- Email: [email protected]
Cavitating primary pulmonary fibrosarcoma in a child
932
Brief communications
The Journal of Thoracic and Cardiovascular Surgery
(especially if with hemoptysis) in excess of that expected from an aspirated pin may be suggestive of a blowgun dart aspiration.
2
3
4
5
6
7
8
9
REFERENCES Vander Salm TJ: Airway obstruction and foreign bodies, Manual of Clinical Problems in Surgery, BS Cutler, TF Dodson, WE Silva, TJ Vander Salm, eds., Boston, 1984, Little, Brown & Company, pp 390-392 Abdulmajid OA, Ebeid AM, Motaweh MM, Kleibo IS: Aspirated foreign bodies in the tracheobronchial tree. Report of 250 cases. Thorax 31:635-640, 1976 Aytac A, Yurdakul Y, Ikizler C, Olga R, Saylam A: Inhalation of foreign bodies in children. Report of 500 cases. J THORAC CARDIOVASC SURG 74:145-151, 1977 Black RE, Choi KJ, Syme WC, Johnson DG, Matlak ME: Bronchoscopic removal of aspirated foreign bodies in children. Am J Surg 148:778-781, 1984 Blazer S, Naveh Y, Friedman A: Foreign body in the airway. A review of 200 cases. Am J Dis Child 134:68-71, 1980 Daniilidis J, Symeonidis B, Triaridis K, Kouloulas A: Foreign body in the airways. A review of 90 cases. Arch Otolaryngol 103:570-573, 1977 Rajendran KM, Satyanand S: An unusual case of live fish obstructing the airway. A case report. J Anaesth 38:492493, 1966 Saw HS, Ganendran A, Somasundaram K: Fogarty catheter extraction of foreign bodies from tracheobronchial trees of small children. J THORAC CARDIOVASC SURG 77:240-242, 1979 Vaheri EA, Tammisto T, Tarkkanen L: Foreign bodies in the lower respiratory tract. A statistical review. Acta Otolaryngol (Stockh) (PSuppl) 224:423-428, 1966
Fig. 1. Anteroposterior (A) and lateral (B) chest radiographs of the mass, which appeared to be a multiloculated cystic structure with air-fluid levels.
Cavitating primary pulmonary fibrosarcoma in a child Jane F. Goldthorn, M.D., Marilyn H. Duncan, M.D., Ann M. Kosloske, M.D., and William S. Ball, M.D.,
Albuquerque, N. M. From the Departments of Surgery, Pediatrics, and Pathology, The University of New Mexico Hospital, Albuquerque, N. M.
Primary pulmonary sarcomas are rare tumors at all ages. They are usuallysolidand often remain silent until large. Prognosisis related to size and histologic characteristics. Curative efforts havebeendirected toward completesurgical removal. Presented Address for reprints: Jane F. Goldthorn, M.D., Division of Pediatric Surgery, Department of Surgery, Texas Tech University, Health Science Center, Lubbock, Texas 79430.
in this report is an ll-year-old girl who was thought to have a bronchogenic cyst. At operation a 14 em cavitating primary pulmonary fibrosarcoma was found, which was incompletely resected. The combined treatment modalities of surgical therapy and chemotherapy have resulted in a disease-free period of 36 months. Primary pulmonary sarcomas are rare tumors in adults and children.!" They are often asymptomatic until they reach a large size. Regardless of cell origin, these tumors are usually solid, well-circumscribed masses. Cavitation within pulmonary sarcomas is infrequent. 4 Presented in this report is the case history of a young girl who had the preoperative diagnosis of bronchogenic cyst but, at operation, was found to have a cavitating primary pulmonary fibrosarcoma.
Volume 91
Brief communications 9 3 3
Number 6 June. 1986
,
J'
..
~
.~
a Fig. 2. Representative section of the highly cellular neoplasm composed of monotonous spindle-shaped cells (hematoxylin and eosin stain; original magnification XIOO).
Case report. An II-year-old Hispanic girl was considered well until she coughed up brownish-red material during an upper respiratory tract infection. Chest radiographs demonstrated a fluid-filled, multiloculated structure, 14 cm in diameter, in the lower lobe of the right lung (Fig. I). Bronchoscopic and esophagoscopic studies were performed at the referring hospital, but no site of bleeding was identified. Three weeks later she was electivelyadmitted for excisionof the mass. At that time she still had an occasional cough but denied recurrence of the hemoptysis, chest pain, anorexia, or weight loss. She was a well-developed, active child who appeared healthy. The only abnormality was diminished breath sounds along the right lateral part of the chest. The complete blood count, serum electrolytes, glucose, creatinine, bloodurea nitrogen, serum glutamic-oxaloacetic transaminase, alkaline phosphatase, total protein, and total bilirubin values were all within normal limits. Radiographically, there was increased density in the central portion of the lung mass. On barium esophagogram, the esophagus was deviated anteriorly and to the left by the mass. A clinical diagnosis of bronchogenic cyst was made. At exploratory thoracotomy thick glistening pleura was encountered before entry into the pulmonary cavity. Extensive adhesions bound atelectatic areas of the right upper and middle lobes to the mass, which was located in the superior segment of the right lower lobe. The mass was found to be an encapsulated solid tumor, densely adherent to the retropleural space. A frozen section of fragments from the capsule demonstrated an anaplastic malignant tumor. Necrotic, semisolid material filled the central portion of the tumor. Right lower lobectomy, carinallymph node biopsies, and transdiaphragmatic needle biopsy of the liver in an area of palpable nodularity were performed. All gross evidence of tumor was removed, but microscopically disease-free margins were not achieved where the tumor bordered the esophagus. The surgical specimen contained a mass 8 ern in diameter. On cut section there was a lobular wall of tumor with a central cavity. Microscopically, the mass was a highly cellular,
malignant neoplasm composed of primitive spindle-shaped cells that were uniform in size, shape, and nuclear appearance (Fig. 2). The mitotic count was up to 3.7 figures per high-power field. There was cystic and hemorrhagic degeneration within the central portion of the tumor. On electron microscopic study, the tumor cells had copious cytoplasm poorly filled by ribosomes, small mitochondria, profiles of Golgi bodies, individually large but sparse profiles of roughsurfaced endoplasmic reticulum, and scattered lysosomal dense bodies. The cells appeared to be primitive mesenchymal cells with some fibroblastic differentiation. No tumor was found in the lymph nodes or liver biopsy tissue. The postoperative staging studies included computed tomography of the brain, lungs, and liver, ultrasonography of the abdomen, bone survey and scan, liver-spleen scan, and a second bronchoscopy. No additional foci of tumor were identified. She recovered rapidly from the operation and was discharged 10 days later. Chemotherapy was begun within 2 weeks after the operation and was maintained for 18 months with alternating monthly cyclesof the following: vincristine, 2 mg intravenously (2 mg/m 2/dose) and doxorubicin (Adriamycin), 60 mg intravenously (50 mg/m'/dose), vincristine and cyclophosphamide (Cytoxan), 900 mg intravenously (800 mg/m'/dose), and vincristine and actinomycin D, 2.3 mg intravenously (2 mg/m'/dose), single dose. She has been on no treatment regimen for 18 months and has remained disease free, 36 months after diagnosis.
Discussion. Primary pulmonary sarcomas most often develop in middle-aged or elderly adults, although their occurrence in children is well documented.!" Dyspnea, cough, hemoptysis, and chest pain are the usual symptoms. 1-3 Occasionally a small tumor, one that is less than 10 em in diameter, is asymptomatic. Radiographically, pulmonary sarcomas are smooth, sharply defined, single or lobulated masses. Although cavitating leiomyosarco-
934
The Journal of Thoracic and Cardiovascular Surgery
Brief communications
mas have been observed, this appearance is unusual and has not been previously documented in a primary pulmonary fibrosarcoma.' The prognosis of a primary pulmonary fibrosarcoma or leiomyosarcoma depends on the size of the tumor, its histologic characteristics, and the completeness with which it can be excised.v' Age and sex distribution are less important factors in determining survival. Nascimento, Unni, and Bematz' reported that all of their patients with tumors larger than 5 em in diameter died. Hartman and Shockat ' also found that leiomyosarcomas in children have a better prognosis than in adults. In the series of patients reported by Guccion and Rosen,' small leiomyosarcomas in children behaved benignly. These tumors, when small, usually demonstrated a high grade of malignancy and caused death through metastatic disease. The larger tumors resembled mesotheliomas and seldom metastasized, but caused death by local invasion.3 In a report from the Intergroup Rhabdomyosarcoma Study, Crist and associates' also found that survival in their patients with intrathoracic sarcomas correlated with the stage of the tumor at diagnosis. Patients with intrathoracic tumors commonly had only subtotal resections in which gross disease remained in the primary site. In other reported series, the best opportunity for cure or palliation was by complete surgical excision.>' Combined modality therapy, including surgical therapy, chemotherapy, and radiotherapy, is currently being used in the treatment of childhood intrathoracic sarcomas.' Because intrapulmonary sarcomas are extremely rare, the role of chemotherapy and radiotherapy in management of these tumors has not yet been systematically evaluated. Holland-Meritz and Heyn' recently reported the occurrence of a fatal cystic pulmonary blastoma in a 4-year-old child. This tumor in their patient was originally thought to be a pneumatocele that appeared when the child was 6 weeks of age. In the young girl in this report, the initial clinical and radiographic presentations of the pulmonary mass were consistent with a congenital cyst of foregut origin, such as a bronchogenic cyst.6 The surgical discovery of a highly malignant cavitating primary pulmonary fibrosarcoma further strongly illustrates that cystic intrapulmonary masses in children should not be treated with complacency. Although there are a few long-term survivors who had large intrapulmonary fibrosarcomas, the usual course is death within 2 years of diagnosis, particularly if the tumor is incompletely resected.' Our patient has had no evidence of local recurrence or distant metastases 3 years after surgical resection of grossly visible disease and an aggressive chemotherapy program. Combined
treatment with surgical therapy and chemotherapy may offer hope for cure in children with primary pulmonary sarcoma.
2
3 4
5 6
REFERENCES Hartman GE, Shockat Sl: Primary pulmonary neoplasms of childhood. A review. Am Thorac Surg 36:108-119, 1983. Crist WM, Raney RB lr, Newton W, Lawrence W. Tefft M, Foulkes MD: Intrathoracic soft tissue sarcomas in children. Cancer 50:598-604, 1982. Nascimento AG, Unni KK, Bernatz PE: Sarcomas ofthe lung. Mayo Clin Proc 57:355-359, 1982 Guccion lG, Rosen SH: Bronchopulmonaryleiomyosarcoma and fibrosarcoma. Cancer 30:836-847, 1972 Holland-Moritz RM, Heyn RM: Pulmonary blastoma associated with cystic lesions in children. Med Pediatr Oncol 12:85-88, 1984 Ravitch MM, Sabiston DC: Mediastinal infections, cysts, and tumors, Pediatric Surgery, ed 3, MM Ravitch, ed., Chicago, 1979, Year Book Medical Publishers, Inc., pp 492-512.
Reoperation for prosthetic valve degeneration after Konno aortoventriculoplasty Brian McIntyre, M.D., Robert A. Guyton, M.D., Ellis L. Jones, M.D., Joseph M. Craver, M.D., Willis H. Williams, M.D., and Charles R. Hatcher, Jr., M.D., Atlanta, Ga. From the Henrietta Egleston Hospital for Children, the Emory University Hospital, and the Carlyle Fraser Heart Center, Crawford W. Long Hospital, Department ofSurgery, Emory University School of Medicine, Atlanta, Ga. Since the introductionof the aortoventriculoplasty procedure by Konnoin 1975, 16 patients haveundergone aortic root augmentation by this method at the Woodndf Medical Center of Emory University. In four patients bioprostheticvalve degeneration 2 to 5 years after the Konno procedure bas necessitated repeat operation and replacementof the prosthetic aortic valve. Modificationof the septal patch or repeat right ventriculotomy hasnot been required.One patient requiredan additionallateral anul~rgement procedure(Manouguian) whereas the other three have demonstrated stability or growth of the aortic anulus. We have not observed recurrent subvaIvuIar stenosisor problems with the right ventricular outflow tract. Repeat operation after aortoventriculoplasty bas been relatively simple and bas usually involved only the supra-annular aorta. Address for reprints: Robert A. Guyton, M.D., Carlyle Fraser Heart Center, Crawford W. Long Memorial Hospital, 25 Prescott St., N.E., Atlanta, Ga. 30308.
Brief communications
The Journal of Thoracic and Cardiovascular Surgery
(especially if with hemoptysis) in excess of that expected from an aspirated pin may be suggestive of a blowgun dart aspiration.
2
3
4
5
6
7
8
9
REFERENCES Vander Salm TJ: Airway obstruction and foreign bodies, Manual of Clinical Problems in Surgery, BS Cutler, TF Dodson, WE Silva, TJ Vander Salm, eds., Boston, 1984, Little, Brown & Company, pp 390-392 Abdulmajid OA, Ebeid AM, Motaweh MM, Kleibo IS: Aspirated foreign bodies in the tracheobronchial tree. Report of 250 cases. Thorax 31:635-640, 1976 Aytac A, Yurdakul Y, Ikizler C, Olga R, Saylam A: Inhalation of foreign bodies in children. Report of 500 cases. J THORAC CARDIOVASC SURG 74:145-151, 1977 Black RE, Choi KJ, Syme WC, Johnson DG, Matlak ME: Bronchoscopic removal of aspirated foreign bodies in children. Am J Surg 148:778-781, 1984 Blazer S, Naveh Y, Friedman A: Foreign body in the airway. A review of 200 cases. Am J Dis Child 134:68-71, 1980 Daniilidis J, Symeonidis B, Triaridis K, Kouloulas A: Foreign body in the airways. A review of 90 cases. Arch Otolaryngol 103:570-573, 1977 Rajendran KM, Satyanand S: An unusual case of live fish obstructing the airway. A case report. J Anaesth 38:492493, 1966 Saw HS, Ganendran A, Somasundaram K: Fogarty catheter extraction of foreign bodies from tracheobronchial trees of small children. J THORAC CARDIOVASC SURG 77:240-242, 1979 Vaheri EA, Tammisto T, Tarkkanen L: Foreign bodies in the lower respiratory tract. A statistical review. Acta Otolaryngol (Stockh) (PSuppl) 224:423-428, 1966
Fig. 1. Anteroposterior (A) and lateral (B) chest radiographs of the mass, which appeared to be a multiloculated cystic structure with air-fluid levels.
Cavitating primary pulmonary fibrosarcoma in a child Jane F. Goldthorn, M.D., Marilyn H. Duncan, M.D., Ann M. Kosloske, M.D., and William S. Ball, M.D.,
Albuquerque, N. M. From the Departments of Surgery, Pediatrics, and Pathology, The University of New Mexico Hospital, Albuquerque, N. M.
Primary pulmonary sarcomas are rare tumors at all ages. They are usuallysolidand often remain silent until large. Prognosisis related to size and histologic characteristics. Curative efforts havebeendirected toward completesurgical removal. Presented Address for reprints: Jane F. Goldthorn, M.D., Division of Pediatric Surgery, Department of Surgery, Texas Tech University, Health Science Center, Lubbock, Texas 79430.
in this report is an ll-year-old girl who was thought to have a bronchogenic cyst. At operation a 14 em cavitating primary pulmonary fibrosarcoma was found, which was incompletely resected. The combined treatment modalities of surgical therapy and chemotherapy have resulted in a disease-free period of 36 months. Primary pulmonary sarcomas are rare tumors in adults and children.!" They are often asymptomatic until they reach a large size. Regardless of cell origin, these tumors are usually solid, well-circumscribed masses. Cavitation within pulmonary sarcomas is infrequent. 4 Presented in this report is the case history of a young girl who had the preoperative diagnosis of bronchogenic cyst but, at operation, was found to have a cavitating primary pulmonary fibrosarcoma.
Volume 91
Brief communications 9 3 3
Number 6 June. 1986
,
J'
..
~
.~
a Fig. 2. Representative section of the highly cellular neoplasm composed of monotonous spindle-shaped cells (hematoxylin and eosin stain; original magnification XIOO).
Case report. An II-year-old Hispanic girl was considered well until she coughed up brownish-red material during an upper respiratory tract infection. Chest radiographs demonstrated a fluid-filled, multiloculated structure, 14 cm in diameter, in the lower lobe of the right lung (Fig. I). Bronchoscopic and esophagoscopic studies were performed at the referring hospital, but no site of bleeding was identified. Three weeks later she was electivelyadmitted for excisionof the mass. At that time she still had an occasional cough but denied recurrence of the hemoptysis, chest pain, anorexia, or weight loss. She was a well-developed, active child who appeared healthy. The only abnormality was diminished breath sounds along the right lateral part of the chest. The complete blood count, serum electrolytes, glucose, creatinine, bloodurea nitrogen, serum glutamic-oxaloacetic transaminase, alkaline phosphatase, total protein, and total bilirubin values were all within normal limits. Radiographically, there was increased density in the central portion of the lung mass. On barium esophagogram, the esophagus was deviated anteriorly and to the left by the mass. A clinical diagnosis of bronchogenic cyst was made. At exploratory thoracotomy thick glistening pleura was encountered before entry into the pulmonary cavity. Extensive adhesions bound atelectatic areas of the right upper and middle lobes to the mass, which was located in the superior segment of the right lower lobe. The mass was found to be an encapsulated solid tumor, densely adherent to the retropleural space. A frozen section of fragments from the capsule demonstrated an anaplastic malignant tumor. Necrotic, semisolid material filled the central portion of the tumor. Right lower lobectomy, carinallymph node biopsies, and transdiaphragmatic needle biopsy of the liver in an area of palpable nodularity were performed. All gross evidence of tumor was removed, but microscopically disease-free margins were not achieved where the tumor bordered the esophagus. The surgical specimen contained a mass 8 ern in diameter. On cut section there was a lobular wall of tumor with a central cavity. Microscopically, the mass was a highly cellular,
malignant neoplasm composed of primitive spindle-shaped cells that were uniform in size, shape, and nuclear appearance (Fig. 2). The mitotic count was up to 3.7 figures per high-power field. There was cystic and hemorrhagic degeneration within the central portion of the tumor. On electron microscopic study, the tumor cells had copious cytoplasm poorly filled by ribosomes, small mitochondria, profiles of Golgi bodies, individually large but sparse profiles of roughsurfaced endoplasmic reticulum, and scattered lysosomal dense bodies. The cells appeared to be primitive mesenchymal cells with some fibroblastic differentiation. No tumor was found in the lymph nodes or liver biopsy tissue. The postoperative staging studies included computed tomography of the brain, lungs, and liver, ultrasonography of the abdomen, bone survey and scan, liver-spleen scan, and a second bronchoscopy. No additional foci of tumor were identified. She recovered rapidly from the operation and was discharged 10 days later. Chemotherapy was begun within 2 weeks after the operation and was maintained for 18 months with alternating monthly cyclesof the following: vincristine, 2 mg intravenously (2 mg/m 2/dose) and doxorubicin (Adriamycin), 60 mg intravenously (50 mg/m'/dose), vincristine and cyclophosphamide (Cytoxan), 900 mg intravenously (800 mg/m'/dose), and vincristine and actinomycin D, 2.3 mg intravenously (2 mg/m'/dose), single dose. She has been on no treatment regimen for 18 months and has remained disease free, 36 months after diagnosis.
Discussion. Primary pulmonary sarcomas most often develop in middle-aged or elderly adults, although their occurrence in children is well documented.!" Dyspnea, cough, hemoptysis, and chest pain are the usual symptoms. 1-3 Occasionally a small tumor, one that is less than 10 em in diameter, is asymptomatic. Radiographically, pulmonary sarcomas are smooth, sharply defined, single or lobulated masses. Although cavitating leiomyosarco-
934
The Journal of Thoracic and Cardiovascular Surgery
Brief communications
mas have been observed, this appearance is unusual and has not been previously documented in a primary pulmonary fibrosarcoma.' The prognosis of a primary pulmonary fibrosarcoma or leiomyosarcoma depends on the size of the tumor, its histologic characteristics, and the completeness with which it can be excised.v' Age and sex distribution are less important factors in determining survival. Nascimento, Unni, and Bematz' reported that all of their patients with tumors larger than 5 em in diameter died. Hartman and Shockat ' also found that leiomyosarcomas in children have a better prognosis than in adults. In the series of patients reported by Guccion and Rosen,' small leiomyosarcomas in children behaved benignly. These tumors, when small, usually demonstrated a high grade of malignancy and caused death through metastatic disease. The larger tumors resembled mesotheliomas and seldom metastasized, but caused death by local invasion.3 In a report from the Intergroup Rhabdomyosarcoma Study, Crist and associates' also found that survival in their patients with intrathoracic sarcomas correlated with the stage of the tumor at diagnosis. Patients with intrathoracic tumors commonly had only subtotal resections in which gross disease remained in the primary site. In other reported series, the best opportunity for cure or palliation was by complete surgical excision.>' Combined modality therapy, including surgical therapy, chemotherapy, and radiotherapy, is currently being used in the treatment of childhood intrathoracic sarcomas.' Because intrapulmonary sarcomas are extremely rare, the role of chemotherapy and radiotherapy in management of these tumors has not yet been systematically evaluated. Holland-Meritz and Heyn' recently reported the occurrence of a fatal cystic pulmonary blastoma in a 4-year-old child. This tumor in their patient was originally thought to be a pneumatocele that appeared when the child was 6 weeks of age. In the young girl in this report, the initial clinical and radiographic presentations of the pulmonary mass were consistent with a congenital cyst of foregut origin, such as a bronchogenic cyst.6 The surgical discovery of a highly malignant cavitating primary pulmonary fibrosarcoma further strongly illustrates that cystic intrapulmonary masses in children should not be treated with complacency. Although there are a few long-term survivors who had large intrapulmonary fibrosarcomas, the usual course is death within 2 years of diagnosis, particularly if the tumor is incompletely resected.' Our patient has had no evidence of local recurrence or distant metastases 3 years after surgical resection of grossly visible disease and an aggressive chemotherapy program. Combined
treatment with surgical therapy and chemotherapy may offer hope for cure in children with primary pulmonary sarcoma.
2
3 4
5 6
REFERENCES Hartman GE, Shockat Sl: Primary pulmonary neoplasms of childhood. A review. Am Thorac Surg 36:108-119, 1983. Crist WM, Raney RB lr, Newton W, Lawrence W. Tefft M, Foulkes MD: Intrathoracic soft tissue sarcomas in children. Cancer 50:598-604, 1982. Nascimento AG, Unni KK, Bernatz PE: Sarcomas ofthe lung. Mayo Clin Proc 57:355-359, 1982 Guccion lG, Rosen SH: Bronchopulmonaryleiomyosarcoma and fibrosarcoma. Cancer 30:836-847, 1972 Holland-Moritz RM, Heyn RM: Pulmonary blastoma associated with cystic lesions in children. Med Pediatr Oncol 12:85-88, 1984 Ravitch MM, Sabiston DC: Mediastinal infections, cysts, and tumors, Pediatric Surgery, ed 3, MM Ravitch, ed., Chicago, 1979, Year Book Medical Publishers, Inc., pp 492-512.
Reoperation for prosthetic valve degeneration after Konno aortoventriculoplasty Brian McIntyre, M.D., Robert A. Guyton, M.D., Ellis L. Jones, M.D., Joseph M. Craver, M.D., Willis H. Williams, M.D., and Charles R. Hatcher, Jr., M.D., Atlanta, Ga. From the Henrietta Egleston Hospital for Children, the Emory University Hospital, and the Carlyle Fraser Heart Center, Crawford W. Long Hospital, Department ofSurgery, Emory University School of Medicine, Atlanta, Ga. Since the introductionof the aortoventriculoplasty procedure by Konnoin 1975, 16 patients haveundergone aortic root augmentation by this method at the Woodndf Medical Center of Emory University. In four patients bioprostheticvalve degeneration 2 to 5 years after the Konno procedure bas necessitated repeat operation and replacementof the prosthetic aortic valve. Modificationof the septal patch or repeat right ventriculotomy hasnot been required.One patient requiredan additionallateral anul~rgement procedure(Manouguian) whereas the other three have demonstrated stability or growth of the aortic anulus. We have not observed recurrent subvaIvuIar stenosisor problems with the right ventricular outflow tract. Repeat operation after aortoventriculoplasty bas been relatively simple and bas usually involved only the supra-annular aorta. Address for reprints: Robert A. Guyton, M.D., Carlyle Fraser Heart Center, Crawford W. Long Memorial Hospital, 25 Prescott St., N.E., Atlanta, Ga. 30308.