PATHOLOGY OF VASCULAR SKIN LESIONS - PART 6 doc

156 Sangüeza and Requena / Pathology of Vascular Skin Lesions References 1. Biberstein HH, Jessner M. A cirsoid aneurysm of the skin. Dermatologica 1956;113:129–41. 2. Girard C, Graham JH, Johnson WC. Arteriovenous hemangioma (arteriovenous shunt). J Cutan Pathol 1974;1:73–87. 3. Rosen T. Arteriovenous hemangioma. Cutis 1979;24:57–9. 4. Carapeto FJ, Garcia-Pérez A, Winkelmann RK. Acral arteriovenous tumor. Acta Derm Venereol 1977;57:155–8. 5. Carapeto FJ, Armijo M. Tumeur acrale arterio-veineuse. Ann Dermatol Venereol 1978;105:977–9. 6. Persson JR. Acral arteriovenous tumor. Acta Derm Venereol 1978;58:95. 7. Connelly MG, Winkelmann RK. Acral arteriovenous tumor: a clinicopathologic review. Am J Surg Pathol 1985;9:15–21. 8. Neumann RA, Knobler M, Schuller Petrovic S, et al. Giant arteriovenous hemangioma (cirsoid aneu- rysm) of the nose. J Dermatol Surg Oncol 1989;15:739–42. 9. Koutlas IG, Jessurum J. Arteriovenous hemangioma: a clinicopathological and immunohistochemical study. J Cutan Pathol 1994;21:343–9. 10. Lazarou G, Goldberg MI. Vulvar arteriovenous hemangioma. A case report. J Reprod Med 2000;45:439–41. 11. Calzavara Pinton P, Carlino A, Manganoni AM, Donzelli C, Faccheti F. Sindrome del nevo epidermico con malformazioni e amartomi vascolari multipli. G Ital Dermatol Venereol 1990;125:251–4. 12. Akiyama M, Inamoto N. Arteriovenous hemangiomas on the foreheads of patients with chronic liver disease. Arch Intern Med 1998;158:1469. 13. Satomi H, Imakado S, Ichikawa E, Fujisawa H, Otsuka F. A case of arteriovenous hemangioma asso- ciated with liver cirrhosis. Dermatology 1999;199:278. 14. Akiyama M, Inamoto N. Arteriovenous haemangioma in chronic liver disease: clinical and histopatho- logic features of four cases. Br J Dermatol 2001;144:604–9. 15. Kutzner H, Winzer M, Mentzel T. Symplastisches Hamangiom. Hautarzt 2000;51:327–31. 16. Kishimoto S, Takenaka H, Shibagaki R, Nagata M, Katoh N, Yasuno H. Verruciform xanthoma arising in an arteriovenous haemangioma. Br J Dermatol 1998;139:546–8. 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM156 Chapter 8 / Benign Neoplasms 157 Fig. 19. Clinical features of hobnail hemangioma. Some lesions, such as the one shown in this figure, have a targetoid appearance, with a central angiomatous papule surrounded by an ecchy- motic halo. 5. HOBNAIL HEMANGIOMA (TARGETOID HEMOSIDEROTIC HEMANGIOMA) Santa Cruz and Aronberg (1) first described this lesion under the term targetoid hemo- siderotic hemangioma. After the original description, a few cases of mostly single lesions were reported in the literature (2–14). Recently, Guillou et al. (15) revisited the lesion and coined the term hobnail hemangioma, which is currently the preferred term. C LINICAL FEATURES Most lesions are characterized by a brown to violaceous central papule, surrounded by a thin, pale area and a peripheral ecchymotic ring (Fig. 19). The ecchymotic halo ulti- mately disappears, in contrast to the central papule which persists. However, there are hemangiomas with histopathologic features identical to those of targetoid hemosiderotic hemangioma that do not have the targetoid appearance. Thus the term hobnail heman- gioma appears to be a more appropriate name (1,16–17). H ISTOPATHOLOGIC FEATURES Histopathologically, hobnail hemangioma has a distinctive biphasic appearance. The center of the lesion is composed of dilated, irregular, thin-walled ectatic vascular spaces positioned in the superficial dermis (Fig. 20). These vascular spaces sometimes exhibit intraluminal papillary projections and fibrin thrombi at different stages of organization. Prominent, plump, endothelial cells with a hobnail appearance line the papillary projec- tions. The deep areas are distinct from the peripheral areas of the lesion because they show irregular, angulated, thin-walled, slit-shaped vascular channels that dissect collagen bundles of the dermis. In these areas, dermal fibrosis, hemosiderin deposits, extravasated red blood cells, and a sparse mononuclear inflammatory infiltrate are frequent findings. Hobnail hemangioma focally exhibits features reminiscent of retiform hemangioendot- helioma, Kaposi’s sarcoma, benign lymphangioendothelioma, and Dabska’s tumor (1,17,18). 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM157 158 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig. 20. Histopathologic features of hobnail hemangioma. (A) Scanning power shows a vascular proliferation involving the upper half of the dermis. (B) Higher magnification shows irregular vascular spaces between collagen bundles of the dermis. (C) Still higher magnification demon- strates that the irregular slit-like spaces are lined by endothelial cells that seem to dissect between collagen bundles of the dermis. Note also the abundant extravasation of erythrocytes. 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM158 Chapter 8 / Benign Neoplasms 159 Immunohistochemical studies have demonstrated that the endothelial cells lining the vascular spaces of hobnail hemangioma stain weakly for factor VIII-related antigen and strongly with Ulex europaeus I lectin, CD 34, and CD31 (1–3,16). Smooth muscle actin- positive pericytes are observed focally around some of the abnormal vascular spaces (1). Four out of eight cases studied stained positive for vascular endothelial growth factor receptor-3 (VEGFR-3), which suggests a lymphatic line of differentiation for this distinc- tive vascular neoplasm(17). The histopathologic differential diagnosis with the patch stage of Kaposi’s sarcoma can be difficult. Hobnail hemangioma shows dilated vessels with intraluminal papillary projections lined by prominent endothelial cells, a feature that is absent in Kaposi’s sarcoma. The vascular channels at the periphery of the lesion do not surround preexisting vascular and adnexal structures in the dermis, as is the case in the patch stage of Kaposi’s sarcoma. Furthermore, in contrast to patch lesions of Kaposi’s sarcoma, there is abundant hemosiderin and absence of plasma cells in the inflammatory infiltrate. Evidence seems to support the fact that hobnail hemangioma with a targetoid appear- ance results from trauma to a preexisting hemangioma or angiokeratoma, with subse- quent development of thrombi and recanalization. The central and superficial areas of the lesion show intraluminal projections and thrombi, closely resembling the intravascular papillary endothelial hyperplasia of Masson, lending further support to the view that hobnail hemangiomas are traumatized hemangiomas or angiokeratomas. Once thrombo- sis and canalization occur, the preexisting vessels at the center of the lesion persist, whereas the peripheral ecchymotic halo disappears. Support for this sequence is shared by other authors (14,19). Carlson et al. (14) compared the clinicopathologic features of 33 cases of hobnail hemangioma with those of 20 cases of solitary angiokeratoma and found an overlap of the clinical and histopathologic features in both groups. The authors concluded that the histopathologic findings of extravasated red blood cells, hemosiderin, telangiectases, lymphangiectases, and fibrosis implicate trauma as the cause of these acquired vascular proliferations. Christenson and Seabury Stone (19) described a patient with lesions showing histopathologic features of hobnail hemangioma, which developed after trauma of an inflammatory cutaneous lesion. T REATMENT Surgical excision is curative, and recurrences have been not reported. References 1. Santa Cruz DJ, Aronberg J. Targetoid hemosiderotic hemangioma. J Am Acad Dermatol 1988;19:550–8. 2. Rapini RP, Golitz LE. Targetoid hemosiderotic hemangioma. J Cutan Pathol 1990;17:233–5. 3. Vion B, Frenk E. Targetoid hemosiderotic hemangioma. Dermatology 1992;184:300–2. 4. Benes P, Douglass M, Lowe L. Targetoid hemosiderotic hemangioma; a series of four cases [Abstract]. J Cutan Pathol 1993;20:533. 5. Krahl D, Petzoldt D. Targetoides hamosiderotisches Hamangiom. Eine weitere Differentialdiagnose des Kaposi-Sarkoms. Hautarzt 1994;45:34–7. 6. Lowe L. Self assessment—1993. Targetoid hemosiderotic hemangioma. J Cutan Pathol 1994;21:568–9. 7. Perrin C, Rodot S, Ortonne JP, Michiels JF. L’hémangiome en cible hémosidériniqué. Ann Dermatol Venereol 1995;122:111–4. 8. Margaroth GS, Tigelaar RE, Longley J, Luck LE, Leffell DJ. Targetoid hemangioma associated with pregnancy and the menstrual cycle. J Am Acad Dermatol 1995;32:282–4. 9. Held JL, Malhotra R. Targetoid hemosiderotic hemangioma. Fitzpatrick’s J Clin Dermatol 1995;3:70. 10. Fariña MC, Montalvo N, Piqué E, et al. Hemangioma hemosiderótico en diana: un nuevo caso y revisión de la literatura. Actas Dermosifiliogr 1995;86:383–7. 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM159 160 Sangüeza and Requena / Pathology of Vascular Skin Lesions 11. Ho C, McCalmont TH. Targetoid hemosiderotic hemangioma: report of 24 cases, with emphasis on unusual features and comparison to early Kaposi’s sarcoma [Abstract]. J Cutan Pathol 1995;22:67. 12. Ly S, Versapuech J, Vergier B, Beylot-Barry M, Beylot C. Guess what? Targetoid hemosiderotic hemangioma. Eur J Dermatol 1998;8:583–5. 13. Avci O, Soyal MC, Sagol O, Gunes AT. Targetoid hemosiderotic hemangioma [Letter]. J Eur Acad Derm Venereol 1998;11:186–7. 14. Carlson JA, Daulat S, Goodheart HP. Targetoid hemosiderotic hemangioma—a dynamic vascular tumor: report of 3 cases with episodic and cyclic changes and comparison with solitary angiokeratomas. J Am Acad Dermatol 1999;41:215–24. 15. Guillou L, Calonje E, Speight P, Rosai J, Fletcher CD. Hobnail hemangioma: a pseudomalignant vascular lesion with a reappraisal of targetoid hemosiderotic hemangioma. Am J Surg Pathol 1999;23:97–105. 16. Santoja C, Torrelo A. Hobnail hemangioma. Dermatology 1995;191:154–6. 17. Mentzel T, Partanen TA, Kutzner H. Hobnail hemangioma (“targetoid hemosiderotic hemangioma”): clinicopathologic and immunohistochemical analysis of 62 cases. J Cutan Pathol 1999;26:279–86. 18. Requena L, Sangueza OP. Cutaneous vascular proliferations. Part II. Hyperplasias and benign neoplasms. J Am Acad Dermatol 1997;37:887–919. 19. Christenson LJ, Seabury Stone M. Trauma-induced simulator of targetoid hemosiderotic hemangioma. Am J Dermatopathol 2001;23:221–3. 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM160 Chapter 8 / Benign Neoplasms 161 Fig. 21. Microvenular hemangioma. Angiomatous plaque on the anterior chest of a girl. 6. MICROVENULAR HEMANGIOMA Microvenular hemangioma is a recently described benign vascular neoplasm. Hunt et al. (1) coined this name in 1991, but the lesions described in 1989 by Bantel et al (2) as microcapillary hemangiomas probably represent the same entity. So far, 25 cases have been reported in the literature (1–9). C LINICAL FEATURES Microvenular hemangioma is an acquired, slowly growing asymptomatic lesion with angiomatous appearance. It is usually solitary, varying in size from 0.5 to 2 cm. It most commonly affects the upper limbs, particularly the forearms. However, lesions on the trunk (Fig. 21), face, and lower limbs have also been recorded (3,4,9). In some patients, a histogenetic relationship between microvenular hemangioma and hormonal factors such as pregnancy and hormonal contraceptives has been postulated (2,4), but this feature has not been corroborated by other authors. An example of microvenular hemangioma developed in a patient with Wiskott-Aldrich syndrome (9). Hemangiomas identical to microvenular hemangiomas can be seen in patients with POEMS syndrome (10). H ISTOPATHOLOGIC FEATURES Histopathologically, microvenular hemangioma appears as a poorly circumscribed proliferation of irregularly branched, round to oval, thin-walled blood vessels lined by a single layer of endothelial cells. They involve the entire reticular dermis, and a variable degree of dermal sclerosis is present in the stroma (Fig. 22). The lumina of the neoplastic blood vessels are inconspicuous and often collapse with only a few erythrocytes in them. Immunohistochemically, the cells lining the lumina show positivity for factor VIII- related antigen and Ulex europaeus I lectin (1–3), which qualifies them as endothelial cells. Some smooth muscle actin, positive perithelial cells have also been described surrounding these vascular spaces (3,5). The main differential diagnosis of microvenular hemangioma is with Kaposi’s sarcoma in the patch stage. Kaposi’s sarcoma shows irregular anastomosing vascular spaces, newly formed ectatic vascular channels surrounding preexisting normal blood vessels and adnexa (promontory sign), plasma cells, hyaline (eosinophilic) globules, and small 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM161 162 Sangüeza and Requena / Pathology of Vascular Skin Lesions interstitial fascicles of spindle cells. All these features are absent in microvenular hemangioma. Characteristic of microvenular hemangioma is the presence of monomorphous small, elongated blood vessels with inconspicuous lumina involving the full thickness of the reticular dermis. T REATMENT Lesions of microvenular hemangioma are cured by simple excision. References 1. Hunt SJ, Santa Cruz DJ, Barr RJ. Microvenular hemangioma. J Cutan Pathol 1991;18:235–40. 2. Bantel E, Grosshans E, Ortonne JP. Zuz Kenntnis mickrokapillärer angiome, beobachtungen bei schwangeren bzw. unter hormoneller antikonzeption stehenden Frauen. Z Hautkr 1989;64:1071–4. 3. Aloi F, Tomasini C, Pippione M. Microvenular hemangioma. Am J Dermatopathol 1993;15:534–8. 4. Satge D, Grande-Goburdhun J, Grosshans E. Hémangiome microcapillaire. Ann Dermatol Venereol 1993;120:297–8. Fig. 22. Histopathologic features of microvenular hemangioma. (A) Scanning power shows a proliferation of irregular blood vessels with inconspicuous lumina involving the dermis. (B) Higher magnification demonstrates irregular vascular spaces lined by endothelial cells. (C) Still higher magnification shows plump endothelial cells lining the inconspicuous lumina. 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM162 Chapter 8 / Benign Neoplasms 163 5. Horn MS, Stern JB. Small red nodule on the leg of a young woman. Microvenular hemangioma. Arch Dermatol 1995;131:483. 6. Black RJ, McCusker GM, Eedy DJ. Microvenular haemangioma. Clin Exp Dermatol 1995;20:260–2. 7. Sanz Trelles A, Ojeda Martos A, Jiménez Fernández A, Vera Castaño A. Microvenular hemangioma: a new case in a child. Histopathology 1998;32:89–90. 8. Fukunaga M, Ushigome S. Microvenular hemangioma. Pathol Int 1998;48:237–9. 9. Rikihisa W, Yamamoto O, Kohda F, et al. Microvenular hemangioma in a patient with Wiskott-Aldrich syndrome. Br J Dermatol 1999;141:752–4. 10. Ackerman AB, Guo Y, Vitale P. Clues to Diagnosis in Dermatopathology II. Chicago, ASCP Press, 1992:285–8. 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM163 164 Sangüeza and Requena / Pathology of Vascular Skin Lesions 7. TUFTED ANGIOMA Tufted angioma is an unusual, acquired vascular neoplasm first described by Wilson Jones in 1976 (1). A similar lesion was previously described by MacMillan and Cham- pion (2) in 1971 under the name progressive capillary hemangioma. The neoplasm described by Nakagawa as angioblastoma also appears to be identical to acquired tufted angioma (3–5), although not all authors agree on this point (6). C LINICAL FEATURES Clinically, lesions of tufted angioma most commonly affect children and young adults, but both congenital and very late onset cases have been described (7–11). Most cases are sporadic, although a family with several members affected by tufted angioma has been reported (12). In this particular family, the mode of transmission was in an autosomal dominant fashion. The lesions have a predilection for the neck, upper chest, back, and shoulders (4,13,14), although examples of tufted angioma have also been reported on the head, extremities (Fig. 23), and oral mucosa (15–18). Tufted angioma grows slowly and insidiously and may eventually come to cover a large area of the trunk or neck. In most cases the growth is halted after some years, but there is a slight tendency toward spon- taneous regression (19). The clinical appearance of the lesions is variable. Some of them are characterized by enlarging erythematous or brown macules or plaques with an angiomatous appearance; other lesions may resemble granulomas or a connective tissue abnormality. In some cases the lesions are tender (20,21) whereas in other cases, there is hyperhidrosis on the surface (21,22). Raised papules resembling pyogenic granulomas are sometimes seen within the area of the lesion (23), and occasionally the lesions may show a linear arrangement (24).Tufted angiomas have been associated with nevus flammeus (25,26) and other vascular malformations (27), pregnancy (28), and nonregressing lipodystrophy centrifugalis abdominalis (29). There is also a single example of tufted angioma described in a liver transplant recipient (30). In some cases the lesions spread by infiltration, leading to sclerosing plaques (31). Many cases of Kasabach-Merritt syndrome are associated with tufted angioma (32–38). It has been demonstrated that in most cases of Kasabach- Fig. 23. Clinical features of tufted hemangioma. An angiomatous plaque involving the inner aspect of the arm. 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM164 Chapter 8 / Benign Neoplasms 165 Fig. 24. Histopathologic features of tufted hemangioma. (A) Low power shows multiple cellular lobules at different levels of the dermis separated by connective tissue. (B) Higher magnification demonstrates that each lobule is composed of aggregates of endothelial cells with some small vascular lumina. (C) Still higher magnification showing aggregations of endothelial cells with small capillary lumina containing erythrocytes. 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM165 [...]... Residual lesions after Kasabach-Merritt phenomenon in 41 patients J Am Acad Dermatol 2000;42:225–35 08/Sangüeza/13 3-2 16/ F 167 01/14/2003, 2:58 PM 168 Sangüeza and Requena / Pathology of Vascular Skin Lesions 38 Alvarez-Mendoza A, Lourdes TS, Ridaura-Sanz C, Ruiz-Maldonado R Histopathology of vascular lesions found in Kasabach-Merritt syndrome: review based on 13 cases Pediatr Dev Pathol 2000;3: 5 56 60 39... magnification shows aggregates of endothelial cells with small capillary lumina 08/Sangüeza/13 3-2 16/ F 175 01/14/2003, 2:58 PM 1 76 Sangüeza and Requena / Pathology of Vascular Skin Lesions vascular channels Proliferating markers Ki -6 7 and MPM-2 stain only a few of the nuclei of the endothelial cells of the newly formed blood vessels TREATMENT In the described series, treatment consisted of local surgical excision... leukemia A report of unusual case with immunohistochemical and immunofluorescence correlation and review of the literature J Cutan Pathol 1999; 26: 2 06 12 08/Sangüeza/13 3-2 16/ F 173 01/14/2003, 2:58 PM 174 Sangüeza and Requena / Pathology of Vascular Skin Lesions 9 ACQUIRED ELASTOTIC HEMANGIOMA Acquired elastotic hemangioma is a recently described variant of cutaneous hemangioma (1) These lesions develop... pseudopapillary pattern 08/Sangüeza/13 3-2 16/ F 183 01/14/2003, 2:58 PM 184 Sangüeza and Requena / Pathology of Vascular Skin Lesions 12 GIANT-CELL ANGIOBLASTOMA CLINICAL FEATURES Congenital infiltrating giant cell angioblastoma is the descriptive term used by Gonzalez-Crussi et al (1) to report a single case of a unique vascular neoplasm that involved the right hand and forearm of a 3-month-old girl More recently,... Pediatr Hematol Oncol 1998;20: 567 –9 19 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 20 Alvarez-Mendoza A, Lourdes TS, Ridaura-Sanz C, Ruiz-Maldonado R Histopathology of vascular lesions found in Kasabach-Merritt syndrome: review based on 13 cases Pediatr Dev Pathol 2000;3:5 56 60 21 Mentzel T, Mazzoleni G,... (1) postulated that the deposition of immunoglobulins within the endothelial cells stimulates the proliferation of these cells in a glomeruloid fashion The description of cases of reactive angioendotheliomatosis with a glomeruloid pattern in patients with cryoglobu- 08/Sangüeza/13 3-2 16/ F 169 01/14/2003, 2:58 PM 170 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig 25 Patient with POEMS syndrome... between them and extravasated erythrocytes (D) Large dilated and congestive vascular spaces are seen in other areas of the lesion 08/Sangüeza/13 3-2 16/ F 178 01/14/2003, 2:58 PM Chapter 8 / Benign Neoplasms 08/Sangüeza/13 3-2 16/ F 179 179 01/14/2003, 2:58 PM 180 Sangüeza and Requena / Pathology of Vascular Skin Lesions only slit-like lumina Basal lamina was occasionally present but was discontinuous and... (24,40,44– 46) For the treatment of these lesions, soft radiation has been recommended (13) Satisfactory results have been also reported with surgery (47), pulse dye laser (11,48), high-dose systemic steroids (49), and interferon-α, (50–54) although there is a report of one case that failed to respond to intralesional injections of interferon-α (55) References 1 2 3 4 5 6 7 8 08/Sangüeza/13 3-2 16/ F Wilson... glomeruloid arrangement In intravascular papillary endothelial hyperplasia or Masson’s tumor, fibrinoid or fibrous cores 08/Sangüeza/13 3-2 16/ F 171 01/14/2003, 2:58 PM 172 Sangüeza and Requena / Pathology of Vascular Skin Lesions form the intravascular papillary projections, but well-formed capillaries are absent Acquired tufted angioma is composed of solid aggregates of endothelial cells forming lobules... Fascicles of spindle cells and dilated congestive blood vessels of thin walls (C) Still higher magnification shows prominent vacuolization of the cells 08/Sangüeza/13 3-2 16/ F 187 01/14/2003, 2:58 PM 188 Sangüeza and Requena / Pathology of Vascular Skin Lesions syndrome developed angiosarcomas in other regions of the body separated from those affected by spindle cell hemangioma (11,24,28) Although many lesions . 2000;42:225–35. 08/Sangüeza/13 3-2 16/ F 01/14/2003, 2:58 PM 167 168 Sangüeza and Requena / Pathology of Vascular Skin Lesions 38. Alvarez-Mendoza A, Lourdes TS, Ridaura-Sanz C, Ruiz-Maldonado R. Histopathology of vascular lesions. capillary lumina containing erythrocytes. 08/Sangüeza/13 3-2 16/ F 01/14/2003, 2:58 PM 165 166 Sangüeza and Requena / Pathology of Vascular Skin Lesions Merritt syndrome the underlying lesion is not. 1995; 86: 383–7. 08/Sangüeza/13 3-2 16/ F 01/14/2003, 2:58 PM159 160 Sangüeza and Requena / Pathology of Vascular Skin Lesions 11. Ho C, McCalmont TH. Targetoid hemosiderotic hemangioma: report of 24