3417009.

10. Shaib Y, El-Serag HB. 2004; The epidemiology of cholangiocarcinoma. Semin Liver Dis. 24:115–125. DOI: 10.1055/s-2004-828889. PMID: 15192785.

11. Saha SK, Zhu AX, Fuchs CS, Brooks GA. 2016; Forty‐year trends in cholangiocarcinoma incidence in the U.S.: intrahepatic disease on the rise. Oncologist. 21:594–599. DOI: 10.1634/theoncologist.2015-0446. PMID: 27000463. PMCID: PMC4861366.

12. Roti E, degli Uberti EC, Bondanelli M, Braverman LE. 2008; Thyroid papillary microcarcinoma: a descriptive and meta-analysis study. Eur J Endocrinol. 159:659–673. DOI: 10.1530/EJE-07-0896. PMID: 18713843.