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Weekly Chest CasesArchive of Old Cases

Case No : 43 Date 1998-08-24

  • Courtesy of Jin-Hwan Kim, M.D. / Chungnam National University Hospital
  • Age/Sex 64 / F
  • Chief Complaintright chest discomfort, dizziness, and cold sweating before meals. The breathing sound was decreased in the right middle and lower lung zones. Her FBS were 17 - 23 mg/dl.
  • Figure 1
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  • Figure 4

Chest PA I

Diagnosis With Brief Discussion

Diagnosis
Localized fibrous tumor of the pleura with low malignant potential
Radiologic Findings
Chest radiograph shows homogenous opacity occupying lower half of the right hemithorax. The shape of the mass was altered examination to examination. On right lateral decubitus image, the mass moved laterally. CT scans demonstrated a soft tissue density mass with moderate heterogenous enhancement and feeding vessels after intravenous contrast material injection. The right lower lobe was compressed by the tumor. On T1-weighted MR images, the mass had an intermediate and low heterogeneous signal. On T2-weighted images, the mass had low central signal and high peripheral signal intensity. At thoracotomy, encapsulated mass of the pleura weighing 1200g was removed. The tumor was attached to the visceral pleura of the left lower lobe by a single stalk. Hypogycemia was corrected after surgery. Sections through the mass showed a white-gray fibrous appearance and necrosis. Histologically, the lesion was localized malignant fibrous tumor of the pleura.
Brief Review
The term "localized benign (or malignant) fibrous tumor of the pleura (LFTP)" is preferred because histologically these tumors do not contain epithelial cells but derive from a submesothelial mesenchymal cell with fibroblastic differentiation. LFTP accounts for fewer than 5% of pleural tumors and these tumors are unrelated to asbestos exposure. Approximately 70 to 80% of these tumors arise from visceral pleura, with the remainder arising from the parietal pleura. A small number are intrapulmonary in location. Most are pedunclated. Large areas of hemorrhage or necrosis are more suggestive of malignant behavior. In Briselli and colleague's review, the best predictor of a good prognosis was the presence of pedicle. Clinically, LFTP occurs in both sexes and in all age groups but predominantly affects persons more than 50 years. Approximately 50% of patients are asymmtomatic, and 40% of symptomatic patients complain chest pain, cough and dyspnea. Extrathoracic manifestations include hypertrophic osteoarthropathy in 4 - 35% and hypoglycemia. Hypoglycemia, noted in less than 5% of patients, is usually associated with very large tumor and is thought to be due to glucose consumption by the mass.
Radiologically, a small to medium-sized tumor appears as a solitary, sharply delineated, often lobulated nodule or mass of variable size from 1 to 39 cm, and forms obtuse angle with the chest wall. Large tumors can appear as opacifications of a portion of one hemithorax, and often forms acute angle with the chest wall as the mass is pedunculated or becomes larger than the pleural origin. LFTP exist in the costal compartment of pleura, within an interlobar fissure, along pleural surface of mediastinum, along the diaphragmatic pleura and rarely within the lung parenchyma. These tumor predominate in the middle and lower half of the chest. A pedunculated LFTP can change postion and appearance with respiration or with a change in position. Calcification have been reported in 7% of tumors. Pleural effusion was present in 8 - 17% of cases but is more likely to be associated with malignant tumors than the benign. On unenhanced CT scans, LFTPs are well-delineated, often lobulated soft-tissue attenuation mass. Calcifications are noted in large tumors and related to the areas of necrosis. The tumors have rich vascularization and shows intense and homogeneous enhancement. Nonenhancing areas on CT scans correspond to necrosis, myxoid degenration, or hemorrhage within the tumor. Identification of pedicle is a clue to the diagnosis. CT findings that suggest a malignant fibrous tumor include a diameter larger than 10 cm, central necrosis, and ipsilateral pleural effusion. MRI is superior to CT for the morphology of a tumor, the relationships of the tumor to adjacent structures and, characterization of tissue. Tumors are intermediate to low signal intensity on T1-weighted images and of low signal intensity on proton density-weighted and T2-weighted images. These findings relate to hypocellularity and abundant collagen stroma. Areas of high signal intensity on T2-weighted images are related to areas of necrosis or myxoid degeneration. Intense enhancement has been reported after injection of gadolinium. Recent fibrosis and malignant fibrosis show low signal intensity on T1-weighted images and high signal intensity on T2-weighted images because of increased vascularity, edema, and increased cellularity. Contrast enhancement is also reported. Falaschi et al reported that mesothelioma, metastasis, and non-Hodgkin's lymphoma had high signal intensity on proton-density weighted images and T2-weighted images. Moreover benign lesions revealed low signal intensity on T2-weighted images.
References
1. Ferretti GR, Chiles C, Choplin RH, Coulomb M. Localized benign fibrous tumor of the pleura. AJR 1997;169:683-686
2. England DM, Hochholzer L, Mccarthy MJ. Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases. Am J Surg Pathol 1989;13:640-658.
3. Falaschi F, Battolla L, Mascalchi M, et al. Usefulness of MR signal intensity in distinguishing benign from malignant pleural disease. AJR 1996;166:963-968.
4. Ferretti GR, Chiles C, Cox JE, Choplin RH, Coulomb M. Localized benign fibrous tumors of the pleura: MR appearance. JCAT 1997;21(1):115-120.
5. Muller NL. Imaging of the pleura. Radiology 1993;186:297-309.
6. Kuhlman JE, Singha NK. Complex disease of the pleural space: radiographic and CT evaluation. Radiographics 1997;17:63-79.
7. Desser TS, Stark P. Pictorial essay: Solitary Fibrous Tumor of the Pleura. Journal of Thoracic Imaging 1998;13:27-35
Keywords
Pleura, Benign tumor,

No. of Applicants : 30

▶ Correct Answer : 24/30,  80.0%
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