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Benign mediastinal teratoma masquerading as a large multiloculated effusion
CASE REPORT ROBINSON ET AL MEDIASTINAL TERATOMA
Ann Thorac Surg 1994;58:545-8
545
septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and hypoplasia of the aortic isthmus: a newly recognized syndrome. Am J Cardiol 1982;49:108-16. 4. Kitigawa T, Katoh I, Taki H, et al. New operative method for distal aortopulmonary septal defect. Ann Thorac Surg 1991;51: 680-2. 5. Sell JE, Jonas RA, Mayer JE, Blackstone EH, Kirklin JW, Castaneda AR. The results of a surgical program for interrupted aortic arch. J Thorac Cardiovasc Surg 1988;96:864-77.
Benign Mediastinal Teratoma Masquerading as a Large Multiloculated Effusion Fig 5. The complete repair with the descending aorta anastomosed to the defect in the ascending aorta and the right pulmonary artery anastomosed to the main pulmonary artery anteriorly.
artery [4]. The pulmonary artery is closed behind the aorta, and the ascending aorta is reanastomosed end to end. This technique maintains pulmonary artery continuity without intraaortic baffling. However, an associated arch interruption might leave inadequate aortic length to repair both the pulmonary artery and the interruption causing obstruction of the RPA along its course posterior to the aorta. Experience with repair of interrupted aortic arch suggests that mobilization of the arch vessels and, if necessary, division of the subclavian arteries creates adequate length for primary anastomosis. In neonates, this technique may decrease the recurrent arch obstruction often seen when Dacron conduits or arch vessels are used to bridge the interruption [5]. In our patient, the location and size of the septal defect allowed a wide, tension-free aortic anastomosis to lie well above the left main bronchus. Persistent ductal tissue in the distal segment may have contributed to restenosis, which was readily dilated at catheterization. Our experience with the arterial switch operation in neonates suggests that anterior transfer of the pulmonary arteries is tolerated well as long as the branches are dissected completely. In this case, mobilizing the RPA to the hilum allowed anastomosis to the main pulmonary artery anterior to the aorta, without a conduit. This arrangement maintains tissue continuity, allowing for continued growth, and places the transposed pulmonary arteries in an accessible position for further pulmonary reconstruction or angioplasty. References 1. Gross RE. Surgical closure of an aortic septal defect. Circulation 1952;5:858-63. 2. Ding W, Su Z, Cao D, Jonas RA. One-stage repair of absence of the aortopulmonary septum and interrupted aortic arch. Ann Thorac Surg 1990;49:664-6. 3. Berry TE, Bharati S, Muster AI, et al. Distal aortopulmonary © 1994 by The Society of Thoracic Surgeons
Lary A. Robinson, MD, Layton F. Rikkers, MD, and John R. Dobson, MD Departments of Surgery and Pathology, University of Nebraska Medical Center, Omaha, Nebraska
Benign mediastinal teratomas are uncommon germ cell tumors often discovered while still asymptomatic. Almost all arise in the anterosuperior mediastinal compartment, and most symptoms, when present, result from compression of adjacent structures. We report a case of a large teratoma arising from the anterior mediastinum that presented a confusing clinical picture of a multiloculated pleural effusion. It was successfully treated by surgical excision, with no long-term recurrence. (Ann Thome Surg 1994;58:545-8)
B
en ign mediastinal teratomas are uncommon neoplasms, accounting for 5% to 8% of all tumors in this region [1,2]. More than 95% of benign teratomas arise from the anterosuperior mediastinum [1, 2], and in adults the majority of patients usually are asymptomatic [2]. Despite containing a variety of differentiated tissues, teratomas generally are nonfunctional and cause symptoms by their size and location. We now report a patient with a confusing presentation of a large symptomatic mass thought preoperatively to be a large, multiloculated pleural fluid collection, possibly a chronic empyema. Although similar unusual presentations of this tumor have been documented in case reports of seven children, as summarized by Shackelford and McAlister [3], we now report an adult case of this type. A 38-year-old farm housewife was seen by her family physician in 1985 complaining of a 1.8-kg weight loss and pleuritic right anterior chest pain that increased with deep breathing, cough, or recumbency. She had a 3-month history of a "pushing" sensation inferior to the right costal Accepted for publication Nov 11, 1993. Address reprint requests to Dr Robinson, Thoracic Oncology Program, H. Lee Moffitt Cancer Center, 12902 Magnolia Dr, Tampa, FL 33612-9497.
0003-4975/94/$7.00
546
CASE REPORT ROBINSON ET AL MEDIASTINAL TERATOMA
Fig 1. Posteroanterior chest radiogram demonstrating? large right subpleural density with a slight shift of the heart to the left.
margin and a 3-week history of arthralgias in most major joints without swelling. She denied fevers, chills, productive cough, hemoptysis, or any significant medical problems. On physical examination, the patient was a mildly dyspneic woman with a temperature of 37.1°C and pulse of 92 beats/min. She had decreased breath sounds over the lower half of the right hemithorax and an enlarged, nontender liver. The remainder of her physical examination was normal. An initial chest radiogram demonstrated a large right pleural effusion. A thoracentesis yielded 800 mL of cloudy yellow fluid with a glucose level of 101 mg/dl, amylase level of 151 IU /L, protein level of 4.6 g/ d l., and white blood cell count of 13,400 cells/ ILL. After a 7-day course of cephalothin, an additional 250 ml, was removed because of fluid reaccumulation with increasing chest pain and dyspnea. The patient then was transferred to our medical center with continuing right pleuritic pain and low-grade fevers. A chest radiogram (Fig 1) revealed a large, subpulmonic right pleural effusion. Computed tomographic scans of the chest and abdomen revealed right lower lobe collapse and consolidation, and an extensive multiloculated fluid collection in the right hemithorax consistent with an empyema (Fig 2). Several punctate calcifications also were seen in the fluid collection. The liver was quite enlarged with scattered small hypodense lesions. Serial serum liver enzyme determinations showed a gradual increase over 3 weeks, but the total bilirubin level was normal. She had a hemoglobin level of 9.4 g/ dl, and a white blood count of 21,300 cells/ ILL. Serum iron content and total iron binding capacity were both low. A third thoracentesis yielded 400 ml. of gray-green purulent fluid, which had a glucose level of 37 mg/ dL (serum glucose level, 102 mg/ dl.), protein level of 1.2 g/dl
Ann Thome Surg 1994;58:545-8
(serum protein level, 6.9 g/dL), lactate dehydrogenase level of 1,903 IU/l (serum lactate dehydrogenase level, 101 IU /L), amylase level of 798 IU /L (serum amylase level, 36 IU/L), and pH of 7.10. On cell count, only disintegrating white blood cells were seen. Cytologies and all cultures on pleural fluid were negative. A pleural fluid carcinoembryonic antigen assay was markedly elevated at 107.0 ng/ml, with a serum carcinoembryonic antigen level of 2.8 ng/ml (normal < 3.0 ng/mL). Numerous other laboratory studies were normal. The patient was then taken to operation with the presumed diagnosis of a chronic multiloculated empyema or a cystic malignancy with liver metastases. An open liver biopsy demonstrated a large, firm, smooth liver. Microscopically, well-circumscribed focal nodular macrovesicular fatty metamorphosis in zones 1 and 2 was seen. At thoracotomy, an encapsulated nodular mass with multiple large cysts filled the right hemithorax and displaced the lung superiorly. The mass had apparently prolapsed from the anterior mediastinum into the pleural cavity as its growth progressed. Although densely adherent to the visceral and parietal pleura, the mass was removed intact including a long, thin stalk arising from the mediastinal fat anterior to the heart. The large mass (Fig 3) was composed of multiple smooth-walled cysts, which contained turbid, yellow and brown fluid. Microscopically (Fig 4), welldifferentiated elements from all three primordial tissue layers were found. The final diagnosis was benign cystic teratoma. The patient had an uncomplicated postoperative course and was discharged after 7 days. Now, 8 years later, the patient is healthy and has had no recurrence on chest radiograms.
Comment Teratomas are the most common type of germ cell tumor, and the mediastinum is their most common extragonadal location. Overall, germ cell tumors are the fourth most common mediastinal neoplasm, occurring almost always in the anterosuperior compartment. In this location, germ cell tumors are second only to thymomas and lymphomas
Fig 2. Representative scan from a contrast-enhanced computed tomographic scan of the chest. This image is taken at a level 7 cm inferior to the carina and demonstrates an extensive multi/oculated right pleural fluid collection containing several punctate areas of calcification, displacing the right lung superiorly and the heart slightly to the left.
Ann Thorac Surg 1994;58:545-8
in frequency [1]. Mediastinal teratomas present most commonly in the second and third decades of life with a slight female preponderance of the benign neoplasms and a definite male dominance of malignant tumors [2]. Slightly more than half of patients with benign teratomas are asymptomatic on diagnosis. The most common symptom is chest pain, which may be accompanied by dyspnea, fever, and cough. The cough may be productive of hair or sebum, indicating a communication between the tracheobronchial tree and the teratoma. Physical examination in most patients is unremarkable. Chest dullness and diminished breath sounds were found in 41% of patients in one series [2]. Less common findings include a bulging deformity of the chest wall, clubbing, cyanosis, wheezing, a draining fistula between the tumor and the skin, acanthosis nigricans, and superior vena caval obstruction [2]. The arthralgias and hepatomegaly seen in this patient are unusual in teratoma patients. Hepatomegaly with focal macrovesicular fatty metamorphosis, which was present in this patient, usually results from diabetes, alcoholism, obesity, hyperalimentation, or Cushing's syndrome. However this individual was not afflicted with any of these disorders. The enlarged liver may have been caused by chronic "systemic" disease, a less common cause. Chest radiograms are useful for initial detection, but chest computed tomography is the most valuable [4]. Typically, a thick-walled cystic mass with little or no fat is seen often protruding into the lung fields. Although distinctive when present, only 26% to 33% of teratomas have radiographic calcifications or well-formed teeth [2, 4]. The present patient had a few tiny calcifications (see Fig 2), but the multicystic mass was located entirely in the base of the right hemithorax and mimicked inflammatory pleural disease. This unusual location of the tumor, the associated respiratory distress, and the confusion of it with a large pleural effusion was seen in 7 prior pediatric cases [3]. Generally, laboratory studies are not helpful in the
Fig 3. Photograph of the fresh specimen showing a multicystic 17.5 cm x 13 cm x 7.6 em mass weighing 829 g. The cut edge of the tiny stalk supplying this tumor is seen at the top right corner of the mass.
CASE REPORT ROBINSON ET AL MEDIASTINAL TERATOMA
547
Fig 4. Photomicrograph of a representative section of the tumor demonstrates all three primordial tissue layers. Endoderm: The left onethird of the section shows pancreas with abundant lighter-staining islets (A), and respiratory epithelium (B) is seen in the lower center. Mesoderm: In the upper center, mature connective tissue and fat (C) are found. Ectoderm: At the far right (0), stratified squamous epithelium (skin) with subcutaneous eccrine glands are demonstrated. (Hematoxylin and eosin; xIS before 51% reduciion.)
diagnosis of teratoma; they were even misleading in this case. Multiple thoracenteses yielded different results, probably from puncture of different cysts. The high amylase content in the fluid suggested either pancreatic disease or a malignancy, but it was most likely secondary to amylase production by pancreatic tissue or salivary gland tissue found in the teratoma. Most confusing was the combination of a markedly elevated carcinoembryonic antigen level (107.0 ng/mL) in the thoracentesis fluid and a normal level in the serum. In one study of 105 patients, a carcinoembryonic antigen level greater than 10 ng/mL in pleural fluid was 100% diagnostic of malignancy [5]. Treatment of these histologically benign tumors requires surgical excision for a definitive diagnosis and symptom relief. Removal often is difficult because of dense adhesions to vital intrathoracic structures. The long-term outlook with operation is excellent, with low surgical mortality and no recurrences. Pathologically, benign teratomas are well encapsulated, usually cystic, and may be quite large (up to 1,820 g) [2]. Histologically, teratomas contain all three primordial tissue layers by definition. The simplest form is the teratodermoid (dermoid) cyst, which predominately contains ectodermal derivatives. The solid component of mature teratomas has well-differentiated elements of ectoderm (skin, hair), mesoderm (bone, fat, muscle), and endoderm (respiratory epithelium, pancreas). Islets of Langerhans are found in most pancreas sections and tend to have a predominance of somatostatin-producing D cells [6]. Immunohistochemical staining of pancreatic tissue in the tumor from the present patient also demonstrated an abundance of somatostatin-producing D cells in islets (Fig 5). In addition, our patient also had tissue with positive staining for insulin and for neuron-specific enolase. In one documented case involving a child, this endocrine tissue was functional in an insulin-producing
548
CASE REPORT WEISS CORONARY SINUS RUPTURE
Ann Thorac Surg 1994;58:548-50
Management of Difficult Coronary Sinus Rupture Steven J. Weiss, MD Albert Einstein Medical Center, Philadelphia, Pennsylvania
Fig 5. Immunohistochemical stain of pancreatic tissue from the tumor demonstrates abundant somatostatin-producing 0 cell, (dark staining cells) in the periphery of the islets of Langerhans. (X150 before 51 % reduction.)
teratoma causing hypoglycemia and an elevated plasma immunoreactive insulin level, which resolved after removal of the tumor [7]. Malignant teratomas occur in 19% to 44% of the total group of tumors and diller from their benign counterpart by having embryonic tissue with less well differentiated structures, less cystic components, and a uniformly dismal prognosis [81. The histogenesis of mediastinal teratomas is quite controversial, but most authorities agree that they are not metastatic from primary gonadal tumors. Some postulate that these neoplasms arise from primordial germ cells left behind during migration from the yolk sac to the urogenital ridge, whereas others believe that this tumor has its origin in faulty embryogenesis of the thymus [8]. Whatever their origin, benign mediastinal teratomas are eminently curable with operation in spite of unusual, dramatic presentations. Interestingly, in the most current series from the Mayo Clinic, there has been an apparent decline in the frequency of this unusual tumor in recent years [2].
References 1. Davis RD, Oldham HN, Sabiston DC. Primary cysts and neoplasms of the mediastinum: recent changes in clinical presentation, methods of diagnosis, management, and results. Ann Thorac Surg 1987;44:229-37. 2. Lewis BD, Hurt RD, Payne WS, Farrow GM, Knapp RH, Muhm JR. Benign teratomas of the mediastinum. J Thorac Cardiovasc Surg 1983;86:727-31. 3. Shackelford DG, McAlister WHo Mediastinal teratoma confused with loculated pleural fluid. Pediatr RadioI1976;5:118-9. 4. Suzuki M, Takashima T, Itoh H, Choutoh S, Kawamura I, Watanabe Y. Computed tomography of mediastinal teratomas. J Comput Assist Tomogr 1983;7:74-6. 5. Vladutiu AO. Carcinoembryonic antigen in pleural effusions. Lancet 1978;2:423-4. 6. Dunn PJS. Pancreatic endocrine tissue in benign mediastinal teratoma. J Clin PathoI1984;37:1105-9. 7. Honicky RE, dePapp EW. Mediastinal teratoma with endocrine function. Am J Dis Child 1973;126:650-3. 8. Pachter MR, Lattes R. "Germinal" tumors of the mediastinum: a clinicopathologic study of adu!t teratomas, teratocarcinomas, choriocarcinomas and seminomas. Dis Chest 1964;45:301-10. © 1994 by The Society of Thoracic Surgeons
A technique of handling severe coronary sinus rupture when direct suture repair or pericardial patch techniques have failed is described. A pericardial patch is sewn over the coronary sinus with running 5-0 Prolene suture, and an 8-mm thin-walled Gore-Tex conduit then is constructed between the patch and the right atrium to decompress the repair and allow an alternative route for coronary sinus return to the right atrium. (Ann Thorae Surg 1994;58:548-50)
T
he current incidence of coronary sinus injury has been estimated at 0.6% [1]. As the technique for cannulation of the coronary sinus for administration of retrograde cardioplegia becomes more widespread, it is likely that the occurrence of coronary sinus injury also will increase. Engineering refinements of the cannulas and increasing expertise will likely reduce but not eliminate this occurrence. A 70-year-old man with a past medical history of inferoposterior myocardial infarction, paroxysmal atrial fibrillation, abdominal aortic aneurysm, and chronic renal insufficiency presented with crescendo angina refractory to medications. Cardiac catheterization revealed occlusion of a dominant right coronary artery and severe left anterior descending and large diagonal lesions not amenable to angioplasty. Left ventricular function was mildly diminished and there was a 1 + to 2 + aortic insufficiency. He underwent triple coronary bypass: saphenous veins to the posterior descending coronary artery and diagonal coronary artery, and left internal mammary artery to the left anterior descending artery. My standard myocardial protection technique is continuous warm retrograde blood cardioplegia. During the operation the retrograde cannula was inserted easily through a high right atrial pursestring into the coronary sinus. A 15F self-inflating balloon cannula (RCSP catalog no. 94415; DLP Inc, Grand Rapids, MI) was used. At no time did the pressure measured in the coronary sinus exceed the recommended 40 mm Hg safe limit [2]. During the procedure, however, cardioplegia was visualized escaping from the coronary sinus just before its insertion into the right atrium due to a burst at the site of the balloon, with the balloon protruding through the rupture. Accepted for publication Nov 13, 1993. Address reprints to Dr Weiss, Albert Einstein Medical Center, 5501 Old York Rd, HB-3, Philadelphia, PA 19141.
0003-4975/94/$7.00
Ann Thorac Surg 1994;58:545-8
545
septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and hypoplasia of the aortic isthmus: a newly recognized syndrome. Am J Cardiol 1982;49:108-16. 4. Kitigawa T, Katoh I, Taki H, et al. New operative method for distal aortopulmonary septal defect. Ann Thorac Surg 1991;51: 680-2. 5. Sell JE, Jonas RA, Mayer JE, Blackstone EH, Kirklin JW, Castaneda AR. The results of a surgical program for interrupted aortic arch. J Thorac Cardiovasc Surg 1988;96:864-77.
Benign Mediastinal Teratoma Masquerading as a Large Multiloculated Effusion Fig 5. The complete repair with the descending aorta anastomosed to the defect in the ascending aorta and the right pulmonary artery anastomosed to the main pulmonary artery anteriorly.
artery [4]. The pulmonary artery is closed behind the aorta, and the ascending aorta is reanastomosed end to end. This technique maintains pulmonary artery continuity without intraaortic baffling. However, an associated arch interruption might leave inadequate aortic length to repair both the pulmonary artery and the interruption causing obstruction of the RPA along its course posterior to the aorta. Experience with repair of interrupted aortic arch suggests that mobilization of the arch vessels and, if necessary, division of the subclavian arteries creates adequate length for primary anastomosis. In neonates, this technique may decrease the recurrent arch obstruction often seen when Dacron conduits or arch vessels are used to bridge the interruption [5]. In our patient, the location and size of the septal defect allowed a wide, tension-free aortic anastomosis to lie well above the left main bronchus. Persistent ductal tissue in the distal segment may have contributed to restenosis, which was readily dilated at catheterization. Our experience with the arterial switch operation in neonates suggests that anterior transfer of the pulmonary arteries is tolerated well as long as the branches are dissected completely. In this case, mobilizing the RPA to the hilum allowed anastomosis to the main pulmonary artery anterior to the aorta, without a conduit. This arrangement maintains tissue continuity, allowing for continued growth, and places the transposed pulmonary arteries in an accessible position for further pulmonary reconstruction or angioplasty. References 1. Gross RE. Surgical closure of an aortic septal defect. Circulation 1952;5:858-63. 2. Ding W, Su Z, Cao D, Jonas RA. One-stage repair of absence of the aortopulmonary septum and interrupted aortic arch. Ann Thorac Surg 1990;49:664-6. 3. Berry TE, Bharati S, Muster AI, et al. Distal aortopulmonary © 1994 by The Society of Thoracic Surgeons
Lary A. Robinson, MD, Layton F. Rikkers, MD, and John R. Dobson, MD Departments of Surgery and Pathology, University of Nebraska Medical Center, Omaha, Nebraska
Benign mediastinal teratomas are uncommon germ cell tumors often discovered while still asymptomatic. Almost all arise in the anterosuperior mediastinal compartment, and most symptoms, when present, result from compression of adjacent structures. We report a case of a large teratoma arising from the anterior mediastinum that presented a confusing clinical picture of a multiloculated pleural effusion. It was successfully treated by surgical excision, with no long-term recurrence. (Ann Thome Surg 1994;58:545-8)
B
en ign mediastinal teratomas are uncommon neoplasms, accounting for 5% to 8% of all tumors in this region [1,2]. More than 95% of benign teratomas arise from the anterosuperior mediastinum [1, 2], and in adults the majority of patients usually are asymptomatic [2]. Despite containing a variety of differentiated tissues, teratomas generally are nonfunctional and cause symptoms by their size and location. We now report a patient with a confusing presentation of a large symptomatic mass thought preoperatively to be a large, multiloculated pleural fluid collection, possibly a chronic empyema. Although similar unusual presentations of this tumor have been documented in case reports of seven children, as summarized by Shackelford and McAlister [3], we now report an adult case of this type. A 38-year-old farm housewife was seen by her family physician in 1985 complaining of a 1.8-kg weight loss and pleuritic right anterior chest pain that increased with deep breathing, cough, or recumbency. She had a 3-month history of a "pushing" sensation inferior to the right costal Accepted for publication Nov 11, 1993. Address reprint requests to Dr Robinson, Thoracic Oncology Program, H. Lee Moffitt Cancer Center, 12902 Magnolia Dr, Tampa, FL 33612-9497.
0003-4975/94/$7.00
546
CASE REPORT ROBINSON ET AL MEDIASTINAL TERATOMA
Fig 1. Posteroanterior chest radiogram demonstrating? large right subpleural density with a slight shift of the heart to the left.
margin and a 3-week history of arthralgias in most major joints without swelling. She denied fevers, chills, productive cough, hemoptysis, or any significant medical problems. On physical examination, the patient was a mildly dyspneic woman with a temperature of 37.1°C and pulse of 92 beats/min. She had decreased breath sounds over the lower half of the right hemithorax and an enlarged, nontender liver. The remainder of her physical examination was normal. An initial chest radiogram demonstrated a large right pleural effusion. A thoracentesis yielded 800 mL of cloudy yellow fluid with a glucose level of 101 mg/dl, amylase level of 151 IU /L, protein level of 4.6 g/ d l., and white blood cell count of 13,400 cells/ ILL. After a 7-day course of cephalothin, an additional 250 ml, was removed because of fluid reaccumulation with increasing chest pain and dyspnea. The patient then was transferred to our medical center with continuing right pleuritic pain and low-grade fevers. A chest radiogram (Fig 1) revealed a large, subpulmonic right pleural effusion. Computed tomographic scans of the chest and abdomen revealed right lower lobe collapse and consolidation, and an extensive multiloculated fluid collection in the right hemithorax consistent with an empyema (Fig 2). Several punctate calcifications also were seen in the fluid collection. The liver was quite enlarged with scattered small hypodense lesions. Serial serum liver enzyme determinations showed a gradual increase over 3 weeks, but the total bilirubin level was normal. She had a hemoglobin level of 9.4 g/ dl, and a white blood count of 21,300 cells/ ILL. Serum iron content and total iron binding capacity were both low. A third thoracentesis yielded 400 ml. of gray-green purulent fluid, which had a glucose level of 37 mg/ dL (serum glucose level, 102 mg/ dl.), protein level of 1.2 g/dl
Ann Thome Surg 1994;58:545-8
(serum protein level, 6.9 g/dL), lactate dehydrogenase level of 1,903 IU/l (serum lactate dehydrogenase level, 101 IU /L), amylase level of 798 IU /L (serum amylase level, 36 IU/L), and pH of 7.10. On cell count, only disintegrating white blood cells were seen. Cytologies and all cultures on pleural fluid were negative. A pleural fluid carcinoembryonic antigen assay was markedly elevated at 107.0 ng/ml, with a serum carcinoembryonic antigen level of 2.8 ng/ml (normal < 3.0 ng/mL). Numerous other laboratory studies were normal. The patient was then taken to operation with the presumed diagnosis of a chronic multiloculated empyema or a cystic malignancy with liver metastases. An open liver biopsy demonstrated a large, firm, smooth liver. Microscopically, well-circumscribed focal nodular macrovesicular fatty metamorphosis in zones 1 and 2 was seen. At thoracotomy, an encapsulated nodular mass with multiple large cysts filled the right hemithorax and displaced the lung superiorly. The mass had apparently prolapsed from the anterior mediastinum into the pleural cavity as its growth progressed. Although densely adherent to the visceral and parietal pleura, the mass was removed intact including a long, thin stalk arising from the mediastinal fat anterior to the heart. The large mass (Fig 3) was composed of multiple smooth-walled cysts, which contained turbid, yellow and brown fluid. Microscopically (Fig 4), welldifferentiated elements from all three primordial tissue layers were found. The final diagnosis was benign cystic teratoma. The patient had an uncomplicated postoperative course and was discharged after 7 days. Now, 8 years later, the patient is healthy and has had no recurrence on chest radiograms.
Comment Teratomas are the most common type of germ cell tumor, and the mediastinum is their most common extragonadal location. Overall, germ cell tumors are the fourth most common mediastinal neoplasm, occurring almost always in the anterosuperior compartment. In this location, germ cell tumors are second only to thymomas and lymphomas
Fig 2. Representative scan from a contrast-enhanced computed tomographic scan of the chest. This image is taken at a level 7 cm inferior to the carina and demonstrates an extensive multi/oculated right pleural fluid collection containing several punctate areas of calcification, displacing the right lung superiorly and the heart slightly to the left.
Ann Thorac Surg 1994;58:545-8
in frequency [1]. Mediastinal teratomas present most commonly in the second and third decades of life with a slight female preponderance of the benign neoplasms and a definite male dominance of malignant tumors [2]. Slightly more than half of patients with benign teratomas are asymptomatic on diagnosis. The most common symptom is chest pain, which may be accompanied by dyspnea, fever, and cough. The cough may be productive of hair or sebum, indicating a communication between the tracheobronchial tree and the teratoma. Physical examination in most patients is unremarkable. Chest dullness and diminished breath sounds were found in 41% of patients in one series [2]. Less common findings include a bulging deformity of the chest wall, clubbing, cyanosis, wheezing, a draining fistula between the tumor and the skin, acanthosis nigricans, and superior vena caval obstruction [2]. The arthralgias and hepatomegaly seen in this patient are unusual in teratoma patients. Hepatomegaly with focal macrovesicular fatty metamorphosis, which was present in this patient, usually results from diabetes, alcoholism, obesity, hyperalimentation, or Cushing's syndrome. However this individual was not afflicted with any of these disorders. The enlarged liver may have been caused by chronic "systemic" disease, a less common cause. Chest radiograms are useful for initial detection, but chest computed tomography is the most valuable [4]. Typically, a thick-walled cystic mass with little or no fat is seen often protruding into the lung fields. Although distinctive when present, only 26% to 33% of teratomas have radiographic calcifications or well-formed teeth [2, 4]. The present patient had a few tiny calcifications (see Fig 2), but the multicystic mass was located entirely in the base of the right hemithorax and mimicked inflammatory pleural disease. This unusual location of the tumor, the associated respiratory distress, and the confusion of it with a large pleural effusion was seen in 7 prior pediatric cases [3]. Generally, laboratory studies are not helpful in the
Fig 3. Photograph of the fresh specimen showing a multicystic 17.5 cm x 13 cm x 7.6 em mass weighing 829 g. The cut edge of the tiny stalk supplying this tumor is seen at the top right corner of the mass.
CASE REPORT ROBINSON ET AL MEDIASTINAL TERATOMA
547
Fig 4. Photomicrograph of a representative section of the tumor demonstrates all three primordial tissue layers. Endoderm: The left onethird of the section shows pancreas with abundant lighter-staining islets (A), and respiratory epithelium (B) is seen in the lower center. Mesoderm: In the upper center, mature connective tissue and fat (C) are found. Ectoderm: At the far right (0), stratified squamous epithelium (skin) with subcutaneous eccrine glands are demonstrated. (Hematoxylin and eosin; xIS before 51% reduciion.)
diagnosis of teratoma; they were even misleading in this case. Multiple thoracenteses yielded different results, probably from puncture of different cysts. The high amylase content in the fluid suggested either pancreatic disease or a malignancy, but it was most likely secondary to amylase production by pancreatic tissue or salivary gland tissue found in the teratoma. Most confusing was the combination of a markedly elevated carcinoembryonic antigen level (107.0 ng/mL) in the thoracentesis fluid and a normal level in the serum. In one study of 105 patients, a carcinoembryonic antigen level greater than 10 ng/mL in pleural fluid was 100% diagnostic of malignancy [5]. Treatment of these histologically benign tumors requires surgical excision for a definitive diagnosis and symptom relief. Removal often is difficult because of dense adhesions to vital intrathoracic structures. The long-term outlook with operation is excellent, with low surgical mortality and no recurrences. Pathologically, benign teratomas are well encapsulated, usually cystic, and may be quite large (up to 1,820 g) [2]. Histologically, teratomas contain all three primordial tissue layers by definition. The simplest form is the teratodermoid (dermoid) cyst, which predominately contains ectodermal derivatives. The solid component of mature teratomas has well-differentiated elements of ectoderm (skin, hair), mesoderm (bone, fat, muscle), and endoderm (respiratory epithelium, pancreas). Islets of Langerhans are found in most pancreas sections and tend to have a predominance of somatostatin-producing D cells [6]. Immunohistochemical staining of pancreatic tissue in the tumor from the present patient also demonstrated an abundance of somatostatin-producing D cells in islets (Fig 5). In addition, our patient also had tissue with positive staining for insulin and for neuron-specific enolase. In one documented case involving a child, this endocrine tissue was functional in an insulin-producing
548
CASE REPORT WEISS CORONARY SINUS RUPTURE
Ann Thorac Surg 1994;58:548-50
Management of Difficult Coronary Sinus Rupture Steven J. Weiss, MD Albert Einstein Medical Center, Philadelphia, Pennsylvania
Fig 5. Immunohistochemical stain of pancreatic tissue from the tumor demonstrates abundant somatostatin-producing 0 cell, (dark staining cells) in the periphery of the islets of Langerhans. (X150 before 51 % reduction.)
teratoma causing hypoglycemia and an elevated plasma immunoreactive insulin level, which resolved after removal of the tumor [7]. Malignant teratomas occur in 19% to 44% of the total group of tumors and diller from their benign counterpart by having embryonic tissue with less well differentiated structures, less cystic components, and a uniformly dismal prognosis [81. The histogenesis of mediastinal teratomas is quite controversial, but most authorities agree that they are not metastatic from primary gonadal tumors. Some postulate that these neoplasms arise from primordial germ cells left behind during migration from the yolk sac to the urogenital ridge, whereas others believe that this tumor has its origin in faulty embryogenesis of the thymus [8]. Whatever their origin, benign mediastinal teratomas are eminently curable with operation in spite of unusual, dramatic presentations. Interestingly, in the most current series from the Mayo Clinic, there has been an apparent decline in the frequency of this unusual tumor in recent years [2].
References 1. Davis RD, Oldham HN, Sabiston DC. Primary cysts and neoplasms of the mediastinum: recent changes in clinical presentation, methods of diagnosis, management, and results. Ann Thorac Surg 1987;44:229-37. 2. Lewis BD, Hurt RD, Payne WS, Farrow GM, Knapp RH, Muhm JR. Benign teratomas of the mediastinum. J Thorac Cardiovasc Surg 1983;86:727-31. 3. Shackelford DG, McAlister WHo Mediastinal teratoma confused with loculated pleural fluid. Pediatr RadioI1976;5:118-9. 4. Suzuki M, Takashima T, Itoh H, Choutoh S, Kawamura I, Watanabe Y. Computed tomography of mediastinal teratomas. J Comput Assist Tomogr 1983;7:74-6. 5. Vladutiu AO. Carcinoembryonic antigen in pleural effusions. Lancet 1978;2:423-4. 6. Dunn PJS. Pancreatic endocrine tissue in benign mediastinal teratoma. J Clin PathoI1984;37:1105-9. 7. Honicky RE, dePapp EW. Mediastinal teratoma with endocrine function. Am J Dis Child 1973;126:650-3. 8. Pachter MR, Lattes R. "Germinal" tumors of the mediastinum: a clinicopathologic study of adu!t teratomas, teratocarcinomas, choriocarcinomas and seminomas. Dis Chest 1964;45:301-10. © 1994 by The Society of Thoracic Surgeons
A technique of handling severe coronary sinus rupture when direct suture repair or pericardial patch techniques have failed is described. A pericardial patch is sewn over the coronary sinus with running 5-0 Prolene suture, and an 8-mm thin-walled Gore-Tex conduit then is constructed between the patch and the right atrium to decompress the repair and allow an alternative route for coronary sinus return to the right atrium. (Ann Thorae Surg 1994;58:548-50)
T
he current incidence of coronary sinus injury has been estimated at 0.6% [1]. As the technique for cannulation of the coronary sinus for administration of retrograde cardioplegia becomes more widespread, it is likely that the occurrence of coronary sinus injury also will increase. Engineering refinements of the cannulas and increasing expertise will likely reduce but not eliminate this occurrence. A 70-year-old man with a past medical history of inferoposterior myocardial infarction, paroxysmal atrial fibrillation, abdominal aortic aneurysm, and chronic renal insufficiency presented with crescendo angina refractory to medications. Cardiac catheterization revealed occlusion of a dominant right coronary artery and severe left anterior descending and large diagonal lesions not amenable to angioplasty. Left ventricular function was mildly diminished and there was a 1 + to 2 + aortic insufficiency. He underwent triple coronary bypass: saphenous veins to the posterior descending coronary artery and diagonal coronary artery, and left internal mammary artery to the left anterior descending artery. My standard myocardial protection technique is continuous warm retrograde blood cardioplegia. During the operation the retrograde cannula was inserted easily through a high right atrial pursestring into the coronary sinus. A 15F self-inflating balloon cannula (RCSP catalog no. 94415; DLP Inc, Grand Rapids, MI) was used. At no time did the pressure measured in the coronary sinus exceed the recommended 40 mm Hg safe limit [2]. During the procedure, however, cardioplegia was visualized escaping from the coronary sinus just before its insertion into the right atrium due to a burst at the site of the balloon, with the balloon protruding through the rupture. Accepted for publication Nov 13, 1993. Address reprints to Dr Weiss, Albert Einstein Medical Center, 5501 Old York Rd, HB-3, Philadelphia, PA 19141.
0003-4975/94/$7.00