Anaplastic spindle and giant cell carcinoma of the thyroid with rhabdoid inclusions

Definition

The is a high grade malignancy arising by dedifferentiation of a pre-existing papillary or follicular carcinoma. The dedifferentiation may occur within the primary tumour of may be found only in a metastasis4, sometimes years after the primary thyroid carcinoma has been treated.

Histopathology

The anaplastic component usually dominates, but sometimes may be a minor component of the tumours. The anaplastic tumour itself comprises a variable proportion of spindle, polyhedral and multinucleate giant cells. There may be foci of squamous differentiation, of osteoclast-like giant cells or corresponding mononuclear cells, foci resembling angiosarcoma or foci of osteoid.

The mononuclear polyhedral cells may contain rhabdoid eccentric hyaline inclusions, displacing the cell nuclei. [The inclusions are similar to those that have been reported in poorly differentiated follicular carcinoma of the thyroid2.]

The differentiated component may be papillary carcinoma (conventional, tall cell or follicular variants), follicular carcinoma or oncocytic carcinoma. Some cases arise against a background of adenomatous goitre or in the absence of thyroid pathology

Immunohistochemistry

 

Vimentin

11/111, 1/13

 

Cytokeratin

7/111, 1/13

Desmin

0/111, 0/13

Thyroglobulin

0/111, 0/13

TTF-1

0/13

Calcitonin

0/111

Myoglobin

1/13

Sarcomeric actin

1/13

SMA

0/13

S-100

0/13

   

Ultrastructure

The rhabdoid inclusions consist of paranuclear whorls of intermediate filaments. [They differ from thyroglobulin inclusion which are filled with granular electron-dense material.]

Molecular genetics

In come cases, RET/PTC rearrangements argue for origin from a papillary carcinoma3.

This is a high grade malignancy with a high mortality.

References

1 Albores-Saavedra J, Hernandez M, Sanchez-Sosa S, et al. Histologic variants of papillary and follicular carcinomas associated with anaplastic spindle and giant cell carcinomas of the thyroid: an analysis of rhabdoid and thyroglobulin inclusions. Am J Surg Pathol 2007; 31:729-36

2 Albores-Saavedra J,Sharma S. Poorly differentiated follicular thyroid carcinoma with rhabdoid phenotype: a clinicopathologic, immunohistochemical and electron microscopic study of two cases. Mod Pathol 2001; 14:98-104

3 Maria Letizia Lai, Gavino Faa, Stefano Serra, Giancarlo Senes, Giovanni M. Daniele, Francesco Boi, Stefano Mariotti, Michel Beauchemin, Sylvia L. Asa. Rhabdoid Tumor of the Thyroid Gland: A Variant of Anaplastic Carcinoma. Archives of Pathology and Laboratory Medicine: Vol. 129, No. 3, pp. e55–e57.

4 Sato K, Waseda R, Tatsuzawa Y, et al. Papillary thyroid carcinoma with anaplastic transformation showing a rhabdoid phenotype solely in the cervical lymph node metastasis. Pathol Res Pract 2006; 202:55-9

This page last revised 3.6.2007.

©SMUHT/PW Bishop