J Gastrointest Canc (2015) 46:87–89 DOI 10.1007/s12029-015-9683-6
CASE REPORT
Abrikossoff Cell Tumor of the Esophagus: Case Presentation of a Rare Endoscopic Entity and Review of Literature Michalis Galanopoulos & Christos Liatsos & Georgios Nakos & Evangelos Kalafatis
Published online: 20 January 2015 # Springer Science+Business Media New York 2015
Introduction Granular cell tumor (GCT) is an extremely uncommon entity first described by Abrikossoff in 1926 when he reported a series of five patients with such tumors found in the tongue [1], and he was also the first in reporting GCT existence in the esophagus 5 years later. GCTs are soft-tissue neoplasms composed of Schwann cells with granular cytoplasm [2] and are mainly asymptomatic. In the gastrointestinal (GI) tract, they occur at a rate of 6 to 8 % [3, 4] and especially their occurrence in esophagus is even rarer, accounting for approximately 2 % of all GCTs [4]. Herein, we present a middle-aged male patient in whom, incidentally, a GCT was discovered in distal part of esophagus.
on physical examination. The patient denied smoking, alcohol, analgesic, or any other drug abuse, and his chemistry panel and complete blood count were normal. During the upper GI endoscopy, a grayish-white intramural firm lesion was found, 1 cm in diameter (Fig. 1) covered by intact overlying mucosa (Fig. 2) in the distal esophagus next to gastroesophageal junction and biopsies were taken. The tissues were sent for histopathological evaluation which confirmed the presence of a benign tumor composed of nests of polygonal large cells with abundant eosinophilic granular cytoplasm and small round nuclei without mitotic activity. The diagnosis of GCT established with the immunohistochemical staining which was positive for the glycoprotein CD68 (Fig. 3) and for the S100 protein. Our patient was discharged after 2 days with follow-up instructions.
Case Presentation Α 36-year-old male patient with free medical history, apart from a gastroesophageal reflux disease (GERD) under double-dose PPIs for 2 months, came to our department (June 2014) for upper GI endoscopic control in the context of investigation for persistent GERD symptoms and epigastric pain. There was no remarkable family history, and on admission, vital signs (blood pressure, heart rate, respiration rate, and body temperature) were within normal limits. The patient was in good general health and had no significant weight loss M. Galanopoulos (*) : C. Liatsos : E. Kalafatis Department of Gastroenterology, 401 Military General Hospital of Athens, Mesogeion Avenue 138 & Katechaki Str, Athens 11525, Greece e-mail: [email protected] G. Nakos Department of Pathology, 401 Military General Hospital of Athens, Mesogeion Avenue 138 & Katechaki Str, Athens 11525, Greece
Discussion According to the literature, the overall occurrence rate of GCTs cannot be estimated precisely because much of the literature on granular cell tumors consists of reports on single cases. Nonetheless, as we mentioned before, their presence in the esophagus occur in 1 to 2 % approximately, more often in the distal part. They are considered benign neoplasms of neural derivation although there have been reports of malignant lesions in the literature for which histological criteria by Fanburg-Smith and associates [5] have been proposed. Their location has been reported anywhere in the human body, but usually they appear in the oral cavity, skin, respiratory tract, biliary and nervous system, and breast. GCTs are more frequently noted in men [3] and commonly seen in the fourth to sixth decade of life. Since 1931, when Abrikossoff noted down the first esophageal GCT, about 350 case reports have been described [6].
88
Fig. 1 Endoscopic image showing a grayish-white intramural firm lesion, cm in diameter, covered by intact overlying mucosa in the distal esophagus next to the gastroesophageal junction
Most esophageal GCTs are discovered incidentally during upper GI endoscopy for investigation of GERD symptoms (as it happens in our case) or nonspecific ones such as dyspepsia, heartburn, vomiting, etc. In order to attribute a symptom (mainly dysphagia or retrosternal pain) to the tumor, its size should be bigger than 2 cm in diameter. As concern its endoscopic appearance, a small yellowish to white-colored lesion with firm to heavy consistency is often seen. They are presented, most of the times, superficially as sessile polyps or plaques, restricted to submucosal layer based on endoscopic ultrasound (EUS) which was unavailable in our case. Apart from the depth of the tumor, EUS can distinguish CGT from cysts, lipomas, and polyps with inflammation. Although endoscopic appearance and EUS are critical for the diagnosis of GCT, the gold standard examination in order to establish the diagnosis is the histologic evaluation.
J Gastrointest Canc (2015) 46:87–89
Fig 3 Strong cytoplasmic immunoreactivity of the neoplastic cells for CD68 (CD68×200)
Thus, the main characteristics consist of nests of round or polygonal large cells with round, central nuclei and with eosinophilic cytoplasm and markedly enlarged lysosomes with granular appearance. The immunohistochemical stainings show on the one hand that GCT are negative for smooth muscle actin and desmin (distinguishing GCTs from leiomyomas where the latter ones are positive), and on the other, immunoreactivity for protein S100, CD68, CD57 nestin, and vimentin [7]. Concerning the treatment of these neoplasms, the current opinions lead to a more conservative approach (regular endoscopy and EUS follow-up) because of their typical “non-malignant” clinical course [8, 9]. However, if the tumor is bigger than 1 cm in diameter resulting to dysphagia or being suspected for malignancy, endoscopic or surgical resection should be performed [9]. In conclusion, esophageal granular cell tumors are nervous originating neoplasms with benign characteristics, most of the times asymptomatic with the diagnosis based on histology. Their identification is very important in order to distinguish lesions with the same, sometimes, appearance such as squamous esophageal carcinoma, and for this reason, the combination of endoscopic image, EUS, histological features, and immunohistochemical stainings contribute to the correct diagnosis.
Conflict of Interest The authors declare that they have no conflicts of interest.
References Fig. 2 Polypoid configuration of the granular cell tumor within the esophagus submucosa. The stratified squamous epithelium remains intact (H&E×100)
1. Abrikossoff A. Uber Myome, ausgehend von der quergestreiften willkurlichen Muskulatur. Virchows Arch Pathol Anat. 1926;260: 215–33.
J Gastrointest Canc (2015) 46:87–89 2. Ordóñez NG. Granular cell tumor: a review and update. Adv Anat Pathol. 1999;6(4):186–203. 3. Parfitt JR, McLean CA, Joseph MG, Streutker CJ, Al-Haddad S, Driman DK. Granular cell tumours of the gastrointestinal tract: expression of nestin and clinicopathological evaluation of 11 patients. Histopathology. 2006;48(4):424–30. 4. Johnston J, Helwig EB. Granular cell tumors of the gastrointestinal tract and perianal region: a study of 74 cases. Dig Dis Sci. 1981;26: 807–16. 5. Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic. Surg Pathol. 1998;22:779–94.
89 6. Chen WS, Zheng XL, Jin L, Pan XJ, Ye MF. Novel diagnosis and treatment of esophageal granular cell tumor: report of 14 cases and review of the literature. Ann Thorac Surg. 2014;97(1):296–302. 7. Hulagu S, Senturk O, Aygun C, et al. Granular cell tumor of esophagus removed with endoscopic submucosal dissection. Turk J Gastroenterol. 2007;18:188–91. 8. Fotiadis C, Manolis EN, Troupis TG, Gorgoulis VG, Sechas MN. Endoscopic resection of a large granular cell tumor of the esophagus. J Surg Oncol. 2000;75:277–9. 9. De Rezende L, Lucendo AJ, Alvarez-Arguelles H. Granular cell tumors of the esophagus: report of five cases and review of diagnostic and therapeutic techniques. Dis Esophagus. 2007;20:436–43.
CASE REPORT
Abrikossoff Cell Tumor of the Esophagus: Case Presentation of a Rare Endoscopic Entity and Review of Literature Michalis Galanopoulos & Christos Liatsos & Georgios Nakos & Evangelos Kalafatis
Published online: 20 January 2015 # Springer Science+Business Media New York 2015
Introduction Granular cell tumor (GCT) is an extremely uncommon entity first described by Abrikossoff in 1926 when he reported a series of five patients with such tumors found in the tongue [1], and he was also the first in reporting GCT existence in the esophagus 5 years later. GCTs are soft-tissue neoplasms composed of Schwann cells with granular cytoplasm [2] and are mainly asymptomatic. In the gastrointestinal (GI) tract, they occur at a rate of 6 to 8 % [3, 4] and especially their occurrence in esophagus is even rarer, accounting for approximately 2 % of all GCTs [4]. Herein, we present a middle-aged male patient in whom, incidentally, a GCT was discovered in distal part of esophagus.
on physical examination. The patient denied smoking, alcohol, analgesic, or any other drug abuse, and his chemistry panel and complete blood count were normal. During the upper GI endoscopy, a grayish-white intramural firm lesion was found, 1 cm in diameter (Fig. 1) covered by intact overlying mucosa (Fig. 2) in the distal esophagus next to gastroesophageal junction and biopsies were taken. The tissues were sent for histopathological evaluation which confirmed the presence of a benign tumor composed of nests of polygonal large cells with abundant eosinophilic granular cytoplasm and small round nuclei without mitotic activity. The diagnosis of GCT established with the immunohistochemical staining which was positive for the glycoprotein CD68 (Fig. 3) and for the S100 protein. Our patient was discharged after 2 days with follow-up instructions.
Case Presentation Α 36-year-old male patient with free medical history, apart from a gastroesophageal reflux disease (GERD) under double-dose PPIs for 2 months, came to our department (June 2014) for upper GI endoscopic control in the context of investigation for persistent GERD symptoms and epigastric pain. There was no remarkable family history, and on admission, vital signs (blood pressure, heart rate, respiration rate, and body temperature) were within normal limits. The patient was in good general health and had no significant weight loss M. Galanopoulos (*) : C. Liatsos : E. Kalafatis Department of Gastroenterology, 401 Military General Hospital of Athens, Mesogeion Avenue 138 & Katechaki Str, Athens 11525, Greece e-mail: [email protected] G. Nakos Department of Pathology, 401 Military General Hospital of Athens, Mesogeion Avenue 138 & Katechaki Str, Athens 11525, Greece
Discussion According to the literature, the overall occurrence rate of GCTs cannot be estimated precisely because much of the literature on granular cell tumors consists of reports on single cases. Nonetheless, as we mentioned before, their presence in the esophagus occur in 1 to 2 % approximately, more often in the distal part. They are considered benign neoplasms of neural derivation although there have been reports of malignant lesions in the literature for which histological criteria by Fanburg-Smith and associates [5] have been proposed. Their location has been reported anywhere in the human body, but usually they appear in the oral cavity, skin, respiratory tract, biliary and nervous system, and breast. GCTs are more frequently noted in men [3] and commonly seen in the fourth to sixth decade of life. Since 1931, when Abrikossoff noted down the first esophageal GCT, about 350 case reports have been described [6].
88
Fig. 1 Endoscopic image showing a grayish-white intramural firm lesion, cm in diameter, covered by intact overlying mucosa in the distal esophagus next to the gastroesophageal junction
Most esophageal GCTs are discovered incidentally during upper GI endoscopy for investigation of GERD symptoms (as it happens in our case) or nonspecific ones such as dyspepsia, heartburn, vomiting, etc. In order to attribute a symptom (mainly dysphagia or retrosternal pain) to the tumor, its size should be bigger than 2 cm in diameter. As concern its endoscopic appearance, a small yellowish to white-colored lesion with firm to heavy consistency is often seen. They are presented, most of the times, superficially as sessile polyps or plaques, restricted to submucosal layer based on endoscopic ultrasound (EUS) which was unavailable in our case. Apart from the depth of the tumor, EUS can distinguish CGT from cysts, lipomas, and polyps with inflammation. Although endoscopic appearance and EUS are critical for the diagnosis of GCT, the gold standard examination in order to establish the diagnosis is the histologic evaluation.
J Gastrointest Canc (2015) 46:87–89
Fig 3 Strong cytoplasmic immunoreactivity of the neoplastic cells for CD68 (CD68×200)
Thus, the main characteristics consist of nests of round or polygonal large cells with round, central nuclei and with eosinophilic cytoplasm and markedly enlarged lysosomes with granular appearance. The immunohistochemical stainings show on the one hand that GCT are negative for smooth muscle actin and desmin (distinguishing GCTs from leiomyomas where the latter ones are positive), and on the other, immunoreactivity for protein S100, CD68, CD57 nestin, and vimentin [7]. Concerning the treatment of these neoplasms, the current opinions lead to a more conservative approach (regular endoscopy and EUS follow-up) because of their typical “non-malignant” clinical course [8, 9]. However, if the tumor is bigger than 1 cm in diameter resulting to dysphagia or being suspected for malignancy, endoscopic or surgical resection should be performed [9]. In conclusion, esophageal granular cell tumors are nervous originating neoplasms with benign characteristics, most of the times asymptomatic with the diagnosis based on histology. Their identification is very important in order to distinguish lesions with the same, sometimes, appearance such as squamous esophageal carcinoma, and for this reason, the combination of endoscopic image, EUS, histological features, and immunohistochemical stainings contribute to the correct diagnosis.
Conflict of Interest The authors declare that they have no conflicts of interest.
References Fig. 2 Polypoid configuration of the granular cell tumor within the esophagus submucosa. The stratified squamous epithelium remains intact (H&E×100)
1. Abrikossoff A. Uber Myome, ausgehend von der quergestreiften willkurlichen Muskulatur. Virchows Arch Pathol Anat. 1926;260: 215–33.
J Gastrointest Canc (2015) 46:87–89 2. Ordóñez NG. Granular cell tumor: a review and update. Adv Anat Pathol. 1999;6(4):186–203. 3. Parfitt JR, McLean CA, Joseph MG, Streutker CJ, Al-Haddad S, Driman DK. Granular cell tumours of the gastrointestinal tract: expression of nestin and clinicopathological evaluation of 11 patients. Histopathology. 2006;48(4):424–30. 4. Johnston J, Helwig EB. Granular cell tumors of the gastrointestinal tract and perianal region: a study of 74 cases. Dig Dis Sci. 1981;26: 807–16. 5. Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic. Surg Pathol. 1998;22:779–94.
89 6. Chen WS, Zheng XL, Jin L, Pan XJ, Ye MF. Novel diagnosis and treatment of esophageal granular cell tumor: report of 14 cases and review of the literature. Ann Thorac Surg. 2014;97(1):296–302. 7. Hulagu S, Senturk O, Aygun C, et al. Granular cell tumor of esophagus removed with endoscopic submucosal dissection. Turk J Gastroenterol. 2007;18:188–91. 8. Fotiadis C, Manolis EN, Troupis TG, Gorgoulis VG, Sechas MN. Endoscopic resection of a large granular cell tumor of the esophagus. J Surg Oncol. 2000;75:277–9. 9. De Rezende L, Lucendo AJ, Alvarez-Arguelles H. Granular cell tumors of the esophagus: report of five cases and review of diagnostic and therapeutic techniques. Dis Esophagus. 2007;20:436–43.
