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Background: Cutaneous vascular proliferations that occur in the field of prior radiotherapy include angiosarcoma and small, cutaneous lesions with a pseudosarcomatous pattern that previously were reported as atypical vascular lesions or benign lymphangiomatous papules., Methods: The objective of this study was to investigate the clinicopathologic features and outcomes of 56 radiation-induced vascular proliferations that occurred in 36 patients who received previous treatment for breast carcinoma. Data from all patients were retrieved from the files of the French Sarcoma Group. Immunostaining with D2.40 antibody was performed in 24 lesions., Results: All patients (median age, 52 years) had received external radiotherapy. Small papules developed within the field of prior radiotherapy (median latency interval, 66 months). Microscopically, the lesions were relatively well circumscribed, and they were located mostly in the superficial/middermis. They were composed of dilated or irregular-jagged vascular channels that were lined by a single layer of bland endothelial cells, and they demonstrated either a predominately lymphangioendothelioma-like or lymphangioma/lymphangioma circumscriptum-like growth pattern. Micropapillary tufts were common findings. Ten lesions showed additional cytologic and/or architectural atypia. Twenty of 24 lesions showed D2.40 positivity. Follow-up information was available for 31 patients (median follow-up, 48 months): Five women developed new cutaneous lesions, and 1 woman had spontaneous regression of her lesions. None of the patients developed cutaneous angiosarcoma. Five patients were lost to follow-up., Conclusions: Although vascular proliferations in irradiated skin may mimic angiosarcoma morphologically, the large majority of these lesions showed a benign clinical outcome. Despite relatively limited follow-up, the current results indicate the benign nature of these vascular proliferations.

Background: Among the molecules important to angiogenesis and lymphangiogenesis is vascular endothelial growth factor receptor 3 (VEGFR-3), a member of the receptor tyrosine kinases of endothelial cells. This receptor is expressed consistently in normal lymphatics, lymphangiomas, and in Kaposi sarcoma, but data regarding other vascular tumors are scant., Methods: In this study the authors immunohistochemically examined VEGFR-3 expression in 82 benign, 31 borderline, and 72 malignant vascular tumors using a monoclonal antibody to VEGFR-3, heat-induced epitope retrieval, and an avidin-biotin-peroxidase detection system., Results: Although normal mesenchymal tissues showed VEGFR-3 only in the lymphatics, benign and malignant vascular tumors and neovascularization of nonendothelial tumors showed widespread VEGFR-3 distribution. All lymphangiomas and Kaposi sarcomas showed consistent VEGFR-3 reactivity. Among the hemangiomas, spindle cell hemangiomas and 80% of capillary (including all lobular capillary hemangiomas) were positive whereas the endothelium of cavernous, venous, and epitheloid hemangiomas were positive in a minority of cases (20%, 27%, and 33%, respectively). Among the borderline lesions, Kaposiform hemangioendotheliomas were intensely positive whereas epithelioid hemangioendotheliomas were positive in 11 of 29 cases (38%). Angiosarcomas showed VEGRF-3 reactivity in the majority of cases (48 of 60 cases; 80%). The nonepithelioid variants more often were positive (40 of 45 cases; 89%) than the epithelioid variants, of which 8 of 15 (53%) showed positive tumor cells. Nonvascular tumors (including perivascular tumors, other sarcomas, melanomas, carcinomas, and large cell lymphomas) consistently were negative whereas tumor neovascularization commonly was VEGFR-3 positive., Conclusions: The results of the current study show that although VEGFR-3 shows specificity toward lymphatics in normal tissues, this receptor is distributed extensively in benign and malignant vascular tumors and therefore can be considered a novel marker in the assessment of endothelial cell differentiation of vascular neoplasms., (Copyright 1999 American Cancer Society.)

Background: Pancreatic lymphangiomas are rare benign tumors, of which only a few cases have been reported in the literature. In this study, the authors present a series of primary pancreatic lymphangiomas., Methods: Cases of nonepithelial pancreatic cystic tumors (lymphangiomas) diagnosed between 1966 and 1994 were retrieved from the Endocrine Pathology Registry of the Armed Forces Institute of Pathology. Histologic features (in 10 cases) as well as histochemical and immunohistochemical studies (in 6 cases) were reviewed. Long term patient follow-up data were obtained in 9 cases., Results: The patients included 8 females and 2 males ages 2-61 years (mean age, 28.9 years) at initial presentation. The tumors were circumscribed and occurred predominantly (in 6 of 10 cases) in the tail of the pancreas. The multicystic, serous, or chylous fluid-filled cystic tumors ranged from 3 to 20 cm (average, 12.7 cm) in greatest dimension. Histologically, the tumors consisted of multilocular cystic spaces of various sizes, lined by endothelial cells. The stroma contained smooth muscle and mature lymphocytes. Immunohistochemistry determined the endothelial lining cells to be factor VIII-R antigen and CD31 positive (in all cases tested) but usually CD34 negative. All patients for whom follow-up data were obtained (n=9) were alive without evidence of disease an average of 7.2 years after initial diagnosis., Conclusions: Pancreatic lymphangiomas occur predominantly in females within a wide age range. Multilocular, fluid-filled cysts, with endothelial immunoreactivity for factor VIII-R antigen and CD31, are characteristic of these tumors. Complete surgical excision of these benign tumors resulted in excellent long term prognoses for all patients studied.

A review of 2369 cases of resected primary colorectal carcinoma identified 15 cases of diffusely infiltrating colorectal carcinoma. Depending on the histologic pattern and extent, these cases were classified as linitis plastica (LP) type (seven cases) or lymphangiosis (LA) type (eight cases). The LP type consisted of poorly differentiated adenocarcinoma or signet ring cell carcinoma; it showed an annularly thickened and rigid bowel wall. The LA type consisted of moderately differentiated adenocarcinoma. The colon wall of the LA type was hemilaterally thickened at the mesenteric side. The prognosis was extremely poor for both types. The LP type mainly had peritoneal dissemination (70%), but the LA type frequently had distant metastasis (88%). Early diagnosis and curative operation are important for both types; however, the LA type also requires therapy for distant metastasis.

Of the several types of treatment for cystic hygromas and lymphangiomas, surgical excision has been the preferred treatment. However, there is a high recurrence rate because lymphangiomas tend to infiltrate the surrounding tissues. Bleomycin in a microsphere-in-oil (S/O) emulsion was used in this study as a sclerosing agent for lymphangiomas. Experimental studies using domestic rabbits showed that the bleomycin emulsion caused more marked fibrotic changes at the injection site than other formulations, such as a blank emulsion and bleomycin solution. In clinical trials, 27 of 33 patients received bleomycin S/O emulsion injected directly into the tumors with satisfactory results. Histologic pictures of the clinically resected specimens confirmed the findings of the experimental studies. Comparative studies of treatments between bleomycin S/O emulsion and surgery indicated that injection therapy of bleomycin S/O emulsion would be more beneficial than surgical excisions.

Three cases of pulmonary lymphangiomyomatosis are described, with emphasis on the ultrastructural changes. The clinicopathologic features corresponded to those previously described. Each patient was a female in the reproductive years; breathlessness and recurrent pneumothoraces were the predominant clinical features. Histologically, the lungs showed a focal interstitial infiltrate of short, spindle-shaped mononuclear cells compatible with primitive smooth muscle, which was associated with irregular emphysema and hemosiderosis. Electron microscopy confirmed the smooth muscle nature of the pulmonary infiltrate and showed the presence of cells intermediate between smooth muscle and fibroblasts. Abnormalities were also noted in the pulmonary connective tissue that are possibly related to the fragility of the lung in this condition.

An 89-year-old asymptomatic white woman was found to have a pelvic mass with retroperitoneal extension. Biopsy of the lesion demonstrated lymphangioleiomyomatosis. Review of her records revealed that 36 years ago a mass in the same location had been found incidentally and biopsy was performed during abdominoperineal resection for rectal carcinoma. Comparison of the old and recent biopsy specimens showed an identical histologic appearance. This case, therefore, represents a benign, localized form of lymphangioleiomyomatosis; the long survival without therapy may be related to the fact that this patient was postmenopausal.

A 54-year-old woman, presenting with chyluria and progressive dyspnea, is reported. Necropsy revealed co-existent lymphangiomyomatosis and pneumoconiosis in the lungs. Retroperitoneal lymphangiomyomas surrounded the distal ureters, and cystoscopy revealed pyelolymphatic backflow into renal lymphatic sinuses. The finding of chyluria with atypical smooth muscle hyperplasia within the ureters is extremely rare. Previously reported cases of lymphangiomyomatosis are discussed and compared with our own. Electron micrographs of involved lymph nodes are presented and the findings briefly evaluated.

Lymphangiomyomyoma is a tumor of lymphatic channels and lymph nodes, clinically manifested by recurrent chylous pleural effusions and ascites. The disease is usually progressive and unresponsive to surgery, chemotherapy, or irradiation. A case of lymphangiomyomatosis in a 24-year-old woman who presented with chylous ascites is described. The patient was treated with dietary fat restriction supplemented with medium chain triglycerides and has remained free of recurrent effusions for 2 years.

Lymphangioma circumscriptum, a rare long-term complication of chronic lymphedema, has been described only twice following radical mastectomy. The case discussed in this report developed 25 years after radical mastectomy and radiation therapy. Both this condition and lymphangiosarcoma, its malignant counterpart, may arise in lymphedematous extremities regardless of the etiology of the lymphatic obstruction.