- Email: [email protected]
An unusually large pulmonary sclerosing haemangioma
CORRESPONDENCE
5. Kuwano H, Iwashita A, Enjoji M. Pseudosarcomatous carcinoma of the anal canal. Dis Colon Rectum 1983; 26: 123–8. 6. Di Vizio D, Insabato L, Conzo G, Zafonte B, Ferrara G, Pettinato G. Sarcomatoid carcinoma of the colon: a case report with literature review. Tumori 2001; 87: 431–5. 7. Roncaroli F, Montironi R, Feliciotti F, Losi L, Eusubi V. Sarcomatoid carcinoma of the anorectal junction with neuroendocrine and rhabdomyoblastic features. Am J Surg Pathol 1995; 19: 217–23. 8. Kench JG, Frommer DJ. Sarcomatoid carcinoma of the ampulla of Vater. Pathology 1997; 29: 89–91. 9. Iezzoni JC, Mills SE. Sarcomatoid carcinomas (carcinosarcomas) of the gastrointestinal tract: a review. Semin Diagn Pathol 1993; 10: 176–87. 10. Kalogeropoulos N, Antonakopoulos G, Agapitos M, Papacharalampous N. Spindle cell carcinoma (pseudosarcoma) of the anus: a light, electron microscopic and immunocytochemical study of a case. Histopathology 1985; 9: 987–94.
267
cellular material in sheet-like and papilliform arrangements, but a definite diagnosis was not reached. At thoracotomy, an 11-cm mass was found replacing the left lower lobe, and a left lower lobectomy was performed. The post-operative recovery was uneventful, and he remains well 5 years later. The solitary mass in the left lower lobe was large and spherical, measuring 11 cm in greatest diameter. The mass was well-circumscribed, but not obviously encapsulated, and had a variegated cut surface with a mottled yellow-white appearance and areas of calcification and haemorrhage (Fig. 2). Microscopic examination revealed varying histological appearances, with sclerotic, haemorrhagic and papillary patterns (Fig. 3). A population of
DOI: 10.1080/00313020600699185
An unusually large pulmonary sclerosing haemangioma Sir, Sclerosing haemangioma of the lung (SHL) is a rare, benign tumour, first described by Liebow and Hubbell in 1956.1 Initially regarded as being of endothelial origin, the histogenesis of SHL has remained somewhat controversial, though recent immunohistochemical and ultrastructural studies have demonstrated epithelial characteristics.2–5 SHL is a well-defined clinicopathological entity, which typically presents as a well-circumscribed round or oval mass, averaging 2.8 cm in size.1,5,6 An exceptionally large example of this rare benign neoplasm occurring in a young adult male of Asian descent is described in this report. A 24-year-old man presented with symptoms related to a prolonged upper respiratory illness including cough for approximately 8 weeks and two episodes of haemoptysis. A large left lower lobe mass was demonstrated on chest Xray. A computed tomography (CT) scan revealed the mass was solid with areas of calcification (Fig. 1). No enlarged mediastinal lymph nodes, hepatic metastases or mediastinal invasion were evident on the CT. Fine needle aspiration cytology performed under CT guidance yielded epithelial
Fig. 1 CT scan demonstrated a large, solid pulmonary mass with scattered calcification.
Fig. 2 Macroscopic appearances of the excision specimen. A small rim of lung tissue surrounds the well-circumscribed and variegated mass.
Fig. 3 Microscopic appearance of the tumour, demonstrating one of the various patterns which characterise sclerosing haemangioma of the lung (H&E, 640).
268
CORRESPONDENCE
mildly pleomorphic cells characterised by clear or vacuolated cytoplasm formed small solid aggregates in the stroma. A similar population of cuboidal cells lined the papillary structures. Solid areas contained large foci of sclerosis, cholesterol cleft formation, areas of dystrophic calcification, and scattered mononuclear inflammatory cell infiltrates. Blood-filled cystic spaces and large foamy histiocytes were also a feature. Immunohistochemical studies revealed positive staining for cytokeratins, epithelial membrane antigen (EMA), thyroid transcription factor (TTF-1) and carcinoembryonic antigen (CEA) in the lesional cells lining papillary structures and forming small solid aggregates. CD31, CD34, factor VIII and S100 protein were negative. SHL is a well-defined clinicopathological entity, which must be distinguished from a number of other neoplastic and inflammatory pulmonary processes. SHL may exhibit a variety of microscopic patterns,2 and this diversity of appearances, together with its relative rarity, frequently lead to diagnostic difficulty in fine needle aspiration, biopsy, intra-operative frozen section, and resection specimens.7,8 The microscopic differential diagnosis is broad, and is determined by the extent and presence of the various histological patterns. In different settings, SHL may need to be distinguished from entities as diverse as bronchioalveolar carcinoma, epithelioid haemangioendothelioma, carcinoid tumour, alveolar adenoma, papillary adenocarcinoma and mesothelioma. The diagnosis may be particularly difficult when there is evidence of cytological atypia, or if one particular microscopic pattern predominates.7 In this case the diagnosis of SHL was based upon the diversity of microscopic appearances, which included the characteristic solid, haemorrhagic, papillary and sclerotic patterns, together with the macroscopic and microscopic evidence of circumscription. The immunohistochemical profile of positivity for cytokeratins, EMA, CEA, TTF-1 and the absence of staining for CD31, CD34, factor VIII and S100 protein confirm the diagnosis. SHL occurs more frequently in women, and is often asymptomatic, frequently being detected as a small ‘coin lesion’ on chest X-ray. Symptomatic lesions may present with cough or haemoptysis. The majority are small, approximately three-quarters measure less than 4 cm.1,5,6 Surgical resection is curative. Metastatic spread of SHL may occur, but is rare, and does not appear to affect the otherwise excellent prognosis.9 Initially regarded as being of vascular origin, the histogenesis of SHL has remained somewhat controversial, though recent immunohistochemical and ultrastructural studies have demonstrated epithelial differentiation and, more specifically, have suggested an origin from primitive respiratory epithelium, incompletely differentiated type 2 pneumocytes or Clara cells.2–5 Two major populations of lesional cells in SHL are recognised.4,5,10 The first is characterised by polygonal cells with abundant pale cytoplasm which demonstrate an EMA-positive, cytokeratin-negative, TTF1-positive immunophenotype. This population forms solid sheets, and is found in cords within areas of sclerosis, the cores of papillae, and lining haemorrhagic angiomatoid spaces. The second population comprises cuboidal cells, which line papillary structures, and have an EMA-positive, cytokeratin-positive and TTF1-positive immunophenotype.4,5,11,12 Clonality studies of SHL have
Pathology (2006), 38(3), June
demonstrated that both cell populations demonstrate the same pattern of monoclonality, leading to the suggestion that both populations are derived from the same cell.10 It has been suggested that the papillary lining cells represent a combination of truly neoplastic cells, similar to those identified in solid areas, together with a second population of entrapped and metaplastic alveolar epithelium.5,12 In summary, we report an exceptionally large example of SHL, a rare pulmonary neoplasm. Peter Robbins* David Holthouse{ Mark Newman{ *Department of Tissue Pathology, PathCentre, Nedlands, and {Department of Cardiothoracic Surgery, Sir Charles Gairdner Hospital, Perth, Western Australia Contact Dr P. Robbins. E-mail: [email protected]
1. Liebow AA, Hubbell DS. Sclerosing haemangioma (histiocytoma, xanthoma) of the lung. Cancer 1956; 9: 53–75. 2. Nagata N, Dairaku M, Sueishi K, Tanaka K. Sclerosing hemangioma of the lung. An epithelial tumor composed of immunohistochemically heterogenous cells. Am J Clin Pathol 1987; 88: 552–9. 3. Yousem SA, Wick MR, Singh G, et al. So-called sclerosing hemangiomas of lung. An immunohistochemical study supporting a respiratory epithelial origin. Am J Surg Pathol 1988; 12: 582–90. 4. Chan AC, Chan JK. Pulmonary sclerosing hemangioma consistently expresses thyroid transcription factor-1 (TTF-1): a new clue to its histogenesis. Am J Surg Pathol 2000; 24: 1531–6. 5. Devouassoux-Shisheboran M, Hayashi T, Linnoila RI, Koss MN, Travis WD. A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with immunohistochemical studies: TTF-1 is expressed in both round and surface cells, suggesting an origin from primitive respiratory epithelium. Am J Surg Pathol 2000; 24: 906–16. 6. Katzenstein AL, Gmelich JT, Carrington CB. Sclerosing hemangioma of the lung: a clinicopathologic study of 51 cases. Am J Surg Pathol 1980; 4: 343–56. 7. Chan AC, Chan JK. Can pulmonary sclerosing haemangioma be accurately diagnosed by intra-operative frozen section? Histopathology 2002; 41: 392–403. 8. Nicholson AG, Magkou C, Snead D, et al. Unusual sclerosing haemangiomas and sclerosing haemangioma-like lesions, and the value of TTF-1 in making the diagnosis. Histopathology 2002; 41: 404–13. 9. Miyagawa-Hayashino A, Tazelaar HD, Langel DJ, Colby TV. Pulmonary sclerosing hemangioma with lymph node metastases: report of 4 cases. Arch Pathol Lab Med 2003; 127: 321–5. 10. Niho S, Suzuki K, Yokose T, Kodama T, Nishiwaki Y, Esumi H. Monoclonality of both pale cells and cuboidal cells of sclerosing hemangioma of the lung. Am J Pathol 1998; 152: 1065–9. 11. Rodriguez-Soto J, Colby TV, Rouse RV. A critical examination of the immunophenotype of pulmonary sclerosing hemangioma. Am J Surg Pathol 2000; 24: 442–50. 12. Illei PB, Rosai J, Klimstra DS. Expression of thyroid transcription factor-1 and other markers in sclerosing hemangioma of the lung. Arch Pathol Lab Med 2001; 125: 1335–9.
DOI: 10.1080/00313020600699623
Childhood tufted angioma Sir, Tufted angioma, also known as angioblastoma, is a rare and slow-growing benign cutaneous vascular tumour which presents mainly in early childhood. The lesions commonly occur in the trunk, neck or extremities. We describe a
5. Kuwano H, Iwashita A, Enjoji M. Pseudosarcomatous carcinoma of the anal canal. Dis Colon Rectum 1983; 26: 123–8. 6. Di Vizio D, Insabato L, Conzo G, Zafonte B, Ferrara G, Pettinato G. Sarcomatoid carcinoma of the colon: a case report with literature review. Tumori 2001; 87: 431–5. 7. Roncaroli F, Montironi R, Feliciotti F, Losi L, Eusubi V. Sarcomatoid carcinoma of the anorectal junction with neuroendocrine and rhabdomyoblastic features. Am J Surg Pathol 1995; 19: 217–23. 8. Kench JG, Frommer DJ. Sarcomatoid carcinoma of the ampulla of Vater. Pathology 1997; 29: 89–91. 9. Iezzoni JC, Mills SE. Sarcomatoid carcinomas (carcinosarcomas) of the gastrointestinal tract: a review. Semin Diagn Pathol 1993; 10: 176–87. 10. Kalogeropoulos N, Antonakopoulos G, Agapitos M, Papacharalampous N. Spindle cell carcinoma (pseudosarcoma) of the anus: a light, electron microscopic and immunocytochemical study of a case. Histopathology 1985; 9: 987–94.
267
cellular material in sheet-like and papilliform arrangements, but a definite diagnosis was not reached. At thoracotomy, an 11-cm mass was found replacing the left lower lobe, and a left lower lobectomy was performed. The post-operative recovery was uneventful, and he remains well 5 years later. The solitary mass in the left lower lobe was large and spherical, measuring 11 cm in greatest diameter. The mass was well-circumscribed, but not obviously encapsulated, and had a variegated cut surface with a mottled yellow-white appearance and areas of calcification and haemorrhage (Fig. 2). Microscopic examination revealed varying histological appearances, with sclerotic, haemorrhagic and papillary patterns (Fig. 3). A population of
DOI: 10.1080/00313020600699185
An unusually large pulmonary sclerosing haemangioma Sir, Sclerosing haemangioma of the lung (SHL) is a rare, benign tumour, first described by Liebow and Hubbell in 1956.1 Initially regarded as being of endothelial origin, the histogenesis of SHL has remained somewhat controversial, though recent immunohistochemical and ultrastructural studies have demonstrated epithelial characteristics.2–5 SHL is a well-defined clinicopathological entity, which typically presents as a well-circumscribed round or oval mass, averaging 2.8 cm in size.1,5,6 An exceptionally large example of this rare benign neoplasm occurring in a young adult male of Asian descent is described in this report. A 24-year-old man presented with symptoms related to a prolonged upper respiratory illness including cough for approximately 8 weeks and two episodes of haemoptysis. A large left lower lobe mass was demonstrated on chest Xray. A computed tomography (CT) scan revealed the mass was solid with areas of calcification (Fig. 1). No enlarged mediastinal lymph nodes, hepatic metastases or mediastinal invasion were evident on the CT. Fine needle aspiration cytology performed under CT guidance yielded epithelial
Fig. 1 CT scan demonstrated a large, solid pulmonary mass with scattered calcification.
Fig. 2 Macroscopic appearances of the excision specimen. A small rim of lung tissue surrounds the well-circumscribed and variegated mass.
Fig. 3 Microscopic appearance of the tumour, demonstrating one of the various patterns which characterise sclerosing haemangioma of the lung (H&E, 640).
268
CORRESPONDENCE
mildly pleomorphic cells characterised by clear or vacuolated cytoplasm formed small solid aggregates in the stroma. A similar population of cuboidal cells lined the papillary structures. Solid areas contained large foci of sclerosis, cholesterol cleft formation, areas of dystrophic calcification, and scattered mononuclear inflammatory cell infiltrates. Blood-filled cystic spaces and large foamy histiocytes were also a feature. Immunohistochemical studies revealed positive staining for cytokeratins, epithelial membrane antigen (EMA), thyroid transcription factor (TTF-1) and carcinoembryonic antigen (CEA) in the lesional cells lining papillary structures and forming small solid aggregates. CD31, CD34, factor VIII and S100 protein were negative. SHL is a well-defined clinicopathological entity, which must be distinguished from a number of other neoplastic and inflammatory pulmonary processes. SHL may exhibit a variety of microscopic patterns,2 and this diversity of appearances, together with its relative rarity, frequently lead to diagnostic difficulty in fine needle aspiration, biopsy, intra-operative frozen section, and resection specimens.7,8 The microscopic differential diagnosis is broad, and is determined by the extent and presence of the various histological patterns. In different settings, SHL may need to be distinguished from entities as diverse as bronchioalveolar carcinoma, epithelioid haemangioendothelioma, carcinoid tumour, alveolar adenoma, papillary adenocarcinoma and mesothelioma. The diagnosis may be particularly difficult when there is evidence of cytological atypia, or if one particular microscopic pattern predominates.7 In this case the diagnosis of SHL was based upon the diversity of microscopic appearances, which included the characteristic solid, haemorrhagic, papillary and sclerotic patterns, together with the macroscopic and microscopic evidence of circumscription. The immunohistochemical profile of positivity for cytokeratins, EMA, CEA, TTF-1 and the absence of staining for CD31, CD34, factor VIII and S100 protein confirm the diagnosis. SHL occurs more frequently in women, and is often asymptomatic, frequently being detected as a small ‘coin lesion’ on chest X-ray. Symptomatic lesions may present with cough or haemoptysis. The majority are small, approximately three-quarters measure less than 4 cm.1,5,6 Surgical resection is curative. Metastatic spread of SHL may occur, but is rare, and does not appear to affect the otherwise excellent prognosis.9 Initially regarded as being of vascular origin, the histogenesis of SHL has remained somewhat controversial, though recent immunohistochemical and ultrastructural studies have demonstrated epithelial differentiation and, more specifically, have suggested an origin from primitive respiratory epithelium, incompletely differentiated type 2 pneumocytes or Clara cells.2–5 Two major populations of lesional cells in SHL are recognised.4,5,10 The first is characterised by polygonal cells with abundant pale cytoplasm which demonstrate an EMA-positive, cytokeratin-negative, TTF1-positive immunophenotype. This population forms solid sheets, and is found in cords within areas of sclerosis, the cores of papillae, and lining haemorrhagic angiomatoid spaces. The second population comprises cuboidal cells, which line papillary structures, and have an EMA-positive, cytokeratin-positive and TTF1-positive immunophenotype.4,5,11,12 Clonality studies of SHL have
Pathology (2006), 38(3), June
demonstrated that both cell populations demonstrate the same pattern of monoclonality, leading to the suggestion that both populations are derived from the same cell.10 It has been suggested that the papillary lining cells represent a combination of truly neoplastic cells, similar to those identified in solid areas, together with a second population of entrapped and metaplastic alveolar epithelium.5,12 In summary, we report an exceptionally large example of SHL, a rare pulmonary neoplasm. Peter Robbins* David Holthouse{ Mark Newman{ *Department of Tissue Pathology, PathCentre, Nedlands, and {Department of Cardiothoracic Surgery, Sir Charles Gairdner Hospital, Perth, Western Australia Contact Dr P. Robbins. E-mail: [email protected]
1. Liebow AA, Hubbell DS. Sclerosing haemangioma (histiocytoma, xanthoma) of the lung. Cancer 1956; 9: 53–75. 2. Nagata N, Dairaku M, Sueishi K, Tanaka K. Sclerosing hemangioma of the lung. An epithelial tumor composed of immunohistochemically heterogenous cells. Am J Clin Pathol 1987; 88: 552–9. 3. Yousem SA, Wick MR, Singh G, et al. So-called sclerosing hemangiomas of lung. An immunohistochemical study supporting a respiratory epithelial origin. Am J Surg Pathol 1988; 12: 582–90. 4. Chan AC, Chan JK. Pulmonary sclerosing hemangioma consistently expresses thyroid transcription factor-1 (TTF-1): a new clue to its histogenesis. Am J Surg Pathol 2000; 24: 1531–6. 5. Devouassoux-Shisheboran M, Hayashi T, Linnoila RI, Koss MN, Travis WD. A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with immunohistochemical studies: TTF-1 is expressed in both round and surface cells, suggesting an origin from primitive respiratory epithelium. Am J Surg Pathol 2000; 24: 906–16. 6. Katzenstein AL, Gmelich JT, Carrington CB. Sclerosing hemangioma of the lung: a clinicopathologic study of 51 cases. Am J Surg Pathol 1980; 4: 343–56. 7. Chan AC, Chan JK. Can pulmonary sclerosing haemangioma be accurately diagnosed by intra-operative frozen section? Histopathology 2002; 41: 392–403. 8. Nicholson AG, Magkou C, Snead D, et al. Unusual sclerosing haemangiomas and sclerosing haemangioma-like lesions, and the value of TTF-1 in making the diagnosis. Histopathology 2002; 41: 404–13. 9. Miyagawa-Hayashino A, Tazelaar HD, Langel DJ, Colby TV. Pulmonary sclerosing hemangioma with lymph node metastases: report of 4 cases. Arch Pathol Lab Med 2003; 127: 321–5. 10. Niho S, Suzuki K, Yokose T, Kodama T, Nishiwaki Y, Esumi H. Monoclonality of both pale cells and cuboidal cells of sclerosing hemangioma of the lung. Am J Pathol 1998; 152: 1065–9. 11. Rodriguez-Soto J, Colby TV, Rouse RV. A critical examination of the immunophenotype of pulmonary sclerosing hemangioma. Am J Surg Pathol 2000; 24: 442–50. 12. Illei PB, Rosai J, Klimstra DS. Expression of thyroid transcription factor-1 and other markers in sclerosing hemangioma of the lung. Arch Pathol Lab Med 2001; 125: 1335–9.
DOI: 10.1080/00313020600699623
Childhood tufted angioma Sir, Tufted angioma, also known as angioblastoma, is a rare and slow-growing benign cutaneous vascular tumour which presents mainly in early childhood. The lesions commonly occur in the trunk, neck or extremities. We describe a