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Granular cell myoblastoma: Another unusual esophageal lesion
Granular cell myoblastoma: Another unusual esophageal lesion The case is presented of a 48-year-old woman with esophageal granular cell myoblastoma who was treated by local excision. A review of the literature is also included. So far as we can determine, this is the thirteenth such case reported.
Thomas Calhoun, M.D., F.A.C.S., Ezekiel O. O. Odelowo, M.B., B.S., Subhi Ali, M.D., Lewis H. Kurtz, M.D., F.A.C.S., and Robert H. Simmons, M.D., F.A.C.S., Washington, D. C.
kJince the first description of granular cell myoblastoma in 1926 by Abrikosoff,1 in the esophagus, the origin and truly neoplastic nature of the lesion have been the subject of much controversy. Paskin and colleagues1- estimated that fewer than 1,000 cases had been reported by 1971. Of these, only 11 involved the esophagus. Elias and associates" reported an autopsy finding of granular cell myoblastoma of the lower esophagus in a 59-year-old man dying of extensively metastasized carcinoma of the larynx. So far as we know, ours is the thirteenth case of granular cell myoblastoma of the esophagus to be reported in the literature. Case report F. W., a 48-year-old black woman with diabetes, presented for a routine follow-up clinical appointment on Oct. 31, 1973. The level of blood sugar was noted to be 555 mg. per cent. (Jtner complaints included a 12 to 20 pound weight loss over a 2 month period and 1 week of diarrhea, polyuria, and polydipsia. She specifically denied any dysphagia. The woman had had diabetes for 2 years and had been maintained on diabinese, From the Department of Surgery and Medicine, Howard University College of Medicine, Washington, D. C. 20001, and the Department of Pathology, D. C. General Hospital, Washington, D. C. Received for publication June 21, 1974.
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taken twice a day. She had been hospitalized in 1963 with jaundice. Findings from the physical examination were essentially normal except for moderate dehydration. Laboratory data. The white blood count was 17,200 per cubic millimeter, the red blood count 4,500,000 per cubic millimeter, hemoglobin 14.1 Gm., and hematocrit 42 per cent. The electrolytes, in milliequivalents per liter, were as follows: sodium 133, potassium 3.2, chlorine 84, and carbon dioxide 12. The blood urea nitrogen was 5 and glucose 555 mg. per cent. Urine sugar was 4+ and acetone 3+. A chest roentgenogram was normal. Abdominal x-ray films showed calcification in the tail of the pancreas and calcified uterine fibroids. Upper gastrointestinal series showed an extramucosal, smooth filling defect at the level of the clavicle (Fig. 1) and a suspicious (inconstant) defect at the level of the carina (Fig. 2 ) . Barium colon enema and a liver scan were normal. After the diabetes was controlled, esophagogastroscopy by means of the fiberoptic endoscope was performed by the gastrointestinal division. The extramucosal lesion was identified and biopsied.* She was transferred to surgery for re-esophagoscopy and excision of the 4 by 3.5 cm. lesion through a right cervical incision (Fig. 3 ) . Frozen and permanent section reports showed the mass to be a granular cell myoblastoma (Fig. 4, A and B). She was discharged asymptomatic with her diabetes controlled 2 weeks after the opera*These lesions should not be biopsied via the endoscope due to the increased likelihood of severe bleeding.
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Fig. 2. Spot film shows suspicious defect.
Fig. I. Esophagogram shows filling defect. tion, and a follow-up esophagogram has appeared completely normal.
Comments Whereas the acanthosis and pseudoepitheliomatous hyperplasia observed in the epidermis overlying granular cell myoblastomas have been related to metabolites released from the tumor cells,' the tumor has not been ascribed any hormonal activity. The diabetic state of our patient did not appear to have been altered by the presence of the tumor. Sites. One third of all granular cell myoblastomas have been located in the tongue. Other common sites include the skin, subcutaneous tissues, and breast. The tumor occurs in voluntary muscles in no greater than 5 per cent of all cases.' It has also been reported by various authors in scattered sites such as the gum as myoblastoma epulis, gallbladder and common bile duct, pituitary stalk, larynx, bronchus and lungs, vulva, colorectum, periadrenal fat, and stomach.
Fig. 3. In situ lesion at the surgeon's thumb.
Origin. The histologic description of the tumor is ascribed to Weber1" (1884). However, Abrikosoff1 advanced the theory of the origin of the tumor from striated muscle, hence the names Abrikosoffs tumor and granular cell myoblastoma."' 1!i Based on electron microscopic and histochemical studies of the tumor, Fisher and Wechsler
The Journal of
474
Calhoun et al.
Thoracic and Cardiovascular Surgery
Fig. 4. A, Low-power view. B, High-power view. suggested granular cell schwannoma as a more suitable name," and Fust and Custer1" gave the name granular cell neurofibroma. These two names emphasized a neural derivation of the lesion. Pathology. The lesion is usually small but may reach 10 cm. in diameter.- It consists of firm or soft, yellow or gray nodules of polygonal cells containing pink-staining granular cells that infiltrate subcutaneous tissues (Fig. 5, A and B). When the lesion is near the skin, the overlying epidermis may be affected by acanthosis and pseudoepitheliomatous hyperplasia which can be easily mistaken for squamous cell carcinoma and an indication for radical surgery. Discussing the lesion in the breast, Ackerman- stated that no other lesion may so closely mimic mammary carcinoma. Although few pathologistsr'- ,:i deny that granular cell myoblastoma is a neoplasm, cases have been reported in which the lesion was found to infiltrate, recur, or metastasize.'- 7 Clinical presentation. Granular cell myo-
blastoma in the esophagus, as in other parts of the body, may present diagnostic and therapeutic problems to the surgeon because accurate preoperative diagnosis is rare. Classically, the lesion is asymptomatic. It becomes symptomatic, however, if it is large enough. Differential diagnosis on esophagoscopy and esophagography of an extramucosal, noninvasive mass will include the benign tumors of the esophagus. Carcinosarcoma should also be considered." Treatment. The tumor should be excised to exclude malignant tumor, to prevent progression to a size that can produce symptoms (e.g., by pressure on or obstruction of vital structures), and for cosmetic reasons." Enucleation is inadequate if cells infiltrate the pseudocapsule or surrounding tissue, for recurrence is certain. Local wide excision that ensures free margins is dictated by the local anatomy. If permanent sections show atypism or numerous mitoses, wider excision or resection is indicated." The effect of lymph node dissection is unknown. Radium applied
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Fig. 5. A, Low-power view. B, High-power view.
to the tumor on the tongue had no effect on the tumor but caused sloughing of the tongue.11 The effect of supervoltage irradiation is unknown. REFERENCES 1 Abrikosoff, A.: Ober Myome, ausgebend von der guergestreiften wiukurlichen Muskalatur, Virchow. Arch. [Pathol. Anat.] 260: 215, 1926. 2 Ackerman, L. V.: in Surgical Pathology, St. Louis, 1968, The C. V. Mosby Company, p. 736. 3 Anderson, W.: in Boyd's Pathology for the Surgeon, ed. 8, Philadelphia and London, 1967, W. B. Saunders Company, pp. 693, 719-720. 4 Archer, F . L., Harrison, R. W., and Moulder, P. V.: Granular Cell Myoblastoma of the Trachea and Carina Treated by Resection and Reconstruction, J. THORAC. CARDIOVASC. SURG.
45: 539, 1963. 5 Azzopardi, J. G.: On Histogenesis of Granular Cell Myoblastoma, J. Pathol. 71: 85, 1956. 6 Calhoun, T., Ali, S. D., Muna, D., Kurtz, L. H., Simmons, R. L., and Nash, E. C : Carcinosarcoma of the Esophagus: Case Report and Review of the Literature, J. THORAC. CARDIOVASC. SURG. 66: 315, 1973.
7 Colberg, J. E., and Hubay, C. A.: Granular
Cell Myoblastoma, Surgery 5 3 : 226, 1963. 8 Elias, E. G., Valenzuela, L., and Pickerin, J. W.: Granular Cell Myoblastoma, J. Surg. Oncol. 2: 33, 1970. 9 Fisher, E. R., and Wechsler, H.: Granular Cell Myoblastoma—A Misnomer, Cancer 15: 936, 1962. 10 Fust, J. A., and Custer, R. P.: On Neurogenesis of the So-Called Granular Cell Myoblastoma, Am. J. Clin. Pathol. 19: 522, 1949. 11 Horn, R. C , Jr., and Stout, A. P.: Granular Cell Myoblastoma, Surg. Gynecol. Obstet. 76: 315, 1943. 12 Paskin, D. L., Hull, J. D., and Cookson, P. J.: Granular Cell Myoblastoma: A Comprehensive Review of 15-Year Experience, Ann. Surg. 175: 501, 1972. 13 Shear, M.: The Histogenesis of the So-Called Granular Cell Myoblastoma, J. Pathol. 80: 225, 1960. 14 Walter, W. L.: Granular Cell Myoblastoma of the Larynx With Presentation of 2 Cases, Ann. Otol. Rhinol. Laryngol. 69: 328, 1960. 15 Ward, P. H., and Oshiro, H.: Laryngeal Granular-Cell Myoblastoma, Arch. Otolaryngol. (Chicago) 76: 239, 1962. 16 Weber, C. D.: Cited by Blashi, S. F . : Myoblastoma of the Larynx, Ann. Otol. Rhinol. Laryngol. 69: 115, 1960.
Thomas Calhoun, M.D., F.A.C.S., Ezekiel O. O. Odelowo, M.B., B.S., Subhi Ali, M.D., Lewis H. Kurtz, M.D., F.A.C.S., and Robert H. Simmons, M.D., F.A.C.S., Washington, D. C.
kJince the first description of granular cell myoblastoma in 1926 by Abrikosoff,1 in the esophagus, the origin and truly neoplastic nature of the lesion have been the subject of much controversy. Paskin and colleagues1- estimated that fewer than 1,000 cases had been reported by 1971. Of these, only 11 involved the esophagus. Elias and associates" reported an autopsy finding of granular cell myoblastoma of the lower esophagus in a 59-year-old man dying of extensively metastasized carcinoma of the larynx. So far as we know, ours is the thirteenth case of granular cell myoblastoma of the esophagus to be reported in the literature. Case report F. W., a 48-year-old black woman with diabetes, presented for a routine follow-up clinical appointment on Oct. 31, 1973. The level of blood sugar was noted to be 555 mg. per cent. (Jtner complaints included a 12 to 20 pound weight loss over a 2 month period and 1 week of diarrhea, polyuria, and polydipsia. She specifically denied any dysphagia. The woman had had diabetes for 2 years and had been maintained on diabinese, From the Department of Surgery and Medicine, Howard University College of Medicine, Washington, D. C. 20001, and the Department of Pathology, D. C. General Hospital, Washington, D. C. Received for publication June 21, 1974.
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taken twice a day. She had been hospitalized in 1963 with jaundice. Findings from the physical examination were essentially normal except for moderate dehydration. Laboratory data. The white blood count was 17,200 per cubic millimeter, the red blood count 4,500,000 per cubic millimeter, hemoglobin 14.1 Gm., and hematocrit 42 per cent. The electrolytes, in milliequivalents per liter, were as follows: sodium 133, potassium 3.2, chlorine 84, and carbon dioxide 12. The blood urea nitrogen was 5 and glucose 555 mg. per cent. Urine sugar was 4+ and acetone 3+. A chest roentgenogram was normal. Abdominal x-ray films showed calcification in the tail of the pancreas and calcified uterine fibroids. Upper gastrointestinal series showed an extramucosal, smooth filling defect at the level of the clavicle (Fig. 1) and a suspicious (inconstant) defect at the level of the carina (Fig. 2 ) . Barium colon enema and a liver scan were normal. After the diabetes was controlled, esophagogastroscopy by means of the fiberoptic endoscope was performed by the gastrointestinal division. The extramucosal lesion was identified and biopsied.* She was transferred to surgery for re-esophagoscopy and excision of the 4 by 3.5 cm. lesion through a right cervical incision (Fig. 3 ) . Frozen and permanent section reports showed the mass to be a granular cell myoblastoma (Fig. 4, A and B). She was discharged asymptomatic with her diabetes controlled 2 weeks after the opera*These lesions should not be biopsied via the endoscope due to the increased likelihood of severe bleeding.
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March, 1975
Fig. 2. Spot film shows suspicious defect.
Fig. I. Esophagogram shows filling defect. tion, and a follow-up esophagogram has appeared completely normal.
Comments Whereas the acanthosis and pseudoepitheliomatous hyperplasia observed in the epidermis overlying granular cell myoblastomas have been related to metabolites released from the tumor cells,' the tumor has not been ascribed any hormonal activity. The diabetic state of our patient did not appear to have been altered by the presence of the tumor. Sites. One third of all granular cell myoblastomas have been located in the tongue. Other common sites include the skin, subcutaneous tissues, and breast. The tumor occurs in voluntary muscles in no greater than 5 per cent of all cases.' It has also been reported by various authors in scattered sites such as the gum as myoblastoma epulis, gallbladder and common bile duct, pituitary stalk, larynx, bronchus and lungs, vulva, colorectum, periadrenal fat, and stomach.
Fig. 3. In situ lesion at the surgeon's thumb.
Origin. The histologic description of the tumor is ascribed to Weber1" (1884). However, Abrikosoff1 advanced the theory of the origin of the tumor from striated muscle, hence the names Abrikosoffs tumor and granular cell myoblastoma."' 1!i Based on electron microscopic and histochemical studies of the tumor, Fisher and Wechsler
The Journal of
474
Calhoun et al.
Thoracic and Cardiovascular Surgery
Fig. 4. A, Low-power view. B, High-power view. suggested granular cell schwannoma as a more suitable name," and Fust and Custer1" gave the name granular cell neurofibroma. These two names emphasized a neural derivation of the lesion. Pathology. The lesion is usually small but may reach 10 cm. in diameter.- It consists of firm or soft, yellow or gray nodules of polygonal cells containing pink-staining granular cells that infiltrate subcutaneous tissues (Fig. 5, A and B). When the lesion is near the skin, the overlying epidermis may be affected by acanthosis and pseudoepitheliomatous hyperplasia which can be easily mistaken for squamous cell carcinoma and an indication for radical surgery. Discussing the lesion in the breast, Ackerman- stated that no other lesion may so closely mimic mammary carcinoma. Although few pathologistsr'- ,:i deny that granular cell myoblastoma is a neoplasm, cases have been reported in which the lesion was found to infiltrate, recur, or metastasize.'- 7 Clinical presentation. Granular cell myo-
blastoma in the esophagus, as in other parts of the body, may present diagnostic and therapeutic problems to the surgeon because accurate preoperative diagnosis is rare. Classically, the lesion is asymptomatic. It becomes symptomatic, however, if it is large enough. Differential diagnosis on esophagoscopy and esophagography of an extramucosal, noninvasive mass will include the benign tumors of the esophagus. Carcinosarcoma should also be considered." Treatment. The tumor should be excised to exclude malignant tumor, to prevent progression to a size that can produce symptoms (e.g., by pressure on or obstruction of vital structures), and for cosmetic reasons." Enucleation is inadequate if cells infiltrate the pseudocapsule or surrounding tissue, for recurrence is certain. Local wide excision that ensures free margins is dictated by the local anatomy. If permanent sections show atypism or numerous mitoses, wider excision or resection is indicated." The effect of lymph node dissection is unknown. Radium applied
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Granular cell myoblastoma
Number 3
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March, 1975
Fig. 5. A, Low-power view. B, High-power view.
to the tumor on the tongue had no effect on the tumor but caused sloughing of the tongue.11 The effect of supervoltage irradiation is unknown. REFERENCES 1 Abrikosoff, A.: Ober Myome, ausgebend von der guergestreiften wiukurlichen Muskalatur, Virchow. Arch. [Pathol. Anat.] 260: 215, 1926. 2 Ackerman, L. V.: in Surgical Pathology, St. Louis, 1968, The C. V. Mosby Company, p. 736. 3 Anderson, W.: in Boyd's Pathology for the Surgeon, ed. 8, Philadelphia and London, 1967, W. B. Saunders Company, pp. 693, 719-720. 4 Archer, F . L., Harrison, R. W., and Moulder, P. V.: Granular Cell Myoblastoma of the Trachea and Carina Treated by Resection and Reconstruction, J. THORAC. CARDIOVASC. SURG.
45: 539, 1963. 5 Azzopardi, J. G.: On Histogenesis of Granular Cell Myoblastoma, J. Pathol. 71: 85, 1956. 6 Calhoun, T., Ali, S. D., Muna, D., Kurtz, L. H., Simmons, R. L., and Nash, E. C : Carcinosarcoma of the Esophagus: Case Report and Review of the Literature, J. THORAC. CARDIOVASC. SURG. 66: 315, 1973.
7 Colberg, J. E., and Hubay, C. A.: Granular
Cell Myoblastoma, Surgery 5 3 : 226, 1963. 8 Elias, E. G., Valenzuela, L., and Pickerin, J. W.: Granular Cell Myoblastoma, J. Surg. Oncol. 2: 33, 1970. 9 Fisher, E. R., and Wechsler, H.: Granular Cell Myoblastoma—A Misnomer, Cancer 15: 936, 1962. 10 Fust, J. A., and Custer, R. P.: On Neurogenesis of the So-Called Granular Cell Myoblastoma, Am. J. Clin. Pathol. 19: 522, 1949. 11 Horn, R. C , Jr., and Stout, A. P.: Granular Cell Myoblastoma, Surg. Gynecol. Obstet. 76: 315, 1943. 12 Paskin, D. L., Hull, J. D., and Cookson, P. J.: Granular Cell Myoblastoma: A Comprehensive Review of 15-Year Experience, Ann. Surg. 175: 501, 1972. 13 Shear, M.: The Histogenesis of the So-Called Granular Cell Myoblastoma, J. Pathol. 80: 225, 1960. 14 Walter, W. L.: Granular Cell Myoblastoma of the Larynx With Presentation of 2 Cases, Ann. Otol. Rhinol. Laryngol. 69: 328, 1960. 15 Ward, P. H., and Oshiro, H.: Laryngeal Granular-Cell Myoblastoma, Arch. Otolaryngol. (Chicago) 76: 239, 1962. 16 Weber, C. D.: Cited by Blashi, S. F . : Myoblastoma of the Larynx, Ann. Otol. Rhinol. Laryngol. 69: 115, 1960.