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PubMed1983

[Non-epitherial malignant neoplasms of the breast--a clinicopathological study of 4 cases].

Between 1972 and 1981, 4 women with breast sarcoma underwent treatment at our department; they accounted for 1.1% (4 of 355) of all female breast malignancies. There was one case of malignant lymphoma and liposarcoma each, and 2 cases of malignant cystosarcoma phyllodes. The patients averaged 31.0 years of age. Of the two cases of cystosarcoma phyllodes, one had coexisting lobular carcinoma in situ arising in a fibroadenoma of the contralateral breast, the other had a coexisting lung cancer. Mastectomy was performed in 3 cases, irradiation in one (malignant lymphoma). All patients received concurrent chemotherapy. The regional lymph nodes were not involved, and 2 patients are alive and well without recurrence.

Adolescent↗
PubMed1988

Rhabdomyosarcoma in patients older than 40 years of age.

The original diagnosis of rhabdomyosarcoma (RM) was critically evaluated by histology, immunohistochemistry, and electron microscopy in a retrospective series of 25 patients older than 40 years of age. Only two of the 25 patients (8%) were verified to have RM by strict criteria. By light microscopy, the true RM had a spindle or round cell appearance and were subtyped as embryonal RM, although some pleomorphism was present. Sarcomeric differentiation was revealed by electron microscopy, and desmin and muscle actin by immunohistochemistry. Both of these tumors arose in the urogenital organs, one in the urinary bladder and the other in the prostatic region. Both patients died within 3 months of the diagnosis. The other tumors not verified as RM were pleomorphic or spindle cell sarcomas (n = 17), ten of which were considered to represent malignant fibrous histiocytoma, or had desmin and/or muscle actin, and were verified as leiomyosarcomas by electron microscopy (n = 2). There were single cases of undifferentiated carcinoma, probable neuroendocrine carcinoma, extraskeletal osteosarcoma, and pleomorphic liposarcoma. The average survival for the non-RM tumor patients was 32 months. The results show that true RM do occur in the elderly, but they are very rare. Such tumors may have clinicopathologic properties similar to embryonal RM in children both in regard to the site of origin and to the histologic picture. True RM seems to carry a worse prognosis than other pleomorphic sarcomas, and this emphasizes the need for accurate diagnosis.

Adult↗
PubMed1994

Adult paratesticular sarcomas: a report of eight cases.

Eight adult paratesticular sarcomas seen at a Regional Cancer Centre over a 7-year period are described. There were three cases of rhabdomyosarcoma (RMS), three cases of leiomyosarcoma (LMS), and two cases of liposarcoma (LS). The RMS occurred in a younger age group (3rd decade) than the LMS and LS (6th and 7th decades). Most of our patients presented with advanced disease after orchiectomy at other hospitals, three with recurrent or residual disease and four with metastasis. The single patient with RMS who received intensive adjuvant therapy is free of disease at 84 months in spite of the advanced stage at presentation. All three patients with LMS had an unfavourable clinical course. Both the patients with LS had well differentiated (WD) tumours and presented with recurrences, one over several years, following initial local excisions. The necessity for early adequate surgical and adjuvant therapy and the need for a uniform treatment policy are discussed.

Adult↗
PubMed2006

[Soft tissue sarcoma of the extremities: current state of the art of adjuvant therapy].

Today, most sarcoma patients can be spared an amputation through the use of adjuvant radiotherapy. Treatment by an experienced multidisciplinary team offers the best chance of achieving permanent tumor control. Histopathologically-free resection margins are of the greatest importance. The indication for radiotherapy is determined by the recurrence risk profile of the individual patient. In addition to the well-proven postoperative irradiation, neoadjuvant radiotherapy is also successful. In the event of an unfavorably sited tumor, intra-operative irradiation can be applied in combination with either form. Patients with large G3 tumors can be given adjuvant chemotherapy to reduce the risk of distant metastases. On account of its appreciable toxicity, however, it should be reserved for patients younger than 65 in a good state of health.

Antibiotics, Antineoplastic↗
PubMed2005

Preoperative chemoradiotherapy (modified Eilber protocol) provides maximum local control and minimal morbidity in patients with soft tissue sarcoma.

BACKGROUND: Local recurrence rates of 15% to 30% after treatment of soft tissue sarcoma (STS) are still common but unacceptable. Our hypothesis was that a refined neoadjuvant chemotherapy and radiation protocol (modified Eilber protocol) improves local control rates while minimizing major morbidity. METHODS: Consecutive patients with STS deep to the fascia of the extremity or trunk during 1984 to 1996 were treated with 3 days of doxorubicin (30 mg/day) and sequential radiation (300 cGy/day for 10 days). Wide excision with limb preservation was performed 4 to 8 weeks after radiation completion. Treatment complications, margins, local recurrence, and survival were prospectively documented. RESULTS: Of 75 patients, 66% had tumors >5 cm, and 71% were grade 2/3. In eight patients, negative margins were not achieved, and four of these had amputation (95% limb salvage). Three of the remaining four had local recurrence with a 5- and 7-year actuarial local control rate of 50% and 25%, respectively. In contrast, of the 67 patients with negative margins, a local control rate of 97% at 5 years and 94% at 7 years and an overall survival of 63% were achieved. Although margin (P = .001) and stage (P = .035) were correlated, these were not significant on multivariate Cox regression analysis. Risk factors for death included tumor stage (hazard ratio, 1.54; P = .001) and tumor grade (hazard ratio, 1.4; P = .02). Three patients (4%) required reoperation for tissue loss, and eight patients (10.6%) developed minor wound complications. CONCLUSIONS: This modified Eilber protocol seems to maximize local control and minimize major wound complications for extremity/truncal STS.

Adolescent↗
PubMed1984

[Basement membranes and tumor pathology].

The distribution of four basement membrane components, type IV collagen (C IV), laminin (LM), heparan sulfate proteoglycan (HSP) and fibronection (FN) has been studied by indirect immunofluorescence using specific antibodies, in benign and malignant proliferations of the mammary gland and in soft tissue tumors. In breast carcinomas, specially intraductal cancers, there is a progressive and concomitant loss of these macromolecules around tumoral cells, preceding an overt tumoral invasion. In sarcomas, FN is frequently seen between malignant cells but the regular pericellular labeling observed around normal muscular cells, Schwann cells and adipocytes is absent. Nevertheless, the persistance of some pericellular staining with anti-C IV, anti-LM, anti-HSP and anti-FN antisera, in most differentiated territories of liposarcomas, leiomyosarcomas and neurifibrosarcomas can help to the diagnosis of such lesions.

Basement Membrane↗
PubMed1983

[Primary soft tissue tumors of the retroperitoneal space].

Primary soft tissue tumors of the retroperitoneal space are seldom. In the last 40 years there are only some reports on larger tumor series. But their comparison is merely possible reservedly because in the past the classification of soft tissue tumors was repeatedly changed. Using the diagnostic criteria of the WHO we examined 96 primary tumors of the retroperitoneal soft tissue concerning the distribution of the histological types. 25 cases were classified as benign tumors. 44% of the lesions were benign schwannomas, in the frequency they were followed by leiomyomas (16%), fibromatoses (12%), lipomas (8%) and fibromas (8%). All fibromatoses were localized in the mesenteric radix. 71 tumors were considered to be malignant. The most frequent malignant growths were leiomyosarcomas (24%). Malignant fibrous histiocytomas and liposarcomas constituted 15.5% and 14% of the malignant tumors, resp. Rhabdomyosarcomas were relatively seldom and only of the embryonal type. They exclusively occurred in childhood. Fibrosarcomas were just found in 3% of cases and synovial sarcomas were completely lacking. 17% of all malignant tumors could not be classified with certainty. Whereas the diagnosis of benign soft tissue tumors is not too difficult as a rule the malignant lesions not seldom resist an exact classification. General problems in diagnosis are discussed, differential diagnostic criteria for a clear separation of different tumor entities are presented. Finally the importance of a histological grading of malignant retroperitoneal soft tissue tumors is emphasized.

Adolescent↗
PubMed2001

[Differential diagnosis of bone and soft tissue tumors by MRI].

Musculoskeletal neoplasms are rare, and both the medical history and complaints of the patients are usually uncharacteristic and of limited information. After a clinical evaluation and biplane conventional radiography, the clinician must classify the patient as having a nonprogressive or progressive primary benign, primary malignant, or metastatic bone tumor. In the case of a probably benign, nonprogressive bone tumor, the patient has to be observed continuously or an additional biopsy should be performed. In the case of a probably malignant lesion, the patient should be referred for further staging and treatment to an orthopedic oncologist. Conventional biplane radiography, scintiscan, computed tomography scan, and magnetic resonance imaging (MRI) are indispensable in staging and treatment planning for patients with musculoskeletal tumors. For limb salvage procedures, delineation of the tumor from adjacent tissue structures is crucial. Hence, MRI of the entire anatomic structure involved, together with adjacent joints, is of the utmost importance, both in the coronal and axial planes. The significance of MRI in clinical follow-up depends on keeping the sequences and imaging planes used constant. Differentiating pseudotumors from true neoplasms still poses a challenge. The cellular pattern and matrix characteristics of a lesion cannot definitely be identified as neoplastic even with application of all imaging modalities including MRI. Information on epidemiology, clinical picture, radiology, and histology of the lesion is necessary to draw a firm conclusion. Biopsy is still the first choice in making the diagnosis.

Adult↗
PubMed2003

Hyperfractionated 192Ir brachytherapy for recurrent retroperitoneal sarcoma: a technique for delivery of local tumor boost dose.

BACKGROUND: Radical surgery is the treatment of first choice for retroperitoneal sarcoma. However, locoregional relapse is frequently observed leading to death in the majority of patients. The role of radiotherapy is not well defined in the management of retroperitoneal sarcoma. Yet, there is evidence that adjuvant irradiation does improve local tumor control. MATERIAL AND METHODS: In order to deliver sufficiently high radiation doses to the retroperitoneum, different techniques for application of a local tumor boost dose in addition to external beam treatment have been proposed. We present a technique of hyperfractionated (192)Ir brachytherapy (HFIR) of the tumor bed via intraoperatively implanted plastic catheters. Postoperative CT-based image-guided brachytherapy was performed. In two consecutive patients with recurrent retroperitoneal sarcoma, treatment was delivered twice daily with single doses of 1.5-2.0 Gy in 5-10 mm tissue depth up to a total dose of 18-32.5 Gy. RESULTS: HFIR of the tumor bed was easily accomplished facilitating delivery of high radiation doses to the retroperitoneum. No major late effects of treatment have been observed with a follow-up of 15 and 28 months, respectively. Details of the brachytherapy procedure are presented. CONCLUSION: HFIR via intraoperatively implanted catheters in the retroperitoneum is a technique suitable for application of a local tumor boost dose. Thus, sufficiently high doses of radiation mandatory for long-lasting local tumor control can be delivered in the tumor bed of the retroperitoneum without exceeding normal tissue radiotolerance in this unfavorable disease.

Brachytherapy↗
PubMed1998

Dendritic fibromyxolipoma: clinicopathologic study of a distinctive benign soft tissue lesion that may be mistaken for a sarcoma.

Twelve cases are described of a distinctive benign soft tissue lesion that may be mistaken for a sarcoma. The tumors occurred in 11 men and a woman aged 33 to 81 years (mean, 64 years), and measured from 2 to 11 cm in greatest diameter (mean, 6 cm). They were grossly described as soft, well-circumscribed, yellow-gray, with a mucoid cut surface. All cases were superficially located in the subcutis or muscular fascia of the head and neck region or the chest and back. Histologically, the tumors were characterized by a proliferation of spindle or stellate fibroblastic cells variably admixed with mature adipose tissue embedded in an abundant myxoid and collagenized stroma. The spindle and stellate fibroblastic cells were characterized by slender dendritic prolongations of their cytoplasm, which appeared to extend for short distances along connective tissue planes. Electron microscopy in two cases confirmed the dendritic nature of the fibroblastic cells, which showed elongated cytoplasmic processes lacking external lamina and displaying foci of pinocytotic activity. Immunohistochemical studies in 11 cases showed strong positivity of the spindle cells with vimentin, CD34 and bcl-2, and negative staining for smooth muscle actin, muscle-specific actin (HHF35), desmin, S-100 protein, keratin, and EMA. Because of their prominent myxoid stroma and relatively large size, some of these tumors were initially misinterpreted as low-grade sarcomas. Clinical follow-up in five cases, however, showed that the patients were alive and well without evidence of recurrence between 5 and 13 years (mean follow-up, 8 years) after simple local excision. The present cases appear to represent a distinctive form of benign soft tissue neoplasm that should be distinguished from myxoid liposarcoma and other benign and malignant myxoid tumors of superficial soft tissues.

Adult↗
PubMed1979

Induction of new-bone formation in the host bed by human bone-tumor transplants in athymic nude mice.

UNLABELLED: Specimens of twelve osteosarcomas, five chondrosarcomas, one giant-cell tumor, and five extraskeletal soft-tissue sarcomas were transplanted into male athymic nude (nu/nu) mice. Survival of the transplant was determined by the volume-doubling time and the sex chromatin of the tumor cells obtained from two female patients. By these criteria and the similarity of the histological composition of the original tumor and the transplant, survival occurred in four of twelve osteosarcomas and four of five chondrosarcomas. Without any local infiltration of lymphocytes or plasma cells, or other evidence of cell-mediated immunity, the surviving tumors regressed by the fourth week after the operation. Transformed osteoblasts and osteoprogenitor cells were replaced by fibrous connective tissue or fibrogenic tumor-tissue cells. Osteocytes degenerated and disappeared from the lacunae. The one giant-cell tumor transplant survived, growing very slowly, but by the end of the first week after transplantation whorls of mononucleated cells appeared in sites previously occupied by multinucleated cells. Transplants of leiomyosarcoma, liposarcoma, and synovioma (one tumor of each) degenerated. One of two fibrosarcomas survived transplantation. The most striking reaction of the mouse host bed was to encompass six of twelve osteosarcomas and four of five chondrosarcomas in deposits of normal living cartilage, bone, and bone marrow. The incidence of new bone inducedy by living transplants was only slightly greater than by implants of freeze-dried killed osteosarcoma tissue. Not one of five extraskeletal sarcomas, living or dead, induced bone formation. These observations suggest that an osteoinductive agent is transmitted by some osteosarcomas and chondrosarcomas. This agent initiates differentiation of host mesenchymal cells into normal non-tumorous cartilage and bone, which later colonized by bone marrow. CLINICAL RELEVANCE: Our observations present experimental evidence of the origin of the envelope of normal non-tumorous bone that may surround tumorous bone. In 1926, Phemister recognized the clinical significane of this envelope as a pitfall in the differential diagnosis of malignant bone tumors, chondrosarcoma, myositis ossificans circumscripta, and other neoplasms. He emphasized the importance of examining the entire specimen for the distribution of deposits of tumorous and normal bone. The induction of normal bone formation in the host bed surrounding transplants of osteosarcomas and some chondrosarcomas (but not transplants of fibrosarcoma, liposarcoma, or leiomyosarcoma) is evidence of a specific tumor-cell characteristic. Thus, the bone inductive response is not an unspecific reaction to injury from expansion or of tumor growth but a biological response to tumor-cell products. Transplants of human malignant tumors growing in the thymus-deficient mouse can be treated by combinations of radiation, amputation, and new chemotherapeutic agents...

Adolescent↗