- Email: [email protected]
Chondroblastoma with adamantinoma differentiation of the calcaneus
174
Pathology (2011), 43(2), February
CORRESPONDENCE
Contact Dr M. J. Wilsher. E-mail: [email protected] 1. Kimura N, Miura Y, Miura Y, et al. Adrenal and retroperitoneal mixed neuroendocrine-neural tumors. Endocr Pathol 1991; 2: 139–47. 2. Comstock JM, Willmore-Payne C, Holden JA, et al. Composite phaeochromocytoma. A clinical and molecular comparison with ordinary phaeochromocytoma and neuroblastoma. Am J Clin Pathol 2009; 132: 69–73. 3. Gupta R, Sharma A, Arora R, et al. Composite phaeochromocytoma with malignant peripheral nerve sheath tumour and rhabomyosarcomatous differentiation in a patient without von Recklinghausen disease. J Clin Pathol 2009; 62: 659–61. 4. Juarez D, Brown RW, Ostowski M, et al. Phaeochromocytoma associated with neuroendocrine carcinoma. A new type of composite phaeochromocytoma. Arch Pathol Lab Med 1999; 123: 1274–9. 5. Chetty R. Familial paraganglioma syndromes. J Clin Pathol 2010; 63: 488– 91. 6. Mustafa E, Mustafa Y, Cengiz A, et al. Pancreatic involvement in Von Hippel-Lindau disease. Indian J Cancer 2004; 41: 159–61. 7. Sood GK, Chaudry A, Malhotra V, et al. Pancreatic cystadenoma associated with von Recklinghausen’s disease. Indian J Gastroenterol 1991; 10: 148–9. 8. Goldstein RE, O’Neill JA, Holcomb GW III et al. Clinical experience over 48 years with phaeochromocytoma. Ann Surg 1999; 229: 755–66. 9. Strong VE, Kennedy T, Al-Ahmadie H, et al. Prognostic indicators of malignancy in adrenal phaeochromocytomas: clinical, histopathologic, and cell cycle/apoptosis gene expression analysis. Surgery 2008; 143: 759–68. 10. Kimura N, Watanabe T, Fukase M, et al. Neurofibromin and NF1 gene analysis in composite phaeochromocytoma and tumours associated with von Recklinghausen’s disease. Mod Pathol 2002; 15: 183–8. 11. Candanedo-Gonzalez FA, Alvarado-Cabero I, Gamboa-Dominguez A, et al. Sporadic type phaeochromocytoma with neuroblastoma: clinicomorphologic, DNA content, and Ret gene analysis. Endocr Pathol 2001; 12: 343–50. 12. McNichol A-M. Differential diagnosis of phaeochromocytomas and paragangliomas. Endocr Pathol 2001; 12: 407–15. 13. Kimura N, Fukase M, Wakita A, et al. Loss of the neurofibromin-NF1 gene product and composite phaeochromocytoma. Ann NY Acad Sci 2002; 971: 536–8. 14. Jett K, Friedman JM. Clinical and genetic aspects of neurofibromatosis 1. Genet Med 2010; 12: 1–11. 15. Alamsamimi M, Mirkheshiti N, Mohajery M-R, et al. Bilateral invasive ductal carcinoma in a woman with neurofibromatosis type 1. Arch Iranian Med 2009; 12: 412–4. 16. Sakaguchi N, Sano K, Ito M, et al. A case of von Recklinghausen’s disease with bilateral phaeochromocytoma-malignant peripheral nerve sheath tumors of the adrenal and gastrointestinal autonomic nerve tumours. Am J Surg Pathol 1996; 20: 889–97. 17. Kimura N, Watanabe T, Noshiro T, et al. Histological grading of adrenal and extra-adrenal phaeochromocytomas and relationship to prognosis: a clinicopathological analysis of 116 adrenal phaeochromocytomas and 30 extra-adrenal sympathetic paragangliomas including 38 malignant tumours. Endocrine Pathol 2005; 16: 23–32. 18. Satake H, Inoue K, Kamada M, et al. Malignant composite phaeochromocytoma of the adrenal gland in a patient with von Recklinghausen’s disease. J Urol 2001; 165: 1199–200.
19. Grogan JR, Saeian K, Taylor AJ, et al. Making sense of mucin-producing pancreatic tumors. AJR 2001; 176: 921–9. 20. Hsiao W-C, Lin P-W, Chang K-C. Benign retroperitoneal schwannoma mimicking a pancreatic cystic tumour: case report and literature review. Hepatogastoenterology 1998; 45: 2418–20. 21. Inzani F, Rindi G, Tamborrino E, et al. Extra-adrenal composite paraganglioma with ganglioneuroma component presenting as a pancreatic mass. Endocr Pathol 2009; 20: 191–5. 22. Chi E-K, Kim WH, Park K-Y. A case of a composite adrenal medullary tumour of phaeochromocytoma and ganglioneuroma masquerading as acute pancreatitis. Korean J Intern Med 2006; 21: 141–5.
DOI: 10.1097/PAT.0b013e32834274a3
Chondroblastoma with adamantinoma differentiation of the calcaneus Sir, Chondroblastoma is a rare, benign tumour of the bone and most frequently involves the epiphysis of the long bones, especially the femur and tibia. Chondroblastoma of the calcaneus is very rare and mostly presented in case reports.1,2 Adamantinoma of the long tubular bones is a rare primary low grade malignant bone tumour with disputed histogenesis, first described and named by Fischer in 1913 because of its close morphological resemblance to adamantinoma of the jaws. In 85–90% of cases reported, adamantinoma of the bone is localised to the tibia, mostly on the median third of the diaphysis; nonetheless, it has also been described in the fibula, ulna, humerus and femur.3,4 Adamantinoma of the foot is extremely rare. To our knowledge, there is only one case of calcaneal adamantinoma reported in the English literature.5 Herein, we present the extremely rare case of chondroblastoma with adamantinoma differentiation arising in the calcaneus. The patient was an 18-year-old male referred to our hospital due to high temperature of focal skin and swelling of the left ankle for 2 years but no left ankle pain. Radiographs showed a lytic expansile mass measuring 51 26 mm with a sclerotic margin in the cortical plate of the left calcaneus (Fig. 1), indicative of aneurysmal bone cyst. The patient did not show specific results in laboratory tests. He was treated by curettage and bone grafting. Macroscopically, the surgical specimen was 50 25 20 mm in size. The cut section of tumour was firm and yellowish-grey with focal cystic change.
Fig. 1 (A) Plain film showing a lytic expansile mass with sclerotic margin in the cortical plate of the left calcaneus. (B) Axial CT scan of the left calcaneus utilising bone window reveals an expansive intraosseous lesion surrounded by an obvious bony sclerosis.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
CORRESPONDENCE
Microscopically, the tumour was predominantly composed of a proliferation of round cells in lobular pattern. The tumour cells had round or ovoid nuclei, indistinct nucleoli and a moderate to abundant amount of eosinophilic cytoplasm. Some nuclei were indented with longitudinal grooves. Cellular atypia was mild. Mitotic activity was rarely found and atypical mitosis was absent. Foci of chondroid and osteoid matrix with partial ossification were present. Osteoclastic-like giant cells were scattered in the lesion. In some areas of the tumour, large clusters of basaloid epithelial islands, tubular formation of the epithelial cells, and squamous cells with focal keratinisation were present (Fig. 2), which were characterised by ameloblastic
Fig. 2 (A) Chondroblastoma coexisting with adamantinoma at lower power. (B) Chondroblastoma area. (C) Adamantinoma differentiation area (H&E).
175
epithelium differentiation. Immunohistochemical studies showed the mononuclear round cells were diffusely positive for vimentin and S-100 protein. Ameloblastic epithelial cells were positive for cytokeratin (CK) (Fig. 3), epithelial membrane antigen (EMA), CK19, CK8/18 and p63. The proliferative index (Ki-67) was approximately 1% of tumour cells. Osteoclastic-like giant cells were highlighted by CD68. Tumour cells were negative for actin, CD31 and CD34. These findings led to the diagnosis of chondroblastoma with adamantinoma differentiation in the left calcaneus. The patient underwent curettage treatment and bone grafting without adjuvant radiotherapy and chemotherapy. The patient was alive without recurrence at 6 months following surgery. Chondroblastoma is a rare benign bone tumour and mostly involves the epiphysis of long bones such as the femur, humerus, and tibia. Most patients are between 10 and 25 years of age at diagnosis and there is a male predominance. Adamantinoma is also a rare primary low grade malignant bone tumour. It accounts for 0.33% of all malignant bone tumours in the material of Dahlin6 and 0.48% in that of Schajowicz.7 Adamantinoma is a rare biphasic tumour and predominantly affects young adults, with the overwhelming majority (about 85%) arising in the tibia.3 Rare cases have been reported in bones of the upper extremities, pelvis, and ribs. There is only one case of calcaneal adamantinoma, reported by Chandrasekar et al. in 2009.5 To our knowledge, we present the first case of chondroblastoma with adamantinoma differentiation of the left
Fig. 3 (A) Tumour cells in the chondroblastoma area were positive for S-100 protein by immunohistochemistry. (B) Tumour cells in the adamantinoma differentiation area were positive for CK by immunohistochemistry.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
176
Pathology (2011), 43(2), February
CORRESPONDENCE
calcaneus in an 18-year-old male patient, who presented with swelling of the left ankle for 2 years. The differential diagnosis includes epithelioid chondroblastoma, mixed tumour/myoepithelioma, clear cell chondrosarcoma, synovial sarcoma and metastatic carcinoma. Epithelioid differentiation of chondroblastoma has been reported in the rare cases that occur in the temporal bone and other bones of the skull.8,9 Areas of epithelioid differentiation, typically with more closely packed chondroblasts with eosinophilic cytoplasm, is complicated by the expression of cytokeratin and EMA.10 In general, cytokeratin and EMA expression by chondroblastoma will be in the minority of cells. It is important that there is no ameloblastic epithelium differentiation in epithelioid chondroblastoma. Mixed tumour/myoepithelioma is composed of varying proportions of uniform-appearing, epithelioid cells with eosinophilic to clear cytoplasm, arranged in nests, cords, and ductules and/or spindle cells embedded in a hyalinised to chondromyxoid matrix. Divergent differentiation, including squamous, adipocytic, and bone and cartilaginous metaplasia, may be observed. Myoepithelioma typically lacks a definite ductal component. The histopathological and immunohistochemical findings of mixed tumour/myoepithelioma are similar to our present case. However, clear cut histological evidence of chondroblastoma with grooved and vesicular nuclei in our case may help render a correct interpretation. Furthermore, primary mixed tumour/myoepithelioma of the bone is extremely rare.11 Clear cell chondrosarcoma may be confused with chondroblastoma. However, clear cell chondrosarcoma tends to occur in most patients between 25 and 50 years of age. The tumour consists of large cells with abundant clear or pale cytoplasm and atypical nuclei, which are absent in chondroblastoma. Synovial sarcoma, which is a biphasic or monophasic tumour, should be differentiated from our current case. There is no definite chondroblastoma component in synovial sarcoma. Absence of cellular atypia, mitotic activity, infiltrative growth and presence of chondroblastoma is easy to distinguish from metastatic carcinoma. Treatment of chondroblastoma is determined by the anatomical location and degree of bone and/or joint involvement. The most accepted treatment is curettage and bone grafting. Adamantinoma is a low grade malignant tumour and known to be resistant to both radio- and chemotherapy. Szendroi et al. reported that wide segment resection gives good results in primary adamantinoma, and also in recurrences.3 In our case, the patient was treated by curettage and bone grafting. The postoperative course was uneventful, and the patient was alive without sign of recurrence at 6 month follow-up. The recurrence rate of adamantinoma is reported in the literature to be as high as 30–35% and they can recur even after 10–20 years following recognition of the tumour.12 Therefore, a life-long follow-up of the patient is necessary. Dawei Liu* Ping Xiao* Zhenhua Gao{ Ling Xue* Liantang Wang* Yongdong Liu* Anjia Han* Departments of *Pathology, and {Radiology, the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
Contact Dr A. Han. E-mail: [email protected] 1. Guedes A, Barreto B, Soares Barreto BL, Athanazio DA, Athanazio PR. Calcaneal chondroblastoma with secondary aneurysmal bone cyst: a case report. J Foot Ankle Surg 2010; 49: 298. 2. Davila JA, Amrami KK, Sundaram M, Adkins MC, Unni KK. Chondroblastoma of the hands and feet. Skeletal Radiol 2004; 33: 582–7. 3. Szendroi M, Antal I, Arato G. Adamantinoma of long bones: a long-term follow-up study of 11 cases. Pathol Oncol Res 2009; 15: 209–16. 4. Papagelopoulos PJ, Mavrogenis AF, Galanis EC, Savvidou OD, Inwards CY, Sim FH. Clinicopathological features, diagnosis, and treatment of adamantinoma of the long bones. Orthopedics 2007; 30: 211–5. 216–7. 5. Chandrasekar CR, Mohammed R, Rafalla AA, Grimer RJ. Adamantinoma of the calcaneum–a case report. Foot (Edinb) 2009; 19: 58–61. 6. Dahlin DC. Adamantinoma of long bones. In: Charles CT, editor. Bone Tumors. General Aspects and Data on 6221 Cases. Springfield: LippincottRaven, 1978; 296–306. 7. Schajowicz F. Tumors and Tumor Like Lesions of Bones and Joints. New York: Springer, 1981; 384–91. 8. Deyrup AT, Montag AG. Epithelioid and epithelial neoplasms of bone. Arch Pathol Lab Med 2007; 131: 205–16. 9. Bertoni F, Unni KK, Beabout JW, Harner SG, Dahlin DC. Chondroblastoma of the skull and facial bones. Am J Clin Pathol 1987; 88: 1–9. 10. Semmelink HJ, Pruszczynski M, Wiersma-van TA, Smedts F, Ramaekers FC. Cytokeratin expression in chondroblastomas. Histopathology 1990; 16: 257–63. 11. Jain VK, Jain D, Jawed A, Prasad A, Sachdev N, Kumar S. Primary myoepithelioma of bone. Pathology 2010; 42: 190–3. 12. Jundt G, Remberger K, Roessner A, Schulz A, Bohndorf K. Adamantinoma of long bones. A histopathological and immunohistochemical study of 23 cases. Pathol Res Pract 1995; 191: 112–20.
DOI: 10.1097/PAT.0b013e328342747a
Composite haemangioendothelioma: report of four cases with emphasis on atypical clinical presentation Sir, To date, there have been approximately 20 cases of composite haemangioendothelioma (HE) reported.1,2 Owing to its rarity and complex morphological variations, composite HE continues to pose diagnostic challenges in the distinction from other mimicking vascular tumours, particularly for those occurring in atypical clinical settings. To further broaden the clinicopathological and immunohistochemical spectrum of composite HE, we describe four new cases, including two occurring in the hitherto unreported hypopharynx and one in a patient with neurofibromatosis type 2 (NF2). Composite HE was first designated by Nayler et al. in 2000 as one of the rarest borderline vascular tumours with an admixture of concurrent benign (e.g., spindle cell haemangiomas), intermediate (e.g., retiform HE), and malignant (e.g., angiosarcoma-like) vascular lesions of various types.2 Retiform and epithelioid HEs represent the two most common histological types of composite HE.1,2 However, the variations in composition of vascular tumour types appear not correlated with clinical outcomes. Local relapses are not uncommon in composite HE and tend to occur several years after tumour resection, while the potential of distal and regional nodal metastases is considered very low.1–3 Clinically, composite HE preferentially manifests as a dermal and/or subcutaneous lesion in the distal extremities of young to middle-aged adults with a slow evolution.1–3 However, there were indeed a handful of previously published congenital composite HEs that had been noted since birth with longstanding presentation before
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
Pathology (2011), 43(2), February
CORRESPONDENCE
Contact Dr M. J. Wilsher. E-mail: [email protected] 1. Kimura N, Miura Y, Miura Y, et al. Adrenal and retroperitoneal mixed neuroendocrine-neural tumors. Endocr Pathol 1991; 2: 139–47. 2. Comstock JM, Willmore-Payne C, Holden JA, et al. Composite phaeochromocytoma. A clinical and molecular comparison with ordinary phaeochromocytoma and neuroblastoma. Am J Clin Pathol 2009; 132: 69–73. 3. Gupta R, Sharma A, Arora R, et al. Composite phaeochromocytoma with malignant peripheral nerve sheath tumour and rhabomyosarcomatous differentiation in a patient without von Recklinghausen disease. J Clin Pathol 2009; 62: 659–61. 4. Juarez D, Brown RW, Ostowski M, et al. Phaeochromocytoma associated with neuroendocrine carcinoma. A new type of composite phaeochromocytoma. Arch Pathol Lab Med 1999; 123: 1274–9. 5. Chetty R. Familial paraganglioma syndromes. J Clin Pathol 2010; 63: 488– 91. 6. Mustafa E, Mustafa Y, Cengiz A, et al. Pancreatic involvement in Von Hippel-Lindau disease. Indian J Cancer 2004; 41: 159–61. 7. Sood GK, Chaudry A, Malhotra V, et al. Pancreatic cystadenoma associated with von Recklinghausen’s disease. Indian J Gastroenterol 1991; 10: 148–9. 8. Goldstein RE, O’Neill JA, Holcomb GW III et al. Clinical experience over 48 years with phaeochromocytoma. Ann Surg 1999; 229: 755–66. 9. Strong VE, Kennedy T, Al-Ahmadie H, et al. Prognostic indicators of malignancy in adrenal phaeochromocytomas: clinical, histopathologic, and cell cycle/apoptosis gene expression analysis. Surgery 2008; 143: 759–68. 10. Kimura N, Watanabe T, Fukase M, et al. Neurofibromin and NF1 gene analysis in composite phaeochromocytoma and tumours associated with von Recklinghausen’s disease. Mod Pathol 2002; 15: 183–8. 11. Candanedo-Gonzalez FA, Alvarado-Cabero I, Gamboa-Dominguez A, et al. Sporadic type phaeochromocytoma with neuroblastoma: clinicomorphologic, DNA content, and Ret gene analysis. Endocr Pathol 2001; 12: 343–50. 12. McNichol A-M. Differential diagnosis of phaeochromocytomas and paragangliomas. Endocr Pathol 2001; 12: 407–15. 13. Kimura N, Fukase M, Wakita A, et al. Loss of the neurofibromin-NF1 gene product and composite phaeochromocytoma. Ann NY Acad Sci 2002; 971: 536–8. 14. Jett K, Friedman JM. Clinical and genetic aspects of neurofibromatosis 1. Genet Med 2010; 12: 1–11. 15. Alamsamimi M, Mirkheshiti N, Mohajery M-R, et al. Bilateral invasive ductal carcinoma in a woman with neurofibromatosis type 1. Arch Iranian Med 2009; 12: 412–4. 16. Sakaguchi N, Sano K, Ito M, et al. A case of von Recklinghausen’s disease with bilateral phaeochromocytoma-malignant peripheral nerve sheath tumors of the adrenal and gastrointestinal autonomic nerve tumours. Am J Surg Pathol 1996; 20: 889–97. 17. Kimura N, Watanabe T, Noshiro T, et al. Histological grading of adrenal and extra-adrenal phaeochromocytomas and relationship to prognosis: a clinicopathological analysis of 116 adrenal phaeochromocytomas and 30 extra-adrenal sympathetic paragangliomas including 38 malignant tumours. Endocrine Pathol 2005; 16: 23–32. 18. Satake H, Inoue K, Kamada M, et al. Malignant composite phaeochromocytoma of the adrenal gland in a patient with von Recklinghausen’s disease. J Urol 2001; 165: 1199–200.
19. Grogan JR, Saeian K, Taylor AJ, et al. Making sense of mucin-producing pancreatic tumors. AJR 2001; 176: 921–9. 20. Hsiao W-C, Lin P-W, Chang K-C. Benign retroperitoneal schwannoma mimicking a pancreatic cystic tumour: case report and literature review. Hepatogastoenterology 1998; 45: 2418–20. 21. Inzani F, Rindi G, Tamborrino E, et al. Extra-adrenal composite paraganglioma with ganglioneuroma component presenting as a pancreatic mass. Endocr Pathol 2009; 20: 191–5. 22. Chi E-K, Kim WH, Park K-Y. A case of a composite adrenal medullary tumour of phaeochromocytoma and ganglioneuroma masquerading as acute pancreatitis. Korean J Intern Med 2006; 21: 141–5.
DOI: 10.1097/PAT.0b013e32834274a3
Chondroblastoma with adamantinoma differentiation of the calcaneus Sir, Chondroblastoma is a rare, benign tumour of the bone and most frequently involves the epiphysis of the long bones, especially the femur and tibia. Chondroblastoma of the calcaneus is very rare and mostly presented in case reports.1,2 Adamantinoma of the long tubular bones is a rare primary low grade malignant bone tumour with disputed histogenesis, first described and named by Fischer in 1913 because of its close morphological resemblance to adamantinoma of the jaws. In 85–90% of cases reported, adamantinoma of the bone is localised to the tibia, mostly on the median third of the diaphysis; nonetheless, it has also been described in the fibula, ulna, humerus and femur.3,4 Adamantinoma of the foot is extremely rare. To our knowledge, there is only one case of calcaneal adamantinoma reported in the English literature.5 Herein, we present the extremely rare case of chondroblastoma with adamantinoma differentiation arising in the calcaneus. The patient was an 18-year-old male referred to our hospital due to high temperature of focal skin and swelling of the left ankle for 2 years but no left ankle pain. Radiographs showed a lytic expansile mass measuring 51 26 mm with a sclerotic margin in the cortical plate of the left calcaneus (Fig. 1), indicative of aneurysmal bone cyst. The patient did not show specific results in laboratory tests. He was treated by curettage and bone grafting. Macroscopically, the surgical specimen was 50 25 20 mm in size. The cut section of tumour was firm and yellowish-grey with focal cystic change.
Fig. 1 (A) Plain film showing a lytic expansile mass with sclerotic margin in the cortical plate of the left calcaneus. (B) Axial CT scan of the left calcaneus utilising bone window reveals an expansive intraosseous lesion surrounded by an obvious bony sclerosis.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
CORRESPONDENCE
Microscopically, the tumour was predominantly composed of a proliferation of round cells in lobular pattern. The tumour cells had round or ovoid nuclei, indistinct nucleoli and a moderate to abundant amount of eosinophilic cytoplasm. Some nuclei were indented with longitudinal grooves. Cellular atypia was mild. Mitotic activity was rarely found and atypical mitosis was absent. Foci of chondroid and osteoid matrix with partial ossification were present. Osteoclastic-like giant cells were scattered in the lesion. In some areas of the tumour, large clusters of basaloid epithelial islands, tubular formation of the epithelial cells, and squamous cells with focal keratinisation were present (Fig. 2), which were characterised by ameloblastic
Fig. 2 (A) Chondroblastoma coexisting with adamantinoma at lower power. (B) Chondroblastoma area. (C) Adamantinoma differentiation area (H&E).
175
epithelium differentiation. Immunohistochemical studies showed the mononuclear round cells were diffusely positive for vimentin and S-100 protein. Ameloblastic epithelial cells were positive for cytokeratin (CK) (Fig. 3), epithelial membrane antigen (EMA), CK19, CK8/18 and p63. The proliferative index (Ki-67) was approximately 1% of tumour cells. Osteoclastic-like giant cells were highlighted by CD68. Tumour cells were negative for actin, CD31 and CD34. These findings led to the diagnosis of chondroblastoma with adamantinoma differentiation in the left calcaneus. The patient underwent curettage treatment and bone grafting without adjuvant radiotherapy and chemotherapy. The patient was alive without recurrence at 6 months following surgery. Chondroblastoma is a rare benign bone tumour and mostly involves the epiphysis of long bones such as the femur, humerus, and tibia. Most patients are between 10 and 25 years of age at diagnosis and there is a male predominance. Adamantinoma is also a rare primary low grade malignant bone tumour. It accounts for 0.33% of all malignant bone tumours in the material of Dahlin6 and 0.48% in that of Schajowicz.7 Adamantinoma is a rare biphasic tumour and predominantly affects young adults, with the overwhelming majority (about 85%) arising in the tibia.3 Rare cases have been reported in bones of the upper extremities, pelvis, and ribs. There is only one case of calcaneal adamantinoma, reported by Chandrasekar et al. in 2009.5 To our knowledge, we present the first case of chondroblastoma with adamantinoma differentiation of the left
Fig. 3 (A) Tumour cells in the chondroblastoma area were positive for S-100 protein by immunohistochemistry. (B) Tumour cells in the adamantinoma differentiation area were positive for CK by immunohistochemistry.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
176
Pathology (2011), 43(2), February
CORRESPONDENCE
calcaneus in an 18-year-old male patient, who presented with swelling of the left ankle for 2 years. The differential diagnosis includes epithelioid chondroblastoma, mixed tumour/myoepithelioma, clear cell chondrosarcoma, synovial sarcoma and metastatic carcinoma. Epithelioid differentiation of chondroblastoma has been reported in the rare cases that occur in the temporal bone and other bones of the skull.8,9 Areas of epithelioid differentiation, typically with more closely packed chondroblasts with eosinophilic cytoplasm, is complicated by the expression of cytokeratin and EMA.10 In general, cytokeratin and EMA expression by chondroblastoma will be in the minority of cells. It is important that there is no ameloblastic epithelium differentiation in epithelioid chondroblastoma. Mixed tumour/myoepithelioma is composed of varying proportions of uniform-appearing, epithelioid cells with eosinophilic to clear cytoplasm, arranged in nests, cords, and ductules and/or spindle cells embedded in a hyalinised to chondromyxoid matrix. Divergent differentiation, including squamous, adipocytic, and bone and cartilaginous metaplasia, may be observed. Myoepithelioma typically lacks a definite ductal component. The histopathological and immunohistochemical findings of mixed tumour/myoepithelioma are similar to our present case. However, clear cut histological evidence of chondroblastoma with grooved and vesicular nuclei in our case may help render a correct interpretation. Furthermore, primary mixed tumour/myoepithelioma of the bone is extremely rare.11 Clear cell chondrosarcoma may be confused with chondroblastoma. However, clear cell chondrosarcoma tends to occur in most patients between 25 and 50 years of age. The tumour consists of large cells with abundant clear or pale cytoplasm and atypical nuclei, which are absent in chondroblastoma. Synovial sarcoma, which is a biphasic or monophasic tumour, should be differentiated from our current case. There is no definite chondroblastoma component in synovial sarcoma. Absence of cellular atypia, mitotic activity, infiltrative growth and presence of chondroblastoma is easy to distinguish from metastatic carcinoma. Treatment of chondroblastoma is determined by the anatomical location and degree of bone and/or joint involvement. The most accepted treatment is curettage and bone grafting. Adamantinoma is a low grade malignant tumour and known to be resistant to both radio- and chemotherapy. Szendroi et al. reported that wide segment resection gives good results in primary adamantinoma, and also in recurrences.3 In our case, the patient was treated by curettage and bone grafting. The postoperative course was uneventful, and the patient was alive without sign of recurrence at 6 month follow-up. The recurrence rate of adamantinoma is reported in the literature to be as high as 30–35% and they can recur even after 10–20 years following recognition of the tumour.12 Therefore, a life-long follow-up of the patient is necessary. Dawei Liu* Ping Xiao* Zhenhua Gao{ Ling Xue* Liantang Wang* Yongdong Liu* Anjia Han* Departments of *Pathology, and {Radiology, the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
Contact Dr A. Han. E-mail: [email protected] 1. Guedes A, Barreto B, Soares Barreto BL, Athanazio DA, Athanazio PR. Calcaneal chondroblastoma with secondary aneurysmal bone cyst: a case report. J Foot Ankle Surg 2010; 49: 298. 2. Davila JA, Amrami KK, Sundaram M, Adkins MC, Unni KK. Chondroblastoma of the hands and feet. Skeletal Radiol 2004; 33: 582–7. 3. Szendroi M, Antal I, Arato G. Adamantinoma of long bones: a long-term follow-up study of 11 cases. Pathol Oncol Res 2009; 15: 209–16. 4. Papagelopoulos PJ, Mavrogenis AF, Galanis EC, Savvidou OD, Inwards CY, Sim FH. Clinicopathological features, diagnosis, and treatment of adamantinoma of the long bones. Orthopedics 2007; 30: 211–5. 216–7. 5. Chandrasekar CR, Mohammed R, Rafalla AA, Grimer RJ. Adamantinoma of the calcaneum–a case report. Foot (Edinb) 2009; 19: 58–61. 6. Dahlin DC. Adamantinoma of long bones. In: Charles CT, editor. Bone Tumors. General Aspects and Data on 6221 Cases. Springfield: LippincottRaven, 1978; 296–306. 7. Schajowicz F. Tumors and Tumor Like Lesions of Bones and Joints. New York: Springer, 1981; 384–91. 8. Deyrup AT, Montag AG. Epithelioid and epithelial neoplasms of bone. Arch Pathol Lab Med 2007; 131: 205–16. 9. Bertoni F, Unni KK, Beabout JW, Harner SG, Dahlin DC. Chondroblastoma of the skull and facial bones. Am J Clin Pathol 1987; 88: 1–9. 10. Semmelink HJ, Pruszczynski M, Wiersma-van TA, Smedts F, Ramaekers FC. Cytokeratin expression in chondroblastomas. Histopathology 1990; 16: 257–63. 11. Jain VK, Jain D, Jawed A, Prasad A, Sachdev N, Kumar S. Primary myoepithelioma of bone. Pathology 2010; 42: 190–3. 12. Jundt G, Remberger K, Roessner A, Schulz A, Bohndorf K. Adamantinoma of long bones. A histopathological and immunohistochemical study of 23 cases. Pathol Res Pract 1995; 191: 112–20.
DOI: 10.1097/PAT.0b013e328342747a
Composite haemangioendothelioma: report of four cases with emphasis on atypical clinical presentation Sir, To date, there have been approximately 20 cases of composite haemangioendothelioma (HE) reported.1,2 Owing to its rarity and complex morphological variations, composite HE continues to pose diagnostic challenges in the distinction from other mimicking vascular tumours, particularly for those occurring in atypical clinical settings. To further broaden the clinicopathological and immunohistochemical spectrum of composite HE, we describe four new cases, including two occurring in the hitherto unreported hypopharynx and one in a patient with neurofibromatosis type 2 (NF2). Composite HE was first designated by Nayler et al. in 2000 as one of the rarest borderline vascular tumours with an admixture of concurrent benign (e.g., spindle cell haemangiomas), intermediate (e.g., retiform HE), and malignant (e.g., angiosarcoma-like) vascular lesions of various types.2 Retiform and epithelioid HEs represent the two most common histological types of composite HE.1,2 However, the variations in composition of vascular tumour types appear not correlated with clinical outcomes. Local relapses are not uncommon in composite HE and tend to occur several years after tumour resection, while the potential of distal and regional nodal metastases is considered very low.1–3 Clinically, composite HE preferentially manifests as a dermal and/or subcutaneous lesion in the distal extremities of young to middle-aged adults with a slow evolution.1–3 However, there were indeed a handful of previously published congenital composite HEs that had been noted since birth with longstanding presentation before
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.