444
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M.D., JR., SSCP.R HARRY~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~Chairman~~~~~~~~~~~~~. and .so it rofessor, Deprten of .Rad..iolo.gy. Howard ahigtn DC ...!rst
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The case presented is a 71 year old black male who complained of a two week history of productive cough following a two to three day episode of flu symptoms. Significant past history included an automobile accident in 1953 in which multiple pelvic and left rib fractures, and a penetrating injury to the left hemi-diaphragm were incurred. Physical findings were unremarkable except for decreased breath sounds at the left base. The admission chest radiographs are shown in Fig. 1. What is your diagnosis?
ing inferior margin which appears continuous with the mediastinal line (Fig. 2). These findings are most consistent with an extrapleural location. The lesion is well defined above the clavicle, which indicates its posterior and intrathoracic location (cervico-thoracic sign).1 There is no evidence of intralesional calcification or cavitation, and no obvious rib or vertebral body de-
. . .s. . ... . . FIG 1 Admission PA and Lateral Chest Radiographs left &
right respectively.
RADIOGRAPHIC FINDINGS
The posterior-anterior and lateral chest radiographs demonstrate a solitary homogeneous oval mass density projected in the left apical lungfield adjacent to and blending with the mediastinum. The lesion measures approximately 4 x 4 cms. and has well defined pencil sharp lateral, inferior and anterior margins and a taper-
FIG. 2. Magnified Frontal View of Mass. Note "Pencil Sharp" margins (Arrows).
struction. Multiple buckshot pellets are in the mid anterior chest wall. Elevation of the left hemi-diaphragm and calcification of the left hemi-diaphragmatic pleura
Vol. 68, No. 5
The
Unuisuial Mediastinal Mass
are incidental findings, which are related to the patient's previous automobile injury and resultant surgery. DISCUSSION
The common diagnostic consideration for a mediastinal mass in the absence of parenchymal involvement, are benign cysts, dermoid cysts, teratomas, thyroid and thymus tumors, tumors of neural origin, mesenchymal tumors and lymphomas. Compartmental localization is the most important feature in differential diagnosis of a mediastinal lesion. However, there is considerable overlap of diagnostic entities within the various mediastinal compartments and relatively few characteristic findings in most mediastinal lesions. A case in point is the case presented: A homogeneous posterior mediastinal mass without evidence of rib or spine involvement in a 71 year old asymptomatic man. Neuroenteric cysts, neurogenic tumors (neurofibromas, ganglioneuroma), mesenchymal tumors (lipoma, fibroma, myoma, etc.), and lymphoma (lymphosacoma, reticulum cell sarcoma) would be the usual diagnostic considerations. Other uncommon considerations might include chemodectomas, glomus tumor and extramedullary hematopoiesis.2 However, resection of the mass and subsequent pathological interpretation was consistent with plasmacytoma of the posterior mediastinum and left chest wall. Post operative work-up for multiple myeloma, including bone marrow aspiration, urine for Bence-Jones, protein and immunoelectrophoresis, was negative. It was uncertain from the pathology report whether or not the tumor represented a lymph node replaced by plasma cells. Extramedullary plasmacytomas are comparatively rare and several reports of cases and literature reviews indicate an even rarer incidence of intrathoracic involvement.3'5 They occur in a wide variety of organs and tissues; however, approximately 80-85% involve the upper respiratory tract and oral cavity.3 They are found predominantly in men and are most common in the fifth, sixth and seventh decades of life.' This age
445
and sex distribution is similar to that of multiple myeloma. Extramedullary plasmacytomas, although histologically very similar to multiple myeloma, do not share the same poor prognosis; however, regardless of site or origin, they have an unpredictable course.7 Four patterns can occur: 1) response to local treatment without recurrence or dissemination; 2) invasion of surrounding structures without further dissemination; 3) subsequent distant involvement of lymph nodes, bones and other organs; and 4) evolution to multiple myeloma. There is no way of predicting which pattern will develop in a given case. As in the case presented, abnormal laboratory findings are not, as a rule, seen with extramedullary plasmacytomas. However, the high incidence of evolution to multiple myeloma (approximately 30%)3 and recent reports of multiple myeloma presenting as an extraosseous tumor, emphasizes the need for careful initial search and long term follow-up to detect chemical and bony changes in all patients with extramedullary plasmacytomas. 8 LITERATURE CITED
1. FELSON, B. Chest Roentgenology. W. B. Saunders Co., 1973. 2. FRASER, R. G. and T. A. P. PARE. Diagnosis of Disease of The Chest., W. B. Saunders Co., 1970. 3. DOLIN, S. and J. P. DEWAR. Extramedullary Plasmacytoma. Amer. J. Path., 32:83, 1956. 4. KILBURN, K. and A. M. SCHMIDT. Intrathoracic Plasmacytoma. Arch. Int. Med., 106:862869, 1960. 5. K. WARE, S. G., et al. Extensive Ossification In A Pulmonary Plasmacytoma. Thorax, 30:206-210, 1965. 6. DINES, D. et al. Solitary Plasmacytoma of The Trachea. Am. Rev. Resp. Dis., 92:949-951, 1965. 7. CARSON, C.P., et al. Plasma Cell Myeloma. Am. J. Clin. Path., 25:849, 1955. 8. GUPTA, R. M., et al. Extramedullary Plasmacytoma Ig G Type I Presenting as Mediastinal Syndrome. Brit. J. Dis. Chest, 69:65-70, 1974.
I. .~ ~ ~.:_ _. . _ . =
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION
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SEPTEMBER, 1976
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M.D., JR., SSCP.R HARRY~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~Chairman~~~~~~~~~~~~~. and .so it rofessor, Deprten of .Rad..iolo.gy. Howard ahigtn DC ...!rst
::::,: ::
......
The case presented is a 71 year old black male who complained of a two week history of productive cough following a two to three day episode of flu symptoms. Significant past history included an automobile accident in 1953 in which multiple pelvic and left rib fractures, and a penetrating injury to the left hemi-diaphragm were incurred. Physical findings were unremarkable except for decreased breath sounds at the left base. The admission chest radiographs are shown in Fig. 1. What is your diagnosis?
ing inferior margin which appears continuous with the mediastinal line (Fig. 2). These findings are most consistent with an extrapleural location. The lesion is well defined above the clavicle, which indicates its posterior and intrathoracic location (cervico-thoracic sign).1 There is no evidence of intralesional calcification or cavitation, and no obvious rib or vertebral body de-
. . .s. . ... . . FIG 1 Admission PA and Lateral Chest Radiographs left &
right respectively.
RADIOGRAPHIC FINDINGS
The posterior-anterior and lateral chest radiographs demonstrate a solitary homogeneous oval mass density projected in the left apical lungfield adjacent to and blending with the mediastinum. The lesion measures approximately 4 x 4 cms. and has well defined pencil sharp lateral, inferior and anterior margins and a taper-
FIG. 2. Magnified Frontal View of Mass. Note "Pencil Sharp" margins (Arrows).
struction. Multiple buckshot pellets are in the mid anterior chest wall. Elevation of the left hemi-diaphragm and calcification of the left hemi-diaphragmatic pleura
Vol. 68, No. 5
The
Unuisuial Mediastinal Mass
are incidental findings, which are related to the patient's previous automobile injury and resultant surgery. DISCUSSION
The common diagnostic consideration for a mediastinal mass in the absence of parenchymal involvement, are benign cysts, dermoid cysts, teratomas, thyroid and thymus tumors, tumors of neural origin, mesenchymal tumors and lymphomas. Compartmental localization is the most important feature in differential diagnosis of a mediastinal lesion. However, there is considerable overlap of diagnostic entities within the various mediastinal compartments and relatively few characteristic findings in most mediastinal lesions. A case in point is the case presented: A homogeneous posterior mediastinal mass without evidence of rib or spine involvement in a 71 year old asymptomatic man. Neuroenteric cysts, neurogenic tumors (neurofibromas, ganglioneuroma), mesenchymal tumors (lipoma, fibroma, myoma, etc.), and lymphoma (lymphosacoma, reticulum cell sarcoma) would be the usual diagnostic considerations. Other uncommon considerations might include chemodectomas, glomus tumor and extramedullary hematopoiesis.2 However, resection of the mass and subsequent pathological interpretation was consistent with plasmacytoma of the posterior mediastinum and left chest wall. Post operative work-up for multiple myeloma, including bone marrow aspiration, urine for Bence-Jones, protein and immunoelectrophoresis, was negative. It was uncertain from the pathology report whether or not the tumor represented a lymph node replaced by plasma cells. Extramedullary plasmacytomas are comparatively rare and several reports of cases and literature reviews indicate an even rarer incidence of intrathoracic involvement.3'5 They occur in a wide variety of organs and tissues; however, approximately 80-85% involve the upper respiratory tract and oral cavity.3 They are found predominantly in men and are most common in the fifth, sixth and seventh decades of life.' This age
445
and sex distribution is similar to that of multiple myeloma. Extramedullary plasmacytomas, although histologically very similar to multiple myeloma, do not share the same poor prognosis; however, regardless of site or origin, they have an unpredictable course.7 Four patterns can occur: 1) response to local treatment without recurrence or dissemination; 2) invasion of surrounding structures without further dissemination; 3) subsequent distant involvement of lymph nodes, bones and other organs; and 4) evolution to multiple myeloma. There is no way of predicting which pattern will develop in a given case. As in the case presented, abnormal laboratory findings are not, as a rule, seen with extramedullary plasmacytomas. However, the high incidence of evolution to multiple myeloma (approximately 30%)3 and recent reports of multiple myeloma presenting as an extraosseous tumor, emphasizes the need for careful initial search and long term follow-up to detect chemical and bony changes in all patients with extramedullary plasmacytomas. 8 LITERATURE CITED
1. FELSON, B. Chest Roentgenology. W. B. Saunders Co., 1973. 2. FRASER, R. G. and T. A. P. PARE. Diagnosis of Disease of The Chest., W. B. Saunders Co., 1970. 3. DOLIN, S. and J. P. DEWAR. Extramedullary Plasmacytoma. Amer. J. Path., 32:83, 1956. 4. KILBURN, K. and A. M. SCHMIDT. Intrathoracic Plasmacytoma. Arch. Int. Med., 106:862869, 1960. 5. K. WARE, S. G., et al. Extensive Ossification In A Pulmonary Plasmacytoma. Thorax, 30:206-210, 1965. 6. DINES, D. et al. Solitary Plasmacytoma of The Trachea. Am. Rev. Resp. Dis., 92:949-951, 1965. 7. CARSON, C.P., et al. Plasma Cell Myeloma. Am. J. Clin. Path., 25:849, 1955. 8. GUPTA, R. M., et al. Extramedullary Plasmacytoma Ig G Type I Presenting as Mediastinal Syndrome. Brit. J. Dis. Chest, 69:65-70, 1974.
