대한흉부영상의학회 Weekly Chest Cases

Case No : 1366 Date 2023-12-26

Courtesy of Yura Ahn / Asan medical center
Age/Sex 20 / F
Chief ComplaintCough and chest radiograph abnormality History: none, never smoker

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Diagnosis With Brief Discussion

Diagnosis: NUT carcinoma
Radiologic Findings: Fig 1. Chest PA shows consolidation or mass in LLLZ including retrocardiac area and left pleural effusion.
Fig 2. Axial chest CT scan shows a large mass in LLL.
Fig 3. Coronal chest CT scan shows a large mass in LLL conglomerated with enlarged multiple lymph nodes in the AP window, subcarina, and left interlobar area.
Fig 4. Chest PA obtained 3 months before initial diagnosis shows a mass in the retrocardiac area, suggesting the rapidly growing nature of the mass.
Brief Review: NUT carcinoma, first identified in 1991 as a severe form of thymic carcinoma, is a type of poorly differentiated carcinoma characterized by genetic alterations in the NUTM1 gene (located at 15q14). Originally perceived as a variant of squamous cell carcinoma predominantly affecting young individuals' midline structures above the diaphragm, it's now understood that these tumors can develop in any organ. This condition can affect people of all ages, but it is most frequently diagnosed in children and young adults, with a typical onset in the early twenties. There is no specific gender more likely to develop this disease. Approximately half of the cases are found in the thoracic cavity, while the head and neck region accounts for around 40%. Notably, thoracic NUT carcinoma isn't caused by smoking, and most patients have little to no history of smoking. These tumors are often in advanced stages when discovered. NUT carcinoma is known for its high aggressiveness, with an average survival period of just 6.5 months post-diagnosis.
CT scans of NUT carcinoma showed a primary lung mass, at least 5 cm in diameter that merged with the swelling of lymph nodes in the hilum and mediastinum. This was frequently accompanied by atelectasis due to obstruction and involvement of the pleura on the same side.
References: 1. Akihiko Yoshida. Histopathology. 2024 Jan;84(1):86-101.
2. Sholl LM, Nishino M, Pokharel S, Mino-Kenudson M, French CA, Janne PA, Lathan C. Primary Pulmonary NUT Midline Carcinoma: Clinical, Radiographic, and Pathologic Characterizations. J Thorac Oncol. 2015 Jun;10(6):951-9.
Keywords

No. of Applicants : 79

▶ Correct Answer : 14/79, 17.7%

- , Japan SUZUNE TSUKAMOTO
- , Japan HIROAKI ARAKAWA
- The University of Tokyo Hospital , Japan JUN KANZAWA
- Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
- Osaka University , Japan AKINORI HATA
- Japanese Red Cross Medical Center , Japan YASUSHI AOYAGI
- Kyoto University Hospital , Japan YASUHISA KURATA
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
- Osaka metropolitan university Hospital , Japan TATSUSHI OURA
- Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- Matsunami General Hospital , Japan TARO TAKEDA
- , Korea (South) JIN WOO YOON
- Jiangsu province hospital , China WANGJIAN ZHA

▶ Correct Answer as Differential Diagnosis : 9/79, 11.4%

- Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
- Kyoto University , Japan AKIHIKO SAKATA
- Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
- , Italy PAOLO BALDASSARI
- Gifu University Hospital , Japan Yo Kaneko
- Kyoto University , Japan SHO KOYASU
- Sotiria Hospital, Athens , Greece VASILIOS TZILAS
- , Japan YUMI MAEHARA
- Kyoto university , Japan AKIHIKO SAKATA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Cases by Disease Category

Case No : 1366 Date 2023-12-26

Discussion

No. of Applicants : 79