2019. 03. 21. 대한흉부영상의학회 월례집담회 증례발표 전북대학교병원 양혜진

위내시경상 발견한 subepithelial tumor 510628 Case 1 (66/F) C.C. ) 위내시경상 발견한 subepithelial tumor PMHx. ) N/S

Esophagus의 left lateral side에 약 2. 4x2 Esophagus의 left lateral side에 약 2.4x2.6cm크기의 내부에 calcification을 포함하는 round shape의 mass가 있으며 homogenous enhancement를 보이고 있음.    esophagus와의 wall에 irregularity가 동반되어 있어 malignancy 가능성이 있음 R/O malignant looking mediastinal mass (line)  - D/Dx with Tb lymphadenitis Superior mediastinal mass LNs Esophagus

Malignant mediastinal mass with esophageal invasion? Retrotracheal Tb lymphadenitis? Malignant esophageal neoplasm? Esophageal leiomyoma? Esophageal leiomyoma : common, calcification - 보통 lumen을 좁히며 intramural growth 하므로 약간 다르다 Malignant esophageal neoplasm Retrotracheal LN?? Malignant mediastinal mass with esophageal invasion

Rarely schwannomas occur in the superior/middle mediastinal compartment arising from the vagus or phrenic nerves. (2% of all mediastinal neurogenic tumors) Radiologically they are sharply demarcated with rare calcifications. Contrast enhanced CT shows a sharply demarcated mass with low densities and mild enhancement, rarely with calcifications and no fat. Journal of the association of physicians of india 2016;65:92-95 J Cardiothorac Surg. 2009; 4: 68.

Blood tinged sputum, cough 1735612 Case 2 (67/M) C.C. ) Blood tinged sputum, cough PMHx. ) DM (1995), NSTEMI (2015)

2017-06-28 Chest CT Both SCL 가 최대 1.5cm 이하로 커져 있음 2. Mediastinum 내 대부분의 LN와 10R, 11R LN가 커져있으며,  heterogeneous enhancement를 보이고 있음. 3. No evidence of tracheal mass or stenosis. 4. RLL 의 superior segment에서 spiculated margin과 heterogeneous enhancement를 보이는 약 4*4*5.3cm 크기의 mass가 관찰됨    -  이 mass는 pleura와 바로 맞닿아 있고, Rt. main bronchus를 narrowing 시키고 있어, visceral pleura 이상의 부분과, Rt. main bronchus 에 invasion이 있는 것으로 판단됨.    - 또한 이 mass는 mediastinal pleura와도 맞닿아 있어 mediastinum 으로의 invasion도 배제할 수는 없음    - RUL 의 posterior segment 에 tiny nodule이 관찰되며 benign 으로 판단됨 5. Both pleural space 에 이상 소견은 보이지 않음.    - T12의 Rt. side에서 osteolytic change가 관찰되며, metastasis를 배제할 수 없음 Suggestive of lung cancer in RLL with multiple LN metastasis in mediastinum and both SCL    - if cancer, T3이상 N3 (AJCC 8th)

2017-06-28 Chest CT Both SCL 가 최대 1.5cm 이하로 커져 있음 2. Mediastinum 내 대부분의 LN와 10R, 11R LN가 커져있으며,  heterogeneous enhancement를 보이고 있음. 3. No evidence of tracheal mass or stenosis. 4. RLL 의 superior segment에서 spiculated margin과 heterogeneous enhancement를 보이는 약 4*4*5.3cm 크기의 mass가 관찰됨    -  이 mass는 pleura와 바로 맞닿아 있고, Rt. main bronchus를 narrowing 시키고 있어, visceral pleura 이상의 부분과, Rt. main bronchus 에 invasion이 있는 것으로 판단됨.    - 또한 이 mass는 mediastinal pleura와도 맞닿아 있어 mediastinum 으로의 invasion도 배제할 수는 없음    - RUL 의 posterior segment 에 tiny nodule이 관찰되며 benign 으로 판단됨 5. Both pleural space 에 이상 소견은 보이지 않음.    - T12의 Rt. side에서 osteolytic change가 관찰되며, metastasis를 배제할 수 없음 Suggestive of lung cancer in RLL with multiple LN metastasis in mediastinum and both SCL    - if cancer, T3이상 N3 (AJCC 8th)

2017-07-04 Lung, PTNB Inflammatory cell Fibrotic background

Findings are suggestive of inflammatory myofibroblastic tumor. 2017-07-04 Lung, PTNB Inflammatory cell Findings are suggestive of inflammatory myofibroblastic tumor. Actin : positive IgG, IgG4 : non significant ALK, CD31 : negative Fibroblast

1. Lt SCL에 enlarged LN들은 이전과 큰 변화 없으며, Rt SCL area에는 post op change가 관찰됨. 2. Mediastinum 내 대부분의 LN와 10R LN가 커져있음 3. No evidence of tracheal mass or stenosis. 4. RLL pleura와 맞닿아 있고, Rt. main bronchus를 narrowing 시키던 mass like lesion은 Bx. 검사상     inflammatory myofibroblastic tumor 로 확인되었으며 이전보다 extent가 더 감소되었음.   - RUL 의 posterior segment 에 tiny nodule은 크기가 작아져 현재 잘 구분되지 않음.    - Both lung에 acute pneumonic consolidation은 관찰되지 않음. 5. Both pleural space 에 이상 소견은 보이지 않음.    - T12의 Rt. side에서 osteolytic change가 이전과 변화 없이 관찰되며, Bx. 검사상 benign으로 확인됨. (arrow) CONCLUSION Suggestive of inflammatory myofibroblastic tumor in RLL   - decreased extent compared with 2017-08-21 Suggestive of multiple reactive LNs in Lt. SCL, and mediastinum 2017-06-28 Prednisone (17.8.13~) : 6T 2017-08-26

Compatible with inflammatory myofibroblastic tumor Actin : positive Desmin : focal positive ALK : negative IgG, IgG4: not significant 2017-08-26 2017-11-07 2018-01-09 2018-02-14 2018-01-24, Lung, PTNB ; Compatible with inflammatory myofibroblastic tumor

2017-08-26 2018-01-09

2018.01.25 Liver Bx) compatible with inflammatory myofibroblatic tumor 2018-01-09 Abd CT . inflammatory myofibroblastic tumor의 extent는 이전과 비교시 큰 변화 보이지 않으며 여전히 RLL pleura와 맞닿아 있고, Rt. main bronchus를 narrowing 시키고 있음. compatible with inflammatory myofibroblatic tumor 2018.01.25 Liver Bx) compatible with inflammatory myofibroblatic tumor

Prednisone : 3T(1/9) → 12T(1/31~) 고용량 스테로이드 치료 2018-03-20 HRCT 2018. 04. 17 expire

Inflammatory pseudotumor (IPT) Appl Immunohistochem Mol 2016;24:721–726) Virchows Arch (2013) 463:743–747 Arch Pathol Lab Med—Vol 134, March 2010 The term “IPT” was used previously for all fibro-inflammatory lesions because of its propensity to mimic clinically and radiologically a malignant process. However, recent concepts have separated Inflammatory myofibroblastic tumor (IMT) : neoplastic lesion Inflammatory pseudotumor (IPT) : reactive inflammatory lesion

IMT (Inflammatory myofibroblastic tumor) : neoplastic process IMT has distinct clinical, pathologic, and molecular features and should be differentiated clearly from IPT. IMT (Inflammatory myofibroblastic tumor) : neoplastic process Previously considered under the umbrella of IPTs Can show local invasion or distant metastasis Primary Tx. : surgical resection Limited success with steroids, radiotherapy and chemotherapy IPT (Inflammatory pseudotumor) : reactive non-neoplastic process Belong to the spectrum of IgG4-related sclerosing conditions Tumefactive lesions with radiologic feature s/o malignancy Responded simple and effective corticosteroid treatment Appl Immunohistochem Mol 2016;24:721–726) Virchows Arch (2013) 463:743–747 Arch Pathol Lab Med—Vol 134, March 2010

IMT : myofibroblastic spindle cell proliferation more marked The major criteria for subclassfication between IMT and IPT is “The number of IgG4 positive cells and ALK expression”. IMT : myofibroblastic spindle cell proliferation more marked Strong cytoplasmic immunoreactivity for SMA (myofibroblastic nature) ALK-1 : positive (40~66.7%) ALK (+) : younger patients, favorable prognosis ALK (-) : older patients, greater nuclear pleomorphism/atypia, more distant metastasis and poor prognosis IPT : lymphoplasmacytic infiltrate more marked Do not show ALK-1 expression in the fibroblast and myofibroblast Higher mean number of IgG4 cell Mean IgG4/IgG ratio : 48.2% (IMT : 10.7%) Appl Immunohistochem Mol 2016;24:721–726) Virchows Arch (2013) 463:743–747 Arch Pathol Lab Med—Vol 134, March 2010

→ Biopsy is usually required to make an diagnosis Radiologic findings is variable and nonspecific, because of the amount of fibrosis and cellular infiltration. Lung : usually single peripheral, well-defined, lobulated mass with lower lobe predominance, heterogeneous enhancement Calcifications can occur (15%, children > adult) Necrosis (10%), cavitation (5%), central scarring (rare) FDG avid (false positive) MR: usually low SI on both T1/2, due to fibrosis Can be aggressive with invasion to bronchi, mediastinum, chest wall Relapse and metastasis can occur → Biopsy is usually required to make an diagnosis AJR 2012; 198:W217–W227 RadioGraphics 2003; 23:719–729

Fig. 1—8-year-old boy with history of cough, fever, and malaise. Contrast-enhanced CT image shows mass replacing right upper lobe. Mass has ring of dystrophic calcification (arrow). Right hemothorax is related to previous open biopsy. Right pneumonectomy revealed inflammatory myofibroblastic tumor Fig. 2—52-year-old male smoker with lung mass. PET/CT images show pleura-based left upper lobe mass with avid FDG uptake from inflammatory pseudotumor Fig. 4—14-year-old girl with history of asthma. A and B, Axial (A) and coronal multiplanar reformation (B) contrast-enhanced CT images show middle and posterior mediastinal mass encasing carina. Coronal image shows extent of mass. Pathologic result was inflammatory myofibroblastic tumor IMT, 16/F IMT, 8/M IPT, 52/M AJR 2012; 198:W217–W227 RadioGraphics 2003; 23:719–729