Ann Thorac Cardiovasc Surg 2015; 21: 567–569
Case Report
Online June 2, 2015 doi: 10.5761/atcs.cr.15-00112
A Case of Mediastinal Granular Cell Tumor with Horner’s Syndrome Yoshinobu Shikatani, MD,1 Mikio Okazaki, MD,1 Nobuhiko Sakao, MD,1 Shungo Yukumi, MD,3 Hisayuki Shigematsu, MD,1 Sohei Kitazawa, MD,2 and Yoshifumi Sano, MD1
Granular cell tumor (GCT) is found in various organs but is rare in the mediastinum. We report a case of mediastinal GCT in a 19-year-old woman who presented with left ptosis and miosis. CT and MRI revealed a 29-mm well-circumscribed tumor located close to the first thoracic vertebra with features suggesting a neurogenic tumor. The tumor was completely excised using single-port video-assisted thoracoscopic surgery. Histopathological and immunohistochemical analysis revealed that the tumor was a benign GCT. Postoperatively, left ptosis and miosis had improved slightly. To our knowledge, this is the first report regarding mediastinal GCT presenting with preoperative Horner’s syndrome. Keywords: mediastinal tumor, granular cell tumor, Horner’s syndrome
Introduction
Case Report
Granular cell tumor (GCT), although typically observed in the digestive system, head, neck, breast and skin regions, can occur almost anywhere in the body; >5% of patients have multiple lesions.1) At the time of diagnosis, most patients are aged 30–50 years; there is a 2:1 female to male ratio.2) The occurrence of GCTs in the mediastinum is extremely rare,3) and Horner’s syndrome has not been previously described as a symptom of GCT. We report a rare case of mediastinal GCT with preoperative Horner’s syndrome.
A 19-year-old woman gave her informed consent for the publication of this study. She was referred to our hospital for extended examination of a superior mediastinal mass that had been detected using a chest X-ray and a CT scan. She had no previous history of significant illness. On physical examination left ptosis and miosis were noted, but anhidrosis was not clear. The results of clinical laboratory tests were within the normal range. A CT scan showed a well-circumscribed and homogeneous mass measuring 29 mm × 22 mm and located close to the vertebral column (Th1; Fig. 1A and 1B). Both T1- and T2-weighted MRIs showed inhomogeneous low intensity regions inside the tumor, and high intensity regions on the tumor surface (Fig. 2A and 2B). The location of the tumor and symptoms suggested that it had a neurogenic origin with complicating Horner’s syndrome. The patient underwent single-port video-assisted thoracoscopic resection of the tumor. Resection was performed through a 2.5-cm skin incision located in the fourth intercostal space in the middle axillary line using a rigid 5-mm thirty-degree thoracoscope. A solid circumscribed tumor was found on the first costeovertebral joint attached to the
Center of Chest Medicine and Surgery, Ehime University, Toon, Ehime, Japan 2Department of Diagnostic Pathology, Ehime University, Toon, Ehime, Japan 3Surgery, National Hospital Organization Ehime Medical Center, Toon, Ehime, Japan 1
Received: April 7, 2015; Accepted: April 16, 2015 Corresponding author: Mikio Okazaki, MD. Center of Chest Medicine and Surgery, Ehime University, Shitsukawa, Toon, Ehime 791-0295, Japan Email: [email protected] ©2015 The Editorial Committee of Annals of Thoracic and Cardiovascular Surgery. All rights reserved.
Ann Thorac Cardiovasc Surg Vol. 21, No. 6 (2015)
567
Shikatani Y, et al. (A)
(B)
(A)
(B)
(C)
(D)
Fig. 1 C ontrast-enhanced chest CT image. (A) Axial section showing a poorly enhanced well-circumscribed 29 mm × 22 mm × 17 mm mass, closely attached to the vertebral column. (B) Coronal section.
(A)
(B)
Fig. 2. C hest MRI. The tumor exhibited inhomogeneous low intensities. High intensities were found on tumor surface on T1- and T2-weighted images. (A) Coronal section: T1-weighted image. (B) Coronal section: T2-weighted image.
sympathetic nerve. The tumor was completely excised without sympathetic nerve injury. Postoperatively, no complications developed and the left Horner’s syndrome improved slightly. The patient’s left miotic pupillary diameter increased from 2.5 mm to 3 mm, and the diameter of her left palpebral fissure improved from 7 mm to 8 mm. She was discharged on the third postoperative day. The excised tumor was a solid encapsulated whitish mass measuring 30 mm × 18 mm. Histopathological examination revealed that the mass was mostly composed of sheets of rounded or short spindle cells with eosinophilic cytoplasmic granular structures (Fig. 3A and 3B), and was partly composed of normal ganglion cells at its periphery (Fig. 3C). Immunohistochemical studies revealed strong and diffuse cell reactivity for S-100 (Fig. 3D), and partial reactivity for neuron-specific enolase (NSE). Staining was negative for c-KIT, chromogranin-A, synaptophysin, CD56 and neurofilaments. The MIB-1 labeling index was
568
Fig. 3 H ematoxylin-eosin (HE) stained tumor sections (A) with diffuse proliferation of spindle to oval-shaped cells (×100), and (B) with abundant cytoplasm filled with numerous eosinophilic granules (×400). Non-tumorous ganglion cells can be seen in the peripheral area. (C) and (D) Immunohistochemically stained tumor sections. The tumor cells are strongly positive for S-100 protein (×40).
Case Report
Online June 2, 2015 doi: 10.5761/atcs.cr.15-00112
A Case of Mediastinal Granular Cell Tumor with Horner’s Syndrome Yoshinobu Shikatani, MD,1 Mikio Okazaki, MD,1 Nobuhiko Sakao, MD,1 Shungo Yukumi, MD,3 Hisayuki Shigematsu, MD,1 Sohei Kitazawa, MD,2 and Yoshifumi Sano, MD1
Granular cell tumor (GCT) is found in various organs but is rare in the mediastinum. We report a case of mediastinal GCT in a 19-year-old woman who presented with left ptosis and miosis. CT and MRI revealed a 29-mm well-circumscribed tumor located close to the first thoracic vertebra with features suggesting a neurogenic tumor. The tumor was completely excised using single-port video-assisted thoracoscopic surgery. Histopathological and immunohistochemical analysis revealed that the tumor was a benign GCT. Postoperatively, left ptosis and miosis had improved slightly. To our knowledge, this is the first report regarding mediastinal GCT presenting with preoperative Horner’s syndrome. Keywords: mediastinal tumor, granular cell tumor, Horner’s syndrome
Introduction
Case Report
Granular cell tumor (GCT), although typically observed in the digestive system, head, neck, breast and skin regions, can occur almost anywhere in the body; >5% of patients have multiple lesions.1) At the time of diagnosis, most patients are aged 30–50 years; there is a 2:1 female to male ratio.2) The occurrence of GCTs in the mediastinum is extremely rare,3) and Horner’s syndrome has not been previously described as a symptom of GCT. We report a rare case of mediastinal GCT with preoperative Horner’s syndrome.
A 19-year-old woman gave her informed consent for the publication of this study. She was referred to our hospital for extended examination of a superior mediastinal mass that had been detected using a chest X-ray and a CT scan. She had no previous history of significant illness. On physical examination left ptosis and miosis were noted, but anhidrosis was not clear. The results of clinical laboratory tests were within the normal range. A CT scan showed a well-circumscribed and homogeneous mass measuring 29 mm × 22 mm and located close to the vertebral column (Th1; Fig. 1A and 1B). Both T1- and T2-weighted MRIs showed inhomogeneous low intensity regions inside the tumor, and high intensity regions on the tumor surface (Fig. 2A and 2B). The location of the tumor and symptoms suggested that it had a neurogenic origin with complicating Horner’s syndrome. The patient underwent single-port video-assisted thoracoscopic resection of the tumor. Resection was performed through a 2.5-cm skin incision located in the fourth intercostal space in the middle axillary line using a rigid 5-mm thirty-degree thoracoscope. A solid circumscribed tumor was found on the first costeovertebral joint attached to the
Center of Chest Medicine and Surgery, Ehime University, Toon, Ehime, Japan 2Department of Diagnostic Pathology, Ehime University, Toon, Ehime, Japan 3Surgery, National Hospital Organization Ehime Medical Center, Toon, Ehime, Japan 1
Received: April 7, 2015; Accepted: April 16, 2015 Corresponding author: Mikio Okazaki, MD. Center of Chest Medicine and Surgery, Ehime University, Shitsukawa, Toon, Ehime 791-0295, Japan Email: [email protected] ©2015 The Editorial Committee of Annals of Thoracic and Cardiovascular Surgery. All rights reserved.
Ann Thorac Cardiovasc Surg Vol. 21, No. 6 (2015)
567
Shikatani Y, et al. (A)
(B)
(A)
(B)
(C)
(D)
Fig. 1 C ontrast-enhanced chest CT image. (A) Axial section showing a poorly enhanced well-circumscribed 29 mm × 22 mm × 17 mm mass, closely attached to the vertebral column. (B) Coronal section.
(A)
(B)
Fig. 2. C hest MRI. The tumor exhibited inhomogeneous low intensities. High intensities were found on tumor surface on T1- and T2-weighted images. (A) Coronal section: T1-weighted image. (B) Coronal section: T2-weighted image.
sympathetic nerve. The tumor was completely excised without sympathetic nerve injury. Postoperatively, no complications developed and the left Horner’s syndrome improved slightly. The patient’s left miotic pupillary diameter increased from 2.5 mm to 3 mm, and the diameter of her left palpebral fissure improved from 7 mm to 8 mm. She was discharged on the third postoperative day. The excised tumor was a solid encapsulated whitish mass measuring 30 mm × 18 mm. Histopathological examination revealed that the mass was mostly composed of sheets of rounded or short spindle cells with eosinophilic cytoplasmic granular structures (Fig. 3A and 3B), and was partly composed of normal ganglion cells at its periphery (Fig. 3C). Immunohistochemical studies revealed strong and diffuse cell reactivity for S-100 (Fig. 3D), and partial reactivity for neuron-specific enolase (NSE). Staining was negative for c-KIT, chromogranin-A, synaptophysin, CD56 and neurofilaments. The MIB-1 labeling index was
568
Fig. 3 H ematoxylin-eosin (HE) stained tumor sections (A) with diffuse proliferation of spindle to oval-shaped cells (×100), and (B) with abundant cytoplasm filled with numerous eosinophilic granules (×400). Non-tumorous ganglion cells can be seen in the peripheral area. (C) and (D) Immunohistochemically stained tumor sections. The tumor cells are strongly positive for S-100 protein (×40).
