Sclerosing angiomatoid nodular transformation of the spleen related to IgG4-associated disease: report of a case.

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular mass, with fewer than 100 cases documented. It is generally recognized as a vascular lesion that develops in the red pulp of the spleen; however, its pathogenesis is not clearly defined. We report a case of SANT of the spleen, which presents evidence to support the hypothesis that this disease entity is associated with IgG4-associated disease. Microscopically, the tumor was composed of multiple vascular structures separated by fibrous connective tissue and immunohistochemical examination revealed positive staining for CD31, CD34, factor VIII, and IgG4. Further research based on large number of cases is warranted to clarify the pathogenesis of this tumor.

The basic chromospheres of skin that might not be differentiated visually: observations on aneurysmal fibrous histiocytomas and malignant melanomas.

AIM: Aneurysmal fibrous histiocytoma (AFH) is a variant of fibrous histiocytoma, which has a cleft-like cavernous blood-filled space in the tumor. It appears as a single reddish black tumor with variable levels of pain and size from its bleeding. And, it must be differentiated from other similar looking malignant conditions such as malignant melanoma. The visual mimicry of AFH to melanoma was raised by some careful dermatologists, but never be confirmed objectively by colorimetric analysis. MATERIALS, SUBJECTS, AND METHODS: In this study, we simply analyzed conventionally photographed digital images of thirty-seven cases of fibrous histiocytomas, including three AFH cases into colorimetrically useful color space, CIELAB, of which coordinates are L*, a*, and b* representing lightness, red to green, and yellow to blue axis, respectively. In addition, we also analyzed the clinical digital images of seven cases of malignant melanomas. Using statistical package, each coordinates of CIELAB were compared using Wilcoxon rank sum test between AFH and melanomas. The CIELAB coordinates of AFH and non-aneurysmal fibrous histiocytomas were compared statistically as well. RESULTS: Comparing with banal fibrous histiocytomas, the colors of AFH showed significantly smaller a* and b* coordinates (P = 0.008, 0.008, respectively), which implies more green and blue hue of AFH lesion. Rather, they were more like melanomas (P = 0.2839, 0.2040, respectively). As for L*, there were no significant differences for all comparisons. CONCLUSIONS: As a result, more objective analysis of the digital images using colorimetric color space confirmed the visual mimicry of AFH to melanoma.

Collapsing angiokeloidal dermatofibroma.

A heterogeneous group of benign fibrohistiocytic lesions has been assembled under the umbrella term, dermatofibroma. These lesions share a morphology of bland spindled cells encompassed by and intercalating through thick dermal collagen; unique variants have been described based on secondary histologic features, some of which include aneurysmal, myxoid, lipidized, signet ring, angiomatous, and keloidal. Here, we present a distinct dermatofibroma variant henceforth known as collapsing angiokeloidal dermatofibroma identified in 2 patients with slowly growing nodules of the buttock and the arm. Microscopically, the lesions have a characteristic dermatofibroma appearance but are accompanied by unusual diffuse small caliber vessels whose walls are collapsed by a thick, eosinophilic, keloid-like substance. The eosinophilic material resembles the adjacent dermal collagen; however, it does not stain for type-4 collagen or type-1 procollagen, amyloid, or glycogen. Although the exact composition of the keloidal material remains ambiguous, the architectural novelty of collapsing angiokeloidal dermatofibroma serves to further expand the morphologic spectrum of benign fibrous histiocytomas, although highlighting the difficulty in distinguishing between it and similar lesions.

Presentation of an epithelioid cell histiocytoma on the ventral tongue.

The epithelioid cell histiocytoma (ECH) is a polypoidal benign tumor of superficial connective tissue that is often diagnosed as a pyogenic granuloma. ECHs are speculated to originate from dermal dendritic subunits and are composed of 2 primary cell populations, ie, CD34+ primitive fibroblastic dendrocytes and factor XIIIa+ histiocytes. Although dendritic subunits are distributed throughout most collagenous tissues inclusive of oral mucosa, to date, all reported cases of ECH have been cutaneous lesions. ECHs' putative pathogenesis entails activation of CD34+ "sentinel" reserve dendrocytes, followed by an influx of histiocytes and mast cells. Juxtacrine communication increases release of wound healing factors; suggesting a reactive etiologic component. In this current case, the location (ventral tongue) and history (recent increase in size) suggest the possibility that trauma could have initiated the dendritic subunit "wound healing" cascade. Consistent with its benign course, the ECH is managed by local excision, and has an excellent prognosis.

Histological vascular invasion by tumors is a risk factor for distant metastasis in malignant fibrous histiocytoma.

BACKGROUND: The appearance of distant metastasis is a fatal sign in patients with soft tissue sarcoma. Therefore, we studied the risk factors associated with distant metastasis that appear in malignant fibrous histiocytoma. MATERIALS AND METHODS: The study group comprised sixty patients treated for malignant fibrous histiocytoma at our hospital between 1991 and 2002. We retrospectively studied whether age, tumor size, tumor site, developmental form, surgical margin, local recurrence, histological subtype, vascular invasion and histological grade are risk factors associated with the appearance of distant metastasis. RESULTS: A univariate analysis showed that, for those patients older than 70 years of age, insufficient surgical margin, the presence of local recurrence and vascular invasion are significant risk factors associated with the appearance of distant metastasis. A multivariate analysis showed only the presence of vascular invasion to be a significant risk factor as well. CONCLUSION: Histological vascular invasion by tumors is a risk factor associated with distant metastasis that appears in malignant fibrous histiocytoma. Taking early measures for patients with vascular invasion is necessary to improve the prognosis.

Cyclooxygenase-2 in human malignant fibrous histiocytoma: correlations with intratumoral microvessel density, expression of vascular endothelial growth factor and thymidine phosphorylase.

Cyclooxygenase-2 (COX-2) has been found to be up-regulated in several types of human malignant tumors and proposed to have a role in the angiogenic process. This study examined the expression of COX-2 in two human malignant fibrous histiocytoma (MFH) cell lines by Western blotting, which showed a specific single band at 72 kDa. Immunohistochemistry was conducted in 35 MFHs and 30 benign fibrohistiocytic tumors (BFHTs), comparing the expression of vascular endothelial growth factor (VEGF) and thymidine phosphorylase (TP), as well as intratumoral microvessel density (IMVD). COX-2 expression was noted in 22 (62.9%) MFHs, but in no BFHT. IMVD values were significantly higher in the MFHs (90.6+/-8.0) than BFHTs (27.9+/-3.1), and also in the COX-2 positive (104.5+/-11.3) than negative (67.2+/-5.8) MFHs. VEGF and TP expression was also associated with a significantly higher level of COX-2, as well as greater IMVD. The highest IMVD values were noted in the 17 MFHs (120.8+/-11.5) expressing all three factors. Clinical analysis demonstrated poorer survival in the 18 COX-2 positive MFHs than in the 10 negative ones, although the small number of cases did not reveal a significant difference. The results overall indicated that COX-2 expression is associated with intratumoral angiogenesis, which might provide favorable conditions for tumor progression in human MFHs.

Orbital malignant fibrous histiocytoma with extension to the base of the skull--case report.

An 18-year-old woman presented with a malignant fibrous histiocytoma (MFH) originating in the orbit and invading the frontal and temporal base of the skull manifesting as exophthalmos and double vision that had persisted for 2 months. Magnetic resonance imaging revealed a tumor in the left orbit that extended as far as the frontal and temporal base of the skull. The tumor was treated by radical resection with conservation of the eyeball and its contents, followed by orbit wall reconstruction. The histological diagnosis was MFH. Local radiotherapy was administered postoperatively. The preoperative symptoms improved, and there has been no evidence of local recurrence or metastasis in the year since the surgery. In this case, radical resection of the tumor was essential. Furthermore, the adjuvant therapy was apparently successful, probably because this histological type of tumor is highly sensitive to radiotherapy.

Angioarchitecture of tumors induced by two different cloned cell lines established from a transplantable rat malignant fibrous histiocytoma.

Angiogenesis, a biologic process whereby endothelial cells divide and migrate to form new blood vessels, is a key step in tumor growth, invasion, and metastasis. In the present study, we investigated the differences in angioarchitecture between two different tumors induced by cloned cell lines (MT-8 and MT-9), derived from a transplantable rat malignant fibrous histiocytoma, by scanning electron microscopy of vascular corrosion casts. During a 3-week observation period after implantation, the growth of MT-8 tumors appeared to be faster than that of MT-9 tumors. Histologically, MT-8 tumors were of the uniformly undifferentiated sarcoma type arranged in characteristic organoid structures, and MT-9 tumors showed a storiform growth pattern. In MT-8 tumors, neovascularization occurred by sprouting at postimplantation (PI) week 1, and the newly formed capillaries gradually became more tortuous. In MT-9 tumors, at PI week 1, the corrosion casts of newly formed capillaries mainly showed a wavy course but no finger-like outgrowths of capillaries were seen. At PI weeks 2 and 3, the sprouting was seen specifically in MT-9 tumors, forming basket-like structures and glomeruloid structures of capillaries. These results indicate that angiogenesis or angioarchitecture of MT-8 tumors is different from that of MT-9 tumors, depending on the differences in their tumor histology and by the features like absence or presence of basket-like structures and glomeruloid structures of capillaries.

Multinucleate cell angiohistiocytoma: a fibrohistiocytic proliferation with increased mast cell numbers and vascular hyperplasia.

BACKGROUND: Multinucleate cell angiohistiocytoma (MCAH) is an uncommon lesion clinically characterized by multiple papules usually located on the face and acral regions of elderly women. Histopathologically, MCAH is characterized by dermal vascular hyperplasia associated with increased number of factor XIIIa-positive fibrohistiocytic cells and multinucleate cells with scalloped borders. METHODS: We report the clinical, histopathological and immunohistochemical features of three cases of MCAH, with ulstrastructural study in one of them. The patients were a woman and two men of 56, 40 and 70 years of age, respectively. They all had multiple dull-red papules, which had appeared over several years and were located on the face, the trunk and the dorsa of the hands, respectively. RESULTS: The reticular dermis presented a fibrohistiocytic proliferation of factor XIIIa-positive cells, with abundant bizarre multinucleate cells and vascular hyperplasia. Increased mast cell numbers were seen in all cases, often in apposition to multinucleate cells. CONCLUSION: Histopathological differential diagnosis of MCAH includes mainly angiofibromas and dermatofibromas, even though vascular hyperplasia can be prominent and has led to many authors to classify MCAH among vascular tumors. Bizarre multinucleate cells can be found in reactive, neoplastic and inflammatory lesions in many sites of the body, and mast cells can play a role in their morphogenesis.

Platelet and coagulation activation with vascular endothelial growth factor generation in soft tissue sarcomas.

Angiogenesis and activated blood coagulation are involved in tumor growth and metastasis. Although some have suggested that activation of coagulation in tumors is not linked to activation of platelets, no data exist to either support or refute this concept. However, platelet turnover in cancer patients is often increased, and platelets are carriers of angiogenic growth factors. We hypothesized that platelets are involved in tumor-associated angiogenesis. To obtain evidence supporting this hypothesis, we have studied whether the angiogenic and coagulation pathways and platelets are concomitantly activated in cancer patients with soft tissue sarcomas (STSs) using a novel method to detect activated platelets in tumor specimens. Twelve patients with STS were selected on the basis of having intratumoral accumulation of fluid, which was aspirated. These accumulations demonstrated very high concentrations of vascular endothelial growth factor and coagulation factors (including thrombin-antithrombin-complex). Tumor specimens showed dense vascularization with intense vascular endothelial growth factor expression and the presence of activated platelets. Taken together, these results support the concept that angiogenesis, blood coagulation, and platelets are concomitantly activated in STS and support the hypothesis that platelets contribute to tumor-induced angiogenesis.

Color Doppler sonography of focal lesions of the skin and subcutaneous tissue.

We evaluated with color Doppler sonography 71 visible and palpable nodules of the skin and subcutaneous tissue from 51 patients. The nodules were classified as avascular (type I), hypovascular with a single vascular pole (type II), hypervascular with multiple peripheral poles (type III), and hypervascular with internal vessels (type IV). Of the 32 malignant nodules, 9% showed a type I pattern, 50% had a type III pattern, and 41% had a type IV pattern; of the 39 benign nodules, 86% showed a type I pattern and 14% had a type II pattern. The sensitivity and specificity of hypervascularity in malignant lesions were 90% and 100%, respectively, whereas the sensitivity and specificity of hypovascularity in benign lesions were 100% and 90%, respectively. The authors conclude that color Doppler sonography is able to increase the specificity of ultrasonography in the evaluation of nodular lesions of the skin.

Assessment of chemotherapy-induced changes in bone sarcomas: clinical experience with 99Tcm-MDP three-phase dynamic bone scintigraphy.

The aim of this study was to evaluate the value of three-phase dynamic bone scintigraphy (TPBS) in the assessment of the response of bone sarcomas to pre-operative chemotherapy and to correlate serial scintigraphic changes with histological findings. The study group comprised 27 patients (osteogenic sarcoma, n = 20; Ewing's sarcoma, n = 5; malignant fibrous histiocytoma, n = 2) with a mean age of 19.2 years. All patients received 99Tcm-methylene diphosphonate TPBS before and after pre-operative chemotherapy. Each phase of the imaging procedure was interpreted qualitatively and quantitatively. The percentage of tumour necrosis was analysed on resection materials following surgery. Histologically, 12 patients were non-responsive (tumour necrosis less than 90%) and 15 patients were responsive (tumour necrosis more than 90%). A decrease in the tumour blood flow ratio and extension were the most notable findings in the responders. The mean change in the tumour blood flow ratio following therapy was 58.7 +/- 8.3% and 19.9 +/- 26.6% (P < 0.005) in responders and non-responders respectively. The accuracy of three-phase imaging and static bone scintigraphy was 88% and 74% respectively. Since bone scintigraphy is a valuable technique owing to its ability to detect distant metastases in clinically early disease, TPBS should be helpful in monitoring therapy effects without any additional cost or radiation dose.

Fistula formation from neovascularity developing in malignant fibrous histiocytoma of the heart.

Neovascularity in cardiac tumours is uncommon and fistula formation from such neovascularity is extremely rare. Fistula formation from neovascularity developing in a malignant fibrous histiocytoma of the heart is described in a 48-year-old man. The the case illustrates that cardiac tumour is not only a cause of cardiac output obstruction but also of coronary artery fistula.

A case of malignant fibrous histiocytoma of the ilium, evaluation by blood-pool scintigraphy.

A rare case of malignant fibrous histiocytoma (MFH) of the ilium was presented and blood-pool scintigraphic images and angiographic images were correlated. Hypervascular tumor and lacking of contrast material with arteriovenous shunts were shown by angiography. Intensive tracer accumulation was shown by blood-pool scintigraphy. After radiation therapy and transcatheter arterial embolization of the tumor, tracer accumulation was noticeable reduced. These findings suggested a vascular tumor such as angiosarcoma, but surgery revealed MFH of the bone. Blood-pool scintigraphy was useful in the evaluation of the vascular characteristics of the tumor.