- 著者
- Mika Kawagoe Kohei Odajima Shinichiro Asakawa Michito Nagura Shigeyuki Arai Osamu Yamazaki Yoshifuru Tamura Ryuji Ohashi Takamasa Iwakura Hideo Yasuda Shigeru Shibata Yoshihide Fujigaki
- 出版者
- The Japanese Society of Internal Medicine
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- pp.1251-22, (Released:2023-03-22)
- 参考文献数
- 27
Exostosin 1 (EXT1) and exostosin 2 (EXT2)-associated membranous nephropathy (MN) may be associated with active autoimmune disease. We encountered an elderly man who presented with EXT1/EXT2-associated lupus-like MN with full house immune deposits, monoclonal gammopathy of uncertain significance and Sjögren's syndrome. The patient exhibited various other immune abnormalities. Although he did not fulfill the criteria of clinical systemic lupus erythematosus (SLE), he met a stand-alone renal criterion of SLICC 2012. Whether or not a stand-alone renal criterion with EXT1/EXT2 positivity, as in the present patient, can efficiently guide decisions regarding the diagnosis and treatment of SLE remains a clinical dilemma.
- 著者
- Tomoo Jikuzono Shigekazu Suzuki Osamu Ishibashi Shoko Kure Atsuko Sakanushi Munenaga Nakamizo Masashi Kawamoto Ryuji Ohashi Tetsu Yamada Iwao Sugitani
- 出版者
- The Medical Association of Nippon Medical School
- 雑誌
- Journal of Nippon Medical School (ISSN:13454676)
- 巻号頁・発行日
- pp.JNMS.2022_89-207, (Released:2021-04-19)
- 被引用文献数
- 2
Background: Adenoid cystic carcinoma of the trachea (ACCT) is a rare cancer; ACCT with thyroid invasion is particularly rare. We first suspected anaplastic thyroid carcinoma (ATC) but diagnosed ACC after performing fine needle aspiration cytology (FNAC). A tracheal origin was confirmed after operation.Case Description: We report the case of a 77-year-old female presenting to our hospital with acute inspiratory dyspnea requiring emergency tracheotomy. Physical examination revealed a right anterior neck swelling with a hard and unmovable mass. Computed tomography (CT) and ultrasonography (US) showed tumor extension to the right thyroid lobe, and between the first and third tracheal rings, which caused severe stenosis of the lumen. Next, we performed FNAC. Clinical findings were highly suspicious for ACCT with thyroid invasion. Thirty-five days after the first visit to our department, the patient underwent total laryngectomy, cervical esophagectomy, and thyroidectomy with bilateral selective neck dissections at another hospital. The tumor was located in the right posterior wall of the trachea, with extension into the right thyroid gland. Pathological examination showed an infiltrative carcinomatous proliferation with tubular and cribriform patterns. The tumor was classified as pT4N1. A definite diagnosis was made after histopathological analyis of the surgical specimen confirmed ACCT. The tumor was found to be positive for FABP7, a putative prognostic marker of ACC, and metastasized to the lungs 3 years after the surgery.Conclusions: ACCT with thyroid invasion is an extremely rare malignant neoplasm. FNAC was useful for differentiating ACCT from other diagnoses and enabled appropriate surgical treatment.