PATHOLOGY OF VASCULAR SKIN LESIONS - PART 7 potx

BENIGN VASCULAR PROLIFERATIONS IN IRRADIATED SKINBenign vascular proliferations are well recognized lesions in previously irradiatedareas of the skin.. The striking tufts of endothelial

30 Hisaoka M, Hashimoto H, Iwamasa T Diagnostic implication of Kaposi’s sarcoma-associated virus with special reference to the distinction between spindle cell hemangioendothelioma and Kaposi’s sarcoma Arch Pathol Lab Med 1998;122:72–6.

herpes-31 Yañez S, Val-Bernal JF, Mira C, Echevarria MA, González-Vela MC, Arce F Spindle cell dothelioma associated with multiple skeletal enchondromas: a variant of Maffucci’s syndrome Gen Diagn Pathol 1998;143:331–5.

hemangioen-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.

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 F EATURES

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.

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 F EATURES

Benign lymphangioendotheliomas appear histologically as delicate, thin-walled,endothelium-lined spaces entrapped between collagen bundles (Fig 38) The appearancemay be confined to the papillary dermis, but it can extend into the reticular dermis andsubcutaneous fat In superficial areas, the vascular channels are arranged horizontally,often becoming smaller and more convoluted at deeper levels The newly formed vesselsmay dissect preexisting vessels as well as adnexal structures of the dermis The vascularspaces are variably empty or occupied by proteinaceous material Some vessels showstromal papillary projections that call to mind papillary endothelial hyperplasia Eryth-rocytes and hemosiderin deposits are characteristically absent Endothelial cells arepresent in greater numbers in lesional vessels than in normal lymphatic channels Theymay 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, whichcombines 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 differentialdiagnosis can be substantiated by the absence of erythrocytes, hemosiderin deposits, andplasma cells in lesions of benign lymphangioendothelioma; their presence frequentlycharacterizes early lesions of Kaposi’s sarcoma Benign lymphangioendothelioma mayalso mimic low-grade angiosarcoma However, contrastingly, angiosarcoma occurs pre-dominantly on the face and scalp of elderly patients, and it bears atypical endothelial cellsthat form multilayers in less differentiated areas Extravasated erythrocytes, hemosiderindeposits, and a mixed or plasma-cell rich inflammatory infiltrate are common features inangiosarcoma

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).

Fig 38 Histopathologic features of benign lymphangioendothelioma (A) Irregular slit-like cular spaces are present involving different levels of the dermis (B) The vascular spaces appear

vas-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.

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.

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 tosis Cutis 2001;67:29–30.

kera-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.

15 BENIGN VASCULAR PROLIFERATIONS IN IRRADIATED SKIN

Benign vascular proliferations are well recognized lesions in previously irradiatedareas 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 F EATURES

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 iectases They result from acquired permanent dilation of lymphatic capillaries that haveappeared in areas of the skin affected by obstruction or destruction of the lymphatics It

telang-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 F EATURES

Under low magnification, the lesions appear as relatively well-circumscribed 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 vascularspaces, devoid of content, are lined by a discontinuous single layer of endothelial cellswith 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

capil-a single lcapil-ayer of endothelium, project into the lumincapil-a of the dilcapil-ated lymphcapil-atic The stromcapil-a

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.

consists of fibrillary collagen rich with spindle, or stellate, fibroblasts Nodular infiltrates

of lymphocytes with germinal centers are occasionally present in the vicinity of thedilated vascular channels Rarely, vascular proliferations are poorly circumscribed andfocally intermingled with irregular jagged vascular spaces that may permeate the entiredermis Endothelial cells line the latter inconspicuously Irregular slit-like vascular spacesmay 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, butthey do so only focally or not at all for CD34 Although a minority of newly formed vesselsshow an attenuated muscle layer, external to the endothelial cells, which has on occasionimmunoreactivity 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-differentiatedangiosarcoma 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.

cular proliferations, as induced by radiation, do not contain erythrocytes or hemosiderindeposits, or stromal plasma cells The striking tufts of endothelial cells and the intravas-cular papillary projections, evidenced in the vascular proliferations of irradiated skin, areabsent 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 theendothelial cells are monomorphous, have inconspicuous nucleoli, and lack mitotic fig-ures In contrast, the endothelial cells of an angiosarcoma may form stratified layers thatirregularly line anastomostic channels, to a degree not seen in the atypical vascularproliferations of irradiated skin.

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 tion therapy Cancer 1978;42:1989–91.

radia-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 therapy for breast carcinoma J Cutan Pathol 1995;22:164–7.

radio-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.

16 GLOMUS TUMORS

Glomus tumors are uncommon neoplasms that arise from modified smooth musclecells normally present in specialized arteriovenous shunts in acral sites, mainly the fin-gertips These anatomic structures, known as the Sucquet-Hoyer canals, contribute totemperature regulation Sucquet-Hoyer canals are lined by endothelial cells and possessseveral 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 F EATURES

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 minorpressure 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 Theyare termed glomangiomas descriptively in accordance with their angiomatous appearance

In contrast to the solitary glomus lesion, glomangiomas present during childhood as smallbluish 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 Merritt syndrome (28) Occasionally, glomangiomas present as a solitary telangiectatic plaque-like lesion (29) (Fig 43) Mounayer et al (30) recently reported seven patients

Kasabach-with multiple large facial plaque-like glomangiomas that mimicked common facialvenous 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 thewalls of the large tortuous venous channels

Glomangiomyomas consist of tumors composed of neoplastic cells that show a gradualtransition from glomus cells to elongated, mature smooth muscle cells (Fig 45).

H ISTOPATHOLOGIC F EATURES

Histopathologically, solitary glomus tumors are customarily solid, well-circumscribednodules 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.

nuclei and scant eosinophilic cytoplasm (Fig 46) Endothelium-lined vascular spacescreate a core in the center of some of these clusters The uniformity of the neoplastic cellsand their lack of pleomorphism are characteristic attributes of glomus tumors Some

lesions acquire a mucinous stroma (2) Abundant nerve fibers (10) and mast cells (31) are

seen in some solitary glomus tumors Rare histopathologic variants include (1) lesionswith large, hyperchromatic nuclei that probably represent a degenerative phenomenon

(32); (2) glomus tumors composed of glomus cells with abundant granular cytoplasm,

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.

which have been termed “oncocytic” glomus tumors (9,33,34); (3) intravascular glomus tumors (35,36); (4) intraneural glomus tumors (8,37); (5) epithelioid glomus tumor (38); and (6) infiltrating glomus tumors (39,40) The lesions termed glomangiomyomas con-

sist of glomus tumors in which there is an admixture of glomus cells and elongated,

mature smooth muscle cells (32) (Fig 47)

Histopathologically, glomangiomas are less well-circumscribed lesions than solitaryglomus tumors Some are made up of several nodules within the dermis Some have anappearance that calls to mind a hemangioma (Fig 48) These are composed of irregulardilated endothelium-lined vascular channels that contain red blood cells and, distinc-tively, have small intramural aggregations of glomus cells Glomus cells may form cords

or small clusters in adjacent stroma, but numerically they are sparse relative to theirnumbers in a solitary glomus tumor As stated, some lesions can be difficult to distinguishfrom a conventional hemangioma; this is particularly true when thrombosis and phlebo-lith formation occurs within the vascular channels of a glomangioma (Fig 49)

Glomus cells were once considered pericytes (31) However, ultrastructural studies

have demonstrated that the cells of the normal glomus, as well as the neoplastic cells ofboth the solitary and the multiple glomus tumors, contain pinocytotic vesicles and aresurrounded by a basal lamina Abundant numbers of myofilaments condense focally toform dense bodies within the cytoplasm, in testimony to the cell’s nature as a modified

smooth muscle cell (2,9,20,22,31,40–48).

Immunohistochemically, glomus cells express vimentin, muscle-specific actin, and

α-smooth muscle actin (36,49–55) Desmin positivity has been described by some authors

Fig 45 Clinical features of glomangiomyoma An exophytic and pedunculated lesion with an

angiomatous appearance on the back of an adult woman.

(51,52,56), but this finding has been not corroborated by others (36,48,53) Laminin and type IV collagen outline the glomus cells (51) Highly cellular glomus tumors can be mistaken for solid apocrine hidradenomas (50) However, immunohistochemistry resolves

this histopathologic dilemma since apocrine hidradenomas express immunoreactivity for

cytokeratins and carcinoembryonic antigen, whereas glomus tumors do not (50).

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Fig 46 Histopathologic features of subungual glomus tumor (A) Scanning power shows a mostly solid cellular proliferation (B) Higher magnification shows that the lesion is composed of clusters

of round monomorphous cells (C) Still higher magnification demonstrates that neoplastic

cells have large round or plump nuclei and scant eosinophilic cytoplasm, all characteristics of glomus cells.

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Fig 47 Histopathologic features of glomangiomyoma (A) Low power shows dense cellular proliferations involving the entire thickness of the dermis (B) Neoplastic cells show intermediate characteristics between glomus cells and mature smooth muscle cells (C) Cellular aggregates are

mostly solid, with inconspicuous vascular lumina.

Fig 48 (Opposite page) Histopathologic features of glomangioma (A) Low power shows cellular

aggregates and dilated vascular structures at different levels of the dermis (B) Higher tion demonstrates that the vascular channels are surrounded by round monomorphous cells (C)

magnifica-Still higher magnification demonstrates that the cells surrounding the vascular channels show characteristics of glomus cells.

Fig 49 Histopathologic features of large facial glomangioma mimicking a common facial venous malformation (A) Low power shows dilated vascular channels involving different levels of the dermis (B) Higher magnification demonstrates that these dilated vascular channels are surrounded

by clusters of glomus cells.

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