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Ectopic Thymic Carcinoma in the Neck

2010; Elsevier BV; Volume: 90; Issue: 2 Linguagem: Inglês

10.1016/j.athoracsur.2009.12.078

1552-6259

Wen‐Teng Yao, Chih‐Hao Chen, Jie-Jen Lee, Be‐Fong Chen, Tsang‐Pai Liu,

Vascular Malformations and Hemangiomas

Thymic carcinoma is a rare tumor arising from the thymus, which is most commonly located in the anterior mediastinum. We report a 24-year-old woman who presented with a neck tumor. The patient underwent complete resection and the pathology sections showed thymic carcinoma. The patient received adjuvant chemotherapy and radiotherapy. After a 6-month follow-up, the patient is well without any evidence of recurrence. Thymic carcinoma is a rare tumor arising from the thymus, which is most commonly located in the anterior mediastinum. We report a 24-year-old woman who presented with a neck tumor. The patient underwent complete resection and the pathology sections showed thymic carcinoma. The patient received adjuvant chemotherapy and radiotherapy. After a 6-month follow-up, the patient is well without any evidence of recurrence. Thymic carcinoma is an uncommon tumor arising from the thymus, which may be accompanied by a cough, chest pain, phrenic nerve palsy, or superior vena cava syndrome. This tumor is most commonly located in the anterior mediastinum.A 24-year-old woman presented with a neck mass of gradual enlargement accompanied by difficulty swallowing and neck soreness for 1 month. There was chest pain and hoarseness, but no cough. Because the tumor had enlarged with time, the patient sought medical attention at our hospital. A physical examination showed a cervical firm and fixed mass, nontender, and well-demarcated with extension to the left clavicle. Laboratory data included thyroid-stimulating hormone, thyroglobulin, and T4 concentration that were all within normal range. A chest roentgenogram showed mild deviation of the trachea in the cervical region. A contrast-enhanced computed tomographic scan of the neck revealed a tumor approximately 5 cm in diameter that was located near the left thyroid gland. The mass showed heterogeneous contrast enhancement with central low density (Figs 1A, B). A thyroid tumor was suspected and a fine-needle aspiration report indicated anaplastic carcinoma. A low collar incision was made (approximately 8 cm in length). The subcutaneous tissue, fascia, and muscle were carefully separated. The tumor was lying near the carotid sheath and next to the infrahyoid muscles. The trachea was found to be deviated to the right side due to compression. Although with some adhesions, there was no evidence of direct invasion to the major vessels, bone, trachea, esophagus, thyroid gland, and thymus. The patient underwent a complete resection.The tumor measured 5.5 × 5 × 2.3 cm and had a nodular outer surface. Histology revealed that the tumor grew in irregular nests with focal squamoid differentiation (Figs 2A, B), and the thymus tissue was found near the tumor (Fig 3A). The tumor cells showed immunoreactivity for CD5, CD117, P63, synaptophysin, and chromogranins (Fig 3B). The thyroglobulin and thyroid transcription factor-1 could not be stained.Fig 2(A) Histologically, the tumor grows in irregular nests (Hematoxylin and eosin, ×40) with (B) focal squamoid differentiation. (Hematoxylin and eosin, ×200.)View Large Image Figure ViewerDownload (PPT)Fig 3(A) Thymus tissue can be seen near the tumor. (Hematoxylin and eosin, ×100.) (B) The tumor cells showed immunoreactivity for CD5 immunohistochemical stain. (Immunoperoxidase, ×100.)View Large Image Figure ViewerDownload (PPT)A postoperative chemotherapy regiment of cisplatin with gemcitabine was administered to the patient. She received a total of three courses of concurrent chemoradiotherapy. The total radiotherapy doses were 5,580 cGy. No recurrence was found after 6 months. A postoperative contrast-enhanced computed tomographic scan showed no evidence of malignancy in the thorax.CommentWhen we encounter a neck tumor, the patient's age, clinical presentation, and physical findings are important. In the pediatric population, 80% to 90% of head and neck masses represent benign conditions. Lymphoma is the most common head and neck malignancy in children [1Dickson P.V. Davidoff A.M. Malignant neoplasms of the head and neck.Sem Ped Surg. 2006; 15: 92-98Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar]. Physical examinations including oral cavity, pharynx, larynx, thyroid, salivary glands, and skin of the head and neck should be checked for identifying primary origin. In patients who have negative findings in their physical examinations, a secondary survey can help, which may include image study, fine-needle aspiration biopsy, or open biopsy.Thymic carcinoma can occur in all age groups, but this is found to be most frequent in adults between 30 and 60 years of age. In our case, the patient's age was young (24 years), and due to the unusual location, we did not believe that a thymic carcinoma was the diagnosis. Physical examinations and imaging studies resulted in negative findings of the oral cavity, nasopharynx, oropharynx, hypopharynx, larynx, and esophagus; thus the neck tumor was unlikely to have originated from these locations. Ultimately, a fine-needle aspiration showed anaplastic carcinoma, which led us to believe the tumor had originated from the thyroid. However, the pathologic reports proved thymic carcinoma.Thymic carcinoma is a rare tumor and tends to be aggressive. This represents less than 1% of thymic malignancies. Five-year survival rates for all patients are approximately 30% to 50% [2Eng T.Y. Fuller C.D. Jagirdar J. Bains Y. Thomas Jr, C.R. Thymic carcinoma: state of the art review.Int J Radiat Oncol Biol Phys. 2004; 59: 654-664Abstract Full Text Full Text PDF PubMed Scopus (132) Google Scholar]. Thymic carcinoma is a poorly differentiated epithelial neoplasm in histology and has been classified as type C thymoma by the World Health Organization (WHO). The most common location of thymic carcinoma is in the anterior mediastinum. Several reports revealed the different locations of thymic carcinoma. One case report revealed an intrapericardial primary thymic carcinoma [3Calderon A.M. Merchan J.A. Rozo J.C. et al.Intrapericardial primary thymic carcinoma in a 73-year-old man.Tex Heart Inst J. 2008; 35: 458-461PubMed Google Scholar]. Hsu and colleagues [4Hsu I.L. Wu M.H. Lai W.W. et al.Cervical ectopic thymoma.J Thorac Cardiovasc Surg. 2007; 133: 1658-1659Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar] reported 2 patients with cervical ectopic thymoma, with one of them being a thymic carcinoma.The case that we present is a thymic squamous cell carcinoma growing in the cervical region. Due to its ectopic location, it was initially confused with a thyroid tumor. Squamous cell carcinoma is a common cell type in previous studies of thymic carcinomas. Chung [5Chung D.A. Thymic carcinoma—analysis of nineteen clinicopathological studies.Thorac Cardiovasc Surg. 2000; 48: 114-119Crossref PubMed Scopus (57) Google Scholar] reviewed 19 clinicopathologic studies of thymic carcinoma; there were 40 of 140 patients who presented with squamous cell carcinoma histology [5Chung D.A. Thymic carcinoma—analysis of nineteen clinicopathological studies.Thorac Cardiovasc Surg. 2000; 48: 114-119Crossref PubMed Scopus (57) Google Scholar]. Besides, thymic carcinoma lacks organotypical features of thymic differentiation in histology. Current studies propose that detection of the specific immunohistochemical markers in the tumor cells, especially CD5, could be used to support the diagnosis of primary thymic carcinoma [6Moran C.A. Suster S. Thymic carcinoma: current concepts and histologic features.Hematol Oncol Clin North Am. 2008; 22: 393-407Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar].The treatment of thymic carcinoma remains undefined because of the low incidence. The favored treatment protocols are surgical resection plus radiotherapy and cisplatinum-based chemotherapy. We presented a case of thymic carcinoma with the initial presentation of a neck mass that had been successfully treated by surgical resection with chemotherapy and radiotherapy. Although we found only two reported cases of carcinoma in ectopic thymus in the neck, thymic carcinoma should be considered in the differential diagnosis of a neck mass [4Hsu I.L. Wu M.H. Lai W.W. et al.Cervical ectopic thymoma.J Thorac Cardiovasc Surg. 2007; 133: 1658-1659Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar, 7Nomori H. Morinaga S. Kobayashi R. Mimura T. Cervical thymic cancer infiltrating the trachea and thyroid.Eur J Cardiothorac Surg. 1994; 8: 222-224Crossref PubMed Scopus (11) Google Scholar]. Thymic carcinoma is an uncommon tumor arising from the thymus, which may be accompanied by a cough, chest pain, phrenic nerve palsy, or superior vena cava syndrome. This tumor is most commonly located in the anterior mediastinum. A 24-year-old woman presented with a neck mass of gradual enlargement accompanied by difficulty swallowing and neck soreness for 1 month. There was chest pain and hoarseness, but no cough. Because the tumor had enlarged with time, the patient sought medical attention at our hospital. A physical examination showed a cervical firm and fixed mass, nontender, and well-demarcated with extension to the left clavicle. Laboratory data included thyroid-stimulating hormone, thyroglobulin, and T4 concentration that were all within normal range. A chest roentgenogram showed mild deviation of the trachea in the cervical region. A contrast-enhanced computed tomographic scan of the neck revealed a tumor approximately 5 cm in diameter that was located near the left thyroid gland. The mass showed heterogeneous contrast enhancement with central low density (Figs 1A, B). A thyroid tumor was suspected and a fine-needle aspiration report indicated anaplastic carcinoma. A low collar incision was made (approximately 8 cm in length). The subcutaneous tissue, fascia, and muscle were carefully separated. The tumor was lying near the carotid sheath and next to the infrahyoid muscles. The trachea was found to be deviated to the right side due to compression. Although with some adhesions, there was no evidence of direct invasion to the major vessels, bone, trachea, esophagus, thyroid gland, and thymus. The patient underwent a complete resection. The tumor measured 5.5 × 5 × 2.3 cm and had a nodular outer surface. Histology revealed that the tumor grew in irregular nests with focal squamoid differentiation (Figs 2A, B), and the thymus tissue was found near the tumor (Fig 3A). The tumor cells showed immunoreactivity for CD5, CD117, P63, synaptophysin, and chromogranins (Fig 3B). The thyroglobulin and thyroid transcription factor-1 could not be stained. A postoperative chemotherapy regiment of cisplatin with gemcitabine was administered to the patient. She received a total of three courses of concurrent chemoradiotherapy. The total radiotherapy doses were 5,580 cGy. No recurrence was found after 6 months. A postoperative contrast-enhanced computed tomographic scan showed no evidence of malignancy in the thorax. CommentWhen we encounter a neck tumor, the patient's age, clinical presentation, and physical findings are important. In the pediatric population, 80% to 90% of head and neck masses represent benign conditions. Lymphoma is the most common head and neck malignancy in children [1Dickson P.V. Davidoff A.M. Malignant neoplasms of the head and neck.Sem Ped Surg. 2006; 15: 92-98Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar]. Physical examinations including oral cavity, pharynx, larynx, thyroid, salivary glands, and skin of the head and neck should be checked for identifying primary origin. In patients who have negative findings in their physical examinations, a secondary survey can help, which may include image study, fine-needle aspiration biopsy, or open biopsy.Thymic carcinoma can occur in all age groups, but this is found to be most frequent in adults between 30 and 60 years of age. In our case, the patient's age was young (24 years), and due to the unusual location, we did not believe that a thymic carcinoma was the diagnosis. Physical examinations and imaging studies resulted in negative findings of the oral cavity, nasopharynx, oropharynx, hypopharynx, larynx, and esophagus; thus the neck tumor was unlikely to have originated from these locations. Ultimately, a fine-needle aspiration showed anaplastic carcinoma, which led us to believe the tumor had originated from the thyroid. However, the pathologic reports proved thymic carcinoma.Thymic carcinoma is a rare tumor and tends to be aggressive. This represents less than 1% of thymic malignancies. Five-year survival rates for all patients are approximately 30% to 50% [2Eng T.Y. Fuller C.D. Jagirdar J. Bains Y. Thomas Jr, C.R. Thymic carcinoma: state of the art review.Int J Radiat Oncol Biol Phys. 2004; 59: 654-664Abstract Full Text Full Text PDF PubMed Scopus (132) Google Scholar]. Thymic carcinoma is a poorly differentiated epithelial neoplasm in histology and has been classified as type C thymoma by the World Health Organization (WHO). The most common location of thymic carcinoma is in the anterior mediastinum. Several reports revealed the different locations of thymic carcinoma. One case report revealed an intrapericardial primary thymic carcinoma [3Calderon A.M. Merchan J.A. Rozo J.C. et al.Intrapericardial primary thymic carcinoma in a 73-year-old man.Tex Heart Inst J. 2008; 35: 458-461PubMed Google Scholar]. Hsu and colleagues [4Hsu I.L. Wu M.H. Lai W.W. et al.Cervical ectopic thymoma.J Thorac Cardiovasc Surg. 2007; 133: 1658-1659Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar] reported 2 patients with cervical ectopic thymoma, with one of them being a thymic carcinoma.The case that we present is a thymic squamous cell carcinoma growing in the cervical region. Due to its ectopic location, it was initially confused with a thyroid tumor. Squamous cell carcinoma is a common cell type in previous studies of thymic carcinomas. Chung [5Chung D.A. Thymic carcinoma—analysis of nineteen clinicopathological studies.Thorac Cardiovasc Surg. 2000; 48: 114-119Crossref PubMed Scopus (57) Google Scholar] reviewed 19 clinicopathologic studies of thymic carcinoma; there were 40 of 140 patients who presented with squamous cell carcinoma histology [5Chung D.A. Thymic carcinoma—analysis of nineteen clinicopathological studies.Thorac Cardiovasc Surg. 2000; 48: 114-119Crossref PubMed Scopus (57) Google Scholar]. Besides, thymic carcinoma lacks organotypical features of thymic differentiation in histology. Current studies propose that detection of the specific immunohistochemical markers in the tumor cells, especially CD5, could be used to support the diagnosis of primary thymic carcinoma [6Moran C.A. Suster S. Thymic carcinoma: current concepts and histologic features.Hematol Oncol Clin North Am. 2008; 22: 393-407Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar].The treatment of thymic carcinoma remains undefined because of the low incidence. The favored treatment protocols are surgical resection plus radiotherapy and cisplatinum-based chemotherapy. We presented a case of thymic carcinoma with the initial presentation of a neck mass that had been successfully treated by surgical resection with chemotherapy and radiotherapy. Although we found only two reported cases of carcinoma in ectopic thymus in the neck, thymic carcinoma should be considered in the differential diagnosis of a neck mass [4Hsu I.L. Wu M.H. Lai W.W. et al.Cervical ectopic thymoma.J Thorac Cardiovasc Surg. 2007; 133: 1658-1659Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar, 7Nomori H. Morinaga S. Kobayashi R. Mimura T. Cervical thymic cancer infiltrating the trachea and thyroid.Eur J Cardiothorac Surg. 1994; 8: 222-224Crossref PubMed Scopus (11) Google Scholar]. When we encounter a neck tumor, the patient's age, clinical presentation, and physical findings are important. In the pediatric population, 80% to 90% of head and neck masses represent benign conditions. Lymphoma is the most common head and neck malignancy in children [1Dickson P.V. Davidoff A.M. Malignant neoplasms of the head and neck.Sem Ped Surg. 2006; 15: 92-98Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar]. Physical examinations including oral cavity, pharynx, larynx, thyroid, salivary glands, and skin of the head and neck should be checked for identifying primary origin. In patients who have negative findings in their physical examinations, a secondary survey can help, which may include image study, fine-needle aspiration biopsy, or open biopsy. Thymic carcinoma can occur in all age groups, but this is found to be most frequent in adults between 30 and 60 years of age. In our case, the patient's age was young (24 years), and due to the unusual location, we did not believe that a thymic carcinoma was the diagnosis. Physical examinations and imaging studies resulted in negative findings of the oral cavity, nasopharynx, oropharynx, hypopharynx, larynx, and esophagus; thus the neck tumor was unlikely to have originated from these locations. Ultimately, a fine-needle aspiration showed anaplastic carcinoma, which led us to believe the tumor had originated from the thyroid. However, the pathologic reports proved thymic carcinoma. Thymic carcinoma is a rare tumor and tends to be aggressive. This represents less than 1% of thymic malignancies. Five-year survival rates for all patients are approximately 30% to 50% [2Eng T.Y. Fuller C.D. Jagirdar J. Bains Y. Thomas Jr, C.R. Thymic carcinoma: state of the art review.Int J Radiat Oncol Biol Phys. 2004; 59: 654-664Abstract Full Text Full Text PDF PubMed Scopus (132) Google Scholar]. Thymic carcinoma is a poorly differentiated epithelial neoplasm in histology and has been classified as type C thymoma by the World Health Organization (WHO). The most common location of thymic carcinoma is in the anterior mediastinum. Several reports revealed the different locations of thymic carcinoma. One case report revealed an intrapericardial primary thymic carcinoma [3Calderon A.M. Merchan J.A. Rozo J.C. et al.Intrapericardial primary thymic carcinoma in a 73-year-old man.Tex Heart Inst J. 2008; 35: 458-461PubMed Google Scholar]. Hsu and colleagues [4Hsu I.L. Wu M.H. Lai W.W. et al.Cervical ectopic thymoma.J Thorac Cardiovasc Surg. 2007; 133: 1658-1659Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar] reported 2 patients with cervical ectopic thymoma, with one of them being a thymic carcinoma. The case that we present is a thymic squamous cell carcinoma growing in the cervical region. Due to its ectopic location, it was initially confused with a thyroid tumor. Squamous cell carcinoma is a common cell type in previous studies of thymic carcinomas. Chung [5Chung D.A. Thymic carcinoma—analysis of nineteen clinicopathological studies.Thorac Cardiovasc Surg. 2000; 48: 114-119Crossref PubMed Scopus (57) Google Scholar] reviewed 19 clinicopathologic studies of thymic carcinoma; there were 40 of 140 patients who presented with squamous cell carcinoma histology [5Chung D.A. Thymic carcinoma—analysis of nineteen clinicopathological studies.Thorac Cardiovasc Surg. 2000; 48: 114-119Crossref PubMed Scopus (57) Google Scholar]. Besides, thymic carcinoma lacks organotypical features of thymic differentiation in histology. Current studies propose that detection of the specific immunohistochemical markers in the tumor cells, especially CD5, could be used to support the diagnosis of primary thymic carcinoma [6Moran C.A. Suster S. Thymic carcinoma: current concepts and histologic features.Hematol Oncol Clin North Am. 2008; 22: 393-407Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar]. The treatment of thymic carcinoma remains undefined because of the low incidence. The favored treatment protocols are surgical resection plus radiotherapy and cisplatinum-based chemotherapy. We presented a case of thymic carcinoma with the initial presentation of a neck mass that had been successfully treated by surgical resection with chemotherapy and radiotherapy. Although we found only two reported cases of carcinoma in ectopic thymus in the neck, thymic carcinoma should be considered in the differential diagnosis of a neck mass [4Hsu I.L. Wu M.H. Lai W.W. et al.Cervical ectopic thymoma.J Thorac Cardiovasc Surg. 2007; 133: 1658-1659Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar, 7Nomori H. Morinaga S. Kobayashi R. Mimura T. Cervical thymic cancer infiltrating the trachea and thyroid.Eur J Cardiothorac Surg. 1994; 8: 222-224Crossref PubMed Scopus (11) Google Scholar].