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Kaposiform haemangioendothelioma: case report and review of the literature
Kaposiform haemangioendothelioma
The Authors Hassan Shaaban MSc, FRCS, Specialist Registrar (LAT) in Plastic Surgery Ardeshir Bayat BSe, MRCS, Research Fellow in Plastic Surgery Peter Davenport FRCS, Consultant Plastic Surgeon Mamta Shah PhD, FRCS(Plast), Consultant Plastic Surgeon
163 Department of Plastic Surgery, Booth Hall Children's Hospital, Charleston Road, Blackley, Manchester M9 7AA, UK. Correspondence to Mr Hassan Sbaaban. Paper received 25 April 2001. Accepted 24 September 2001, after revision.
British Journal of Plastic Surgery (2002)
9 2002 The British Association of Plastic Surgeons doi: 10.1054/bjps.2001.3769
Kaposiform haemangioendothelioma: case report and review of the literature J. G. Cooper, S. L. Edwards* and J. D. Holmes
Departments of Plastic Surgery and *Pathology, Aberdeen Royal Infirmary, Aberdeen, UK SUMMARY. Kaposiform haemangioendothelioma (KHE) is a rare vascular tumour, predominantly of infancy and early childhood, that has a close association with Kasabach-Merritt syndrome. Despite benign histology, this tumour frequently behaves aggressively, causing significant morbidity and mortality as a result of the compression and invasion of surrounding structures as well as from associated haematological and lymphoproliferative syndromes. There is a need for a high index of suspicion when presented with large, enlarging or abnormal vascular lesions in infancy and, less commonly, in adulthood. An early diagnosis of KHE can lead to prompt treatment, which may be life saving. To date, there have been only four reported cases of KHE occurring in patients over the age of 18 years. We report an otherwise well 26-year-old woman who presented with a KHE of the left thigh, and briefly review the literature. We believe this to be the second reported case of KHE in the UK and the first in an adult patient. 9 2002 The British Association of Plastic Surgeons
Keywords: kaposiform haemangioendothelioma, vascular tumour, Kasabach-Merritt syndrome.
Kaposiform haemangioendothelioma (KHE) is a rare soft-tissue tumour of vascular origin that occurs predominantly in infancy and early childhood. Despite benign histology, KHE displays 'borderline malignant' behaviour, in that it is a locally proliferative and frequently aggressive vascular neoplasm involving skin, subcutis and deep soft tissues, including those of the retroperitoneum.l Distant metastatic spread has not been reported, although there has been one case of local perinodal involvement. 2 Associated lymphatic proliferation is common, and there is a strong association with KasabachMerritt syndrome. 1,3-5 There have been only four reported cases occurring in patients over the age of 18 years.l,6 We report a case of KHE in the left thigh of a 26-yearold female. We believe this to be the second reported case of KHE in the UK and the first in an adult patient]
Case report An otherwise healthy 26-year-old female presented to our unit with a slowly enlarging vascular lesion on the lateral aspect of her left thigh, which was becoming tender, nodular and uncomfortable. The lesion had been present since birth and had been biopsied in infancy, leading to a diagnosis of benign angioma. There was no history of thrombocytopenia or other bleeding diathesis. The lesion, which measured 10cm • 15 cm, was dark red in colour and well circumscribed with an indurated and nodular surface (Fig. 1). Presented in part at the Scottish and Irish Plastic Surgery Meeting held in The Ulster Hospital, Belfast, 28 October 2000.
Figure 1--The 10cm x 15cm dark-red vascular lesion on the patient's left thigh, with a definite edge and an indurated nodular surface.
164 Clinically, this was thought to be a malignant soft-tissue turnout. Fine needle aspiration cytology proved unhelpful, and, therefore, an incisional biopsy was performed to obtain a definitive diagnosis. Histology demonstrated an unusual vascular lesion extending throughout the dermis and into the subcutaneous fat. The vascular patterns varied throughout the lesion from areas
British Journal of Plastic Surgery of tightly packed capillaries to slit-like and sinusoidal spaces lined with mature endothelial cells and a large number of spindle cells (Fig. 2). These features pointed to a diagnosis of KHE. A review of the literature suggested that excision, if feasible, offered the best chance of cure/The lesion was further delineated by MRI scanning, which demonstrated that the tumour was confined to the skin and subcutaneous tissues (Fig. 3). Subsequently, surgery in the form of wide local excision down to the deep fascia and application of a split-thickness skin graft was undertaken.
Discussion
Figure 2--High power demonstrates the variable pattern of vasculature, including tightly packed capillaries (arrow head), slit-like spaces (thin arrow) and sinusoids (thick arrow).
Figure 3--MRI scan demonstrating a soft-tissue tumour in the left thigh involving the skin and subcutaneous tissues to a maximum depth of 2.5 cm (arrow head).
KHE has clinical and histological similarities to benign haemangioma and Kaposi's sarcoma. 1 Approximately 40 biopsy-proven or probable cases of KHE have been reported, 1'3-6'8'9 the majority occurring in infancy and early childhood. Only four cases have been described in adults. 1,6 Kasabach and Merritt first described the association of a large vascular tumour and a bleeding diathesis in 1940.1~ Recent research suggests that Kasabach-Merritt syndrome does not complicate true haemangiomata, but that KHE is frequently the culprit. 3'4 In infants KHE is often a disease of the retroperitoneum, with a dismal prognosis owing to local compression and infiltration of vital structures and a high incidence of Kasabach-Merritt syndrome with its consequent haemorrhagic complications, s KHE occurring in more peripheral sites can still be complicated by both Kasabach-Merritt syndrome 3-s and lymphatic proliferation.l,4 All of the four cases previously reported in adults were of the cutaneous variant, and none showed evidence of Kasabach-Merritt syndrome or of preceding immunosuppression. 1'6 Our patient is no exception. Cutaneous KHE adopts a characteristic violet colour and typically grows in an aggressive infiltrative manner involving the overlying skin. The presence of telangiectasia within the lesion itself or any associated lymphatic proliferation should raise suspicion of KMS) The diagnosis is often made only on histopathological and immunohistochemical grounds, and the differential diagnosis primarily includes Kaposi's sarcoma 1'6'~1-~3 and benign haemangioma, 11 but also includes angiosarcoma, 11 spindle-cell haemangioendothelioma 6 and tufted angioma. Tufted angioma shares many features with KHE, including an association with Kasabach-Merritt syndrome, 4 and may represent part of a continuum of rare vascular tumours. 4,5 MRI has been advocated as the best tool for further imaging of KHE, and characteristic features have been documented that differentiate KHE from other vascular tumours. 3 Treatment should be by wide local excision of the tumour where possibleJ Systemic treatment should be directed towards attributable systemic disease and lesions not amenable to resection. Since KHE is rare, no set protocol for systemic treatment has been established. Varying degrees of therapeutic success have been reported with interferon 2-alpha, ticlopidine hydrochloride plus aspirin, chemotherapy, corticosteroids, radiotherapy and embolisation used alone or in combination. 3'4
Kaposiform haemangioendothelioma T h o u g h K H E is rare, it is a tumour that plastic surgeons and dermatologists could well encounter in both children and adults. Frequently, the presentation of a vascular t u m o u r poses no diagnostic or therapeutic problem, but a healthy index o f suspicion is required with new, unusual or enlarging vascular tumours, and there should be a low threshold for biopsy o f these lesions. K n o w l e d g e o f K H E and its associated complications should lead to a p r o m p t diagnosis and early initiation o f treatment, w h i c h m a y reduce residual t u m o u r and sequelae. 5
Acknowledgements We acknowledge the help of Professor C. D. M. Fletcher, Professor of Pathology, Harvard Medical School, in making the diagnosis. We are also grateful to the Department of Medical Illustration, Aberdeen Royal Infirmary, for their help with the preparation of the photographs in this paper.
References 1. Zukerberg LR, Nickoloff BJ, Weiss SW. Kaposiform hemangioendothelioma of infancy and childhood: an aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol 1993; 17: 321-8. 2. Lai FM-M, Allen PW, Yuen PM, Leung PC. Locally metastasizing vascular tumor: spindle cell, epithelioid, or unclassified hemangioendothelioma? Am J Clin Pathol 1991; 96: 660-3. 3. Sarkar M, Mulliken JB, Kozakewich HPW, Robertson RL, Burrows PE. Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with kaposiform hemangioendothelioma and not with common infantile hemangioma. Plast Reconstr Surg 1997; 100: 1377-86. 4. Enjolras O, Wassef M, Mazoyer E, et al. Infants with KasabachMerritt syndrome do not have 'true' hemangiomas. J Pediatr 1997; 130: 631-40. 5. Enjolras O, Mulliken JB, Wassef M, et al. Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol 2000; 42: 225-35.
165 6. Mentzel T, Mazzoleni G, Deitos AP, Fletcher CDM. Kaposiform hemangioendothelioma in adults: clinicopathologic and immunohistochemical analysis of three cases. Am J Clin Pathol 1997; 108: 450-5. 7. McPartlin DW, Ghufoor K, Patel SK, Jayaraj S. A rare case of cutaneous kaposiform haemangioendothelioma. Int J Clin Practice 1999; 53: 562-3. 8, Vin-Christian K, McCalmont TH, Frieden IJ. Kaposiform hemangioendothelioma: an aggressive, locally invasive vascular tumor that can mimic hemangioma of infancy. Arch Dermatol 1997; 133: 1573-8. 9. Beaubien ER, Ball NJ, Storwick GS. Kaposiform hemangioendothelioma: a locally aggressive vascular tumor. J Am Acad Dermatol 1998; 38: 799-802. 10. Kasabach HH, Merritt KK. Capillary hemangioma with extensive purpura: report of a case. Am J Dis Child 1940; 59: 1063-70. 11. Tsang WYW, Chan JKC. Kaposi-like infantile hemangioendothelioma: a distinctive vascular neoplasm of the retroperitoneum. Am J Surg Pathol 1991; 15: 982-9. 12. Fukunaga M, Ushigome S, Ishikawa E. Kaposiform haemangioendothelioma associated with Kasabach-Merritt syndrome. Histopathology 1996; 28: 281--4. 13. Zhang Y-M, Bachmann S, Hemmer C, et al. Vascular origin of Kaposi's sarcoma: expression of leukocyte adhesion molecule- 1, thrombomodulin, and tissue factor. Am J Pathol 1994; 144: 51-9.
The Authors Jamie G. Cooper, SHO in Plastic Surgery John D. Holmes MChir, FRCS(Plast), Consultant Plastic and Reconstructive Surgeon Department of Plastic Surgery,
Sharon L. Edwards, Registrar in Pathology Department of Pathology, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB25 2ZN, UK. Correspondence to Mr Jamie G. Cooper. Paper received 16 February 2001. Accepted 5 December 2001, after revision.
British Journal of Plastic Surgery (2002) 9 2002 The British Association of Plastic Surgeons doi: l 0.1054/bjps.2001.3782
Subungual melanoma in situ: two independent streaks in one nail bed J. H. W. Clarkson, R. M. R. McAllister, S. H. Cliff* and B. Powell
Plastic Surgical Unit, St Georges Hospital London; and *East Surrey Hospital UK SUMMARY.
W e report an unusual case o f two nail-bed streaks under one nail, revealing m e l a n o m a in situ affecting the nail bed and plate, but not the nail fold. This unusual m o d e of presentation together with the absence o f any nailfold i n v o l v e m e n t has not been reported previously. W e highlight the need for early histological assessment o f nail-bed lesions and discuss subungual m e l a n o m a . 9 2002 T h e British Association o f Plastic S u r g e o n s
Case report We report an unusual case of two independent melanocytic streaks discovered under the left thumbnail of a 35-year-old Caucasian male. Both transpired to be melanoma in situ. The patient presented with a history of two brown streaks running down the nail of his left thumb. He had noticed the first
3 years before, and it was joined by the second after about a year. Initially it was diagnosed by his General Practitioner as a fungal lesion and treated accordingly. The streaks gradually became increasingly pigmented, leading him to present to a dermatology clinic in some concern. On examination, two distinct parallel pigmented streaks were observed, running the full length of the nail bed, extending
The Authors Hassan Shaaban MSc, FRCS, Specialist Registrar (LAT) in Plastic Surgery Ardeshir Bayat BSe, MRCS, Research Fellow in Plastic Surgery Peter Davenport FRCS, Consultant Plastic Surgeon Mamta Shah PhD, FRCS(Plast), Consultant Plastic Surgeon
163 Department of Plastic Surgery, Booth Hall Children's Hospital, Charleston Road, Blackley, Manchester M9 7AA, UK. Correspondence to Mr Hassan Sbaaban. Paper received 25 April 2001. Accepted 24 September 2001, after revision.
British Journal of Plastic Surgery (2002)
9 2002 The British Association of Plastic Surgeons doi: 10.1054/bjps.2001.3769
Kaposiform haemangioendothelioma: case report and review of the literature J. G. Cooper, S. L. Edwards* and J. D. Holmes
Departments of Plastic Surgery and *Pathology, Aberdeen Royal Infirmary, Aberdeen, UK SUMMARY. Kaposiform haemangioendothelioma (KHE) is a rare vascular tumour, predominantly of infancy and early childhood, that has a close association with Kasabach-Merritt syndrome. Despite benign histology, this tumour frequently behaves aggressively, causing significant morbidity and mortality as a result of the compression and invasion of surrounding structures as well as from associated haematological and lymphoproliferative syndromes. There is a need for a high index of suspicion when presented with large, enlarging or abnormal vascular lesions in infancy and, less commonly, in adulthood. An early diagnosis of KHE can lead to prompt treatment, which may be life saving. To date, there have been only four reported cases of KHE occurring in patients over the age of 18 years. We report an otherwise well 26-year-old woman who presented with a KHE of the left thigh, and briefly review the literature. We believe this to be the second reported case of KHE in the UK and the first in an adult patient. 9 2002 The British Association of Plastic Surgeons
Keywords: kaposiform haemangioendothelioma, vascular tumour, Kasabach-Merritt syndrome.
Kaposiform haemangioendothelioma (KHE) is a rare soft-tissue tumour of vascular origin that occurs predominantly in infancy and early childhood. Despite benign histology, KHE displays 'borderline malignant' behaviour, in that it is a locally proliferative and frequently aggressive vascular neoplasm involving skin, subcutis and deep soft tissues, including those of the retroperitoneum.l Distant metastatic spread has not been reported, although there has been one case of local perinodal involvement. 2 Associated lymphatic proliferation is common, and there is a strong association with KasabachMerritt syndrome. 1,3-5 There have been only four reported cases occurring in patients over the age of 18 years.l,6 We report a case of KHE in the left thigh of a 26-yearold female. We believe this to be the second reported case of KHE in the UK and the first in an adult patient]
Case report An otherwise healthy 26-year-old female presented to our unit with a slowly enlarging vascular lesion on the lateral aspect of her left thigh, which was becoming tender, nodular and uncomfortable. The lesion had been present since birth and had been biopsied in infancy, leading to a diagnosis of benign angioma. There was no history of thrombocytopenia or other bleeding diathesis. The lesion, which measured 10cm • 15 cm, was dark red in colour and well circumscribed with an indurated and nodular surface (Fig. 1). Presented in part at the Scottish and Irish Plastic Surgery Meeting held in The Ulster Hospital, Belfast, 28 October 2000.
Figure 1--The 10cm x 15cm dark-red vascular lesion on the patient's left thigh, with a definite edge and an indurated nodular surface.
164 Clinically, this was thought to be a malignant soft-tissue turnout. Fine needle aspiration cytology proved unhelpful, and, therefore, an incisional biopsy was performed to obtain a definitive diagnosis. Histology demonstrated an unusual vascular lesion extending throughout the dermis and into the subcutaneous fat. The vascular patterns varied throughout the lesion from areas
British Journal of Plastic Surgery of tightly packed capillaries to slit-like and sinusoidal spaces lined with mature endothelial cells and a large number of spindle cells (Fig. 2). These features pointed to a diagnosis of KHE. A review of the literature suggested that excision, if feasible, offered the best chance of cure/The lesion was further delineated by MRI scanning, which demonstrated that the tumour was confined to the skin and subcutaneous tissues (Fig. 3). Subsequently, surgery in the form of wide local excision down to the deep fascia and application of a split-thickness skin graft was undertaken.
Discussion
Figure 2--High power demonstrates the variable pattern of vasculature, including tightly packed capillaries (arrow head), slit-like spaces (thin arrow) and sinusoids (thick arrow).
Figure 3--MRI scan demonstrating a soft-tissue tumour in the left thigh involving the skin and subcutaneous tissues to a maximum depth of 2.5 cm (arrow head).
KHE has clinical and histological similarities to benign haemangioma and Kaposi's sarcoma. 1 Approximately 40 biopsy-proven or probable cases of KHE have been reported, 1'3-6'8'9 the majority occurring in infancy and early childhood. Only four cases have been described in adults. 1,6 Kasabach and Merritt first described the association of a large vascular tumour and a bleeding diathesis in 1940.1~ Recent research suggests that Kasabach-Merritt syndrome does not complicate true haemangiomata, but that KHE is frequently the culprit. 3'4 In infants KHE is often a disease of the retroperitoneum, with a dismal prognosis owing to local compression and infiltration of vital structures and a high incidence of Kasabach-Merritt syndrome with its consequent haemorrhagic complications, s KHE occurring in more peripheral sites can still be complicated by both Kasabach-Merritt syndrome 3-s and lymphatic proliferation.l,4 All of the four cases previously reported in adults were of the cutaneous variant, and none showed evidence of Kasabach-Merritt syndrome or of preceding immunosuppression. 1'6 Our patient is no exception. Cutaneous KHE adopts a characteristic violet colour and typically grows in an aggressive infiltrative manner involving the overlying skin. The presence of telangiectasia within the lesion itself or any associated lymphatic proliferation should raise suspicion of KMS) The diagnosis is often made only on histopathological and immunohistochemical grounds, and the differential diagnosis primarily includes Kaposi's sarcoma 1'6'~1-~3 and benign haemangioma, 11 but also includes angiosarcoma, 11 spindle-cell haemangioendothelioma 6 and tufted angioma. Tufted angioma shares many features with KHE, including an association with Kasabach-Merritt syndrome, 4 and may represent part of a continuum of rare vascular tumours. 4,5 MRI has been advocated as the best tool for further imaging of KHE, and characteristic features have been documented that differentiate KHE from other vascular tumours. 3 Treatment should be by wide local excision of the tumour where possibleJ Systemic treatment should be directed towards attributable systemic disease and lesions not amenable to resection. Since KHE is rare, no set protocol for systemic treatment has been established. Varying degrees of therapeutic success have been reported with interferon 2-alpha, ticlopidine hydrochloride plus aspirin, chemotherapy, corticosteroids, radiotherapy and embolisation used alone or in combination. 3'4
Kaposiform haemangioendothelioma T h o u g h K H E is rare, it is a tumour that plastic surgeons and dermatologists could well encounter in both children and adults. Frequently, the presentation of a vascular t u m o u r poses no diagnostic or therapeutic problem, but a healthy index o f suspicion is required with new, unusual or enlarging vascular tumours, and there should be a low threshold for biopsy o f these lesions. K n o w l e d g e o f K H E and its associated complications should lead to a p r o m p t diagnosis and early initiation o f treatment, w h i c h m a y reduce residual t u m o u r and sequelae. 5
Acknowledgements We acknowledge the help of Professor C. D. M. Fletcher, Professor of Pathology, Harvard Medical School, in making the diagnosis. We are also grateful to the Department of Medical Illustration, Aberdeen Royal Infirmary, for their help with the preparation of the photographs in this paper.
References 1. Zukerberg LR, Nickoloff BJ, Weiss SW. Kaposiform hemangioendothelioma of infancy and childhood: an aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol 1993; 17: 321-8. 2. Lai FM-M, Allen PW, Yuen PM, Leung PC. Locally metastasizing vascular tumor: spindle cell, epithelioid, or unclassified hemangioendothelioma? Am J Clin Pathol 1991; 96: 660-3. 3. Sarkar M, Mulliken JB, Kozakewich HPW, Robertson RL, Burrows PE. Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with kaposiform hemangioendothelioma and not with common infantile hemangioma. Plast Reconstr Surg 1997; 100: 1377-86. 4. Enjolras O, Wassef M, Mazoyer E, et al. Infants with KasabachMerritt syndrome do not have 'true' hemangiomas. J Pediatr 1997; 130: 631-40. 5. Enjolras O, Mulliken JB, Wassef M, et al. Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol 2000; 42: 225-35.
165 6. Mentzel T, Mazzoleni G, Deitos AP, Fletcher CDM. Kaposiform hemangioendothelioma in adults: clinicopathologic and immunohistochemical analysis of three cases. Am J Clin Pathol 1997; 108: 450-5. 7. McPartlin DW, Ghufoor K, Patel SK, Jayaraj S. A rare case of cutaneous kaposiform haemangioendothelioma. Int J Clin Practice 1999; 53: 562-3. 8, Vin-Christian K, McCalmont TH, Frieden IJ. Kaposiform hemangioendothelioma: an aggressive, locally invasive vascular tumor that can mimic hemangioma of infancy. Arch Dermatol 1997; 133: 1573-8. 9. Beaubien ER, Ball NJ, Storwick GS. Kaposiform hemangioendothelioma: a locally aggressive vascular tumor. J Am Acad Dermatol 1998; 38: 799-802. 10. Kasabach HH, Merritt KK. Capillary hemangioma with extensive purpura: report of a case. Am J Dis Child 1940; 59: 1063-70. 11. Tsang WYW, Chan JKC. Kaposi-like infantile hemangioendothelioma: a distinctive vascular neoplasm of the retroperitoneum. Am J Surg Pathol 1991; 15: 982-9. 12. Fukunaga M, Ushigome S, Ishikawa E. Kaposiform haemangioendothelioma associated with Kasabach-Merritt syndrome. Histopathology 1996; 28: 281--4. 13. Zhang Y-M, Bachmann S, Hemmer C, et al. Vascular origin of Kaposi's sarcoma: expression of leukocyte adhesion molecule- 1, thrombomodulin, and tissue factor. Am J Pathol 1994; 144: 51-9.
The Authors Jamie G. Cooper, SHO in Plastic Surgery John D. Holmes MChir, FRCS(Plast), Consultant Plastic and Reconstructive Surgeon Department of Plastic Surgery,
Sharon L. Edwards, Registrar in Pathology Department of Pathology, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB25 2ZN, UK. Correspondence to Mr Jamie G. Cooper. Paper received 16 February 2001. Accepted 5 December 2001, after revision.
British Journal of Plastic Surgery (2002) 9 2002 The British Association of Plastic Surgeons doi: l 0.1054/bjps.2001.3782
Subungual melanoma in situ: two independent streaks in one nail bed J. H. W. Clarkson, R. M. R. McAllister, S. H. Cliff* and B. Powell
Plastic Surgical Unit, St Georges Hospital London; and *East Surrey Hospital UK SUMMARY.
W e report an unusual case o f two nail-bed streaks under one nail, revealing m e l a n o m a in situ affecting the nail bed and plate, but not the nail fold. This unusual m o d e of presentation together with the absence o f any nailfold i n v o l v e m e n t has not been reported previously. W e highlight the need for early histological assessment o f nail-bed lesions and discuss subungual m e l a n o m a . 9 2002 T h e British Association o f Plastic S u r g e o n s
Case report We report an unusual case of two independent melanocytic streaks discovered under the left thumbnail of a 35-year-old Caucasian male. Both transpired to be melanoma in situ. The patient presented with a history of two brown streaks running down the nail of his left thumb. He had noticed the first
3 years before, and it was joined by the second after about a year. Initially it was diagnosed by his General Practitioner as a fungal lesion and treated accordingly. The streaks gradually became increasingly pigmented, leading him to present to a dermatology clinic in some concern. On examination, two distinct parallel pigmented streaks were observed, running the full length of the nail bed, extending