Slit-like Vascular Spaces Research Articles

Positive staining of tumor-stage Kaposi sarcoma with lymphatic marker D2-40

Kaposi sarcoma (KS) is a vascular tumor first described by Moritz Kaposi (Kohn) in 1872 and recently linked to human herpesvirus 8. All forms of KS share the same pathologic features, including atypical spindle cells and slitlike vascular spaces. The exact origin of the neoplastic endothelial cells (vascular vs lymphatic) has recently been debated. We sought to further investigate the origin of neoplastic cells in KS. D2-40, a novel monoclonal antibody to a sialoglycoprotein that reacts with a fixation-resistant epitope in lymphatic endothelium, was used to investigate possible lymphatic origin of neoplastic cells in KS. We report 5 cases of tumor-stage KS that showed positive staining with lymphatic marker D2-40. Small study size is a limitation. The origin of neoplastic cells in KS may be lymphatic or mixed lymphatic and vascular. Further studies are needed to elucidate the exact origin of neoplastic cells in KS.

Sclerosing Angiomatoid Nodular Transformation

AJR:192, May 2009 known, and a strong correlation with another disease process has not been found [3, 4]. SANT has a distinctive gross appearance. It is a well-circumscribed mass composed of numerous nodules derived from red pulp alternating with bands of dense fibrous tissue that coalesce to form a central, stellate fibrous scar [3]. Microscopically, SANT is characterized by multiple nodular aggregates of plump endothelial cells and pericytes lining prominent slitlike vascular spaces [2–5]. The nodules are associated with extravasated erythrocytes, and the intervening collagenous stroma contains variable numbers of reactive myofibroblasts, hemosiderin-laden macrophages, lymphocytes, and plasma cells. The endothelial cells exhibit minimal cytologic atypia, with rare mitotic activity [2–5]. Limited imaging information is conveyed by the pathology literature. Two articles provide correlative enhanced CT images [4, 5]. Both have images or descriptions depicting a hypodense mass in the early portal venous phase, with areas of peripheral enhancement that become homogeneous on more delayed imaging. Description of MR findings in one case indicates that the mass showed low signal on T1 and T2 sequences [4]. A recent report, however, describes a single case showing early peripheral enhancement progressing centripetally in a radial pattern [6]. Although the small number of cases with radiologic description precludes definitive assessment, suggestive characteristics of SANT include a well-circumscribed solitary mass of the spleen that initially shows low attenuation with areas of peripheral enhancement after the administration of contrast material. This may reflect the gross pathologic appearance of central fibrous stroma and more peripheral coalescent nodules of angiomatoid Sclerosing Angiomatoid Nodular Transformation

Solitary Kaposi's Sarcoma of the Lower Lid in an HIV-Negative Patient

Purpose: To report a case of solitary Kaposi’s sarcoma (KS) of eyelid in an HIV-negative patient, which has not yet been reported in domestics. Case summary: A 76-year-old woman was seen at our clinic with a purplish nodular mass on her left lower lid that had grown up for 4 months prior. There were no abnormal findings in the body except eyelid. In the routine laboratory examinations, the result of HIV test was negative. The lesion was completely excised and the eyelid was reconstructed by semicircular rotational flap procedure. The histopathologic examination showed spindle cells of mild atypism, intermixed with slit-like vascular spaces and red blood cell extravastaion. These findings coincided with KS and polymerase chain reaction was positive for human herpes virus-8. During 10 months follow up, there was no evidence of tumor recurrence and systemic abnormalities. Conclusions: We experienced that Kaposi’s sarcoma can occur solitarily in the eyelid of HIV-negative patient and favorable prognosis can be obtained by only surgical resection.

Retroperitoneal lymphangioleiomyomatosis associated with endosalpingiosis

A case of retroperitoneal lymphangioleiomyomatosis (LAM) arising from endosalpingiosis is described. A 25-year-old woman with no history of tuberous sclerosis or hormonal therapy presented with a painless, palpable abdominal mass. Computed tomographic and magnetic resonance imaging studies of the abdomen demonstrated a 4 cm cystic mass in the retroperitoneum. Macroscopically, the excised retroperitoneal cyst was multilocular and measured 4.0 x 3.5 x 3.5 cm. Histologically, the lesion demonstrated three components. The first comprised multiple cysts or glands lined by columnar epithelial cells with cilia. The second component was a condensation of small stromal cells immediately subjacent to the cystic epithelium or glands. The third component was a thick exterior wall composed of plump spindle cells with clear to palely eosinophilic cytoplasm in a fascicular pattern, and slit-like vascular spaces, resembling LAM. Immunohistochemically, the epithelium and glands were positive for cytokeratin 7. The stromal cells were positive for vimentin and CD10. The cells of the LMA-like component showed positive staining for HMB45, alpha-smooth muscle actin, muscle actin and h-caldesmon. The lesion, LAM arising from endosalpingiosis, represents a distinctive pathologic entity that should be recognized and studied further. This type of lesion should be included in the differential diagnosis of retroperitoneal cystic lesions.

Clinical and pathological characteristics of primary intraspinal hemangiopericytoma and choice of treatment

Primary intraspinal hemangiopericytoma is a rare malignant mesenchymal tumor with high rates of recurrence and metastasis. Surgery is the main therapeutic procedure for this lesion. This clinical research was undertaken to analyze the pathological characteristics, clinical course, and the choice of treatment for this lesion. Twenty-three patients with primary intraspinal hemangiopericytomas were treated from 1987 to 2004. The clinical and imaging features, pathological findings, therapeutic procedures, and prognosis were analyzed retrospectively. Primary intraspinal hemangiopericytoma is more likely to attack middle-aged persons. The tumor mainly manifests as muscle weakness and sensor abnormalities. Microscopic examination showed slit-like vascular spaces and oral- or spindle-shaped cells with slightly acidic cytoplasm and oral nuclei. Tumors were subtotally resected in 11 patients, subtotally resected with postoperative radiotherapy in 4, totally resected in 5, and totally resected with postoperative radiotherapy in 3. Two patients were given spinal stabilization after total resection. Recurrence and metastatic rates were 50% and 0 in intradural patients. They were 73% and 27% in extradural patients, respectively. The tumor should be resected en bloc with the neighboring dural mater to reduce recurrence and metastasis. Patients with subtotal resection need adjuvant radiotherapy. Patients with evident spinal involvement may benefit from spinal stabilization. The prognosis of the lesion arising from the dural mater is better.

Multifocal kaposiform haemangioendothelioma

Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive vascular spindle cell proliferation, with resemblance to Kaposi's sarcoma. This tumour usually occurs in skin and retroperitoneum of infants and young children and is often complicated by the Kasabach-Merritt phenomenon (KMP). A 3-year-old boy presented with a right submandibular swelling due to lymphadenopathies, a violaceous skin lesion at the left commissure of the lips and an ill-defined lesion in the right thyroid lobe. There were some signs of KMP. Histological examination revealed a typical infiltrative multilobular spindle cell proliferation with slit-like vascular spaces in these three localisations. Immunohistochemical stains showed positivity for CD34 and CD31 and many alpha-smooth muscle actin-positive spindle cells around the vascular spaces. There was no Herpes virus type 8 expression. The presented case is unique in two ways. First, thyroid involvement of KHE has never been described in the literature until now. Secondly, and most remarkably, the multifocal presentation in three anatomically distinct and separated localisations is extremely unusual.

Sclerosing angiomatoid nodular transformation (SANT): report of 25 cases of a distinctive benign splenic lesion.

Twenty-five cases of a morphologically distinctive vascular lesion of the spleen are described. The patients were 17 women and 8 men, ranging in age from 22 to 74 years (mean, 48.4 years; median, 56 years). The most common presentations were incidental finding of an asymptomatic splenic mass (13 patients), abdominal pain or discomfort (6 patients), and splenomegaly (4 patients). None of the patients had evidence of recurrent disease after splenectomy. The splenic lesion was solitary, measuring 3 to 17 cm, and sharply demarcated from the surrounding parenchyma. The cut surface revealed a mass of coalescing red-brown nodules embedded in a dense fibrous stroma. All cases showed a remarkably consistent multinodular appearance at low-power examination. The individual nodules had an angiomatoid appearance, in the sense that they were composed of slit-like, round or irregular-shaped vascular spaces lined by plump endothelial cells and interspersed by a population of spindly or ovoid cells. Some of the nodules (particularly the smaller ones) were surrounded by concentric rings of collagen fibers. Numerous red blood cells were present, as well as scattered inflammatory cells. Nuclear atypia was minimal, mitotic figures were extremely rare, and necrosis was consistently absent. The internodular stroma consisted of variably myxoid to dense fibrous tissue with scattered plump myofibroblasts, plasma cells, lymphocytes, and siderophages. Immunostaining revealed 3 distinct types of vessels in the angiomatoid nodules: CD34+/CD8-/CD31+ capillaries, CD34-/CD8+/CD31+ sinusoids, and CD34-/CD8-/CD31+ small veins, recapitulating the composition of the normal splenic red pulp. These features are therefore different from those of littoral cell angioma, conventional hemangioma, and hemangioendothelioma of the spleen. We interpret these angiomatoid nodules as altered red pulp tissue that had been entrapped by a nonneoplastic stromal proliferative process. The characteristic morphologic appearance, immunophenotype, and benign clinical course suggest that this is a distinctive nonneoplastic vascular lesion of the spleen that we propose to designate as sclerosing angiomatoid nodular transformation (SANT).

Benign dermal angioproliferation mimicking Kaposi’s sarcoma: report of two cases

Kaposi’s sarcoma and angiosarcoma are malignant endothelial neoplasms with a progressive clinical course and distinctive histopathology. We present two adult renal dialysis patients who developed violaceous, non-tender, poorly demarcated plaques on the scalp and thigh respectively that clinically resembled angiosarcoma. Skin biopsies showed a dermal proliferation of atypical spindle cells associated with slit-like vascular spaces, abundant hemorrhage and nuclear atypia suggestive of Kaposi’s sarcoma, but lacking fully-transformed nuclear features of malignancy, mitoses, and plasma cells. The atypical cells expressed the endothelial markers CD31 and CD34. Both lesions evaluated by reverse transcriptase in situ polymerase chain reaction for human herpes virus 8 (HHV8) were negative. One of the two patients was receiving low-dose prednisone for idiopathic pulmonary fibrosis. Based upon the kaposiform histomorphology in the absence of unequivocal features of malignant degeneration, the absence of typical risk factors for Kaposi’s sarcoma, relative unresponsiveness to radiation, and the lack of HHV8 expression, we postulate that both lesions represent benign kaposiform angoendotheliomata with overlap features between Kaposi’s sarcoma and a benign angioma. These cases compliment that described by Kuntsfeld and Petzelbauer (J Am Acad Dermatol 2001;45:601) and represent, to the authors’ knowledge, the second and third reported cases of this distinctive entity.

Benign vascular proliferations in irradiated skin.

Several types of cutaneous vascular proliferations have been described in areas of irradiated skin, including both benign lesions, such as benign lymphangiomatous papules, atypical vascular lesions, or benign lymphangioendothelioma, and malignant neoplasms such as high-grade angiosarcomas. This report describes the clinicopathologic features of 15 cases of different types of benign cutaneous vascular proliferations arisen within irradiated skin. All patients were female ranging in age from 33 to 72 years, and they had received postoperative external radiotherapy for treatment of breast carcinoma (14 cases) or ovarian carcinoma (one case). In those cases in which the latency interval period between radiotherapy and the development of the vascular lesion was known from the clinical records, the latency interval period elapsed between radiotherapy and diagnosis of the vascular lesion ranged from 3 to 20 years. The most common clinical presentation of the cutaneous lesions consisted of papules, small vesicles, or erythematous plaques on the irradiated field. Histopathologically, most lesions consisted of irregular dilated vascular spaces, with a branching and anastomosing pattern, thin walls, and lymphatic appearance involving the superficial dermis. A discontinuous single layer of endothelial cells with flattened nuclei lined these vascular channels, and numerous small stromal papillary formations also lined by endothelial cells projected into the lumina of the dilated lymphatic vessels. These cases were classified as benign lymphangiomatous papules or plaques. Two cases showed different histopathologic findings because they consisted of poorly circumscribed and focally infiltrating irregular jagged vascular spaces involving the entire dermis and lined by inconspicuous endothelial cells. In some areas these irregular slit-like vascular spaces dissected collagen bundles of the dermis. These cases were classified as atypical vascular proliferations mimicking benign lymphangioendothelioma or patch-stage Kaposi's sarcoma. All cases showed similar immunohistochemical findings and the endothelial cells lining the vascular spaces expressed immunoreactivity for CD31, but they stained only focally positive for CD34 or were negative for this marker. Immunohistochemical investigations for alpha-smooth muscle actin failed to demonstrate a complete peripheral ring of actin-positive pericytes in most of the neoformed vascular structures. This immunohistochemical profile also supported the lymphatic nature of these vascular proliferations developed in irradiated skin. Although some of these lesions may mimic histopathologically patch-stage Kaposi's sarcoma or well-differentiated angiosarcoma, the follow-up of the patients of this series demonstrated that the vascular proliferations arisen in irradiated skin invariably showed a benign biologic behavior.

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Subcutaneous spindle cell hemangioendothelioma was observed in lower limb of a woman and forearm of a man. Cavernous and slit-like sinusoid vascular spaces were combined with solid spindle cell areas in both tumours. Spindle cells showed local immunohistochemical positivity of Factor XIII. Cavernous spaces comprised numerous thrombi in different phase of organization and sinusoids were full of lingering erythrocytes and lots of siderophages. Recurrence of the tumour occurred in the woman. Biologic nature of the lesion is not evident for the time being because its reactive proliferative character cannot be excluded.

FIBROMA OF TENDON SHEATH

The clinical picture, the light and electron microscopic appearance, and the histochemical findings are described in six cases of fibroma of tendon sheath. The ages at onset for the four men and two women range between 16 and 54 years. The lesions were well circumscribed, rounded or oval, and had a diameter of about 1 cm. Light-microscopically the lesions consisted of a dense fibrocollagenous tissue with a variable degree of collagenization and cellularity, the peripheral zone often being most cellular and containing slit-like vascular spaces. Electron-microscopically two main cell types were identified: myofibroblasts and fibroblasts, the former dominating in cellular areas, the latter dominating in collagenized areas. The histochemical analysis indicated the presence of sulphated glucosaminoglycans within the collagenous matrix. Immunoperoxidase staining for factor VIII RAG-related antigen gave prominence to the endothelium of capillary-like blood vessels. The differential diagnosis against giant cell tumor of tendon sheath is discussed. The observations indicate that fibroma of tendon sheath is a distinct entity of probable reactive, non-neoplastic nature.

Angiography in myeloma (plasmacytoma). A correlated angiographic and histologic study.

Angiography was performed in ten cases of myeloma (plasmacytoma), of which nine were solitary on admission. All lesions were hypervascular bone tumors with extension of neoplastic growth into adjacent soft tissue. Contrast uptake of the tumors occurred regularly and usually was non-homogeneous. In nearly all cases irregular tumor vessels and early venous drainage was evident with arteriovenous shunting in three. Pathologic-anatomic correlation demonstrated 'tumor vessels' to be newly formed vascular spaces lacking the normal constituents of vessel walls. The contrast uptake presumably was caused by passage of contrast into the newly formed, slit-like capillary vascular spaces. Angiography usually permitted separation of myeloma from benign, hypervascular bone lesions. The procedure proved to be of particular value in indicating definite malignancy, since myeloma was considered initially as the probable diagnosis in only one of the series. It was not possible, however, to differentiate myeloma from other malignant tumors by plain radiography or angiography. Irreversible renal failure occurred in one patient after angiography.