대한흉부영상의학회 Weekly Chest Cases

Case No : 464 Date 2006-09-18

Courtesy of Joo Sung Sun, MD , Kyung Joo Park, MD. / Ajou University School of Medicine, Suwon, Korea.
Age/Sex 75 / F
Chief ComplaintLeft subcostal pain for 5 months and dyspnea

Figure 1
Figure 2
Figure 3

Diagnosis With Brief Discussion

Diagnosis: Thoracic Desmoid Tumor (Fibromatosis)
Radiologic Findings: In the left hemithorax, homogenous hypodense mass was noted. Lung parenchyma adjacent to this mass was compressed and left diaphragm was caudally displaced. Posterior arc of left 8th rib was also eroded and adjacent back muscle was involved.

During operation, large feeding vessel was identified in this mass. Exploratory thoracotomy revealed a tumor originating from the left dorsal region. Visceral plerua was intact and parietal plerua was involved. Resection margin was positive.

Microscopically, small spindle cells with some variation without atypia or other malignant features were found in a fibrous backgound. Immnunohistochemical staing of CD34 was negative.
Brief Review: Desmoid tumor of the chest is a rare soft tissue neoplasm derived from fascial or musculoaponeurotic structure. Most of the reported thoracic desmoid tumors were originated from the chest wall. However, intrathoracic desmoid tumors are rare. Histologic feature of intrathoracic desmoid tumors is similar to those of desmoid tumors at more conventional sites. On immunohistochemical analysis, cytoplasms of the tumor cells are strongly positive for vimentin, and some tumor cells could be positive for α-smooth muscle actin, but all tumor cells are negative for CD34. These findings are characteristic in intrathoracic desmoid tumor and are the differential points from solitary fibrous tumor of the pleura. Desmoid tumors involving the chest and adjacent structures are locally aggressive with a high recurrence rate. Wide radical resection should be attempted.
References: 1. Iqbal M, Rossoff LJ, Kahn L, Lackner RP. Intrathoracic desmoid tumor mimicking primary lung neoplasm. Ann Thorac Surg 2001;71:1698-1700.

2. Abbas AE, Deschamps C, Cassivi SD, Nichols FC 3rd, Allen MS, Schleck CD, Pairolero PC. Chest-wall desmoid tumors: results of surgical intervention. Ann Thorac Surg 2004;78:1219-1223.

3. Takeshima Y, Nakayori F, Nakano T, Shimizu K, Yamashita Y, Toge T, Inai K. Extra-abdominal desmoid tumor presenting as an intrathoracic tumor: case report and literature review. Pathol Int 2001;51:824-828.
Keywords: Chest wall, Benign tumor,

No. of Applicants : 38

▶ Correct Answer : 2/38, 5.3%

- Ondokuz Mayis University, Samsun, Turkey Cetin Celenk
- Hopital Calmette, Chru, Lille, France Toledano Manuel

▶ Correct Answer as Differential Diagnosis : 2/38, 5.3%

- Pgimer, Chandigarh, India Ram Prakash Galwa
- Annecy Hospital, France Oliver Segall

▶ Semi-Correct Answer : 19/38, 50.0%

- RubyHall Clinic, Pune, India Nikhil Unune
- Marien Hospital, Germany Davis Chiramel
- Inje University Ilsan Paik Hospital, Korea Bae Geun Oh
- Kaums, Kashan, Iran Ebrahim Razi
- National Taiwan University Hospital, Taiwan Yu-Feng Wei
- Hangang Sacred Heart Hospital, Korea Eil Seong Lee
- Shalamar Hospital, Lahore, Pakistan Asim Shaukat
- Inha University Hospital , Korea Sang Uk Park
- Sam Anyang Hospital, Korea Jae Seung Seo
- Monaldi Hospital,Naples, Italy Gaetano Rea
- Airforce Hospital, Korea Hyun Seok Choi
- Annemasse, Polyclinique de Savoie, France Gay-Dpassier Philippe
- Zulekha Hospital, Dubai Julie Arora
- Zulekha Hospital, Dubai Saurabh Khandelwal
- CHU Grenoble, France Bing Fabrice
- Doctors Hospital, Nassau, Bahamas N.B.S.Mani
- China Medical University Hospital,Taiwan Jun-Jun Yeh
- Radiologie Guiton, La Rochelle, France Jean-Luc Bigot
- Annecy Hospital, France Gilles Genin

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 464 Date 2006-09-18

Discussion

No. of Applicants : 38