대한흉부영상의학회 Weekly Chest Cases

Case No : 1308 Date 2022-11-14

Courtesy of Joo Hyeok Choi, Hyewon Choi, Min Jae Cha / Chung-Ang University Hospital
Age/Sex 36 / F
Chief Complaintintermittent chest pain (Onset: 6 months ago)

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Diagnosis With Brief Discussion

Diagnosis: Desmoid fibromatosis
Radiologic Findings: Fig. 1-2. About 8.5cm sized mild, heterogeneously enhancing mass was noted in Rt. Side anterior chest wall showing indistinct margin with pectoralis minor muscle.
Fig. 3. On bone window setting, subtle cortical irregularity and erosion was noted at Rt. 1st rib, anterior arch.
Fig. 4. Three years ago, the mass was noted at Rt. 1st. intercostal space, measuring 3.8cm x 2.1cm.
Fig. 5-6. It shows iso SI on T1-weighted image (figure 5) and high SI on T2-weighted image (figure 6). On T2 weighted image, we can also see irregular band like hypo SI lesion within the mass.
Brief Review: Desmoid fibromatosis is a locally aggressive but non-metastasizing myofibrobalstic neoplasm with infiltrative growth and propensity for local recurrence. The majority of intrathoracic desmoid fibromatosis arise from the chest wall, but cases originating from the pleura, pulmonary parenchyma and mediastinum also have been reported.
Intrathoracic desmoid fibromatosis most often arise in adulthood and show an equal sex distribution. It may cause pain, dyspnea, and kyphoscoliosis or may be detected as an incidental radiologic finding.
The etiology of tumor includes genetic factors and some cases may be associated with prior trauma.

[Pathogenesis]
The majority of cases (as many as 95%) are sporadic, resulting from somatic mutations in CTNNB1; a smaller subset occurs in patients harboring germline APC mutations (Gardner syndrome).

[Imaging findings]
Desmoid-type fibromatoses are commonly intermuscular masses of deep soft tissue. On CT scan, it shows soft tissue mass of variable attenuation and enhancement. It can appear both sharp margin and indistinct margin with infiltration into adjacent structures. Necrosis and calcification is rare.
On MRI, it appears iso to mildly hyperintense relative to muscle on T1- weighted images, and iso- to hyperintense relative to muscle on T2-weighted images. The unusual characteristic of heterogeneous, bandlike low-signal intensity areas on T1- and T2-weighted images is a distinguishing feature of desmoid-type fibromatosis. The nonenhancing hypointense bands are thought to represent areas of increased fibrosis composed of a higher degree of collagen content. A higher percentage of cellularity (as opposed to collagen) correlates with higher signal intensity on T2-weighted and short inversion time inversion recovery images. On PET-CT scan, it shows mild FDG uptake.

[Prognosis and prediction]
The clinical behaviors of desmoid fibromatosis is unpredictable. The recurrence rates as high as 33%. Magin status dose not consistently correlate with recurrence risk.
References: 1) WHO classification of Tumours Editorial Board. Thoracic Tumours. International Agency for Research on Cancer; 2021
2) Elizabeth S. McDonald, Eunhee S. Yi, Doris E. Wenger. Extra-abdominal Desmoid-type fibromatosis. RadioGraphics 2008; 28:901
Keywords

No. of Applicants : 92

▶ Correct Answer : 20/92, 21.7%

- , Japan KYOKO NAGAI
- Showa General Hospital , Japan MASAFUMI KAIUME
- Hospital of Sacred Heart, Montreal , Canada ANDREA OJANGUREN
- Seoul National University Hospital , Korea (South) HYUNGIN PARK
- , Italy PAOLO BALDASSARI
- Beaulieu clinic Geneva , Switzerland GILLES GENIN
- Osaka Metropolitan University , Japan HIROTAKA TAKITA
- Wonkwang University Hospital , Korea (South) HYOJOO KIM
- Soonchunhyang University Hospital Bucheon , Korea (South) HYEJOO PARK
- , Japan YUKIKO USUI
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- Gifu University Hospital , Japan Yo Kaneko
- Kyoto University Hospital , Japan YASUHISA KURATA
- Other , Korea (South) CHAEHUN LIM
- Seoul National University Bundang Hospital , Korea (South) YOONAH DO
- , Japan SOTA MASUOKA
- , Japan YUKI HAYASHI
- , Korea (South) HM PARK
- the first affiliatited hospital of nanjing medical univercity , China HAI XU
- Hanyang University Hospital , Korea (South) SEUNG JIN YOO

▶ Correct Answer as Differential Diagnosis : 23/92, 25.0%

- Jichi Medical University Hospital , Japan JUN KANZAWA
- ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
- McGill University Health Center , Canada ALEXANDRE SEMIONOV
- IRSA LA ROCHELLE , France JEAN LUC BIGOT
- Seoul Medical Center , Korea (South) HYUK GI HONG
- , Japan SHINTARO KANO
- , Japan KENTARO KOTANI
- Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
- Chungbuk National University Hospital , Korea (South) MIHYEON PARK
- Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
- Osaka metropolitan university Hospital , Japan TATSUSHI OURA
- Chonnam National University Hwasun Hospital , Korea (South) JEONG YEOP LEE
- Other , Korea (South) KYU-CHONG LEE
- , Japan MATSUNO MAI
- Kyoto University Hospital , Japan SATOSHI IKEDA
- University of Tsukuba Hospital , Japan SODAI HOSHIAI
- Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
- Toranomon Hospital , Japan KAORU SUMIDA
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- Nerimahikarigaoka hospital , Japan TAKANA HAYASHI
- , Japan YUMI MAEHARA
- Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY

▶ Semi-Correct Answer : 15/92, 16.3%

- , Canada AHMED BENTRIDI
- Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
- National Center of Neurology and Psychiatry , Japan MOTO NAKAYA
- Asan Medical Center , Korea (South) EUNJI CHOI
- Other , Korea (South) SEONGSU KANG
- Chonbuk National University Hospital , Korea (South) JUNGHWAN KIM
- Korea University Guro Hospital , Korea (South) HEEJUN PARK
- Yamanashi Prefectural Central Hospital , Japan HIROAKI WATANABE
- Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
- Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
- Dokkyo Medical University , Japan HIROAKI ARAKAWA
- Nishida Hospital , Japan SHOJI OKUDA
- The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
- Juntendo University , Japan YUTAKA IKENOUCHI
- Jiangsu province hospital , China WANGJIAN ZHA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Cases by Disease Category

Case No : 1308 Date 2022-11-14

Discussion

No. of Applicants : 92