A 71-year-old man was referred to our department complaining of dyspnea, hypoventilation and dysphonia, determined by swelling in the neck region and related to a considerable increase in size of the thyroid gland.
On clinical examination, the thyroid gland appeared firm during the acts of deglutition. The patient reported that he had a goiter for more than 20 years and he had never undergone any drug therapy.
An ultrasound examination dating back to 1997 documented a complete subversion of the echotexture of the whole gland and the presence of a large nodule in the right lobe displaying a complex echotexture. A further ecography in 2000 documented an increase in volume of the thyroid, which was also the cause of the right carotid bulb dislocation.
Laboratory investigations revealed significantly elevated thyroglobulin values. A preoperative fine needle aspiration was not significant; it consisted of an acellular smear within a background of inflammatory and necrotic cells without any cytologic specification.
Consequently, we decided not to repeat this procedure because of the worsening of the patient’s dyspnea, for which a surgical management for liberation of the airways was planned.
A total thyroidectomy was performed. Although the surgical operation was expected to be very complex, it was performed in a completely linear way. The left lobe was easily separable from the surrounding tissue; it appeared to be in the throes of a nodular transformation. The right lobe appeared uniformly in a nodular transformation and it penetrated the upper part of the neck, adhering to the vessels, from which, however, it was easily dissociated. Lymph nodes were not visible. However, surgical times were also lower than expected, particularly for the removal of the right half, which was relatively easy, and the mass appeared well encapsulated and demarcated (Figure ).
A macroscopic photograph of thyroid angiosarcoma showing a well capsulated nodule that appeared cystic and hemorrhagic on the cut surface.
The specimen was sent to surgical pathology for definitive diagnosis. The tissue samples were fixed in formalin, then routinely processed and embedded in paraffin. The sections were stained with hematoxylin and eosin. Additional 5-μm sections were cut and subjected to immunohistochemical studies using antibodies to the following antigens: CD34, CD31, factor VIII-related antigen, pan-cytokeratin (Pan-CK) and thyroglobulin. Grossly, the right thyroid lobe measured 10 × 8.5 × 6 cm, the left lobe was 5 × 4 × 1.5 cm and the pyramidal lobe was 4 × 2 × 1 cm in size. The right thyroid lobe was totally occupied by a well circumscribed nodule macroscopically confined within the capsule. The nodule measured 9 × 5 cm and, on the cut sections, appeared cystic and hemorrhagic, with large necrotic tissue areas (Figure ). The tumor was extensively sampled. On histological examination, the periphery of the lesion showed epithelioid areas that were made up of large rounded cells of relatively high nuclear grade, with eosinophilic cytoplasm and prominent nucleoli arranged in rudimentary vascular channels. These neoplastic channels were irregular in shape, and they were lined by a single layer of malignant endothelium forming intraluminal papillary projections (Figure ). An extensive central area of necrosis and hemorrhages was a characteristic feature of the lesion. The capsular surface was not involved with the tumor and a rim of residual thyroid tissue was observed in some peripheral areas. Immunohistochemically, neoplastic cells were strongly positive for CD31, CD34, and factor VIII-related antigen, showing evidence of their endothelial differentiation (Figure ). Diagnosis of this condition can be difficult as the histological features may mimic other malignant vascular lesions. In view of the clinical history and morphological and immunohistochemical findings, a diagnosis of primary angiosarcoma of the thyroid gland was made.
Hematoxylin and eosin staining (10 ×) shows a peripheral rim of normal thyroid tissue and a central area of necrosis and hemorrhages.
Hematoxylin and eosin staining (40 ×) showing numerous vascular channels lined by neoplastic endothelium.
Vascular neoplastic channels with strong, diffuse CD 34 immunoreactivity.
As regards adjuvant therapy, our patient has not been subjected to any kind of special treatment because, even today, there are no guidelines and there are various schools of thought as to the best treatment options.
The prognosis was not favorable. The postoperative course was not simple: it was decided to admit the patient to our intensive coronary unit for precautionary reasons. After 3 days, due to an atelectasis of the left lung, intubation and subsequently tracheostomy were needed. Breathing difficulties continued for a long time (about 60 days after surgery). Our patient was monitored and follow-up was started but it was too short to be meaningful because unfortunately, our patient died about 3 months later as a result of of cardiac and respiratory complications related to his oncologic condition.