847
0 1992
The Japanese Society of Pathology
Pleura I Ma ligna nt Fibrous Histiocy to ma Concomitant with Pulmona ry Adenoca rcinoma
Kazuki Nabeshimal, Teruhiko Inoue', Yoshihisa Tanaka2, and Masashi Koono'
A rare autopsy case, in which pleural malignant fibrous histiocytoma (MFH) and peripheral pulmonary adenocarcinoma were present concurrently in the right thorax, is described. Clinically, only a pleural mass was detected because of massive pleural effusion. Since cytologic examination of the effusion showed only adenocarcinoma cells, the pleural mass was considered to be enlarged mediastinal lymph nodes due t o metastasis of adenocarcinoma. Histopathologically, the pleural mass showed the features of a common type of MFH, accompanied by metastatic adenocarcinoma cells in the pleural lymphatics. No mixture of MFH and adenocarcinoma cells was present. lmmunohistochemicaIly, the MFH lesion showed positive staining for alpha-1-antitrypsin, alpha-1-chymotrypsin, and factor Xllla, but no reactivity for cytokeratins. The adenocarcinoma lesion showed positive staining for carcinoembryonic antigen (CEA), and contained hyaluronidase-resistant mucin. To our knowledge, this is the second reported case of pleural MFH with pulmonary adenocarcinoma. Acta Pathol Jpn 42 : 847-851, 1992. Key words : Pleural MFH, Pulmonary adenocarcinoma, Dou-
ble malignant tumors
Malignant fibrous histiocytoma (MFH) is now considered the most common soft tissue sarcoma of late adulthood, and occurs frequently in the extremities, trunk and peritoneum (1, 2). However, primary pleural MFH is rare, only five cases having been reported so far (3-7). Among these five cases, one was accompanied by pulmonary adenocarcinoma in the same hemithorax (7). Here we report an additional case together with a summary of Received April 3, 1992. Accepted for publication August 3, 1992. 'Second Department of Pathology and *Department of Radiology, Miyazaki Medical College, Miyazaki. Mailing address : Masashi Koono, Second Department of Pathology, Miyazaki Medical College, 5200 Kihara, Kiyotake, Miyazaki 889-16, Japan.
the clinicopathological features of pleural MFH reported previo us1y.
CLINICAL SUMMARY A 63-year-old man consulted his home doctor because of general malaise and dyspnea on May 31, 1990. Chest X-ray revealed massive right pleural effusion. Cytologic studies of the pleural effusion showed adenocarcinoma cells. He was admitted to Miyazaki Medical College Hospital on June 14, 1990. On admission, the right chest showed impaired transmission of breath sounds. Laboratory studies showed mild hypoproteinemia (6.29 g/dl) and a slight increase in the level of serum carcinoembryonic antigen (CEA) (6.8 ng/ml). As for the intrapulmonary lesion, no further information could be obtained because of the massive pleural effusion. After partial removal of the pleural fluid, a nodular mass appeared in the medial upper portion of the right thorax (Fig. l),which was then thought to be an enlarged mediastinal lymph node due to metastasis of adenocarcinoma. Although the primary tumor remained obscure radio logically, c hem0t hera py (CDD P and VDS), hy perthermia therapy, adjuvant chemotherapy, and radiation to the right mediastinum were performed. In spite of the combined therapies, the pleural effusion was difficult to control. The patient's respiratory status gradually deteriorated, accompanied b y episodes of left pneumothorax and pneumonia. He died of respiratory insufficiency on November 10, 1990.
PATHOLOGICAL FINDINGS At autopsy, there was about 220 ml and 300 ml of bloody pleural effusion in the left and right pleural cavities, respectively. The left side also showed pneumothorax. The whole right lung had collapsed and showed dense adhesions predominantly on the posterior
848
Pleural MFH and Pulmonary Adenocarcinoma (Nabeshima et a/.)
Figurel. CT of the thorax at soft tissue windows. I n the right posteromedial hemithorax the scan shows a non calcified and non homogeneous mass
Figure 2. Cut surface of the right lung and the rnediastinum The upper medial parietal pleura shows a nodular mass of MFH (arrow). The lower lobe has a small nodular lesion of adenocarcinoma (arrow head) as well as small metastatic tumors
Figure 3. a : Spindle-shaped tumor cells of MFH are arraged in fascicles in a storiform pattern, mixed with giant cells. HE. b : A higher-magnification view of MFH tumor cells. HE. (inset) The tumor cells show positive immunostaining for alpha-l -antitrypsin.
and lateral surfaces. There were two different isolated tumors in the right thorax; one was a sessile mass attached directly to the parietal pleural wall by a broad base just above the hilus of the right lung, and the other was an intrapulmonary nodule in the medial basal portion of the right lung (Fig. 2). The pleural mass was round and grayish with small areas of necrosis and hemorrhage, and measured 3 c m in maximum dimension. It had expanded with bidirectional erosion into the lung parenchyma and the mediastinal soft tissue. The intrapulmonary nodule was whitish, measured 1.5 cm in maximum dimension, and adhered tightly to the parietal
pleura. Microscopically, the right upper pleural tumor consisted predominantly of proliferated atypical spindleshaped cells mixed with plump or multinucleated bizarre giant tumor cells. The spindle-shaped tumor cells showed a storiform arrangement (Figs. 3a, b). The tumor cell nuclei were moderately bizarre and hyperchromatic. High mitotic activity and necrotic foci were also present in parts. lmmunohistochemically, the tumor cells showed diffuse, sometimes intense intracytoplasmic staining for alpha-1-antitrypsin (Fig. 3b, inset) and alpha-1 -antichymotrypsin, and focal staining for vimentin and factor Xllla, but were negative for desmin,
849
Acta Pathologica Japonica 42 (1 1) : 1992
Figure 4. a : Adenocarcinoma cells proliferate in a nodular pattern. Tubular structures are present in the nodules. HE. b : A higher-magnification view of adenocarcinoma cells. HE. (inset) The carcinoma cells show intense immunostaining for CEA. c : Adenocarcinoma cells invade the parietal pleura accompanied by desrnoplastic reaction. HE.
$100 protein and cytokeratins. The right lower lobe tumor showed nodular proliferation of atypical cuboidal or low columnar cells with hyperchromatic nuclei. The cells formed tubules or were lined up along the alveolar walls (Figs. 4a, b). The pleura adhered to the surface of the tumor, and tumor cells invaded the parietal pleura accompanied by desmoplastic reaction (Fig. 4c). Alcian blue staining focally demonstrated intracytoplasmic positive mucin staining, which remained positive even after hyaluronidase predigestion. In addition, the tumor cells showed intense immunostaining for CEA (Fig. 4b, inset) and cytokeratins. This adenocarcinoma showed widespread metastases and involved the pleural lymphatics which were present at the periphery of the MFH mass (Figs. 5a, b). However, adenocarcinoma cells remained in the lymphatics and no mixture of adenocarcinoma and
MFH cells was observed.
DISCUSSION MFH was first described in the early 1960s by OBrien and Stout (8). Although its exact histogenesis still remains unclear, it is thought to be a primitive mesenchymal tumor showing partial fibroblastic and histiocytic differentiation (1, 2, 9). The classic form of MFH, which was described as a storiform-pleomorphic subtype by Enzinger and Weiss (10) o r a common type by Enjoji et a/. (2), is characterized by a storiform fascicular or pleomorphic growth pattern, and the presence of fibroblast- and histiocyte-like cells, mixed with scattered bizzare multinucleated giant cells. The microscopic features of the pleural MFH described here were those of the
850
Pleural MFH and Pulmonary Adenocarcinoma (Nabeshima et a/.)
Figure 5. a, b : At the periphery of the MFH mass, metastatic adenocarcinoma cells are present in the lymphatics. HE.
classic form. In this case, pleural MFH and peripheral pulmonary adenocarcinoma were present concurrently in the same hemithorax. Since adenocarcinoma had invaded the parietal pleura with desmoplastic reaction and metastasized to the pleural lymphatics at the periphery of the MFH mass, malignant mesothelioma and sarcomatoid carcinoma of the lung were included in the differential diagnosis. However, at the periphery of the MFH mass, adenocarcinoma cells remained in the lymphatics and were not intermingled with MFH cells. In addition, an intrathoracic mass attached to the parietal pleura by a broad base suggested a neoplasm of pleural origin rather than sarcomatoid carcinoma of the lung. Immunohistochemically, the cells in the MFH lesion showed diffuse reactivity for alpha-1 -antitrypsin and alpha-1 -antichymotrypsin as well as focal reactivity for factor Xllla, but were negative for cytokeratins. Cytokeratins are known to be frequently present in mesothelioma cells (11) and spindle cells in sarcomatoid carcinoma (12). Although alpha-1-antitrypsin and alpha-1 -antichymotrypsin are not specific for MFH cells (13), simultaneous demonstration of factor Xllla (14) and the lack of cytokeratins (11, 12) favor a diagnosis of MFH. On the other hand, the cells in the adenocarcinoma lesions were diffusely and intensely immunoreactive for CEA and also had intracytoplasmic hyaluronidase-resistant mucin. Mesothelioma cells are known to be usually negative for CEA and hyaluronidase-resistant mucin (11). Thus, although the results of the antibody studies are not specific for a diagnosis of MFH, when taken in conjunction with the gross and microscopical features of the tumor, they do support the diagnosis.
Another entity included in the differential diagnosis is localized malignant fibrous tumor of the pleura. Although there is disagreement regarding its histogenesis, i.e. mesothelial versus submesothelial, reports of localized fibrous tumor of the pleura have appeared increasingly in the recent literature (15, 16). The majority of these tumors show a patternless or hemangiopericytic pattern microscopically, with infrequent giant cells and moderate to markedly positive immunostaining for vimentin. However, our present tumor showed a storiform cell arrangement with pleomorphism and frequent bizarre giant cells. Together with the immunostaining results described above, these findings favor the diagnosis of MFH. Primary pleural MFH is rare, and only five cases (3-7) have been reported previously. Including our case, the ages of the 6 patients ranged from 5 1 to 86 years with a median age of 66 years. There was no predilectional site of tumor involvement in the thorax. Two cases showed circumferential tumor thickening of the pleura, whereas the other four cases were localized pleural tumors averaging 6.8 c m in maximum dimension. In three of the 6 cases, MFH was aggressive, causing death with widespread metastases within a short period (median survival time, 8.2 months) after the discovery of the tumor, although no background history was available in these aggressive cases. In two other cases, low aggressiveness of the primary pleural MFH was suggested (5,6) because there was a long background history of the tumor, 2 3 and 1 2 years respectively. However, in one of these two less aggressive cases, the patient died due to the tumor about three months after development of tumor-associated symptoms. There was no correla-
Acta Pathologica Japonica 42 (11) : 1992 tion between histological subtype and growth behavior, since all six cases showed the microscopic features of the common (storiform-pleomorphic) type of MFH. Including our present case, two of the six cases of pleural MFH were accompanied by pulmonary adenocarcinoma. Although the number of cases is not sufficient to allow a firm conclusion to be drawn, and no apparent etiological connection has been found between these tumors, this coincidence appears to be extremely high. Other tumors reported to be associated with MFH include breast carcinoma and multiple neurofibromatosis (17, 18). In the present case, as the pleural effusion contained only adenocarcinoma cells, the presence of MFH was overlooked. In addition, MFH cells were detected in the pleural fluid in only one case, although five of the six cases showed pleural effusion. Therefore, in the cases of pleural mass or diffuse pleural thickening, it seems worthwhile to include MFH in the differential diagnosis even if no MFH cells are present in the pleural effusion. Acknowledgements : The authors thank Mr. S. Sat0 for his skiilful technical assistance in immunohistochemistry, and Mr. T. Miyamoto for photographic assistance.
6. 7.
8. 9.
10.
11.
12. 13. 14.
REFERENCES 1. Weiss SW and Enzinger FM. Malignant fibrous histiocytoma: An analysis of 200 cases. Cancer 41 : 2250-2266, 1978. 2. Enjoji M, Hashimoto H, Tsuneyoshi M, and lwasaki H. Malignant fibrous histiocytoma : A clinicopathologic study of 130 cases. Acta Pathol Jpn 3 0 : 727-741, 1980. 3. Nishimura T, Fujiyoshi Y, Nagayama K, Tanaka T, and Tanimura A. A case of malignant fibrous histiocytoma of the pleura. Kurume Med J 32 : 223-227, 1985. 4. Okada R, Ito M, Kitagawa G, et a/. A case of malignant fibrous histiocytoma of the pleura. Nippon Kyobu Rinsho 43: 248-252, 1984 (in Japanese). 5. Sforza V, del Vecchio MT, Gotti G, Tucci E, and Santopietro R. Primary malignant fibrous histio-
15.
16.
17. 18.
85 1
cytoma of the pleura : A case report. Appl Pathol 4 : 162-169, 1986. Yang HY, Weaver LL, and Foti PR. Primary malignant fibrous histiocytoma of the pleura: A case report. Acta Cytol 27 : 683-687, 1983. Hiasa Y, Shimoyama T, Kitahori Y, et a/. Malignant fibrous histiocytoma with widespread metastasis and pulmonary cancer. Acta Pathol Jpn 36:113-122, 1986. OBrien JE and Stout AP. Malignant fibrous xanthomas. Cancer 17: 1445-1455, 1964. Tsuneyoshi M, Enjoji M, and Shinohara N. Malignant fibrous histiocytoma : An electron microscopic study of 17 cases. Virchows Arch [Pathol Anat] 392: 135-145, 1981. Enzinger FM and Weiss SW. Malignant fibrohistiocytic tumors. In Enzinger FM and Weiss SW, eds. Soft Tissue Tumors (2nd ed). Mosby, St. Louis, 1988 : 269-300. Otis CN, Carter D, Cole S, and Battifora H. Immunohistochemical evaluation of pleural mesothelioma and pulmonary adenocarcinoma : A bi-institutional study of 47 cases. Am J Surg Pathol 11 : 445-456, 1987. Ro JY, Chen JL, Lee JS, et a/. Sarcomatoid carcinoma of the lung : lmmunohistochemical and ultrastructural studies of 14 cases. Cancer 69: 376-386, 1992. Soini Y and Miettinen M. Alpha-1-antitrypsin and lysozyrne : Their limited significance in fibrohistiocytic tumors. Am J Clin Pathol 91 : 515-521, 1989. Nemes Z and Thomazy V. Factor Xllla and the classic histiocytic markers in malignant fibrous histiocytoma : A comparative immunohistochemical study. Hum Pathol 19: 822-829, 1988. Briselli M,Mark EJ, and Dickersin GR. Solitary fibrous tumors of the pleura: Eight new cases and review of 360 cases in the literature. Cancer 47 : 2678-2689, 1981. England DM, Hochholzer L, and McCarthy MJ. Localized benign and malignant fibrous tumors of the pleura : A clinicopathologic review of 223 cases. Am J Surg Pathol 13: 640-658, 1989. Tsuneyoshi M and Enjoji M. Postirradiation sarcoma (malignant fibrous histiocytoma) following breast carcinoma. Cancer 45: 1419-1423, 1980. Johnson PS, Katz DA, and Penn R. Malignant fibrous histiocytoma arising in a patient with multiple neurofibromatosis: A case report and a literature review. J Surg Oncol 12: 97-105, 1979.
0 1992
The Japanese Society of Pathology
Pleura I Ma ligna nt Fibrous Histiocy to ma Concomitant with Pulmona ry Adenoca rcinoma
Kazuki Nabeshimal, Teruhiko Inoue', Yoshihisa Tanaka2, and Masashi Koono'
A rare autopsy case, in which pleural malignant fibrous histiocytoma (MFH) and peripheral pulmonary adenocarcinoma were present concurrently in the right thorax, is described. Clinically, only a pleural mass was detected because of massive pleural effusion. Since cytologic examination of the effusion showed only adenocarcinoma cells, the pleural mass was considered to be enlarged mediastinal lymph nodes due t o metastasis of adenocarcinoma. Histopathologically, the pleural mass showed the features of a common type of MFH, accompanied by metastatic adenocarcinoma cells in the pleural lymphatics. No mixture of MFH and adenocarcinoma cells was present. lmmunohistochemicaIly, the MFH lesion showed positive staining for alpha-1-antitrypsin, alpha-1-chymotrypsin, and factor Xllla, but no reactivity for cytokeratins. The adenocarcinoma lesion showed positive staining for carcinoembryonic antigen (CEA), and contained hyaluronidase-resistant mucin. To our knowledge, this is the second reported case of pleural MFH with pulmonary adenocarcinoma. Acta Pathol Jpn 42 : 847-851, 1992. Key words : Pleural MFH, Pulmonary adenocarcinoma, Dou-
ble malignant tumors
Malignant fibrous histiocytoma (MFH) is now considered the most common soft tissue sarcoma of late adulthood, and occurs frequently in the extremities, trunk and peritoneum (1, 2). However, primary pleural MFH is rare, only five cases having been reported so far (3-7). Among these five cases, one was accompanied by pulmonary adenocarcinoma in the same hemithorax (7). Here we report an additional case together with a summary of Received April 3, 1992. Accepted for publication August 3, 1992. 'Second Department of Pathology and *Department of Radiology, Miyazaki Medical College, Miyazaki. Mailing address : Masashi Koono, Second Department of Pathology, Miyazaki Medical College, 5200 Kihara, Kiyotake, Miyazaki 889-16, Japan.
the clinicopathological features of pleural MFH reported previo us1y.
CLINICAL SUMMARY A 63-year-old man consulted his home doctor because of general malaise and dyspnea on May 31, 1990. Chest X-ray revealed massive right pleural effusion. Cytologic studies of the pleural effusion showed adenocarcinoma cells. He was admitted to Miyazaki Medical College Hospital on June 14, 1990. On admission, the right chest showed impaired transmission of breath sounds. Laboratory studies showed mild hypoproteinemia (6.29 g/dl) and a slight increase in the level of serum carcinoembryonic antigen (CEA) (6.8 ng/ml). As for the intrapulmonary lesion, no further information could be obtained because of the massive pleural effusion. After partial removal of the pleural fluid, a nodular mass appeared in the medial upper portion of the right thorax (Fig. l),which was then thought to be an enlarged mediastinal lymph node due to metastasis of adenocarcinoma. Although the primary tumor remained obscure radio logically, c hem0t hera py (CDD P and VDS), hy perthermia therapy, adjuvant chemotherapy, and radiation to the right mediastinum were performed. In spite of the combined therapies, the pleural effusion was difficult to control. The patient's respiratory status gradually deteriorated, accompanied b y episodes of left pneumothorax and pneumonia. He died of respiratory insufficiency on November 10, 1990.
PATHOLOGICAL FINDINGS At autopsy, there was about 220 ml and 300 ml of bloody pleural effusion in the left and right pleural cavities, respectively. The left side also showed pneumothorax. The whole right lung had collapsed and showed dense adhesions predominantly on the posterior
848
Pleural MFH and Pulmonary Adenocarcinoma (Nabeshima et a/.)
Figurel. CT of the thorax at soft tissue windows. I n the right posteromedial hemithorax the scan shows a non calcified and non homogeneous mass
Figure 2. Cut surface of the right lung and the rnediastinum The upper medial parietal pleura shows a nodular mass of MFH (arrow). The lower lobe has a small nodular lesion of adenocarcinoma (arrow head) as well as small metastatic tumors
Figure 3. a : Spindle-shaped tumor cells of MFH are arraged in fascicles in a storiform pattern, mixed with giant cells. HE. b : A higher-magnification view of MFH tumor cells. HE. (inset) The tumor cells show positive immunostaining for alpha-l -antitrypsin.
and lateral surfaces. There were two different isolated tumors in the right thorax; one was a sessile mass attached directly to the parietal pleural wall by a broad base just above the hilus of the right lung, and the other was an intrapulmonary nodule in the medial basal portion of the right lung (Fig. 2). The pleural mass was round and grayish with small areas of necrosis and hemorrhage, and measured 3 c m in maximum dimension. It had expanded with bidirectional erosion into the lung parenchyma and the mediastinal soft tissue. The intrapulmonary nodule was whitish, measured 1.5 cm in maximum dimension, and adhered tightly to the parietal
pleura. Microscopically, the right upper pleural tumor consisted predominantly of proliferated atypical spindleshaped cells mixed with plump or multinucleated bizarre giant tumor cells. The spindle-shaped tumor cells showed a storiform arrangement (Figs. 3a, b). The tumor cell nuclei were moderately bizarre and hyperchromatic. High mitotic activity and necrotic foci were also present in parts. lmmunohistochemically, the tumor cells showed diffuse, sometimes intense intracytoplasmic staining for alpha-1-antitrypsin (Fig. 3b, inset) and alpha-1 -antichymotrypsin, and focal staining for vimentin and factor Xllla, but were negative for desmin,
849
Acta Pathologica Japonica 42 (1 1) : 1992
Figure 4. a : Adenocarcinoma cells proliferate in a nodular pattern. Tubular structures are present in the nodules. HE. b : A higher-magnification view of adenocarcinoma cells. HE. (inset) The carcinoma cells show intense immunostaining for CEA. c : Adenocarcinoma cells invade the parietal pleura accompanied by desrnoplastic reaction. HE.
$100 protein and cytokeratins. The right lower lobe tumor showed nodular proliferation of atypical cuboidal or low columnar cells with hyperchromatic nuclei. The cells formed tubules or were lined up along the alveolar walls (Figs. 4a, b). The pleura adhered to the surface of the tumor, and tumor cells invaded the parietal pleura accompanied by desmoplastic reaction (Fig. 4c). Alcian blue staining focally demonstrated intracytoplasmic positive mucin staining, which remained positive even after hyaluronidase predigestion. In addition, the tumor cells showed intense immunostaining for CEA (Fig. 4b, inset) and cytokeratins. This adenocarcinoma showed widespread metastases and involved the pleural lymphatics which were present at the periphery of the MFH mass (Figs. 5a, b). However, adenocarcinoma cells remained in the lymphatics and no mixture of adenocarcinoma and
MFH cells was observed.
DISCUSSION MFH was first described in the early 1960s by OBrien and Stout (8). Although its exact histogenesis still remains unclear, it is thought to be a primitive mesenchymal tumor showing partial fibroblastic and histiocytic differentiation (1, 2, 9). The classic form of MFH, which was described as a storiform-pleomorphic subtype by Enzinger and Weiss (10) o r a common type by Enjoji et a/. (2), is characterized by a storiform fascicular or pleomorphic growth pattern, and the presence of fibroblast- and histiocyte-like cells, mixed with scattered bizzare multinucleated giant cells. The microscopic features of the pleural MFH described here were those of the
850
Pleural MFH and Pulmonary Adenocarcinoma (Nabeshima et a/.)
Figure 5. a, b : At the periphery of the MFH mass, metastatic adenocarcinoma cells are present in the lymphatics. HE.
classic form. In this case, pleural MFH and peripheral pulmonary adenocarcinoma were present concurrently in the same hemithorax. Since adenocarcinoma had invaded the parietal pleura with desmoplastic reaction and metastasized to the pleural lymphatics at the periphery of the MFH mass, malignant mesothelioma and sarcomatoid carcinoma of the lung were included in the differential diagnosis. However, at the periphery of the MFH mass, adenocarcinoma cells remained in the lymphatics and were not intermingled with MFH cells. In addition, an intrathoracic mass attached to the parietal pleura by a broad base suggested a neoplasm of pleural origin rather than sarcomatoid carcinoma of the lung. Immunohistochemically, the cells in the MFH lesion showed diffuse reactivity for alpha-1 -antitrypsin and alpha-1 -antichymotrypsin as well as focal reactivity for factor Xllla, but were negative for cytokeratins. Cytokeratins are known to be frequently present in mesothelioma cells (11) and spindle cells in sarcomatoid carcinoma (12). Although alpha-1-antitrypsin and alpha-1 -antichymotrypsin are not specific for MFH cells (13), simultaneous demonstration of factor Xllla (14) and the lack of cytokeratins (11, 12) favor a diagnosis of MFH. On the other hand, the cells in the adenocarcinoma lesions were diffusely and intensely immunoreactive for CEA and also had intracytoplasmic hyaluronidase-resistant mucin. Mesothelioma cells are known to be usually negative for CEA and hyaluronidase-resistant mucin (11). Thus, although the results of the antibody studies are not specific for a diagnosis of MFH, when taken in conjunction with the gross and microscopical features of the tumor, they do support the diagnosis.
Another entity included in the differential diagnosis is localized malignant fibrous tumor of the pleura. Although there is disagreement regarding its histogenesis, i.e. mesothelial versus submesothelial, reports of localized fibrous tumor of the pleura have appeared increasingly in the recent literature (15, 16). The majority of these tumors show a patternless or hemangiopericytic pattern microscopically, with infrequent giant cells and moderate to markedly positive immunostaining for vimentin. However, our present tumor showed a storiform cell arrangement with pleomorphism and frequent bizarre giant cells. Together with the immunostaining results described above, these findings favor the diagnosis of MFH. Primary pleural MFH is rare, and only five cases (3-7) have been reported previously. Including our case, the ages of the 6 patients ranged from 5 1 to 86 years with a median age of 66 years. There was no predilectional site of tumor involvement in the thorax. Two cases showed circumferential tumor thickening of the pleura, whereas the other four cases were localized pleural tumors averaging 6.8 c m in maximum dimension. In three of the 6 cases, MFH was aggressive, causing death with widespread metastases within a short period (median survival time, 8.2 months) after the discovery of the tumor, although no background history was available in these aggressive cases. In two other cases, low aggressiveness of the primary pleural MFH was suggested (5,6) because there was a long background history of the tumor, 2 3 and 1 2 years respectively. However, in one of these two less aggressive cases, the patient died due to the tumor about three months after development of tumor-associated symptoms. There was no correla-
Acta Pathologica Japonica 42 (11) : 1992 tion between histological subtype and growth behavior, since all six cases showed the microscopic features of the common (storiform-pleomorphic) type of MFH. Including our present case, two of the six cases of pleural MFH were accompanied by pulmonary adenocarcinoma. Although the number of cases is not sufficient to allow a firm conclusion to be drawn, and no apparent etiological connection has been found between these tumors, this coincidence appears to be extremely high. Other tumors reported to be associated with MFH include breast carcinoma and multiple neurofibromatosis (17, 18). In the present case, as the pleural effusion contained only adenocarcinoma cells, the presence of MFH was overlooked. In addition, MFH cells were detected in the pleural fluid in only one case, although five of the six cases showed pleural effusion. Therefore, in the cases of pleural mass or diffuse pleural thickening, it seems worthwhile to include MFH in the differential diagnosis even if no MFH cells are present in the pleural effusion. Acknowledgements : The authors thank Mr. S. Sat0 for his skiilful technical assistance in immunohistochemistry, and Mr. T. Miyamoto for photographic assistance.
6. 7.
8. 9.
10.
11.
12. 13. 14.
REFERENCES 1. Weiss SW and Enzinger FM. Malignant fibrous histiocytoma: An analysis of 200 cases. Cancer 41 : 2250-2266, 1978. 2. Enjoji M, Hashimoto H, Tsuneyoshi M, and lwasaki H. Malignant fibrous histiocytoma : A clinicopathologic study of 130 cases. Acta Pathol Jpn 3 0 : 727-741, 1980. 3. Nishimura T, Fujiyoshi Y, Nagayama K, Tanaka T, and Tanimura A. A case of malignant fibrous histiocytoma of the pleura. Kurume Med J 32 : 223-227, 1985. 4. Okada R, Ito M, Kitagawa G, et a/. A case of malignant fibrous histiocytoma of the pleura. Nippon Kyobu Rinsho 43: 248-252, 1984 (in Japanese). 5. Sforza V, del Vecchio MT, Gotti G, Tucci E, and Santopietro R. Primary malignant fibrous histio-
15.
16.
17. 18.
85 1
cytoma of the pleura : A case report. Appl Pathol 4 : 162-169, 1986. Yang HY, Weaver LL, and Foti PR. Primary malignant fibrous histiocytoma of the pleura: A case report. Acta Cytol 27 : 683-687, 1983. Hiasa Y, Shimoyama T, Kitahori Y, et a/. Malignant fibrous histiocytoma with widespread metastasis and pulmonary cancer. Acta Pathol Jpn 36:113-122, 1986. OBrien JE and Stout AP. Malignant fibrous xanthomas. Cancer 17: 1445-1455, 1964. Tsuneyoshi M, Enjoji M, and Shinohara N. Malignant fibrous histiocytoma : An electron microscopic study of 17 cases. Virchows Arch [Pathol Anat] 392: 135-145, 1981. Enzinger FM and Weiss SW. Malignant fibrohistiocytic tumors. In Enzinger FM and Weiss SW, eds. Soft Tissue Tumors (2nd ed). Mosby, St. Louis, 1988 : 269-300. Otis CN, Carter D, Cole S, and Battifora H. Immunohistochemical evaluation of pleural mesothelioma and pulmonary adenocarcinoma : A bi-institutional study of 47 cases. Am J Surg Pathol 11 : 445-456, 1987. Ro JY, Chen JL, Lee JS, et a/. Sarcomatoid carcinoma of the lung : lmmunohistochemical and ultrastructural studies of 14 cases. Cancer 69: 376-386, 1992. Soini Y and Miettinen M. Alpha-1-antitrypsin and lysozyrne : Their limited significance in fibrohistiocytic tumors. Am J Clin Pathol 91 : 515-521, 1989. Nemes Z and Thomazy V. Factor Xllla and the classic histiocytic markers in malignant fibrous histiocytoma : A comparative immunohistochemical study. Hum Pathol 19: 822-829, 1988. Briselli M,Mark EJ, and Dickersin GR. Solitary fibrous tumors of the pleura: Eight new cases and review of 360 cases in the literature. Cancer 47 : 2678-2689, 1981. England DM, Hochholzer L, and McCarthy MJ. Localized benign and malignant fibrous tumors of the pleura : A clinicopathologic review of 223 cases. Am J Surg Pathol 13: 640-658, 1989. Tsuneyoshi M and Enjoji M. Postirradiation sarcoma (malignant fibrous histiocytoma) following breast carcinoma. Cancer 45: 1419-1423, 1980. Johnson PS, Katz DA, and Penn R. Malignant fibrous histiocytoma arising in a patient with multiple neurofibromatosis: A case report and a literature review. J Surg Oncol 12: 97-105, 1979.
