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At least 19 recordsLinked to original sources
PubMed1981

Lymphangiosarcoma.

Forty-four cases of lymphangiosarcoma treated at Memorial Sloan-Kettering Cancer Center were reviewed. With the exception of four patients with primary lymphangiosarcoma of the scalp, all patients had had chronic lymphedema of the involved limb for many years, usually following mastectomy for breast carcinoma, but occasionally due to other cases. Although chronic lymphedema could be implicated in the etiology of lymphangiosarcoma in all patients with neoplasms of an extremity, a significant number of patients did not have a history of radiation therapy at the site where their tumor developed. Histologically, although there were no differences in the lymphangiosarcomas between any of the groups of patients, the morphology of the tumors was influenced in the individual patient by the size and anatomic site of the sarcoma. Early amputation seemed to give the best chance of long-term survival, with responses to wide resection, chemotherapy, and radiation therapy generally only of short duration. Amputation after local recurrence was ineffective in preventing pulmonary metastases and death. At present, early amputation appears to be the treatment of choice for patients with lymphangiosarcoma.

Adult↗
PubMed2003

Cutaneous lymphangiosarcoma in a young dog: clinical, anatomopathological and lectinhistochemical description.

Lymphangiosarcoma is a rare tumour in domestic animals arising from lymphatic endothelial cells. Occasionally, microscopic differentiation with haemangiosarcoma may be difficult. The aim of the present study was to describe a lymphangiosarcoma in a 1-year-old female Doberman Pinscher dog and to characterize its lectinhistochemical binding pattern as compared with that of haemangiosarcoma. The dog was presented because of a cutaneous painful swelling located in the left axilla. Histological diagnosis confirmed lymphangiosarcoma. The dog was killed. Necropsy revealed mediastinal lymph nodes' involvement. Twenty lectins were tested in tissue sections of this case as well as in four haemangiosarcomas from other dogs. Staining intensity was issued upon optical density determinations. Percentage of lectinhistochemical staining area was also conducted. RCA-I showed the most intense and wide distributed labelling pattern for lymphangiosarcoma. PHA-E was the counterpart for haemangiosarcoma. Should similar results be obtained in further studies, such differences could aid in the differential diagnosis between lymphangiosarcoma and haemangiosarcoma when histological pictures were not conclusive.

Animals↗
PubMed1981

Lymphangiosarcoma after filarial infection.

A case of lymphangiosarcoma of a lower extremity is described in a patient with chronic lymphedema of that leg from a filarial infection in childhood. Histologically, the neoplasm resembled lymphangiosarcomas that arise in arms that become lymphedematous after mastectomies, but was different in that it also contained areas of calcification consistent with prior filarial infection. Calcifications were also present in muscle uninvolved by the lymphangiosarcoma of this case. The prolonged survival of this patient is unlike that of most patients with lymphangiosarcoma, which is generally shorter. Although lymphedema after filariasis is common, this is the first case of a lymphangiosarcoma arising in chronic lymphedema of filarial origin.

Filariasis↗
PubMed1995

Lymphangiosarcoma following mastectomy.

This study reviews 16 cases of lymphangiosarcoma of the upper extremity after mastectomy for breast cancer (Stewart-Treves syndrome) was done at the author's institution from 1970 to 1992. Lymphangiosarcoma was diagnosed an average of 10.6 years (range, 5.6-18 years) after the diagnosis of breast cancer. Presenting signs included a bruise (6 patients); increased swelling (4); a red, raised lesion (2); a palpable mass (2); a blister appearance (1); and a non-healing eschar with continual bleeding (1). After biopsy, surgical treatment included forequarter amputation (8 patients), wide excision with grafting (5), and above-elbow amputation (1). Local recurrence of the lymphangiosarcoma occurred on the chest wall in 11 of the 16 patients after an average of 10.9 months. Metastasis occurred in 11 patients. (Metastasis occurred in 1 patient without local recurrence, and 1 patient with locally recurrent lymphangiosarcoma died of breast cancer before evidence of metastatic lymphangiosarcoma.) There are 2 long-term survivors who have lived 9.3 and 6 years, respectively, since the operation. One had been treated with a forequarter amputation and the other with wide excision. Early recognition and surgical treatment seem to offer the only chance for long-term survival.

Adult↗
PubMedDate not supplied

Lymphangiosarcoma of the pubic region: a rare complication arising in congenital non-hereditary lymphedema.

Lymphangiosarcoma is a rare, aggressive, vascular neoplasm arising in chronic congenital or acquired lymphedema. Although it is most frequently associated with post-mastectomy lymphedema (Stewart-Treves's syndrome), lymphangiosarcoma can exceptionally arise in congenital hereditary lymphedema (Milroy's syndrome and Meige's syndrome) and non-hereditary lymphedema (congenital, praecox or forme tarde lymphedemas). We report a case of lymphangiosarcoma of the pubic region, supported by immunohistochemical studies, in a 42-year-old woman affected by congenital, non-hereditary lymphedema of the left genital region and homolateral lower limb. In addition, molecular analysis demonstrated the absence of Kaposi's sarcoma-associated Herpes virus (KSHV) DNA sequences in tumour lesions. To our knowledge, this is the first case of lymphangiosarcoma associated with congenital non-hereditary lymphedema confined to the pubic region. The literature concerning the cases of lymphangiosarcoma arising in congenital hereditary and non-hereditary lymphedema is reviewed. Moreover, we emphasized the importance of regular clinical controls in all patients affected by chronic lymphedema. In fact, although the prognosis of this neoplasm is very poor, a prompt diagnosis and a rapid, ablative surgery associated with radiation therapy can increase the possibility of survival of these patients.

Adult↗
PubMed1979

Postmastectomy lymphangiosarcoma: experience with three patients and electron microscopic observations in one.

The clinical features are presented of three patients in whom lymphangiosarcoma developed after radical mastectomy followed by local irradiation. The median time between radical mastectomy and the diagnosis by biopsy of lymphangiosarcoma was 9.9 years. No form of therapy, including high-dose combination chemotherapy, appeared able to control the disease. All three patients died with residual lymphangiosarcoma and in two it was the direct cause of death. These results stress that prevention of postmastectomy lymphangiosarcoma is vital. This can be accomplished by avoiding radical mastectomy followed by local irradiation. Ultrastructural observations in one case suggest that the tumour has a primitive vasoformative origin.

Adult↗
PubMed1993

Lymphangiosarcoma of arm after chronic lymphedema: a rare long-term complication after radical mastectomy in breast cancer patients. Case report and overview.

Lymphangiosarcoma after mastectomy was first described by Stewart and Treves in 1948. Today, this tumor associated with chronic lymphedema has become a rare entity, due to less radical surgery. Chronic lymphedema and lymphangiectasia of limbs preceding lymphangiosarcoma may not only be induced by radical mastectomy. Also post-traumatic, congenital, filarial-associated or spontaneous chronic lymphedema may be associated with lymphangiosarcoma. A time interval of years seems to be required before malignant changes supervene and lymphangiosarcoma develops. This paper describes a case with lethal and arising in an edematous arm years after radical mastectomy and irradiation. Current concepts of etiology, histopathology, immunohistology, diagnostic investigation, treatment and prognosis are presented.

Aged↗
PubMed1995

Lymphangiosarcoma in late-onset hereditary lymphedema: case report and nosological implications.

Hereditary lymphedemas that are not associated with other malformations usually affect the lower limbs and are inherited in an autosomal dominant fashion. These non-syndromic hereditary lymphedemas are categorized by their age of onset, being either congenital (Milroy disease) or having an onset in childhood or around puberty (Meige disease). We describe a family in which three individuals in three generations had unusually late onset of lymphedema in their mid-twenties or thirties. The proband additionally developed a very rare lymphangiosarcoma. This tumor, usually associated with post-mastectomy lymphedema, has not been described in late-onset hereditary lymphedema. Because of an unusually high incidence of multiple primary tumors in association with lymphangiosarcoma in the literature (approximately 10%) and the proband's own familial cancer background, we speculate that an inherited predisposition to malignancy may underlie the development of lymphedema-associated lymphangiosarcoma.

Adult↗
PubMed2002

Isolated limb perfusion with tumor necrosis factor and melphalan for nonresectable sSewart-Treves lymphangiosarcoma.

BACKGROUND: Cutaneous Stewart-Treves lymphangiosarcomas represent a rare group of tumors characterized by a high grade of vascularization and by localization in an extremity with lymphedema. The multifocality and the localization makes these tumors eligible for treatment with isolated limb perfusion (ILP). ILP with tumor necrosis factor (TNF) and melphalan is a safe and highly effective procedure that can achieve limb salvage in >or=80% of all patients with nonresectable extremity soft tissue sarcoma or melanoma. METHODS: In 10 patients with multifocal Stewart-Treves lymphangiosarcoma of the extremities, 16 ILPs with TNF plus melphalan were performed. All patients would have been candidates for exarticulation of the extremity. RESULTS: We observed an 87% overall response rate (complete and partial responses); one patient had a mixed response, and one patient did not respond to the therapy. In nine perfusions (56%), a complete response was achieved, and five perfusions (31%) resulted in a partial response. Limb salvage was achieved in eight patients (80%), with a mean follow-up duration of 34.8 months (range, 3 to >or=115 months). Regional toxicity was limited and systemic toxicity minimal to moderate, with no toxic deaths. CONCLUSIONS: Multifocal Stewart-Treves lymphangiosarcomas in extremities with chronic lymphedema can be successfully treated by ILP with TNF and melphalan.

Aged↗
PubMed1995

Lymphedema-induced lymphangiosarcoma.

A series of eight patients with chronic lymphedema-related lymphangiosarcoma is presented. All but one case showed a typical rapid progression and fatal outcome, as has been reported in other series. In one patient the lymphangiosarcoma developed on the chest wall, the axilla and the arm where persisting lymphedema and fibrosis occurred after bilateral mastectomy and bilateral postoperative radiotherapy. In this patient an asymptomatic course and slow locoregional progression of lesions was seen. The clinical picture, the etiological considerations and the therapeutic options of the lymphedema-induced lymphangiosarcoma with regard to the literature are discussed.

Aged↗
PubMed1999

Lymphangiosarcoma in chronic lymphoedema. Stewart-Treves syndrome.

A case of post-mastectomy lymphangiosarcoma is reported. Lymphangiosarcoma is an extremely rare but highly lethal complication of chronic lymphoedema. Our patient was treated by amputation and died 6 months later. Treatment of post-mastectomy lymphangiosarcoma is still unsatisfactory. Early recognition and radical ablative surgery seem to provide best chance for survival.

Amputation, Surgical↗
PubMed2004

Feline lymphangiosarcoma--definitive identification using a lymphatic vascular marker.

Three cases of feline exudative dermatitis associated with lymphangiosarcoma are described. The animals, an 11-year-old, neutered male and two 10-year-old, neutered female short hair European cats, presented with a 2-month history of transparent liquid oozing from the skin of the groin and caudal abdomen. On physical examination the neutered male cat and one of the females were slightly depressed and showed loss of weight. Skin lesions were similar in all cats and characterized by the presence of alopecia and moist dermatitis in the ventral abdomen, groin and inner thigh. The hair at the periphery appeared matted by the fluid. In all three cases, histopathological examination of skin biopsies from the abdomen identified poorly defined neoplasia involving dermis and subcutis, characterized by proliferation of spindle cells aligned along pre-existing collagen bundles. The dissection of collagen bundles gave rise to irregular shaped anastomosing, often blind-ending vascular channels and trabeculae. Vascular spaces were mostly optically empty. These histological features were strongly suggestive of lymphangiosarcoma. Neoplastic cells were positive for the blood vascular marker Von Willebrand factor, and a lymphatic vascular marker LYVE-1 (Lymphatic Vessel Endothelial receptor - 1), demonstrating the mixed vascular origin of the tumour. Ultrastructural findings confirmed the final diagnosis of lymphangiosarcoma.

Abdomen↗
PubMed1999

Lymphangiosarcomas in cats: a retrospective study of 12 cases.

Clinical, macroscopic, and histologic features of 12 lymphangiosarcomas in cats are described. Nine tumors were located in the subcutaneous tissue at the caudoventral abdominal wall (eight cats) or in the neck (one cat). The remaining three cats had lymphangiosarcomas around the cranial mesenteric artery (two cats) or precardial in the mediastinum (one cat). Macroscopically, the tumors were noncircumscribed, white, edematous, and intermixed with fat tissue. Histologic features varied from cleft-forming and cavernous growth to papilliform and solid patterns. Follow-up data were available for seven cats with subcutaneous lymphangiosarcomas. All these cats died or were euthanatized within 6 months after surgery because of poor wound healing, local recurrence, or distant metastases. The cats with abdominal or thoracic masses were either euthanatized at surgery or within 6 months after the first surgery because of recurrent chylothorax, chyloperitoneum, or distant metastases.

Abdominal Muscles↗
PubMed2005

Lymphangiosarcoma of dogs: a review.

Lymphangiosarcoma in dogs, an extremely rare tumour with only 16 cases reported in the literature, is reviewed. Lymphangiosarcoma in humans, also very rare, and known in post-mastectomy, chronically-lymphoedematous patients as 'Stewart-Treves' syndrome, is briefly outlined, as well as the various other causes of lymphoedema, both primary and secondary, which usually precede malignancy. Comparisons between human and canine lymphoedema are made when such references were found. The genetic links to primary lymphoedema and the manifestation thereof in humans are mentioned. Lymphangiosarcoma in the majority of human and canine patients is an aggressively malignant tumour with few patients surviving despite various attempted treatments. The tumour most commonly arises in the subcutaneous tissues and rapidly invades underlying tissues and may spread widely internally via haematogenous and lymphatic routes, with frequent pleural and chest involvement. The tumour has been reported mostly in medium- to large-breed dogs, in slightly more males than females, and in an age-range of 8 weeks to 13 years, with more cases aged 5 years and older. Methods of diagnosis, with the variations encountered, including routine histopathology, immunohistochemistry, electron microscopy, tissue culture characteristics and endothelial expression of glycocongugates, are discussed.

Age Factors↗
PubMed2005

Lymphangiosarcoma in a 3.5-year-old Bullmastiff bitch with vaginal prolapse, primary lymph node fibrosis and other congenital defects.

Lymphangiosarcoma is an extremely rare tumour in dogs with only 16 cases reported in the literature. Lymphoedema, which may be primary due to defects in the lymphatic system, or secondary to various other pathologies, often precedes malignancy. Of the 16 canine reports, only 1 dog was confirmed as having had prior primary lymphoedema due to aplasia of the popliteal lymph nodes. A case of lymphangiosarcoma is described in a 3.5-year-old purebred, Bullmastiff bitch which presented with vaginal blood 'spotting' for 3 weeks after cessation of oestrus, during which intromission by the male had been unsuccessful. During ovariohysterectomy a large multicystic, proliferative, spongy, fluid-filled, brownish-red mass surrounding the cervix and projecting into the abdominal space was removed with the cervix, and a diagnosis of lymphangiosarcoma made on histological and electron microscopic examination of the tissue. Ultrastructurally, no basement membrane or pericytes were found, only some of the neoplastic endothelial cells were linked by tight junctions while there were gaps between others, and neither micropinocytotic vesicles nor Weibel-Palade bodies occurred in the cells examined. Very few of the endothelial cells lining the many interlinking, tortuous maze of channels, stained slightly positive immunohistochemically for factor VIII-related antigen. The channels were filled mostly with serous fluid, and occasionally mixed leucocytes and some erythrocytes. The endothelium was often associated with underlying blocks of collagenous material, as well as loosely-arranged aggregates of lymphocytes, other mononuclear cells and occasional neutrophils in the connective tissue septae and more prominently perivascularly. The bitch was discharged on antibiotic treatment but returned 2 weeks later with apparent prolapsed vagina which failed to reduce over the next week. Laparotomy revealed the tumour to have spread extensively in the caudal abdomen to involve the broad ligament and the ventral rectal serosa, and the 'prolapsed' tissue was found to be expanded vaginal wall. The bitch was euthanased and necropsied, Histological examination confirmed lymphangiosarcomatous invasion of the submucosal and muscular layers of the retroperitoneal, traumatised, prolapsed part of the vagina, the urethra and the ventral rectal wall. The broad ligament was diffusely invaded with tumour which had proliferated into the caudal abdominal space, and 3 small intra-trabecular foci of tumour were found in the right popliteal lymph node near the hilus. Mitotic figures were generally scarce. There was mild subcutaneous oedema of the ventral trunk extending from the axillae to the inner proximal thighs, which had not been evident clinically, and the lymph nodes (peripheral more so than internal) microscopically showed marked trabecular and perivascular fibrosis especially in hilar regions. Other congenital defects were hepatic capsular and central venous fibrosis with lymphatic duplication and dilatation in all areas of connective tissue, ventrally-incongruous half-circular tracheal rings, and multifocal renal dysplasia affecting the right kidney. There was locally-extensive subacute pyelonephritis of the left kidney.

Animals↗
PubMedDate not supplied

Chylothorax in a dog with pulmonary lymphangiosarcoma.

Lymphangiosarcoma of the pulmonary pleura was found to be the cause of persistent chylothorax in an eight-year-old, intact male golden retriever. After a two-month course of medical management, a lymphangiogram and thoracic duct ligation were performed with the objective of decreasing further effusion. At surgery, gross lung pathology was biopsied and yielded a histopathological diagnosis of pulmonary pleural lymphangiosarcoma. A relatively rare tumor, lymphangiosarcoma of the pulmonary pleura has not previously been documented as a source of chylothorax in the dog.

Animals↗
PubMed1975

Lymphangiosarcoma arising in congenital lymphedema.

An 85-year-old woman with congenital lymphedema of the right upper extremity developed a small purplish papule on the forearm, which was the first clinical evidence of lymphangiosarcoma. The lesion grew rapidly and became necrotic and ulcerated. The patient experienced severe pain. A mid-arm amputation was performed. Microscopical examination of the amputated limb showed widespread histological involvement of skin, muscle, and subcutaneous tissue by lymphangiosarcoma. The need for continuous monitoring of patients with both primary and secondary lymphedema is emphasized, since early diagnosis of the developing lymphangiosarcoma and rapid surgical intervention provide the best prognosis for survival.

Aged↗
PubMed1990

Response of postmastectomy lymphangiosarcoma to radiotherapy: report of four cases.

We have reported on four previously unpublished cases of postmastectomy lymphangiosarcoma. All of these cases had suffered from a long-lasting lymphedema of the upper extremity. Only one patient had survived over one year. After radiotherapy she lived four years from first lesion of lymphangiosarcoma. She died locally disease-free in spite of two earlier recurrences, which were also treated with radiotherapy. We believe that only in early cases can the radiotherapy give a good palliation in postmastectomy lymphangiosarcoma.

Aged↗