The Journal of Foot & Ankle Surgery 53 (2014) 239–242
Contents lists available at ScienceDirect
The Journal of Foot & Ankle Surgery journal homepage: www.jfas.org
JFAS Instructional Course
Primary Epithelioid Angiosarcoma of the Calcaneum: A Diagnostic Dilemma Gopisankar Balaji G, MS Ortho, DNB Ortho 1, Justin S.V. Arockiaraj, MS Ortho, DNB Ortho 2, Alfred Cyril Roy, MS Ortho 2, Burad Deepak, MD Path 3 1 2 3
Assistant Professor in Orthopaedics, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India Assistant Professor in Orthopaedics, Department of Orthopaedics Unit 1, Christian Medical College, Vellore, Tamil Nadu, India Assistant Professor in Pathology, Department of Orthopaedics Unit 1, Christian Medical College, Vellore, Tamil Nadu, India
a r t i c l e i n f o
a b s t r a c t
Level of Clinical Evidence: 4
Primary epithelioid angiosarcoma of the bone is very rare. We report a rare case of epithelioid angiosarcoma of the calcaneum and the difficulties we had in diagnosing this condition. A 22-year-old woman presented with complaints of pain and swelling of the left ankle of 8 years’ duration. Examination revealed swelling and tenderness over the lateral aspect of calcaneum. Plain radiographs showed an osteolytic lesion of the calcaneum. She underwent curettage and bone grafting, with bone substitutes. Histopathologic examination showed epithelioid angiosarcoma in contrast to the needle biopsy, which had showed an aneurysmal bone cyst. The patient was counseled about the need for amputation. She refused limb ablative surgery, and the likelihood of local recurrence and systemic spread and the need for close follow-up were explained. At the end of 3 years of follow-up, she was pain free and had no evidence of recurrence. We present this case because of the rare site, histopathologic challenges in diagnosing the condition, and unique presentation of the disease. Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved.
Keywords: amputation biopsy bone graft substitute bone tumor calcaneus diagnosis
Angiosarcoma is an aggressive, malignant vascular tumor arising from vascular endothelial cells. Primary angiosarcoma of bone is very rare, accounting for 6% of all angiosarcomas (1). We report a rare case of epithelioid angiosarcoma of the calcaneum and the diagnostic challenges we had with this case.
Blood investigations revealed a hemoglobin of 12.1 g%, calcium of 8.9 mg%, phosphorous of 4.4 mg%, and alkaline phosphatase of 151
Case Report A 22-year-old woman presented to our outpatient department with complaints of pain and swelling of the left ankle of 8 years’ duration. The pain had been insidious in onset, dully aching, and slowly progressive. She had been experiencing difficulty in walking because of the pain. She had no history of fever or trauma. She had no comorbid illness. The physical examination revealed diffuse swelling and tenderness over the lateral aspect of the calcaneal region. The ankle range of movement was painless. She had no distal neurovascular deficits. Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: Gopisankar Balaji G, MS Ortho, DNB Ortho, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry 605006, India. E-mail address: [email protected] (G.Balaji G).
Fig. 1. Plain radiograph of the left ankle (anteroposterior [A] and lateral [B] views) showing an osteolytic lesion of the calcaneum.
1067-2516/$ - see front matter Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved. http://dx.doi.org/10.1053/j.jfas.2013.10.010
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G. Balaji G et al. / The Journal of Foot & Ankle Surgery 53 (2014) 239–242
Fig. 2. (A) Sagittal section of computed tomography image showing expansile lytic lesion with multiple loculations and cortical thinning of the calcaneum. (B) Coronal section of computed tomography showing an expansile lytic lesion with cortical thinning and a subtalar joint.
U/L. The parathyroid hormone level was 12 pg/mL. Plain radiographs of the left ankle revealed an osteolytic lesion in the calcaneum with multiple loculations (Fig. 1). Computed tomography showed an expansile lytic lesion with multiple loculations involving the calcaneum and predominantly extending up to the lateral aspect of the calcaneotalar joint (Fig. 2). Bony cortical margins had thinned out at the point of maximum expansion. Expanded loculations showed hyperdense fluid, with a few showing the fluid levels. Bone scintigraphy showed increased tracer uptake in the left calcaneum. The findings for the rest of the skeleton were normal. The differential diagnosis included aneurysmal bone cyst, giant cell tumor, simple bone cyst, and fibrous dysplasia. Needle biopsy of the left calcaneum revealed an aneurysmal bone cyst. Definitive surgery was scheduled. With the patient under general anesthesia, using a lateral approach to the calcaneum, extended curettage was performed, and the defect was filled with an autologous iliac crest graft and bone substitutes (Fig. 3). She was placed in a below the knee plaster cast. The drain was removed after 48 hours. The histopathology report showed fragments of hemorrhagic tumor with extensive iron encrustation. In some areas, many congested and crowded, small, thin-walled blood vessels were seen, lined by neoplastic endothelial cells, with pleomorphic and hyperchromatic nuclei displaying occasional mitosis. In some areas, the tumor cells showed an epithelioid morphology (Fig. 4) and were present in sheets. Foci of tumor-type necrosis were present.
Fig. 3. Perioperative view showing the calcaneal tumor through a lateral approach.
The endothelial cells, including the epithelioid cells, were immunopositive for CD31 (Fig. 5), a more specific endothelial cell marker than CD34. The expression of CD34 was negative. The immunomorphologic features, including the presence of tumor-type necrosis, favored a diagnosis of intermediate-grade epithelioid angiosarcoma. Disparate findings were present between the clinical presentation and the histopathology report. The metastatic workup findings were negative. The patient was advised to undergo limb ablative surgery because of the finding of a malignancy. However, the patient and her husband were not willing for her to undergo amputation. They accepted the risk of local recurrence and systemic spread. She was placed in a below the knee cast after suture removal and advised to perform non-weightbearing crutch walking for 3 months. She was closely followed up. At the end of 6 months, she had no pain. The calcaneal wound had healed well, and no soft tissue masses were present. Radiography revealed a well-consolidated calcaneum. She was advised to begin full weightbearing walking without support. At the end of 3 years of follow-up, she had no pain and no recurrence
Fig. 4. Photomicrographs showing sheets of epithelioid tumor cells with moderately pleomorphic nuclei (hematoxylin and eosin stain, original magnification 40).
G. Balaji G et al. / The Journal of Foot & Ankle Surgery 53 (2014) 239–242
241
Fig. 5. Photomicrograph demonstrating tumor cells with positivity for CD31, an endothelial cell marker (CD31 immunohistochemistry stain, original magnification 20).
Fig. 7. Clinical photograph at the end of 3 years of follow-up showing full painless ankle plantarflexion in the open kinetic chain.
(Fig. 6). Her ankle and subtalar range of movement was full and pain free (Figs. 7 and 8). She was able to walk without any support. No metastasis had developed.
Angiosarcomas are malignant vascular tumors derived from mesenchymal cells. It occurs mostly in the skin and soft tissues. It has also been seen in the spleen, liver, breast, heart, and bone. A great amount of controversy has existed regarding the nature and terminology of malignant vascular tumors. The term angiosarcoma has had many confusing synonyms, including angioendothelioma, hemangioendothelial sarcoma, hemangioendothelioma, and malignant hemangioendothelioma (1,2). Patients with angiosarcomas usually will have a poor prognosis. Primary angiosarcoma of the bone is very rare, accounting for less than 1% of primary malignant neoplasms in the bone (3). The most commonly involved bones have been the femur and tibia, followed by
the pelvis, vertebra, and upper limb bones (4). Epithelioid angiosarcoma is a rare variant. It will be characterized by large cells with epithelioid morphology. These cases can present as solitary or multifocal disease. Multicentric disease will be present in approximately 25% of patients (1). Primary epithelioid angiosarcoma of the calcaneum is very rare. This tumor has appeared in all age groups, with a slightly increased incidence in males. The etiology of angiosarcoma is unclear. The few causes reported have included radiation, trauma, a high estrogen level, and malignant transformation of a benign hemangioma (5). Pain and swelling have been the most common complaints. Often, soft tissue swelling will be present. The clinical course is gradually progressive and eventually leads to metastasis to other bones and organs, namely the lungs. Radiographs will usually show an osteolytic lesion with minimal periosteal reaction and ill-defined borders. A permeative and destructive pattern has also sometimes been seen. The cortex will be partially or totally eroded. Multicentric angiosarcomas will show
Fig. 6. Lateral radiograph of the ankle at 3-year postoperative follow-up examination.
Fig. 8. Clinical photograph at the end of 3 years of follow-up showing full painless ankle dorsiflexion in the open kinetic chain.
Discussion
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G. Balaji G et al. / The Journal of Foot & Ankle Surgery 53 (2014) 239–242
small, punched out lesions, with characteristic clustering of the lesions in a single anatomic region or single limb (4). Computed tomography and magnetic resonance imaging can be helpful in determining the tumor extent and soft tissue involvement. Bone scintigraphy should be performed to rule out multicentricity, because the risk is high. Fine needle aspiration cytology and needle biopsy play an important role in diagnosing skeletal tumors. The accuracy rate with fine needle cytology has been 87.8% (6). However, angiosarcomas will be difficult to recognize on fine needle aspiration smears, when they lack the typical dual, spindle, and epithelioid cell populations (7). Advanced imaging, such as positron emission tomography, gallium enhancement, and bone marrow scanning, along with computed tomography of the abdomen and high-resolution computed tomography of the thorax can be of great value for identifying whether the lesion is primary or secondary. Because angiosarcoma of bone has a slightly greater incidence in the elderly, the differential diagnosis has included myeloma, metastasis, osteosarcoma, fibrosarcoma, and lymphoma. Usually, it has not been confused with benign conditions, unlike our case. It has also presented as deep vein thrombosis on a few occasions (8,9). Our clinical and radiologic diagnosis was that of a benign bone lesion, mostly giant cell tumor and an aneurysmal bone cyst, because of the prolonged duration of symptoms and the clinical and radiologic findings. Although aneurysmal bone tumor is not so common in the third decade of life, the needle biopsy finding was reported as an aneurysmal bone cyst. Hence, we chose to perform curettage and bone grafting. However, the final report was that of angiosarcoma. A vascular tumor with epithelioid morphology and CD31-positive tumor cells can be seen even in an epithelioid hemangioma; however, the presence of the tumor-type necrosis prompted us to diagnose epithelioid angiosarcoma. Just as occurred in our present patient, the histologic features of angiosarcoma can mimic those of an aneurysmal bone cyst. Usually 50% of aneurysmal bone cysts will occur as a secondary lesion in another tumor (3). The diagnosis of angiosarcoma can be confirmed by the presence of immunohistochemistry markers. Hence, a secondary aneurysmal bone cyst in an underlying angiosarcoma can be a possibility and should be ruled out, because the treatment and prognosis will change completely. The role of radiotherapy and chemotherapy for angiosarcoma remains
uncertain (3). Budd (10) has reported that chemotherapy with doxorubicin and ifosfamide produced a good response in angiosarcoma. The 5-year survival rate has been reported to be 12% to 31% for patients with angiosarcoma (3). We have reported the present unique case because of its rarity in bone, the rare site in the calcaneum, the rare variant of angiosarcoma, the unique presentation of the tumor, and the histopathologic challenges in diagnosing this condition. Clinicians must be aware of this condition and also that angiosarcoma can mimic an aneurysmal bone cyst. For pathologists, it can be extremely challenging, because the pathologic features of both conditions resemble each other. In conclusion, clinicopathologic correlation is very important in differentiating angiosarcoma from an aneurysmal bone cyst. An early diagnosis will help in timely intervention, although the prognosis of angiosarcoma has been poor.
References 1. Saglik Y, Yildiz Y, Atalar H, Basarir K. Primary angiosarcoma of the fibula: a case report. Acta Orthop Belg 73:799–803, 2007. 2. Deshpande V, Rosenberg AE, O’Connell JX, Nielsen GP. Epitheliod angiosarcoma of bone: a series of 10 cases. Am J Surg Pathol 27:709–716, 2003. 3. Tse LF, Ek ETH, Slavin JL, Schlicht SM, Choong PFM. Intraosseous angiosarcoma with secondary aneurysmal bone cysts presenting as an elusive diagnostic challenge. Int Semin Surg Oncol 5:10, 2008. 4. Balicki D, Buhrmann R, Maclean J, et al. Multicentric epithelioid angiosarcoma of the bone: pitfalls in clinical and morphologic diagnosis. Blood Cells Mol Dis 22:205–213, 1996. 5. Carter JH, Dickerson R, Needy C. Angiosarcoma of bone: a review of the literature and presentation of a case. Ann Surg 144:107–117, 1956. 6. Nnodu OE, Giwa SO, Eyesan SU, Abdulkareem FB. Fine needle aspiration cytology of bone tumoursdthe experience from the National Orthopaedic and Lagos University Teaching Hospitals, Lagos, Nigeria. CytoJournal 3:16, 2006. 7. Pohar-Marinsek Z, Lamovec J. Angiosarcoma in FNA smears: diagnostic accuracy, morphology, immunocytochemistry and differential diagnoses. Cytopathology 21:311–319, 2010. 8. Gagner JP, Yim JH, Yang GC. Fine-needle aspiration cytology of epithelioid angiosarcoma: a diagnostic dilemma. Diagn Cytopathol 33:429–433, 2005. 9. Greenwald U, Newman E, Taneja S, et al. Malignant epithelioid angiosarcoma of the external iliac vein presenting as venous thrombosis. Ann Vasc Surg 18: 493–496, 2004. 10. Budd GT. Management of angiosarcoma. Curr Oncol Rep 4:515–519, 2002.
Contents lists available at ScienceDirect
The Journal of Foot & Ankle Surgery journal homepage: www.jfas.org
JFAS Instructional Course
Primary Epithelioid Angiosarcoma of the Calcaneum: A Diagnostic Dilemma Gopisankar Balaji G, MS Ortho, DNB Ortho 1, Justin S.V. Arockiaraj, MS Ortho, DNB Ortho 2, Alfred Cyril Roy, MS Ortho 2, Burad Deepak, MD Path 3 1 2 3
Assistant Professor in Orthopaedics, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India Assistant Professor in Orthopaedics, Department of Orthopaedics Unit 1, Christian Medical College, Vellore, Tamil Nadu, India Assistant Professor in Pathology, Department of Orthopaedics Unit 1, Christian Medical College, Vellore, Tamil Nadu, India
a r t i c l e i n f o
a b s t r a c t
Level of Clinical Evidence: 4
Primary epithelioid angiosarcoma of the bone is very rare. We report a rare case of epithelioid angiosarcoma of the calcaneum and the difficulties we had in diagnosing this condition. A 22-year-old woman presented with complaints of pain and swelling of the left ankle of 8 years’ duration. Examination revealed swelling and tenderness over the lateral aspect of calcaneum. Plain radiographs showed an osteolytic lesion of the calcaneum. She underwent curettage and bone grafting, with bone substitutes. Histopathologic examination showed epithelioid angiosarcoma in contrast to the needle biopsy, which had showed an aneurysmal bone cyst. The patient was counseled about the need for amputation. She refused limb ablative surgery, and the likelihood of local recurrence and systemic spread and the need for close follow-up were explained. At the end of 3 years of follow-up, she was pain free and had no evidence of recurrence. We present this case because of the rare site, histopathologic challenges in diagnosing the condition, and unique presentation of the disease. Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved.
Keywords: amputation biopsy bone graft substitute bone tumor calcaneus diagnosis
Angiosarcoma is an aggressive, malignant vascular tumor arising from vascular endothelial cells. Primary angiosarcoma of bone is very rare, accounting for 6% of all angiosarcomas (1). We report a rare case of epithelioid angiosarcoma of the calcaneum and the diagnostic challenges we had with this case.
Blood investigations revealed a hemoglobin of 12.1 g%, calcium of 8.9 mg%, phosphorous of 4.4 mg%, and alkaline phosphatase of 151
Case Report A 22-year-old woman presented to our outpatient department with complaints of pain and swelling of the left ankle of 8 years’ duration. The pain had been insidious in onset, dully aching, and slowly progressive. She had been experiencing difficulty in walking because of the pain. She had no history of fever or trauma. She had no comorbid illness. The physical examination revealed diffuse swelling and tenderness over the lateral aspect of the calcaneal region. The ankle range of movement was painless. She had no distal neurovascular deficits. Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: Gopisankar Balaji G, MS Ortho, DNB Ortho, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry 605006, India. E-mail address: [email protected] (G.Balaji G).
Fig. 1. Plain radiograph of the left ankle (anteroposterior [A] and lateral [B] views) showing an osteolytic lesion of the calcaneum.
1067-2516/$ - see front matter Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved. http://dx.doi.org/10.1053/j.jfas.2013.10.010
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G. Balaji G et al. / The Journal of Foot & Ankle Surgery 53 (2014) 239–242
Fig. 2. (A) Sagittal section of computed tomography image showing expansile lytic lesion with multiple loculations and cortical thinning of the calcaneum. (B) Coronal section of computed tomography showing an expansile lytic lesion with cortical thinning and a subtalar joint.
U/L. The parathyroid hormone level was 12 pg/mL. Plain radiographs of the left ankle revealed an osteolytic lesion in the calcaneum with multiple loculations (Fig. 1). Computed tomography showed an expansile lytic lesion with multiple loculations involving the calcaneum and predominantly extending up to the lateral aspect of the calcaneotalar joint (Fig. 2). Bony cortical margins had thinned out at the point of maximum expansion. Expanded loculations showed hyperdense fluid, with a few showing the fluid levels. Bone scintigraphy showed increased tracer uptake in the left calcaneum. The findings for the rest of the skeleton were normal. The differential diagnosis included aneurysmal bone cyst, giant cell tumor, simple bone cyst, and fibrous dysplasia. Needle biopsy of the left calcaneum revealed an aneurysmal bone cyst. Definitive surgery was scheduled. With the patient under general anesthesia, using a lateral approach to the calcaneum, extended curettage was performed, and the defect was filled with an autologous iliac crest graft and bone substitutes (Fig. 3). She was placed in a below the knee plaster cast. The drain was removed after 48 hours. The histopathology report showed fragments of hemorrhagic tumor with extensive iron encrustation. In some areas, many congested and crowded, small, thin-walled blood vessels were seen, lined by neoplastic endothelial cells, with pleomorphic and hyperchromatic nuclei displaying occasional mitosis. In some areas, the tumor cells showed an epithelioid morphology (Fig. 4) and were present in sheets. Foci of tumor-type necrosis were present.
Fig. 3. Perioperative view showing the calcaneal tumor through a lateral approach.
The endothelial cells, including the epithelioid cells, were immunopositive for CD31 (Fig. 5), a more specific endothelial cell marker than CD34. The expression of CD34 was negative. The immunomorphologic features, including the presence of tumor-type necrosis, favored a diagnosis of intermediate-grade epithelioid angiosarcoma. Disparate findings were present between the clinical presentation and the histopathology report. The metastatic workup findings were negative. The patient was advised to undergo limb ablative surgery because of the finding of a malignancy. However, the patient and her husband were not willing for her to undergo amputation. They accepted the risk of local recurrence and systemic spread. She was placed in a below the knee cast after suture removal and advised to perform non-weightbearing crutch walking for 3 months. She was closely followed up. At the end of 6 months, she had no pain. The calcaneal wound had healed well, and no soft tissue masses were present. Radiography revealed a well-consolidated calcaneum. She was advised to begin full weightbearing walking without support. At the end of 3 years of follow-up, she had no pain and no recurrence
Fig. 4. Photomicrographs showing sheets of epithelioid tumor cells with moderately pleomorphic nuclei (hematoxylin and eosin stain, original magnification 40).
G. Balaji G et al. / The Journal of Foot & Ankle Surgery 53 (2014) 239–242
241
Fig. 5. Photomicrograph demonstrating tumor cells with positivity for CD31, an endothelial cell marker (CD31 immunohistochemistry stain, original magnification 20).
Fig. 7. Clinical photograph at the end of 3 years of follow-up showing full painless ankle plantarflexion in the open kinetic chain.
(Fig. 6). Her ankle and subtalar range of movement was full and pain free (Figs. 7 and 8). She was able to walk without any support. No metastasis had developed.
Angiosarcomas are malignant vascular tumors derived from mesenchymal cells. It occurs mostly in the skin and soft tissues. It has also been seen in the spleen, liver, breast, heart, and bone. A great amount of controversy has existed regarding the nature and terminology of malignant vascular tumors. The term angiosarcoma has had many confusing synonyms, including angioendothelioma, hemangioendothelial sarcoma, hemangioendothelioma, and malignant hemangioendothelioma (1,2). Patients with angiosarcomas usually will have a poor prognosis. Primary angiosarcoma of the bone is very rare, accounting for less than 1% of primary malignant neoplasms in the bone (3). The most commonly involved bones have been the femur and tibia, followed by
the pelvis, vertebra, and upper limb bones (4). Epithelioid angiosarcoma is a rare variant. It will be characterized by large cells with epithelioid morphology. These cases can present as solitary or multifocal disease. Multicentric disease will be present in approximately 25% of patients (1). Primary epithelioid angiosarcoma of the calcaneum is very rare. This tumor has appeared in all age groups, with a slightly increased incidence in males. The etiology of angiosarcoma is unclear. The few causes reported have included radiation, trauma, a high estrogen level, and malignant transformation of a benign hemangioma (5). Pain and swelling have been the most common complaints. Often, soft tissue swelling will be present. The clinical course is gradually progressive and eventually leads to metastasis to other bones and organs, namely the lungs. Radiographs will usually show an osteolytic lesion with minimal periosteal reaction and ill-defined borders. A permeative and destructive pattern has also sometimes been seen. The cortex will be partially or totally eroded. Multicentric angiosarcomas will show
Fig. 6. Lateral radiograph of the ankle at 3-year postoperative follow-up examination.
Fig. 8. Clinical photograph at the end of 3 years of follow-up showing full painless ankle dorsiflexion in the open kinetic chain.
Discussion
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G. Balaji G et al. / The Journal of Foot & Ankle Surgery 53 (2014) 239–242
small, punched out lesions, with characteristic clustering of the lesions in a single anatomic region or single limb (4). Computed tomography and magnetic resonance imaging can be helpful in determining the tumor extent and soft tissue involvement. Bone scintigraphy should be performed to rule out multicentricity, because the risk is high. Fine needle aspiration cytology and needle biopsy play an important role in diagnosing skeletal tumors. The accuracy rate with fine needle cytology has been 87.8% (6). However, angiosarcomas will be difficult to recognize on fine needle aspiration smears, when they lack the typical dual, spindle, and epithelioid cell populations (7). Advanced imaging, such as positron emission tomography, gallium enhancement, and bone marrow scanning, along with computed tomography of the abdomen and high-resolution computed tomography of the thorax can be of great value for identifying whether the lesion is primary or secondary. Because angiosarcoma of bone has a slightly greater incidence in the elderly, the differential diagnosis has included myeloma, metastasis, osteosarcoma, fibrosarcoma, and lymphoma. Usually, it has not been confused with benign conditions, unlike our case. It has also presented as deep vein thrombosis on a few occasions (8,9). Our clinical and radiologic diagnosis was that of a benign bone lesion, mostly giant cell tumor and an aneurysmal bone cyst, because of the prolonged duration of symptoms and the clinical and radiologic findings. Although aneurysmal bone tumor is not so common in the third decade of life, the needle biopsy finding was reported as an aneurysmal bone cyst. Hence, we chose to perform curettage and bone grafting. However, the final report was that of angiosarcoma. A vascular tumor with epithelioid morphology and CD31-positive tumor cells can be seen even in an epithelioid hemangioma; however, the presence of the tumor-type necrosis prompted us to diagnose epithelioid angiosarcoma. Just as occurred in our present patient, the histologic features of angiosarcoma can mimic those of an aneurysmal bone cyst. Usually 50% of aneurysmal bone cysts will occur as a secondary lesion in another tumor (3). The diagnosis of angiosarcoma can be confirmed by the presence of immunohistochemistry markers. Hence, a secondary aneurysmal bone cyst in an underlying angiosarcoma can be a possibility and should be ruled out, because the treatment and prognosis will change completely. The role of radiotherapy and chemotherapy for angiosarcoma remains
uncertain (3). Budd (10) has reported that chemotherapy with doxorubicin and ifosfamide produced a good response in angiosarcoma. The 5-year survival rate has been reported to be 12% to 31% for patients with angiosarcoma (3). We have reported the present unique case because of its rarity in bone, the rare site in the calcaneum, the rare variant of angiosarcoma, the unique presentation of the tumor, and the histopathologic challenges in diagnosing this condition. Clinicians must be aware of this condition and also that angiosarcoma can mimic an aneurysmal bone cyst. For pathologists, it can be extremely challenging, because the pathologic features of both conditions resemble each other. In conclusion, clinicopathologic correlation is very important in differentiating angiosarcoma from an aneurysmal bone cyst. An early diagnosis will help in timely intervention, although the prognosis of angiosarcoma has been poor.
References 1. Saglik Y, Yildiz Y, Atalar H, Basarir K. Primary angiosarcoma of the fibula: a case report. Acta Orthop Belg 73:799–803, 2007. 2. Deshpande V, Rosenberg AE, O’Connell JX, Nielsen GP. Epitheliod angiosarcoma of bone: a series of 10 cases. Am J Surg Pathol 27:709–716, 2003. 3. Tse LF, Ek ETH, Slavin JL, Schlicht SM, Choong PFM. Intraosseous angiosarcoma with secondary aneurysmal bone cysts presenting as an elusive diagnostic challenge. Int Semin Surg Oncol 5:10, 2008. 4. Balicki D, Buhrmann R, Maclean J, et al. Multicentric epithelioid angiosarcoma of the bone: pitfalls in clinical and morphologic diagnosis. Blood Cells Mol Dis 22:205–213, 1996. 5. Carter JH, Dickerson R, Needy C. Angiosarcoma of bone: a review of the literature and presentation of a case. Ann Surg 144:107–117, 1956. 6. Nnodu OE, Giwa SO, Eyesan SU, Abdulkareem FB. Fine needle aspiration cytology of bone tumoursdthe experience from the National Orthopaedic and Lagos University Teaching Hospitals, Lagos, Nigeria. CytoJournal 3:16, 2006. 7. Pohar-Marinsek Z, Lamovec J. Angiosarcoma in FNA smears: diagnostic accuracy, morphology, immunocytochemistry and differential diagnoses. Cytopathology 21:311–319, 2010. 8. Gagner JP, Yim JH, Yang GC. Fine-needle aspiration cytology of epithelioid angiosarcoma: a diagnostic dilemma. Diagn Cytopathol 33:429–433, 2005. 9. Greenwald U, Newman E, Taneja S, et al. Malignant epithelioid angiosarcoma of the external iliac vein presenting as venous thrombosis. Ann Vasc Surg 18: 493–496, 2004. 10. Budd GT. Management of angiosarcoma. Curr Oncol Rep 4:515–519, 2002.
