Granular Cell Myoblastoma Arising From the Thoracic Sympathetic Nerve Chain By Phillip M. Rosenbloom,
George Ii. Barrows,
Donald
R. Kmetz, and Timothy
G. Canty
G
RANULAR cell myoblastoma has been of interest both to the clinician and the pathologist for many years. This uncommon tumor presents the usual diagnostic problem of a mass, but the actual diagnosis of granular cell myoblastoma is rarely made prior to biopsy. The origin of the tumor from either myoblastic or neural tissue remains controversial. Light and electron microscopic studies of a granular cell myoblastoma uniquely located in the posterior mediastinum and associated with the sympathetic chain support the neural origin of this tumor. CASE
REPORT
An 1 I-yr-old boy was admitted for evaluation of refractory asthma. Physical examination revealed a well-developed, well-nourished, young adolescent in mild respiratory distress with diffuse bilateral expiratory wheezing. The admission hemogram, urinalysis, and routine blood chemistries showed no abnormalities, and a chest roentgenogram revealed clear lung fields and a posterior mediastinal mass on the left (Fig. IA). Liver chemistries, liver-spleen scan, bone scan, and bone marrow were normal. Previous chest roetgenograms taken 6 mo and 2 wk prior to admission were retrieved and showed the same mass. His asthma was readily controlled, and, at left thoracotomy, a solid mass 5 cm in diameter and fixed to the left sympathetic chain was removed en bloc, freeing it from the underlying ribs. The patient was discharged on his seventh postoperative day with an expected partial left Horner’s syndrome. Follow-up to date has shown no further problems. PATHOLOGY
The excised tumor consisted of a lobulated mass with a small amount of attached connective tissue. The specimen was not encapsulated but the margins were distinct, and a 3-cm segment of sympathetic chain containing a ganglion was contiguous with the tumor. On cut surface, the tissue was homogenous tan (Fig. 1B). Light microscopy revealed a sheet-like distribution of uniform, large round cells with eosinophilic granules typical of granular cell tumors (Fig. 2A).‘-3 Neural tissue was intimately connected to the tumor, but no striated or smooth muscle was present (Fig. 2B). Electron microscopy demonstrated ultrastructure similar to that of previously described granular cell tumors. 4-6The tumor cells contained ovoid structures and intracytoplasmic filaments (Fig. 2C). The ovoid structures consisted of both osmophilic concentric lamellae and homogenous material. In addition, unmyelinated axons and aggregates of tubular filaments were present (Fig. 2D). From the Departments of Surgery and Pathology, University of Louisville School of Medicine, Louisville, Ky. Presented before the 6th Annual Meeting of the American Pediatric Surgical Association, San Juan, Puerto Rico, April 10-12. 1975. Address for reprint requests: Timothy G. Canty. M.D., Surgeon-in-Chief, Children’s Division, Norton-Children’s Hospital, Louisville, Ky. 40202. o 1975 by Grune & Stratton, Inc. Journal of Pediatric Surgery, Vol. 10, No. 5 (October), 1975
819
ROSENBLOOM
Fig. 1.
(A) Adminission
of the aorta. (6) Operative
chest roentgenogmm specimen showing
shows
left mediastinal
ET AL
mass above the density
homogenour cut surface of tumor.
DISCUSSION
Although granular cell tumors occur most commonly in the third to fourth decades of life, up to 10% of these lesions occur in children under 10 yr with the majority occurring under 1 yr. Another 5% occur in the second decade.‘*2 Granular cell tumors are not restricted to any one area or tissue of the body and can occur singly or as multiple tumors. 7*8The most frequent location is the head and neck, especially the tongue. Skin lesions are next in frequency.‘-3*8*9 These tumors have also been described in the tracheobronchial tree,3*6vsgastrotree,8 intestinal tract, iL%i%11biliary urinary bladder,’ anogenital region,‘9~8*‘2 and the pituitary. 2~‘3The unique presentation of a granular cell myoblastoma occurring in the posterior mediastinum and associated with the sympathetic chain has not previously been described. The origin and description of these lesions have been the basis of discussion among pathologists for over a century.’ In 1926, Abrikossoff classified them as a specific tumor entity and hypothesized that these granular cell tumors were derived from myoblasts. *‘A~However the frequent observations of these lesions in nonmuscle-bearing areas as well as’histochemical studies of these tumors has led several investigators to question whether granular cell myoblastomas did indeed have a myoblastic origin. Others suggested that they were storage tumors of mesenchymal origin, containing glycogeni5 mucins,16 or protein,‘7*‘8 The derivation of granular cell tumors from neural tissue was first proposed by of these tumors to neurofibromata and the Feyrter in 1935. I9 The resemblance
GRANULAR
CELL MYOMASTOMA
Fig. 2.
(A) Photomicrograph (high-power) of tumor showing granular nature of tumor cells. (high-power) of tumor showing contiguity with nerve tissue in lower portion of section. (C) Electron microgmph of tumor showing osmophillic (OS)and concentric lamellated structures(cl) in right upper corner. (D) Electron micrograph showing intermingling of unmyelinated axons (ax), tubular filaments (tf), and granule containing cells (gr).
(B) Photomicrogmph
occurrence in a peripheral nerve gave further support to neural origin.‘*” Electron microscopic studies of cytoplasmic contents further suggested that granular cell tumors were actually derived from Schwann cells.5*6 The light and electron microscopic observations of the tumor presented here support the theory for a derivation from Schwann cells.
822
ROSENBLOOM
ET AL.
REFERENCES 1. Murphy G, Dockerty M, Borders A: Myoblastoma. Am J Pathol25: 1157,1949 2. Strong E, McDivitt R, Brasfield R: Granular cell myoblastoma. Cancer 25:415, 1970 3. Vance S, Hudson R: Granular cell myoblastoma. Am J Clin Pathol 52:208, 1969 4. Carstens P: Ultrastructure of granular cell myoblastoma. Acta Path01 Microbial Stand 78: 685, 1970 5. Fisher E, Weshler H: Granular cell myoblastoma-A misnomer. Cancer 5:936, 1962 6. Garancis J, Komorowski R, Kuzma J: Granular cell myoblastoma. Cancer 25:542, 1970 7. Moscovic E, Azar H: Multiple granular cell tumors (“myoblastomas”). Cancer 20:2032, 1967 8. Paskin D, Hull J, Cookson P: Granular cell myoblastoma: A comprehensive review of 15 years experience. Ann Surg 175:501, 1972 9. Fust J, Custer P: On the neurogenesis of so-called granular cell myoblastoma. Am J Clin Pathol 19:522, 1949 10. Aston S, Tompkins R: Granular cell myoblastoma of stomach. Ann Surg 177:228, 1973 11. Bangle R: An early granular cell myoblastoma confined within a small peripheral myelinated nerve. Cancer 6:790, 1953
12. Gifford R, Birch H: Granular cell myoblastoma of multicentric origin involving the vulva: A case report. Am J Obstet Gynecol 117: 184, 1973 13. Satyamurti S, Huntington H: Granular cell myoblastoma of the pituitary. J Neurosurg 37:483, 1972 14. Abrikossoff A: Uber Myome, ausgehend von der quergestreiften willkiirlichen Muskulatur. Virchows Arch (Pathol Anat) 260:215, 1926 15. Leroux R, Delarue J: Sur trois cas de tumeurs a cellules granuleuses de la cavite buctale. Bull Assoc Franc Etude Cancer 28:427, 1939 16. Holle G: Uber die Deutung der songenannten Myoblastenmyome als Speicherzellgeschwtilste auf Grund einer besonderen Wrberischen Reaktion. Zentralbl Allg Pathol 76: 244, 1941 17. Lauche A: Sind die Sog. “Myoblastenmyome” Speicherzellgeschwtilste? Virchows Arch (Path01 Anat) 312:335, 1944 18. Ringertz N: Uber das Sog. Myoblastenmyome: Mit Beschreibun 7 neuer Falle. Acta Pathol Microbial Stand 19:112, 1942 19. Feyrter F: Uber eine eigenartige geschwulstform des nervengewebes im menschlichen verdauungsschlauch. Virchows Arch (Pathol Anat) 295:480, 1935
George Ii. Barrows,
Donald
R. Kmetz, and Timothy
G. Canty
G
RANULAR cell myoblastoma has been of interest both to the clinician and the pathologist for many years. This uncommon tumor presents the usual diagnostic problem of a mass, but the actual diagnosis of granular cell myoblastoma is rarely made prior to biopsy. The origin of the tumor from either myoblastic or neural tissue remains controversial. Light and electron microscopic studies of a granular cell myoblastoma uniquely located in the posterior mediastinum and associated with the sympathetic chain support the neural origin of this tumor. CASE
REPORT
An 1 I-yr-old boy was admitted for evaluation of refractory asthma. Physical examination revealed a well-developed, well-nourished, young adolescent in mild respiratory distress with diffuse bilateral expiratory wheezing. The admission hemogram, urinalysis, and routine blood chemistries showed no abnormalities, and a chest roentgenogram revealed clear lung fields and a posterior mediastinal mass on the left (Fig. IA). Liver chemistries, liver-spleen scan, bone scan, and bone marrow were normal. Previous chest roetgenograms taken 6 mo and 2 wk prior to admission were retrieved and showed the same mass. His asthma was readily controlled, and, at left thoracotomy, a solid mass 5 cm in diameter and fixed to the left sympathetic chain was removed en bloc, freeing it from the underlying ribs. The patient was discharged on his seventh postoperative day with an expected partial left Horner’s syndrome. Follow-up to date has shown no further problems. PATHOLOGY
The excised tumor consisted of a lobulated mass with a small amount of attached connective tissue. The specimen was not encapsulated but the margins were distinct, and a 3-cm segment of sympathetic chain containing a ganglion was contiguous with the tumor. On cut surface, the tissue was homogenous tan (Fig. 1B). Light microscopy revealed a sheet-like distribution of uniform, large round cells with eosinophilic granules typical of granular cell tumors (Fig. 2A).‘-3 Neural tissue was intimately connected to the tumor, but no striated or smooth muscle was present (Fig. 2B). Electron microscopy demonstrated ultrastructure similar to that of previously described granular cell tumors. 4-6The tumor cells contained ovoid structures and intracytoplasmic filaments (Fig. 2C). The ovoid structures consisted of both osmophilic concentric lamellae and homogenous material. In addition, unmyelinated axons and aggregates of tubular filaments were present (Fig. 2D). From the Departments of Surgery and Pathology, University of Louisville School of Medicine, Louisville, Ky. Presented before the 6th Annual Meeting of the American Pediatric Surgical Association, San Juan, Puerto Rico, April 10-12. 1975. Address for reprint requests: Timothy G. Canty. M.D., Surgeon-in-Chief, Children’s Division, Norton-Children’s Hospital, Louisville, Ky. 40202. o 1975 by Grune & Stratton, Inc. Journal of Pediatric Surgery, Vol. 10, No. 5 (October), 1975
819
ROSENBLOOM
Fig. 1.
(A) Adminission
of the aorta. (6) Operative
chest roentgenogmm specimen showing
shows
left mediastinal
ET AL
mass above the density
homogenour cut surface of tumor.
DISCUSSION
Although granular cell tumors occur most commonly in the third to fourth decades of life, up to 10% of these lesions occur in children under 10 yr with the majority occurring under 1 yr. Another 5% occur in the second decade.‘*2 Granular cell tumors are not restricted to any one area or tissue of the body and can occur singly or as multiple tumors. 7*8The most frequent location is the head and neck, especially the tongue. Skin lesions are next in frequency.‘-3*8*9 These tumors have also been described in the tracheobronchial tree,3*6vsgastrotree,8 intestinal tract, iL%i%11biliary urinary bladder,’ anogenital region,‘9~8*‘2 and the pituitary. 2~‘3The unique presentation of a granular cell myoblastoma occurring in the posterior mediastinum and associated with the sympathetic chain has not previously been described. The origin and description of these lesions have been the basis of discussion among pathologists for over a century.’ In 1926, Abrikossoff classified them as a specific tumor entity and hypothesized that these granular cell tumors were derived from myoblasts. *‘A~However the frequent observations of these lesions in nonmuscle-bearing areas as well as’histochemical studies of these tumors has led several investigators to question whether granular cell myoblastomas did indeed have a myoblastic origin. Others suggested that they were storage tumors of mesenchymal origin, containing glycogeni5 mucins,16 or protein,‘7*‘8 The derivation of granular cell tumors from neural tissue was first proposed by of these tumors to neurofibromata and the Feyrter in 1935. I9 The resemblance
GRANULAR
CELL MYOMASTOMA
Fig. 2.
(A) Photomicrograph (high-power) of tumor showing granular nature of tumor cells. (high-power) of tumor showing contiguity with nerve tissue in lower portion of section. (C) Electron microgmph of tumor showing osmophillic (OS)and concentric lamellated structures(cl) in right upper corner. (D) Electron micrograph showing intermingling of unmyelinated axons (ax), tubular filaments (tf), and granule containing cells (gr).
(B) Photomicrogmph
occurrence in a peripheral nerve gave further support to neural origin.‘*” Electron microscopic studies of cytoplasmic contents further suggested that granular cell tumors were actually derived from Schwann cells.5*6 The light and electron microscopic observations of the tumor presented here support the theory for a derivation from Schwann cells.
822
ROSENBLOOM
ET AL.
REFERENCES 1. Murphy G, Dockerty M, Borders A: Myoblastoma. Am J Pathol25: 1157,1949 2. Strong E, McDivitt R, Brasfield R: Granular cell myoblastoma. Cancer 25:415, 1970 3. Vance S, Hudson R: Granular cell myoblastoma. Am J Clin Pathol 52:208, 1969 4. Carstens P: Ultrastructure of granular cell myoblastoma. Acta Path01 Microbial Stand 78: 685, 1970 5. Fisher E, Weshler H: Granular cell myoblastoma-A misnomer. Cancer 5:936, 1962 6. Garancis J, Komorowski R, Kuzma J: Granular cell myoblastoma. Cancer 25:542, 1970 7. Moscovic E, Azar H: Multiple granular cell tumors (“myoblastomas”). Cancer 20:2032, 1967 8. Paskin D, Hull J, Cookson P: Granular cell myoblastoma: A comprehensive review of 15 years experience. Ann Surg 175:501, 1972 9. Fust J, Custer P: On the neurogenesis of so-called granular cell myoblastoma. Am J Clin Pathol 19:522, 1949 10. Aston S, Tompkins R: Granular cell myoblastoma of stomach. Ann Surg 177:228, 1973 11. Bangle R: An early granular cell myoblastoma confined within a small peripheral myelinated nerve. Cancer 6:790, 1953
12. Gifford R, Birch H: Granular cell myoblastoma of multicentric origin involving the vulva: A case report. Am J Obstet Gynecol 117: 184, 1973 13. Satyamurti S, Huntington H: Granular cell myoblastoma of the pituitary. J Neurosurg 37:483, 1972 14. Abrikossoff A: Uber Myome, ausgehend von der quergestreiften willkiirlichen Muskulatur. Virchows Arch (Pathol Anat) 260:215, 1926 15. Leroux R, Delarue J: Sur trois cas de tumeurs a cellules granuleuses de la cavite buctale. Bull Assoc Franc Etude Cancer 28:427, 1939 16. Holle G: Uber die Deutung der songenannten Myoblastenmyome als Speicherzellgeschwtilste auf Grund einer besonderen Wrberischen Reaktion. Zentralbl Allg Pathol 76: 244, 1941 17. Lauche A: Sind die Sog. “Myoblastenmyome” Speicherzellgeschwtilste? Virchows Arch (Path01 Anat) 312:335, 1944 18. Ringertz N: Uber das Sog. Myoblastenmyome: Mit Beschreibun 7 neuer Falle. Acta Pathol Microbial Stand 19:112, 1942 19. Feyrter F: Uber eine eigenartige geschwulstform des nervengewebes im menschlichen verdauungsschlauch. Virchows Arch (Pathol Anat) 295:480, 1935
