Granular Cell T u m o r o f the Esophagus m R e p o r t o f a Case and Review o f the L i t e r a t u r e m Noriyuki TOHNOSU,1 Yoshifumi MATSUI,1 Masahiko OZAVa,' Yoshio KOIDE, 1
Kazuaki OKUVAMA,'Teruo Kouzu,' Shoichi ONODA,1 Kaichi IsoNo ~ and Hiroshi HomE 2 ABSTRACT: We report herein a case of a 46 year old man presenting with a gastric ulcer in w h o m an endoscopy h a p p e n e d to detect an elevated lesion in the lower esophagus. Endoscopic biopsy proved sufficient for determining the diagnosis o f a granular cell tumor (GCT). Electron and 9microscopic studies suggest that GCT are derived from Schwann cells. Although commonly f o u n d in the tongue and skin, GCT are rarely seen in the gastrointestinal tract, especially in the esophagus. However, advances in endoscopic techniques will increase the opportunity of detecting GCT of the esophagus. KEY WORDS: granular cell tumor o f the esophagus, endoscopic diagnosis, Schwann cell
INTRODUCTION
CASE REPORT
The n u m b e r of granular cell tumors (GCT)
A 46 year old male with a two year history of gastric ulcer complained of occasional epigastralgia and r e c u r r e n t h e a r t b u r n in April, 1988 and was referred to Chiba University Hospital from a nearby clinic. An esophago-gastrogram revealed an elevated lesion in the posterior wall of the lower esophagus (Fig. 1) and an ulcer nische in the angle o f the stomach (Fig. 2). O n endoscopy an elevated polypoid lesion was seen at the 4 o'clock position, 41 cm from the upper incisor teeth. T h e tumor wasyellowish, submucosal and had a small dell on its top (Fig. 3). T h e mucosal surface of the tumor appeared intact after staining with Lugol's solution. Endoscopic sonography disclosed a hypoechoic, well-defined and smoothly-bordered submucosal tumor 12 mm in size (Fig_ 4). T h e biopsy specimen from the lesion demonstrated the typical GCT patterns (Fig. 5-a, 5-b and 5-c) a n d surgery was performed
of the esophagus reported in J a p a n remains small compared to Western countries and although GCT o f the esophagus is a rare disease, a correct diagnosis is easily made by an endoscopic biopsy specimen alone if it is taken adequately. T h e purpose o f this paper is to present a case, review the literature and discuss the groSS appearance and management o f GCT, as well as its histogenesis using i m m u n o h i s t o c h e m i c a l a n d ultrastructural studies.
1The Second Department of Surgery, School of Medicine, Chiba University, Chiba,Japan "-TheCentralLaboratory Division, Chiba Children's Hospital, Chiba,Japan Reprint requests to: Noriyuki Tohnosu, MD, The Second Department of Surgery, School of Medicine, Chiba University, 1-8-1 Inohana, Chiba 280,Japan
JAPANESEJOURNALOF SURGERY,VOL.21, NO. 4 pp. 444-449, 1991
Volume 21 Number 4
Granular cell tumor of the esophagus
445
Fig. 2. Double contrast gastrogram showing an ulcer nische (arrow)in the angle of the stomach.
Fig. 1~ Double contrast esophagogram revealing an elevated lesion (arrow) in the posterior wall of the lower esophagus.
o n J u n e 1st, 1988. ,A l e f t a n t e r o l a t e r a l thoracotomy was m a d e through the 7th intercostal space following laparotomy and resection o f the lower esophagus a n d total stomach done to perform esophagojejunostomy with a Roux-Y loop. No ascites or pleural effusion were seen and only inflammatory swelling was noted in the regional lymph n o d e s o f the stomach. T h e removed specimen revealed a whitish, elevated lesion 12 X 7 X 3 m m in size located 15 m m above the gastroesophagealjunction (Fig. 6-a). T h e cross section o f the formalin-fixed specimen demonstrated a lesion apparently limited to the s u b m u c o s a (Fig. 6-b). Electron microscopy o f the removed specimen f o u n d multiple cells filled with a lot o f dense granules adjacent to the Schwann cells showing sparse granules. T h e same b a s e m e n t m e m b r a n e
Fig. 3. An endoscopic photo. Endoscopic view of the tumor located at the 4 o'clock position, 41 cm from the upper incisor teeth. The tumor was yellowish, submucosal and had a small dell on its top. contained both granular cells a n d Schwann cell (Fig. 7). T h e patient's clinical course was unevent-
Tohnosu et al.
446
Jpn; J; Surg. jury 1991 DiscussioN
Fig. 4. Endoscopic sonography showing a hypoechoic, well-defined and smoothlybordered submucosal tumor 12 mm in size (arrows). ful a n d he was discharged after 40 days o f h o s p i t a l i z a t i o n . H e has b e e n e x a m i n e d periodically by endoscopy with no evidence o f recurrence.
a
Abrikossoit a provided the first account o f a granular cell tumor o f the tongue as "granular cell myoblastoma" in 1926. Since then 119 cases o f GCT o f the esophagus have b e e n reported in Western countries as o f 19853 In Japan, Sai 3 reported the first case in 1975 since w h e n only 69 cases have b e e n described. GCT are relatively rare lesions frequently f o u n d in the tongue, skin, breast and gastrointestinal tract but less c o m m o n l y seen in the esophagus3 ,4,~ As the histogenesis of the tumors, GCT were initially thought to originate from the striated muscle cells, 1,6 histiocytes, 7 or even as a reaction to trauma, s,9 Recent studies, however, especially those using the S-100 protein antibody (immunohistochemical staining) support the idea that GCT arise from Schwann cells. 4,1~ This is
b
Fig. 5. Histology of the biopsy specimen. a Hematoxylin and eosin stain (• Spindle-shaped cells with the bright and acidphilic cytoplasm proliferate. The overlying squamous epithelium shows pseudoepitheliomatous hyperplasia and the islet-shaped basal cell nests are partly isolated. b PAS stain ()
Kazuaki OKUVAMA,'Teruo Kouzu,' Shoichi ONODA,1 Kaichi IsoNo ~ and Hiroshi HomE 2 ABSTRACT: We report herein a case of a 46 year old man presenting with a gastric ulcer in w h o m an endoscopy h a p p e n e d to detect an elevated lesion in the lower esophagus. Endoscopic biopsy proved sufficient for determining the diagnosis o f a granular cell tumor (GCT). Electron and 9microscopic studies suggest that GCT are derived from Schwann cells. Although commonly f o u n d in the tongue and skin, GCT are rarely seen in the gastrointestinal tract, especially in the esophagus. However, advances in endoscopic techniques will increase the opportunity of detecting GCT of the esophagus. KEY WORDS: granular cell tumor o f the esophagus, endoscopic diagnosis, Schwann cell
INTRODUCTION
CASE REPORT
The n u m b e r of granular cell tumors (GCT)
A 46 year old male with a two year history of gastric ulcer complained of occasional epigastralgia and r e c u r r e n t h e a r t b u r n in April, 1988 and was referred to Chiba University Hospital from a nearby clinic. An esophago-gastrogram revealed an elevated lesion in the posterior wall of the lower esophagus (Fig. 1) and an ulcer nische in the angle o f the stomach (Fig. 2). O n endoscopy an elevated polypoid lesion was seen at the 4 o'clock position, 41 cm from the upper incisor teeth. T h e tumor wasyellowish, submucosal and had a small dell on its top (Fig. 3). T h e mucosal surface of the tumor appeared intact after staining with Lugol's solution. Endoscopic sonography disclosed a hypoechoic, well-defined and smoothly-bordered submucosal tumor 12 mm in size (Fig_ 4). T h e biopsy specimen from the lesion demonstrated the typical GCT patterns (Fig. 5-a, 5-b and 5-c) a n d surgery was performed
of the esophagus reported in J a p a n remains small compared to Western countries and although GCT o f the esophagus is a rare disease, a correct diagnosis is easily made by an endoscopic biopsy specimen alone if it is taken adequately. T h e purpose o f this paper is to present a case, review the literature and discuss the groSS appearance and management o f GCT, as well as its histogenesis using i m m u n o h i s t o c h e m i c a l a n d ultrastructural studies.
1The Second Department of Surgery, School of Medicine, Chiba University, Chiba,Japan "-TheCentralLaboratory Division, Chiba Children's Hospital, Chiba,Japan Reprint requests to: Noriyuki Tohnosu, MD, The Second Department of Surgery, School of Medicine, Chiba University, 1-8-1 Inohana, Chiba 280,Japan
JAPANESEJOURNALOF SURGERY,VOL.21, NO. 4 pp. 444-449, 1991
Volume 21 Number 4
Granular cell tumor of the esophagus
445
Fig. 2. Double contrast gastrogram showing an ulcer nische (arrow)in the angle of the stomach.
Fig. 1~ Double contrast esophagogram revealing an elevated lesion (arrow) in the posterior wall of the lower esophagus.
o n J u n e 1st, 1988. ,A l e f t a n t e r o l a t e r a l thoracotomy was m a d e through the 7th intercostal space following laparotomy and resection o f the lower esophagus a n d total stomach done to perform esophagojejunostomy with a Roux-Y loop. No ascites or pleural effusion were seen and only inflammatory swelling was noted in the regional lymph n o d e s o f the stomach. T h e removed specimen revealed a whitish, elevated lesion 12 X 7 X 3 m m in size located 15 m m above the gastroesophagealjunction (Fig. 6-a). T h e cross section o f the formalin-fixed specimen demonstrated a lesion apparently limited to the s u b m u c o s a (Fig. 6-b). Electron microscopy o f the removed specimen f o u n d multiple cells filled with a lot o f dense granules adjacent to the Schwann cells showing sparse granules. T h e same b a s e m e n t m e m b r a n e
Fig. 3. An endoscopic photo. Endoscopic view of the tumor located at the 4 o'clock position, 41 cm from the upper incisor teeth. The tumor was yellowish, submucosal and had a small dell on its top. contained both granular cells a n d Schwann cell (Fig. 7). T h e patient's clinical course was unevent-
Tohnosu et al.
446
Jpn; J; Surg. jury 1991 DiscussioN
Fig. 4. Endoscopic sonography showing a hypoechoic, well-defined and smoothlybordered submucosal tumor 12 mm in size (arrows). ful a n d he was discharged after 40 days o f h o s p i t a l i z a t i o n . H e has b e e n e x a m i n e d periodically by endoscopy with no evidence o f recurrence.
a
Abrikossoit a provided the first account o f a granular cell tumor o f the tongue as "granular cell myoblastoma" in 1926. Since then 119 cases o f GCT o f the esophagus have b e e n reported in Western countries as o f 19853 In Japan, Sai 3 reported the first case in 1975 since w h e n only 69 cases have b e e n described. GCT are relatively rare lesions frequently f o u n d in the tongue, skin, breast and gastrointestinal tract but less c o m m o n l y seen in the esophagus3 ,4,~ As the histogenesis of the tumors, GCT were initially thought to originate from the striated muscle cells, 1,6 histiocytes, 7 or even as a reaction to trauma, s,9 Recent studies, however, especially those using the S-100 protein antibody (immunohistochemical staining) support the idea that GCT arise from Schwann cells. 4,1~ This is
b
Fig. 5. Histology of the biopsy specimen. a Hematoxylin and eosin stain (• Spindle-shaped cells with the bright and acidphilic cytoplasm proliferate. The overlying squamous epithelium shows pseudoepitheliomatous hyperplasia and the islet-shaped basal cell nests are partly isolated. b PAS stain ()
