Granular cell myoblastoma of the esophagus: endoscopic diagnosis and therapy

Granular cell myoblastoma of the esophagus: endoscopic diagnosis and therapy

0016-5107/85/3103-0022$02.00/0 GASTROINTESTINAL ENDOSCOPY Copyright © 1985 by the American Society for Gastrointestinal Endoscopy Case Re po rts Gran...

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0016-5107/85/3103-0022$02.00/0 GASTROINTESTINAL ENDOSCOPY Copyright © 1985 by the American Society for Gastrointestinal Endoscopy

Case Re po rts Granular cell myoblastoma of the esophagus: endoscopic diagnosis and therapy Fabio Farinati, MD Giacomo Carlo Sturniolo, MD Attilio Cecchetto, MD Massimo Rugge, MD Remo Naccarato, MD

Granular cell myoblastoma is a rare neoplasm, usually found in the tongue, subcutaneous tissues, and breast. Esophageal localization is unusual. Only 44 cases have been reported in the literature since Abrikossof,1 in 1931, first described a case of granular cell myoblastoma of the proximal portion of the esophagus. The neoplasm affects females more often than males, occurs in middle age, and has a small chance of becoming malignant. 2 Patients complain of dysphagia or chest and substernal pain. Frequently there are multiple foci or an association with other tumors such as gastric, laryngeal, and esophageal carcinoma. 3- 4

Figure 1. Endoscopic appearance of the polypoid lesion in the esophagus (arrow).


A 35-year-old man presented with mild epigastric discomfort for 3 months. A barium meal suggested a duodenal ulcer, but endoscopy revealed only a sessile polypoid lesion of the lower third of the esophagus. The diameter of the polyp was about 1.5 em, and it was covered by a smooth, white mucosa (Fig. 1). A biopsy of the lesion demonstrated hyperkeratosis of the epithelium. An endoscopic follow-up 3 months later showed persistence of the lesion, and we decided to remove the lesion endoscopically. Histological examination of the polyp demonstrated a granular cell myoblastoma of the esophagus characterized by irregularly arranged strands and nests of large granular polyhedral acidophilic cells, embedded in a delicate nest of reticular tissue. The cell borders were generally sharp and formed syncytial polynucleated masses. The finely granular cytoplasm reacted positively with periodic acid-Schiff, and the small nuclei were densely hyperchromatic (Fig. 2).


The esophageal myoblastoma is structurally identical to those developing in striated muscle, but the From Cattedra di Gastroenterologia, Clinica Medica I, and Istituto diAnatomia Patologica, Universita di Padova, Padova, Italy. Reprint requests: Prof. Remo Naccarato, Cattedra di Gastroenterologia, Clinica Medica I, Policlinico Universitario, Via Giustiniani 2, 35100, Padova, Italy.


Figure 2. Myoblastoma of the esophagus showing the typical granular cells (x48).

hypothesis of a myomatous origin for this tumor is in doubt. An origin from undifferentiated mesenchymal cells was in fact postulated by Aparicio and Lumsdel5 and by Sobel et al.,6 who considered the Schwann cell GASTROINTESTINAL ENDOSCOPY

the cell of origin. In this regard, Pearse 7 considered myoblastoma a variant of perineural fibroblastoma in which the cells had accumulated lipid complexes. This concept has received further support from ultrastructural and histochemical studies. R- Io Early endoscopy in patients with upper digestive tract complaints is the reason for both the relatively increased frequency for the diagnosis of granular cell myoblastoma and the reduction in the number of cases with extensive invasion of surrounding tissues l l and with lymph node metastasis. 12 Nevertheless, cases with dysphagia caused by large polypoid lesions still occur. 13 Biopsy of smooth, submucosal lesions is usually unsatisfactory.14 All polypoid lesions of digestive tract should be removed whenever feasible since only this approach guarantees an accurate diagnosis. Endoscopic resection may be used not only in pedunculated polyps but also in sessile ones, if it is possible to create a pseudostalk with the wire snare. This method has been successfully applied by Sandler et a1.,15 although Wypkema et a1. 16 removed a small myoblastoma by excisional biopsies. Neither group of authors could demonstrate any recurrence after a variable period of follow-up. In a recent report l7 of six cases, endoscopic excision was not undertaken, however. We believe that these esophageal lesions should be treated by endoscopic polypectomy (without requiring previous biopsy) not only in pedunculated polyps but also, whenever possible, in sessile polyps, and that surgical resection should only be resorted to in invasive or recurrent cases. This approach is justified by the possibility of malignant degeneration, the safety of endoscopic excision, and the difficulty of surgical resection.

Triple lumen pylorus-a complication of peptic ulcer Frank M. Moses, MD, Maj, MC David A. Peura, MD, Lt Col, MC Lawrence F. Johnson, MD, Col, MC

A gastroduodenal fistula consists of an additional channel found adjacent to the pylorus connecting the gastric antrum and the duodenal bulb. Since the first major review by Notkin in 1939,1 several publications From the Department of Medicine, Walter Reed Army Medical Center; and the Uniformed Services University of the Health Sciences. Washington, D.C. Reprint requests: David A. Peura. MD. Director, Clinical Services. Gastroenterology Service. Walter Reed Army Medical Center, Washington. D.C. 20012. The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. VOLUME 31. NO. I, 1985

REFERENCES 1. Abrikossof AL. Weitere untersuchunge uber myoblastenmyome. Virchows Arch [Pathol Anat] 1931;280:723. 2. Howe RW, Postletwait RW. Granular cell myoblastoma of the esophagus. Surgery 1981;89:701. 3. Clemencon G, Gloor F. Myoblastic myoma (granular cell tumor) of the esophagus, Z. Gastroenterol 1975;13:529. 4. Elias EG, Valenzuela L, Pickren JW. Granular cell myohlastoma. J Surg Oncol 1970;2:33. 5. Aparicio SR, Lumsdel CEo Light and electron microscopic studies on the granular cell myoblastoma of the tongue. J Pathol 1969;97:339. 6. Sobel HJ, Schwarz R, Marquet E. Light and electron microscopic study of the origin of granular cell myoblastoma. J Pathol 1973;109:101. 7. Pearse AGE. The histogenesis of granular cell myoblastoma (granular cell perineural fibroblastoma). J Pathol Bacteriol 1950;62:351. 8. Burston J, John R, Spencer M. Myoblastoma of the neurohipophysis. J Pathol Bacteriol 1962;83:455. 9. Fisher ER, Wechsler H. Granular cell myoblastoma. A misnomer: electron-microscopic and histochemical evidence concerning its Schwann cell derivation and nature (granular cell Schwannoma). Cancer 1962;15:936. 10. McCormack LJ, Hazard JB, Dickinson JA. Malignant epithelioid neurilemoma (Schwannoma). Cancer 1954;7:725. 11. Crawford ES, De Bakey ME. Granular cell myoblastoma. Two unusual cases. Cancer 1953;6:786. 12. Obiditish-Mayer I, Salzer-Kuntschik M. Malignes, "Gekonntzelliges neuron" sogennantes "myoblastenmyom" des esophagus. Beitr Pathol Anat 1961;125:357. 13. Farrel KH, Devine KD, Harrison EG, OIenn AM. Granular cell myoblastoma of the esophagus. Incidence and surgical treatment. Ann Otol Rhinol Laryngol 1973;82:784. 14. Gerschwind ME, Chiat H, Addei KA, Ferraro LR. Granular cell tumors ofthe oesophagus. Gastrointest RadioI1978;2:327. 15. Sandler RS, Wood DR, Bozymsky EM. Endoscopic removal of a granular cell tumor of the esophagus. Gastrointest Endosc 1981;27:70. 16. Wypkema W, Schmaman A, Berson D. Granular cell myoblastoma of the esophagus. A report of two cases. S Afr Moo J 1967;41:911. 17. Frazin G, Capitanio A, Manfrini G, Musolar R, Tomba A, Fratton A. Granular cell tumors of the esophagus: report of 6 cases and review of the literature. Ital J Gastroenterol 1982;4:230.

have reported isolated cases and reviews of the gastroduodenal fistula. 2-5The etiology of the gastroduodenal fistula is thought to be acquired in the majority of cases.5-15 A triple lumen pylorus has been reported as an unexpected finding at autopsy. 2 We report a patient who developed a triple lumen pylorus as a complication of peptic ulcer disease. Our case is unique in that the triple lumen pylorus was diagnosed by endoscopy and radiology and clinically followed for 2lf2 years. CASE REPORT

A 57-year-old white woman was well until 3 years before referral to Walter Reed Army Medical Center, when duodenal ulcer disease was diagnosed and symptomatically treated with antacids. She was then well until 8 months before referral when recurrent abdominal discomfort developed and an upper gastrointestinal series demonstrated a 23