대한흉부영상의학회 Weekly Chest Cases

Case No : 1364 Date 2023-12-12

Courtesy of Sung Shine Shim, Woocheol Kwon / Ewha Womans University Seoul Hospital
Age/Sex 81 / F
Chief ComplaintBoth lower extremities (proximal limb) weakness for 3 months

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Diagnosis With Brief Discussion

Diagnosis: small cell cancer associated with Lambert-Eaton myasthenic syndrome
Radiologic Findings: Histological examination of bronchoscopic-guided biopsy disclosed small cell lung cancer. Following a diagnosis of LEMS, the patient underwent one course of cisplatin + etoposide chemotherapy.

Fig 1-3. Chest CT images show an enhancing mass in subcarina and enlarged LNs in paratracheal area.
Fig 4-5. On the fused PET/CT images, the mass and LNs show focal hypermetabolic uptake.
Fig 6-7. Lung window setting of CT images show multifocal bronchiectasis with bronchiolitis. Decreased volume of left lung combined with effusion is noted.
Brief Review: The Lambert–Eaton myasthenic syndrome (LEMS) is an autoimmune disease characterized by weakness, reduced reflexes, and autonomic involvement. LEMS is typically characterized as a paraneoplastic syndrome associated with small cell lung carcinoma (SCLC). The presence of LEMS in association with other neuroendocrine lung tumors, including carcinoids or large cell lung carcinoma, is highly unusual.
It is hypothesized that the neurological dysfunction in SCLC LEMS is triggered by the development of autoantibodies to the P/Q voltage-gated calcium channels (VGCC) ectopically expressed in lung by the tumor, which cross-react with channels at the neuromuscular junction. The causal relationship is supported by remission of LEMS in the few patients with complete regression of their tumors following treatment. The majority of patients have neurological symptoms before the diagnosis of malignancy is made.
Treatment of LEMS initially involves symptomatic management. In cases of SCLC-LEMS treatment of the tumor is key, as this may result in remission of LEMS. Immunological therapy may be required in more severe cases.
References: 1. O’Neill JH, Murray NMF, Newsom-Davis J. The Lambert-Eaton myasthenic syndrome – a review of 50 cases. Brain. 1988;111(Pt 3): 577–596.
2. Zhang RR, Han T, Guo F, et al. Lambert-Eaton myasthenic syndrome in a patient with small-cell lung cancer: A case report. Oncol Lett. 2015 Sep;10(3):1339-1342.
3. Brueck M, Vogel S, Waeger S, et al. Lambert-Eaton myasthenic syndrome with acute respiratory failure associated with small cell lung cancer. Dtsch Med Wochenschr. 2004 Jan 30;129(5):193-196.
4. Kejian Zhang , Wei Liu, Yang Li et al. Mediastinal small cell cancer associated with Lambert-Eaton myasthenic syndrome: A case report. Exp Ther Med. 2015 Jul;10(1):117-120.
Keywords

No. of Applicants : 70

▶ Correct Answer : 22/70, 31.4%

- Oita University, Faculty of Medicine , Japan FUMITO OKADA
- Jiangsu province hospital , China WANGJIAN ZHA
- Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
- Fukuoka university , Japan KEISUKE SATO
- Osaka metropolitan university Hospital , Japan TATSUSHI OURA
- GHICL , France MANUEL TOLEDANO
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- , Japan KENTARO KOTANI
- , Japan SHUNJIRO NOGUCHI
- Qatif Central Hospital , Saudi Arabia HANI MAKKY ALSALAM
- Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
- , China LI GUO
- , Japan YUMI MAEHARA
- Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
- , Japan SUZUNE TSUKAMOTO
- Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
- Toyota Kosei Hospital , Japan YUKI HAYASHI
- Hyogo Prefectural Kobe Children , Japan SHUHEI NORIMOTO
- Tokyo Medical and Dental University , Japan MIKIYA FUJII
- , Japan HIROAKI ARAKAWA
- Yokkaichi municipal hospital , Japan HIKARI FUKUI
- Nerimahikarigaoka hospital , Japan TAKANA HAYASHI

▶ Correct Answer as Differential Diagnosis : 5/70, 7.1%

- McGill University Health Center , Canada ALEXANDRE SEMIONOV
- The Catholic University of Korea Yoeuido St. Mary , Korea (South) CHAWOONG JEON
- , Japan KYOKO NAGAI
- Vita Hospital , Brazil DIOGO LAGO PINHEIRO
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI

▶ Semi-Correct Answer : 2/70, 2.9%

- Others , India PURUSHOTHAMA RAO TUMMALA
- Matsunami General Hospital , Japan TARO TAKEDA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1364 Date 2023-12-12

Discussion

No. of Applicants : 70