- Email: [email protected]
Solitary lymphangioma of the femur. A case report
J Orthop Sci (2002) 7:501–504
Solitary lymphangioma of the femur. A case report Akio Sakamoto, Shuichi Matsuda, Kazuhiro Tanaka, Tomoyuki Shukuri, Katsumi Harimaya, and Yukihide Iwamoto Department of Orthopaedic Surgery, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan
Abstract Lymphangioma of the bone is a benign lesion, characterized by newly formed lymph vessels. It usually occurs in a diffuse manner or as multiple lesions. Solitary lymphangioma of the bone is an extremely rare condition. We herein describe a case of solitary lymphangioma of the femur in a 55year-old woman who presented with knee pain. Radiographs showed an eccentric osteolytic lesion in the distal femur. This is the first reported case of solitary lymphangioma of the bone occurring in the femur. Key words Solitary lymphangioma · Femur · CT · MRI
Introduction Lymphangioma of the bone is a benign lesion that is generally considered to be a hamartomatous malformation rather than a true neoplasm.5 These lesions occur diffusely or as multiple lesions. Solitary lymphangioma of the bone is an extremely rare condition that was first described in 1947.2 Fewer than 10 cases have previously been reported, all of which occurred in the tibia, humerus, mandible, or vertebra.3,6,9,10 We present a case of solitary lymphangioma of the bone that arose in the femur, a site that has not previously been reported.
studies were unremarkable. Radiographs showed an eccentric osteolytic lesion with moderate osseous expansion located within the medial condyle of the femur (Fig. 1). Computed tomography (CT) revealed a multilocular lesion with coarse bone trabeculae and thinning of the cortex (Fig. 2). Magnetic resonance imaging (MRI) showed a homogenous lesion with low intensity to isointensity on T1-weighted images and high intensity on T2-weighted images (Figs. 3 and 4). The signal intensity of the lesion on the T1-weighted images was greater than that of skeletal muscle, approaching that of fat. No enhancement of the lesion was seen after gadolinium injection. No soft-tissue mass was evident. We performed an open biopsy of the lesion. The cyst lesion was filled with light-brown colored fluid. Histologically, a specimen from the cystic wall showed dilated, thin-walled cystic spaces, which were lined by a single layer of endothelial cells that bordered the bone trabeculae (Figs. 5 and 6). These cystic spaces contained few red blood cells. The features of the specimen were consistent with those of lymphangioma of the bone. The lesion was treated by curettage and autogenous bone grafting. After surgery for the symptomatic lesions in the femur, we carried out bone scintigraphy (technetium) to rule out multiple lesions of lymphangiomatosis. No other lesions were demonstrated (Fig. 7).
Case report A 55-year-old woman was referred to our institute because she suffered from left knee pain while walking. At palpation, the patient had tenderness. The knee was not swollen, and there was no sensation of heat or redness over the knee. Range of motion in the knee joint was full and no instability was seen. Muscle weakness was not observed in the lower extremity. Laboratory Offprint requests to: A. Sakamoto Received: December 7, 2001 / Accepted: February 18, 2002
Discussion The pathogenesis of lymphangioma is not well understood. Insufficiency of lymph flow, leading to back flow into the bone, has been presumed to be the cause.7 Congenital malformation, inflammation, neoplasm, and lymphatic obstruction have also been reported as possible causes of the lesion.4,11 When making a diagnosis of solitary lymphangioma of the bone, the possibility of lymphangiomatosis should be ruled out, because
502
A. Sakamoto et al.: Solitary lymphangioma of femur
Fig. 1 Anterior and lateral views of the left femur show a multiloculated expansive osteolytic lesion within the medial condyle
Fig. 3 Magnetic resonance imaging (MRI) shows a solitary homogenous lesion with low- to iso-signal intensity on T1weighted image (TR ⫽ 500, TE ⫽ 15)
Fig. 2 Computed tomography (CT) reveals coarse bone trabeculae within the cystic lesion with a thinning of the cortex
lymphangioma tends to occur diffusely or as multiple lesions. In the present case, bone scintigraphy (technetium) was found to be useful for excluding the presence of other lesions, as reported in previous studies.11 The histological features of lymphangioma and hemangioma are sometimes similar. For this reason, lymphangioma has also been referred to as “angiomatosis” of the bone by several authors.7,12 It has been reported that the fluid within the cyst in lymphangioma, which is straw colored, is different from that in hemangioma, which is in fact blood.1 In the present case, straw (light-brown) colored fluid was seen within the
Fig. 4 MRI shows a solitary homogenous lesion with high signal intensity on T2-weighted image (TR ⫽ 3000, TE ⫽ 800)
A. Sakamoto et al.: Solitary lymphangioma of femur
503
Fig. 5 Solitary lymphangioma of the bone. Cancellous bone trabeculae are bordered by a thin-walled lymphatic channel (hematoxylin and eosin, original magnification ⫻80)
Fig. 7 There is no other uptake besides that in the distal femur, according to bone scintigraphy. The right iliac bone, where bone was harvested for the graft, also has a high signal intensity
Fig. 6 Solitary lymphangioma of the bone, showing dilated, thin-walled cystic spaces, which are lined by endothelial cells (hematoxylin and eosin, original magnification ⫻130)
cyst, and this helped us to make a diagnosis of lymphangioma of the bone. Radiographs of lymphangioma of the bone show multiple or multilocular expansive osteolytic lesions associated with a fluid-filled cyst. In some cases, the margin has soap-bubble features,11 suggesting the possibility of an aneurysmal bone cyst. In the present case, CT showed coarse bone trabeculae within the lesion, a feature that is unlikely in the case of an aneurysmal bone cyst. The MRI appearance of a solitary lymphangioma occurring in the humerus has already been
described.10 The lesion in that report demonstrated low intensity on T1-weighted images and high intensity on T2-weighted images, without enhancement by gadolinium. The findings in the current case were consistent with the above. The clinical symptoms and the natural course of solitary lymphangioma of the bone have not been well documented because of its rare incidence. Localized bone involvement is generally asymptomatic, and in most cases it is probably discovered incidentally, although pathological fractures occasionally occur. It has also been reported that solitary lymphangioma of the bone usually grows slowly, becoming stable during adulthood.10 There have even been some reported cases of regression.8 In cases of lymphangioma of the bone where symptoms are present or where there is the possibility of pathological fracture, surgical treatment is the preferred option.10 In summary, we present a case of solitary lymphangioma of the bone arising in the femur. CT revealed coarse bone trabeculae within the lesion. The MRI features showed low intensity to iso intensity on T1-weighted images without enhancement, and high intensity on T2-weighted images. Long-term follow-up should be maintained, because the natural history of this lesion is not yet fully understood.
504 Acknowledgments. The English used in this manuscript was revised by Miss K. Miller (Royal English Language Centre, Fukuoka, Japan). This study was supported in part by Grantsin-Aid for Scientific Research (12557125 and 10307034) from the Japan Society for the Promotion of Science and a Grantin-Aid for Cancer Research from the Ministry of Health and Welfare, Japan.
References 1. Asch MJ, Cohen AH, Moore TC. Hepatic and splenic lymphangiomatosis with skeletal involvement: report of a case and review of the literature. Surgery 1974;76:334–9. 2. Bickel WH, Broders AC. Primary lymphangioma of the ileum: report of a case. J Bone Joint Surg 1947;29:517–22. 3. Bullough PG, Goodfellow JW. Solitary lymphangioma of bone. A case report. J Bone Joint Surg Am 1976;58:418–9.
A. Sakamoto et al.: Solitary lymphangioma of femur 4. Cohen J, Craig JM. Multiple lymphangiectases of bone. J Bone Joint Surg Am 1955;37:585–96. 5. Dorfman HD, Crerniak B. Bone tumors. St. Louis: Mosby; 1998. p. 855–79. 6. Ellis GL, Brannon RB. Intraosseous lymphangiomas of the mandible. Skeletal Radiol 1980;5:253–6. 7. Gilsanz V, Yeh HC, Baron MG. Multiple lymphangiomas of the neck, axilla, mediastinum, and bones in an adult. Radiology 1976;120:161–2. 8. Gutierrez RM, Spjut HJ. Skeletal angiomatosis: report of three cases and review of the literature. Clin Orthop 1972;85:82– 97. 9. Keenen TL, Buehler KC, Campbell JR. Solitary lymphangioma of the spine. Spine 1995;20:102–5. 10. Martinat P, Cotten A, Singer B, et al. Solitary cystic lymphangioma. Skeletal Radiol 1995;24:556–8. 11. Schopfner CE, Allen RP. Lymphangioma of bone. Radiology 1961;76:449–53. 12. Steiner GM, Farman J, Lawson JP. Lymphangiomatosis of bone. Radiology 1969;93:1093–8.
Solitary lymphangioma of the femur. A case report Akio Sakamoto, Shuichi Matsuda, Kazuhiro Tanaka, Tomoyuki Shukuri, Katsumi Harimaya, and Yukihide Iwamoto Department of Orthopaedic Surgery, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan
Abstract Lymphangioma of the bone is a benign lesion, characterized by newly formed lymph vessels. It usually occurs in a diffuse manner or as multiple lesions. Solitary lymphangioma of the bone is an extremely rare condition. We herein describe a case of solitary lymphangioma of the femur in a 55year-old woman who presented with knee pain. Radiographs showed an eccentric osteolytic lesion in the distal femur. This is the first reported case of solitary lymphangioma of the bone occurring in the femur. Key words Solitary lymphangioma · Femur · CT · MRI
Introduction Lymphangioma of the bone is a benign lesion that is generally considered to be a hamartomatous malformation rather than a true neoplasm.5 These lesions occur diffusely or as multiple lesions. Solitary lymphangioma of the bone is an extremely rare condition that was first described in 1947.2 Fewer than 10 cases have previously been reported, all of which occurred in the tibia, humerus, mandible, or vertebra.3,6,9,10 We present a case of solitary lymphangioma of the bone that arose in the femur, a site that has not previously been reported.
studies were unremarkable. Radiographs showed an eccentric osteolytic lesion with moderate osseous expansion located within the medial condyle of the femur (Fig. 1). Computed tomography (CT) revealed a multilocular lesion with coarse bone trabeculae and thinning of the cortex (Fig. 2). Magnetic resonance imaging (MRI) showed a homogenous lesion with low intensity to isointensity on T1-weighted images and high intensity on T2-weighted images (Figs. 3 and 4). The signal intensity of the lesion on the T1-weighted images was greater than that of skeletal muscle, approaching that of fat. No enhancement of the lesion was seen after gadolinium injection. No soft-tissue mass was evident. We performed an open biopsy of the lesion. The cyst lesion was filled with light-brown colored fluid. Histologically, a specimen from the cystic wall showed dilated, thin-walled cystic spaces, which were lined by a single layer of endothelial cells that bordered the bone trabeculae (Figs. 5 and 6). These cystic spaces contained few red blood cells. The features of the specimen were consistent with those of lymphangioma of the bone. The lesion was treated by curettage and autogenous bone grafting. After surgery for the symptomatic lesions in the femur, we carried out bone scintigraphy (technetium) to rule out multiple lesions of lymphangiomatosis. No other lesions were demonstrated (Fig. 7).
Case report A 55-year-old woman was referred to our institute because she suffered from left knee pain while walking. At palpation, the patient had tenderness. The knee was not swollen, and there was no sensation of heat or redness over the knee. Range of motion in the knee joint was full and no instability was seen. Muscle weakness was not observed in the lower extremity. Laboratory Offprint requests to: A. Sakamoto Received: December 7, 2001 / Accepted: February 18, 2002
Discussion The pathogenesis of lymphangioma is not well understood. Insufficiency of lymph flow, leading to back flow into the bone, has been presumed to be the cause.7 Congenital malformation, inflammation, neoplasm, and lymphatic obstruction have also been reported as possible causes of the lesion.4,11 When making a diagnosis of solitary lymphangioma of the bone, the possibility of lymphangiomatosis should be ruled out, because
502
A. Sakamoto et al.: Solitary lymphangioma of femur
Fig. 1 Anterior and lateral views of the left femur show a multiloculated expansive osteolytic lesion within the medial condyle
Fig. 3 Magnetic resonance imaging (MRI) shows a solitary homogenous lesion with low- to iso-signal intensity on T1weighted image (TR ⫽ 500, TE ⫽ 15)
Fig. 2 Computed tomography (CT) reveals coarse bone trabeculae within the cystic lesion with a thinning of the cortex
lymphangioma tends to occur diffusely or as multiple lesions. In the present case, bone scintigraphy (technetium) was found to be useful for excluding the presence of other lesions, as reported in previous studies.11 The histological features of lymphangioma and hemangioma are sometimes similar. For this reason, lymphangioma has also been referred to as “angiomatosis” of the bone by several authors.7,12 It has been reported that the fluid within the cyst in lymphangioma, which is straw colored, is different from that in hemangioma, which is in fact blood.1 In the present case, straw (light-brown) colored fluid was seen within the
Fig. 4 MRI shows a solitary homogenous lesion with high signal intensity on T2-weighted image (TR ⫽ 3000, TE ⫽ 800)
A. Sakamoto et al.: Solitary lymphangioma of femur
503
Fig. 5 Solitary lymphangioma of the bone. Cancellous bone trabeculae are bordered by a thin-walled lymphatic channel (hematoxylin and eosin, original magnification ⫻80)
Fig. 7 There is no other uptake besides that in the distal femur, according to bone scintigraphy. The right iliac bone, where bone was harvested for the graft, also has a high signal intensity
Fig. 6 Solitary lymphangioma of the bone, showing dilated, thin-walled cystic spaces, which are lined by endothelial cells (hematoxylin and eosin, original magnification ⫻130)
cyst, and this helped us to make a diagnosis of lymphangioma of the bone. Radiographs of lymphangioma of the bone show multiple or multilocular expansive osteolytic lesions associated with a fluid-filled cyst. In some cases, the margin has soap-bubble features,11 suggesting the possibility of an aneurysmal bone cyst. In the present case, CT showed coarse bone trabeculae within the lesion, a feature that is unlikely in the case of an aneurysmal bone cyst. The MRI appearance of a solitary lymphangioma occurring in the humerus has already been
described.10 The lesion in that report demonstrated low intensity on T1-weighted images and high intensity on T2-weighted images, without enhancement by gadolinium. The findings in the current case were consistent with the above. The clinical symptoms and the natural course of solitary lymphangioma of the bone have not been well documented because of its rare incidence. Localized bone involvement is generally asymptomatic, and in most cases it is probably discovered incidentally, although pathological fractures occasionally occur. It has also been reported that solitary lymphangioma of the bone usually grows slowly, becoming stable during adulthood.10 There have even been some reported cases of regression.8 In cases of lymphangioma of the bone where symptoms are present or where there is the possibility of pathological fracture, surgical treatment is the preferred option.10 In summary, we present a case of solitary lymphangioma of the bone arising in the femur. CT revealed coarse bone trabeculae within the lesion. The MRI features showed low intensity to iso intensity on T1-weighted images without enhancement, and high intensity on T2-weighted images. Long-term follow-up should be maintained, because the natural history of this lesion is not yet fully understood.
504 Acknowledgments. The English used in this manuscript was revised by Miss K. Miller (Royal English Language Centre, Fukuoka, Japan). This study was supported in part by Grantsin-Aid for Scientific Research (12557125 and 10307034) from the Japan Society for the Promotion of Science and a Grantin-Aid for Cancer Research from the Ministry of Health and Welfare, Japan.
References 1. Asch MJ, Cohen AH, Moore TC. Hepatic and splenic lymphangiomatosis with skeletal involvement: report of a case and review of the literature. Surgery 1974;76:334–9. 2. Bickel WH, Broders AC. Primary lymphangioma of the ileum: report of a case. J Bone Joint Surg 1947;29:517–22. 3. Bullough PG, Goodfellow JW. Solitary lymphangioma of bone. A case report. J Bone Joint Surg Am 1976;58:418–9.
A. Sakamoto et al.: Solitary lymphangioma of femur 4. Cohen J, Craig JM. Multiple lymphangiectases of bone. J Bone Joint Surg Am 1955;37:585–96. 5. Dorfman HD, Crerniak B. Bone tumors. St. Louis: Mosby; 1998. p. 855–79. 6. Ellis GL, Brannon RB. Intraosseous lymphangiomas of the mandible. Skeletal Radiol 1980;5:253–6. 7. Gilsanz V, Yeh HC, Baron MG. Multiple lymphangiomas of the neck, axilla, mediastinum, and bones in an adult. Radiology 1976;120:161–2. 8. Gutierrez RM, Spjut HJ. Skeletal angiomatosis: report of three cases and review of the literature. Clin Orthop 1972;85:82– 97. 9. Keenen TL, Buehler KC, Campbell JR. Solitary lymphangioma of the spine. Spine 1995;20:102–5. 10. Martinat P, Cotten A, Singer B, et al. Solitary cystic lymphangioma. Skeletal Radiol 1995;24:556–8. 11. Schopfner CE, Allen RP. Lymphangioma of bone. Radiology 1961;76:449–53. 12. Steiner GM, Farman J, Lawson JP. Lymphangiomatosis of bone. Radiology 1969;93:1093–8.