대한흉부영상의학회 Weekly Chest Cases

Case No : 1044 Date 2017-10-30

Courtesy of Kum Ju Chae, Gong Yong Jin / Chonbuk National University College of Medicine
Age/Sex 64 / F
Chief ComplaintDyspnea, wheezing

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Diagnosis With Brief Discussion

Diagnosis: Endotracheal neurofibroma
Radiologic Findings: Fig 1. Chest PA shows endotracheal mass-like lesion in the lower trachea.
Fig 2. Non-enhanced axial chest CT image shows endotracheal mass attached to the left tracheal wall with extraluminal extension to the mediastinal fat.
Fig 3. Enhanced chest CT image demonstrates homogeneously enhancing mass in the lower trachea.
Fig 4. Bronchosopic examination revealed a polypoid mass attached to the left tracheal wall.
Fig 5. Histopathologic examination showed the tumor to be a neurofibroma, staining positive for S100.
Brief Review: Intrathoracic neurogenic tumors mostly occur in the posterior mediastinum and rarely in the lungs. Neurogenic tumors constitute 0.2% of pulmonary neoplasms, and 25% of pulmonary neurogenic tumors are endobronchial, which often cause obstructive symptoms. Most tumors are solitary and not associated with neurofibromatosis type I, but multiple neurofibromas and those with diffuse or plexiform morphology are associated with neurofibromatosis type 1.
On histology, neurofibromas are composed of haphazardly arranged spindle cells with wavy nuclei, eosinophilic cytoplasm, and variably collagenous and myxoid matrix. At CT, endobronchial neurogenic tumors have been described as round or lobulated well-defined homogeneous masses. MR imaging may also be useful regarding the evaluation of the internal architecture of neurofibromas. The MR imaging features, such as the target sign on T2-weighted MR images, have been reported to be characteristic for neurofibromas.
The treatment of endotracheal or endobronchial neurofibromas is determined by the size and the location of the tumor. Minimally invasive management can be achieved with endoscopic resection with or without laser. Surgical resection may be indicated in cases where the diagnosis is uncertain or when there is a concern for hemorrhagic complications, invasion of adjacent structures, or technical difficulties for bronchoscopic management. The prognosis is excellent in the absence of neurofibromatosis.
References: 1. Ko JM, Jung JI, Park SH, et al. Benign tumors of the tracheobronchial tree: CT-pathologic correlation. AJR 2006; 186: 1304-1313
2. Park CM, Goo JM, Lee HJ, et al. Tumors in the tracheobronchial tree: CT and FDG PET features. Radiographics 2009; 29:55-71
3. Willmann J K, Weishaupt D, Kestenholz P B, et al. Endotracheal neurofibroma in neurofibromatosis type 1: an unusual manifestation. Eur. Radiol. 2002; 12:190-192
Keywords: Airway, Trachea, Neoplasm, Benign neoplasm, Neurogenic tumor,

No. of Applicants : 100

▶ Correct Answer as Differential Diagnosis : 2/100, 2.0%

- The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
- Ajou University Hospital , Korea (South) YOO YOUNGJIN

▶ Semi-Correct Answer : 17/100, 17.0%

- Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
- Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
- Higashi-Ohmi General Medical Center , Japan AKITOSHI INOUE
- King Abdulaziz University Hospital , Saudi Arabia Amr M. Ajlan
- IRSA LA ROCHELLE , France, Metropolitan BIGOT
- The University of Tokyo Hospital , Japan Yusuke Watanabe
- Other , Korea (South) SEONG SU KANG
- Kyemyung University Dongsan Hospital , Korea (South) MINJAE GU
- The Jikei university , Japan TAKU GOMI
- Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
- 怨紐 , Korea (South) jin young kim
- CLINIQUE STE CLOTILDE , Reunion PATRICK MASCAREL
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
- Other , Korea (South) HAYEON LEE
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- University of Tsukuba , Japan Toshitaka Ishiguro
- University of Utah , United States AKIHIKO SAKATA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1044 Date 2017-10-30

Discussion

No. of Applicants : 100