Infected mature teratoma of lower posterior mediastinum
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《美国医学杂志》
Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi-110002, India
A six-year-old girl was referred to us for management of right-sided chronic empyema thoracis. She was apparently well till 1 months ago, when she developed high-grade fever of sudden onset, chest pain and respiratory distress. A local physician drained two liters of pus through closed tube thoracotomy and but she continued to be symptomatic. There was no history of dysphagia, hemoptysis, expectoration, or significant weight loss. There was no past or family history of tuberculosis. On examination, the child was febrile and breath sounds were absent in the right infrascapular region. Routine investigations were normal; tuberculosis was excluded. The radiological investigations that were done included chest roentgenogram and CT scan of chest. Regretfully, the diagnosis was missed on both radiological investigations preoperatively. The chest roentgenogram had revealed multiple calcifications (teeth) in the right paravertebral gutter. CT scan had revealed a well-encapsulated large posterior mediastinal mass growing into right hemithorax. This mass had heterogeneous attenuation of soft tissue, fluid, fat and calcium Figure1. This was initially dismissed as loculated empyema thoracis with collapse consolidation of right lower lobe and nodular hilar calcifications.
Right lateral thoracotomy revealed a well-encapsulated cyst measuring 10 × 10 cm containing 200 ml. of turbid foul smelling fluid and a highly differentiated tumor that resembled a mandible with nine well-defined teeth, a well formed tongue and identifiable tonsillar fossae, hyoid bone and laryngeal inlet. The tumor was excised completely. Histopathology confirmed the tumor to be a mature teratoma. The serum alpha-fetoprotein levels done postoperatively were in normal range.
The postoperative period was complicated by a spontaneous esophageal perforation a week after the thoracotomy. The child was managed with I/V fluids, antibiotics and feeding jejunostomy. The esophageal perforation closed spontaneously after 7 weeks. The child is under close follow up for four years now; she is doing well with no evidence of recurrence of the tumor or any esophageal problem.
Benign teratomas of the mediastinum are rare and account for approximately 8% of all tumors and 75% of primary germ cell tumor of the region. The majority of the teratomas are located anteriorly; only 2-8% of these arise from the posterior mediastinum. A total of 22 such cases were reviewed by Magu, Rattan and Mishra 5 years ago.[1] An extensive review of English literature revealed at least 16 more cases of the entity from world literature. [2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15] Of these cases, at least 6 cases presented in adulthood.
The various speculations regarding the origin of the posterior mediastinal teratomas include yolk sac endoderm in the gonad during early embryogenesis, thymus, notochordal remnants, lower branchial arches, and the epidural region. [10], [13]
Posterior mediastinal teratoma commonly presents with pain and dyspnea due to mass effect, chest wall deformity, or repeated episodes of pneumonia. Features of spinal cord compression may be present.[3],[13] Occasionally, there may be an abdominal extension of the tumor.[1], [9] The tumor is notorious for its being totally asymptomatic for many years in occasional patients.[12] The preoperative differential diagnosis includes neurogenic tumors, liposarcoma, neuroenteric cyst, extramedullary hematopoiesis or extralobar pulmonary sequestration.[10],[11],[15]
Posterior mediastinal teratomas frequently involve mediastinal structures such as the esophagus, aorta and chest wall, thus mandating a thorough preoperative evaluation[1]. A chest roentgenogram may demonstrate mediastinal mass, mediastinal widening, calcifications and teeth. CT scan could confirm the diagnosis by demonstrating a well-defined heterogeneous mass containing soft-tissue, fluid, fat and calcium attenuation. At times, the mass could be cystic.[7],16 MRI could be even better in delineating the mass and the surgical planes.
The tumor can be easily excised through a lateral thoracotomy. Intra-spinal extension and extensive pleural adhesions preclude the use of thoracoscopic technique in most of the cases. Late recurrence is known to occur rarely; so long term follow-up is mandatory. Although majority of the teratomas in this location are 'mature' on histopathology, 'immature' teratoma with its predisposition of blood-borne metastasis has also been reported.[14]
The authors suggest a high index of suspicion of the entity in a patient whose lateral chest radiograph shows 'clustered' calcifications in posterior mediastinum.
References
1. Magu S, Rattan KN, Mishra DS. Posterior mediastinal teratomas. Indian J Pediatr 2000; 67 : 236-240.
2. Philip WP, Harrison K, Cruickshank DB. A posterior mediastinal dermoid tumor with marked anatomical differentiation. Thorax 1954; 9 : 245-247.
3. Wychulis AR, Payne WS, Clagget OT, Woolner LB. Surgical treatment of mediastinal tumors. J Thorac Cardiovasc Surg 1971; 62 : 379-391.
4. Kolandaivelu G, Govindan R. Posterior mediastinal teratoma with spinal compression. Indian J Chest Dis Allied Sci 1981; 23 : 44-46.
5. Lewis BD, Hurt RD, Payne SW, Farrow GM, Knapp RH, Muhm RJ. Benign teratomas of the mediastinum. J Thorac Cadiovasc Surg 1983; 86 : 727-731.
6. Husain A, Kernec J, Rioux CL, Gandon Y. Uncommon aspects of mediastinal teratoma. CT, X ray and MRI study. J Radiol 1989; 70 : 569-571.
7. Harun MH, Yaacob I. Congenital posterior mediastinal teratoma-a case report. Singapore Med J 1993; 34 : 567-568.
8. Catania A, D'Andrea V, Panzironi G, Biancari F, De Antoni E. Conservative surgical treatment for a giant thoracoabdominal benign teratoma. Acta Chir Belg 1997; 97 : 130-132.
9. Moeller KH, Rosado-de-Christenson ML, Templeton PA. Mediastinal mature teratoma: imaging features. Am J Roentgenol 1997; 169 : 985-990.
10. Massie RJ, van Asperen PP, Mellis CM. A review of open biopsy for mediastinal masses. J Paediatr Child Health 1997; 33 : 230-233.
11. Kurosaki Y, Tanaka YO, Itai Y. Mature teratoma of the posterior mediastinum. Eur Radiol 1998; 8 : 100-102.
12. Kaneko M, Ohkawa H, Iwakawa M, Ikebukuro K. Extensive epidural teratoma in early infancy treated by multi-stage surgery. Pediatr Surg Int 1999; 15 : 280-283.
13. van Echten J, de Jong B, Sinke RJ, Weghuis DO, Sleijfer DT, Oosterhuis JW. Definition of a new entity of malignant extragonadal germ cell tumors. Genes Chromosomes Cancer 1995; 12 : 8-15.
14. Sinclair DS, Bolen MA, King MA. Mature teratoma within the posterior mediastinum. J Thorac Imaging 2003; 18 : 53-55.
15. Unnikrishnan M, Kundu M, Sandhyamani S, Gadhinglajkar SV. Giant posterior mediastinal benign cystic teratomatous ganglioneurofibro-lipoma in the first decade. Eur J Cardiothorac Surg 2004; 26 : 823.(Sarin YK, Sinha A, Sengar)
A six-year-old girl was referred to us for management of right-sided chronic empyema thoracis. She was apparently well till 1 months ago, when she developed high-grade fever of sudden onset, chest pain and respiratory distress. A local physician drained two liters of pus through closed tube thoracotomy and but she continued to be symptomatic. There was no history of dysphagia, hemoptysis, expectoration, or significant weight loss. There was no past or family history of tuberculosis. On examination, the child was febrile and breath sounds were absent in the right infrascapular region. Routine investigations were normal; tuberculosis was excluded. The radiological investigations that were done included chest roentgenogram and CT scan of chest. Regretfully, the diagnosis was missed on both radiological investigations preoperatively. The chest roentgenogram had revealed multiple calcifications (teeth) in the right paravertebral gutter. CT scan had revealed a well-encapsulated large posterior mediastinal mass growing into right hemithorax. This mass had heterogeneous attenuation of soft tissue, fluid, fat and calcium Figure1. This was initially dismissed as loculated empyema thoracis with collapse consolidation of right lower lobe and nodular hilar calcifications.
Right lateral thoracotomy revealed a well-encapsulated cyst measuring 10 × 10 cm containing 200 ml. of turbid foul smelling fluid and a highly differentiated tumor that resembled a mandible with nine well-defined teeth, a well formed tongue and identifiable tonsillar fossae, hyoid bone and laryngeal inlet. The tumor was excised completely. Histopathology confirmed the tumor to be a mature teratoma. The serum alpha-fetoprotein levels done postoperatively were in normal range.
The postoperative period was complicated by a spontaneous esophageal perforation a week after the thoracotomy. The child was managed with I/V fluids, antibiotics and feeding jejunostomy. The esophageal perforation closed spontaneously after 7 weeks. The child is under close follow up for four years now; she is doing well with no evidence of recurrence of the tumor or any esophageal problem.
Benign teratomas of the mediastinum are rare and account for approximately 8% of all tumors and 75% of primary germ cell tumor of the region. The majority of the teratomas are located anteriorly; only 2-8% of these arise from the posterior mediastinum. A total of 22 such cases were reviewed by Magu, Rattan and Mishra 5 years ago.[1] An extensive review of English literature revealed at least 16 more cases of the entity from world literature. [2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15] Of these cases, at least 6 cases presented in adulthood.
The various speculations regarding the origin of the posterior mediastinal teratomas include yolk sac endoderm in the gonad during early embryogenesis, thymus, notochordal remnants, lower branchial arches, and the epidural region. [10], [13]
Posterior mediastinal teratoma commonly presents with pain and dyspnea due to mass effect, chest wall deformity, or repeated episodes of pneumonia. Features of spinal cord compression may be present.[3],[13] Occasionally, there may be an abdominal extension of the tumor.[1], [9] The tumor is notorious for its being totally asymptomatic for many years in occasional patients.[12] The preoperative differential diagnosis includes neurogenic tumors, liposarcoma, neuroenteric cyst, extramedullary hematopoiesis or extralobar pulmonary sequestration.[10],[11],[15]
Posterior mediastinal teratomas frequently involve mediastinal structures such as the esophagus, aorta and chest wall, thus mandating a thorough preoperative evaluation[1]. A chest roentgenogram may demonstrate mediastinal mass, mediastinal widening, calcifications and teeth. CT scan could confirm the diagnosis by demonstrating a well-defined heterogeneous mass containing soft-tissue, fluid, fat and calcium attenuation. At times, the mass could be cystic.[7],16 MRI could be even better in delineating the mass and the surgical planes.
The tumor can be easily excised through a lateral thoracotomy. Intra-spinal extension and extensive pleural adhesions preclude the use of thoracoscopic technique in most of the cases. Late recurrence is known to occur rarely; so long term follow-up is mandatory. Although majority of the teratomas in this location are 'mature' on histopathology, 'immature' teratoma with its predisposition of blood-borne metastasis has also been reported.[14]
The authors suggest a high index of suspicion of the entity in a patient whose lateral chest radiograph shows 'clustered' calcifications in posterior mediastinum.
References
1. Magu S, Rattan KN, Mishra DS. Posterior mediastinal teratomas. Indian J Pediatr 2000; 67 : 236-240.
2. Philip WP, Harrison K, Cruickshank DB. A posterior mediastinal dermoid tumor with marked anatomical differentiation. Thorax 1954; 9 : 245-247.
3. Wychulis AR, Payne WS, Clagget OT, Woolner LB. Surgical treatment of mediastinal tumors. J Thorac Cardiovasc Surg 1971; 62 : 379-391.
4. Kolandaivelu G, Govindan R. Posterior mediastinal teratoma with spinal compression. Indian J Chest Dis Allied Sci 1981; 23 : 44-46.
5. Lewis BD, Hurt RD, Payne SW, Farrow GM, Knapp RH, Muhm RJ. Benign teratomas of the mediastinum. J Thorac Cadiovasc Surg 1983; 86 : 727-731.
6. Husain A, Kernec J, Rioux CL, Gandon Y. Uncommon aspects of mediastinal teratoma. CT, X ray and MRI study. J Radiol 1989; 70 : 569-571.
7. Harun MH, Yaacob I. Congenital posterior mediastinal teratoma-a case report. Singapore Med J 1993; 34 : 567-568.
8. Catania A, D'Andrea V, Panzironi G, Biancari F, De Antoni E. Conservative surgical treatment for a giant thoracoabdominal benign teratoma. Acta Chir Belg 1997; 97 : 130-132.
9. Moeller KH, Rosado-de-Christenson ML, Templeton PA. Mediastinal mature teratoma: imaging features. Am J Roentgenol 1997; 169 : 985-990.
10. Massie RJ, van Asperen PP, Mellis CM. A review of open biopsy for mediastinal masses. J Paediatr Child Health 1997; 33 : 230-233.
11. Kurosaki Y, Tanaka YO, Itai Y. Mature teratoma of the posterior mediastinum. Eur Radiol 1998; 8 : 100-102.
12. Kaneko M, Ohkawa H, Iwakawa M, Ikebukuro K. Extensive epidural teratoma in early infancy treated by multi-stage surgery. Pediatr Surg Int 1999; 15 : 280-283.
13. van Echten J, de Jong B, Sinke RJ, Weghuis DO, Sleijfer DT, Oosterhuis JW. Definition of a new entity of malignant extragonadal germ cell tumors. Genes Chromosomes Cancer 1995; 12 : 8-15.
14. Sinclair DS, Bolen MA, King MA. Mature teratoma within the posterior mediastinum. J Thorac Imaging 2003; 18 : 53-55.
15. Unnikrishnan M, Kundu M, Sandhyamani S, Gadhinglajkar SV. Giant posterior mediastinal benign cystic teratomatous ganglioneurofibro-lipoma in the first decade. Eur J Cardiothorac Surg 2004; 26 : 823.(Sarin YK, Sinha A, Sengar)