- Email: [email protected]
Obstructing endobronchial fibrous histiocytoma: Potential for lung salvage
Obstructing ByEdward
Endobronchial Fibrous Histiocytoma: Potential for Lung Salvage Tagge, Eduardo
Yunis, Jon Chopyk,
and Eugene Wiener
Pittsburgh, Pennsylvania l In the past 10 years two children, ages 3$$ and 7 years, have been seen with central endobronchial fibrous histiocytoma causing complete atelectasis. Each child had a 4- to B-month history of cough, dyspnea and fever. After antibiotic failure, x-rays demonstrated complete unilateral lung collapse. Bronchoscopy in case 1 demonstrated total occlusion of the left mainstem bronchus with 80% stenosis of the right. Left pneumonectomy was ultimately performed with removal of tumor from the right bronchus. Ten years later the patient is well. Case 2 had an extrinsic etiology ruled out by computed tomography scan. Ventilation/perfusion scan showed no ventilation and only faint perfusion of the involved lung. Pulmonary function testing showed moderate to severe restrictive changes with air trapping. Total obliteration of the left mainstem with tumor projecting into the carina was observed on bronchoscopy. Endoscopic resection was not felt to be safe. Thoracotomy showed a totally atelectatic, edematous lung filled with pus. The left main bronchus was opened, the tumor removed, and the distal bronchus lavaged and selectively ventilated. Partial lung expansion was obtained. The child is well 1% years later with a normal chest radiograph, pulmonary function tests, and bronchoscopy. Fibrous histiocytomas of the lung, if in a central endobronchial location, can cause complete lung collapse. A combination of tumor resection, lavage, and selective ventilation should be attempted as an alternative to pneumonectomy, even if the lung appears to be nonsalvageable. Copyright
o 1991 by W. B. Saunders Company
INDEX WORDS: Fibrous histiocytoma; inflammatory tumor; plasma cell granuloma; bronchoplasty.
pseudo-
F
IBROUS HISTIOCYTOMA is one of many names for an uncommon, benign tumor that occurs most commonly in young adults. Other names include inflammatory pseudotumor, plasma cell granuloma, xanthoma, and fibroxanthoma. In a literature review by Berardi et al’ of 181 inflammatory pseudotumors of the lung, only 8.1% occurred in children between 1 and 10 years of age. These lesions can occur throughout the body, most commonly in the lung. They usually reside in the lung parenchyma, but occasionally are endobronchial. Two patients presented with complete lung collapse from obstructing endobronchial fibrous histiocytomas. Their management is compared, emphasizing an aggressive approach to salvage of pulmonary tissue. CASE REPORTS
Case 1 A 31/2-year-old white boy bad a 6-month history of intermittent cough and fever. He was treated with antibiotics on numerous JournalofPediatric
Surgery,
Vol26,
No 9 (September),
1991: pp 1067-1069
occasions for presumed tonsillitis. A preoperative chest radiograph for elective tonsillectomy demonstrated complete collapse of the left lung. Bronchoscopy demonstrated total occlusion of the left mainstem bronchus and 80% compromise of the right bronchus by an endobronchial mass. Biopsy results were consistent with inflammatory pseudotumor. The patient subsequently dt?veloped respiratory distress and high fevers. He underwent urgent left pneumonectomy with removal of tumor from the right mainstem bronchus. He was discharged 1 month later. At his recent lo-year follow-up, bronchoscopy demonstrated no recurrent disease.
Case 2 A 7-year-old white boy had a 4-month history of intermittent productive cough, wheezing, and left shoulder pain. During that time he was treated with three separate courses of antibiotics and bronchodilators, with apparent improvement. On recurrence of symptoms, a chest radiograph demonstrated complete collapse of the left lung (Fig 1). A chest computed tomography (CT) scan did not visualize either an extrinsic mass or pleural fluid (Fig 2). Ventilation/perfusion scan showed no ventilation and only faint perfusion of the left lung. Pulmonary function tests showed a moderate to severe restrictive defect with air trapping (Table 1). Bronchoscopy demonstrated a mass totally occluding the left mainstem bronchus. It was not felt to be amenable to endoscopic removal. Biopsy showed spindle cells and a chronic inflammatory infiltrate consistent with a fibrous histiocytoma. At operation the right mainstem was selectively intubated with a single-lumen endotracheal tube. After left thoracotomy the lung was noted to be totally atelectatic, with marked inflammation, edema, and extensive adenopathy. A longitudinal bronchotomy was made just distal to the tumor. A large amount of thick yellow pus was suctioned from the bronchus. The tumor, which measured 2 x 2 x 1 cm, was completely removed from the bronchial wall. Frozen section showed no evidence of malignancy. The distal bronchus was cannulated with a sterile cardiac venous cannula. After extensive suctioning and irrigation, a second anesthesia machine was connected to the cannula, allowing selective ventilation of the left lung. Intermittent positive-pressure was applied by hand, slowly inflating the diseased lung. Eventually ventilation of the right lung was stopped. Blood gases obtained after 5 minutes of left lung ventilation were: pH 7.32, PO, 422, PCO, 49, HCO, 25 (SO, 100%). The bronchus was closed with interrupted nonabsorable sutures. The patient was ventilated for 1 week postoperation. Several bedside bronchoscopies were performed to selectively suction the
From the University of Pittsburgh School of Medicine and the Divisions of Pediatric Surgery, Pathology, and Anesthesiology, Children S Hospital of Pittsbutgh, Pittsburgh, PA. Presented at the 42nd Annual Meeting of the Surgical Section of the American Academy of Pediatrics, Boston, Massachusetts, October 6- 7, 1990. Address reprint requests to Eugene S. Wiener, MD, 4415 Fifth Ave, Pittsburgh, PA 15213. Copyright o 1991 by W.B. Saunders Company 0022-3468/91/2609-0012$03.00/O 1067
TAGGE ET AL
1068
Table 1. Pulmonary Function Tests of Case 2 Preoperative Observed
(L)
Studies % Predicted
Postoperative Observed (L)
Studies % Predicted
FVC
0.71
42
1.77
97
FEV,
0.66
43
1.70
104
vc
0.71
37
1.77
89
TLC
1.15
46
2.35
91
FRC
0.70
63
1.12
97
RV
0.44
77
0.58
95
Abbreviations:
FVC, forced vital capacity; FEV,, forced expiratory
volume of 1 second; VC, vital capacity; TLC, total lung capacity; FRC, functional residual capacity; RV, residual volume.
Fig 1. Preoperative chest radiograph complete collapse of the left lung.
of case 2, demonstrating
left lung. Serial chest radiographs showed gradual lung inflation. The patient was discharged 12 days postoperation. Follow-up bronchoscopies at 3 and 12 months showed no evidence of recurrence. His chest radiograph and pulmonary function tests returned to normal (Fig 3, Table 1). DISCUSSION
Bahadori and Liebow,’ in a review of 40 patients with pulmonary plasma cell granulomas, found that a quarter of the cases occurred in children less than 10 years of age. Atelectasis was the presenting finding in four children. Although the lung is the most common location for this tumor, extrapulmonary lesions have been described in other organs including uterus, bladder, pancreas, and small bower mesentery.3
Fig 2. Preoperative chest CT scan of case 2, demonstrating complete collapse of the left lung and hernietion of the right lung across the midline.
Grossly, these lesions may vary from 1 to 36 cm in diameter. They are slow growing and usually sharply defined, thus allowing their submucosal removal as in this case. Histologically, these are benign lesions with no cellular anaplasia and few mitotic figures. Mononuclear cells, particularly plasma cells, as well as spindle cells and myofibroblasts can be present. Mast cells or foamy histiocytes are found in variable numbers. This wide variety gives rise to the confusing terminology of these tumors. Therapeutic options have increased over the recent past. Prior therapy consisted almost exclusively of lobectomy or pneumonectomy,2’4 if for no other reason than because the diagnosis could not be made preoperatively. The availability, specificity, and ease of bronchoscopy have significantly decreased that problem. Most investigators agree with Mandelbaum et al: who state that once the diagnosis is made lung resection should be minimized. The rarity of tumor
Fig 3. Three-month postoperative chest radiograph demonstrating complete clearing of the left lung field.
of cese 2,
ENDOBRONCHIAL
1069
FIBROUS HISTIOCYTOMA
recurrence make this lesion, when it is endobronchial, an excellent candidate for lung salvage by endoscopic or bronchoplastic techniques. Bronchoplasty involves incision/excision of the bronchial wall, removal of the tumor with minimal if any lung tissue, and subsequent reconstruction. One-lung anesthesia, with either a double-lumen tube or selective intubation of the opposite bronchus, is essential. The repair depends on the location and extent of bronchial involvement and the condition of the distal lung. Preoperative bronchoscopy and radiologic studies, localizing the extent of the lesion, allow precise placement of the bronchotomy and construction of tissue flaps for difficult bronchial reconstructions.6 Preoperative studies are not helpful in assessing potential function of a totally collapsed lung. Gross
examination is extremely subjective and biopsy has formidable potential complications. In case 2, selective involved lung anesthesia with a second ventilator was helpful. A Sminute ventilation trial of only the diseased lung, with maintenance of adequate arterial blood gases, indicated adequate residual function. We agree with Black et al6 that only obviously unsalvageable tissue should be resected. In summary, fibrous histiocytomas, although usually found incidentally, may present with symptoms from complete lung collapse. Bronchoscopy aids in establishing the diagnosis and planning the subsequent operation. Bronchoplastic techniques with lung salvage should be attempted. Recurrence rarely occurs and long-term survival is excellent.
REFERENCES 1. Berardi RS, Lee SS, Chen HP, et al: Inflammatory pseudotumors of the lung. Surg Gynecol Obstet 156:89-96,1983 2. Bahadori M, Liebow AA: Plasma cell granulomas of the lung. Cancer 31:191-208,1973 3. Scott L, Blair G, Taylor G, et al: Inflammatory pseudotumors in children. J Pediatr Surg 23:755-758,1988 4. Scully RE: Case 10-1982, from case records of the Massachu-
setts General Hospital. Postinflammatory pseudotumor of lung (plasma-cell granuloma). N Engl J Med 306~596-602,1982 5. Mandelbaum I, Brashear RE, Hull MT: Surgical treatment and course of pulmonary pseudotumor (plasma cell granuloma). J Thorac Cardiovasc Surg 82:77-82,198l 6. Black CT, Luck SR, Raffensperger JG: Bronchoplastic techniques for pediatric lung salvage. J Pediatr Surg 23:653-656,1988
Discussion R Filler (Toronto, Ontario): I don’t think I have seen one of these fairly unusual lesions. I think that all of us can see that the authors’ learning curve was fairly rapid in terms of saving one lung through a series of procedures as compared with pneumonectomy, which was needed for the first patient. One wonders how much of this could have been done through a bronchoscope, perhaps with laser surgery. I don’t have any great feeling about how easily the
tumor came out, but it did look as if it was fairly well circumscribed and that there was not a great deal of bronchial wall damage. I wonder if endoscopic removal would have been an alternative in this case? E. Tagge (response): Endoscopic resection was considered. We did not feel that laser therapy could be performed safely due to complete bronchial occlusion and inability to determine the lesion’s relationship to the bronchial wall.
Endobronchial Fibrous Histiocytoma: Potential for Lung Salvage Tagge, Eduardo
Yunis, Jon Chopyk,
and Eugene Wiener
Pittsburgh, Pennsylvania l In the past 10 years two children, ages 3$$ and 7 years, have been seen with central endobronchial fibrous histiocytoma causing complete atelectasis. Each child had a 4- to B-month history of cough, dyspnea and fever. After antibiotic failure, x-rays demonstrated complete unilateral lung collapse. Bronchoscopy in case 1 demonstrated total occlusion of the left mainstem bronchus with 80% stenosis of the right. Left pneumonectomy was ultimately performed with removal of tumor from the right bronchus. Ten years later the patient is well. Case 2 had an extrinsic etiology ruled out by computed tomography scan. Ventilation/perfusion scan showed no ventilation and only faint perfusion of the involved lung. Pulmonary function testing showed moderate to severe restrictive changes with air trapping. Total obliteration of the left mainstem with tumor projecting into the carina was observed on bronchoscopy. Endoscopic resection was not felt to be safe. Thoracotomy showed a totally atelectatic, edematous lung filled with pus. The left main bronchus was opened, the tumor removed, and the distal bronchus lavaged and selectively ventilated. Partial lung expansion was obtained. The child is well 1% years later with a normal chest radiograph, pulmonary function tests, and bronchoscopy. Fibrous histiocytomas of the lung, if in a central endobronchial location, can cause complete lung collapse. A combination of tumor resection, lavage, and selective ventilation should be attempted as an alternative to pneumonectomy, even if the lung appears to be nonsalvageable. Copyright
o 1991 by W. B. Saunders Company
INDEX WORDS: Fibrous histiocytoma; inflammatory tumor; plasma cell granuloma; bronchoplasty.
pseudo-
F
IBROUS HISTIOCYTOMA is one of many names for an uncommon, benign tumor that occurs most commonly in young adults. Other names include inflammatory pseudotumor, plasma cell granuloma, xanthoma, and fibroxanthoma. In a literature review by Berardi et al’ of 181 inflammatory pseudotumors of the lung, only 8.1% occurred in children between 1 and 10 years of age. These lesions can occur throughout the body, most commonly in the lung. They usually reside in the lung parenchyma, but occasionally are endobronchial. Two patients presented with complete lung collapse from obstructing endobronchial fibrous histiocytomas. Their management is compared, emphasizing an aggressive approach to salvage of pulmonary tissue. CASE REPORTS
Case 1 A 31/2-year-old white boy bad a 6-month history of intermittent cough and fever. He was treated with antibiotics on numerous JournalofPediatric
Surgery,
Vol26,
No 9 (September),
1991: pp 1067-1069
occasions for presumed tonsillitis. A preoperative chest radiograph for elective tonsillectomy demonstrated complete collapse of the left lung. Bronchoscopy demonstrated total occlusion of the left mainstem bronchus and 80% compromise of the right bronchus by an endobronchial mass. Biopsy results were consistent with inflammatory pseudotumor. The patient subsequently dt?veloped respiratory distress and high fevers. He underwent urgent left pneumonectomy with removal of tumor from the right mainstem bronchus. He was discharged 1 month later. At his recent lo-year follow-up, bronchoscopy demonstrated no recurrent disease.
Case 2 A 7-year-old white boy had a 4-month history of intermittent productive cough, wheezing, and left shoulder pain. During that time he was treated with three separate courses of antibiotics and bronchodilators, with apparent improvement. On recurrence of symptoms, a chest radiograph demonstrated complete collapse of the left lung (Fig 1). A chest computed tomography (CT) scan did not visualize either an extrinsic mass or pleural fluid (Fig 2). Ventilation/perfusion scan showed no ventilation and only faint perfusion of the left lung. Pulmonary function tests showed a moderate to severe restrictive defect with air trapping (Table 1). Bronchoscopy demonstrated a mass totally occluding the left mainstem bronchus. It was not felt to be amenable to endoscopic removal. Biopsy showed spindle cells and a chronic inflammatory infiltrate consistent with a fibrous histiocytoma. At operation the right mainstem was selectively intubated with a single-lumen endotracheal tube. After left thoracotomy the lung was noted to be totally atelectatic, with marked inflammation, edema, and extensive adenopathy. A longitudinal bronchotomy was made just distal to the tumor. A large amount of thick yellow pus was suctioned from the bronchus. The tumor, which measured 2 x 2 x 1 cm, was completely removed from the bronchial wall. Frozen section showed no evidence of malignancy. The distal bronchus was cannulated with a sterile cardiac venous cannula. After extensive suctioning and irrigation, a second anesthesia machine was connected to the cannula, allowing selective ventilation of the left lung. Intermittent positive-pressure was applied by hand, slowly inflating the diseased lung. Eventually ventilation of the right lung was stopped. Blood gases obtained after 5 minutes of left lung ventilation were: pH 7.32, PO, 422, PCO, 49, HCO, 25 (SO, 100%). The bronchus was closed with interrupted nonabsorable sutures. The patient was ventilated for 1 week postoperation. Several bedside bronchoscopies were performed to selectively suction the
From the University of Pittsburgh School of Medicine and the Divisions of Pediatric Surgery, Pathology, and Anesthesiology, Children S Hospital of Pittsbutgh, Pittsburgh, PA. Presented at the 42nd Annual Meeting of the Surgical Section of the American Academy of Pediatrics, Boston, Massachusetts, October 6- 7, 1990. Address reprint requests to Eugene S. Wiener, MD, 4415 Fifth Ave, Pittsburgh, PA 15213. Copyright o 1991 by W.B. Saunders Company 0022-3468/91/2609-0012$03.00/O 1067
TAGGE ET AL
1068
Table 1. Pulmonary Function Tests of Case 2 Preoperative Observed
(L)
Studies % Predicted
Postoperative Observed (L)
Studies % Predicted
FVC
0.71
42
1.77
97
FEV,
0.66
43
1.70
104
vc
0.71
37
1.77
89
TLC
1.15
46
2.35
91
FRC
0.70
63
1.12
97
RV
0.44
77
0.58
95
Abbreviations:
FVC, forced vital capacity; FEV,, forced expiratory
volume of 1 second; VC, vital capacity; TLC, total lung capacity; FRC, functional residual capacity; RV, residual volume.
Fig 1. Preoperative chest radiograph complete collapse of the left lung.
of case 2, demonstrating
left lung. Serial chest radiographs showed gradual lung inflation. The patient was discharged 12 days postoperation. Follow-up bronchoscopies at 3 and 12 months showed no evidence of recurrence. His chest radiograph and pulmonary function tests returned to normal (Fig 3, Table 1). DISCUSSION
Bahadori and Liebow,’ in a review of 40 patients with pulmonary plasma cell granulomas, found that a quarter of the cases occurred in children less than 10 years of age. Atelectasis was the presenting finding in four children. Although the lung is the most common location for this tumor, extrapulmonary lesions have been described in other organs including uterus, bladder, pancreas, and small bower mesentery.3
Fig 2. Preoperative chest CT scan of case 2, demonstrating complete collapse of the left lung and hernietion of the right lung across the midline.
Grossly, these lesions may vary from 1 to 36 cm in diameter. They are slow growing and usually sharply defined, thus allowing their submucosal removal as in this case. Histologically, these are benign lesions with no cellular anaplasia and few mitotic figures. Mononuclear cells, particularly plasma cells, as well as spindle cells and myofibroblasts can be present. Mast cells or foamy histiocytes are found in variable numbers. This wide variety gives rise to the confusing terminology of these tumors. Therapeutic options have increased over the recent past. Prior therapy consisted almost exclusively of lobectomy or pneumonectomy,2’4 if for no other reason than because the diagnosis could not be made preoperatively. The availability, specificity, and ease of bronchoscopy have significantly decreased that problem. Most investigators agree with Mandelbaum et al: who state that once the diagnosis is made lung resection should be minimized. The rarity of tumor
Fig 3. Three-month postoperative chest radiograph demonstrating complete clearing of the left lung field.
of cese 2,
ENDOBRONCHIAL
1069
FIBROUS HISTIOCYTOMA
recurrence make this lesion, when it is endobronchial, an excellent candidate for lung salvage by endoscopic or bronchoplastic techniques. Bronchoplasty involves incision/excision of the bronchial wall, removal of the tumor with minimal if any lung tissue, and subsequent reconstruction. One-lung anesthesia, with either a double-lumen tube or selective intubation of the opposite bronchus, is essential. The repair depends on the location and extent of bronchial involvement and the condition of the distal lung. Preoperative bronchoscopy and radiologic studies, localizing the extent of the lesion, allow precise placement of the bronchotomy and construction of tissue flaps for difficult bronchial reconstructions.6 Preoperative studies are not helpful in assessing potential function of a totally collapsed lung. Gross
examination is extremely subjective and biopsy has formidable potential complications. In case 2, selective involved lung anesthesia with a second ventilator was helpful. A Sminute ventilation trial of only the diseased lung, with maintenance of adequate arterial blood gases, indicated adequate residual function. We agree with Black et al6 that only obviously unsalvageable tissue should be resected. In summary, fibrous histiocytomas, although usually found incidentally, may present with symptoms from complete lung collapse. Bronchoscopy aids in establishing the diagnosis and planning the subsequent operation. Bronchoplastic techniques with lung salvage should be attempted. Recurrence rarely occurs and long-term survival is excellent.
REFERENCES 1. Berardi RS, Lee SS, Chen HP, et al: Inflammatory pseudotumors of the lung. Surg Gynecol Obstet 156:89-96,1983 2. Bahadori M, Liebow AA: Plasma cell granulomas of the lung. Cancer 31:191-208,1973 3. Scott L, Blair G, Taylor G, et al: Inflammatory pseudotumors in children. J Pediatr Surg 23:755-758,1988 4. Scully RE: Case 10-1982, from case records of the Massachu-
setts General Hospital. Postinflammatory pseudotumor of lung (plasma-cell granuloma). N Engl J Med 306~596-602,1982 5. Mandelbaum I, Brashear RE, Hull MT: Surgical treatment and course of pulmonary pseudotumor (plasma cell granuloma). J Thorac Cardiovasc Surg 82:77-82,198l 6. Black CT, Luck SR, Raffensperger JG: Bronchoplastic techniques for pediatric lung salvage. J Pediatr Surg 23:653-656,1988
Discussion R Filler (Toronto, Ontario): I don’t think I have seen one of these fairly unusual lesions. I think that all of us can see that the authors’ learning curve was fairly rapid in terms of saving one lung through a series of procedures as compared with pneumonectomy, which was needed for the first patient. One wonders how much of this could have been done through a bronchoscope, perhaps with laser surgery. I don’t have any great feeling about how easily the
tumor came out, but it did look as if it was fairly well circumscribed and that there was not a great deal of bronchial wall damage. I wonder if endoscopic removal would have been an alternative in this case? E. Tagge (response): Endoscopic resection was considered. We did not feel that laser therapy could be performed safely due to complete bronchial occlusion and inability to determine the lesion’s relationship to the bronchial wall.