Ann Thorac Surg 2005;80:1525-1527
© 2005 The Society of Thoracic Surgeons
Case report
Synchronous Mediastinal Ganglioneuroma and Retroperitoneal Pheochromocytoma
Shin-ichi Takeda, MD
a
,
*
,
Masato Minami, MD
b
,
Yoshifumi Inoue, MD
b
,
Hikaru Matsuda, MD
b
a Department of Thoracic Surgery, Toneyama National Hospital, Toyonaka, Osaka, Japan
b Department of Surgery, Osaka University Graduate School of Medicine, Suita City, Osaka, Japan
Accepted for publication March 25, 2004.
* Address reprint requests to Dr Takeda, Toneyama National Hospital, Toneyama 5-1-1 Toyonaka 560-8552 Japan (Email: stakeda{at}toneyama.hosp.go.jp).
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Abstract
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We herein report the case of a 52-year-old woman who has mediastinal ganglioneuroma and retroperitoneal pheochromocytoma with different histologic entity. The patient first visited our hospital for surgical treatment of superior-posterior mediastinal tumor, which was successfully removed by thoracoscopic approach to reveal a benign ganglioneuroma. A retroperitoneal tumor was incidentally found postoperatively that was removed using a laparoscopic procedure. The retroperitoneal tumor was a nonfunctioning pheochromocytoma, which was different from the mediastinal neurogenic tumor. This association, although rare, should be recognized for management of neurogenic tumors in the thorax.
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Introduction
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Most neurogenic tumors in the thorax are located in the posterior mediastinum and represent 15% to 25% of all mediastinal tumors [1–3]; however, the majority of these tumors (both benign and malignant) are asymptomatic, but they are easily diagnosed using current radiologic imaging techniques. Classification of neurogenic tumors is based on cell origin, and patient age may be the most important clinical measurement to distinguish between histologic type and rate of malignancy. Over the past 50 years, our hospital has treated a total of 146 patients with neurogenic tumors in the thorax, although only 1 patient with a synchronous neurogenic mediastinal tumor. Except for Recklinghausen's neurofibromatosis cases, the coexistence of mediastinal and retroperitoneal neurogenic tumors is quite rare.
A 52-year-old woman was first admitted for surgical treatment of a superior-posterior mediastinal tumor. Her past history was essentially not contributory, and she had been asymptomatic without episodes of paroxysmal hypertension or neurologic dysfunction. Chest roentgenogram images showed a well circumscribed, rounded mass, 2 cm in size in the superior-posterior mediastinum. Chest computed tomography and magnetic resonance imaging revealed a known solid mediastinal mass (Fig 1) in the superior-posterior mediastinum extending up to the thoracic inlet. Findings of a physical examination were unremarkable and also showed neurologic abnormalities. Laboratory examination results were essentially normal without elevation of serum tumor markers. Under a diagnosis of benign neurogenic tumor of the mediastinum, a scheduled operation was performed. The tumor was located in the superior mediastinum surrounded by the thoracic inlet. Therefore we adopted a thoracoscopic approach assisted by fine manipulation through a supraclavicular approach [4] and added a 5-cm supraclavicular skin incision. The tumor was successfully removed without complication or discomfort, and a postoperative histologic diagnosis of ganglioneuroma was obtained (Fig 2). Postoperatively, a computed tomographic chest scan for a check-up of the thoracic cavity downward to the upper abdomen was done to reveal a vascular rich 2.5-cm retroperitoneal mass. At the time of initial surgery, such a check-up was not performed. Abdominal magnetic resonance imaging (Fig 3) confirmed that it was a known retroperitoneal tumor. The patient never complained of headaches, dizziness, or general malaise, and her blood pressure was normal. Furthermore, a laboratory test revealed unremarkable findings. A laparoscopic resection was performed for the retroperitoneal tumor, and a diagnosis of pheochromocytoma of the retroperitoneum was obtained as shown in Figure 4. The two benign ganglioneuroma of the mediastinum and retroperitoneal pheochromocytoma were histologically distinct neurogenic tumors. The patient had an uneventful recovery and was doing well without relapse 2 years postoperatively.
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Fig 1. Chest magnetic resonance imaging revealed a well-circumscribed, rounded mass in the superior-posterior mediastinum.
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Fig 3. Abdominal magnetic resonance imaging revealed an inhomogeneous mass 2.5 cm in size (white arrow) in the retroperitoneum.
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Comment
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As the name implies, neurogenic tumors originate in the embryonic neural crest cells, which constitute the ganglia, paraganglionic, and parasympathetic systems, and recently, benign neurogenic tumors have been proposed as good candidates for video-assisted thoracic surgery excision. We treated 146 patients with intrathoracic neurogenic tumors (136 in the mediastinum, 9 in the chest wall, and 1 intrapulmonary) at our institution over the past 50 years [2, 3]. However, this is the only case of a coexistent mediastinal ganglioneuroma and a retroperitoneal pheochromocytoma, which were removed by two separate endoscopic operations. A review of the English literature revealed no cases of synchronous mediastinal and retroperitoneal neurogenic tumors with different histopathologies.
The recent advent of computed tomographic scanning and magnetic resonance imaging techniques have led to an increase in incidental discovery of adrenal (retroperitoneal) tumors, namely incidentalomas, which comprised 0.3% to 2% of all tumors in the reported series [5, 6]. The current case may have merely been a rare occurrence of mediastinal and retroperitoneal neurogenic tumors. The retroperitoneal (adrenal) pheochromocytoma was just found soon after surgical removal of the mediastinal tumor, thus it fits the criterion for synchronous neurogenic tumors. An optimal diagnostic and therapeutic approach must balance diagnostic costs, discomfort, consequences of false positive results, and curative therapy when treating patients with retroperitoneal incidentolomas [5]. Although size remains a good predictor of histologic features and clinical behavior, small sized malignant tumors can occur as well. In the English literature, pheochromocytomas were found in 9% to 11% of the patients who underwent surgery for incidentalomas [6, 7], whereas retroperitoneal ganglioneuromas accounted for 4% to 9% [5, 6]. The present case may illustrate a rare coexistence of neurogenic tumors with different histologies in the mediastinum and retroperitoneum, which were both resected using an endoscopic procedure. A routine check-up of the abdominal cavity has not been established in the diagnostic and therapeutic modalities for benign mediastinal tumors. However, the coexistence of retroperitoneal neurogenic tumors, although rare, should be recognized during the management of patients with a mediastinal neurogenic tumor.
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References
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- Ribet ME, Cardot GR. Neurogenic tumors of the thorax Ann Thorac Surg 1994;58:1091-1095.[Abstract]
- Takeda S, Miyoshi S, Minami M, et al. Intrathoracic neurogenic tumors J Jpn Assoc Chest Surg 2001;15:266(abstract).
- Takeda S, Miyoshi S, Akashi A, et al. Clinical spectrum of primary mediastinal tumorsa comparison of adult and pediatric populations in one Japanese institutional experience. J Surg Oncol 2003;83:24-30.[Medline]
- Akashi A, Ohashi S, Yoden Y, et al. Thoracoscopic surgery combined with a supraclavicular approach for removing superior mediastinal tumor Surg Endosc 1997;11:74-76.[Medline]
- Murai M, Baba S, Nakashima J, Tachibana M. Management of incidentally discovered adrenal mass World J Urol 1999;17:9-14.[Medline]
- Mantero F, Terzolo M, Arnaldi G, Osella G, Masini AM, Ali A. A survey on adrenal incidentaloma in Italy J Clin Endocrinol Metab 2000;85:637-644.[Abstract/Free Full Text]
- Barnett Jr CC, Varma DG, El-Naggar AK, Dackiw APB, Porter GA. Limitation of size as a criterion in the evaluation of adrenal mass Surgery 2000;128:973-983.[Medline]
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Abstract
Introduction
