| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
J Thorac Cardiovasc Surg 2006;132:1219-1222
© 2006 The American Association for Thoracic Surgery
Clinical-Pathologic Conference |
Washington University School of Medicine, Barnes-Jewish Hospital, Saint Louis, Mo.
Received for publication April 25, 2006; accepted for publication June 7, 2006. * Address for reprints: G. Alexander Patterson, MD, 3108 Queeny Tower, 1 Barnes-Jewish Hospital, St Louis, MO 63110. (Email: pattersona{at}wudosis.wustl.edu).
 |
Case Presentation |
|---|
 Top Case Presentation References |
|---|
She was in excellent health, with no significant past medical history. She took no medication and never had an operation. She is a lifetime nonsmoker and does not consume alcohol. On physical examination, she was a healthy appearing 20-year-old woman. She was afebrile and had no cervical or supraclavicular adenopathy. The results of her cardiac examination were normal. She had absent breath sounds on the right, with diminished chest wall excursion. Her extremity examination revealed no clubbing, cyanosis, or edema.
Dr Bhalla
Her initial chest radiograph is a dramatic demonstration of a mass extending into the right main-stem bronchus and distal trachea (Figure 1). The left main-stem bronchus appears uninvolved. There is complete collapse of the right lung, with herniation of the heart toward the right. A computed tomographic (CT) scan was obtained that shows an enhancing mass in the distal trachea and right main-stem bronchus (Figure 2). There are a few lymph nodes present around the airway, and there is another lymph node anterior to the mass in the region of the right hilum. The right lung is collapsed, and the mediastinum is shifted toward the right. In addition, there is a small pericardial effusion, pleural effusion, and some dilated bronchi.
|
|
Dr Miller
This is a classic nuclear medicine study for right bronchial obstruction. The right lung has no ventilation, and the left lung is ventilated normally. In the perfusion scan the left lung is normal, and the right lung has very diminished perfusion. The differential diagnosis for this is a central obstructing mass, foreign body aspiration, mucous plug, or fibrosing mediastinitis.
Dr Kozower
She was taken to the operating room for fiberoptic and rigid bronchoscopy. The mass completely occluded the right main-stem bronchus and was adherent to the posterior wall of the carina (Figure 3). The left main-stem bronchus was patent.
|
Dr Kozower
We took a biopsy specimen of the mass, and the frozen section diagnosis was consistent with a grade I neuroendocrine tumor or typical carcinoid. We then cored out the tumor with a rigid bronchoscope. It was difficult to establish a patent airway in the right bronchus, but there was some aeration of the right lung after the procedure. We decided to wait 2 weeks before resection to see whether the right lung ventilation would improve and to give her a chance to clear some of her secretions.
Our surgical plan was to perform a carinal resection with right upper lobe sleeve resection. The procedure began with a cervical mediastinoscopy to stage the mediastinal lymph nodes and help dissect the distal trachea and right main-stem bronchus. A right posterolateral thoracotomy was then performed. The obstructing mass extended from the posterior wall of the distal trachea to the distal bronchus intermedius. It appeared to originate from the right main-stem bronchus, and there was no way to preserve the right upper lobe. The bronchus intermedius was divided first, followed by the left main-stem bronchus and trachea. We resected the right upper lobe en bloc with the airway margins and used cross-field ventilation to facilitate the procedure. We then performed a radical mediastinal lymphadenectomy to remove any macroscopic disease. Negative airway margins were confirmed with frozen section analysis before starting the anastomoses.
We performed a right hilar release to elevate the bronchus intermedius and decrease tension on the airway anastomoses. The reconstruction was begun by sewing the left main-stem bronchus to the bronchus intermedius to create a new carina. The neocarina was then sutured to the distal trachea. Her postoperative course went quite smoothly, and she was discharged to home in 6 days. She is doing well 3 months postoperatively, and her bronchoscopy reveals a patent and viable airway with minimal granulation tissue (Figure 4).
|
|
Thirty-one lymph nodes were sent for examination, and 6 showed metastatic carcinoid tumor at levels 4R (right paratracheal) and 7 (subcarinal). Several of the positive lymph nodes had been received for frozen section analysis. Because cytologic features of carcinoid tumor are not well represented on tissue that has been frozen, confirmatory immunohistochemistry was performed on those nodes. The synaptophysin stain was strongly positive and highlights the role of neuroendocrine markers in the diagnosis of carcinoid tumor.
The spectrum of neuroendocrine tumors was classified by Dressler and associates1
on the basis of histologic characteristics and clinical behavior. Grade I neuroendocrine lung cancers correspond to classic typical carcinoid tumors. Grade II neuroendocrine lung cancers correspond most closely with atypical carcinoid cancers. Grade III tumors were divided into small cell neuroendocrine lung cancer and large cell neuroendocrine lung cancers.
Grade I neuroendocrine tumors are commonly divided into central and peripheral varieties. They are characterized histologically by the lack of cytologic atypia, low mitotic rate, and absent necrosis. Grade I neuroendocrine lung cancers can be arranged in nested architecture, ribbons, festoons, trabeculae, or papillae; show solid sheet-like growth; or both. Because central carcinoids usually have a significant intraluminal component, patients often present with wheezing, obstruction, and pneumonia. Overall, cases of typical carcinoids have an excellent prognosis. Unfavorable prognostic features include size greater than 3 cm, vessel invasion, and lymph node invasion. Lymph node metastases are rare in typical carcinoids.2
Dr Kozower
This is a classic presentation for a young woman with a carcinoid tumor. Unfortunately, she has extensive N2 disease, and there are relatively little data to predict her prognosis or help guide her treatment. Martini and associates3 published a small series on the treatment and prognosis of bronchial carcinoids involving regional lymph nodes. It was a small retrospective review of 25 patients treated between 1953 and 1992. Ten patients had N1 disease, and 15 patients had N2 disease. There were 13 atypical and 12 typical carcinoids. The 5-year actuarial survival for the whole group was relatively high at 75%. Importantly, the 5-year survival was 92% for the 12 patients with typical carcinoids, despite having positive lymph nodes. In addition, there was no difference in survival between patients with N1 disease and those with N2 disease. The authors concluded that the grade of neuroendocrine carcinoma is a more important predictor of survival than the presence of lymph node metastases. In addition, the use of adjuvant radiation therapy for N2 disease did not appear beneficial.
Our patient is 20 years old and has a grade I neuroendocrine carcinoma. However, the final pathology indicates that she has multiple-station N2 disease and a positive airway margin. How comfortable should surgeons be with frozen section analysis of the margins, and does she need any additional treatment at this time?
Dr Ritter
The margins on the frozen section slides were negative, and it is difficult to interpret the final diagnosis. If you have a truly negative margin and we cut deeper into that section to find tumor, I would argue that the margin is in fact still negative. The reality is that the margin is very close, but I am not so sure that I would consider it to be positive.
Dr Patterson
The margins in this case are extremely important, and I also think we have a negative margin. In a case like this, where the margins will alter our treatment decisions, we excise a section and orient it with a suture at the true margin. The margin in question in this case is the proximal left main bronchus. The pathologists, as Dr Ritter pointed out, sectioned the distal edge of that airway intraoperatively, and it was negative. However, when they cut deeper into the specimen, further from our margin, there was tumor. I realize that the margin was very close, but it is a difficult place to excise more bronchus when you are trying to perform a carinal sleeve resection.
The reality is that even if the margin is positive, I would not do anything about it. Technically, we cannot re-resect her. Radiation will not help because these tumors do not respond well to radiation and you would expose a carinal anastomosis to the risks of radiation therapy. I would argue that because the bronchial margin is close and because of her positive lymph nodes, she should have surveillance bronchoscopies and chest CT scans done at regular intervals. If she has a local recurrence at the anastomosis, I would be inclined to treat her endoscopically. If she had a recurrence years from now, one could argue that some sort of local resection could be attempted. I think that the bigger problem for her is her nodal metastases. She is 20 years old and has multistation N2 disease.
Dr Kozower
After reviewing the literature, it is difficult to determine how to treat her, and adjuvant therapy does not appear to be very helpful. How many patients have been treated with grade I neuroendocrine carcinoma and bulky N2 nodes? Dr Safdar, do you have any comments on the use of adjuvant therapy in this case?
Dr Safdar
I agree that we do not have good adjuvant therapy for low-grade neuroendocrine carcinomas. We have occasionally radiated some of these patients, but as Dr Patterson stated, she is not a good radiation therapy candidate.
This is an excellent example of a case that demonstrates that the histology of certain tumors can only tell you so much. There is not a single clue from the primary tumor that would predict that it is likely to metastasize. It is large, but there is no necrosis, and the mitotic rate is negligible. It illustrates that our understanding of neuroendocrine carcinomas remains imprecise.
|
Footnotes |
|---|
From the Washington University School of Medicine, Barnes-Jewish Hospital, Saint Louis, Mo.
Thoracic Surgery
Dr Benjamin D. Kozower
Dr G. Alexander Patterson
Radiology
Dr Sanjeev Bhalla
Dr Tom Miller
Surgical Pathology
Dr Robert Jarrett
Dr Jon Ritter
Medical Oncology
Dr Shabbir Safdar
|
References |
|---|
|
TopCase PresentationReferences |
|---|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| ANN THORAC SURG | ASIAN CARDIOVASC THORAC ANN | EUR J CARDIOTHORAC SURG |
| J THORAC CARDIOVASC SURG | ICVTS | ALL CTSNet JOURNALS |
