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PubMed1978

Chemotherapy of sarcoma. A comparison of three regimens.

Three regimens have been used in the chemotherapeutic management of 75 adults with sarcoma. Methotrexate alone was used in 41 cases, a five-drug regimen was given in 12 cases, and adriamycin, T.I.C.-mustard, and methotrexate were given to 22 patients. Remissions, sometimes prolonged, were achieved with each regimen, and none was obviously better than the others. Because of the higher response-rate of primary or locally recurrent tumours it is suggested that sensitivity to drugs should be assessed earlier in the disease. It is proposed that a randomised study of methotrexate versus combination chemotherapy should be started in particular sarcoma subgroups, such as leiomyosarcoma and fibrosarcoma.

Adult↗
PubMed1978

Vascular and lymphatic tumors in infancy, childhood and adulthood: challenge of diagnosis and treatment.

The essential features of the major benign and malignant vascular tumors affecting infants, children and adults have been presented. Major emphasis has been placed upon the classification and treatment of hemangiomas, although malignant vascular tumors are discussed, and extensive and selected references are cited. The classification system of hemangiomas presented in this monograph is based upon the clinical prognosis of the angioma. The principles of treatment for hemangiomas based upon our clinical classification are discussed and may be summarized as follows: 1. The diagnostic features and prognosis vary with the variety of the angioma. 2. Spontaneous regression occurs only with juvenile hemangiomas, and the major involution occurs before age 5. 3. Patients with rapidly growing capillary cavernous hemangiomas should be treated with steroids. 4. Treatment modalities that increase scarring of the surface epithelium are contraindicated. 5. Intradermal and cirsoid angiomas do not spontaneously regress. 6. Congenital A-V fistulas require aggressive surgery. 7. The ultimate deformity resulting from angiomas may be greatly reduced by plastic surgical techniques. 8. Radiation therapy is never indicated for the treatment of hemangiomas. 9. The psychologic impact of the congenital deformity on patients is generally more detrimental than comparable postsurgical or traumatic deformities.

Adult↗
PubMed1989

Malignant angioendotheliomatosis: an angiotropic lymphoma.

Malignant angioendotheliomatosis is a rare, systemic, usually fatal disease characterized by massive proliferation of large, neoplastic, mononuclear cells within the lumen of small blood vessels. Recent studies suggested that the tumor cells are of lymphoid origin. We studied two cases of malignant angioendotheliomatosis by Southern blot hybridization analysis that showed rearrangements of the immunoglobulin heavy chain and kappa light chain (case 2), indicating the presence of a monoclonal B cell lymphoma. Our results provide further evidence that malignant angioendotheliomatosis is an angiotropic lymphoma.

Aged↗
PubMed2000

Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases.

Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibroushistiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.

Adult↗
PubMed2006

Platelet-derived growth factor receptor-beta in Gorham's disease.

BACKGROUND: A 17-year-old male presented with pain in his lower-left chest. He had no significant medical history and was previously in good health. He had a fractured ninth left anterior rib and the tenth, eleventh and twelfth ribs were absent, which was thought to be a congenital anomaly. Several months later, he presented again with back pain, an enlarging mass in the lower-left chest wall, erosion of the lateral pedicles of the lower thoracic vertebrae and pleural effusion. INVESTIGATIONS: Physical examination, chest X-ray, MRI of the spine, incisional biopsy, serial CT imaging of the hemithorax, immunohistochemistry, flow cytometry, and enzyme-linked immunosorbent assays. DIAGNOSIS: Gorham's lymphangiomatosis with expression of platelet-derived growth factor receptor-beta and elevated circulating platelet-derived growth factor-BB. MANAGEMENT: Spine stabilization, thalidomide, celecoxib, interferon-alpha2b, pamidronate, zoledronate, thoracotomy, pleurectomy, talc pleurodesis, and imatinib mesylate.

Adolescent↗
PubMed1999

Chronic lymphoedema and angiosarcoma.

Angiosarcoma has frequently been described arising within chronic lymphoedema of the upper limb following mastectomy and radiotherapy for carcinoma of the breast. We report a case of angiosarcoma arising in a lymphoedematous leg that had been subjected to radiotherapy 20 years previously for Hodgkin's disease. The diagnosis was expedited once the patient noticed the development of bleeding nodules. Prognosis of angiosarcoma is poor with treatment options being wide-excision surgery, palliative radiotherapy or chemotherapy. Unusual bruised areas or bleeding nodules developing within chronic lymphoedematous limbs should be biopsied to exclude the diagnosis.

Aged↗
PubMed1975

[The diagnosis of carcinoma of the breast in relation to growth rate (author's transl)].

The doubling time (DT) was estimated quantitatively for 16 carcinomas of the breast according to the method described by Collins and co-workers in 1956. This is based on the concept of constant and exponential growth. Observation interval for these mammographically confirmed tumors was between 83 and 1,034 days. The DT was calculated to vary from 45 to 260 days; in order to reach a diameter of 1 cm. after 30 divisions would require a period of 3.7 to 21.4 years. Mammography frequently demonstrates small, clinically occult, tumours. Axillary lymph node metastases are relatively rare from small tumours; growth rate of 70% of breast carcinomas in such that an annual clinical and radiological check-up will prove to be the best means of reducing mortality from carcinoma of the breast. The risk inherent in the radiation resulting from annual mammography is acceptable in women over 35 years. This leaves the problem of rapidly growing carcinomas which would escape early diagnosis by early examinations. Half-yearly examinations of women in high risk groups (1. Previous mastectomy for carcinoma, 2. Biopsy-proven mastopathy with atypical proliferation) comprising about 30% of carcinomas with a short doubling time would appear to be reasonable.

Adenocarcinoma↗
PubMed1975

[Lymphography in childhood (author's transl)].

Fifty-one lymphograms were performed in children; the indications, technique and results are discussed. Indications are the search for retroperitoneal involvement in lymphogranulomatosis, lympho-sarcoma and reticulum cell sarcoma; in the search for metastases from malignant tumours, particularly abdominal neuroblastoma, soft tissue sarcomas of the abdomen and lower extremities, testicular tumours and malignant melanomas and finally, for primary lymph-oedema and lymphangiomas. Technique is the same as for adults, but requires particular manual dexterity. Children under six years require general anaesthesia. Amongst 28 children with malignant lymphomas, pathological changes in the retroperitoneal lymph nodes were found in seven. In six, this resulted in a change of the staging. Five out of 16 lymphograms in children with malignant tumours showed evidence of lymph node metastases. All six lymphangiograms in children with lymphoedema and lymphangiomas were abnormal.

Abdominal Neoplasms↗
PubMed1996

[Angiosarcoma of the breast after conservatively operated breast carcinoma--a sequela of adjuvant radiotherapy?].

Angiosarcoma represents only 2.4% of soft tissue malignancies. Primary angiosarcoma of the breast is also a rare neoplasm. The incidence of this disease has been reported to be 0.04% of all breast malignancies. The aetiology is unknown. Angiosarcoma associated with chronic lymphoedematous arm of a postmastectomy woman was described by Stewart and Treves. In this paper we describe a 62-year-old female who underwent wide excision, axillary node dissection and adjuvant radiotherapy for primary breast cancer. After 5 years she noticed a purple discolouration of the treated left breast. Biopsy of these areas confirmed an angiosarcoma. No haematogenous dissemination was found. She underwent simple mastectomy without systemic therapy. A chest X-ray 3 months later revealed diffuse bilateral lung metastases. The incidence of breast conserving therapies is growing fast. Until now we have no evidence on the role of irradiation as an aetiological reason for angiosarcoma after breast-conserving surgery and axillary node dissection. Therefore, we have to pay increasing attention to these patients. A patient with history of radiotherapy after breast conserving therapy and skin discolouration must undergo a biopsy of these areas. The only therapy of angiosarcoma is an early mastectomy. The success of chemotherapy is uncertain. Possibly adjuvant chemotherapy with actinomycin D for the group with poorly differentiated tumours appears beneficial. Prospective studies might help to resolve this question.

Breast↗
PubMed1981

Immunohistochemical identification of factor VIII-related antigen in endothelial cells of cutaneous lesions of alleged vascular nature.

Immunohistochemical staining for factor VIII-related antigen (FVIII-RAG) with the peroxidase-antiperoxidase technic was used as a marker for endothelial cells in a variety of nevoid, reactive, and malignant vascular cutaneous proliferations. The endothelial cells of small normal cutaneous vessels gave the strongest reaction. The cells of hemangioma and angiokeratoma generally were stained, but with less consistency. In lymphangioma, pyogenic granuloma, and pigmented purpuric dermatosis, positivity was either patchy or of lesser intensity. Numerous strongly positive endothelial cells lining well-formed blood vessels were present in lesions of Kaposi's sarcoma, but the proliferating spindle cells forming "vascular slits" were uniformly negative. The cells of angiosarcoma were essentially negative, except for isolated elements in the better-differentiated areas. Plasma was also strongly positive; this may aid in distinguishing vascular from lymphatic channels. Anti-FVIII-RAG immunoperoxidase staining is a helpful aid in evaluating cutaneous vascular proliferations.

Angiokeratoma↗
PubMed2001

Two cases of Kaposi's sarcoma mimicking Stewart-Treves syndrome found to be human herpesvirus-8 positive.

Although angiosarcoma is the most frequent tumor arising in the clinical setting of chronic lymphedema, as in Stewart-Treves syndrome, Kaposi's sarcoma has also been reported in this setting, although rarely. We describe two women who developed Kaposi's sarcoma in the lymphedematous arm many years after surgery for breast cancer. Case 1 is a 92-year-old and Case 2 is an 81-year-old; they underwent left total mastectomy and axillary node dissection for infiltrating breast carcinoma in 1981 and 1982 respectively. At that time, neither patient received further treatment. Except for persistent lymphedema, both women did well until over fourteen years later when each noted the development of several purple asymptomatic plaques on the edematous arm. In both, the clinical diagnosis at the time of biopsy was angiosarcoma. However, histologic findings in both cases were typical for Kaposi's sarcoma. In addition, a nested polymerase chain reaction (PCR) for the detection of a 233bp segment of KSHV/HHV8 was performed on DNA extracted from the paraffin-embedded specimens and both cases were positive for this sequence. Histologic sections of both cases were also tested for KSHV by in situ hybridization and demonstrated a positive signal in the lesional cells in each case.

Aged↗
PubMed1990

Primary malignant lymphoma arising in postmastectomy lymphedema. Another facet of the Stewart-Treves syndrome.

In rare cases, primary malignant lymphomas may arise in the soft tissues. Only one previous case has arisen in the context of chronic lymphedema. Because of the clinical appearance of such lesions, which resemble violaceous nodular or plaquelike tumors, they may be confused clinically with lymphedema-associated angiosarcomas occurring after radical mastectomy (Stewart-Treves syndrome). Furthermore, the histologic appearance of some lymphomas and angiosarcomas may also be similar. We studied two women with primary postmastectomy lymphedema-related malignant lymphoma in the soft tissues of the upper arm. These tumors arose 11 and 30 years, respectively, after radical removal of ductal mammary carcinomas. Histologically, one neoplasm mimicked metastatic carcinoma or epithelioid angiosarcoma; whereas the other was initially confused with a variety of pathologic entities, including vasculitis, epithelioid hemangioma, and malignant fibrous histiocytoma. The lymphoid nature of both lesions was confirmed by immunoreactivity for leukocyte common antigen in addition to the B-lymphocyte marker, L26. Conversely, vascular and epithelial determinants were absent. One patient's disease pursued an indolent course; she died of unknown causes but with no evidence of lymphoma at last follow-up. The second patient is currently in remission on chemotherapy. Awareness of the existence of lymphedema-related malignant lymphoma and familiarity with methods used for its distinction from epithelioid vascular sarcomas should prevent unnecessary surgery.

Aged↗