roentgenograms of the month A Rapidly Expanding Extrapulmonary Mass· Neil J Ducal. .\1.0., F.C.C .P.
(Chest 1992; 101:1425-26)
A 24-year-old Filipino man was noted on routine
chest roentgenogram to have a 5 X 2.5 Col left apical mass. In retrospect, the abnormality was present, measuring 3 X 1.5 em , on a roentgenogram obtained four months prior to presentation. He was referred for pulmonary evaluation, but he delayed two months before presenting. A roentgenogram obtained at the time of his evaluation revealed the mass to be 6.5 X 3 Col . The patient was asymptomatic, and the physical examination findings were unremarkable. The lungs were clear, no adenopathy was appreciated, and the neurologic findings ill the left upper extremity were normal. A PPD test was negative, and routine blood studies were unrevealing. A computed tomographic scan (Fig 1) revealed the mass to be well circumscribed, homogeneous, and of soft tissue density, with 110 invasion of local structures. Fine-needle aspiration of the mass was performed two weeks later, the mass having increased in size to i X i Col (Fig 2). This was complicated by a hemothorax. The aspirated specimen contained malignant cells, type undetermined . A thoracotomy was performed with removal of the mass, which was noted to be necrotic and hemorrhagic. The mass appeared to be adherent to the pleura, invaginating it from without. *Frnm tilt' Pulmonary Division. Portsmouth Naval Hospital. Portsmouth . Va. The opinions or assertions expressed herein art ' those of till' author and art' not to lit' construed as offidal or as reHpl'lin~ th e views of the Department of the Navy or the Dl'eartllll'nl of Defense. Reprinf requests: Dr. D,,()(/[. ,5-12-1 Rnxlkpl'ltl Drive, Vir/.:inia Beach, Virginia 2.3-16-1
CHEST I 101 I 5 I MAY, 1992
Diagnosis: Malignant fibrous tumor of the pleura Malignant fibrous tumors of the pleura are rare' and are considered to be of mesenchymal origin," arising from submesothelial connective tissue cells.v' They are distinguished from mesothelioma by the lack of association with asbestos exposure, the lack of a uniformly poor prognosis, and the absence of epithelial differentiation." The tumor can also occur outside mesothelium-lined cavities." The pathologic diagnosis remains elusive, with as many as 75 percent of the malignant tumors being initially misdiagnosed." Also, despite the presence of histologic features of malignancy, which include increased mitotic figures, pleomorphism, and high cellularity, the prognosis is more dependent upon the resectability of a given tumor than its histologic picture. Those tumors that are malignant yet easily resectable carry a favorable prognosis. The sexes appear to be equally affected," although some series note a slight female predominance." It appears in nearly all age groups, with the majority of patients presenting in the sixth and seventh decades. It is difficult to clinically separate the malignant from the benign variety The malignant tumors are symptomatic 75 percent of the time, compared with only 33 percent for the benign lesions." Hypoglycemia is an associated finding in 11 percent of the patients with malignant tumors and is three times more common in female than in male patients. Although hypoglycemia resolves with excision, insulinlike activity has not been demonstrated in conjunction with this tumor," Pleural effusion may be present in both benign and malignant varieties of the disease, although it is significantly more common with the malignant form. Cytologic examination of the pleural fluid is usually nondiagnostic. Hemoptysis, while not a sensitive finding, appears to be specific for the presence of malignant disease. Other reported symptoms-weight loss,
dyspnea on exertion, chest pain, and night sweatsappear to have little utility in differentiating benign from malignant disease. These tumors can arise from either the visceral or the parietal pleura, although the former is more common. On gross examination, tumors greater than 10 em tend to be malignant, while those on pedunculated stalks tend to be benign. There is significant overlap in these categories, however," Malignant tumors often show foci of recent necrosis and hemorrhage,:' A review has shown that all of the benign lesions and 45 percent of malignant lesions were curable with simple excision." The unresectable tumors invade locally but do not become diffuse and only rarely metastasize." Metastases have been reported in contralateral pleura, liver, adrenal gland, small intestine, bone, myocardium, skin, and stomach." The prognosis with unresectable tumors is uniformly poor. Most patients will have a rapid demise, with death usually occurring within two years and certainly within five years. 5 REFERENCES 1 Okike N, Bernatz PE, \Voolner LB. Localized mesothelioma of the pleura. JThorac Cardiovasc Surg 1978; 75:363-72 2 El-Naggar A, Ward RM, Ro IY. Fibrous tumor with hemangiopericytic pattern, so-called "localized fibrous tumor of pleura." Lab Invest 1987; 56:21A 3 Bolen l\~ Tumors of serosal tissue origin. Clin Lab Med 1987; 7:31-50 \\TE. Localized primary 4 Dalton WT, Zolliker AS, McCau~hey tumors of the pleura: an analysis of 40 cases. Cancer 1979; 44:1465-75 5 England D M, Ilochholzer L, McCarthy MJ. Localized benign and malignant fibrous tumors of the pleura: a clinicopathologic review of 223 cases. Am J Surg Pathol 1989; 13:640-58 6 Briselli M, Mark EI, Dickersin GR. Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature. Cancer 1981; 47:2678-89
Roentgenogram of the Month (Neil J. Duval)