Pulmonary Blastoma

Pulmonary Blastoma

PULMONARY BLASTOMA REFERENCES 1 Fiumicelli A, Fiumicelli C: Unilateral pulmonary agenesis. Med Rad Photogr 42:154, 1966 2 Hulshoff T, Kalvelage H : C...

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PULMONARY BLASTOMA REFERENCES

1 Fiumicelli A, Fiumicelli C: Unilateral pulmonary agenesis. Med Rad Photogr 42:154, 1966 2 Hulshoff T, Kalvelage H : Contribution to the diagnosis and frequency of congenital lung aplasia . Fortschr Roentgenstr 91 :725, 1959 3 Marioni P: Agenesis of the lung. Dis Chest 41:232, 1962 4 Smith RA, Bech AO: Agenesis of lung . Thorax 13:28, 1958 5 Steinberg I, Stein HL: Angiocardiography in diagnosis of agenesis of a lung . Amer J Roentgen 96:991,1966 6 Turiaf G, et al: Pulmonary agenesis and hypoplasia discovered in adults. J Franc Med Chir Thorac 16:361, 1962 7 B~mer JL : Congenital anomalies of the viscera, Cambridge, Harvard University Press, 1957 8 Emery JL, Wilcock PF : The post-natal development of the lung. Acta Anat (Basel) 65 :10, 1966 9 Kendig EL Jr (ed) Disorders of the Respiratory Tract in Children. Philadelphia, W B Saunders Co, 1967 10 Maltz DL, Nadas AS: Agenesis of the lung . Presentation of eight new cases and review of the literature. Pediatrics 42 :175, 1968 Reprint requests : Dr. Soulen, Department of Radiology, Temple University Hospital, Philadelphia 19140

Pulmonary Blastoma Anooshlraoan Nazari, M.D., F.C.C .P.; Ebrahim Amir-Mokri, M .D.; Abbas Sarram , M.D.; and lssaYaghmai, M .D.

187 ished young white boy with moderate respiratory distress . The right hemithorax was immobile with dullness to percussion and absent breath sounds, both anteriorly and posteriorly. Complete blood count revealed 13,000 white cells per mllmm with slight shift to the left. Total red blood cells were 4,000,000. Results of urinalysis was normal. Chest x-ray film showed homogeneous opacity occupying the upper two-thirds of the right hemithorax (Fig 1) . Because of the patient's respiratory distress, thoracocentesis was performed and 200 ml of serosanguinous fluid was removed ; his general condition improved at the termination of this procedure. The laboratory report on this fluid was negative for tumor cells, mycobacteria or any other pathogenic organism . A repeat thoracocentesis was necessary two days later and again 200 ml of the same material was obtained. The follow-up chest x-ray picture demonstrated accidental pneumothorax with air localized at lower part of the right hemithorax. The patient was closely observed for three days and since he continued having respiratory difficulties, such as he had had before the second thoracocentesis, an exploratory thoracotomy was performed on November 13, 1969. At operation, a tumor was seen occupying the entire right upper lobe and completely occluding the middle and lower lobe bronchi. The amount of adhesions between these three lobes made them extremely difficult to separate. The tumor was tense and thickly adherent to the parietal pleura of the fourth, fifth and sixth ribs anteriorly and laterally . To facilitate mobilization of the right lung it was necessary to aspirate the content of the tumor. After evacuating 700 ml of serosanguinous fluid, right pneumonectomy was carried out. No mediastinal lymph nodes were encountered. However, there were two masses completely separate from the lung, one subpleurally attached to the right hemidiaphragm and the other to the posterior aspect of the lower part of the sternum,

Pulmonary blastoma is a rare and potentially malignant tumor capable of metastasis. To date, there have been 15 cases repor1ed In the literature. This report describes the histogenesis of this type tumor and discusses other rare pulmonary neoplasms.

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u rin g the past decade considerable attention has been given to a group of rare pulmonary tumors which Spencer! in 1961 called "pulmonary blastoma." To date, there have been only 15 reported cases of this type of neoplasm in the literature. It is our belief that the rarity of this tumor and controversy still existing among pathologists as to the histogenesis of this unusual neoplasm, warrants the report of additional cases of this nature and a further review of the literature in order to clarify the clinical behavior and morphologic characteristics of this rare group of pulmonary tumors. CASE REPoRT

A 15-year-old white youth was admitted to the hospital because of right-sided chest pain and mild degree of shortness of breath. He dated his symptoms back to one year ago when he first developed dry cough, low grade fever, and vague right-s ided chest pain. He was seen at that time by his local doctor, a gene ral practitioner, who had treated him for common cold. The patient first consulted us on November 8, 1969. The physical examination revealed a well-developed, well nour-

CHEST, VOL. 60, NO.2, AUGUST 1971

FIGURE 1. PA view of the chest one week before operation reveals a large well-defined mass occupying two-thirds of the right upper lung field. The left lung and bony structures are normal .

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NAZAR I ET AL pletely atelectatic as their main bronchi were compressed by tumor. The cut surface revealed a well demarcated tumor, creamy-white in color showing large areas of hemorrhage and cystic degeneration. At the margins of the tumor compressed pulmonary parenchyma could be identified. Two large masses excised from parietal pleura measured 6 x 4 x 2 cm and 4 x 3 x 2 cm respectively, and appeared well demarcated. Their cut surfaces were quite similar to the main tumor (Fig 3). Microscopic Examination

Histologically, numerous sections taken from the various parts of the tumor, including the subpleural masses, showed a uniform pattern throughout. The tumor was highly cellular and composed of rather small, undifferentiated mesenchymal cells with round or oval nuclei and scanty, poorly demarcated cytoplasm. In many areas there were cleft-like spaces lined by one layer of cuboidal cells which seemed to differentiate from the undifferentiated mesenchyma. The main tumor was bounded by a well-formed fibrous capsule and lacked mitoses. The subpleural masses were also well demarcated and showed some condensation of fibrous connective tissue at the periphery. DISCUSSION

FIGURE 2. PA view of the chest ten months postoperation reveals normal heart and left lung. Complete even density of the right side, due to fibrosis, is noted. which were excised with ease. The chest was closed in routine fashion. The postoperative course was uneventful and the patient is well ten months after right pneumonectomy. The chest x-ray film is illustrated in Figure 2. Gross Examination

The entire right upper lobe was replaced by a large spherical mass with a soft cystic consistency measuring 18 cm in diameter. The right middle and lower lobes were com-

FIGURE 3. Cut surface of the tumor, atelectatic middle and lower lobes are seen at the lower part of the photograph and to the right. The specimen to the left shows the cut surface of the substernal mass which was completely separated from the main tumor.

Pulmonary blastoma still represents a challenging problem. The embryogenesis of the lung is that it develops from laryngotracheal bud which grows and subdivides within the investing mesenchyma. Waddell- in 1949 claimed that the distal air passage is formed from mesenchymal celIs which grow in apposition to the terminations of the laryngotracheal bud. Spencer.s accepting this theory, stated that pulmonary blastoma o ~ embryoma develops from a pleuripotential cell of one germ layer in the same manner as the nephroblastoma arises from multipotential renal blastema, and he suggested that this tumor should be better named pulmonary blastoma than embryoma, as applied by Barnard." The question as to how the age of these patients with pulmonary blastoma can be explained, since there have been only two cases in patients in their second decade of life in contrast to the fact that the peak incidence is in the fifth decade, has been answered by Spencer! who indicates that the lung tissue continues to grow several years after birth (ten years or more) . Another explanation for the appearance of these embryonic tumors has been given by wiliis 4 who suggested that adult mesenchymal tissue may contain undifferentiated cells which may revert to embryonic state. . The pulmonary hamartoma can be differentiated from pulmonary blastoma on the basis that the former is confined to a limited anatomic field, not invasive and usually well differentiated, whereas the latter has a tendency to invade and has unlimited growth. Perhaps the most difficult differentiation exists between pulmonary blastoma and carcinosarcoma. Souza" and Bauermeister6 have classified this tumor as a distinct type of carcinosarcoma which is defined by Willis as either simultaneous malignant neoplasm in epithelial tissue and the surrounding mesodermal stroma or sarcomatous changes occurring secondarily in the stroma of a carci-

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PROGRESSIVE CONGENITAL VALVULAR AORTIC STENOSIS noma. Stackhouse? and associates, reviewing eight cases of pulmonary blastoma, stated that these cases should be considered as a distinctive group or subclassification of carcinosarcoma which simulates immature or developing lung blastema. Although more detail of differences in histogenesis of these two varieties of rare pulmonary tumors are given by others," it is important to recall that carcinosarcoma arises from cells of two separate germ layers, whereas pulmonary blastoma develops from a pleuripotential cell of a single germ layer. The benign or malignant nature of this tumor still remains mysterious. However, after reviewing the literature, it seems reasonable to postulate that it is the metastatic nature of this neoplasm which causes the very poor prognosis . The size of these lesions bears no relation to the extent of metastases and subsequently to ultimate outcome of these patients. The treatment of choice, from the available data, seems to be surgical intervention unless the risk of operation is considered hazardous to the life of the patient. ADDENDUM

The patient was last seen on April 1, 1971, 16 months after operation. He is completely free of symptoms and his chest x-ray film shows negative findings. ACKNOWLEDGMENT: We are grateful to Professor H. Spencer for his confirmation of the histologic appearances of this case. REFERENCES

1 Spencer H: Pulmonary blastoma. J Path Bact 82:161, 1961. 2 Waddell WR: Organoid differentiation of the fetal lung: a histologic study of the differentiation of mammalian fetal lung in utero and in transplants . Arch Path 47 :227,1949 3 Spencer H : Pathology of the Lung. Second edition, New York, Pergamon Press, 1968,985-989 4 Willis RA: Pathology of Tumors. Third edition , London, Butterworth, 1960, 140 5 Souza RC, Peasley ED, Takaro T: Pulmonary blastoma : a . distinctive group of carcinosarcoma of the lung. Ann Thorac Surg 1:259, 1965 6 Bauermeister DE, Jennings ER, Beland AH, et al: Pulmonary blastoma, a form of carcinosarcoma : report of a case of 24 years' duration without treatment. Amer J Clin Path 46:322, 1966 7 Stackhouse EM, Harrison EG Jr. Ellis FH Jr: Pulmonary mixed malignancies of lung : carcinosarcoma and blastoma. J Thorac Cardiovasc Surg 57 :385-398, 1969 8 Barnard WG : Embryoma of lung. Thorax 7 :299, 1952 Reprint requests : Dr. Nazari, 168 North Saba Avenue, Tehran, Iran

FIGURE 1. The QRS voltage is consistent with left ventricular hypertrophy. Early repolarization can be seen in several leads (normal standardization, 1 millivolt-10 mm deflection).

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lthough there have been several reports I-Ii regarding the clinical course of mild congenital aortic valvular stenosis, to date there have been few published articles of patients followed-up with serial cardiac catheterizations to determine whether the stenosis changes in severity with growth of the subject. We have recently seen a 12-year-old boy, proved by cardiac catherization to have mild aortic valvular stenosis at age five years, who on repeat catheterization seven years later was found to have progressed to severe aortic stenosis. CASE REPORT

Our patient was the product of an uncomplicated pregnancy and delivery and weighed 3,340 gm at birth. Heart murmur was detected at six weeks of age. He was first seen at Colorado General Hospital at four years of age, at which time he was asymptomatic . Physical examination revealed a systolic thrill in the suprasternal notch, a systolic ejection click and a grade 4/6 systolic ejection murmur. Diastole was clear and no other murmur or extra heart sound was heard . No lift of left ventricle was noted . At cardiac fluoroscopy, the overall heart size was normal; however, in the left anterior

Progressive Congenital Valvular Aortic Stenosis* George E. Bandy, M.D. and John H . K. Vogel, M.D., F.C.C.P.··

Repeat cardiac catheterization in a 12-year-01d boy (previously shown to have mild valvular aortic stenosis at five years of age) revealed development of severe stenosis, thus reaflinning the need for careful follow-up studies in patients with mUd congenital valvular aortic stenosis. ·From the Cardiovascular Laboratory, Division of Cardiology, Department of Medicine, University of Colorado Medical Center, Denver. • ·Presently at Santa Barbara, California.

CHEST, VOL. 60, NO.2, AUGUST 1971

FIGURE 2. As compared to the tracing of 1960, the QRS voltage has increased in both the frontal and precordial leads and minor ST segment and T wave changes have appeared.