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PubMed2005

Cutaneous mass aspirate from a Golden Retriever: "glandular guile".

A 3-year-old, neutered, male Golden Retriever was presented for evaluation of a 10 X 9 X 5 mm, firm, red, raised, cutaneous mass located over the left cranial thorax and noted incidentally by the owner. On cytologic evaluation of a fine-needle aspirate of the mass, the interpretation was a malignant tumor with predominantly mesenchymal features. Differentials included liposarcoma, atypical amelanotic melanoma, anaplastic sarcoma, and anaplastic carcinoma. Following complete excision of the mass, a diagnosis of sebaceous adenocarcinoma was made based on histologic features, positive immunostaining for pancytokeratin, and negative staining for vimentin, Melan-A, and S-100. There was no evidence of metastasis on physical examination or thoracic radiographs, and the prognosis was good. The unique and previously unreported cytologic features of this small, sebaceous adenocarcinoma were the extreme pleomorphism, including marked anisocytosis, anisokaryosis, and multinuclearity, and the paucity of epithelial features.

Adenocarcinoma, Sebaceous↗
PubMed2003

Solitary bone metastasis from myxoid liposarcoma.

BACKGROUND: Extrapulmonary metastasis is more common in liposarcoma than in other soft tissue sarcomas. However, osseous metastases are rare. CASE REPORT: We report the case of a 61-year-old woman with a solitary histologically proven bone metastasis of the right femur from primary myxoid liposarcoma of the left thigh. In September 2000 resection of the primary tumor was performed. Histology showed a high-grade liposarcoma with round cell differentiation. Postoperative radiotherapy with 60 Gy was performed. In a follow-up MRI examination 11 months after initial diagnosis, a suspicious formation in the marrow space of the right proximal femur was detected. CT-guided biopsy was performed and histology showed a metastasis from liposarcoma. PET examination confirmed the metastasis as solitary. Curettage of the metastasis was performed followed by radiotherapy with 60 Gy. The patient was without evidence of disease in the last follow-up. CONCLUSIONS: No standard treatment exists for the management of solitary extrapulmonary metastases from soft tissue sarcoma. Interdisciplinary cooperation is advised and the therapy concept should be chosen individually.

Biopsy↗
PubMed1992

Differentiation markers in hemangiopericytoma.

Normal pericytes were found to express factor XIIIa (F-XIII) and histocompatibility antigen HLA-DR. These markers were studied in 15 hemangiopericytomas (HPC) and 16 other tumors with an HPC-like pattern. A subpopulation of tumor cells expressing F-XIII and HLA-DR antigens was a constant feature of HPC and supported their pericytic origin. Meningeal HPC did not differ in phenotype from peripheral soft tissue HPC. Most tumors with an HPC-like pattern (including synovial sarcomas, malignant schwannomas, leiomyosarcomas, and liposarcomas) were negative for F-XIII and HLA-DR. Malignant fibrous histiocytomas, however, invariably contained a subpopulation expressing these antigens. Therefore, F-XIII can be considered as a marker of fibrohistiocytic differentiation in HPC. Individual tumor cells in HPC occasionally were positive for factor VIII-related antigen (F-VIII-R-Ag), suggesting that the spectrum of differentiation in HPC may include the endothelial cell line. The differentiation characteristics of HPC support the concept that pericytes are primitive cells that may act as precursors to other mesenchymal cells.

Antigens, Differentiation↗
PubMed1989

[Clinical treatment of 37 cases of soft tissue sarcomas in the past 6 years].

Since 1981, 37 cases of soft tissue sarcomas have been treated in our clinic. There were 20 males and 17 females with an average age of 43.5 years old. Histopathological classifications were liposarcomas 9 cases, MFH 8 cases, synovial sarcoma 5 cases, rhabdomyosarcoma 3 cases etc. Twenty-four cases (65%) were located in lower extremity, 11 cases (30%) in upper extremity, and 2 cases (5%) in trunk. All cases were treated with surgical intervention. Postoperative radiation was undertaken in 4 cases and adjuvant chemotherapy in 12 cases, respectively. There were 28 cases (76%) of excisional surgery; i.e., so-called limb salvage operation, and 9 cases (24%) of amputation or disarticulation. In August 1987, 10 cases had died of disease (DOD) and 27 cases were alive, but 4 cases were alive with disease (AWD). Each of the DOD and AWD cases were in stage III (by Enneking's surgical staging system) at first arrival to our clinic, or multioperated cases or cases with rapid distant metastases soon after operation. In conclusion, it was emphasized that in order to improve the clinical results, the soft tissue sarcomas must be treated earlier and that more effective chemotherapeutical protocols should be developed in the future.

Adolescent↗
PubMed2006

[Primary sarcomas of the breast].

Primary sarcomas of the breast are extremely rare with less than 1% of all malignant tumours of the breast reported in literature. At our Institution 1315 malignant tumours of the breast were diagnosed between 1999-2004; nine of them corresponded to primary sarcomas: angiosarcoma (3), leiomyosarcoma (1), low-grade fibromyxoid sarcoma (1), dematofibrosarcoma protuberans (1), liposarcoma (1), osteosarcoma (1), malignant peripheral nerve sheath tumour (1). Histopathological specimens stained with routine techniques and immunoperoxidase were reviewed; proliferation index and p53 over-expression were also determined. Patients' clinical reports were also reviewed to determine prognosis (favorable and unfavorable). The incidence observed (0.7%) is similar to those already published by others authors. Proliferation index was correlated with type of evolution, being an unfavourable prognosis factor when it was equal or major to 30%. Most of the tumours (67%) showed p53 (mayor or equal to 20% of nuclear staining) over-expression but this did not show a direct relationship with the evolution of each neoplasm.

Adult↗
PubMed2003

Expression of multidrug resistance proteins, P-gp, MRP1 and LRP, in soft tissue sarcomas analysed according to their histological type and grade.

The biological behaviour of different histological types and grades of soft tissue sarcomas (STS) varies. This might result in a differing sensitivity to cytotoxic drugs. Cross-resistance to functionally and structurally distinct natural-product drugs, known as multidrug resistance (MDR), is associated with the overexpression of P-glycoprotein (P-gp), multidrug resistance-associated protein 1 (MRP1) and lung resistance-related protein (LRP). The purpose of this study was to evaluate the expression of P-gp, MRP1 and LRP in STS according to their histological type and grade. In 141 chemotherapy-naive STS patients, the expression of the three MDR proteins was detected by immunohistochemistry. Nine histological types were documented. These were 19% grade 1, 34% grade 2 and 47% grade 3 tumours. Expression of P-gp and LRP was observed more frequently than the expression of MRP1 (P<0.0001). P-gp expression was most pronounced in malignant fibrous histiocytoma (MFH), but was low in leiomyosarcomas. MRP1 was expressed in most malignant peripheral nerve sheath tumours (MPNST). LRP was strongly expressed in MFH and unspecified sarcomas, but was low in liposarcomas. MRP1 and LRP expression was significantly more common in grades 2 and 3 compared with grade 1 tumours. P-gp expression was correlated with MRP1, especially in grade 3 STS. In conclusion, P-gp, MRP1 and LRP are expressed in the majority of STS, but this expression varies according to the histological type. MRP1 and LRP, but not P-gp expression, were found to be correlated to tumour grade. MDR might contribute to the observed differences in clinical behaviour within the heterogeneous group of STS.

ATP Binding Cassette Transporter, Subfamily B↗
PubMed2001

Myxoid liposarcoma of the oral cavity with involvement of the periodontal tissues.

BACKGROUND, AIMS: Liposarcoma is the 2nd most frequent soft tissue sarcoma in adults, but it is extremely rare in the head and neck and, particularly, in the oral cavity. We report on a 25-year-old female who presented with a periodontal mass, extended from the right upper 3rd molar to the right upper 2nd premolar, covered by intact oral mucosa. The clinical differential diagnosis included peripheral giant cell granuloma, salivary gland neoplasms, squamous cell carcinoma of the gingiva, sarcoma and malignant lymphoma. METHODS: To accurately plan subsequent treatment, an excisional biopsy was performed and a myxoid liposarcoma was diagnosed. Consequently, the patient underwent wide excision of the neoplasm with maxillary en-block resection. RESULTS: The post-operative course was uneventful and the patient is alive and well 8 years after the original diagnosis. The authors stress the importance of considering soft tissue sarcomas in the diagnostic approach to patients with unusual periodontal neoplasms and to plan adequate surgical sampling of the lesion (i.e. excisional biopsy). CONCLUSIONS: This appears of pivotal importance as more limited specimens may result in inaccurate pre-operative diagnosis.

Adult↗
PubMed2005

Differential sensitivity of liposarcoma subtypes to chemotherapy.

Liposarcoma is one of the most common soft tissue sarcomas and has a number of different subtypes: well-differentiated; dedifferentiated; myxoid/round cell; and pleomorphic. However, the response of these subgroups to chemotherapy is not well documented. In this study, we have conducted a retrospective analysis of a prospectively maintained database of soft tissue sarcoma patients treated at the Royal Marsden Hospital. Eighty-eight liposarcoma patients who received chemotherapy between August 1989 and June 2004 were identified. The response rates to chemotherapy of the different histological subtypes and overall and progression free survival were investigated. Survival according to histological grade was also assessed. A statistically significant higher response rate to first-line chemotherapy was observed in patients with myxoid liposarcoma compared to de- and well-differentiated tumours, 48% (95%CI; 28-69) and 11% (95%CI; 2-29), P = 0.005. Similarly, those with myxoid liposarcoma had a significantly higher response rate compared to all other liposarcoma patients, 48% (95%CI; 28-69) and 18% (95%CI; 8-31). Patients with lower grade tumours had better overall survival. This retrospective analysis suggests that myxoid liposarcoma is relatively chemosensitive in comparison to a combination of other liposarcomas, and in particular de- and well-differentiated tumours. Further confirmation of these results should be sought by similar analyses of other databases.

Adult↗
PubMedDate not supplied

Myxoid liposarcoma of the ankle.

Liposarcomas are among the most common soft tissue sarcomas, but are rarely found in the foot or ankle. Certain types of liposarcomas, which range from well-localized low-grade entities to highly aggressive neoplastic lesions, may resemble common benign soft tissue lesions. Myxoid liposarcoma is the most common type of liposarcoma and is important to recognize and differentiate from a benign soft tissue lesion because it may be a limb-threatening and/or life-threatening tumor. A thorough literature review and perplexing case study of myxoid liposarcoma of the ankle is presented.

Adult↗
PubMed2003

Buttock soft tissue sarcoma: clinical features, treatment, and prognosis.

BACKGROUND: Primary buttock soft tissue sarcomas in adults are common entities that have been infrequently reported (three clinical series and isolated case reports). We present our experience of buttock sarcomas to better characterize and define the natural history of this condition. METHODS: Buttock tumors occurring in adults (>16 years) between January 1990 and January 2002 were identified from the Royal Marsden Hospital's Sarcoma Unit prospective database. RESULTS: Seventy-three buttock sarcomas were evaluated and treated at the Royal Marsden Hospital during this period. Liposarcoma (n = 19), leiomyosarcoma (n = 13), and synovial sarcoma (n = 9) were the most frequent histological types. There were 8 T1 and 61 T2 tumors, and size was not available in 4 patients. Most tumors (n = 64) were located deep to the deep fascia. There were 15 grade 1, 20 grade 2, and 37 grade 3 tumors, and grade was not available in 1 patient. There were 29 tumors contained within the gluteus maximus. Wide excision was performed in 50 patients. Local recurrence and distant metastasis occurred in 15 and 35 patients with a median time of 18 and 8 months, respectively. The rate of local recurrence at 2 years was 20.9% (SE, 6.8%). The 2-year overall and disease-free survival rates were 64.1% (SE, 6.7%) and 48.5% (SE, 6.4%), respectively. CONCLUSIONS: Buttock sarcomas present special surgical difficulties because of proximity of the sciatic nerve and the ability of tumors at this site to extend into the pelvis and perineum. Size and grade of the tumor were independent predictors for disease-free and overall survival.

Adolescent↗
PubMedDate not supplied

The ultrastructure of liposarcomas with attention to "dedifferentiation".

Liposarcomas are among the most common soft tissue sarcomas. It is recognized that dedifferentiation can occur within a well-differentiated liposarcoma, but there is limited information concerning the ultrastructure of the dedifferentiated cells. A series of 8 cases has been studied by light and electron microscopy and compared with well-differentiated, myxoid, and pleomorphic liposarcomas. No definite evidence of lipoblastic differentiation could be found in the dedifferentiated cases. The tumor cells resembled atypical cells in the well-differentiated liposarcomas, supporting the close relationship between these two types of tumors. However, since no conclusive line of differentiation could be found in the dedifferentiated cases, this study supports the contention that these neoplasms are undifferentiated counterparts of well-differentiated liposarcomas.

Aged↗
PubMed1998

Retroperitoneal liposarcomas. Study of 10 cases.

INTRODUCTION: Retroperitoneal liposarcomas are unusual tumors. Their biological and clinical features different from other retroperitoneal sarcomas. AIM: To analyze our series of retroperitoneal liposarcomas. PATIENTS AND METHODS: Retrospective study of 10 patients with retroperitoneal liposarcoma diagnosed in the last 16 years at our institution. Symptoms, diagnosis, treatment and prognosis were analyzed. RESULTS: Radical resection was done in all cases. Postoperative mortality was 10%. Complementary treatment was considered in each patient. Recurrent disease occurred in 8 patients (14 recurrences), during follow up. In 42% of them recurrences were resected. Actuarial 5-year survival rate was 44%. CONCLUSION: Poor prognosis of retroperitoneal liposarcoma is due to its high recurrence rate.

Adult↗
PubMed2000

Retroperitoneal sarcomas.

The approach to the management of retroperitoneal tumors begins with a complete history and physical examination. Imaging of the abdomen and pelvis by computed tomography (CT) provides both an imaging modality and a method by which to obtain tissue for diagnosis. Because a histologic diagnosis is essential in treatment planning, adequate tissue can usually be obtained by a CT-guided core biopsy. If the diagnosis is sarcoma, additional tests necessary for staging include plain chest radiography and evaluation of the liver by either CT scan or magnetic resonance imaging (MRI). The treatment options for primary retroperitoneal sarcomas include chemotherapy, radiation therapy, surgery, or a combination of these modalities; therefore, a multidisciplinary group best manages treatment planning. Primary radiation therapy for cure is seldom effective for retroperitoneal sarcomas but can provide palliation in select cases. Systemic chemotherapy for chemosensitive lesions, such as poorly differentiated liposarcoma, malignant fibrous histiocytoma (MFH), synovial cell sarcoma, and primitive neuroectodermal tumors (PNET), can be useful when used in a neoadjuvant manner. Consequently, surgical resection continues to be the mainstay of treatment for retroperitoneal sarcomas and requires en bloc resection of the primary tumor. Frequently this includes adjacent organs such as colon, small bowel, kidney, adrenal, and pancreas. Postoperative adjuvant therapy with chemotherapy or radiation has not been proven to be of any additional benefit. Overall treatment results are predominantly influenced by tumor stage, grade, size, and margins of surgical resection. Follow-up CT scans at 6-month intervals and surgical resection of recurrences can be valuable.

Antineoplastic Agents↗
PubMed2005

Divergent differentiation in malignant soft tissue neoplasms: the paradigm of liposarcoma and malignant peripheral nerve sheath tumor.

In tumors clonality does not always translate into morphologic uniformity. While most sarcomas exhibit only one line of histologic differentiation, a minority may display a strikingly diverse phenotype in addition to the main lineage. This phenomenon not only presents a diagnostic problem but also raises questions about the commitment of tumor cells toward a specific phenotype. Among sarcomas, malignant peripheral nerve sheath tumor (MPNST) and dedifferentiated liposarcoma are two entities in which divergent differentiation is a relatively frequent event. Diagnostically it is crucial to recognize the "primary" sarcoma in such tumors correctly and distinguish it from the "secondary" divergent elements. The presence of the latter could be the first morphologic clue to a specific sarcoma type. Even though it may be difficult to explain the pathogenesis of divergent differentiation, divergence still illustrates that the phenotype of a tumor cell is not set in stone but can be modulated or switched by a number of factors.

Cell Transformation, Neoplastic↗
PubMed1997

Myxoid liposarcoma of the retropatellar fat pad.

Soft-tissue sarcomas are rare lesions, and they are occasionally seen by the orthopedic surgeon. We describe the clinical history of a patient with a myxoid liposarcoma that arose from the retropatellar fat pad of the knee. The differential diagnosis of knee masses and the magnetic resonance image characteristics of myxoid liposarcoma are discussed. There are no previous reports of liposarcomas originating from the retropatellar fat pad. Given the consequences of inappropriate biopsy and surgery of suspected benign lesions, it is fundamentally important to adhere to the principles of musculoskeletal oncology in the evaluation of any soft-tissue mass that could be malignant.

Adipose Tissue↗
PubMed1992

Tissue characterization and assessment of preoperative chemotherapeutic response in musculoskeletal tumors by in vivo 31P magnetic resonance spectroscopy.

This study investigates the potential of in vivo 31P magnetic resonance spectroscopy (MRS) to characterize musculoskeletal tumors and to determine preoperative levels of histological necrosis, which is an important clinical indicator of patient response. Pretherapy MRS was performed on 28 patients with large musculoskeletal tumors: 13 with osteosarcoma, 3 with chondrosarcoma, 5 with malignant fibrous histiocytoma, 1 with desmoid tumor, 1 with Ewing's, 2 with hemangioendothelioma, 1 with myxoid liposarcoma, 1 with synovial cell sarcoma, and 1 with rhabdomyosarcoma. Fifteen patients had follow-up MRS examinations after commencement of chemotherapy (mean of five/patient), eight of whom have now had surgery. Elevated levels of PMEs (P < 0.01), P(i) (P < 0.01), and PDEs (P < 0.02) as well as elevated tumor pH (P < 0.05) were observed in all patients. The synovial cell sarcoma was characterized by high levels of PMEs (> 20%) and low pH (pH 6.76). This contrasted with the spectra obtained from the malignant fibrous histiocytomas which had high levels of PDEs (17 +/- 5%). Reductions in PDE levels postchemotherapy were associated with a high degree of necrosis (> 90%) at surgery, while an increase in PDE levels was associated with a low level of histological necrosis. Likewise, reductions in the ratios PDE/NTP and PDE/PCr and an increase in P(i)/PDE were also associated with a high level of necrosis.

Adult↗
PubMed2003

FDG-PET for preoperative differential diagnosis between benign and malignant soft tissue masses.

OBJECTIVE: To evaluate the standardized uptake value (SUV) of [(18)F]2-deoxy-2-fluoro- d-glucose at positron emission tomography (FDG-PET) for preoperative differential diagnosis between benign and malignant soft tissue masses. DESIGN: One hundred and fourteen soft tissue masses (80 benign, 34 malignant) were examined by FDG-PET prior to tissue diagnosis. The SUVs were calculated and compared between benign and malignant lesions and among different histologic subgroups which included three or more cases. RESULTS: There was a statistically significant difference in SUV between benign (1.80+/-1.42 [SD]) and malignant (4.20+/-3.16) soft tissue masses in total (P<0.0001). However, a considerable overlap in SUV was observed between many benign and malignant lesions. Liposarcomas (2.16+/-1.72) and synovial sarcomas (1.60+/-0.43) did not show significantly higher SUV than any benign lesions. Metastases (4.23+/-2.35) showed no statistically significant difference in SUV as compared with schwannomas (1.75+/-0.84), desmoids (2.77+/-1.32), sarcoidosis (3.62+/-1.53), or giant cell tumors of tendon sheath (GCT of TS; 5.06+/-1.63). Even malignant fibrous histiocytomas (5.37+/-1.40) could not be differentiated from sarcoidosis or GCT of TS, based on the SUV. CONCLUSIONS: A large accumulation of FDG can be observed in both benign and malignant histiocytic, fibroblastic, or neurogenic lesions. SUV at conventional FDG-PET is limited to differentiating benign from malignant soft tissue masses, when all kinds of histologic subtypes are included.

Diagnosis, Differential↗