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. 2022 Jul 25;8(1):139.
doi: 10.1186/s40792-022-01496-5.

Primary thyroid gland myxofibrosarcoma: a case report and review of the literature

Maria Chara Stylianidi  1 , Lena Haeberle  2 , Matthias Schott  3 , Yuriko Mori  4 , Christina Antke  4 , Frederick Lars Giesel  4 , Gerald Antoch  5 , Irene Esposito  2 , Wolfram Trudo Knoefel  1 , Andreas Krieg  6
Affiliations

Primary thyroid gland myxofibrosarcoma: a case report and review of the literature

Maria Chara Stylianidi et al. Surg Case Rep. .

Abstract

Background: Myxofibrosarcoma is a common soft tissue sarcoma of the extremities, which occurs very rarely in the thyroid gland.

Case presentation: We report the case of a 61-year-old male who presented with a swelling of the left side of the neck and a newly emerged hoarseness. Ultrasound depicted a hypoechoic thyroid nodule with microcalcifications that was highly suspicious for malignancy. He underwent a left hemithyroidectomy. Histopathological examination and immunohistochemical studies revealed a myxofibrosarcoma of the thyroid gland.

Conclusion: Myxofibrosarcoma of the thyroid gland is extremely rare. The diagnosis is based on histopathological features. Radical surgery achieving tumor-free resection margins remains the only chance for cure. However, the role of radiotherapy and/or chemotherapy is still under debate. Due to their high tendency for locoregional recurrence, a close follow-up after surgery is mandatory.

Keywords: Myxofibrosarcoma; Soft tissue sarcoma; Thyroid gland.

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Conflict of interest statement

The authors have no competing interest.

Figures

Fig. 1
Fig. 1
Preoperative thyroid sonography. Ultrasound revealed a large hypoechoic nodule with microcalcification (arrows) and irregular margins in the left lobe measuring 40 × 39 × 52 mm. a Transverse plane. b Sagittal plane
Fig. 2
Fig. 2
Histomorphology and immunohistochemistry of high-grade MFS of the thyroid gland. a MFS of the thyroid gland presenting as multi-nodular tumor composed of pleomorphic spindle-shaped cells infiltrating the normal thyroid parenchyma with vascular invasion (hematoxylin–eosin staining (HE), ×25). b Higher magnification demonstrates a myxoid stroma arranged along curvilinear blood vessels (HE, ×50). c Immunohistochemical staining showing strong expression of CD99 in myxoid (left) and more densely packed (right) tumor areas, while remnant thyroid follicles (arrows) stain negative for CD99 (×50). d Remnant thyroid follicles (upper right) exhibit a strong nuclear expression of TTF-1, while surrounding tumor cells remain negative (50x)
Fig. 3
Fig. 3
Postoperative FDG-PET/CT scan. Two weeks after surgery FDG-PET/CT scan revealed a 9.6 × 7.1 × 9 cm left cervical tumor mass expanding from the esophagus to the carotid sheath that infiltrates the trachea, the front-edge of the lower cervical vertebrae and the higher thoracic vertebrae. a Maximum intensity projections of 18F-FDG-PET. b Direct comparison of contrast-enhanced CT, c fusion imaging and d FDG-PET
Fig. 4
Fig. 4
Tumor progression under radiotherapy. Tumor mass in the ventral upper thorax infiltrating the larynx, esophagus and the left common carotid artery
See this image and copyright information in PMC

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