Journal of Surgical Oncology 33:246-249 (1986)

Symptomatic Granular Cell Tumor of the Esophagus

DEBA P. SARMA, MD, FRED H. RODRIGUEZ, Jr., MD, ERIC0 M. DEIPARINE, MD,

AND THOMAS G. WEILBAECHER, MD

From the Department of Pathology and Radiology, Veterans Administration Medical Center and Louisiana State University Medical School, New Orleans

A case of symptomatic granular cell tumor of the esophagus is described. Another 32 cases collected from the English literature are analyzed. The reported cases have occurred mostly in women of less than 50 years of age presenting with dysphagia. Excision of the lesion is curative.

KEY WORDS: granular cell tumor of the esophagus, benign tumor of the esophagus, rare esophageal tumor, granular cell tumor

INTRODUCTION Granular cell tumor is a rare benign neoplasm of the

esophagus. About 71 such cases have been reported in the English literature [ l a ] . About 60% of those patients had clinical symptoms due to the esophageal lesion. In others the tumor was discovered incidentally in the course of investigation for other problems or was found at autopsy.

We are reporting a symptomatic case of esophageal granular cell tumor. A survey of the English literature is made to analyze such symptomatic cases.

CASE REPORT A 73-year-old black man came to the hospital with a

complaint of a sore throat and difficulty in swallowing. Two years previously the patient had undergone a total laryngectomy and left radical neck dissection for squa- mous cell carcinoma. Physical examination did not reveal any evidence of recurrent or metastatic tumor. An eso- phagogram (Fig. 1) revealed a polypoid, 1.8 X 1 cm filling defect located at the thoracic outlet level of the esophagus. Esophagoscopy disclosed a yellow peduncu- lated mass, a biopsy of which showed granular cell tumor (Fig. 2). Sheets of large polygonal cells showed abundant eosinophilic, finely granular cytoplasm and small, round, uniform nuclei. The overlying squamous epithelium was intact with mild pseudoepitheliomatous hyperplasia. Electron microscopic study showed each cell with contin- Fig. 1. Esophagogram showing a filling defect in the upper

esophagus. uous basal lamina and innumerable, irregular, mem- Accepted for publication March 26, 1985. brane-bound lysosomal-type granules (Fig. 3 ) .

The patient refused further therapy for the Address reprint requests to D.P. Sarma, MD, 1601 Perdido Street, lesion. Five years later, during a follow-up evaluation, New Orleans, LA 70146.

01986 Alan R. Liss, Inc.

Esophageal Granular Cell Tumor 247

Fig. 2 . (Hematoxylin and eosin, magnification, X 120).

Biopsy of the lesion showing intact squamous epithelium overlying typical granular cell tumor

Fig. 3. membrane-bound granules in the cytoplasm. (original magnification, X4400).

Ultrastructurally, the granular cells enclosed by continuow basal lainina contain numcrous

248 Sarma et a1

TABLE I. Symptomatic Granular Cell Tumors of the Esophagus

Authorslyear

Crawford et al, 1953 [5]

De Gouveia et at, 1960 161 Rella et al, 1963 [7]

Wypkema et al, 1967 [8]

Gallia, I968 191

Flege et al, 1969 [I01

Paskin et al, 1972 [ I 11 Ming, 1973 1121

Larsen et al, 1973 [I31

Farrell et al, 1973 [I41

Collett, 1974 [ 151

O’Connell et at, 1978 [I61

Gershwind et al, 1978 1171

Domen et al, 1979 [ 181

Gibbons et al, 1980 [I91

Flood, 1981 [20]

Johnston et al, 1981 141

Howe et al, 1981 [211

Sandler et al, 1981 1221

Rubesin et al, 1985 [3]

Vuyk et at, 1985 [2]

Sarma et al,

Clinical Granular cell tumor AgeISexlRace presentation Location Gross observation in other sites

30lFlblack

19lFlwhite 43/F/white

40IFlblack 43lFlblack 48/Fl?

40lMI?

?IF/? 4 I IF/?

40lMlwhite

24/F/?

58/Fl? 58/M/?

48/F/?

36lFlblack

56lFlwhite

28lFlblack

651Flwhite

29/M/?

5 1 /MI?

23lFlwhite 39lFlwhite 43/Ml? 42/F/? 53/M/? 46lFloriental 30/F/white 42lFloriental

39lMlblack

39/M/black

37lFlblack

40/F/white

73lmlblack

Dysphagia

Dysphagia Dy sphagia

Dysphagia Dysphagia Substernal discomfort Substernal pain, regurgitation of food Nausea, vomiting Postprandial pain, regurgitation of food Symptoms of esoph- ageal reflux Dy sphagia

Dysphagia Dysphagia

Dysphagia

Dysphagia

Dysphagia, sub- sternal pain Dysphagia, heart burn

Dysphagia

Dysphagia

Dysphagia, heart burn Dysphagia Dysphagia Dysphagia Dysphagia Dysphagia Epigastric pain Dysphagia Nausea, vomit- ing, anorexia Dysphagia

Dysphagia post- prandial vomiting Dysphagia sub- sternal pain, nausea Dysphagia

Dysphagia

Upper third

Upper third Upper third

Upper third Lower third Lower third

Lower third

Middle third Middle third

Lower third

Upper third

Lower third Lower third

Upper third

Middle and lower third Middle and lower third Lower third

Upper third

Lower third

Lower third

Upper third ?

Lower third Lower third Lower third

? ?

Middle third

Middle third

Lower third

Lower third

Upper third

Upper third

Ill-defined, “golf ball”-size mass Concentric narrowing Intramural “walnut” size mass Annular, 2-cm size Stenotic lesion Intramural mass

Polypoid, I-cm mass

Intramural, 1-cm mass Sessile submucosal mass, 1.5 cm

Yellow, submucosal mass, 1 cm

Stenotic lesion, 4 x 2 x 0.5 cm Stenotic mass Yellow-tan, rubbery mass, 2 cm Yellow, fibrous, nodular mass, 2 X 1 cm Three submucosal masses Two submucosal masses Submucosal mass, 2 x 1 x 1 cm White mucosalO.4 em lesion Yellow, submucosal mass, 2 x 2 cm Yellow, elevated, 1 cm mucosal mass 5 ~ 2 ~ 2 m m

? ? ’l

Two lesions, one 4 x 6 mm submucosal, one 2 X 3 mm polypoid Polypoid mass, 2.5 cm

Two submucosal nodules, 1.5 cm and 1 cm Stenotic, 4-cm white mass Polypoid, yellow, I .8 X 1 cm mass

Skin, tongue, uterous, ovaries No No

No No No

No

Bronchus No

No

No

No No

No

Bronchus, trachea, stomach, pericardium Skin

No

No

No

No

No Skin NO Skin No No No No

No

No

Skin, tongue, breast, vulva

No

(present case) No

Esophageal Granular Cell Tumor 249

Rubesin S, Herlinger H, Sigal H: Granular cell tumors of the esophagus. Gastrointest Radiol 10: 11-15, 1985. Johnston J, Helwig EB: Granular cell tumors of the gastrointes- tinal tract and perianal region. A study of 74 cases. Dig Dis Sci 2:807-816, 1981. Crawford ES, DeBakey ME: Granular-cell myoblastoma. Two unusual cases. Cancer 6:786-789, 1953. DeGouveia OF, Pereira AA, Netto MB, Vilhena AM, Dutra G, Bryk D: Granular cell myoblastoma of the esophagus (Abrikos- soff‘s tumor). Gastroenterology 38:805-809, 1960. Rella AJ, Conte AJ, Farrell JT: Granular cell myoblastoma of the esophagus. Arch Otolaryngol 78:715-718, 1963. Wypkema W, Schmaman A, Berson D: Granular cell myoblas- toma of the oesophagus - a report of 2 cases. S Afr Med J

Gallia FJ: Intramural lesion of esophagus. JAMA 204: 1065- 1066, 1968. Flege JB, Edmonds TT: Granular cell myoblastoma of the esoph- agus. A case report. J Thorac Cardiovasc Surg 58:217-222, 1967. Paskin DL, Hull JD, Cookson PJ: Granular cell myoblastoma; A comprehensive review of 15-years experience. Ann Surg 17S:SOl- 503, 1972. Ming S: Tumors of the esophagus and stomach. Atlas of tumor pathology. Fascicle 7. Washington, DC: Armed Forces Institute for Pathology, pp 73-74. Larsen GL, Armstrong RG, Stanford W. Cline RE: Granular cell myoblasma of the oesophagus. Thorax 28:64-643, 1973. Farrell KH, Devine KD, Harrison EG, Olsen AM: Granular cell myoblastoma of the esophagus. Incidence and surgical treatment. Ann Otolaryngol 82:784-789, 1973. Collett HS: Granular cell myoblastoma of the esophagus. Rocky Mt Med J 71;145-147, 1974. O’Connell DJ, MacMahon H, DeMeester TR: Multicentric trach- eobronchial and oesophageal granual cell myoblastoma. Thorax

Gershwind ME, Chiat H, Addei KA, Ferraro LR: Granular cell tumors of the esophagus. Gastrointest Radiol 2:327-330, 1978. Domen RE, Tang P, Harshman KV: Granular cell myoblastoma of the esophagus after irradiation for carcinoma. South Med J

Gibbons JRP, Bharucha H, Soorae AS: Granular cell tumor of the esophagus. Am J Gastroenterol 74: 161-164, 1980.

41:911-912, 1967.

331596-602, 1978.

72: 1207-1209, 1979.

esophagoscopy revealed the presence of the polypoid lesion that did not change in size. A biopsy showed exactly the same morphology of the tumor as was noted earlier. The patient, however, had developed an infiltrat- ing adenocarcinoma of the sigmoid colon with metastases to paracolonic lymph nodes.

COMMENT

Table I summarizes the essential features of 32 cases of symptomatic granular cell tumor of the esophagus reported in the English literature and one additional case from the present communication. The tumor occurs about three times as often in women as in men. Ages range from 19 to 73 years, with most patients below 50 years of age. There is no racial predominance. About a quarter of the patients may have granular cell tumors in other sites of the body, such as skin, tongue, and bronchus. Dysphagia is the most common presenting symptom. The tumor is usually single. It may be located in any part of the esophagus, with a slight predominance of lesions reported in the lower half. The tumor may be a stenotic lesion, an intramural mass, a submucosal yellow nodule, or a luminal polypoid mass varying in size from a few millimeters to several centimeters.

Granular cell tumor of the esophagus is a benign neo- plasm curable by surgical or sometimes endoscopic ex- cision. Local recurrence is not uncommon, though malignant degeneration has not been reported.

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