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Hemangioma of the testis: report of unusual occurrences of cavernous hemangioma in a fetus and capillary hemangioma in an older man.

We report two cases of hemangiomata of the testes which occurred in a 17-week-old fetus and a 73-year-old man. To our knowledge, these are the first reported cases of cavernous hemangioma of the testis in a fetus and capillary hemangioma of the testis in an older man. Although a hemangioma of the testis is rare, it should be considered in the differential diagnosis of a testicular tumor. Ann Diagn Pathol 5:80-83, 2001.

Adult↗

Imaging characteristics of two subtypes of congenital hemangiomas: rapidly involuting congenital hemangiomas and non-involuting congenital hemangiomas.

BACKGROUND: Common infantile hemangiomas (COMMON) occur in approximately 10% of infants by the age of 1 year, with a female predominance. Some hemangiomas can be fully developed at birth and are thus called congenital hemangiomas (CH). Within this population, two courses have been identified: rapidly involuting CH (RICH) and non-involuting CH (NICH). Little has been reported on the clinical prognosis and imaging features of these entities. OBJECTIVE: To describe the imaging characteristics of two subtypes of CH, i.e. RICH and NICH, and to compare them with COMMON. MATERIALS AND METHODS: We retrospectively gathered data on 26 children presenting with CH, i.e. lesions fully developed at birth. These lesions were divided into two groups according to the clinical course: suspected RICH (n=8) and suspected NICH (n=18). We used US, CT or MRI and angiography to identify the gross anatomy and structure and the vascularization. Imaging findings were compared with the clinical course and pathology results, when available. The imaging findings in these patients were compared retrospectively with those in 26 patients with COMMON randomly chosen from the database of our multidisciplinary clinic. RESULTS: When compared with COMMON imaging characteristics, NICH and RICH had distinctive features on US such as being heterogeneous (72% of NICH and 62.5% of RICH vs 42.3% of COMMON), visible vessels (72% of NICH and 62.5% of RICH vs 15.4% of COMMON), calcifications (17% of NICH and 37.5% of RICH vs no case of COMMON). On CT and/or MRI, we compared imaging features such as well-defined limits (67% of NICH and 60% of RICH vs 100% of COMMON), and fat stranding (29.4% of NICH and RICH vs 7.7% of COMMON). CONCLUSION: Distinctive imaging characteristics are observed in cases of CH with US findings of visible vessels and calcifications statistically significant.

Adolescent↗

Sclerosed hemangioma and sclerosing cavernous hemangioma of the liver: a comparative clinicopathologic and immunohistochemical study with emphasis on the role of mast cells in their histogenesis.

BACKGROUND/AIMS: Sclerosed hemangiomas of the liver are rare. To date, their histopathology, immunohistochemistry, and the role of mast cells (MC) in their histogenesis have not been systematically studied. PATIENTS/METHODS: Clinical, histopathologic and immunohistochemical features of 20 sclerosed hemangiomas were compared with those of 18 sclerosing cavernous hemangiomas. The number of MC was quantified and compared in all cases, using a tryptase immunostain. RESULTS: Compared to patients with sclerosed hemangiomas, those with sclerosing hemangiomas were younger (mean age, 63 versus 71 years); had larger tumors (mean 6 +/- 4.73 versus 3 +/- 2.2 cm); presented with a mass more frequently, and epigastric pain less frequently. Sclerosing hemangiomas, but not sclerosed hemangiomas, were more frequent in males than in females. Sclerosing hemangiomas occurred much more frequently in the right lobe than sclerosed hemangiomas. Sclerosing hemangiomas had less fibrosis, hyalinization, and elastic fibers than sclerosed hemangiomas (p = 0.00004). Numerous thick-walled blood vessels were a feature of sclerosed hemangiomas but not of sclerosing hemangiomas. Collagen IV, and laminin were more uniformly positive in sclerosing hemangiomas than in sclerosed hemangiomas. Increased immunoreactivity for smooth muscle actin was present in sclerosed hemangiomas more often than in sclerosing hemangiomas. FVIII-R Ag, CD34, and CD31 were more diffusely positive in sclerosing hemangiomas than in sclerosed hemangiomas. In sclerosing hemangiomas, the mean number of tryptase-positive MC per high power field (MC/HPF) varied from 8.25 +/- 6.23 in vascular areas to 1.6 +/- 4.01 in sclerotic areas. In comparison, the mean number of MC in sclerosed hemangiomas, was 4.3 +/- 5.01 in vascular areas, and 0.86 +/- 0.58 in sclerotic areas (p = 0.0095). The number of MC was significantly correlated with vascular proliferation and inversely related to the degree of fibrosis (p < 0.0001). CONCLUSIONS: This study demonstrates certain distinct clinical and histopathologic differences between sclerosing cavernous hemangiomas and sclerosed hemangiomas of the liver. We have established the presence of MC in those tumors, and suggest possible involvement of the MC in angiogenesis, and the regression process and development of fibrosis.

Age Factors↗

[Treatment of hemangiomas in infancy and childhood with the flash lamp-pumped dye laser: cutaneous versus mixed cutaneous-subcutaneous hemangiomas].

The efficacy of flashlamp-pumped pulsed dye laser therapy at 585 nm for cutaneous and mixed hemangiomas was assessed in 165 children with 225 separate hemangiomas were treated in 332 sittings over 2.5 years. The patients were divided into three groups: 100 patients with 153 flat cutaneous hemangiomas, 47 patients with 54 mixed cutaneous-subcutaneous hemangiomas and 18 patients with 18 superficial hemangiomas in the involution phase. In the first group of 100 patients with 153 flat cutaneous hemangiomas, 52 (34%) of the lesions showed total lightening. 21 of the 153 lesions showed proliferation of the subcutaneous component, although these lesions were flat at first presentation. Of the 54 mixed hemangiomas, 33 (61%) had continued proliferation of the subcutaneous component. The cutaneous component responded to therapy in 21 (39%) hemangiomas while the subcutaneous component of the mixed hemangiomas remained unchanged. No lesions in this group involuted completely and therapy was discontinued because of a relatively poor response to therapy. 12 (67%) of the 18 patients with superficial hemangiomas in the involution phase had excellent results, and 6 (33%) had good results. We conclude that treatment with the flashlamp-pumped pulsed dye laser is very effective and may be the treatment of choice for selected hemangiomas, especially cutaneous hemangiomas at sites of potential functional impairment (hands or feet, anogenital-region), and on the face. Furthermore, laser therapy should be initiated as early as possible, even in the first days or weeks of life, when the hemangioma is flat, to prevent enlargement, promote involution, or eliminate these vascular lesions.

Age Factors↗

Flashlamp-pumped pulsed dye laser for hemangiomas in infancy: treatment of superficial vs mixed hemangiomas.

OBJECTIVE: To study in a compared manner the efficacy of flashlamp-pumped pulsed dye laser (FPDL) therapy for superficial and mixed hemangiomas. DESIGN: Nonrandomized control trial. SETTING: Department of Lasermedicine, General Hospital Neukölln, Berlin, Germany. PATIENTS: To investigate variation in response to treatment, a prospective study of 165 children with 225 separate hemangiomas treated with the FPDL was undertaken. Patients were aged 2 days to 7 years; mean follow-up was 5 months. INTERVENTIONS: During a 2 1/2-year period, we administered 332 treatments, for a mean+/-SD of 2.0+/-1.1 treatments per patient. MAIN OUTCOME MEASURE: Patients received therapy until the lesion was almost clear or until the lesion did not respond to treatment. Evaluation was performed by comparing pretreatment and posttreatment photographs. In addition, pathologic flow of vessels and thickness were determined before, during, and after completion of therapy with color-coded duplex sonography. RESULTS: In the first group of 100 patients with 153 flat cutaneous hemangiomas, 52 hemangiomas (34%) had excellent results; 80 (52%) had good results; and 21 (14%) showed proliferation of the subcutaneous component, although these lesions were flat at first presentation. Of the 54 mixed hemangiomas, 33 (61%) had continued proliferation of the subcutaneous component. The cutaneous component responded to therapy in 21 hemangiomas (39%), whereas the subcutaneous component of the mixed hemangiomas remained unchanged. No lesions in this group involuted completely, and therapy was discontinued because of relatively poor response. Twelve (67%) of 18 patients with superficial hemangiomas in the involution phase had excellent results and 6 (33%) had good results. CONCLUSIONS: Treatment with the FPDL is effective and may be the treatment of choice for superficial cutaneous hemangiomas at sites of potential functional impairment and on the face. Hemangiomas with a deep component do not benefit from FPDL treatment because the efficacy of the FPDL is limited by its depth of vascular injury. Furthermore, early therapeutic intervention with the FPDL may not prevent proliferative growth of the deeper or subcutaneous component of the hemangioma despite early intervention.

Child↗

Vascular tumors of the breast. II. Perilobular hemangiomas and hemangiomas.

Nearly 100 vascular tumors of the breast have been studied. Sixty-two were angiosarcomas lesions. Twenty-four patients with mammary hemangiomas are the subject of this report. Five of 11 microscopic perilobular hemangiomas, not clinically apparent (2 mm or less), were atypical and had nuclear hyperchromasia or focal anastomoses among vascular channels. Whether treated by excision or mastectomy, all 11 patients remain well with follow-up of up to 10 years. Fourteen clinically or grossly apparent macroscopic hemangiomas (0.3-2.5 cm) included eight characterized as cytologically atypical. With one exception, lesions designated hemangiomas were well circumscribed and tended to be divided into lobules. In more than half, origin from large, non-neoplastic "feeding" vessels that were seen branching into the lesion was demonstrated. Whether treated by excision or mastectomy, no hemangioma has recurred after follow-up, up to 5 years in some cases. The diagnosis of vascular tumors of the breast requires thorough microscopic study of the entire lesion. The majority are angiosarcomas. However, about one-third constitute a spectrum of apparently benign and atypical lesions, in which the single largest group are perilobular hemangiomas and hemangiomas. Size appears to be an important characteristic for distinguishing hemangiomas from angiosarcomas, as few lesions larger than 2 cm qualify as hemangiomas, whereas angiosarcomas are rarely smaller than 2 cm. It is possible that atypical perilobular hemangiomas or atypical hemangiomas are precursors to angiosarcoma, but this relationship remains to be demonstrated.

Adolescent↗

A novel animal model for hemangiomas: inhibition of hemangioma development by the angiogenesis inhibitor TNP-470.

Hemangiomas represent the most frequent tumors of infancy. However, the pathogenesis of these tumors is still largely unknown, and current treatment of juvenile hemangiomas remains unsatisfactory. Here we present a novel animal model to study proliferating hemangiomas and to evaluate the effect of angiostatic compounds on their growth. Intraperitoneal (i.p.) infection of 4-day-old rats with murine polyomavirus resulted in the development of multiple cutaneous, intramuscular (i.m.), and cerebral hemangiomas with 100% frequency. Histological examination of the brain revealed the formation of immature lesions as soon as 4 days postinfection (p.i.). The subsequent exponential growth of the hemangiomas, both in number and size, was associated with severe hemorrhage and anemia. The cerebral, cutaneous, and i.m. lesions consisted of blood-filled cysts, histologically similar to human cavernous hemangiomas and stained positive for proliferating cell nuclear antigen, urokinase-type plasminogen activator, and vascular endothelial growth factor. Mature cerebral hemangiomas also expressed von Willebrand factor. Cerebral lesions caused death of the untreated animals within 19.2 +/- 1.1 days p.i. Remarkably fewer and smaller hemangiomas developed in animals that had been treated s.c. with the angiogenesis inhibitor TNP-470. Accordingly, TNP-470 (50 mg/kg), administered twice a week from 3 days p.i., significantly delayed tumor-associated mortality [mean day of death, 28.2 +/- 3.3 (P < 0.001)]. Even if therapy was initiated when cerebral hemangiomas were already macroscopically visible (i.e., 9 days p.i.), a significant delay in hemangioma-associated mortality was observed. Also, the IFN-inducer polyinosinic-polycytidylic acid caused a delay of 9 days (P < 0.005) in tumor-associated mortality when administered i.p. at 5 mg/kg, twice a week, starting at day 3 p.i. The model described here may be useful for investigating (a) the angiogenic mechanism(s) underlying hemangioma progression; and (b) the effect of anti-angiogenic compounds on vascular tumor growth.

Animals↗

Congenital nonprogressive hemangioma: a distinct clinicopathologic entity unlike infantile hemangioma.

BACKGROUND: Infantile hemangiomas are common tumors, distinctive for their perinatal presentation, rapid growth during the first year of life, and subsequent involution-and for their expression of a unique immunophenotype shared by placental microvessels. Occasional "hemangiomas" differ from the classic form in presenting fully formed at birth, then following a static or rapidly involuting course. These congenitally fully developed lesions have generally been assumed to be clinical variants of more typical, postnatally developing hemangiomas. This assumption has not been tested by rigorous histologic and immunophenotypic comparisons. OBJECTIVE: To compare the histologic and immunohistochemical features of congenital nonprogressive hemangiomas with those of typical, postnatally proliferating, hemangiomas. DESIGN: All cellular vascular tumors resected from infants younger than 4 months at Arkansas Children's Hospital, Little Rock, over the past 20 years (43 lesions from 36 patients) were first characterized histologically and immunohistochemically, then clinically by chart review. SETTING: A university-affiliated pediatric hospital. MAIN OUTCOME MEASURES: Histologic appearance, immunoreactivity for the infantile hemangioma-associated antigens GLUT1 and LeY, and clinical behavior. RESULTS: Congenital nonprogressive hemangiomas differed from postnatally proliferating infantile hemangiomas in histologic appearance and immunohistochemical profile. Distinguishing pathologic features of these tumors were lobules of capillaries set within densely fibrotic stroma containing hemosiderin deposits; focal lobular thrombosis and sclerosis; frequent association with multiple thin-walled vessels; absence of "intermingling" of the neovasculature with normal tissue elements; and lack of immunoreactivity for GLUT1 and LeY. CONCLUSION: Congenital nonprogressive hemangiomas are histologically and immunophenotypically distinct from classically presenting hemangiomas of infancy, unlikely to be related to the latter in pathogenesis.

Female↗

Patterns of infantile hemangiomas: new clues to hemangioma pathogenesis and embryonic facial development.

OBJECTIVES: Large facial infantile hemangiomas have higher rates of complications than small localized hemangiomas, more often require treatment, and can be associated with neurological, ophthalmologic, and cardiac anomalies (PHACE syndrome). The anatomic patterns of these hemangiomas are often referred to as "segmental" despite a lack of precise anatomic definitions. Our study aims to define "segmental" hemangiomas based on clinically observed patterns. Our secondary goal is to relate the observed patterns to currently accepted developmental patterns to gain insight into hemangioma pathogenesis and craniofacial development. METHODS: Photographic data were extracted from a large cohort of patients with infantile hemangiomas. We mapped 294 hemangiomas and recorded common morphologic patterns. Anatomic descriptions of the most common patterns were described and compared with accepted concepts of craniofacial development. RESULTS: Four primary segments were identified (Seg1-Seg4). Seg2 and Seg3 correspond with the previously recognized maxillary and mandibular prominences. Seg1 and Seg4 differ from standard human embryology texts. The frontotemporal segment, Seg1, encompasses the lateral forehead, anterior temporal scalp, and lateral frontal scalp. The segment Seg4, encompassing the medial frontal scalp, nasal bridge, nasal tip, ala, and philtrum, is substantially narrower on the forehead than the previously described frontonasal prominence. CONCLUSIONS: The patterns provide new clues regarding facial development. The observed patterns resemble previously described facial developmental units on the lower face but are distinctly different on the upper face. The patterns suggest that neural crest derivatives may play a role in the development of facial hemangiomas. Finally, these patterns (Seg1-Seg4) help standardize the nomenclature of facial segmental hemangiomas to analyze more effectively hemangioma risks and behavior.

Face↗

Congenital hemangiomas and infantile hemangioma: missing links.

Rapid postnatal growth and slow involution in childhood characterize the common infantile hemangioma. There are other rare vascular tumors that present fully grown at birth and behave quite differently, as designated by the acronyms: rapidly involuting congenital hemangioma (RICH) and noninvoluting congenital hemangioma (NICH). RICH and NICH have similarities in appearance, location, size, equal sex ratio, and both have overlapping radiologic and histologic features with infantile hemangioma. However, neither type of congenital tumor immunostains for glucose transporter-1 protein, a marker of infantile hemangioma. This raises the question of whether these congenital vascular lesions are variations in a spectrum of hemangioma or are entirely different tumors. We describe two groups of patients that suggest a linkage between postnatal and congenital vascular tumors: Link I (n=5), children who had either RICH or NICH coexisting with infantile hemangioma, and Link II (n=10), children initially diagnosed as having RICH, but regression was incomplete and the residuum was that of NICH. We conclude that these infants exhibit "missing links" between the rare RICH and NICH, and the common infantile hemangioma.

Female↗

[Inner-hemangioma ligation and pingyangmycin injection in hemangioma (with 30 cases report)].

OBJECTIVE: To provide a simple and safe surgical approach in treating cavernous and racemose hemangioma. METHODS: First cross-ligate the hemangioma, as to divide the tumor into independent compartments, them inject pingyangmycin into the compartments respectively. RESULTS: There are 30 cases in this group (35 hemangiomas). Twenty-four cases (28 hemangiomas) were totally cured, accounting for 80%; Six cases were improved (7 hemangiomas) for 20%; There was no ineffective case. CONCLUSION: "Inner-hemangioma ligation and injection of pingyangmycin" has broad surgical indications, with desirable outcome in treating cavernous and racemose hemangioma, especially those unsuitable for surgery, or ineffective simply by inner-hemangioma injection.

Adolescent↗

[Radiotherapy of blastomatous hemangiomas - under special consideration of 44 eyelid hemangiomas (author's transl)].

The propriety of blastomatous hemangiomas to show spontaneous regression is underlined. Under certain indications, above all in case of eyelid hemangiomas, a radiotherapy is indicated. The moment of manifestation lies within the first three months of life, and the female sex is affected two times more frequently. The head represents the predilection spot within the distribution scheme of hemangiomas. 44 patients with eyelid hemangiomas who had been treated between 1968 and 1972 by the described irradiation method had check-up appointments five years later. The therapeutical results were evaluated according to certain criteria. 95,5% of the blastomatous hemangiomas showed a very good or satisfactory regression. Only 4,5% did not present any satisfactory success. In an advanced age of the patients, the hemangiomas do not always respond favourably to radiotherapy. In case of a strictly executed indication and a faultless irradiation technique, irradiation damages can be avoided. Our results show that, with regard to the spontaneous regression of blastomatous hemangiomas, total doses of 1000 to 1500 rd are sufficient. Finally the authors cite the standpoints of radiotherapy concerning the treatment of blastomatous hemangiomas and give recommendations for dosage principles.

Age Factors↗

Orbital cavernous hemangioma associated with juxtapapillary exophytic retinal capillary hemangioma.

A 32-year-old man presented with coexisting orbital hemangioma and juxtapapillary exophytic capillary hemangioma. Fluorescein and indocyanine green angiography revealed exophytic juxtapapillary retinal capillary hemangioma. The orbital cavernous hemangioma was surgically removed, and the retinal capillary hemangioma was observed without treatment. Subretinal hemorrhage gradually absorbed and clinical symptoms improved. Juxtapapillary exophytic capillary hemangioma can cause subretinal bleeding secondary to external pressure from an adjacent orbital cavernous hemangioma. Coexistence of both tumors suggests a common etiology.

Adult↗

Congenital hemangiomas: rapidly involuting and noninvoluting congenital hemangiomas.

While infantile hemangiomas are a very common lesion seen in infants and young children, congenital hemangiomas are much more rare and have been only recently described. Two types of congenital hemangiomas exist: rapidly involuting congenital hemangiomas and noninvoluting congenital hemangiomas. The goal of this article is to describe rapidly involuting and noninvoluting congenital hemangiomas as they differ from infantile hemangiomas in their presentation, natural history, histopathologic features, and treatment.

Age Factors↗

Acquired elastotic hemangioma: A clinicopathologic variant of hemangioma.

BACKGROUND: In recent years, many new cutaneous vascular proliferations have been described. This is partly due to the current epidemic of AIDS, which has forced dermatopathologists to establish the diagnosis of the earliest patch-stage lesions of AIDS-related Kaposi's sarcoma and some of the recently described cutaneous vascular proliferations that may histopathologically mimic lesions of early Kaposi's sarcoma. OBJECTIVE: This article reports the clinical, histopathologic, and immunohistochemical findings in 6 cases of a clinicopathologic variant of cutaneous hemangioma, which we have named acquired elastotic hemangioma. METHODS: Six cases of acquired elastotic hemangioma were studied from clinical, histopathologic, and immunohistochemical standpoints. The histopathologic differential diagnosis with other similar cutaneous vascular proliferations is discussed. RESULTS: Acquired elastotic hemangioma appears on the sun-damaged skin of the dorsal aspect of the forearms or on the lateral aspects of the neck of middle-aged or elderly women. Clinically, lesions present as solitary erythematous plaques with variable morphology and in only some cases have a clearly angiomatous appearance. On histopathologic examination, acquired elastotic hemangioma is characterized by a band-like proliferation of capillary blood vessels involving the superficial dermis and arranged horizontally parallel to the epidermis. CONCLUSION: Acquired elastotic hemangioma is a distinctive clinicopathologic variant of hemangioma that should be differentiated from other cutaneous vascular proliferations. On histopathologic examination, it is characterized by capillary proliferation involving the dermis.

Aged↗

Hobnail hemangiomas (targetoid hemosiderotic hemangiomas) are true lymphangiomas.

BACKGROUND: Hobnail hemangioma (targetoid hemosiderotic hemangioma) is a small benign vascular tumor of the superficial and mid-dermis. In contrast to its well-characterized histology, it has been unclear whether this tumor arises from blood vessel endothelial cells (BECs) or lymphatic vessel endothelial cells (LECs). METHODS: We analyzed 10 hobnail hemangiomas by immunohistochemistry, using the recently described lymphatic endothelial cell marker, D2-40. For comparison, CD31, CD34, and alpha-smooth muscle actin expression were studied in consecutive sections of the paraffin-embedded tissues. RESULTS: In all analyzed vessels, D2-40 labeled exclusively LECs, whereas BECs were consistently negative. In contrast to capillary BECs, either neighboring the tumors or intermingled, neoplastic endothelial cells of all 10 hobnail hemangiomas were strongly labeled by D2-40. CONCLUSIONS: The results suggest a lymphatic origin for hobnail hemangiomas. This view is further supported by the CD34 negativity of endothelial cells and the lack of actin-labeled pericytes in hobnail hemangiomas, both characteristic of lymphatic vessels. Moreover, our analysis revealed that microshunts between neoplastic lymphatic vascular channels and small blood vessels occur, explaining some features of hobnail hemangiomas, such as aneurysmatic microstructures, erythrocytes within and beneath neoplastic vascular spaces, inflammatory changes, scarring, and interstitial hemosiderin deposits.

Actins↗

Ipsilateral orbital cavernous hemangioma and choroidal hemangioma.

PURPOSE: A unique case of simultaneous ipsilateral choroidal and orbital hemangiomas is described. METHODS: A 71-year-old man was found to have a left orbital intraconal mass while being worked up for intractable headache. He also had a left juxtapapillary choroidal hemangioma which did not cause any leakage to macular area. RESULTS: The left intraconal tumor was removed by medial transconjunctival orbitotomy. Histopathological examination revealed cavernous hemangioma. The ipsilateral choroidal hemangioma was managed by observation only. During 15 months of follow-up, there has been no growth or accumulation of subretinal fluid. CONCLUSIONS: The occurrence of orbital cavernous and choroidal hemangiomas on the same side suggests common pathogenic derangement acting on two different sites.

Aged↗