PATHOLOGY OF VASCULAR SKIN LESIONS - PART 10 doc

292 Sangüeza and Requena / Pathology of Vascular Skin Lesions References 1. Argenyi ZB, Piette WW, Goeken J. Cutaneous angiomyolipoma: a light microscopic, immunohis- tochemical, and electron microscopic study [Abstract]. J Cutan Pathol 1986;13:434. 2. Argenyi ZB, Piette WW, Goeken JA. Cutaneous angiomyolipoma: a light microscopic, immunohis- tochemical, and electron-microscopic study. Am J Dermatopathol 1991;13:497–502. 3. Mehregan DA, Mehregan DR, Mehregan AH. Angiomyolipoma. J Am Acad Dermatol 1992;27:331–3. 4. Rodriguez Fernandez A, Caro Mancilla A. Cutaneous angiomyolipoma with pleomorphic changes. J Am Acad Dermatol 1993;29:115–6. 5. Val-Bernal FJ, Mira C. Cutaneous angiomyolipoma. J Cutan Pathol 1996;23:364–8. 6. Ortiz Rey JA, Valbuena Ruvira L, Bouso Montero M, Sacristan Lista F. Angiomiolipoma cutáneo. Patología 1996;29:115–8. 7. Fitzpatrick JE, Mellette JR, Hwang RJ, Golitz LE, Zaim T, Clemous D. Cutaneous angiolipoleiomyoma. J Am Acad Dermatol 1990;23:1093–8. 8. Buyukbabani N, Tetikkurt S, Ozturk AS. Cutaneous angiomyolipoma: report of two cases with emphasis on HMB-45 utility. J Eur Acad Dermatol Venereol 1998;11:151–4. 9. Watanabe K, Suzuki T. Mucocutaneous angiomyolipoma. A report of 2 cases arising in the nasal cavity. Arch Pathol Lab Med 1999;123:789–92. 10/Sangüeza/275-298/F 01/14/2003, 4:15 PM292 Chapter 10 / Other Neoplasms 293 Fig. 12. Clinical features of cutaneous angiomyxoma. A subcutaneous nodule on the dorsum of the toe. 6. CUTANEOUS ANGIOMYXOMA CLINICAL FEATURES Cutaneous myxomas have been firmly established in recent years, especially when they are present as part of Carney’s complex. This is an autosomal dominant complex consisting of endocrine hyperactivity (Cushing’s syndrome, testicular tumors, and acromegaly), spotty pigmentation, psammomatous melanotic schwannoma, and multiple myxomas (cutaneous, cardiac, and mammary) (1–5). A patient with multiple cutaneous myxomas and other anomalies of Carney’s complex progressed to scleromyxedema (6). Multiple cutaneous myxomas, however, have also been described without other elements of Carney’s complex (7,8). Solitary myxomas are not related to Carney’s complex and may appear as cutaneous or subcutaneous nodules, especially in acral sites (9–11). When there is a prominent vascular component, the lesion is designated angiomyxoma (9–11). Clinically, cutaneous angiomyxomas appear as slow-growing nodules that have been present for several years before excision (Fig. 12). Some solitary angiomyxomas appear as congenital lesions (12), and, in rare instances, solitary cutaneous angiomyxoma may be large (13). H ISTOPATHOLOGIC FEATURES Histopathologically, the lesions are fairly well-circumscribed, multilobulated nod- ules, with abundant blood vessels, especially at the periphery and delicate septa of col- lagen bundles extending into the lesion (14). Individual nodules appear as sparsely cellular with abundant mucinous material (Fig. 13). Stellate and spindle-shaped stromal cells are scattered throughout the myxoid material. Cytologic atypia is mild at most, and mitotic figures are rare. Sometimes dilated thin-walled blood vessels filled by erythrocytes are scattered within the mucoid material, imparting an angiomatous appearance to the lesion. Often, hyperplastic epithelia from entrapped adnexa, in the form of small epidermoid cysts, thin strands of squamous epithelium, and small buds of basaloid cells, are seen embedded in the lesion (14). A mixed inflammatory infiltrate is common, particularly 10/Sangüeza/275-298/F 01/14/2003, 4:15 PM293 294 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig. 13. Histopathologic features of cutaneous angiomyxoma. (A) Low power shows an exophytic lesion. The dermis exhibits a myxoid appearance. (B) Thin, spindle-shaped cells and capillaries embedded in a myxoid stroma. (C) These spindle-shaped cells show no nuclear atypia. 10/Sangüeza/275-298/F 01/14/2003, 4:15 PM294 Chapter 10 / Other Neoplasms 295 stromal neutrophils, a feature unique to this neoplasm compared with other cutaneous myxoid lesions (14). Immunohistochemical studies have shown that the cellular compo- nent of the lesion is positive with vimentin and α-smooth muscle actin but negative for CD34, S-100 protein, factor XIIIa, Leu-7, Kp1, MAC387, and desmin (10,14), support- ing a myofibroblastic differentiation. Electron microscopic examination of these cells demonstrated ultrastructural characteristics of fibroblasts (9). Some cutaneous myxomas arise in the genital region (15), and they should be differen- tiated from aggressive angiomyxoma. The latter lesion tends to be larger, extends to deeper structures, and has a vascular component different from that of cutaneous angiomyxoma, because the vascular component of aggressive angiomyxoma consists of variable-sized vessels that range from small thin-walled capillaries to large vessels with secondary changes including perivascular hyalinization and thick vessel walls. T REATMENT Cutaneous angiomyxomas are benign neoplasms, but local recurrences have been reported in 38% of cases (1,14), probably because they are subcutaneous and poorly demarcated lesions. References 1. Carney JA, Headington JT, Su WPD. Cutaneous myxomas: a major component of myxomas, spotty pigmentation, and endocrine overactivity. Arch Dermatol 1986;122:790–8. 2. Carney JA. Psammomatous melanotic schwannoma: a distinctive heritable tumor with special associa- tions, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol 1990;14:206–22. 3. Handley J, Carson D, Sloan J, et al. Multiple lentigines, myxoid tumour and endocrine overactivity; four cases of Carney complex. Br J Dermatol 1992;126:367–71. 4. Carney JA. Carney complex: the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas. Semin Dermatol 1995;14:90–8. 5. Egan CA, Stratakis CA, Turner ML. Multiple lentigines associated with cutaneous myxomas. J Am Acad Dermatol 2001;44:282–4. 6. Craig NM, Putterman AM, Roenigk RK, Wang TD, Roenigk HH. Multiple periorbital cutaneous myxo- mas progressing to scleromyxedema. J Am Acad Dermatol 1996;34:928–30. 7. Murphy CM, Grau-Massanes M, Sanchez RL. Multiple cutaneous myxomas. Report of a case without other elements of Carney’s complex. J Cutan Pathol 1995;22:556–62. 8. Bernardeu K, Serpier H, Salmon Ehr V, Metz D, Pluot M, Kalis B. Myxomes cutanés multiples isolés. Ann Dermatol Venereol 1998;125:30–3. 9. Allen PW, Dymock RB, MacCormac LB. Superficial angiomyxomas with and without epithelial com- ponents. Am J Surg Pathol 1988;12:519–30. 10. Wilk M, Schmoeckel C, Kaiser HW, Hepple R, Kreysel HW. Cutaneous angiomyxoma: a benign neoplasm distinct from cutaneous focal mucinosis. J Am Acad Dermatol 1995;33:352–5. 11. Alaiti S, Nelson FP, Ryoo JW. Solitary cutaneous myxoma. J Am Acad Dermatol 2000;43:377–9. 12. Bedlow AJ, Sampson SA, Holden CA. Congenital superficial angiomyxoma. Clin Exp Dermatol 1997;22:237–9. 13. Lockshin NA, Boswell JT. Giant cutaneous angiomyxoma. Cutis 1978;21:673–4. 14. Calonje E, Guerin D, McCormick D, Fletcher CD. Superficial angiomyxoma: clinicopathologic analysis of a series of distinctive but poorly recognized cutaneous tumors with tendency for recurrence. Am J Surg Pathol 1999;23:910–7. 15. Fetsch JF, Laskin WB, Tavassoli FA. Superficial angiomyxoma (cutanous myxoma): a clinicopatho- logic study of 17 cases arising in the genital region. Int J Gynecol Pathol 1997;16:325–34. 10/Sangüeza/275-298/F 01/14/2003, 4:15 PM295 296 Sangüeza and Requena / Pathology of Vascular Skin Lesions 7. AGGRESSIVE ANGIOMYXOMA CLINICAL FEATURES An entirely different lesion from cutaneous angiomyxoma has been described by Stepper and Rosai (1) under the term aggressive angiomyxoma. The lesion occurs in the genital (Fig. 14), perineal, and pelvic regions, and women are chiefly affected (1–5), but there have also been some reported examples in men involving the scrotum, perineal, and perianal areas (6–8). Aggressive angiomyxomas are soft polypoid masses that show an infiltrative growth but lack metastases. H ISTOPATHOLOGIC FEATURES Histopathologically, the tumor is composed of stellate or spindle-shaped cells and either thin or thick hyalinized vascular channels immersed within a myxoid matrix that is rich in collagen bundles (Fig. 15). There is no cellular atypia or mitotic figures. Immunohistochemically, the stromal cells of aggressive angiomyxoma express vimentin, muscle specific actin, and α-smooth muscle actin (2,3,5–9). In addition, some studies have demonstrated that the stromal cells were immunoreactive for desmin (3,5,7,9), whereas myosin, CD34, factor VIII-related antigen, α1-antitrypsin, α1-antichymo- trypsin, S-100 protein, neuron-specific enolase, estrogen and progesterone receptors, and chromogranin were negative. Electron microscopic examination showed fibroblast-like (2) or myofibroblast-like cells (1). Recently, Fletcher et al. (10) have described angiomyofibroblastoma of the vulva, a benign neoplasm distinct from aggressive angiomyxoma. According to these authors, angiomyofibroblastoma is smaller, better circumscribed, more cellular, and has a more abundant vascular component than aggressive angiomyxoma. Immunohistochemically, the stromal cells of angiomyofibroblastoma are desmin-positive and actin-negative (10). T REATMENT Wide local excision is recommended for aggressive angiomyxoma, but local recur- rence is frequent because of the difficulty of excising of this poorly circumscribed neo- plasm completely. Fig. 14. Clinical features of aggressive angiomyxoma. A poorly circumscribed nodular lesion on the pubis of an elderly woman. 10/Sangüeza/275-298/F 01/14/2003, 4:15 PM296 Chapter 10 / Other Neoplasms 297 Fig. 15. Histopathologic features of aggressive angiomyxoma. (A) Low power shows a poorly circumscribed nodular lesion with a myxoid appearance involving the entire thickness of the dermis. (B) Spindle cells embedded in a myxoid stroma. (C) Abundant dilated blood vessels are another component of the lesion. References 1. Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum: report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm. Am J Surg Pathol 1983;7:463–75. 2. Begin LR, Clement PB, Kirk ME, et al. Aggressive angiomyxoma of pelvic soft part: a clinicopathologic study of nine cases. Hum Pathol 1985;16:621–8. 3. Manivel C, Steeper T, Swanson P, Wick M. Aggressive angiomyxoma of the pelvis: an immuno- peroxidase study [Abstract]. Lab Invest 1987;56:46A. 10/Sangüeza/275-298/F 01/14/2003, 4:15 PM297 298 Sangüeza and Requena / Pathology of Vascular Skin Lesions 4. Skalova A, Michal M, Husek K, Zamecnik M, Leivo I. Aggressive angiomyxoma of the pelvioperineal region. Immunohistochemical and ultrastructural study of seven cases. Am J Dermatopathol 1993;15:446–51. 5. Fetsch JF, Laskin WB, Lefkowitz M, Kindblom LG, Meis-Kindblom JM. Aggressive angiomyxoma. A clinicopathologic study of 29 female patients. Cancer 1996;78:79–90. 6. Tsang WYW, Chan JKC, Lee KC, et al. Aggressive angiomyxoma: a report of four cases occurring in men. Am J Surg Pathol 1992;16:1059–65. 7. Clatch RJ, Drake WK, Gonzalez JG. Aggressive angiomyxoma in men: a report of two cases associated with inguinal hernias. Arch Pathol Lab Med 1993;117:911–3. 8. Iezzoni JL, Fechner RE, Wong LS, Rosai J. Aggressive angiomyxoma in males. A report of four cases. Am J Clin Pathol 1995;104:391–6. 9. Fetsch JF, Laskin WB, Lefkowitz M, et al. Aggressive angiomyxoma: a clinicopathologic study of 26 cases [Abstract]. Mod Pathol 1995;8:89A. 10 Fletcher CDM, Tsang WYW, Fisher C, Lee KC, Chan JKC. Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma. Am J Surg Pathol 1992;16:373–82. 10/Sangüeza/275-298/F 01/14/2003, 4:15 PM298 Chapter 11 / Mistaken Vascular Neoplasms 299 299 11 Disorders Erroneously Considered as Vascular Neoplasms CONTENTS KIMURA’S DISEASE “MALIGNANT” ANGIOENDOTHELIOMATOSIS (INTRAVASCULAR LYMPHOMATOSIS) A CRAL PSEUDOLYMPHOMATOUS ANGIOKERATOMA IN CHILDREN (APACHE) Table 1 summarizes a series of disparate disorders that have been erroneously consid- ered as vascular neoplasms and are described in this chapter. 1. KIMURA’S DISEASE CLINICAL FEATURES Kimura’s disease is an inflammatory disorder of the soft tissue that consists of prolif- erations of lymphoid and angiomatous tissues accompanied by lymphadenopathy, peripheral blood eosinophilia, and elevated IgE levels. Kimura et al. (1) originally described the disorder in 1948 as “an unusual granulation combined with hyperplastic changes of lymphatic tissue.” Subsequently, several reports of the disorder appeared in the literature, mostly in Oriental patients (2–8). It usually presents as a massive subcu- taneous swelling (Fig. 1) preferentially located in periauricular and submandibular regions of young men. The rare occurrence of Kimura’s disease in Caucasians caused confusion between Kimura’s disease and angiolymphoid hyperplasia with eosinophilia. In fact, Wells and Whimster (9), in their original description of angiolymphoid hyperpla- sia with eosinophilia in 1969, linked this disorder to Kimura’s disease, considering angiolymphoid hyperplasia to be late stage Kimura’s disease. After that, these terms were used synonymously by several authors (10–15). Rosai et al. (16,17) were the first to note that Kimura’s disease and angiolymphoid hyperplasia with eosinophilia are two differ- Table 1 Disorders Erroneously Considered as Vascular Neoplasms Kimura’s disease “Malignant” angioendotheliomatosis (angiotropic or intravascular lymphoma) APACHE (acral pseudolymphomatous angiokeratoma of children) 11/Sangüeza/299-310/F 01/14/2003, 4:28 PM299 300 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig. 1. Clinical appearance of Kimura’s disease showing swelling of the left forehead of an adult man. ent clinicopathologic entities. Recent reports have also supported this opinion (2–6,8,18). According to these authors, the differential diagnosis between Kimura’s disease and angiolymphoid hyperplasia with eosinophilia can be established on the basis of both clinical and histopathologic features. Clinically, Kimura’s disease occurs mainly in young men who present with large swellings of the subcutaneous soft tissue. The disorder is frequently accompanied by enlarged regional lymph nodes, nephrotic syndrome, peripheral blood eosinophilia, and elevated serum IgE. This disease has a striking predilection for males. Patients with Kimura’s disease also have elevated levels of interleukin (IL)-4, IL-5, and IL-13 mRNA in peripheral blood mononuclear cells (19,20), and the Th2 cytokines probably play a role in the development of this process. In contrast, angiolymphoid hyperplasia with eosino- philia consists of papules or nodules with an angiomatous appearance which are located superficially and which bleed easily. It is not accompanied by either lymphadenopathy or peripheral blood eosinophilia, and the serum level of IgE is normal. In the cases reported, one patient had Kimura’s disease associated with ulcerative colitis (20), and another patient presented with lymphadenopathy, cutaneous nodules, and oral ulcer- ations (21). H ISTOPATHOLOGIC FEATURES Histopathologically, there are also several differences between angiolymphoid hyper- plasia and Kimura’s disease. Viewed at low power, angiolymphoid hyperplasia with eosinophilia appears as an angiomatous lesion, with thin- or thick-walled blood vessels lined by prominent endothelial cells with a “histiocytoid” (16) or “epithelioid” (22,23) appearance that characteristically show vacuolated cytoplasm. In contrast, low-power views of lesions of Kimura’s disease show as a subcutaneous inflammatory disorder, with lymphoid aggregates with prominent germinal center formation and infiltration of count- less numbers of eosinophils (Fig. 2). The blood vessels in lesions of Kimura’s disease are Fig. 2. (Opposite page) Histopathologic features of Kimura’s disease. (A) Low power shows prominent germinal center formation involving the entire thickness of the dermis. (B) Germinal centers show a central pale area and a peripheral ring of mature lymphocytes. (C) Numerous eosinophils are present in the infiltrate. 11/Sangüeza/299-310/F 01/14/2003, 4:28 PM300 Chapter 11 / Mistaken Vascular Neoplasms 301 11/Sangüeza/299-310/F 01/14/2003, 4:28 PM301 [...]... Pyogenic granuloma GLUT-1 and, 10 Pyogenic granuloma (PG) atypical, 99 clinical features, 105 106 histopathologic features, 106 108 immunohistochemistry, 107 intravascular, see Intravascular pyogenic granuloma 01/14/2003, 5:44 PM 318 Index nevus flammeus and, 106 spider angioma and, 73, 106 treatment, 109 QB-END 10, 8 Radiation-induced cutaneous angiosarcoma, 262 atypical vascular lesions and, 262 benign... pictures of all described vascular proliferations of the skin CONTENTS Embryology, Anatomy, and Histology of the Vasculature of the Skin Special Techniques for the Study of Vessels and Vascular Proliferations Classification of Cutaneous Vascular Proliferations Cutaneous Vascular Hamartomas Cutaneous Vascular Malformations Cutaneous Lesions Characterized by Dilation of Preexisting Vessels Cutaneous Vascular. .. Hyperplasias Benign Neoplasms Malignant Neoplasms Other Cutaneous Neoplasms With Significant Vascular Component Disorders Erroneously Considered as Vascular Neoplasms Index 90000 CD-ROM Included Current Clinical Pathology Pathology of Vascular Skin Lesions: Clinicopathologic Correlations ISBN: 1-5 882 9-1 8 2-0 E-ISBN: 1-5 925 9-3 6 0-7 humanapress.com 9 781588 291820 ... 11/Sangüeza/29 9-3 10/ F 304 01/14/2003, 4:28 PM Chapter 11 / Mistaken Vascular Neoplasms 305 Fig 3 Clinical features of intravascular lymphomatosis (A) Multiple nodules on the left flank of a young man (B) Subcutaneous nodules on the posterior left knee 11/Sangüeza/29 9-3 10/ F 305 01/14/2003, 4:28 PM 306 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig 4 Histopathologic features of intravascular... vessels (Continued) 11/Sangüeza/29 9-3 10/ F 309 01/14/2003, 4:28 PM 310 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig 5 Histopathologic features of APACHE (B) The infiltrates are composed of uniform lymphocytes, whereas the vessels show thick walls and prominent endothelial cells (C) Other areas show a predominant vascular component 11/Sangüeza/29 9-3 10/ F 310 01/14/2003, 4:28 PM Index 311... treatment of vascular proliferations of the skin, all generously illustrated with a wide-ranging array of clinical cases and histopathologic specimens FEATURES • Accompanying CD-ROM with color versions of all illustrations appearing in the book • A new system of classification and nomenclature for vascular neoplasms • Comprehensive and up-to-date guide to benign and malignant proliferations of the skin. .. MD PATHOLOGY OF VASCULAR SKIN LESIONS CLINICOPATHOLOGIC CORRELATIONS Omar P Sangüeza, MD Departments of Pathology and Dermatology, Wake Forest University School of Medicine, Winston-Salem, NC Luis Requena, MD Department of Dermatology, Fundación Jiménez Diaz, Universidad Autonoma, Madrid, Spain Cutaneous vascular proliferations are exceedingly common, and with the recent identification of new diseases,... systemic process of unknown etiology rather than a disorder of blood vessels An increased number of mast cells have been described in lesions of Kimura’s disease, and it seems that the number of mast cells and the vascularity of the lesion run parallel, with sparse numbers of mast cells and blood vessels in early lesions and a gradual increase in fully developed lesions (24) Late stage lesions of Kimura’s... embryology, histology, and anatomy of skin vasculature, as well as the use of special techniques for the study of vascular proliferations An accompanying CDROM provides precise color versions of all illustrations appearing in the book Comprehensive and practical, Pathology of Vascular Skin Lesions: Clinicopathologic Correlations provides dermatologists, pathologists, and skin researchers with a complete,... Vitiligo, 29 von Hippel-Lindau syndrome, 42 von Willebrand factor (vWF), 7–8, see also Factor VIII-related antigen Weibel-Palade bodies, 5 Wilson Jones’ angiosarcoma, see Cutaneous angiosarcoma of the face and scalp Wiskott-Aldrich syndrome, 161 Wyburn-Mason syndrome, 41 01/14/2003, 5:44 PM 320 Sangueza_Index_Final Index 320 01/14/2003, 5:44 PM COMPANION CD-ROM TO PATHOLOGY OF VASCULAR SKIN LESIONS: CLINICOPATHOLOGIC . (Continued) 11/Sangüeza/29 9-3 10/ F 01/14/2003, 4:28 PM309 310 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig. 5. Histopathologic features of APACHE. (B) The infiltrates are composed of uniform lym- phocytes,. 11 / Mistaken Vascular Neoplasms 301 11/Sangüeza/29 9-3 10/ F 01/14/2003, 4:28 PM301 302 Sangüeza and Requena / Pathology of Vascular Skin Lesions abundant but never reach the degree of proliferation. flank of a young man. (B) Subcutaneous nodules on the posterior left knee. 11/Sangüeza/29 9-3 10/ F 01/14/2003, 4:28 PM305 306 Sangüeza and Requena / Pathology of Vascular Skin Lesions Most of the