856
0 1990 The Japanese Society of
Pathology
lntraosseous Glomus Tumor in the Sacrum A Case Report
Yasushi Kobayashi', Takanori Kawaguchi', Kazuhiko lmoto2, and Tomio Yamamoto2
Primary intraosseous glomus tumor is rare and may occur in the medullary cavity of bone. A 22-year-old woman with a one-year history of spontaneous and persistent sacral pain was found to have an intraosseous glomus tumor i n this unusual location. Curettage was followed by complete relief from pain. This case of glomus tumor originating in the sacrum is thought to be the first reported in the world literature. Acta Pathol Jpn 40: 856-859, 1990. Key words : Glomus tumor, Intraosseous, Sacrum
INTRODUCTION Glomus tumors arise from the glomus apparatus, which represents an arterio-venous anastomosis. The nail beds and volar surfaces of the fingers are rich in glomus bodies, and tumors arising from them are common. Glomus tumors are benign vascular tumors composed of round to oval uniform cells associated with small vessels or capillaries. The cell of origin may be the neuromyoarterial glomus body (1). These tumors are usually soft tissue lesions, commonly found adjacent to the distal phalanx, and occasionally they erodes bone. However, they can also occur at sites where glomus bodies are normally sparse, or even absent. Glomus tumors rarely arise primarily within bone (2-10). This report describes a patient with an intraosseous glomus tumor in the sacrum, a location hitherto unreported.
CASEREPORT A 22-year-old woman had a one-year history of persistent sacral pain on the right side of the median line. The lesion was not tender to touch or sensitive to changes in temperature. The pain was constant and localized to a small area without any antecedent trauma. There were no abnormal laboratory findings. The pain increased progressively and prevented the patient from sleeping. Roentgenographic examination of the sacrum revealed a well-defined osteolytic lesion in the right lower portion of the sacrum (Fig. 1). The lesion was radiolucent and measured 2 cm in diameter. CT examination of the pelvis also showed a round osteolytic lesion in the corresponding area. The bone cortex was thin, and partially absent in the anterior aspect of the sacrum (Fig. 2). Bone scintigraphy, extradural roentgenography and MRI revealed no abnormal findings. The patient underwent surgery which revealed a small round hole measuring 0.3cm or less in diameter in the center of the thin bone cortex in front of the lesion. However, none of the intraosseous contents including the lesion flowed out from the bone. There was a mass of reddish brown material in the lesion, which was soft and friable. Curettage of the lesion was performed as far as the surrounding cancellous bone. The postoperative course was unremarkable, and the patient has since been entirely without pain or other symptoms.
HISTOPATHOLOGIC OBSERVATION Received April 5, 1990. Accepted for publication September 11, 1990. 'Department of Pathology, 2Department of Orthopedics, Osaka Kousei-Nenkin Hospital, Osaka. Mailing address : Yasushi Kobayashi (/J\$$ g ) , Department of Pathology, Osaka Kousei-Nenkin Hospital, 4-2-78 Fukushirna, Fukushima-ku, Osaka 553, Japan.
Microscopically, the tumor consisted of sheets of uniform round to polygonal epithelioid cells with distinct cell boundaries around blood vessels lined by normal endothelial cells. The nuclei were round or oval, with finely dispersed chromatin, and the nucleoli were somewhat prominent. Mitoses were extremely rare. Some
85 7
Acta Pathologica Japonica 40 (11) : 1990
Figure 1. Tomography of the sacrum showing well defined osteolytic lesion (arrow) in the right lower part.
endot helium-lined vascular c ha nnels were found within the tumor (Figs. 3, 4). There was a fine reticulin framework, and in many areas the reticulin fibers surrounded individual cells (Fig. 5). Nerve fibers and smooth muscle of blood vessels were also found in the tumor (Fig. 6). lmmunohistochemical study by the ABC method showed intense staining for S-100 (DAKO) (Fig. 7) and neuronspecific enolase (DAKO) in the former element, and positive staining for desmin (DAKO) in the latter. Myosin (Biomeda) was also positively stained on the walls of blood vessels and in the cytoplasm of the tumor cells (Fig. 8).
Figure 2. CT scan showing osteolytic lesion and defect of part of the sacral cortex.
a case of intraosseous glomus tumor by Lattes and Bull (4), the possible histogenesis of this tumor was discus sed. Jaffe reported that since glomus bodies normally exist in the medullary cavity of bone, a primary glomus tumor of the bone could be expected to occur (11). Moreover, according to Ushigome and lshikawa (12), pericytes appear to have a close relationship with vascular smooth muscle cells or glomus cells, and might have the potential to develop and transform into these cells. In other words, pericytes appear to be more immature than well differentiated smooth muscle cells or glomus cells. The fundamental structures of hemangioperi-
DISCUSSION The glomus body is a specialized form of arterial venous anastomosis composed of neural, smooth muscle and vascular elements. It is usually located in the dermal-subdermal junction in the subungual region of the fingers and may be involved in distal temperature regulation. Glomus tumor is a distinctive type of vascular neoplasm whose cell type is a modified smooth muscle cell closely resembling the glomus body from which it is derived (1). The earliest report of a glomus tumor within bone was by de la Torre et a/. (3), who in 1939 reported such a tumor in the distal phalanx of the thumb. In a report of
Table 1. Previously
Reported Cases lntraosseous Glomus Tumor
Phalanges Metacarpal bones Ossa digitorum pedis Basis cranii Temporal bone Ulna coccyx Sacrurna Total a
Present case.
14
1 2 2 3
1 2 1
26
of
53.8% 3.8 7.7 7.7 11.5 3.8
7.7 3.8 99.8
85%
Glomus Tumor in Sacrum (Kobayashi et a/.)
Acta Pathologica Japonica 40 (11) : 1990 cytoma, vascular leiomyoma and glomus tumor, i.e. proliferation of tumor cells intimately related t o the endothelium, are all similar. Therefore, f r o m occasional reports of glomus tumors occurring in unusual locations (13) it seems apparent that pericytes may develop into glomus tumors in bone. Although these lesions may be locally erosive, they are solitary. Metastatic spread and malignant degeneration are unknown. Table 1 gives a list of cases of intraosseous glomus tumor reported hitherto. The phalanx is the most frequent site of occurrence. The present case represents the first reported instance of an intraosseous glomus tumor of the sacrum. Acknowledgements : The authors thank Prof. M. Enjoji, Faculty of Medicine, Kyushu University, and Prof. S. Ushigome, Tokyo Jikei University School of Medicine, for helpful suggestions.
REFERENCES 1. Enzinger FM and Weis SW. Soft tissue tumors. CV Mosby, St. Louis, 1985 : 450-462. 2. Dorfman HD, Steiner GC, and Jaffe HL. Vascular tumors of bone. Hum Pathol 2 : 349-375, 1971. 3. de la Torre LI, G6mez Camajo M, and Palacios G.
4.
9.
10. 11. 12. 13.
859
Consideraciones clinicas, inat6micas, radiol6gicas y quirfirgicas del glomus tumoral de masson. Cir Ortoped Traumatol Habana 7 : 11-17, 1939. Lattes R and Bull DC. A case of glomus tumor with primary involvement of bone. Ann Surg 127: 187191, 1943. Lehman W and Kraissl C. Glomus tumor within bone. Surgery 2 5 : 118-121, 1949. MacKenzie DH. lntraosseous glomus tumors : Report of two cases. J Bone Joint Surg 44: 118-121, 1949. Sugiura 1. lntraosseous glomus tumor. A case report. J Bone Joint Surg [Br] 5 8 : 245-247, 1976. Serra JM, Muirragui A, and Tadjalli H. Glomus tumor of the metacarpophalangeal joint: A case report. J Hand Surg [Am] 10: 142-143, 1985. Rozmaryn LM, Sadler AH, and Dorfman HD. Intraosseous glomus tumor in the ulna. A case report. Clin Orthop 220: 126-129, 1987. Sunderraj S, Al-Khalifa AA, Pal AK, Pim HP, and Sabri SH. Primary intraosseous glomus tumor. Histopathology 14: 532-536, 1989. Jaffe HL. Glomus tumor of bone. In Tumors and tumorous conditions of bones and joints. Lea & Febiger, Philadelphia, 1958 : 254-255. Ushigome S and lshikawa E. Pericyte-tumors originating from pericytes and related cells. Recent Adv RES Res 1 5 : 109-128, 1975. Tanaka S, Takeuchi S, and Fukuda M. Glomus tumor of the stomach. Report of a case. Gan No Rinsho 2 0 : 708-713, 1974 (in Japanese).
Figure 3. Glomus tumor cells show proliferation adjacent to bone trabecula. HE. Figure4. The tumor cells are round to polygonal with distinct boundaries. The nuclei are round or oval, with somewhat
prominent nucleoli. Figure 5. Fine reticulin framework evident within the tumor. Silver impregnation. Figure 6. Nerve fibers and blood vessels of various sizes within the tumor. HE. Figure 7. Intense staining for S-100 in nerve fibers. ABC method. Figure8. Myosin is weakly stained on the walls of blood vessels and in the cytoplasm of the tumor cells. ABC method.
0 1990 The Japanese Society of
Pathology
lntraosseous Glomus Tumor in the Sacrum A Case Report
Yasushi Kobayashi', Takanori Kawaguchi', Kazuhiko lmoto2, and Tomio Yamamoto2
Primary intraosseous glomus tumor is rare and may occur in the medullary cavity of bone. A 22-year-old woman with a one-year history of spontaneous and persistent sacral pain was found to have an intraosseous glomus tumor i n this unusual location. Curettage was followed by complete relief from pain. This case of glomus tumor originating in the sacrum is thought to be the first reported in the world literature. Acta Pathol Jpn 40: 856-859, 1990. Key words : Glomus tumor, Intraosseous, Sacrum
INTRODUCTION Glomus tumors arise from the glomus apparatus, which represents an arterio-venous anastomosis. The nail beds and volar surfaces of the fingers are rich in glomus bodies, and tumors arising from them are common. Glomus tumors are benign vascular tumors composed of round to oval uniform cells associated with small vessels or capillaries. The cell of origin may be the neuromyoarterial glomus body (1). These tumors are usually soft tissue lesions, commonly found adjacent to the distal phalanx, and occasionally they erodes bone. However, they can also occur at sites where glomus bodies are normally sparse, or even absent. Glomus tumors rarely arise primarily within bone (2-10). This report describes a patient with an intraosseous glomus tumor in the sacrum, a location hitherto unreported.
CASEREPORT A 22-year-old woman had a one-year history of persistent sacral pain on the right side of the median line. The lesion was not tender to touch or sensitive to changes in temperature. The pain was constant and localized to a small area without any antecedent trauma. There were no abnormal laboratory findings. The pain increased progressively and prevented the patient from sleeping. Roentgenographic examination of the sacrum revealed a well-defined osteolytic lesion in the right lower portion of the sacrum (Fig. 1). The lesion was radiolucent and measured 2 cm in diameter. CT examination of the pelvis also showed a round osteolytic lesion in the corresponding area. The bone cortex was thin, and partially absent in the anterior aspect of the sacrum (Fig. 2). Bone scintigraphy, extradural roentgenography and MRI revealed no abnormal findings. The patient underwent surgery which revealed a small round hole measuring 0.3cm or less in diameter in the center of the thin bone cortex in front of the lesion. However, none of the intraosseous contents including the lesion flowed out from the bone. There was a mass of reddish brown material in the lesion, which was soft and friable. Curettage of the lesion was performed as far as the surrounding cancellous bone. The postoperative course was unremarkable, and the patient has since been entirely without pain or other symptoms.
HISTOPATHOLOGIC OBSERVATION Received April 5, 1990. Accepted for publication September 11, 1990. 'Department of Pathology, 2Department of Orthopedics, Osaka Kousei-Nenkin Hospital, Osaka. Mailing address : Yasushi Kobayashi (/J\$$ g ) , Department of Pathology, Osaka Kousei-Nenkin Hospital, 4-2-78 Fukushirna, Fukushima-ku, Osaka 553, Japan.
Microscopically, the tumor consisted of sheets of uniform round to polygonal epithelioid cells with distinct cell boundaries around blood vessels lined by normal endothelial cells. The nuclei were round or oval, with finely dispersed chromatin, and the nucleoli were somewhat prominent. Mitoses were extremely rare. Some
85 7
Acta Pathologica Japonica 40 (11) : 1990
Figure 1. Tomography of the sacrum showing well defined osteolytic lesion (arrow) in the right lower part.
endot helium-lined vascular c ha nnels were found within the tumor (Figs. 3, 4). There was a fine reticulin framework, and in many areas the reticulin fibers surrounded individual cells (Fig. 5). Nerve fibers and smooth muscle of blood vessels were also found in the tumor (Fig. 6). lmmunohistochemical study by the ABC method showed intense staining for S-100 (DAKO) (Fig. 7) and neuronspecific enolase (DAKO) in the former element, and positive staining for desmin (DAKO) in the latter. Myosin (Biomeda) was also positively stained on the walls of blood vessels and in the cytoplasm of the tumor cells (Fig. 8).
Figure 2. CT scan showing osteolytic lesion and defect of part of the sacral cortex.
a case of intraosseous glomus tumor by Lattes and Bull (4), the possible histogenesis of this tumor was discus sed. Jaffe reported that since glomus bodies normally exist in the medullary cavity of bone, a primary glomus tumor of the bone could be expected to occur (11). Moreover, according to Ushigome and lshikawa (12), pericytes appear to have a close relationship with vascular smooth muscle cells or glomus cells, and might have the potential to develop and transform into these cells. In other words, pericytes appear to be more immature than well differentiated smooth muscle cells or glomus cells. The fundamental structures of hemangioperi-
DISCUSSION The glomus body is a specialized form of arterial venous anastomosis composed of neural, smooth muscle and vascular elements. It is usually located in the dermal-subdermal junction in the subungual region of the fingers and may be involved in distal temperature regulation. Glomus tumor is a distinctive type of vascular neoplasm whose cell type is a modified smooth muscle cell closely resembling the glomus body from which it is derived (1). The earliest report of a glomus tumor within bone was by de la Torre et a/. (3), who in 1939 reported such a tumor in the distal phalanx of the thumb. In a report of
Table 1. Previously
Reported Cases lntraosseous Glomus Tumor
Phalanges Metacarpal bones Ossa digitorum pedis Basis cranii Temporal bone Ulna coccyx Sacrurna Total a
Present case.
14
1 2 2 3
1 2 1
26
of
53.8% 3.8 7.7 7.7 11.5 3.8
7.7 3.8 99.8
85%
Glomus Tumor in Sacrum (Kobayashi et a/.)
Acta Pathologica Japonica 40 (11) : 1990 cytoma, vascular leiomyoma and glomus tumor, i.e. proliferation of tumor cells intimately related t o the endothelium, are all similar. Therefore, f r o m occasional reports of glomus tumors occurring in unusual locations (13) it seems apparent that pericytes may develop into glomus tumors in bone. Although these lesions may be locally erosive, they are solitary. Metastatic spread and malignant degeneration are unknown. Table 1 gives a list of cases of intraosseous glomus tumor reported hitherto. The phalanx is the most frequent site of occurrence. The present case represents the first reported instance of an intraosseous glomus tumor of the sacrum. Acknowledgements : The authors thank Prof. M. Enjoji, Faculty of Medicine, Kyushu University, and Prof. S. Ushigome, Tokyo Jikei University School of Medicine, for helpful suggestions.
REFERENCES 1. Enzinger FM and Weis SW. Soft tissue tumors. CV Mosby, St. Louis, 1985 : 450-462. 2. Dorfman HD, Steiner GC, and Jaffe HL. Vascular tumors of bone. Hum Pathol 2 : 349-375, 1971. 3. de la Torre LI, G6mez Camajo M, and Palacios G.
4.
9.
10. 11. 12. 13.
859
Consideraciones clinicas, inat6micas, radiol6gicas y quirfirgicas del glomus tumoral de masson. Cir Ortoped Traumatol Habana 7 : 11-17, 1939. Lattes R and Bull DC. A case of glomus tumor with primary involvement of bone. Ann Surg 127: 187191, 1943. Lehman W and Kraissl C. Glomus tumor within bone. Surgery 2 5 : 118-121, 1949. MacKenzie DH. lntraosseous glomus tumors : Report of two cases. J Bone Joint Surg 44: 118-121, 1949. Sugiura 1. lntraosseous glomus tumor. A case report. J Bone Joint Surg [Br] 5 8 : 245-247, 1976. Serra JM, Muirragui A, and Tadjalli H. Glomus tumor of the metacarpophalangeal joint: A case report. J Hand Surg [Am] 10: 142-143, 1985. Rozmaryn LM, Sadler AH, and Dorfman HD. Intraosseous glomus tumor in the ulna. A case report. Clin Orthop 220: 126-129, 1987. Sunderraj S, Al-Khalifa AA, Pal AK, Pim HP, and Sabri SH. Primary intraosseous glomus tumor. Histopathology 14: 532-536, 1989. Jaffe HL. Glomus tumor of bone. In Tumors and tumorous conditions of bones and joints. Lea & Febiger, Philadelphia, 1958 : 254-255. Ushigome S and lshikawa E. Pericyte-tumors originating from pericytes and related cells. Recent Adv RES Res 1 5 : 109-128, 1975. Tanaka S, Takeuchi S, and Fukuda M. Glomus tumor of the stomach. Report of a case. Gan No Rinsho 2 0 : 708-713, 1974 (in Japanese).
Figure 3. Glomus tumor cells show proliferation adjacent to bone trabecula. HE. Figure4. The tumor cells are round to polygonal with distinct boundaries. The nuclei are round or oval, with somewhat
prominent nucleoli. Figure 5. Fine reticulin framework evident within the tumor. Silver impregnation. Figure 6. Nerve fibers and blood vessels of various sizes within the tumor. HE. Figure 7. Intense staining for S-100 in nerve fibers. ABC method. Figure8. Myosin is weakly stained on the walls of blood vessels and in the cytoplasm of the tumor cells. ABC method.
