A 52-year-old woman was referred to our hospital because of severe dyspnea and the presence of a neck tumor. She had experienced dyspnea for the past 6 months and had recently noticed bloody sputum. A computed tomography examination of the neck revealed a thyroid tumor about 8 cm in size. The tumor had invaded the trachea, obstructing 90% of the tracheal lumen (fig. ). An emergency tracheotomy was performed to maintain an airway through the tumor tissue. On biopsy, the tumor was diagnosed as CASTLE, suggesting a favorable prognosis if a complete resection could be performed. Magnetic resonance imaging and an esophageal fibroscopy examination revealed a low possibility of esophageal invasion (fig. ). Under general anesthesia, a thyroidectomy was initially performed via a neck incision. The invaded portion of the trachea was sharply dissected. A partial sternotomy was performed at the level of the 2nd intercostal space to identify the distal portion of the intact trachea and to mobilize the inferior tracheal segment. The trachea was transversely opened to find the intact portion. The length of the resected trachea was 6 cm (fig. ). Bilateral recurrent nerves were macroscopically preserved. We performed a suprahyoid release to mobilize the superior tracheal segment, but these mobilizations were insufficient to perform an anastomosis. Thus, to allow additional mobilization, we performed a clamshell thoracotomy and incised the pericardium 360° around the hilum, dissecting the pulmonary ligament bilaterally. These procedures allowed an anastomosis. An end-to-end bronchial anastomosis was carried out using running 4-0 PDS II sutures (Ethicon, Somerville, N.J., USA) for the membranous portion and interrupted 4-0 PDS II sutures for the cartilaginous portion. The bronchial anastomosis was wrapped with an omental pedicle flap that was lifted up through a retrosternal route via a laparotomy. A gastrostomy was also performed for postoperative enteral nutrition. For management after surgery, a chin suture was placed. Neck mobilization was restricted for the placement of the chin suture and airway fixation. After confirming an improvement in the laryngeal edema using a laryngoscopy, extubation was performed on postoperative day (POD) 17. The patient exhibited swallowing disturbances and underwent swallowing rehabilitation. Enteral nutrition was provided through the gastrostomy. The patient was discharged on POD 31, and per os feeding was started on POD 51. Radiotherapy (60 Gy in 40 daily fractions of 1.5 Gy) was performed as an adjuvant therapy. Throughout the clinical course, no major complications, including swallowing disturbances, hoarseness or anastomotic problems, were observed. A histopathological examination of the resected specimen revealed the characteristic morphology of CASTLE (fig. ); immunohistochemistry was positive for CD5 (fig. ) but negative for thyroid transcription factor 1 (TTF-1; fig. ).\nEleven months after surgery, lower back pain and right leg numbness developed and led to gait inability. Multiple lung and bone recurrences were observed, but no local recurrence. In order to ease the back pain, palliative radiotherapy (35 Gy in 14 fractions) to the bone metastasis was administered. Twelve months after surgery, drainage of pleural effusion was performed. She died of pleural metastasis 14 months after the initial diagnosis of CASTLE.
A 76-year-old man was referred to our hospital for investigation of a 20-day history of rapidly deteriorating dyspnea and severe cough. This was associated with dysphagia and cervical pain. The patient was an ex-marble craftsman and an active smoker. His medical history included chronic pulmonary obstructive disease (COPD). A chest X-ray (CXR) showed an imprint at the right side of the trachea. Thyroid ultrasound (US) revealed a large and heterogenous thyroid gland and the presence of a calcified mass, 4.76 × 4.01 cm with high blood perfusion in the right upper lobe of the gland. A contrast-enhanced computed tomography (CT) scan of the neck and thorax reported the corresponding lesion as heterogenous, with irregular borders, extending from the sternoclavicular joints to the upper borders of the thyroid gland. The mass was surrounding the trachea anterolaterally displacing it to the left and the right common artery to the right (). There were no enlarged lymph nodes. Bronchoscopy revealed a fracture of the first tracheal ring but there was no evidence of tumour invasion (). Cytology from bronchial lavage was negative for cancer cells. Laboratory tests were within normal limits with the exception of elevated D-dimers (2,994 μg/L) and elevated white blood cells (19.27 K/μL). Tumour markers were within normal range values.\nOn admission, the patient was intubated, due to acute respiratory failure, and he was admitted to the intensive care unit (ICU). Subsequently, he underwent an emergency procedure and a collar incision was carried out. At surgery, a suspicious looking tumour, which was in continuity with the superior pole of the right thyroid lobe, the sheath of right common carotid artery, and the anterolateral portion of the cartilaginous trachea, was identified. Macroscopically, the tumour looked whitish and scleroelastic with moderately regular borders and was firmly adhered to the aforementioned structures. After the right recurrent laryngeal nerve was identified, the tumour was carefully dissected off the right thyroid lobe and the adventitia of the right common carotid artery. A similar approach was carried out to dissect the tumour off the left lobe; hence, an en bloc tumour resection with total thyroidectomy and a regional lymph node dissection was performed. Rapid frozen section biopsy of the tumour in the right thyroid lobe proved to be malignant with features of squamous cell carcinoma and negative surgical margins.\nPostoperatively, the patient experienced swallowing difficulty and hoarseness and a tracheostomy was performed on day 2.\nFinal pathology examination confirmed frozen section's diagnosis of a squamous cell carcinoma invading the right lobe of the thyroid gland, as well as extracapsular structures of the left lobe. Microscopically, the tumour was described as nonencapsulated, irregularly lobulated, with broad fibrous septa, and lymphoplasmacytic, eosinophilic, histiocytic, and multinuclear infiltration. The neoplastic cells were largely polyhedral or oval with eosinophilic or amphophilic cytoplasm, arranged in islands or nests that showed focal keratinization. The nuclei were vesicular or hyperchromatic with nucleoli. A panel of immunohistochemical stains was performed; the tumour was positive for p63, CD117, CKH, CD5, and CD3 in the lymphocytic part (Figures , , and ). It was also negative for the expression of TTF1.\nThe patient subsequently improved and was discharged home on postoperative day 20. The patient did not receive any additional treatment.