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Congenital Nonprogressive Hemangioma a Distinct Clinicopathologic Entity Unlike Infantile Hemangioma

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Congenital Nonprogressive Hemangioma a Distinct Clinicopathologic Entity Unlike Infantile Hemangioma STUDY Congenital Nonprogressive Hemangioma A Distinct Clinicopathologic Entity Unlike Infantile Hemangioma Paula E. North, MD, PhD; Milton Waner, MD; Charles A. James, MD; Adam Mizeracki, BS; Ilona J. Frieden, MD; Martin C. Mihm, Jr, MD Background: Infantile hemangiomas are common tu- Setting: A university-affiliated pediatric hospital. mors, distinctive for their perinatal presentation, rapid growth during the first year of life, and subsequent in- Main Outcome Measures: Histologic appearance, im- volution—and for their expression of a unique immu- munoreactivity for the infantile hemangioma–associated nophenotype shared by placental microvessels. Occa- antigens GLUT1 and LeY, and clinical behavior. sional “hemangiomas” differ from the classic form in presenting fully formed at birth, then following a static Results: Congenital nonprogressive hemangiomas dif- or rapidly involuting course. These congenitally fully de- fered from postnatally proliferating infantile hemangio- veloped lesions have generally been assumed to be clini- mas in histologic appearance and immunohistochemical cal variants of more typical, postnatally developing hem- profile. Distinguishing pathologic features of these tu- angiomas. This assumption has not been tested by rigorous mors were lobules of capillaries set within densely fi- histologic and immunophenotypic comparisons. brotic stroma containing hemosiderin deposits; focal lobu- lar thrombosis and sclerosis; frequent association with Objective: To compare the histologic and immunohis- multiple thin-walled vessels; absence of “intermingling” tochemical features of congenital nonprogressive heman- of the neovasculature with normal tissue elements; and giomas with those of typical, postnatally proliferating, lack of immunoreactivity for GLUT1 and LeY. hemangiomas. Conclusion: Congenital nonprogressive hemangiomas Design: All cellular vascular tumors resected from in- are histologically and immunophenotypically distinct from fants younger than 4 months at Arkansas Children’s Hos- classically presenting hemangiomas of infancy, unlikely pital, Little Rock, over the past 20 years (43 lesions from to be related to the latter in pathogenesis. 36 patients) were first characterized histologically and im- munohistochemically, then clinically by chart review. Arch Dermatol. 2001;137:1607-1620 NFANTILE (JUVENILE) hemangio- bers of our group2 have reported recently mas are the most common tu- that these congenital examples of other- mors of infancy, affecting ap- wise typical, postnatally proliferating, in- proximately 10% of children.1 fantile hemangiomas are histologically in- From the Departments of These benign vascular tumors distinguishable from classic, postnatally Pathology (Dr North and vary in location and extent of tissue in- presenting examples, including expres- Mr Mizeracki), Head and Neck I Surgery and Otolaryngology volvement, and thus in clinical impact, but sion of an unusual set of tissue-specific vas- (Dr Waner and Mr Mizeracki), share a remarkably predictable biological cular antigens uniquely shared by placen- 3 and Radiology (Dr James), the behavior. Lesions generally appear within tal microvessels. These antigens are not University of Arkansas for weeks after birth, proliferate rapidly dur- expressed by the normal vasculature of Medical Sciences and Arkansas ing the first year of life, and then sponta- skin or subcutis; or by a variety of benign Children’s Hospital, Little Rock; neously involute over a period of several vascular tumors that are histologically the Departments of Pediatrics years. Most begin as relatively inconspicu- similar to hemangioma (including pyo- and Dermatology, University of ous blanched or blushed macules, in some genic granuloma, tufted angioma, and in- California at San Francisco cases evident at birth, that then dramati- fantile kaposiform hemangioendothe- Medical Center, San Francisco cally expand as tumorlike masses. A mi- lioma); or by vascular malformations.3 Two (Dr Frieden); and the Department of Pathology, nority of infantile hemangiomas, per- of these antigens, the erythrocyte-type glu- Harvard University Medical haps 15%, are relatively prominent at birth, cose transporter protein GLUT1, and Lewis School and Massachusetts usually presenting as diffuse, plaquelike Y antigen (LeY), can be detected by im- General Hospital, Boston, Mass lesions that grow rapidly prior to their munohistochemical analysis using forma- (Dr Mihm). characteristic period of involution. Mem- lin-fixed, paraffin-embedded tissue speci- (REPRINTED) ARCH DERMATOL / VOL 137, DEC 2001 WWW.ARCHDERMATOL.COM 1607 ©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 METHODS medical record review. These clinical features included pa- tient sex and race; gestational history; age at resection; age when lesion was first noticed; biological behavior SPECIMENS (whether the lesion grew, regressed, or remained static post- natally); whether multiple lesions were present; gross con- We performed an initial database review, searching for all figuration of the lesion (eg, bulging tumor mass vs exo- surgical specimens archived at Arkansas Children’s Hos- phytic polyp vs diffuse infiltration or plaquelike expansion); pital, Little Rock, between January 1980 and December 2000, and whether the patient experienced complicating condi- for which the diagnosis contained the suffix “-angioma” (903 tions such as Kasabach-Merritt syndrome, heart failure, or cases). These were then restricted to examples resected from ulceration. patients younger than 4 months to exclude most noncon- genital lesions and enrich for “alarming” congenital le- IMMUNOHISTOCHEMICAL ANALYSIS sions, yielding a more manageable and focused group of 59 lesions from 50 patients. Hematoxylin-eosin–stained tis- Paraffin sections were deparaffinized, rehydrated, and sub- sue sections from these cases were then reviewed, and all jected to citrate buffer antigen retrieval, then protein- vascular and lymphatic malformations were excluded (15 blocked before incubation with primary antibodies to cases). This process yielded 44 cellular vascular lesions com- GLUT1 or LeY as previously described.3,4 Bound primary posed of proliferative, mitotically active vascular elements antibody was detected using a DAKO Corp (Carpinteria, resected from infants younger than 4 months. One case, Calif) LSAB+ peroxidase kit using DAB+ chromagen.4 Im- histologically consistent with infantile hemangioma, showed munoreactions for CD34 and ␣ smooth muscle actin were poor preservation of tissue antigenicity (GLUT1-positive performed similarly, but without antigen retrieval, using internal controls were nonreactive) and was subsequently monoclonal antibodies directed against CD34 (clone excluded, leaving a final total of 43 lesions from 36 pa- QBEnd/10, prediluted; Biogenex, San Ramon, Calif) or ␣ tients (1 patient had multiple lesions resected). smooth muscle actin (clone 1A4, DAKO N1584). Nega- During review of the hematoxylin-eosin–stained sec- tive controls were processed in parallel without primary tions, the lesions were comparatively evaluated by investi- antibody. Normal tissue immunoreactivities (perineu- gators blind to clinical history for a variety of general histo- rium and erythrocytes) provided internal positive con- logic features relevant to vascular proliferations, including trols for GLUT1. For LeY, sections of LeY-immunoposi- lobularity, endoneurial pseudoinvasion, lesional mitotic ac- tive oral mucosa were included in each run as positive tivity, endothelial cell atypia (eg, spindled or epithelioid shape controls. Immunoreactivities were scored blindly by the and/or abnormal mitotic figures), epithelial collarette forma- first author (P.E.N.) as none, weak, moderate,orintense tion, planes of tissue involvement, characteristics of the ac- (Ն controls). For LeY, occasional weak immunoreactivity companying larger vasculature, and the presence or absence in a perinuclear, hof-type pattern was discounted. Only of ulceration, thrombosis, hemorrhage, hemosiderin depo- membranous and/or diffuse cytoplasmic-membranous LeY sition, fibrosis, calcification, or necrosis. Histopathologic di- immunoreactivity was scored as positive. agnoses were rendered during the course of these reviews based on published criteria for infantile hemangioma,7 pyo- RADIOGRAPHIC ANALYSIS genic granuloma,8 vascular and lymphatic malformations,7 tufted angioma,9 and infantile kaposiform hemangioendo- Subsequent to the pathologic examinations, available ra- thelioma.10 No examples of infantile myofibromatosis (in- diographic imaging studies (ultrasound, computed tomog- fantile hemangiopericytoma), as defined by Mentzel et al,11 raphy, and magnetic resonance imaging) of all congenital were identified. Lesions that did not fit well into established nonprogressive lesions included in the study were re- diagnostic categories were initially grouped as other. viewed. Findings were compared with those characteris- Diagnoses, including other, were then correlated with tic of well-established vascular tumors and anomalies, in- GLUT1 and LeY immunoreactivites, and with selected clini- cluding infantile hemangiomas and vascular and lymphatic cal features determined (when available) by subsequent malformations, based on published criteria.12 mens, and thus are suitable for retrospective analyses of unusual, fully formed, congenital nonprogressive hem- archival material. Both GLUT1 and LeY are highly ex- angiomas have not been defined.
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