A Case of Concurrent Thymic Carcinoma with Systemic Lupus Erythematosus

흉선암종과 동반된 전신홍반루푸스의 1예

  • Lee, Young Joo (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Choi, Sang Tae (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Kim, Se Hyun (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Jung, Kyung Soo (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Yoon, Sul Hee (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Jeung, Soo Jin (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Yi, Seung Woo (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Kim, Joo Hang (Department of Internal Medicine, Yonsei University College of Medicine)
  • 이영주 (연세대학교 의과대학 내과학교실) ;
  • 최상태 (연세대학교 의과대학 내과학교실) ;
  • 김세현 (연세대학교 의과대학 내과학교실) ;
  • 정경수 (연세대학교 의과대학 내과학교실) ;
  • 윤설희 (연세대학교 의과대학 내과학교실) ;
  • 정수진 (연세대학교 의과대학 내과학교실) ;
  • 이승우 (연세대학교 의과대학 내과학교실) ;
  • 김주항 (연세대학교 의과대학 내과학교실)
  • Received : 2006.11.29
  • Accepted : 2006.12.28
  • Published : 2007.01.30

Abstract

A thymic carcinoma is a rare malignant neoplasm of the thymus epithelium, which can be distinguished from a benign or invasive thymoma. Contrary to a thymoma, the association of a thymic carcinoma and autoimmune disease is rare, with only a few cases having been reported. Herein, a case of thymic carcinoma diagnosed concurrently with systemic lupus erythematosus (SLE) is reported. A 49 year-old man presented at our clinic with myalgia. He was diagnosed with SLE, based on an oral ulcer, lymphopenia, and positive ANA and anti-Sm antibodies. Incidentally, a routine chest X-ray showed a large mediastinal mass. Pathological examination of the mediastinal mass revealed an undifferentiated thymic carcinoma, of WHO classification type C. Further work-up for staging showed multiple bone and lung metastases. With a palliative aim, he received systemic chemotherapy, but refused further chemotherapy after the $2^{nd}$ course. Currently, the patient has not been followed up since the chemotherapy.

흉선암종은 드문 종격동의 악성 종양으로서 흉선상피세포에서 기원하지만 흉선종과는 조직학적 특징이 다르다. 흉선종이 자가면역질환을 종종 동반하는데 반해 흉선암종에서 자가면역질환이 동반되는 예는 드물다. 국내에서는 흉선암종과 자가면역질환이 동반된 예가 보고된 적이 없는데 저자들은 전신 근육통을 주소로 내원한 49세 남자 환자가 전신홍반루프스와 흉선암종을 동시에 진단받고 항암치료를 시작한 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

Keywords

References

  1. Levine GD, Rosai J. Thymic hyperplasia and neoplasia: a review of current concepts. Hum Pathol 1978;9:495-515 https://doi.org/10.1016/S0046-8177(78)80131-2
  2. Cameron, RB. Neoplasms of the mediastinum. In: Devita, Hellman, Rosenberg, editors. Principles and practice of oncology. 6th ed. 2000. p.1024
  3. Sungur A, Ruacan S, Gungen Y, Dalkara T. Myasthenia gravis and primary squamous cell carcinoma of the thymus. Arch Pathol Lab Med 1993;117:937-8
  4. Yonekura S, Nagao T, Arimori S, Kobayashi I, Fukuhara N, Mori T. Thymic carcinoma associated with pinealoma and terminating with peroxidasenegative acute myeloid leukemia. Intern Med 1992;31: 825-7 https://doi.org/10.2169/internalmedicine.31.825
  5. Thomas CV, Manivel JC. Thymic carcinoma and aplastic anemia: report of a previously undocumented association. Am J Hematol 1987;25:333-5 https://doi.org/10.1002/ajh.2830250313
  6. Negron-Soto JM, Cascade PN. Squamous cell carcinoma of the thymus with paraneoplastic hypercalcemia. Clin Imaging 1995;19:122-4 https://doi.org/10.1016/0899-7071(94)00041-A
  7. Suster S, Rosai J. Thymic carcinoma: a clinicopathologic study of 60 cases. Cancer 1991;67:1025-32 https://doi.org/10.1002/1097-0142(19910215)67:4<1025::AID-CNCR2820670427>3.0.CO;2-F
  8. Eng TY, Fuller CD, Jagirdar J, Bains Y, Thomas CR Jr. Thymic carcinoma: state of the art review. Int J Radiat Oncol Biol Phys 2004;59:654-64 https://doi.org/10.1016/j.ijrobp.2003.11.021
  9. Fong PH, Wee A, Chan HL, Tan YO. Primary thymic carcinoma and its association with dermatomyositis and pure red cell aplasia. Int J Dermatol 1992;31: 426-8 https://doi.org/10.1111/j.1365-4362.1992.tb02675.x
  10. Di Cataldo A, Villari L, Milone P, Miano AE, Sambataro MP, Florio G, et al. Thymic carcinoma, systemic lupus erythematosus, and hypertrophic pulmonary osteoarthtopathy. Pediatr Hematol Oncol 2000;17:701-6 https://doi.org/10.1080/08880010050211420