Ann Thorac Surg 2007;84:1375-1376
© 2007 The Society of Thoracic Surgeons
Case Reports
Giant Carcinoid Tumor Mimicking Pulmonary Sequestration
Guven Olgac, MD, FETCSa,*,
Farzin Peirovi, MDc,
Aynur Yilmaz, MDb,
Cemal Asim Kutlu, MD, FETCSa
a Department of Thoracic Surgery, Sureyyapasa Chest Diseases, Chest Surgery Training and Research Hospital, Istanbul, Turkey
b Department of Histopathology, Sureyyapasa Chest Diseases, Chest Surgery Training and Research Hospital, Istanbul, Turkey
c Medica Imaging Center, Istanbul, Turkey
Accepted for publication May 14, 2007.
* Address correspondence to Dr Olgac, Acibadem Mah. Tekin Sok. No: 19, Baris Sitesi, D-Blok, Daire: 8, Kadikoy, Istanbul, 34718, Turkey (Email: guvenolgac{at}gmail.com).
 |
Abstract
|
|---|
A 42-year-old woman who previously underwent two consecutive thoracotomies for a lower lobe mass in her right lung was referred to our clinic for further management. Both procedures were abandoned due to excessive bleeding. Computed tomographic angiography demonstrated an infra-diaphragmatic systemic arterial supply of the mass similar to pulmonary sequestration. However the lobe had a normal venous drainage to the left atrium. Then a right lower lobectomy was undertaken through a hemi-clamshell incision, and histopathology revealed an atypical carcinoid tumor. The patient was discharged home after a satisfactory postoperative period. She still remains disease free at 14 months follow-up.
|
Introduction
|
|---|
Bronchial carcinoid tumors originate from neuroendocrine cells of the bronchial mucosa and constitute 1% of all lung tumors [1]. Atypical carcinoids differ from typical ones owing to their atypical cytology, tendency of lymphatic invasion, derangement in the cell pattern, and significance of their nucleolus. They comprise approximately 10% of all carcinoid tumors. They usually represent as endobronchial masses of 3 to 6 cm in diameter and are associated with coughing, shortness of breath, wheezing, chest pain, and hemoptysis. Giant carcinoid tumors are rarely reported in the literature [2]. Here we report a giant atypical carcinoid tumor with a systemic arterial supply similar to pulmonary sequestration.
A 42-year-old nonsmoking woman was referred to our clinic after two consecutive thoracotomies for a large right lower lobe mass. She had been previously admitted to another hospital with a 3-year history of shortness of breath and chest pain. A chest x-ray film showed a homogeneous opacity occupying half of the right hemithorax, and a computed tomographic scan of the chest demonstrated a calcified mass lesion that was 12 cm in its maximum diameter with no signs of mediastinal invasion. Fiberoptic bronchoscopy and transthoracic fine needle aspiration failed to reveal any histologic diagnosis, and she underwent an explorative thoracotomy. However the operation was abandoned due to massive bleeding during the initial exploration. An aortography was then obtained, which had demonstrated tortuous bronchial arteries supplying the mass, and these were embolized to decrease the chance of life-threatening hemorrhage at the time of the planned second thoracotomy. Two weeks after the first operation, a re-thoracotomy was undertaken, and once again the mass was found to be unresectable owing to uncontrollable bleeding during pneumolysis. The patient denied the offered chemoradiation therapy and decided to seek a second medical opinion.
Before attempting any surgical treatment, we obtained a computed tomographic angiography to scrutinize the arterial supply of the mass. In addition to a previously documented network of tortuous systemic arteries, another artery that was unusually originating from the abdominal aorta and approaching the mass through the diaphragm was determined (Fig 1). This new finding also suggested the possibility of a pulmonary sequestration, but the lower lobe had a normal venous return to the left atrium through the right inferior pulmonary vein.
View larger version (157K):
[in this window]
[in a new window]
|
Fig 1. Computed tomographic angiographic scan demonstrates an aberrant artery arising from the abdominal aorta and excessive network of tortuous bronchial arteries feeding a large left lower lobe mass.
|
|
The patient agreed to undergo another operation for the third time and was scheduled for a re-redo thoracotomy. A right "hemi-clamshell" incision was preferred to achieve a quicker and easier access to the systemic arteries that supply the mass. As computed tomographic angiography visualized a reach network of systemic arteries in conjunction with the mass, great care had to be taken not to damage them during the procedure. The aberrant infra-diaphragmatic arterial branch was found to be entering the pleural cavity behind the pericardium at the level of inferior pulmonary ligament. First, this vessel was preferentially dissected out, doubly ligated, and divided before proceeding to hilar or interlobar dissection of the pulmonary vessels. Even with this maneuver, full mobilization of the mass that had replaced almost the entire lobe could only be achieved with considerable difficulty. In part, this was due to dense adhesions from the previous thoracotomies. Excessive bleeding during the operation required 4 units of blood and a further 3 units in the early postoperative period. Despite these difficulties, the rest of the lower lobectomy was otherwise straightforward.
Macroscopic examination of the specimen demonstrated that only a thin layer of atelectatic lung tissue remained on the surface of the tumor like a capsule (Fig 2). Neither mediastinal nor pulmonary lymphatic invasion was detected at any of the lymph nodes that were sampled nonsystematically at the operation. Microscopic findings were consistent with those of an atypical carcinoid tumor (T2N0M0, stage 1B). The patient’s postoperative progress was smooth making a sufficient progress to be discharged home on postoperative day 4. She remains free of disease with no symptoms at 14 months after the operation.
View larger version (143K):
[in this window]
[in a new window]
|
Fig 2. Resected specimen shows an encapsulated and highly vascular tumor occupying an almost entire left lower lobe.
|
|
|
Comment
|
|---|
Atypical carcinoid tumors have more malignant histologic and clinical behavior in comparison with their typical counterparts, and both lymph node and distant metastasis are more frequently observed in the former type [1]. They hardly exceed 6 cm in diameter, and the resection is performed without much difficulty in most patients. The large series does however report anecdotal cases of giant atypical carcinoid tumors reaching sizes up to 24 cm requiring complex procedures [3]. In one such case [2], where the systemic arterial branch originated from the left internal thoracic artery, intraoperative massive bleeding was experienced, and the first thoracotomy was abandoned due to this bleeding. Then the mass could only be removed using partial cardiopulmonary bypass in a second thoracotomy. The similarities between that case and ours can be seen, but one should note the distinct anatomical difference (ie, the origin of the arterial blood supply). However, we believe that the presentation of this patient is the first case in the literature in which the presence of an infra-diaphragmatic arterial supply to a carcinoid tumor is preoperatively documented.
As far as surgical technique, when unusually excessive bleeding in relation to a lower chest mass is encountered at a thoracotomy, the possibility of sequestration should be considered, and this should alert the surgeon regarding the presence of an aberrant infra-diaphragmatic arterial supply. In this type of situation the operation may be continued only if the bleeding vessel is clearly visible and appears to be safely controlled; otherwise the resection should be deferred and plans for a re-thoracotomy should be made after appropriate angiographic examination.
In conclusion, carcinoid tumors may have unusual presentations; therefore they should always be kept in mind in the differential diagnosis of a giant lung mass associated with an excessive network of systemic arterial blood supply. The variety of possible perioperative scenarios warrants careful planning of the anticipated surgery.
|
References
|
|---|
- Marty-Ane CH, Costes V, Pujol JL, Alauzen M, Baldet P, Mary H. Carcinoid tumors of the lung: do atypical features require aggressive management? Ann Thorac Surg 1995;59:78-83.[Abstract/Free Full Text]
- Sheppard BB, Follette DM, Meyers FJ. Giant carcinoid tumor of the lung Ann Thorac Surg 1997;63:851-852.[Abstract/Free Full Text]
- Fischer S, Kruger M, McRae K, Merchant N, Tsao MS, Keshavjee S. Giant bronchial carcinoid tumors: a multidisciplinary approach Ann Thorac Surg 2001;71:386-393.[Abstract/Free Full Text]
Top
Abstract
Introduction
