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Case of the season
Case of the S e a s o n Bv A.M. Saks
A
31-YEAR-OLD woman presented with a 2-day history of a sharp left-sided pleuritic pain. There was no relevant medical or surgical history and no history of trauma. Results of a physical examination of the chest and lung fields were normal. A chest radiograph showed a rounded, well-delineated opacity continuous with the midportion of the left hemidiaphragm. The lung fields were clear (Fig 1). Ultrasound showed a 6- by 5-cm solid lesion in the periphery of the left lung field, clearly separate from the hemidiaphragm. The lesion had a distinctive, disorganized pattern with heterogeneous echoes, and a branching filament pattern in areas (Fig 2).
Fig 1.
From the Department of Radiology, Rosebank ttospital, Rosebank, Johannesburg, South Africa. Address Reprint Requests to Dr A Saks, Department of Radiology, Rosebank Hospital, SturdeeAve, Rosebank, Johannesburg, South Africa, Copyright © 1995 by W.B. Saunders Company 0037-198X/95/3003-000155. O0/0 222
Fig 2.
Seminars in Roentgenology, Vol XXX, No 3 (Ju}y), 1995: pp 222-223
ADULT PULMONARY BLASTOMA
DIAGNOSIS
Pleuroscopy with biopsy resulted in a diagnosis of pulmonary blastoma. A chest radiograph taken 3 months later shows marked increase in the size of the tumor. There is now a large left-sided pleural effusion and left hilar adenopathy (Fig 3). The patient died shortly after this film was taken. DISCUSSION
Pulmonary blastoma, formerly known as pulmonary embryoma, is the least common malignant pulmonary neoplasm, contributing 0.5% of primary pulmonary malignancies. Fewer than 100 cases are reported. 1,2Twenty-five percent of cases occur in childhood. 2 Associations with cystic adenoid malformation and bronchopulmonary dysplasia have been described. The remaining cases occur in adulthood, with peak incidence in the third and fourth decades. 2 Almost half of all patients are asymptomatic at the time of diagnosis. 3 Blastomas are typically peripheral; hence the term pleuropulmonary blastoma. They form large, soft, and sometimes cystic unilateral masses. 1 There is no predilection for a particular lobe. 3 Pleural effusions, lymphadenopathy, and extrathoracic metastases occur, often late,
223
and are a poor prognostic feature. The histopathologic appearances of the tumor vary considerably. There are primitive epithelial or primitive mesenchymal elements in varying degrees. The epithelial component consists of tubular structures, with branching ducts lined by cuboidal or columnar epithelium. The mesenchymal components vary, from regions of undifferentiated mesenchyma to regions where differentiation can result in formation of mature cartilage, bone, fat, and smooth or striated muscle. Some tumors consist virtually entirely of epithelial components and lack the primitive stromal elements.I, 4 Data on treatment are limited. Surgery and radiation therapy have been used, but their role is not established. Chemotherapy has little impact on the disease. Pulmonary blastoma is usually an aggressive tumor but is somewhat unpredictable. The overall 5-year survival rate is 16%. 1 Reference to pulmonary blastoma is rare in the radiologic literature. 4-6Previous reviews have centered on the pathological characteristics. 3 The classic chest radiograph finding is a large peripheral soft tissue mass in an asymptomatic, younger patient. Computed tomography features have been described as a low-attenuation mass with interspersed areas of solid tissue, producing a whorled appearance. 6 Ultrasound may show a nonspecific peripheral echogenic or mixed echoic mass. The ultrasound appearance in this patient correlates well with the described computed tomography appearances and branching ductal pattern seen pathologically. Although pulmonary blastoma is a rare tumor, the diagnosis should be considered in a younger patient with a large pleuropulmonary mass. Because of the peripheral nature of most cases, ultrasound may aid in the diagnosis. REFERENCES
Fig 3,
1. Corrin B (ed): The Lungs. London, Churchill Livingstone, 1990, pp 391-392 2. McKay MJ, Yung T, Langlands AO: Pulmonary blastoma. Clin Oncol 2:173-176, 1990 3. Koss MN, Hochholzer L, O'Leary T: Pulmonary blastomas. Cancer 67:2368-2381, 1991 4. Ohtomo K, Araki T, Yashiro N, et al: Pulmonary blastoma in children. Radiology 147:101-104, 1983 5. Shady K, Siegel MJ, Glazer HS: CT of focal pulmonary masses in childhood (review). Radiographics 12:505-514, 1992 6. Senac MO Jr, Wood BP, Isaacs H, et al: Pulmonary blastoma: A rare childhood malignancy. Radiology 179:743746, 1991
A
31-YEAR-OLD woman presented with a 2-day history of a sharp left-sided pleuritic pain. There was no relevant medical or surgical history and no history of trauma. Results of a physical examination of the chest and lung fields were normal. A chest radiograph showed a rounded, well-delineated opacity continuous with the midportion of the left hemidiaphragm. The lung fields were clear (Fig 1). Ultrasound showed a 6- by 5-cm solid lesion in the periphery of the left lung field, clearly separate from the hemidiaphragm. The lesion had a distinctive, disorganized pattern with heterogeneous echoes, and a branching filament pattern in areas (Fig 2).
Fig 1.
From the Department of Radiology, Rosebank ttospital, Rosebank, Johannesburg, South Africa. Address Reprint Requests to Dr A Saks, Department of Radiology, Rosebank Hospital, SturdeeAve, Rosebank, Johannesburg, South Africa, Copyright © 1995 by W.B. Saunders Company 0037-198X/95/3003-000155. O0/0 222
Fig 2.
Seminars in Roentgenology, Vol XXX, No 3 (Ju}y), 1995: pp 222-223
ADULT PULMONARY BLASTOMA
DIAGNOSIS
Pleuroscopy with biopsy resulted in a diagnosis of pulmonary blastoma. A chest radiograph taken 3 months later shows marked increase in the size of the tumor. There is now a large left-sided pleural effusion and left hilar adenopathy (Fig 3). The patient died shortly after this film was taken. DISCUSSION
Pulmonary blastoma, formerly known as pulmonary embryoma, is the least common malignant pulmonary neoplasm, contributing 0.5% of primary pulmonary malignancies. Fewer than 100 cases are reported. 1,2Twenty-five percent of cases occur in childhood. 2 Associations with cystic adenoid malformation and bronchopulmonary dysplasia have been described. The remaining cases occur in adulthood, with peak incidence in the third and fourth decades. 2 Almost half of all patients are asymptomatic at the time of diagnosis. 3 Blastomas are typically peripheral; hence the term pleuropulmonary blastoma. They form large, soft, and sometimes cystic unilateral masses. 1 There is no predilection for a particular lobe. 3 Pleural effusions, lymphadenopathy, and extrathoracic metastases occur, often late,
223
and are a poor prognostic feature. The histopathologic appearances of the tumor vary considerably. There are primitive epithelial or primitive mesenchymal elements in varying degrees. The epithelial component consists of tubular structures, with branching ducts lined by cuboidal or columnar epithelium. The mesenchymal components vary, from regions of undifferentiated mesenchyma to regions where differentiation can result in formation of mature cartilage, bone, fat, and smooth or striated muscle. Some tumors consist virtually entirely of epithelial components and lack the primitive stromal elements.I, 4 Data on treatment are limited. Surgery and radiation therapy have been used, but their role is not established. Chemotherapy has little impact on the disease. Pulmonary blastoma is usually an aggressive tumor but is somewhat unpredictable. The overall 5-year survival rate is 16%. 1 Reference to pulmonary blastoma is rare in the radiologic literature. 4-6Previous reviews have centered on the pathological characteristics. 3 The classic chest radiograph finding is a large peripheral soft tissue mass in an asymptomatic, younger patient. Computed tomography features have been described as a low-attenuation mass with interspersed areas of solid tissue, producing a whorled appearance. 6 Ultrasound may show a nonspecific peripheral echogenic or mixed echoic mass. The ultrasound appearance in this patient correlates well with the described computed tomography appearances and branching ductal pattern seen pathologically. Although pulmonary blastoma is a rare tumor, the diagnosis should be considered in a younger patient with a large pleuropulmonary mass. Because of the peripheral nature of most cases, ultrasound may aid in the diagnosis. REFERENCES
Fig 3,
1. Corrin B (ed): The Lungs. London, Churchill Livingstone, 1990, pp 391-392 2. McKay MJ, Yung T, Langlands AO: Pulmonary blastoma. Clin Oncol 2:173-176, 1990 3. Koss MN, Hochholzer L, O'Leary T: Pulmonary blastomas. Cancer 67:2368-2381, 1991 4. Ohtomo K, Araki T, Yashiro N, et al: Pulmonary blastoma in children. Radiology 147:101-104, 1983 5. Shady K, Siegel MJ, Glazer HS: CT of focal pulmonary masses in childhood (review). Radiographics 12:505-514, 1992 6. Senac MO Jr, Wood BP, Isaacs H, et al: Pulmonary blastoma: A rare childhood malignancy. Radiology 179:743746, 1991