Carcinoma showing thymus-like differentiation (CASTLE) with neuroendocrine differentiation.

Pathology Division, Research Center for Innovative Oncology, National Cancer Center Hospital East, Chiba, Japan.
Pathology International (Impact Factor: 1.59). 01/2009; 58(12):775-9. DOI: 10.1111/j.1440-1827.2008.02310.x
Source: PubMed

ABSTRACT Carcinoma showing thymus-like differentiation (CASTLE) is a rare malignant neoplasm that histologically resembles thymic carcinoma and arises in the thyroid gland or adjacent soft tissue of the neck. Herein is reported the case of a 62-year-old male patient with CASTLE exhibiting neuroendocrine differentiation, who was treated with total pharyngolaryngo-esophagectomy and total thyroidectomy. Gross examination of the surgical specimen showed a grayish-white, solid, lobulated tumor, mainly located between the trachea and esophagus, and involving the lower part of the left thyroid lobe. Histologically, the tumor consisted of epithelial cell nests separated by thick fibrous septa. The tumor cells were polygonal in shape, and contained pale cytoplasm and a vesicular nucleus with prominent nucleoli. There were few mitotic figures. Rosette-like arrangements that suggested neuroendocrine differentiation were observed in part of the tumor. The tumor cells were positive for CD5 and neuroendocrine markers including synaptophysin and chromogranin A.

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    ABSTRACT: A 55-year-old woman underwent a total thyroidectomy for carcinoma showing thymus-like differentiation (CASTLE). The patient was referred to our hospital after the tumor was found to have directly invaded the cervical esophagus and the entire circumference of the trachea. A total thyroidectomy was performed, followed by end-to-end anastomosis of the trachea, suprahyoid release and dissection of bilateral pulmonary ligaments. No major complications, including anastomotic dehiscence or stenosis, were observed. The patient experienced some swallowing disturbances and hoarseness during the perioperative period but fully recovered. Radiotherapy to the neck was performed as an adjuvant therapy. Eleven 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. Palliative radiotherapy to the bone metastasis was performed. The patient died of pleural metastasis 14 months after the initial diagnosis of CASTLE.
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    ABSTRACT: Ectopic thymic tissue can be present in the thyroid gland and a carcinoma showing thymus-like differentiation (CASTLE) may arise from such tissue. We are reported the case of a 26-year-old man with CASTLE, with cervical subcutaneous nodules relapse, who showed a good response to treatment with surgery, chemotherapy and radiotherapy. The problematic aspect of this case was the diagnosis; only on review were we able to make a final diagnosis. CASTLE is a very rare neoplasm. It is important to differentiate this cancer from others tumors such as primary or metastatic squamous cell carcinoma of the head and neck or squamous cell thyroid carcinoma, because the therapy and prognosis are different. Diagnosis is complicated and requires careful histological analysis (CD5- and P63-positive with presence of Hassall's corpuscles); unfortunately there is no gold standard treatment so, in this case, we administered a sandwich of chemotherapy and radiotherapy.
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    ABSTRACT: To investigate the clinicopathologic, immunophenotypic, ultrastructural, and molecular features of thyroid carcinoma showing thymus-like elements (CASTLE). We retrospectively analyzed the clinicopathologic data of 10 patients with CASTLE and described the immunophenotypic and ultrastructural features of these tumors. The expression of Epstein-Barr virus-encoded RNA and the gene status of EGFR, C-KIT, and HER-2 were also assessed by molecular techniques. The tumor cells were positive for CD5, CD117, p63, HMWK, EGFR, GLUT-1, Pax8, E-cadherin, bcl-2, and p53 in all cases and for CA-IX, CEA, p16, HER-2, and neuroendocrine markers in some cases. Ultrastructural examination indicated that the tumor cells contained large quantities of tonofilament with abundant intercellular desmosomes, including intracytoplasmic neuroendocrine granules in one case. EGFR gene amplification in two patients and polyploidy of chromosome 7 in one patient were identified by fluorescence in situ hybridization. Sequencing analysis revealed that a synonymous mutation, Q787Q 2363 (G→A), occurred on exon 20 of the EGFR gene in three patients. GLUT-1 can be used as a novel biomarker for CASTLE, and combined detection of GLUT-1 with CD5 and CD117 aids in the diagnosis of this tumor. Aberrant expression of Bcl-2, p53, p16, E-cadherin, EGFR, C-KIT, and HER-2 may play important roles in the development of CASTLE. Copyright© by the American Society for Clinical Pathology.
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