PATHOLOGY OF VASCULAR SKIN LESIONS - PART 7 potx

190 Sangüeza and Requena / Pathology of Vascular Skin Lesions 30. Hisaoka M, Hashimoto H, Iwamasa T. Diagnostic implication of Kaposi’s sarcoma-associated herpes- virus with special reference to the distinction between spindle cell hemangioendothelioma and Kaposi’s sarcoma. Arch Pathol Lab Med 1998;122:72–6. 31. Yañez S, Val-Bernal JF, Mira C, Echevarria MA, González-Vela MC, Arce F. Spindle cell hemangioen- dothelioma associated with multiple skeletal enchondromas: a variant of Maffucci’s syndrome. Gen Diagn Pathol 1998;143:331–5. 32. Gradner TL, Elston DM. Multiple lower extremity and penile spindle cell hemangioendotheliomas. Cutis 1998;62:23–6. 33. Bodemer C, Fraitag S, Amoric JC, Benaceur S, Brunelle F, De Prost Y. Hémangioendotheliome à cellules fusiformes dans une varieté monomelique et multinodulaire chez l’enfant. Ann Dermatol Venereol 1997;124:857–60. 34. Enjolras O, Wassef M, Merland JJ. Syndrome de Maffucci: une fausse malformation veineusse? Un cas avec hémangioendothelioma à cellules fusiformes. Ann Dermatol Venereol 1998;125:512–5. 35. Keel SB, Rosemberg AE. Hemorrhagic epithelioid and spindle cell hemangioma: a newly recognized, unique vascular tumor of bone. Cancer 1999;85:1966–72. 36. Isayama T, Iwasaki H, Ogata K, Naito M. Intramuscular spindle cell hemangioendothelioma. Skeletal Radiol 1999;28:477–80. 37. Tomasini C, Aloi F, Soro E, Elia V. Spindle cell hemangioma. Dermatology 1999;199:274–6. 38. Setoyama M, Shimada H, Miyazono N, Baba Y, Kanzaki T. Spindle cell hemangioendothelioma: succesful treatment with recombinant interleukin-2. Br J Dermatol 2000;142:1238–9. 39. Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors, 4th ed., St. Louis, Mosby, 2001;853–6. 40. Mentzel T, Kutzner H. Hemangioendotheliomas: heterogeneous vascular neoplasms. Dermatopathol Pract Concep 1999:5:102–9. 41. Requena L, Ackerman AB. Hemangioendothelioma? Dermatopathol Pract Concep 1999;5:110–2. 42. Fletcher CDM. The non-neoplastic nature of spindle cell hemangioendothelioma. Am J Clin Pathol 1992;98:545–6. 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM190 Chapter 8 / Benign Neoplasms 191 14. BENIGN LYMPHANGIOENDOTHELIOMA Benign lymphangioendothelioma is a rare lymphatic neoplasm characterized in a series of eight patients by Wilson Jones et al. in 1990 (1). Shortly thereafter these authors coined the name acquired progressive lymphangioma for the same lesion (2). So far, only 37 cases of this uncommon neoplasm have been reported (1–19), under the names acquired progressive lymphangioma (2–5,9–11,13,14) or benign lymphangioendothelioma (1,6–,8,12,15,17–19). The lesion described as acquired progressive lymphangioma of the skin following radiotherapy for breast carcinoma (11) is best interpreted as a benign, radiation-induced vascular proliferation of the breast (see the next chapter). The lesion designated as self-healing pseudoangiosarcoma (16) seemingly represents a transient benign lymphangioendothelioma. C LINICAL FEATURES The lesions of benign lymphangioendothelioma are reddish or bruise-like, slowly enlarging plaques that lack site predilection (Fig. 37). They can be found on the wrist (1), face (3,18), scalp (3,18), neck (18), shoulder (1,18), arm (5,6), forearm (1,18), breast (9,18), back (1,18), abdominal wall (4,16), buttock (14), knee (10), thigh (1,6–8,13,17), toes (18), sole (18), and oral mucosa (18). Typically, the lesion appears during adolescence or in young adults as an asymptomatic plaque that increases in size through the years. With the exception of one patient who had involvement of both forearms (1), and another Fig. 37. Clinical features of benign lymphangioendothelioma. A plaque with angiomatous appear- ance involving the anterior thigh of an adult man. 08/Sangüeza/133-216/F 01/14/2003, 2:58 PM191 192 Sangüeza and Requena / Pathology of Vascular Skin Lesions who presented with two separate lesions involving the face and the arm (3), most patients have solitary lesions. Lesions have developed in sites previously involved by trauma (3,17), notably following femoral arteriography (13), and after a tick bite (15). In one patient, benign lymphangioendothelioma manifested clinically as an actinic keratosis (19). H ISTOPATHOLOGIC FEATURES Benign lymphangioendotheliomas appear histologically as delicate, thin-walled, endothelium-lined spaces entrapped between collagen bundles (Fig. 38). The appearance may be confined to the papillary dermis, but it can extend into the reticular dermis and subcutaneous fat. In superficial areas, the vascular channels are arranged horizontally, often becoming smaller and more convoluted at deeper levels. The newly formed vessels may dissect preexisting vessels as well as adnexal structures of the dermis. The vascular spaces are variably empty or occupied by proteinaceous material. Some vessels show stromal papillary projections that call to mind papillary endothelial hyperplasia. Eryth- rocytes and hemosiderin deposits are characteristically absent. Endothelial cells are present in greater numbers in lesional vessels than in normal lymphatic channels. They may crowd together but regularly lack nuclear atypia. Immunohistochemically, the endothelial cells express affinity for Ulex europaeus I lectin (1,8,14,17), but are unpredictable for factor VIII-related antigen, some cases posi- tive (8,14,17,18) and others negative (1,7,13). In addition, the endothelial cells may show immunoreactivity for CD31(17,18), CD34 (8,17,18), HLA-DR (8), smooth muscle actin (8,18), and ICAM-1 (8). Some studies have demonstrated the presence of type IV col- lagen (5,14,17) and desmin (5,14) in the matrix that surrounds the vascular channels, thus creating conjecture that benign lymphangioendothelioma is a complex hamartoma, which combines blood vessel, lymphatic, and smooth muscle components. Electron micro- scopic studies have revealed the presence of both tight junctions and well-formed, con- tinuous basement membranes in the absence of Weibel-Palade bodies (8,13). Benign lymphangioendothelioma may mimic the patch stage of Kaposi’s sarcoma, and some authorities (20) have considered the two entities to be indistinguishable. Wilson Jones et al. (1), in their original series, emphasized that it is sometimes impossible to make this differentiation in the absence of clinical information. However, the differential diagnosis can be substantiated by the absence of erythrocytes, hemosiderin deposits, and plasma cells in lesions of benign lymphangioendothelioma; their presence frequently characterizes early lesions of Kaposi’s sarcoma. Benign lymphangioendothelioma may also mimic low-grade angiosarcoma. However, contrastingly, angiosarcoma occurs pre- dominantly on the face and scalp of elderly patients, and it bears atypical endothelial cells that form multilayers in less differentiated areas. Extravasated erythrocytes, hemosiderin deposits, and a mixed or plasma-cell rich inflammatory infiltrate are common features in angiosarcoma. T REATMENT Some lesions of benign lymphangioendothelioma have resolved spontaneously (7,16). Surgical excision is generally curative (1,4), but some lesions have persisted after incom- plete removal. Marked improvement of extensive lesions has been reported following therapy with oral prednisolone (3), or oral antibiotics (5), namely, ciprofloxacin and clindamycin, which were given for other reasons (14). 08/Sangüeza/133-216/F 01/14/2003, 2:59 PM192 Chapter 8 / Benign Neoplasms 193 Fig. 38. Histopathologic features of benign lymphangioendothelioma. (A) Irregular slit-like vas- cular spaces are present involving different levels of the dermis. (B) The vascular spaces appear empty and are lined by a discontinuous single layer of endothelial cells. A discrete inflammatory infiltrate of lymphocytes is present in the stroma. (C) Higher magnification demonstrates that endothelial cells lining the cleft-like vessels show no nuclear atypia. 08/Sangüeza/133-216/F 01/14/2003, 2:59 PM193 194 Sangüeza and Requena / Pathology of Vascular Skin Lesions References 1. Wilson Jones E, Winkelmann RK, Zachary CB, Reda AM. Benign lymphangioendothelioma. J Am Acad Dermatol 1990;23:229–35. 2. Wilson Jones E. Malignant vascular tumours. Clin Exp Dermatol 1976;1:287–312. 3. Watanabe M, Kishiyama K, Ohkawara A. Acquired progressive lymphangioma. J Am Acad Dermatol 1983;8:663–7. 4. Tadaki T, Aiba S, Masu S, Tagami H. Acquired progressive lymphangioma as a flat erythematous patch on the abdominal wall of a child. Arch Dermatol 1988;124:699–701. 5. Zhu WY, Penneys NS, Reyes B, Khatib Z, Schachner L. Acquired progressive lymphangioma. J Am Acad Dermatol 1991;24:813–5. 6. Chemaly PH, Cricks B, Besseige H, Grossin M, Belaich S. Lymphangio-endotheliome benin. Ann Dermatol Venereol 1992;119:912–3. 7. Mehregan DR, Mehregan AH, Mehregan DA. Benign lymphangioendothelioma: report of 2 cases. J Cutan Pathol 1992;19:502–5. 8. Herron GS, Rouse RV, Kosek JC, Smoller BR, Egbert BM. Benign lymphangioendothelioma. J Am Acad Dermatol 1994;31:362–8. 9. Meunier L, Barneon G, Meynadier J. Acquired progressive lymphangioma. Br J Dermatol 1994; 131:706–8. 10. Soohoo L, Mercurio MG, Brody R, Zaim MT. An acquired vascular lesion in a child. Acquired progres- sive lymphangioma. Arch Dermatol 1995;131:341–2. 11. Rosso R, Gianelli U, Carnevali L. Acquired progressive lymphangioma of the skin following radio- therapy for breast carcinoma. J Cutan Pathol 1995;22:164–7. 12. Querol I, Cordoba A, Cisneros MT, Viguri A, Urbiola E. Linfangioendotelioma benigno. Med Cut Iber Lat Am 1995;23:243–7. 13. Kato H, Kadoya A. Acquired progressive lymphangioma occurring following femoral arteriography. Clin Exp Dermatol 1996;21:159–62. 14. Grunwald MH, Amichi B, Avinoach I. Acquired progressive lymphangioma. J Am Acad Dermatol 1997;37:656–7. 15. Wilmer A, Kaatz M, Mentzel T, Wollina U. Lymphangioendothelioma after a tick bite. J Am Acad Dermatol 1998;39:126–8. 16. Bencini PL, Sala F, Valeriani D, et al. Self-healing pseudoangiosarcoma. Unusual vascular proliferation resembling a vascular malignancy of the skin. Arch Dermatol 1988;124:692–4. 17. Sevila A, Botella Estrada R, Sanmartín O, et al. Benign lymphangioendothelioma of the thigh simulating a low-grade angiosarcoma. Am J Dermatopathol 2000;22:151–4. 18. Guillou L, Fletcher CD. Benign lymphangioendothelioma (acquired progressive lymphangioma), a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi’s sarcoma. Clinicopathologic analysis of a series. Am J Surg Pathol 2000;24:1047–57. 19. Yiannias JA, Winkelmann RK. Benign lymphangioendothelioma manifested clinically as actinic kera- tosis. Cutis 2001;67:29–30. 20. Sanchez JL, Ackerman AB. Vascular proliferations of the skin and subcutaneous fat. In: Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austen KF, eds. Dermatology in General Medicine, 4th ed., New York, McGraw-Hill, 1993;1209–43. 08/Sangüeza/133-216/F 01/14/2003, 2:59 PM194 Chapter 8 / Benign Neoplasms 195 15. BENIGN VASCULAR PROLIFERATIONS IN IRRADIATED SKIN Benign vascular proliferations are well recognized lesions in previously irradiated areas of the skin. The nomenclature in the literature is complex, compounded by such terms as lymphangiectases (1), benign lymphangiomatous papules (2), lymphangiomas (3–13), atypical vascular lesions (14), and benign lymphangioendothelioma (15). These vascular lesions appear within the field of radiation, and the interval between the application of radiotherapy and the appearance of the cutaneous lesions spans several years (16). C LINICAL FEATURES The cutaneous lesions include papules, small vesicles, and erythematous plaques (Fig. 39). Benign appearing lymphangiomatous papules and plaques are the most com- mon. Confusingly, the term lymphangioma circumscriptum (9,10,12,13) has been applied by some authors to the localized malformations of lymphatic vessels of the superficial dermis and as such bears no relation to the lesion under consideration in this chapter (17). Benign lymphangiomatous papules and plaques are the lymphatic counterpart of telang- iectases. They result from acquired permanent dilation of lymphatic capillaries that have appeared in areas of the skin affected by obstruction or destruction of the lymphatics. It is conjectured that they result from interference in the drainage of lymphatic vessels secondary to radiotherapy (1–13) or surgery (18). Benign lymphangiomatous papules and plaques, however, may also appear in the skin of the elderly without any evidence of primary lymphatic injury (19). H ISTOPATHOLOGIC FEATURES Under low magnification, the lesions appear as relatively well-circumscribed capil- lary proliferations centered in the dermis, without extension into the subcutaneous fat. The epidermis is usually spared (Fig. 40). Most lesions show irregularly dilated lym- phatic spaces that branch and anastomose within the superficial dermis. The vascular spaces, devoid of content, are lined by a discontinuous single layer of endothelial cells with flattened nuclei. Commonly, adjacent vascular channels lie “back-to-back,” sepa- rated only by a thin layer of endothelial cells. Multiple papillary projections, covered by a single layer of endothelium, project into the lumina of the dilated lymphatic. The stroma Fig. 39. Clinical features of a benign vascular proliferation in irradiated skin. This patient with breast carcinoma was treated with mastectomy and subsequent radiotherapy. In addition to the abundant telangiectases secondary to radiodermatitis, there are scattered small papules with an angiomatous appearance. 08/Sangüeza/133-216/F 01/14/2003, 2:59 PM195 196 Sangüeza and Requena / Pathology of Vascular Skin Lesions consists of fibrillary collagen rich with spindle, or stellate, fibroblasts. Nodular infiltrates of lymphocytes with germinal centers are occasionally present in the vicinity of the dilated vascular channels. Rarely, vascular proliferations are poorly circumscribed and focally intermingled with irregular jagged vascular spaces that may permeate the entire dermis. Endothelial cells line the latter inconspicuously. Irregular slit-like vascular spaces may be seen between collagen bundles of the dermis, together with tufts of endothelial cells that protrude into the lumina of the newly formed vessels (16). The endothelial cells that line the vascular spaces express immunoreactivity for CD31, but they do so only focally or not at all for CD34. Although a minority of newly formed vessels show an attenuated muscle layer, external to the endothelial cells, which has on occasion immunoreactivity for α-smooth muscle actin antibody, this marker is usually nonreactive. Reactivity for Ki-67 is negative among the endothelial cell nuclei (16). The immunohis- tochemical profile substantiates the lymphatic nature of these newly formed vessels. Some dermal vascular responses to irradiation, such as the benign lymphangio- endothelioma (15) or an atypical angiomatous proliferation (14), may mimic the histo- pathologic appearance of the patch stage of Kaposi’s sarcoma or even a well-differentiated angiosarcoma. In contrast to the patch stage of Kaposi’s sarcoma, atypical benign vas- Fig. 40. Histopathologic features of benign vascular proliferation in irradiated skin. (A) Low power shows dilated vascular spaces at different levels of the dermis. (B) These vessels show a lymphatic appearance, with thin walls and a single and discontinuous layer of endothelial cells lining their lumina. In some vessels there are small intraluminal papillary projections of endothe- lial cells. 08/Sangüeza/133-216/F 01/14/2003, 2:59 PM196 Chapter 8 / Benign Neoplasms 197 cular proliferations, as induced by radiation, do not contain erythrocytes or hemosiderin deposits, or stromal plasma cells. The striking tufts of endothelial cells and the intravas- cular papillary projections, evidenced in the vascular proliferations of irradiated skin, are absent in the lesions of Kaposi’s sarcoma. In contrast to well-differentiated angiosar- coma, atypical dermal vascular proliferations in irradiated skin do not involve the sub- cutaneous tissues. Distinctively, they have no cytologic atypia. The nuclei of the endothelial cells are monomorphous, have inconspicuous nucleoli, and lack mitotic fig- ures. In contrast, the endothelial cells of an angiosarcoma may form stratified layers that irregularly line anastomostic channels, to a degree not seen in the atypical vascular proliferations of irradiated skin. T REATMENT The vascular proliferations in irradiated skin do not call for therapy, and the accounts in the literature have not provided examples of metastasis. References 1. Ambrojo P, Fernández-Cogolludo E, Aguilar A, et al. Cutaneous lymphangiectases after therapy for carcinoma of the cervix: a case with unusual clinical and histological features. Clin Exp Dermatol 1990;15:57–9. 2. Díaz-Cascajo C, Borghi S, Weyers W, Retzlaff H, Requena L, Metze D. Benign lymphangiomatous papules of the skin following radiotherapy. A report of five new cases and review of the literature. Histopathology 1999;35:319–27. 3. Fisher I, Orkin M. Acquired lymphangioma (lymphangiectasis). Arch Dermatol 1970;101:230–4. 4. Gianelli V, Rockley PF. Acquired lymphangiectases following mastectomy and radiation therapy. Report of a case and review of the literature. Cutis 1996;58:276–8. 5. Handfield-Jones SE, Prendville WJ, Norman S. Vulval lymphangiectasia. Genitourin Med 1989; 65:335–7. 6. Harwood CA, Mortimer PS. Acquired vulvar lymphangiomata mimicking genital warts. Br J Dermatol 1993;129:334–6. 7. Kennedy CTC. Lymphangiectasia of the vulva following hysterectomy and radiotherapy. Br J Dermatol 1990;123 (suppl. 37):92–3. 8. Kurwa A, Waddinton E. Post mastectomy lymphangiomatosis. Br J Dermatol 1968;80:840. 9. LaPolla J, Foucar E, Leshin B et al. Vulvar lymphangioma circumscriptum: a rare complication of therapy for squamous cell carcinoma of the cervix. Gynecol Oncol 1985;22:363–6. 10. Leshin B, Whitaker D, Foucar E. Lymphangioma circumscriptum following mastectomy and radiation therapy. J Am Acad Dermatol 1986;15:1117–9. 11. Plotnick H, Richfield D. Tuberous lymphangiectatic varices secondary to radical mastectomy. Arch Dermatol Syphilol 1956;74:466–8. 12. Prioleau PG, Santa Cruz DJ. Lymphangioma circumscriptum following radical mastectomy and radia- tion therapy. Cancer 1978;42:1989–91. 13. Young AW Jr, Wind RM, Tovell HM. Lymphangioma of vulva: acquired following treatment for cervical cancer. NY State J Med 1980;80:987–9. 14. Finenberg S, Rosen PP. Cutaneous angiosarcoma and atypical vascular lesions of the skin and breast after radiation therapy for breast carcinoma. Am J Clin Pathol 1994;102:757–63. 15. Rosso R, Gianelli U, Carnevali L. Acquired progressive lymphangioma of the skin following radio- therapy for breast carcinoma. J Cutan Pathol 1995;22:164–7. 16. Requena L, Kutzner H, Mentzel T, Durán R, Rodríguez-Peralto JL. Benign vascular proliferations in irradiated skin. Am J Surg Pathol 2002;26:328–37. 17. Requena L, Sangueza OP. Cutaneous vascular anomalies. Part I. Hamartomas, malformations, and dilatation of preexisting vessels. J Am Acad Dermatol 1997;37:523–9. 18. Ziv R, Schewach-Millet M, Trau H. Lymphangiectasia: a complication of thoracotomy for bronchial carcinoid. Int J Dermatol 1988;27:123. 19. Cecchi R, Bartoli L, Brunetti L, Pavesi M, Giomi A. Lymphangioma circumscriptum of the vulva of late onset. Acta Derm Venereol 1995;75:79–93. 08/Sangüeza/133-216/F 01/14/2003, 2:59 PM197 198 Sangüeza and Requena / Pathology of Vascular Skin Lesions 16. GLOMUS TUMORS Glomus tumors are uncommon neoplasms that arise from modified smooth muscle cells normally present in specialized arteriovenous shunts in acral sites, mainly the fin- gertips. These anatomic structures, known as the Sucquet-Hoyer canals, contribute to temperature regulation. Sucquet-Hoyer canals are lined by endothelial cells and possess several intramural layers of glomus cells. The vascular channel connects an afferent arteriole to an efferent venule (1). Glomus tumors are considered to originate from the glomus cells; thus they occur preferentially in acral areas (2).However, “renegade” or “ectopic” glomus tumors have been described in extracutaneous sites notably in the bone (3), stomach (4), colon (5), trachea (6), and mediastinum (7). Since glomus bodies are sparse, or presumptively absent in these areas, conceptually glomus tumors may arise from either ectopic glomus cells or from undifferentiated perivascular cells (8). C LINICAL FEATURES Two types of glomus tumors have been described, solitary and multiple. They do not fully share distribution, clinical characteristics, or histopathologic features (9). The soli- tary glomus tumor is more common. It creates a small, purple nodule preferentially in acral areas of the extremities, especially nail beds of the fingers (Fig. 41) and toes (2). Subungual lesions may create a blue-red flush and with time may erode the distal phalanx (10,11). There is a striking predominance among female patients (10). The lesion fre- quently creates severe paroxysmal pain, usually precipitated by exposure to cold or minor pressure. The cause of the pain is a subject of conjecture. However, the recently demon- strated substance P in nerve fibers of glomus tumors incriminates this substance, since it is known to be a primary sensory afferent neurotransmitter for mediating painful stimuli (12). Solitary glomus tumors may on occasion occur in aberrant locations. They typically appear in the early adult years, although not always. In general, they affect both sexes, although there is a female predominance (10,11). Multiple glomus tumors are much less common than their solitary counterpart. They are termed glomangiomas descriptively in accordance with their angiomatous appearance. In contrast to the solitary glomus lesion, glomangiomas present during childhood as small bluish nodules situated deep in the dermis and widely scattered in the skin (Fig. 42). Multiple lesions have been noted to aggregate in an anatomic region (13–18). Gloman- giomas are rarely subungual. They are less commonly painful. Noteably, multiple glo- mangiomas are often inherited in an autosomal dominant manner (9,19–25). The gene is located in chromosome 1p21-22 (26). Presumably, sporadic cases occur from somatic mutation of the same gene. Glomangiomas are often sufficiently large to be raised, soft, and compressible. As such they can be mistaken for lesions of the blue rubber bleb nevus syndrome, even in the absence of intestinal bleeding (27). Patients may develop Kasabach- Merritt syndrome (28). Occasionally, glomangiomas present as a solitary telangiectatic plaque-like lesion (29) (Fig. 43). Mounayer et al. (30) recently reported seven patients with multiple large facial plaque-like glomangiomas that mimicked common facial venous malformations (Fig. 44). These extensive facial glomangiomas were not painful. Unlike facial venous malformations, the large facial glomangiomas described by Mounayer et al. (30) were distinctly nodular, deep blue or purple, and poorly compress- ible. Histopathologic study disclosed one or several rows of glomus cells present in the walls of the large tortuous venous channels. 08/Sangüeza/133-216/F 01/14/2003, 2:59 PM198 Chapter 8 / Benign Neoplasms 199 Glomangiomyomas consist of tumors composed of neoplastic cells that show a gradual transition from glomus cells to elongated, mature smooth muscle cells (Fig. 45). H ISTOPATHOLOGIC FEATURES Histopathologically, solitary glomus tumors are customarily solid, well-circumscribed nodules surrounded by compressed fibrous tissue. The neoplasm is cytologically formed of clusters of round or polygonal monomorphous glomus cells with large, round, plump Fig. 41. Clinical features of subungual glomus tumor. A purple nodule is seen under the nail plate. Fig. 42. Clinical features of multiple glomus tumors. Multiple small blue nodules scattered on the back of an adult woman. 08/Sangüeza/133-216/F 01/14/2003, 2:59 PM199 [...]... The lesions present as small, pink-to-violaceous macules (Figs 4 and 5), preferentially along the lines of skin cleavage (71 ) The macules evolve into oblong papules and nodules In contrast to the lesions of classic Kaposi’s sarcoma, which mainly affect the distal parts 09/Sangüeza/21 7- 2 74 /F 221 01/16/2003, 10:12 AM 222 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig 5 Clinical features of. .. Kaposi’s sarcoma, but the lesions resolve entirely upon withdrawal of the offending agent However, with use of prolonged, high-dose 09/Sangüeza/21 7- 2 74 /F 219 01/16/2003, 10:12 AM 220 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig 1 Clinical features of classic Kaposi’s sarcoma An angiomatous plaque involving the lateral ankle and foot of an elderly man Fig 2 Nodular stage of classic Kaposi’s... demonstrates that the cells surrounding the vascular channels show characteristics of glomus cells 08/Sangüeza/13 3-2 16/F 205 01/14/2003, 2:59 PM 206 Sangüeza and Requena / Pathology of Vascular Skin Lesions 17 Yang J-S, Ko J-W, Suh K-S, Kim S-T Congenital multiple plaque-like glomangiomyoma Am J Dermatopathol 1999;21:454 7 18 Carvalho VO, Taniguchi K, Giraldi S, et al Congenital plaquelike glomus tumor... Ultrastructurally, neoplastic cells of cutaneous adult myofibroma appear as undifferentiated mesenchymal forms with features of fibroblasts, myofibroblasts, and 08/Sangüeza/13 3-2 16/F 213 01/14/2003, 2:59 PM 214 08/Sangüeza/13 3-2 16/F Sangüeza and Requena / Pathology of Vascular Skin Lesions 214 01/14/2003, 2:59 PM Chapter 8 / Benign Neoplasms 215 pericytes (12) Neoplastic cells with a myofibroblastic appearance... gutartiges Myofibrom der Haut (Adultes Myofibrom) Hautarzt 1993;44:561–8 6 Wolfe JT, Cooper PH Solitary cutaneous “infantile” myofibroma in a 49-year-old woman Hum Pathol 1990;21:562–4 7 Sahin AA, Ro JY, Ordoñez NG, et al Myofibroblastoma of the tongue An immunohistochemical, ultrastructural, and flow cytometric study Am J Clin Pathol 1990;94 :77 3 7 8 Hogan SF, Salassa JR Recurrent adult myofibromatosis... hemangiopericytoma 2 17 09/Sangüeza/21 7- 2 74 /F 2 17 01/16/2003, 10:12 AM 218 Sangüeza and Requena / Pathology of Vascular Skin Lesions tage; central Africans, in whose countries the disease is endemic; and immunosuppressed patients such as renal transplant recipients (2–4) The situation changed dramatically in 1981, when cases of Kaposi’s sarcoma started to be reported in young homosexual men (5 7) Since then... Requena / Pathology of Vascular Skin Lesions Fig 43 Rare variant of solitary glomangioma with the appearance of a telangiectatic plaque Histopathologic study demonstrated conventional features of glomangioma Fig 44 Large facial glomangioma mimicking a common facial venous malformation nuclei and scant eosinophilic cytoplasm (Fig 46) Endothelium-lined vascular spaces create a core in the center of some of. .. portions of the upper and lower extremities (Fig 52) being the most commonly targeted (12) The lesions may be painful (12) HISTOPATHOLOGIC FEATURES Among cutaneous adult myofibromas, the histologic appearance varies with the duration of the lesion (12) Earlier lesions feature the vascular component In this vascular type of cutaneous adult myofibroma, variable numbers of endothelium-lined vascular Fig... AIDS-associated Kaposi’s sarcoma Lesions of Kaposi’s sarcoma are intermingled with plaques of psoriasis on the anterior chest of the lower extremities, the lesions of AIDS-associated Kaposi’s sarcoma are often distributed over the chest and face (Fig 6) In some cases the oral mucosa is the first affected site, with the mucosa of the hard palate (Fig 7) being the most frequently involved area (72 ) The... lesions of AIDS-related Kaposi’s sarcoma The earliest lesions of Kaposi’s sarcoma, known as the patch stage (71 ), are characterized by inconspicuous changes and may produce the erroneous impression of an inflammatory condition (78 ) At scanning magnification these lesions show sparse, 09/Sangüeza/21 7- 2 74 /F 222 01/16/2003, 10:12 AM Chapter 9 / Malignant Neoplasms 223 Fig 6 Clinical features of advanced . cells. 08/Sangüeza/13 3-2 16/F 01/14/2003, 2:59 PM205 206 Sangüeza and Requena / Pathology of Vascular Skin Lesions 17. Yang J-S, Ko J-W, Suh K-S, Kim S-T. Congenital multiple plaque-like glomangiomyoma circumscriptum of the vulva of late onset. Acta Derm Venereol 1995 ;75 :79 –93. 08/Sangüeza/13 3-2 16/F 01/14/2003, 2:59 PM1 97 198 Sangüeza and Requena / Pathology of Vascular Skin Lesions 16. GLOMUS. 1996;20:233–8. 08/Sangüeza/13 3-2 16/F 01/14/2003, 2:59 PM2 07 208 Sangüeza and Requena / Pathology of Vascular Skin Lesions 17. HEMANGIOPERICYTOMA Since Stout and Murray’s initial description of hemangiopericytoma