Acquired tufted angioma: a unique vascular lesion not previously reported in the oral mucosa

We describe two patients with acquired tufted angioma, a unique vascular lesion not previously reported in the oral mucosa. In one patient, the lesion manifested as a purple-red papule and, in the other, as a blue submucosal swelling. Both lesions were non-painful and neither was associated with a history of trauma. The histopathological features consisted of scattered, irregularly shaped tufts, primarily composed of poorly formed capillary spaces and slit-like vascular channels. Capillary spaces were often closely packed, producing solid areas which stained for smooth muscle actin. Staining for factor VIII-related antigen was positive only within endothelial cells lining well-formed vascular channels. Both lesions were treated by excision; short-term follow-up of one patient revealed no evidence of recurrence. Similarities between this and other vascular processes may have resulted in misdiagnosis of this lesion in the past. The clinical significance of acquired tufted angioma in the oral mucosa is not known.     

Spindle cell hemangioendothelioma: report of three cases

Three cases of spindle cell hemangioendothelioma were studied. Two had multiple nodules and the third had a single, recurrent lesion in the dermis or subcutis of the distal extremities. The tumor initiated at the age of 3 months in one patient and at the ages of 11 or 12 years in the remaining two. Local recurrence after surgical excision was observed in all cases. Microscopically the tumors were composed of two components; cavernous blood spaces and a proliferation of spindle cells. Among these spindle cells, there were occasional epithelioid endothelial cells with intracytoplasmic lumina. Phleboliths were observed in two cases. Most of the endothelial cells lining the cavernous spaces were positive for factor VIII-associated antigen in all cases; the intracytoplasmic lumina were also positive in two cases. The spindle cells were negative for that antigen in all cases. However, they were positive for desmin in one case. Electronmicroscopically, the spindle cells resembled primitive mesenchymal cells.     

ULTRASTRUCTURE OF SO-CALLED ANGIOBLASTOMA OF THE SKIN BEFORE AND AFTER SOFT X-RAY THERAPY

Tissues from a two-month-old Japanese male infant with a typical angioblastoma of the skin on the right groin was examined by light and electron microscopy before and after soft X-ray therapy. Before therapy, nest-like proliferation of the tumor cells with frequent formation of vascular spaces was observed. Ultrastructurally, these tumor cells showed the morphological characteristics of vascular endothelial cells in their size, shape and usual organelles. These cells usually had an abundance of peculiar dense granules with a limiting membrane. Fibrin deposits and thrombosis with aggregations of platelets were observed in the lumina in situ. After soft X-ray therapy with a total dose of 3,600 rads, disappearance of most of the tumor cell-nests and prominent dilatation of the residual vessels were observed. Ultrastructurally, numbers of the vessels showed more or less degenerative and destructive changes. Vessels with ruptured endothelial cell linings, extravasated red cells, and hemosiderin deposits were also frequently observed. From these findings, it is concluded that angioblastoma of the skin should be considered as one of the benign hemangiomas of the skin, and that degenerative changes in the endothelial cells induced by irradiation are almost identical to those in other conditions.     

Pagetoid Bowen''s Disease on the Breast

A 75-Year-old while woman was referred to Memorial Sloan-Kettering Cancer Center for evaluation of a slowly enlarging, asymptomatic plaque that had been present on the left breast for several years. She had developed hypothyroidism and hypertension. There was no personal or family history of skin cancer or broast disease. Results of mammography, performed 1 month earlier, were normal. Physical examination revealed an elderly, moderately obose white woman. Examination of the breasts did not reveal any masses, and there were no palpable axillary lymph nodes.
On the left breast, tbere was a well circumscribed, orangered plaque, 3 cm in diameter, with a peripheral collarette scale. The lesion was located 2 cm from the areola, in the medio-inferior quadrant of the breast (Fig. 1). A punch biopsy of the lesion showed atypical cells in the epidermis characterized by abundant pale cytoplasm (Fig. 2). Result of PAS stain for glycogen was only weakly positive, and results of all stains for mucin were negative. A lichenoid lymphohistiocytic infiltrate was seen in the dermis. A complete excision of the lesion was performed, revealing Bowen's disease. The specimen contained areas of pagetoid cells (Fig. 2) as well as more typical areas of Bowen's disease (Fig. 3).     

Scanning electron microscopic features of Kaposi's sarcoma

In order to clarify the three-dimensional structure of Kaposi's sarcoma (KS) five cases of KS of the skin were observed with a scanning electron microscope (SEM). KS lesions were investigated both in vascular and spindle cell areas and were compared with similar areas observed by light microscopy. KS appears as a spongelike neoplasm consisting of irregular vascular channels lacking endothelial cells and well-developed basement membranes. Vascular spaces and vascular slits are packed with numerous red blood cells (RBCs). SEM features of KS share some similarities with those of hemangiopericytoma, a vascular tumor composed of mature endothelial cells surrounded by pericytes. These findings are consistent with the hypothesis according to which the proliferating cells of KS are viewed as immature endothelial cells with partial differentiation into pericytes.     

Cutaneous necrosis by cold agglutinins associated with glomeruloid reactive angioendotheliomatosis

The cold agglutinin syndrome is a haemolytic disorder that can cause skin lesions, mainly on the acral areas, with acrocyanosis being the most frequent manifestation. Cutaneous necrosis due to cold agglutinins is very rare. Reactive angioendotheliomatosis (RAE) is an uncommon condition that exclusively affects the skin, characterized by a hyperplasia of endothelial cells and pericytes that can result in the formation of glomeruloid structures. The association of cold agglutinin syndrome with glomeruloid RAE has not been previously described. We report a 70-year-old man diagnosed as having a B-cell low-grade non-Hodgkin's lymphoma. He had two episodes of cutaneous necrosis in acral areas which were related to exposure to cold and due to IgM anti-I^T cold agglutinins. Biopsy specimens showed vessel proliferations composed of dilated vascular spaces in the dermis and subcutis. Some vessel lumina were partially occluded by eosinophilic thrombi of fibrin and erythrocytes. Numerous closely packed capillaries were observed within pre-existing dilated vessels. This intravascular proliferation of capillaries displayed a glomeruloid pattern. We emphasize the possible presence of a cold agglutinin syndrome in patients with skin necrosis and findings of RAE with a glomeruloid pattern. Cold agglutinaemia may cause these distinctive histological changes.     

Dermoscopic findings of umbilical granuloma

Dermoscopic findings in umbilical granuloma are vascular polymorphism comprised of linear irregular and arborizing vessels with structureless areas distributed over a milky‐red background. The increase of angiogenesis and neovascularization is represented by the linear irregular and arborizing vessels. Structureless areas over an irregularly milky‐red background are originated by the proliferation of vascular endothelial cells and fibroblasts, with capillary and granulation tissue formation.     

Congenital hemangiopericytoma of the skin

A 5-month-old white boy had a pinhead red spot on his left malleolar region since birth. His mother noted progressive growth and, when he was first examined, the lesion measured 30x25x20 mm in size, had a pyramidal shape, covered by erythematous and smooth skin, with teleangiectasia. The tumor was soft on paipation and movable over the deeper structures (Fig. 1a). X-ray examination revealed no bone abnormalities at that time.
Just before the programmed incisional biopsy, the patient had a febrile episode due to upper respiratory tract viral infection during which the tumor became ulcerated, bleeding and painful (Fig. 1b). As the bleeding was constant the lesion was surgically excised, at which time the whole gelatinous mass was easily detached from the aponeurotic structures (Fig. 1c). The tumor measured 36x31 x30 mm and had a whitish, smooth cut surface crossed by small blood vessels.
Histopathologic examination revealed a tumor mass extending from the mid dermis to the subcutaneous fat and composed of tightly packed fusiform cells in close opposition with lined vascular channels (Fig. 2a). There were atypical areas and mitotic figures were frequently seen. A reticulin preparation showed a dense network surrounding vessels and pericytes. The vessels branched in a staghorn configuration (Fig. 2b). The immunoperoxidase technique with monoclonal antibody anti-Factor VIII was positive in the endothelial cells surrounded by tumor cells (Fig. 2c).
Seven months after surgery the patient returned with a painful subcutaneous mass on the upper third of his left leg that was adherent to the tibial bone. X-ray examination disclosed osteolytic lesions at the tibial base and destruction of the peroneal head (Fig. 3).
The patient was submitted to radiotherapy, amputation of the left lower limb in the middle portion of the thigh and chemotherapy. He is clinically well 3 years after treatment.     

Pseudolymphomatous cutaneous angiosarcoma: a rare variant of cutaneous angiosarcoma readily mistaken for cutaneous lymphoma

Cutaneous angiosarcoma is probably the most malignant neoplasm involving the skin. Three clinical variants of cutaneous angiosarcoma are recognized, including angiosarcoma of the scalp and face of elderly patients, angiosarcoma associated with chronic lymphedema, and postirradiation angiosarcoma. Histopathologically, these three variants of angiosarcoma show similar features, which consist of poorly circumscribed, irregularly dilated, and anastomosing vascular channels lined by prominent endothelial cells that dissect through the dermis. Focally, neoplastic endothelial cells show large, hyperchromatic, and pleomorphic nuclei, protruding within vascular lumina and creating small papillations. Usually, inflammatory infiltrate is sparse and consists of a patchy, perivascular lymphoid infiltrate around the neoformed vessels. In rare instances, cutaneous angiosarcomas may exhibit prominent inflammatory infiltrate, and the neoplasm may be mistaken for an inflammatory process, both from clinical and histopathologic points of view. We describe four examples of cutaneous angiosarcomas with dense lymphocytic infiltrates involving the neoplasm. Immunohistochemically, lymphocytes expressed immunoreactivity for CD3, CD5, and CD45 markers, whereas the germinal centers were positive for CD20, CD79a, and Bcl-6. The neoplastic endothelial cells expressed immunoreactivity for the CD31, CD34, podoplanin, Prox-1, Lyve-1, and D2-40. We discuss the possible relationship between neoplastic endothelial lymphatic cells and reactive lymphocytes. Cutaneous angiosarcoma with prominent lymphocytic infiltrate may be readily mistaken for cutaneous follicle center cell lymphoma or cutaneous pseudolymphoma.     

Acquired progressive lymphangioma occurring following femoral arteriography

Our case report concerns an unusual case of acquired progressive lymphangioma (APL) which developed in a 52-year-old Japanese man following femoral artery catheterization for angiography. The histological examination revealed many irregularly shaped and dilated lymphatic channels that were lined by a single layer of endothelial cells. Immunohistochemical staining for von Willebrand factor was negative in the endothelial cells of the dilated channels. Electron microscopic study demonstrated no Weibel-Palade bodies in the cytoplasm of the endothelial cells. The endothelial cells had no distinct basement membrane.     

Focal endothelial cell degeneration and proliferative endarteritis in trauma-induced early lesions of necrobiosis lipoidica diabeticorum

Microangiopathy is an essential component in diabetic vascular pathology. We report ultrastructural observations of ballooning degeneration involving isolated endothelial cells of cutaneous capillaries, while leaving adjacent endothelial cells relatively intact in six diabetic patients with early lesions of necrobiosis lipoidica induced by trauma. Focal proliferation of endothelial cells encroaching upon the vascular lumina (obliterative endarteritis) was also observed. Lectin studies on biopsy specimens of older lesions of necrobiosis lipoidica revealed paucity of dermal blood vessels. These observations enable us to gain further insight into the pathophysiological mechanisms that underlie diabetic microvascular disease.     

Microcystic adnexal carcinoma

A 55-year-old man presented with an ulcer on the right sole present for 8 years. The ulcer measured 6.5 × 3 cm and affected the entire distal sole of the right foot; the margins were everted and an intermittent serosanguineous discharge was present. The general condition of the patient was good, with findings limited to the ulcerated lesion. There was no history of hypertension, diabetes mellitus, or venous stasis. A biopsy taken from the ulcer edge was interpreted as squamous cell carcinoma, Grade I. A transmetatarsal amputation was carried out and the specimen sent for histopathologic examination. Histologically, the epidermis showed ulcerated areas; adjacent areas showed hyperkeratosis and irregular acanthosis. Keratin cysts containing well-developed lamellar keratin were present in the upper dermis (Fig. 1). Nests and strands of squamous and basaloid cells, having scanty eosinophilic cytoplasm, alternated with the cysts. Areas of ductular differentiation were also noted. The epithelial strands were separated by concentric bands of moderately cellular fibrous tissue in the upper and mid-dermis. In the deeper areas of the tumor the epithelial nests became progressively smaller in size, diminishing to small clusters of two or three cells, and were surrounded by a sclerotic stroma. Cytologic atypia was minimal and no significant mitotic figures were identified. The neoplasm showed extensive infiltration of subcutaneous fat and striated muscle with frequent perineural involvement in the deeper parts. There was no extension to bone or perichondrium. Immunoperoxidase staining carried out for carcinoembryonic antigen (CEA) showed positivity in the lumina and lining cells of the ducts (Fig. 2).
Based upon the classical microscopic appearance, a diagnosis of microcystic adnexal carcinoma was made. The patient has been followed for a period of 3 years with no evidence of tumor recurrence.     

A unique case of a benign disseminated angioproliferation combining features of Kaposi's sarcoma and diffuse dermal angioendotheliomatosis

A female patient undergoing chronic hemodialysis had disseminated, violaceous, and partly ulcerated plaques develop on the trunk. Lesions had erupted simultaneously over a period of 4 weeks and resolved within 5 months after steroid treatment. By histopathology, the papillary dermis was densely filled with blood vessels lined by a single layer of differentiated endothelial cells, a growth pattern resembling diffuse dermal angioendotheliomatosis. In some areas, endothelial cells were spindle shaped and formed discontinuous lumina. Red blood cells were interspersed within these slits, giving the lesions a kaposiform appearance. By immunohistochemistry, endothelial cells reacted with the antibodies anti-von Willebrand factor, anti-CD31, and anti-CD34 and with the lectin Ulex europaeus-1. The course of the disease combined with the unusual histopathology makes this case a unique form of a benign disseminated kaposiform angioproliferation.     

Congenital multiple plaque-like glomangiomyoma.

Congenital glomus tumor is a rare clinical variant of glomus tumor, and glomangiomyoma is the least frequent histologic type of glomus tumor. We report a case of congenital multiple plaque-like glomangiomyoma in a 38-year-old man with multiple nodules and plaques on his left arm and forearm. Histopathologic study showed an angiomatous, nonencapsulated tumor with numerous highly folded dilated vascular lumina scattered throughout the dermis. The lumina were lined by a single layer of flat endothelial cells, and one to several rows of glomus cells were observed adjacent to the endothelial cells. Around large vessels, there was a gradual transition from glomus cells to elongated mature smooth muscle cells with thin and long "blunt-ended" nuclei. Immunohistochemically, there were strong positive reactions for cytoplasmic alpha-smooth muscle actin in glomus cells and smooth muscle cells, vimentin in glomus cells and endothelial cells, and desmin in the smooth muscle cells only. To our knowledge, this is the first case report of congenital multiple plaque-like glomus tumor with the microscopic appearance of a glomangiomyoma.     

Reactive angioendotheliomatosis secondary to dermal amyloid angiopathy

Reactive angioendotheliomatosis (RAE) is a rare benign cutaneous vascular proliferation characterized by intravascular hyperplasia of endothelial cells and tuft-like proliferation of vessels. A 75-year-old man had erythematous and violaceous macules, some stellate and others arranged in a livedoid pattern, evolving toward necrosis with central areas having an "atrophie blanche" appearance spread on the trunk, inguinal folds, and right thigh. He was on hemodialysis and had a benign monoclonal gammopathy. Cutaneous biopsy revealed RAE characterized by the proliferation of epithelioid and spindle-shaped cells in superficial and middermis lining vascular channels, arranged in clusters, and sometimes displaying an intravascular growth pattern. These cells stained for CD31, CD34, and actin. Interestingly, prominent amyloid deposits were found in the wall of some vessels in deep dermis, often causing obstruction of their lumina. The cause of RAE is unknown, but it can be associated with infections, antiphospholipid syndrome, dysglobulinemia, cryoproteinemia, and lower extremities arteritis, and it may occur near arteriovenous fistulas. In this patient, we believe that RAE was caused by obliteration of dermal vessels by amyloid deposits. Indeed, it is thought that RAE could be caused by ischemia secondary to vascular obstruction. This is the first reported patient with RAE associated with amyloid deposits.     

Glomeruloid hemangioma in POEMS syndrome shows two different immunophenotypic endothelial cells

The case of a Japanese woman with glomeruloid hemangioma, an initial marker for POEMS syndrome, is reported. Her cutaneous lesions were multiple and consisted of glomeruloid hemangiomas, cherry-type capillary hemangiomas, and a mixture of both. The specimens of glomeruloid hemangiomas were studied by paraffin section immunohistochemistry with a large panel of antibodies and electron microscopy, respectively. The lesions, whose size ranged from minute foci to large nodules, were composed of anastomosing vascular channels resembling renal glomeruli and had irregular lumina, often featuring capillaries and sinusoid-like spaces. The vascular channels were lined by a single layer of endothelial cells, which showed two types of cells. The capillary-type endothelium possessed large vesicular nuclei with open chromatin and large amount of cytoplasm. The sinusoidal endothelium possessed small basal nuclei with dense chromatin as well as scant amount of cytoplasm. The former cells had a characteristic CD31+/CD34+/UEA I+/CD68- phenotype. Some of these cells ultrastructurally showed intracytoplasmic lumen formation. The latter cells had a characteristic CD31+/CD34-/UEA I-/CD68+ phenotype. The present study shows that glomeruloid hemangioma has unique morphologic and immunologic features that differ from the traditional hemangiomas as well as littoral cell angioma of the spleen.     

Lymphangioma-like Kaposi sarcoma

BACKGROUND: Lymphangioma-like Kaposi's sarcoma (LLKS) is a rare morphologic expression of Kaposi's sarcoma (KS) that occurs in virtually all of the well-recognized clinical subtypes of the disease and has the potential to mimic other pathologic processes. In this study, we present the clinical and pathological features of four patients with LLKS. METHODS: Four cases of LLKS were retrieved from the dermatopathology files of our institution. All four tumours were tested immunohistochemically with anti-human herpesvirus-8 (HHV-8) latent nuclear antigen-1 (LNA-1) and anti-CD34 antibodies. RESULTS: Clinically, each patient presented with violaceous patches, papules or plaques; one patient presented with bullous lesions. All of the LLKS biopsy specimens revealed areas with characteristic light microscopic features of KS. Lymphangioma-like foci consisted of ectatic, irregularly shaped vascular spaces lined by mildly atypical endothelial cells. All tumour cells, including those associated with LLKS foci, showed a strong and diffuse reactivity for anti-HHV-8 LNA-1 and anti-CD34. KS progressed slowly in two patients with adequate follow-up. CONCLUSIONS: As LLKS can mimic other disease processes, the correct diagnosis relies heavily on the recognition of salient clinical and histological features of conventional KS, including a strong immunohistochemical expression of HHV-8-associated LNA-1 in lesional cells.     

Granular cell tumor (Abrikossoff tumor) of the clitoris

A 60-year-old woman presented to the Department of Dermatology of the ABC Medical Center in Mexico City with a 2-month history of a progressive growth in the region of the clitoris that had been increasing rapidly. Examination revealed a firm growth measuring 3 cm in diameter; the overlying surface was smooth and free of ulceration. Vaginal speculum examination showed no mucosal irregularities or displacement. Rectal examination was normal. Inguinal lymph nodes were clinically negative. A wide excisional biopsy was performed with ample skin margins.
The specimen consisted of a mass measuring 3.5 × 1.2 × 0.5 cm that was pale tan, homogeneous, and firm. Prepared sections showed a cellular tumor composed of compact irregular strands and nodules of polyhedral cells containing centrally located, round to oval vesicular nuclei with no significant atypia of mitosis. These cells had granular eosinophilic cytoplasm with distinct cell borders (Fig. 1). The cytoplasmic granules were diastase-resistant periodic acid-Schiff (PAS) positive. The tumor cells were positive for S–100 protein and for neuron-specific enolase (NSE), and were negative for actin, desmin, and cytokeratin. The tumor showed compression and obliteration of the cavernous sinuses (Fig. 2) outlined by the S–100 immunostain and actin. (Figs 3 and 4).     

Recurrent primary cutaneous adenoid cystic carcinoma of the scrotum

A 60‐year‐old man had asymptomatic erythematous nodule on the right inguinal area, which had progressively grown for 2 years. Excision was performed at the Department of Urology of our hospital. Two months later, there was local recurrence on the excision site, and it extended locally to make several tender erythematous nodules on the right inguinal area and firm plaque on the scrotum ( Fig. 1 ). Histopathologic examination of the excision specimen revealed large cell masses with tubular or cribriform pattern and many small, solid epithelial islands unattached to the overlying epidermis. The tumor was confined to the dermis and subcutaneous tissue. Round spaces formed by malignant epithelial cells and containing amorphophilic basement membrane‐like material occured inside the tumor masses. The tumor cells were basaloid cells with scanty cytoplasm and deeply stained round‐to‐oval nucleus ( Fig. 2 ). There was clear perineural involvement by the tumor cells. A substance in the pseudoglandular spaces, cystic spaces and the stroma was stained strongly with periodic acid‐Schiff (PAS) and alcian blue (pH 2.5). A PAS‐positive substance was digested by diastase pretreatment. These findings suggested the substance to be a neutral mucopolysaccharide. Immunoperoxidase staining for S‐100 protein and cytokeratin were strongly positive with the tumor cells but not with carcinoembryonic antigen. A histopathologic diagnosis of ACC was made. Salivary glands, bronchi, esophagus, and other organs were examined, but there was no evidence of neoplasm in other organs. A diagnosis of primary cutaneous adenoid cystic carcinoma was made but the patient refused all the treatment, including surgery.

Figure 1 Open in figure viewer PowerPoint Several erythematous nodules on the right inguinal area and indurated plaque on the adjacent scrotum     

Trauma-induced simulator of targetoid hemosiderotic hemangioma

Reported here is a 15-year-old with lesions demonstrating histologic features of targetoid hemosiderotic hemangioma (THH) developing after trauma to inflammatory lesions. These lesions pose as simulators of THH. Targetoid hemosiderotic hemangioma is a benign vascular lesion first described by Santa Cruz and Aaronburg. It classically presents as a single, small, red/brown, targetoid lesion on the trunk or extremities of a young or middle-aged individual. Histologically, it is characterized by ectatic vascular lumina in the papillary dermis lined by a single layer of endothelial cells with an epithelioid or "hobnail" appearance. In the deeper dermis, vascular spaces become slit-like and angulated, appearing to dissect through collagen bundles. A commonly proposed etiology of THH is trauma to a preexisting hemangioma. This case is remarkable for its unusual clinical presentation, histologic simulation of THH, and for its support for the theory that trauma can induce the histologic changes seen in THH.