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PubMed1997

Diet and risk of lymphoid neoplasms and soft tissue sarcomas.

The relationship between frequency of intake of selected indicator foods, lymphoid neoplasms, and soft tissue sarcomas was investigated in an updated case-control study conducted in Northern Italy between 1983 and 1992 on 158 incident, histologically confirmed cases of Hodgkin's disease (HD), 429 cases of non-Hodgkin's lymphoma (NHL), 141 cases of multiple myelomas, 101 cases of soft tissue sarcomas, and 1,157 controls admitted to hospital for acute, nonneoplastic diseases unrelated to long-term modifications of diet. Compared with the lowest tertile, the odds ratio (OR) for the highest tertile of milk intake was 1.8 for NHL and 1.9 for sarcomas. Liver intake was an indicator of the risk of HD (OR = 1.8), NHL (OR = 1.6), and myelomas (OR = 2.0), ham an indicator of HD (OR = 1.7), and butter an indicator of myelomas (OR = 2.8). A high consumption of green vegetables was inversely related to myelomas (OR = 0.4), and frequent use of whole-grain foods was inversely related to NHL (OR = 0.4) and soft tissue sarcomas (OR = 0.2). No material association with meat was observed for any of the neoplasms considered. Likewise, coffee and alcohol intakes were not associated with lymphoid neoplasms and soft tissue sarcomas. The OR for the highest tertile of intake of beta-carotene ranged between 0.5 and 0.7, whereas the OR for retinol ranged between 1.5 and 2.3. Although available data do not point to any specific inference, this study suggests that certain aspects of diet are a correlate or an indicator of the risk of lymphoid neoplasms and soft tissue sarcomas.

Adolescent↗
PubMed2004

Cutaneous and subcutaneous fibrohistiocytic tumors of intermediate malignancy: an update.

The fibrohistiocytic tumors of intermediate malignancy are uncommon mesenchymal tumors, which typically occur in the skin and subcutis and which may pose significant problems for the dermatopathologist. This article reviews the clinical, histopathologic, and genetic features of dermatofibrosarcoma protuberans, giant cell fibroblastoma, angiomatoid (malignant) fibrous histiocytoma, plexiform fibrous histiocytoma, and soft tissue giant cell tumor (of low malignant potential). The differential diagnosis of these tumors with a variety of benign and fully malignant cutaneous soft tissue neoplasms is discussed.

DNA, Neoplasm↗
PubMed1991

Soft tissue myofibroblastomas.

Five well-circumscribed solitary soft tissue tumors composed of myofibroblasts are described and termed myofibroblastomas. By light microscopy these lesions are characterized by short, intersecting, or crisscrossing fascicles of spindle cells, sometimes associated with foci of necrosis and/or mitotic activity with less than three mitoses per 10 high power fields. Myofibroblastomas show well-defined myofibroblastic differentiation ultrastructurally with peripheral myofilaments and vimentin, actin, and desmin immunocytochemistry positivity. The five tumors described occurred in patients of various age groups, including one congenital, and in a variety of soft tissue locations. It is important to recognize this benign soft tissue neoplasm to avoid confusion with other soft tissue tumors and to separate this lesion from other myofibromatosis. This study elucidates the spectrum of light microscopic, ultrastructural, and immunocytochemistry findings of soft tissue myofibroblastomas and establishes this soft tissue tumor as a specific clinico-pathologic entity.

Actins↗
PubMed1986

A familial syndrome of cardiac myxomas, myxoid neurofibromata, cutaneous pigmented lesions, and endocrine abnormalities.

A family syndrome of cardiac myxomas, myxoid neurofibromata, cutaneous pigmented lesions, and endocrine abnormalities is described. Three cases from a family of four are presented. All had cutaneous pigmented lesions. The mother had left and right atrial myxomata, her daughter a left ventricular myxoma, subcutaneous myxoid neurofibromata, and mammary fibroadenosis, and her son has no evidence of cardiac myxoma. Elevated circulating levels of insulin-like growth factors in the one family member tested raises the possibility of abnormal stimulation of somatic growth and may be linked to development of soft tissue neoplasms in these patients.

Adolescent↗
PubMedDate not supplied

Sonography of soft tissue masses of the neck.

In many clinical conditions, high-resolution sonography and color (power) Doppler sonography can be used as the first-line modality for evaluating cervical soft tissue masses. Cervical cysts, lipomas, paragangliomas, neurogenic tumors, hemangiomas, and lymphangiomas often exhibit characteristic sonographic appearances. Sonography can be used for lymph node assessment, and most salivary gland diseases can be diagnosed sonographically. Sonography can be used to guide needle biopsy of soft tissue neoplasms and lymph nodes. In addition, the relationship between a cervical mass and the great vessels can be evaluated.

Branchioma↗
PubMed2006

Deregulation of HMGA2 in an aggressive angiomyxoma with t(11;12)(q23;q15).

Aggressive angiomyxoma is a soft-tissue neoplasm with a predilection for the pelvic and perineal regions and a tendency to recur locally. Cytogenetic data on this tumor type are limited to five cases, three of which showed rearrangement of chromosomal bands 12q13-15. Molecular investigation of two of the tumors identified the HMGA2 gene as the target of the 12q rearrangements. However, the two previously analyzed tumors were different at the molecular level: in one, the rearrangement of 12q13-15 resulted in a fusion product, whereas, in the second case, the breakpoint was telomeric (3') to the HMGA2, leaving the gene intact although expressed in its entire length. To shed more light on the pathobiology of aggressive angiomyxoma and to investigate the molecular mechanisms behind the involvement of the HMGA2 gene in this tumor type (fusion transcript vs deregulated expression), we investigated, cytogenetically and with molecular techniques, one such tumor which presented a t(11;12)(q23;q15) as the sole karyotypic aberration. FISH analyses demonstrated no structural alteration of HMGA2 at the cytogenetic level; however, expression of the full-length gene was detected molecularly.

Adult↗
PubMed1985

Soft-tissue chondromas.

Soft-tissue chondromas are benign, relatively rare lesions. The clinicopathologic and radiologic features in six cases are presented and the literature is reviewed. Five of six cases had calcifications. Peripheral curvilinear calcification was seen in two cases, multiple ringlike areas characteristic of cartilage in one, and discrete ossification in another. Histopathologically, they were diverse, ranging from an immature pattern with a preponderance of chondroblasts to a more mature form with chondrocytes. Although these lesions resemble a wide variety of other soft-tissue lesions, they can usually be differentiated by their small size, characteristic location in the extremities, the nature of their calcifications, and the histopathologic features. The importance of this lesion is that it is benign. It must be distinguished from more aggressive soft-tissue neoplasms such as soft-tissue chondrosarcoma so as to spare the patient unnecessary radical therapy.

Adolescent↗
PubMed1995

Low grade fibromyxoid sarcoma: clinicopathological analysis of eleven new cases in support of a distinct entity.

Low grade fibromyxoid sarcoma is a recently recognized, uncommon soft tissue neoplasm with a tendency to develop in deep soft tissue of young adults. Diagnostic criteria have not been well defined and this tumour has not been widely accepted as a distinct entity. Eleven new cases are reported here for which reproducible histological features are described and in which the immunohistochemical profile of the tumour cells is documented for the first time. Ten of the eleven patients were male and the majority were young or middle-aged adults (median age 45 years). All except one of the tumours were situated in deep soft tissue. Lower limb (four cases) and chest wall (three cases) were the commonest primary sites; one case each arose in the groin, buttock, axilla and retroperitoneum. Follow-up (median duration 6 years) was available in nine patients. Six developed local recurrence and in five cases recurrences were multiple. Pulmonary metastasis occurred in one patient. All tumours were characterized by the presence of bland spindle cells, showing a mainly whorled or focally linear arrangement, set in alternating areas with a fibrous or myxoid stroma. Tumour cells were small, spindle to stellate, with poorly defined, palely eosinophilic cytoplasm and hyperchromatic ovoid nuclei. Most tumour cells showed strong staining with antibodies to vimentin, while occasional cells stained positively for actin, desmin and cytokeratin, in keeping with focal myofibroblastic differentiation. Ultrastructural examination in one case revealed features of fibroblasts. Careful consideration of the morphological and immunohistochemical features of these tumours permits a positive diagnosis of low grade fibromyxoid sarcoma and allows its distinction from a number of other benign and malignant soft tissue neoplasms.

Adolescent↗
PubMedDate not supplied

Palliation of soft tissue cancer pain with radiofrequency ablation.

The purpose of this study was to analyze the feasibility, safety, and efficacy of radiofrequency ablation (RFA) to treat pain from soft tissue neoplasms. RFA was performed on 15 painful soft tissue tumors in 14 patients. Tumors varied in histology and location and ranged in size from 2 to 20 cm. Patient pain was assessed using the Brief Pain Inventory (BPI) at baseline and 1 day, 1 week, 1 month, and 3 months post RFA. All patients had unresectable tumors or were poor operative candidates whose pain was poorly controlled by conventional treatment methods. BPI scores were divided into two categories: pain severity and interference of pain. Although not all scores were statistically significant, all mean scores trended down with increased time post ablation. Based on these outcomes, RFA appears to be a low-risk and well-tolerated procedure for pain palliation in patients with unresectable, painful soft tissue neoplasms. RFA is effective for short-term local pain control and may provide another option for failed chemotherapy or radiation therapy in patients with cancer. However, pain may transiently worsen, and relief is often temporary.

Catheter Ablation↗
PubMed1984

Detection of malignant soft tissue tumors in bone imaging.

A total of 2530 consecutive bone scans were examined to evaluate the number and the type of soft tissue neoplasms detected with bone-seeking phosphonates. Sixty-eight primary or secondary soft tissue neoplasms of 63 patients accumulated 99mTc -methylene diphosphonate and 2 accumulated 99mTc -diphosphonate, and one metastasis was seen as a nonactive 'cold' focus in the urinary bladder. The localization of tumors was: 19 in the lung, 15 in the liver, 11 in the kidney, 10 in the peritoneal cavity or ascites, 5 in the large bowel, 4 in the vascular or lymphatic system, 3 in the connective tissue or muscles, 2 in the ovary, 1 in the urinary bladder, and 1 in the brain. Our results differ in many respects from those reported in the literature. The causes of the differences are discussed.

Abdominal Neoplasms↗
PubMed2000

Soft tissue aspiration cytopathology.

BACKGROUND: Fine-needle aspiration (FNA) biopsy as a diagnostic modality for the pathologic evaluation of soft tissue neoplasms and non-neoplastic soft tissue mass lesions is uncommon and controversial. This procedure contrasts with more traditional diagnostic methods such as marginal excision, incisional (open) biopsy, or even core biopsy to procure tissue from somatic sites. METHODS: The authors reviewed the results of cytopathologic diagnoses obtained by fine-needle aspiration biopsy over a consecutive 11-month period in patients that presented primarily with a palpable soft tissue mass. A few patients with deep non-palpable soft tissue masses also were evaluated by radiologically guided FNA. Cytopathologic diagnoses were verified by different means including tissue examination either by concurrent cell block or subsequent surgical biopsy, flow cytometry, clinical outcome, or repetition of the FNA procedure. Patients were followed for a minimum of one year to evaluate the mass clinically, to determine whether any further therapy was administered, and to assess disease status. RESULTS: Eighty-two aspirates were performed without complications from 77 patients ranging from 12-88 years of age (mean = 50 yrs.) with men outnumbering women 1.5:1. Soft tissue masses were most common in the extremities (41 cases), followed by the trunk (34 cases), retroperitoneum (5 cases), and head and neck (2 cases). Fine-needle aspirates were diagnosed as malignant in 42 (51%), benign in 32 (39%), nondiagnostic in 6 (7%), and atypical in 2 (2%) cases. Malignant aspirates were comprised of 24 sarcomas (57%), 9 carcinomas (21%), 6 malignant lymphomas (14%), and 3 melanomas (7%). Twenty-two aspirates (52%) had an initial diagnosis of malignancy, whereas 18 (43%) represented metastatic and 2 (5%) recurrent neoplasms. Confirmation of the cytopathologic diagnosis was by concurrent or subsequent tissue examination in 57%, flow cytometry in 5%, clinical outcome in 34%, and repeat aspiration in 4%. One false negative and no false positive diagnoses were issued for a sensitivity and specificity of 100% and 97% respectively in distinguishing benign and malignant lesions by FNA. Of the malignant aspirates, 83% could be subtyped whereas 72% of benign aspirates were correctly subtyped. For primary soft tissue sarcomas, 12 of 19 (63%) were accurately subtyped. In 48% of cases a concurrent cell block was obtained and found diagnostically useful in 54% of them. CONCLUSIONS: Aspiration cytopathology of soft tissue mass lesions using FNA biopsy can be an accurate and minimally invasive method for the initial pathologic diagnosis of primary benign and malignant soft tissue masses, for the pathologic confirmation of metastatic tumors to soft tissue, and for the documentation of locally recurrent soft tissue neoplasms. FNA cytopathology is capable of specifically subtyping a large percentage of primary and metastatic soft tissue tumors if cellular material either in the form of a cell block or flow cytometry is obtained in addition to cell smears.

Adolescent↗
PubMed1996

Extra-acral calcifying aponeurotic fibroma: a distinctive case with 23-year follow-up.

Calcifying aponeurotic fibroma (CAF) is an unusual but well-characterized soft tissue neoplasm that typically involves the digits of children and frequently recurs locally. This report describes a case from the subcutis of the lumbosacral region. A 26-year-old man initially presented at age 3 and developed three recurrences over a 23-year period each at approximately 8-year intervals (ages 10, 18, and 26). The microscopic findings in all recurrences showed a lobulated, poorly circumscribed proliferation of dense fibrous tissue containing epithelioid-like fibroblasts, multinucleated cells, and islands of metaplastic chondroid differentiation with focal calcification. Despite origin from an unusual anatomic site, this case reported herein demonstrates the classical morphologic features and clinical history of CAF which showed little in the way of morphologic evolution despite 23 years of persistence. The clinical and histologic features helpful in distinguishing CAF from infantile fibromatosis and soft tissue chondroma are discussed.

Adult↗
PubMed1996

Soft tissue sarcomas in dermatology.

BACKGROUND: Dermatofibrosarcoma protuberans, atypical fibroxanthoma, and cutaneous leiomyosarcoma are uncommon soft tissue sarcomas that tend to have a high recurrence rate when removed with standard excisional surgery, and in some cases have been reported to metastasize. These tumors have been reported in the surgical, medical, and pathology literature. The diagnosis and treatment of these tumors is still evolving. Wide excision and surgical techniques involving meticulous surgical margin control appear to be the most efficacious treatment for these problematic tumors. OBJECTIVE: The surgical, medical, and pathology literature was extensively reviewed to collate the observations of multiple investigators, and to summarize their findings. Literature from medical journals of multiple subspecialties as well as textbooks were reviewed with particular emphasis on diagnosis, histologic features including immunohistochemistry, and useful surgical techniques that facilitate the removal of these difficult tumors. RESULTS: Dermatofibrosarcoma protuberans is a soft tissue neoplasm of intermediate malignancy that shows a high recurrence rate when treated with standard excision. Wide surgical excision using a 3-cm margin greatly reduces the risk of recurrence, but is not practical in some cases. Evidence is continuing to accumulate that Mohs micrographic surgery may be the treatment of choice for this tumor. Atypical fibroxanthoma is best thought of as a superficial form of malignant fibrous histiocytoma. It is histologically indistinguishable from certain forms of malignant fibrous histiocytoma and differs primarily in its size and superficial location. These tumors are best removed surgically. For most smaller tumors a 1-cm margin, which is carried down to the subcutaneous tissue, provides adequate treatment. In cases where tissue conservation is important, Mohs micrographic surgery has been shown to be very effective. Cutaneous leiomyosarcoma is a rare superficial soft tissue sarcoma that has a high incidence of recurrence after excision and can metastasize. Because of the small number of patients treated, treatment recommendations are still evolving. Currently, wide local excision is recommended with a 3-5 cm margin and removal of the underlying subcutaneous tissue. Mohs micrographic surgery has been used in a small number of cases with good success. CONCLUSION: Dermatofibrosarcoma protuberans, atypical fibroxanthoma, and cutaneous leiomyosarcoma are soft tissue sarcomas that have a high rate of recurrence when treated with standard surgical techniques and may occasionally metastasize. When removing these tumors it is very important to take adequate margins. Mohs micrographic surgery is proving to be very useful in treating these difficult neoplasms.

Dermatofibrosarcoma↗
PubMed1989

Clear cell sarcoma. An immunohistochemical analysis of six cases and comparison with other epithelioid neoplasms of soft tissue.

Clear cell sarcoma (CCS) is an uncommon, clinicopathologically distinct neoplasm that typically arises in association with tendons and aponeuroses. It shares several histologic and ultrastructural features with malignant melanoma. Clear cell sarcoma occasionally may be confused with other tumors of soft tissue that have a predominantly epithelioid appearance, including epithelioid leiomyosarcoma, epithelioid neurofibrosarcoma, synovial sarcoma, and epithelioid sarcoma. To assess the potential contribution of immunohistochemistry to this differential diagnosis, a panel of immunostains was applied to examples of each of these neoplasms. All six CCSs contained vimentin, and five were reactive with the melanoma-specific monoclonal antibody HMB-45. In addition, five CCSs expressed neuron-specific enolase, four cases displayed S100 protein, and four examples contained LN3 antigen. Synaptophysin and Leu-7 antigen were present in one case each. Cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, desmin, muscle-specific actin, and leukocyte common antigen were invariably absent. No other primary epithelioid neoplasm of soft tissue reacted with HMB-45. Clear cell sarcoma could be separated from epithelioid leiomyosarcoma by the presence of desmin and muscle-specific actin in the latter neoplasm. Similarly, both synovial sarcoma and epithelioid sarcoma differed from CCS by their expression of cytokeratin and epithelial membrane antigen. Clear cell sarcoma and malignant melanoma were immunohistochemically indistinguishable, supporting the concept that they share a common pattern of differentiation.

Adolescent↗
PubMed1999

Giant cell angiofibroma of the oral cavity: report of a new location for a rare tumor.

Giant cell angiofibroma is a rare, soft tissue tumor that was first described in the orbit. Since then, several case reports have described this tumor in a number of extra-orbital sites, suggesting a wider anatomic distribution than is generally recognized. The tumor typically acts in a benign fashion with only rare local recurrences but no tendency to metastatic disease. Here, we report the first case of a giant cell angiofibroma in the oral cavity. The tumor presented as a soft tissue nodule on the buccal mucosa of a 60-year-old man. The histologic differential diagnosis included a number of other uncommon soft tissue neoplasms, including giant cell fibroblastoma, solitary fibrous tumor, and pleomorphic lipoma. The histologic and immunohistochemical features of this tumor and differentiation from other histologically similar soft tissue neoplasms are briefly discussed.

Angiofibroma↗
PubMed1998

Supernumerary ring chromosomes and nuclear blebs in some low-grade malignant soft tissue tumours: atypical lipomatous tumours and dermatofibrosarcoma protuberans.

We investigated the diagnostic significance of supernumerary ring chromosomes in low-grade soft-tissue neoplasms. Chromosome slides were prepared from 123 samples of soft-tissue tumours using the standard trypsin-Giemsa banding technique. Supernumerary ring chromosomes were found in 6 cases of soft tissue tumours: 5 cases of atypical lipomatous tumour (ALT) and 1 case of dermatofibrosarcoma protuberans (DFSP). By chromosome painting with fluorescence in situ hybridization (FISH), the ring chromosome in 1 ALT was painted over its entire length with the chromosome 12 probe. Nuclear blebs and micronuclei, which were observed in each case of ALT, also contained chromosome 12 material; and these structures may represent a topological distribution of ring or giant marker chromosomes in the interphase nuclei. Our findings suggest that supernumerary ring chromosomes are characteristic of some low-grade soft tissue neoplasms including ALT and DFSP.

Adult↗
PubMed1993

p53 protein expression in non-neoplastic lesions and benign and malignant neoplasms of soft tissue.

Alterations of the p53 tumour suppressor gene, with consequent nuclear p53 protein accumulation, are among the most common genetic lesions in human neoplasms. In the present paper we show p53 immunoreactivity in 65% of malignant and 21% of intermediate malignancy soft tissue tumours, and in 48% of benign/reactive soft tissue lesions. p53 immunoreactivity of sarcomas can be interpreted as an indirect indication of a mutation of the corresponding p53 gene, suggesting that its alteration may have a role in their pathogenesis. Our data on p53 immunoreactivity in benign lesions of the soft tissues are among the first demonstrations of p53 over-expression in benign/reactive conditions. We cannot exclude mutations of the p53 gene in these cases, but it is difficult to sustain this hypothesis in reactive/pseudoneoplastic lesions. Alternatively p53 immunoreactivity in benign processes could be due to an increase in wild-type p53 as a result of different physiological mechanisms (cell type-specific p53 regulation, cell maturation, DNA repair). Our results do not indicate that immunohistochemical demonstration of p53 expression is a marker of malignancy in soft tissue tumours and therefore is of limited use in differential diagnosis. However, they suggest the need for further molecular genetic studies in order to elucidate the biological significance of the abnormal expression of p53 in benign soft tissue lesions.

Humans↗