Non-Alpine thyroid angiosarcoma

Abstract

INTRODUCTION

Angiosarcoma is a very rare entity of soft tissue neoplasm with an aggressive and destructive biological behavior. Thyroid angiosarcoma is usually reported in Alpine regions, with only exceptionally rare cases arising in non-mountainous areas. In the Alpine regions it constitutes 2–10% of all malignant thyroid tumors.

PRESENTATION OF CASE

We report a case of a thyroid non-Alpine angiosarcoma in a 71-year-old female with a 10 years old multinodular goiter. The cervical mass underwent rapid growth in the last year, and she was referred for surgical treatment. A 15 cm mass was found on the right side of the neck invading adjacent tissues and displacing the trachea without obvious invasion of this organ. Fine needle aspiration cytology showed “carcinoma”. Lung metastasis were present. Although difficult, total thyroidectomy was possible with resection of an esophageal implant. Post-operatively, she had respiratory failure that eventually recovered, but, on 39th post-operative day, she died of violent hemoptysis, probably due to invasion by mediastinal metastasis.

DISCUSSION

A clear distinction between angiosarcoma and anaplastic carcinoma of the thyroid is considerably difficult, despite treatments and prognosis are practically the same. However, in recent years, a thyroid malignancy exhibiting phenotypical features of endothelial differentiation was described. Keratin positivity cannot be reported as necessarily indicative of epithelial differentiation.

CONCLUSION

Optimal treatment for thyroid angiosarcoma remains unclear, not only because the prognosis is poor, despite multimodal therapeutic efforts, but also because it is a very rare entity.

1. Introduction

Angiosarcomas are a very rare entity of soft tissue neoplasms with an aggressive and a destructive biological behavior.¹ Thyroid angiosarcoma has usually been reported in Alpine regions, with only exceptionally rare cases arising in non-mountainous areas.¹ In the Alpine regions it constitutes 2–10% of all malignant thyroid tumors.² The incidence of this rare entity may be related to endemic goiter areas and is probably due to thrombotic events and hyperplastic endotelial proliferations of thyroid nodules after recurrent intranodular hemorrhage.^3,4 This occurrences could be considered as a pre-condition for subsequent neoplastic transformation.^4,5

It has been a controversial entity, and it is histologically defined as freely anastomosing channels lined by atypical endothelial cells.⁶ Nevertheless, a clear distinction between angiosarcoma and anaplastic carcinoma of the thyroid is considerably difficult, since they yield similar clinical prognosis and histopathological findings.⁶

We report a clinical case of thyroid angiosarcoma with a discussion on pathological findings, and appropriate surgical and medical treatment.

2. Presentation of case

A 71-year-old female in Oporto, Portugal, presented with a mass in the antero-lateral cervical region of the neck (Fig. 1) According to the patient, she had had a neck mass for 10 years, with a rapid growing over the last year. One month prior to admission, she suffered severe dyspnea, forcing her lo look for medical care.

Fig. 1.

A 71-year-old female presented with a mass in the antero-lateral cervical region of the neck.

Physical examination revealed a 13 cm firm, fixed, slightly tender, poorly demarcated, and warm-to-touch right antero-lateral neck mass. There was no thyroid bruit and no contra-lateral cervical, supra-clavicular or retro-auricular lymph nodes. The thyroid function tests revealed euthyroid status. Fine needle aspiration biopsy of the cervical mass was performed with a 22-gauge needle, whose result was compatible with carcinoma. A cervical and chest CT was also performed and revealed a cervical encapsulated mass (75 × 75 mm) with liquid and dystrophic calcifications displacing adjacent structures (aerodigestive), but without apparent invasion (Fig. 2). The chest CT revealed a “crazy-paving” appearance without enlargement of mediastinic nodes or pleural effusion compatible with lung metastasis.

Fig. 2.

Cervical CT showing a cervical encapsulated mass (75 × 75 mm) displacing adjacent structures (aerodigestive), but without apparent invasion.

Although difficult, total thyroidectomy was possible with resection of an esophageal implant that did not interested the esophageal mucosa. It was also resected a small fragment from right jugular vein that was sorted out with direct suture over the vein (Fig. 3).

Fig. 3.

Total thyroidectomy specimen with an esophageal implant.

Post-operatively, she had respiratory failure and eventually recovered. But on 39th post-operative day, the patient died of violent hemoptysis, probably due to invasion by mediastinal metastasis.

2.1. Pathologic findings

2.1.1. Gross features

Total thyroidectomy specimen, weighed 544 g, with a 10 × 10 × 10 cm large, encapsulated, hemorrhagic, necrotic, and partially cystic tumor with areas of calcifications occupying the right lobe of thyroid.

The left lobe and isthmus measured 12.0 × 9.0 × 4.0 cm, with a colloid and nodular aspect.

Tumoral implants from esophagus and right jugular vein were excised.

2.1.2. Microscopic features

The right lobe was totally substituted by a large tumor, composed of freely anastomosing channels lined by atypical endothelial cells, associated with a papillary configuration and a solid pattern of growth (Fig. 4). The tumor cells vary from spindle to epithelioid. The nuclei of endothelial cells were large, vesicular with prominent and irregularly shaped nucleolus. Mitoses were found. A constant feature was the presence of extensive fresh and old hemorrhage and necrosis. There were images of lymphatic and vascular invasion.

Fig. 4.

Anastomosing channels lined by atypical spindle to epithelioid cells with vesicular nuclei (40×).

The tumor cells were immune-reactive for vimentin (Fig. 5), AE1/AE3 keratin (focal) (Fig. 6), CD31 and CD34 (Fig. 7).

Fig. 5.

Tumor cells are strongly immunoreactive for vimentin (20×).

Fig. 6.

Many of tumor cells are immunoreactive for keratin (20×).

Fig. 7.

Tumor cells are immunoreactive for CD34 (40×).

The lesion excised from the esophagus and jugular vein revealed angiosarcoma metastasis.

3. Discussion

We present a case of an aggressive rapid growing thyroid angiosarcoma with invasion of the right jugular vein and with esophagus and pulmonary metastasis. The medical history is classically described as a longstanding goiter that somehow, in a period of time, begins to grow as a neck mass with symptoms of local compression.⁷ These malignancies typically occur in patients over 60 years old.⁷ Rapid primary tumor growth and the widespread of distant metastasis are common causes of death in this patients.⁸ Early tumor metastasis to regional lymph nodes and lungs are frequent, with bone marrow metastasis occurring in later stages.⁹ However, there are reported cases with longer survivals mainly due to the absence of extraglandular tumor spread at time of surgery.⁸

Nevertheless, a main question remains over decades: are they true endothelial tumors or variants of carcinoma? Most pathologists have been skeptical about the existence of this entity, assuming that most cases were undifferentiated carcinoma with an angiomatoid appearance. However, in recent years, a thyroid malignancy exhibiting phenotypical features of endothelial differentiation was described.¹⁰ Keratin positivity cannot be reported as necessarily indicative of epithelial differentiation.

The distinction may be academic, because treatments and prognosis are practically the same.^5–7

4. Conclusion

Optimal treatment for thyroid angiosarcoma remains unclear, not only because the prognosis is poor, despite multimodal therapeutic efforts, but also because it is a very rare entity.^11,12

Conflicts of interest

The authors report that there are no conflicts of interest.

Funding

None.

Ethical approval statement

Written informed consent was obtained from the patient's family for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Author contributions

Pedro Gouveia played a major role in the treatment of the patient described, in addition to collecting and analyzing the data, and writing the manuscript. Catarina Silva, Fátima Magalhães, Claúdia Santos, Emanuel Guerreiro, Filipe Santos and Teixeira Gomes were participated in the treatment of the patients described, as well as in the data collection, and in the editing of the final manuscript.