- Email: [email protected]
Endoscopic removal of a granular cell tumor of the esophagus
The enlarger light is turned on for an average of 2 to 4 seconds, as determined by counting orally (1-1000, 2-1000, 3-1000, etc.) and then manually shut off. The dark shield is then closed completely and the room lights put on. The picture is easily developed in 60 seconds by following the manufacturer's instructions. Multiple prints, if needed, should be made at this time. Trial and error wi II usually resu It in a very satisfactory print. If the initial print is too dark, a longer period of exposure is needed for the next test print. This is achieved by either keeping the enlarger light on for a few extra seconds or else by opening the lens to the next smallest number. If the picture is too light, less exposure time is required. One can often improve the quality of prints obtained from a poor transparency by
following this procedure. In some instances, a 1-second exposure may still result in too light an image. This can be corrected by screwing a neutral density filter into the bottom of the lens before the picture is to be exposed. This filter decreases the amount of light coming through without affecting color, image, or size. The filters are in series and graded from 1 to 6. The purchase of filters no. 2 and no. 4 will cover most situations. It may be easier to buy a Spiratone (Flushing, NY) variable neutral density filter. The degree of filtration can be adjusted simply by turning the ring which is an integral part of the filter. This eliminates the need for handling multiple filters. After some experience is gained, one is usually able to judge more easily the proper exposure for obtaining an excellent print (Fig. 1).
Endoscopic removal of a granular cell tumor of the esophagus
longitudinal esophageal fold above the lesion contributed to the appearance of a stalk. Biopsies showed unremarkable squamous epithelium with smooth muscle seen in one fragment. The lesion appeared to be freely moveable on a short stalk and we performed transendoscopic resection with a wire snare and electrosurgical unit. Transection of the stalk appeared to require more than the usual amount of current. When the polyp was removed, a O.B-cm diameter burn was noted at the polypectomy site. The patient tolerated the procedure well and had an uneventful subsequent course with no dysphagia. On cut section the polyp revealed a homogeneous, creamy white, moderately firm surface. Microscopically the polyp consisted predominately of large polygonal granular cells covered by a stratified squamous epithelium with slight inflammation (Fig. 3). Follow-up endoscopy at 3 months showed complete healing of the esophagus. No mucosal or submucosal lesions were seen.
Robert S. Sandler, MD David R. Wood, MD Eugene M. Bozymski, MD
Granular cell tumors are rare lesions of neural origin. Since their initial description in 1926 by Abrikossoff, there have been only 22 cases in which the tumor was found in the esophagus. 1 We report the first case in which a granular cell tumor was removed by transendoscopic wire snare. CASE REPORT A 53-year-old woman was referred for evaluation of a 2month history of dysphagia for solid foods and postprandial vomiting. Ten months earlier she was evaluated for epigastric pain at a local hospital. An upper gastrointestinal series revealed tertiary contractions of the distal esophagus and an oral cholecystogram was negative. Her medications on admission included cimetidine, propranolol for hypertension, and aspirin for osteoarthritis. She admitted to moderate alcohol use. Her physical examination was unremarkable except for the presence of occult blood in the stool. Sigmoidoscopy and barium enema examination were normal. An upper gastrointestinal series demonstrated a 2.5-cm diameter polypoid lesion in the distal esophagus (Fig. 1). Esophagoscopy revealed a pinkish tan polypoid lesion 30 cm from the incisors (Fig. 2). The lesion had a firm, rubbery consistency, was covered by normal appearing mucosa, and appeared to be attached by a short stalk. A From the Division of Digcstive Diseases and Nutrition, Department of Medicine, University of North Carolina School of Mcdicine, Chapel Hill, North Carolina. This investigation was supported in part by National Canccr Institute Grant CA 17973. Reprint rcquests: Robert S. Sandler, MD, Division of Digestivc Diseascs and Nutrition, Room 324, Clinical Sciences Building 229H, University of North Carolina School of Medicine, Chapel Hill, North Carolina 27.514.
70
DISCUSSION Granular cell tumors may be found throughout the body but have a predilection for the upper digestive and respiratory tract, with a third of the lesions found in the tongue. 2 - 4 Other common sites include the skin, subcutaneous tissue, and the breast 4 The lesion most commonly occurs in the 3rd to 5th decade 5 Granular cell tumors of the esophagus are more common in women (79%) and are multiple in 25% of cases. Malignant granular cell tumors have been reported but are much less common than the benign variety.6 When the tumors are malignant, they are often slow growing. 6 One should distinguish a multicentric tumor from a malignant tumor with metastases 5 Although these lesions are often termed granular cell myoblastomas, they are thought to be of neural origin. In 1952 Bangle presented evidence based on histologic and histochemical studies that showed that the granular material was derived from degeneration GASTROINTESTINAL ENDOSCOPY
Figure 1. Karium swallow showing a polYPc)I(j lesion in the distal esophagus.
Figure 3. I llstologic appearance' 01 gralluldl ((,II tumm ,hO\\Ing large polygollal cells With grallular cytoplasm (H ," I. x2(0).
Figure 2. Endoscopic view of esophageal polyp on a short stalk.
of myelin sheaths and axis cylinders.? Electron microscopic studies have concluded that the tumor is of Schwann cell origin. 8 Granular cell tumors are often asymptomatic, but the esophageal leSions may produce dysphagia when they reach 1 cm in size. 9 The radiologic and endoscopic appearance of granular cell tumors is nonspecific. The usual preoperative diagnosis is leiomyoma since leiomyomas constitute the majority of benign esophageal tumors. lO Other lesions to consider in the VOLUME 27, NO.2, 1981
differential diagnosis are benign tumors such as fibromas, lipomas, neurofibromas, and hemangiomas 3 Additional esophageal tumors with similar appearance include leiomyosarcoma, carcinosarcoma, inclusion or duplication cysts, fibrovascular polyps, and squamous papillomas. 9 - 11 Grossly, the granular cell tumors are small, firm, and 4 usually not painful or tender. Microscopically, they are formed of masses of large polygonal cells with small hyperchromatic nuclei and abundant cytoplasm with small acidophilic granules. 12 The cells individually or in groups are surrounded by a thin network of con nective tissue fi bers. 13 Management decisions are difficult because of the rarity of this entity. Some authors caution against biopsy because of the risk of bleeding. 4 , 9 Others suggest that the pseudoepitheliomatous hyperplasia of the mucosa overlying the granular cell tumor may be confused with squamous cell carcinoma. 13 However, since granular cell tumors are rare and impossible to identify visually, a biopsy is necessary to establish the diagnosis. One should not biopsy a smooth submucosal lesion that is most likely a leiomyoma as the biopsy creates the potential for an esophageal fistula through the mucosal defect if surgery is performed. 11 Moreover, it is generally impossible to make a tissue diagnosis with submucosal lesions. In cases in which the lesion is pedunculated and appears as a polyp, as in this case, an attempt may be made at transendoscopic snare removal. The rubbery 71
consistency makes removal more difficult than the usual polyp. Most granular cell tumors will not be pedunculated, in which case local wide excision is the treatment of choice. Because the cells infiltrate the surrounding tissue or pseudocapsule, recurrence may follow simple enucleation or snare removal. At 3 months our patient has no evidence for residual disease at repeat endoscopy. It is important to recognize that experience with endoscopic removal of polypoid tumors of the esophagus is limited. The procedure has no proven safety record and should only be attempted by skilled endoscopists when the polyp is clearly on a stalk. Although polypectomy is possible for pedunculated tumors of this type, most will be submucosal and should not be biopsied because of the potential risk of fistula formation. Benign granular cell tumors of the esophagus will generally require wide local excision. REFERENCES 1. REYES CV, SATINDER K, MOLNAR Z: Granular cell tumor of the esophagus: a case report. J Clin Gastroenterol 2:365, 1980
72
2. KESHISHIAN jM, ALFORD TC: Granular cell myoblastoma of the esophagus. Am Surg 30:263, 1964 3. FLEGE I B, EDMONDS TT: Granular cell myoblastoma of the esophagus. J Thorac Cardiovasc Surg 58:217. 1969 4. CALHOUN T, ODELOWO E, All S: Granular cell myoblastoma: another unusual esophageal lesion. J Thorac Cardiovasc Surg 79: 472, 1975 5. FARRELL KH, DEVINE KD, HARRISON EG: Granular cell myoblastoma of the esophagus: incidence and surgical treatment. Ann Otol Rhinol Laryngol 82:784, 1973 6. CRAWFORD ES, DEBAKEY ME: Granular cell myoblastoma: two unusual cases. Cancer 6:786, 1953 7. BANGLE R: A morphological and histochemical study of the granular cell myoblastoma. Cancer 5:950, 1952 8. SOBEL HI. MARQUET E, AVRIN E: Granular cell myoblastoma. Am J Pathol 65:59, 1971 9. GESHWIND MF, C~IIAT H, ADDEI K: Granular cell tumors of the esophagus. Gastrointest Radiol 2:327, 1978 10. CHI PS, ADAMS W: Benign tumors of the esophagus. Arch Surg 60:92,1950 11. POPE CE: Tumors, in Gastrointestinal Diseases, Ed. 2, Sieisenger MH, Fordtran jS, eds. Philadelphia, Saunders, 1978, p. 574 12. DEGouvEIA OF, PEREIRA A, NETTA MB: Granular cell myoblastoma of the esophagus (Abrikossof's tumor). Gastroenterology 38:805, 1960 13. ELIAS EG, VALENZUELA L, PICKREN jW: Granular cell myoblastoma. J Surg Oneal 2:33, 1970
GASTROINTESTINAL ENDOSCOPY
following this procedure. In some instances, a 1-second exposure may still result in too light an image. This can be corrected by screwing a neutral density filter into the bottom of the lens before the picture is to be exposed. This filter decreases the amount of light coming through without affecting color, image, or size. The filters are in series and graded from 1 to 6. The purchase of filters no. 2 and no. 4 will cover most situations. It may be easier to buy a Spiratone (Flushing, NY) variable neutral density filter. The degree of filtration can be adjusted simply by turning the ring which is an integral part of the filter. This eliminates the need for handling multiple filters. After some experience is gained, one is usually able to judge more easily the proper exposure for obtaining an excellent print (Fig. 1).
Endoscopic removal of a granular cell tumor of the esophagus
longitudinal esophageal fold above the lesion contributed to the appearance of a stalk. Biopsies showed unremarkable squamous epithelium with smooth muscle seen in one fragment. The lesion appeared to be freely moveable on a short stalk and we performed transendoscopic resection with a wire snare and electrosurgical unit. Transection of the stalk appeared to require more than the usual amount of current. When the polyp was removed, a O.B-cm diameter burn was noted at the polypectomy site. The patient tolerated the procedure well and had an uneventful subsequent course with no dysphagia. On cut section the polyp revealed a homogeneous, creamy white, moderately firm surface. Microscopically the polyp consisted predominately of large polygonal granular cells covered by a stratified squamous epithelium with slight inflammation (Fig. 3). Follow-up endoscopy at 3 months showed complete healing of the esophagus. No mucosal or submucosal lesions were seen.
Robert S. Sandler, MD David R. Wood, MD Eugene M. Bozymski, MD
Granular cell tumors are rare lesions of neural origin. Since their initial description in 1926 by Abrikossoff, there have been only 22 cases in which the tumor was found in the esophagus. 1 We report the first case in which a granular cell tumor was removed by transendoscopic wire snare. CASE REPORT A 53-year-old woman was referred for evaluation of a 2month history of dysphagia for solid foods and postprandial vomiting. Ten months earlier she was evaluated for epigastric pain at a local hospital. An upper gastrointestinal series revealed tertiary contractions of the distal esophagus and an oral cholecystogram was negative. Her medications on admission included cimetidine, propranolol for hypertension, and aspirin for osteoarthritis. She admitted to moderate alcohol use. Her physical examination was unremarkable except for the presence of occult blood in the stool. Sigmoidoscopy and barium enema examination were normal. An upper gastrointestinal series demonstrated a 2.5-cm diameter polypoid lesion in the distal esophagus (Fig. 1). Esophagoscopy revealed a pinkish tan polypoid lesion 30 cm from the incisors (Fig. 2). The lesion had a firm, rubbery consistency, was covered by normal appearing mucosa, and appeared to be attached by a short stalk. A From the Division of Digcstive Diseases and Nutrition, Department of Medicine, University of North Carolina School of Mcdicine, Chapel Hill, North Carolina. This investigation was supported in part by National Canccr Institute Grant CA 17973. Reprint rcquests: Robert S. Sandler, MD, Division of Digestivc Diseascs and Nutrition, Room 324, Clinical Sciences Building 229H, University of North Carolina School of Medicine, Chapel Hill, North Carolina 27.514.
70
DISCUSSION Granular cell tumors may be found throughout the body but have a predilection for the upper digestive and respiratory tract, with a third of the lesions found in the tongue. 2 - 4 Other common sites include the skin, subcutaneous tissue, and the breast 4 The lesion most commonly occurs in the 3rd to 5th decade 5 Granular cell tumors of the esophagus are more common in women (79%) and are multiple in 25% of cases. Malignant granular cell tumors have been reported but are much less common than the benign variety.6 When the tumors are malignant, they are often slow growing. 6 One should distinguish a multicentric tumor from a malignant tumor with metastases 5 Although these lesions are often termed granular cell myoblastomas, they are thought to be of neural origin. In 1952 Bangle presented evidence based on histologic and histochemical studies that showed that the granular material was derived from degeneration GASTROINTESTINAL ENDOSCOPY
Figure 1. Karium swallow showing a polYPc)I(j lesion in the distal esophagus.
Figure 3. I llstologic appearance' 01 gralluldl ((,II tumm ,hO\\Ing large polygollal cells With grallular cytoplasm (H ," I. x2(0).
Figure 2. Endoscopic view of esophageal polyp on a short stalk.
of myelin sheaths and axis cylinders.? Electron microscopic studies have concluded that the tumor is of Schwann cell origin. 8 Granular cell tumors are often asymptomatic, but the esophageal leSions may produce dysphagia when they reach 1 cm in size. 9 The radiologic and endoscopic appearance of granular cell tumors is nonspecific. The usual preoperative diagnosis is leiomyoma since leiomyomas constitute the majority of benign esophageal tumors. lO Other lesions to consider in the VOLUME 27, NO.2, 1981
differential diagnosis are benign tumors such as fibromas, lipomas, neurofibromas, and hemangiomas 3 Additional esophageal tumors with similar appearance include leiomyosarcoma, carcinosarcoma, inclusion or duplication cysts, fibrovascular polyps, and squamous papillomas. 9 - 11 Grossly, the granular cell tumors are small, firm, and 4 usually not painful or tender. Microscopically, they are formed of masses of large polygonal cells with small hyperchromatic nuclei and abundant cytoplasm with small acidophilic granules. 12 The cells individually or in groups are surrounded by a thin network of con nective tissue fi bers. 13 Management decisions are difficult because of the rarity of this entity. Some authors caution against biopsy because of the risk of bleeding. 4 , 9 Others suggest that the pseudoepitheliomatous hyperplasia of the mucosa overlying the granular cell tumor may be confused with squamous cell carcinoma. 13 However, since granular cell tumors are rare and impossible to identify visually, a biopsy is necessary to establish the diagnosis. One should not biopsy a smooth submucosal lesion that is most likely a leiomyoma as the biopsy creates the potential for an esophageal fistula through the mucosal defect if surgery is performed. 11 Moreover, it is generally impossible to make a tissue diagnosis with submucosal lesions. In cases in which the lesion is pedunculated and appears as a polyp, as in this case, an attempt may be made at transendoscopic snare removal. The rubbery 71
consistency makes removal more difficult than the usual polyp. Most granular cell tumors will not be pedunculated, in which case local wide excision is the treatment of choice. Because the cells infiltrate the surrounding tissue or pseudocapsule, recurrence may follow simple enucleation or snare removal. At 3 months our patient has no evidence for residual disease at repeat endoscopy. It is important to recognize that experience with endoscopic removal of polypoid tumors of the esophagus is limited. The procedure has no proven safety record and should only be attempted by skilled endoscopists when the polyp is clearly on a stalk. Although polypectomy is possible for pedunculated tumors of this type, most will be submucosal and should not be biopsied because of the potential risk of fistula formation. Benign granular cell tumors of the esophagus will generally require wide local excision. REFERENCES 1. REYES CV, SATINDER K, MOLNAR Z: Granular cell tumor of the esophagus: a case report. J Clin Gastroenterol 2:365, 1980
72
2. KESHISHIAN jM, ALFORD TC: Granular cell myoblastoma of the esophagus. Am Surg 30:263, 1964 3. FLEGE I B, EDMONDS TT: Granular cell myoblastoma of the esophagus. J Thorac Cardiovasc Surg 58:217. 1969 4. CALHOUN T, ODELOWO E, All S: Granular cell myoblastoma: another unusual esophageal lesion. J Thorac Cardiovasc Surg 79: 472, 1975 5. FARRELL KH, DEVINE KD, HARRISON EG: Granular cell myoblastoma of the esophagus: incidence and surgical treatment. Ann Otol Rhinol Laryngol 82:784, 1973 6. CRAWFORD ES, DEBAKEY ME: Granular cell myoblastoma: two unusual cases. Cancer 6:786, 1953 7. BANGLE R: A morphological and histochemical study of the granular cell myoblastoma. Cancer 5:950, 1952 8. SOBEL HI. MARQUET E, AVRIN E: Granular cell myoblastoma. Am J Pathol 65:59, 1971 9. GESHWIND MF, C~IIAT H, ADDEI K: Granular cell tumors of the esophagus. Gastrointest Radiol 2:327, 1978 10. CHI PS, ADAMS W: Benign tumors of the esophagus. Arch Surg 60:92,1950 11. POPE CE: Tumors, in Gastrointestinal Diseases, Ed. 2, Sieisenger MH, Fordtran jS, eds. Philadelphia, Saunders, 1978, p. 574 12. DEGouvEIA OF, PEREIRA A, NETTA MB: Granular cell myoblastoma of the esophagus (Abrikossof's tumor). Gastroenterology 38:805, 1960 13. ELIAS EG, VALENZUELA L, PICKREN jW: Granular cell myoblastoma. J Surg Oneal 2:33, 1970
GASTROINTESTINAL ENDOSCOPY