Lobules Of Capillaries Research Articles

The Interposed Smudge-Kaposiform Haemangioendothelioma

Kaposiform hemangioendothelioma emerges as an exceptionally discerned endothelial cell neoplasm. Commonly, tumefaction disseminates within entire dermis with expansion into the subcutaneous tissue and exhibits localized tumour aggressiveness. Characteristically, proliferation of spindle shaped endothelial cells is commingled with lymphatic vessels demonstrating capillary haemangioma-like articulations. Glomeruloid structures are configured of discrete capillary lobules demonstrating a swirling pattern. Nodules of spindle shaped endothelial cells are encompassed by peripheral lymphatic vessels articulating lymphangioma-like areas. Tufted angioma configures as a benign, superficial, vascular neoplasm arising as a congenital lesion or appears within infants and paediatric population. The aggressive neoplasm is associated with enhanced morbidity and may undergo spontaneous retrogression, in contrast to Kaposiform hemangioendothelioma.

Perinatal Presentation of a Congenital Intramedullary Capillary Hemangioma in a Neonate Born with Hydrocephalus and Paraplegia.

Spinal intramedullary capillary hemangioma is a rare condition. Although most spinal/spinal cord hemangiomas are inborn in origin, perinatal presentation is uncommon. We present a neonate with an intramedullary hemangioma, born with communicating hydrocephalus and complete paraplegia. Spinal imagining showed an intradural mass with hemorrhagic foci, a low-lying conus medullaris, and scalloping of dorsal elements. Ventriculoperitoneal shunting was performed in an emergent setting, with cerebrospinal fluid showing a brownish appearance with high viscosity which implied a possible old hemorrhage. Tissue sample was taken from the spinal lesion in an elective setting. Histopathological examination revealed capillary lobules and extralobular large vessels resembling abnormal veins. The mentioned clues suggested that this congenital hemangioma had become symptomatic from the prenatal period. Though it is a are event, it should be kept in mind while evaluating a neonate with communicating hydrocephalus and weakness of extremities with or without cutaneous hemangiomatous stigmata.

The histopathology of congenital haemangioma and its clinical correlations: a long-term follow-up study of 55 cases.

Congenital haemangiomas (CHs) can be subdivided into different subtypes [rapidly involuting CHs (RICHs), non-involuting CHs (NICHs), and partially involuting CHs (PICHs)]. During the first few days of life, RICHs may be associated with transient but sometimes marked thrombocytopenia. We sought to assess the histological aspects and clinicopathological correlations of the three subtypes. We assessed the histopathological features of 10 RICHs, 25 NICHs, and 20 PICHs, described the patients' long-term clinical outcomes, and assessed clinicopathological correlations. All CHs were located in the dermis and hypodermis, and comprised both capillary lobules (with three distinct histopathological patterns) and extralobular large vessels. Most of the extralobular vessels were abnormal veins and abnormal lymphatic vessels. We did not observe significant correlations between the CH subtype, the histopathological pattern, and the time of the histopathological assessment. Interestingly, unexpected intralobular expression of podoplanin was found in neonatal biopsies of five RICHs and PICHs. Four of these five patients had concomitant thrombocytopenia. The podoplanin staining intensity decreased over time as the thrombocytopenia resolved and the tumour shrank. The histopathological features were similar in all three subtypes of CH, and were related to the time since disease onset; we consider that RICH, PICH and NICH form a single entity and differ only in their involuting potential. Along with the transient expression of intralobular podoplanin observed in some specimens from the newborn, the lobular architecture might lead to misdiagnosis of tufted haemangioma or kaposiform haemangioendothelioma.

Hemangioma with dabskoid features: a rare histopathologic variant of acquired hemangioma

Dabska tumor, also known as papillary intralymphatic angioendothelioma (PILA), is a locally aggressive hemangiendothelioma characterized by intravascular papillary proliferations of atypical endothelial cells. Besides PILA, papillary tufts lined by hobnail endothelial cells have been rarely described in vascular proliferations. We report two cases of acquired hemangiomas, which focally showed this finding. We present a 15-year-old male and a 7-year-old girl with erythematous nodules. Both lesions were composed of capillary lobules intermingled with large sinusoidal spaces lined with a single layer of flat endothelial cells, which focally developed intravascular papillary proliferations lined by plump hyperchromatic endothelial cells and a central connective tissue core. Both types of cells were positive with CD31 and ERG and negative for Lyve-1, Prox-1 and podoplanin. Wilm's tumor 1 marker was strongly positive in the capillary hemangioma areas while negative in the intravascular tufts. Both lesions recurred after the first excision but we did not observe further recurrence or evidence of metastasis in the follow-up. In summary, our cases expand the histopathologic findings that may be seen in conventional acquired capillary hemangiomas. The focal presence of dabskoid tufts within an otherwise conventional capillary hemangioma should be not misinterpreted as evidence of malignancy.

Adult-Onset Tufted Angiomas Associated With an Arteriovenous Malformation in a Renal Transplant Recipient

Tufted angioma (TA) is a rare benign vascular neoplasm characterized histopathologically by the proliferation of endothelial cells arranged in lobules in the dermis and subcutaneous fat. To date, about 200 cases have been reported, most of which are of Japanese ethnicity. TA predominantly affects children and young adults, developing in 80% of patients younger than 10 years. A white 72-year-old renal transplant recipient presented with 2 asymptomatic dusky red papules on his right leg. The lesions appeared 5 years after the start of immunosuppressive treatment. Histopathologic examination showed a proliferation of poorly canalized capillary-sized vascular structures with typical "cannonball" pattern in the dermis and subcutaneous fat. Eccrine glands were also evident focally in the stroma of capillary lobules. On immunohistochemistry, endothelial cells in the vascular tufts stained positive for CD31 and CD34 but were negative for factor VIII-related antigen, human herpes virus 8, and podoplanin (clone D2-40); α-smooth muscle actin stained pericytes disposed in a single layer in capillary-sized vessels and in 2-3 or more layers in vessels of larger size, respectively. The microscopic findings were suggestive of TA. In the deep dermis, venules with smooth muscle wall and arterioles, as shown by Van Gieson staining, normally not found at that level, were present and appeared surrounded by capillary lobules. Onset of TA in adulthood is rare and may be associated with pregnancy, varicella zoster virus infection, and pharmacological immunosuppression. A case of acquired adult-onset TA associated with an arteriovenous malformation in an elderly transplanted patient is described.

Intramuscular capillary-type hemangioma: radiologic–pathologic correlation

Infantile hemangiomas demonstrate a pattern of proliferative growth in infancy followed by a slow phase of involution. In contrast a rare type of vascular tumor, intramuscular capillary-type hemangioma, usually presents beyond the period of infancy with nonspecific symptoms and no evidence of involution. The purpose of this study was to characterize the clinical, imaging, histopathological characteristics and management of intramuscular capillary-type hemangioma. We performed a retrospective review of a 20-year period to identify children diagnosed with intramuscular capillary-type hemangioma. Patient demographics, imaging and histopathological findings were recorded. We included 18 children (10 boys, 8 girls) with histologically proven intramuscular capillary-type hemangioma - and adequate imaging. The mean age at presentation was 8.1years (range 1day to 19years). Twelve lesions involved muscles of the extremities, 4 were located in the trunk and 2 were in the head and neck. MRI had been performed in all children and demonstrated a soft-tissue mass with flow voids, consistent with fast flow. The lesion was well-circumscribed in 16 children and intralesional fat was seen in 14. Doppler US demonstrated a heterogeneous lesion, predominantly isoechoic to surrounding muscle, with enlarged arterial feeders. Enlarged feeding arteries, inhomogeneous blush and lack of arteriovenous shunting were noted on angiography (n = 5). The most common histopathological findings were lobules of capillaries with plump endothelium and at least some adipose tissue. The lesions were excised in six children. Two children were lost to follow-up. In the remaining 10, follow-up MRI studies ranging from 3months to 10years showed that the lesion enlarged in proportion to the child (n = 7), demonstrated slow growth (n = 2) or remained stable (n = 1). There was no change in imaging characteristics on follow-up. Intramuscular capillary-type hemangioma is a rare benign vascular tumor of skeletal muscle. The most typical imaging features show a heterogeneous intramuscular mass with fast flow, and intralesional fat. Although the lesion is relatively stable in appearance over time, imaging does not obviate the need for a biopsy to rule out sarcoma. The diagnosis can usually be established by typical findings on histopathology.

How Airway Venous Malformations Differ From Airway Infantile Hemangiomas

To compare airway infantile hemangiomas (IHs) and venous malformations (VMs) clinically, radiographically, endoscopically, and histologically. Retrospective cohort study. Tertiary care pediatric hospital. The study included patients seen in the Vascular Anomaly Clinic, Seattle Children's Hospital, Seattle, Washington, between 2001 and 2008. All patients with airway vascular anomalies were identified by searching the Vascular Anomaly Quality Improvement Database and hospital discharge data. The data, which were analyzed with descriptive statistics and the Fisher exact test, included presenting age, sex, presenting signs, lesion site, and radiographic, endoscopic, and histologic findings.. Seventeen patients with airway lesions were identified, 6 with VMs and 11 with IHs. Patients with VMs presented at a mean (SD) age of 11.3 (13.7) months (age range, 3-39 months), while those with IHs presented at 3 (1.8) months of age (age range, 1-6 months) (P=.03). The patients with IHs were predominantly female (9 of 11 [81%]), while no sex difference was noted among the patients with VMs (3 of 6 [50%]). All patients with IHs presented with stridor and cutaneous lesions, whereas patients with VMs more often presented with hemoptysis or dysphagia (P=.001). Computed tomographic angiograms demonstrated enhancing endolaryngeal lesions in all IHs, while VMs enhanced poorly. Endoscopically, IHs were transglottic, while VMs were postcricoid or epiglottic (P<.001). Histologically, immunostained lesions showed submucosal lobules of capillaries lined by GLUT-1 (glucose transporter isoform 1)-positive endothelium in IHs, whereas VMs consisted of loosely organized venous channels that lacked GLUT-1 staining. Patients with airway IHs and VMs differ in presenting age and signs, sex, airway lesion location, enhancement on computed tomographic angiograms, and histologic appearance.

Retroauricular intravascular lobular capillary haemangioma in a child

Retroauricular intravascular lobular capillary haemangioma in a child

Glomeruloid haemangioma is not always associated with POEMS syndrome

Glomeruloid haemangioma is considered a specific marker of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome and it is usually but not always associated with multicentric Castleman's disease. We report a 78-year-old man who presented with a single, red-violet soft nodule with superficial telangiectases on the scalp. Histopathologically, the lesion consisted of lobules of coiled aggregated capillaries that involved the lumina of dilated vascular structures, mimicking renal glomeruli. A collagenous stroma separated the capillary lobules, and eosinophilic, periodic-acid-Schiff positive globules of varying sizes and shapes were seen within the cytoplasm of endothelial cells. Immunohistochemical studies with antibodies against IgA and IgG, and against the kappa and lambda light chains of immunoglobulins showed immunoreactivity within the eosinophilic globules. Results of complete blood count, liver, renal and thyroid function tests, fasting blood sugar measurement, serum levels of oestradiol, testosterone, prolactin and cortisol, serum protein electrophoresis, immunoelectrophoresis and immunofixation yielded normal or negative results. No Bence-Jones proteinuria was found in a sample from a 24-h urine collection. To our knowledge, only two cases of glomeruloid haemangioma have been previously reported in patients without POEMS syndrome. We describe the third case of glomeruloid haemangioma in a patient without features of POEMS syndrome.

Abortive or minimal-growth hemangiomas: Immunohistochemical evidence that they represent true infantile hemangiomas

Abortive or minimal-growth hemangiomas: Immunohistochemical evidence that they represent true infantile hemangiomas

Postradiation Angiosarcoma of the Skin Featuring Capillary Lobules

To the Editor: With reference to the article entitled “the capillary lobule: a deceptively benign feature of postradiation angiosarcoma of the skin: report of 3 cases” by Di Tommaso and Rosai,1 we encountered an identical postradiation angiosarcoma of the skin characterized by capillary lobules. The patient was a 49-year-old woman who had a lumpectomy followed by radiation therapy for invasive ductal carcinoma of the left breast. Two and a half years later, she developed a 0.6 cm pink-purple lesion in the skin of the irradiated area, which was excised. Microscopically, the most striking feature was the presence of multiple round to oval capillary lobules throughout the dermis, suggestive of a hemangioma. The capillary lobules varied in size and were composed of compact vascular channels lined by slightly hyperchromatic endothelial cells (Fig. 1A). In addition to the capillary lobules, there were some areas with features typical of angiosarcoma, namely, anastomosing thin-wall vascular channels lined by plump hyperchromatic endothelial cells, intraluminal micropapillary formations, ill-formed vascular channels interposed between collagen bundles, and mitotic figures (Fig. 1B). We interpreted the lesion as an unusual angiosarcoma with features resembling hemangioma. A simple mastectomy was performed and revealed a 1.5 cm tumor with identical histopathologic features. The tumor was present 0.3 mm from the closest margin and 1 mm from the closest margin in a reexcision specimen. Six months later, the patient had a recurrent lesion in the same area with the same morphology. It was widely excised with negative margins. The patient is alive with no evidence of recurrence or metastasis 4 months later.FIGURE 1.: The tumor was mainly composed of round to oval capillary lobules scattered in the dermis, mimicking a hemangioma (A). However, some areas showed the typical features of angiosarcoma (B).In summary, the presence of capillary lobules in a postradiation angiosarcoma of the skin may present a diagnostic pitfall as it can readily be mistaken for a benign lesion. We agree with Di Tommaso and Rosai who state that “the detection of capillary lobules in irradiated skin ought to be regarded with great suspicion, and other morphologic signs suggestive of angiosarcoma should be sought in the specimen.” Zhenqiang Gao, MD, PhD Sheng Chen, MD, PhD Department of Pathology, Albert Einstein College of Medicine Long Island Jewish Medical Center, 270-05 76th Avenue New Hyde Park, NY

The Capillary Lobule: A Deceptively Benign Feature of Post-Radiation Angiosarcoma of the Skin

Three cases are presented of a proliferative vascular lesion of the skin developing in the chest wall of women who had received radiation therapy for breast carcinoma. All 3 lesions were characterized microscopically by the presence of the so-called capillary lobule, a formation generally regarded as a sign of benignancy but that in this setting proved to be a forerunner or indicator of angiosarcoma (malignant hemangioendothelioma). Therefore, the detection of capillary lobules in irradiated skin ought to be regarded with great suspicion, and other morphologic signs suggestive of angiosarcoma should be sought in the specimen.

Tufted Angioma:Clinicopathologic Surveys and the Response to Intralesional Steroid

progressive vascular tumor found typically in infants and young children, and sometimes since birth or in young adulthood. Because of the characteristic histologic findings of small, well-defined, tightly packed capillary lobules scattered in the dermis, so called “cannonball” appearance, the lesions were designated as tufted angioma. Synonyms are progressive capillary hemangioma and Nakagawa’s angioblastoma. Even though tufted angiomas progressively enlarge frequently to a large size measured 10 centimeter or more in diameter, Tufted Angioma : Clinicopathologic Surveys and the Response to Intralesional Steroid

Bartonella (Rochalimaea) quintana infections.

Bartonella (formerly Rochalimaea) quintana is the etiological agent of trench fever, a disease extensively reported during the World Wars. Recent molecular biology approaches have allowed dramatic extension of the spectrum of Bartonella infections. B. quintana is now also recognized as an etiological agent of fever and bacteremia, endocarditis, bacillary angiomatosis, and chronic lymphadenopathy. Human immunodeficiency virus-infected patients and/or homeless people are the most vulnerable to infection. Poverty and louse infestation were the main epidemiological factors associated with B. quintana infections during wartime. Although poverty and chronic alcoholism have been associated with modern cases of trench fever and bacteremia due to B. quintana in Europe and the United States, vectors for B. quintana have not been clearly identified and B. quintana has not been isolated from modern-day lice. Microscopic bacillary angiomatosis lesions are characterized by tumor-like capillary lobules, with proliferating endothelial cells. In vitro experiments have shown that B. quintana survives within endothelial cells and stimulates cell proliferation. These observations, together with the finding that lesions may regress when antibiotic therapy is administered, strongly suggest that B. quintana itself stimulates angiogenesis. Bartonella infections are characterized by a high frequency of relapses after brief courses of antibiotic therapy. It is to be noted that in vitro, although Bartonella species are highly susceptible to antibiotics, only the aminoglycosides have proved to be bactericidal. However, the most effective antibiotic regimen for Bartonella infections remains to be established.

Bartonella (Rochalimaea) quintana infections.

Bartonella (formerly Rochalimaea) quintana is the etiological agent of trench fever, a disease extensively reported during the World Wars. Recent molecular biology approaches have allowed dramatic extension of the spectrum of Bartonella infections. B. quintana is now also recognized as an etiological agent of fever and bacteremia, endocarditis, bacillary angiomatosis, and chronic lymphadenopathy. Human immunodeficiency virus-infected patients and/or homeless people are the most vulnerable to infection. Poverty and louse infestation were the main epidemiological factors associated with B. quintana infections during wartime. Although poverty and chronic alcoholism have been associated with modern cases of trench fever and bacteremia due to B. quintana in Europe and the United States, vectors for B. quintana have not been clearly identified and B. quintana has not been isolated from modern-day lice. Microscopic bacillary angiomatosis lesions are characterized by tumor-like capillary lobules, with proliferating endothelial cells. In vitro experiments have shown that B. quintana survives within endothelial cells and stimulates cell proliferation. These observations, together with the finding that lesions may regress when antibiotic therapy is administered, strongly suggest that B. quintana itself stimulates angiogenesis. Bartonella infections are characterized by a high frequency of relapses after brief courses of antibiotic therapy. It is to be noted that in vitro, although Bartonella species are highly susceptible to antibiotics, only the aminoglycosides have proved to be bactericidal. However, the most effective antibiotic regimen for Bartonella infections remains to be established.

Verrucous Hemangioma

Verrucous hemangioma is an uncommon variant of cutaneous hemangioma, showing characteristic clinicopathologic features. Since its formal characterization in 1967, little has been written on this entity. This report describes 10 cases to draw wider attention to this distinct hemangioma. The patients, 5 males and 5 females, were aged 6-44 years at presentation. The lesions were usually noted at birth as flat or dome-shaped reddish or bluish lesions that enlarged slowly and became verrucous. All 10 cases were located in the lower extremities. Among the six cases with follow-up information, two have recurred. Histologically, beneath the warty epidermal surface, haphazardly distributed in the dermis were vascular spaces often of cavernous size, merging into capillaries in the subcutis. In the subcutaneous tissue, the lobules of capillaries com monly merged with the adipose cells in a pseudoinfiltrative pattern. The endothelial cells were flat and nondescript, and showed variable immunoreactivity for factor VIII related antigen but showed strong staining with CD34. All the vascular channels were surrounded by complete rims of muscle-specific actin-positive pericytes. The overlying epidermis lacked evidence of human papillomavirus infection either by immunohisto chemical studies or ultrastructural examination. Thus the warty change represented a reactive phenomenon rather than a result of human papillomavirus infection. Since the lateral and deep extent of the vascular proliferation is often greater than that of the exophytic epidermal component in verrucous hemangioma, the size of the lesion is often underestimated, leading to inadequate excision and recurrence. Awareness of this entity and obtaining a complete clinical history should permit recognition of the lesion as a vascular lesion and not a primary epidermal or melanocytic lesion. Int J Surg Pathol 2(3):171-176, 1995

Giant proliferative hemangiomas arising in a port-wine stain

Giant proliferative hemangiomas arising in a port-wine stain

An eruptive vascular proliferation resembling acquired tufted angioma in the recipient of a liver transplant

An eruptive vascular proliferation resembling acquired tufted angioma in the recipient of a liver transplant

Dermatopathologic Findings in Patients Infected with HIV

Dermatopathologic Findings in Patients Infected with HIV

Recently characterized vascular tumours of skin and soft tissues

This review summarizes the clinicopathological features of a number of vascular tumours that have been characterized only in recent years. These include: glomeruloid haemangioma, a multifocal vascular lesion associated with POEMS syndrome: Kaposi-like infantile haemangioendothelioma, a borderline malignant tumour occurring in the retroperitoneum of infants, mimicking Kaposi's sarcoma histologically; giant cell angioblastoma, characterized by proliferated vessels with a granuloma-like appearance; benign lymphangioendothelioma (progressive lymphangioma), slowly-growing macule or plaque over the trunk or limb, mimicking low-grade angiosarcoma histologically; targetoid haemosiderotic haemangioma, a benign lesion with a distinctive annular appearance and histologically overlapping with benign lymphangioendothelioma; spindle cell haemangioendothelioma, a lesion located mostly in the distal extremities, characterized by cavernous vascular spaces, spindle cells with interspersed narrow vascular channels and scattered plump vacuolated endothelial cells; acquired tufted angioma, characterized by 'cannon-ball' involvement of the dermis by lobules of pericyte-rich capillaries; sinusoidal haemangioma, a distinctive variant of cavernous haemangioma which may be confused with angiosarcoma; and epithelioid angiosarcoma, a highly aggressive tumour of deep soft tissue mimicking metastatic carcinoma and co-expressing endothelial and epithelial markers.