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PubMed1986

Fast neutron radiotherapy for soft tissue sarcomas. University of Washington experience and review of the world's literature.

Sixteen patients with inoperable soft tissue sarcomas were treated definitively with fast neutrons at the University of Washington between August, 1970 and May, 1982. Eleven of these 16 patients were treated with curative intent and form the basis of this report. Actuarial plots are shown for local tumor control and survival. This work is placed in the context of worldwide experience in using fast neutrons to treat unresectable soft tissue sarcomas.

Fast Neutrons↗
PubMed1996

Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant.

Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients. We analysed the clinicopathologic features in a series of 75 patients. All patients were adults (range, 22-91 years; median, 66 years) with an approximately equal incidence in men and women. Thirty-five tumors arose in the lower and 25 in the upper extremities, nine on the trunk, two each in the retroperitoneum and the head and neck region, and one each in the pelvis and penis. Forty-eight cases (69.5%) were located in dermal or subcutaneous tissues. Distinctive histologic features included the following: a commonly nodular growth pattern; a myxoid matrix containing elongated, curvilinear capillaries; and fusiform, round or stellate tumor cells with indistinct cell margins, slightly eosinophilic cytoplasm, and hyperchromatic atypical nuclei. These lesions varied from a hypocellular, mainly myxoid, and purely spindle-cell appearance (low-grade neoplasms) to high-grade, pleomorphic (malignant fibrous histiocytoma-like) lesions with multinucleated giant cells, high mitotic activity, and areas of necrosis. Immunohistochemistry in 44 cases revealed only vimentin and occasional actin positivity. Ultrastructurally, tumor cells had a fibroblastic phenotype. DNA flow cytometry and proliferation analysis showed an association between aneuploidy and histologic grade. An average follow-up of 45 months (range, 5-300 months) in 60 cases has revealed local recurrence in 33 cases (54%). Thirteen patients developed metastases, and 13 tumor-related deaths occurred. A short interval to first local recurrence was associated with poor clinical outcome. The rate of local recurrence was independent of histologic grade, but only intermediate and high-grade neoplasms metastasized. The depth of the primary lesion did not influence the incidence of local recurrence. However, in deep-seated neoplasms, the incidence of metastases was higher and the percentage of tumor-related deaths was twice as high as in superficially located lesions, reflecting the fact that deep-seated lesions tended to be higher-grade, larger tumors. Myxofibrosarcoma tends to become progressively higher grade in recurrences, as demonstrated in five cases in our series. The poorly recognized low-grade myxofibrosarcoma is emphasized, as proper diagnosis and treatment and scrupulous follow-up are mandatory to avoid local recurrence and gradual tumor progression to a higher-grade neoplasm that may then metastasize.

Adult↗
PubMed2000

Endometrial stromal tumors: an update on a group of tumors with a protean phenotype.

Endometrial stromal tumors are reviewed with emphasis on their wide morphologic spectrum and problems in differential diagnosis, highlighting issues that have received particular attention in the recent literature. These neoplasms are divided into two major categories--endometrial stromal nodules and endometrial stromal sarcomas--a distinction made on the basis of the lack of significant infiltration at the periphery of the former. The division of endometrial stromal sarcomas into low-grade and high-grade categories has fallen out of favor and the designation endometrial stromal sarcoma is now considered best restricted to neoplasms that were formally referred to as "low-grade" stromal sarcoma. Endometrial sarcomas without recognizable evidence of a definite endometrial stromal phenotype, designated poorly differentiated "endometrial sarcomas," are almost invariably high grade and often resemble the mesenchymal component of a malignant mullerian mixed tumor. Two features of endometrial stromal tumors that may cause confusion are smooth muscle differentiation and epithelial patterns. Cases in the former category often have a characteristic "starburst" pattern of collagen formation. The most common epithelial patterns resemble those seen in ovarian sex-cord stromal tumors. Much less common is endometrioid gland differentiation. Some endometrial stromal tumors have a prominent fibrous or myxoid appearance and the myxoid tumors should be distinguished from myxoid leiomyosarcoma. Other unusual features of endometrial stromal tumors are also discussed. Lesions in the differential diagnosis of uterine endometrial stromal neoplasms include highly cellular leiomyoma, cellular intravenous leiomyomatosis, adenomyosis with sparse glands, metastatic carcinoma, and lymphoma. Endometrial stromal sarcomas at extrauterine sites may be primary or metastatic from a uterine tumor, the latter sometimes being occult and difficult to definitively establish, particularly if there is a history of a remote hysterectomy for "leiomyomas." Endometrial stromal sarcomas of the ovary, whether primary or metastatic, may be difficult to distinguish from ovarian sex-cord stromal tumors. Extragenital endometrial stromal sarcomas may be confused with diverse lesions such as gastrointestinal stromal tumors, hemangiopericytoma, lymphangiomyomatosis, or mesenchymal cystic hamartoma of the lung. Immunohistochemistry may play a role in evaluating these tumors and in some instances establishing the diagnosis although conventional light microscopic analysis suffices in the majority of cases. The unusual tumor, the "uterine tumor resembling an ovarian sex-cord tumor," is also considered in this review as it is almost certainly of endometrial stromal derivation in many cases. These neoplasms may have a striking resemblance to granulosa cell tumors or Sertoli cell tumors, including those with a retiform pattern, and have recently been shown to be frequently inhibin positive.

Antigens, Neoplasm↗
PubMed2005

Myxoid clear cell sarcoma.

Clear cell sarcoma is a rare soft-tissue tumor presenting typically in the extremities of young adults. It has been also known as malignant melanoma of the soft parts because of the presence of melanin and cytoplasmic melanosomes. However, clear cell sarcoma is, at present, usually considered as a unique lesion because the t(12;22)(q13;q12) translocation is present only in clear cell sarcoma. Myxoid malignant melanoma is now a well-recognized morphologic variant of malignant melanoma. However, a myxoid variant of clear cell sarcoma has not been well described yet. We report a case of myxoid clear cell sarcoma occurring on the heel in a 22-year-old man. The tumor was composed of nests and fascicles of oval to fusiform cells with clear to pale eosinophilic cytoplasm, often separated by fibrous septa. The tumor cells were reactive for S-100 protein, HMB-45, and MART-1. Variably sized cysts lined by one or several layers of tumor cells were observed. Alcian blue and mucicarmine stains demonstrated prominent mucin deposition in the tumor stroma and especially in the lumen of the cysts. Fluorescence in situ hybridization for the Ewing sarcoma gene showed rearrangement in nearly all of the neoplastic cells.

Adult↗
PubMed2005

Myxoinflammatory fibroblastic sarcoma of the neck.

BACKGROUND: Myxoinflammatory fibroblastic sarcoma (MIFS), also named inflammatory myxohyaline tumor of distal extremities with virocyte or Reed-Sternberg cells, is a rare tumor typically presenting as a painless mass in the extremities. PATIENTS: We present an unusual case of MIFS presenting as a subcutaneous neck mass. This is the first reported case of MIFS presenting in the neck. RESULTS: Therefore, this lesion must be considered in the differential diagnosis for painless subcutaneous masses presenting not only in the distal extremities, but also in the neck. CONCLUSION: MIFS has only recently been recognized. The differential diagnosis for MIFS is broad, and it can often be mistaken for several different inflammatory and neoplastic processes, which may require different treatment.

Adult↗
PubMed1982

Primary malignant tumor of the heart.

An autopsy case of a primary malignant tumor of the heart in a 15-year-old boy was reported. The tumor had possibly arisen from the subendocardial region of the septal area of right atrium, occupied the atrial septum and formed a spherical mass with a diameter of ca. 9 cm. It protruded into the right atrial chamber and ruptured the endocardium. Multiple metastases were observed in the lungs and the paratracheal lymph nodes. On the basis of light and electron microscopic features, the tumor was interpreted as a poorly differentiated mesenchymal tumor with partial myxosarcomatous differentiation. The histogenetical aspects of this tumor were discussed with reference to morphology and cytodifferentiation of primitive mesenchymal tissues of the heart.

Adolescent↗
PubMed1987

Myxoid sarcoma arising from the pulmonary trunk.

We reported a case of a myxoid sarcoma of the pulmonary trunk in a 40-year-old women. On light and immunohistochemical studies, spindle-shaped tumor cells revealed the nature of smooth muscle origin.

Adult↗
PubMedDate not supplied

Low-grade myxofibrosarcoma: progression in recurrence.

A case of low-grade myxofibrosarcoma with histologic progression in recurrence occurring in a 51-year-old male is described. The patient had a well-circumscribed encapsulated myxoid mass, which measured 2.5 cm at its greatest diameter, in the subcutis of the left forearm. Microscopically, the tumor was characterized by a proliferation of sparsely distributed spindle or stellate cells, curvilinear small vessels and myxoid stroma. It demonstrated mild pleomorphism without mitotic figures. The patient had the first recurrence at 3 years, which was histologically identical to the primary tumor. The patient had a second recurrence at 11 years, which was predominantly composed of sheets of anaplastic, rounded or oval cells with focal osteoid formation. Immunohistochemically, the tumor cells in the primary and the first recurrent lesions were focally positive for vimentin. In the second recurrence, the tumor cells contained vimentin, alpha-smooth muscle actin and muscle specific actin (HHF35). The primary lesion had a diploid DNA content with low S-phase fractions. The second recurrence showed a polyploidy. The patient was well with no evidence of disease 18 months after the second recurrence. These findings suggest that this neoplasm showed histological progression with an increasing risk of metastasis. Low-grade myxofibrosarcoma, which commonly is misinterpreted as benign, has a tendency for histological and biological progression in local recurrences, underlining the importance of accurate diagnosis and wide surgical excision of the primary lesion.

Actins↗
PubMed1989

Myxofibrosarcoma in the left atrium originally presented as a cardiac myxoma with chondroid differentiation. A clinico-pathological report.

Malignant cardiac tumours located on heart valves are very rare. We report on a myxofibrosarcoma originally presented as a cardiac myxoma (CM), partly with chondroid differentiation, located on the posterior leaflet of the mitral valve. The tumor recurred twice, the first time disguised as a CM with only minor components of myxofibrosarcoma, and finally metastazised as a myxofibrosarcoma. Patient survival (40 months from first surgery) was surprisingly long and this may well reflect both the effectiveness of the combined radiation and cytostatic treatment and the histopathological features of the tumor.

Adult↗
PubMed2006

Skp2 overexpression is highly representative of intrinsic biological aggressiveness and independently associated with poor prognosis in primary localized myxofibrosarcomas.

PURPOSE: Two SCF(Skp2) ubiquitin ligase-related proteins, Skp2 and cyclin-dependent kinase subunit 1 (Cks1), are involved in posttranscriptional degradation of p27(Kip1) tumor suppressor. We analyzed the prognostic utility of p27(Kip1) and its interacting cell cycle regulators in myxofibrosarcomas. EXPERIMENTAL DESIGN: Clinicopathologic features and tissue microarray-based immunohistochemical expression of p27(Kip1), Skp2, Cks1, cyclin E, cyclin A, Ki-67, and minichromosome maintenance protein 2 (Mcm2) were assessed in 70 primary myxofibrosarcomas and correlated with clinical outcomes. Skp2 mRNA expression and the relationship between Skp2 and p27(Kip1) proteins were examined in six cases by semiquantitative reverse transcription-PCR and Western blotting, respectively. RESULTS: High indices of Skp2 (> or =10%), cyclin A (> or =10%), and Mcm2 (> or =50%) were adverse prognosticators at the univariate level. Furthermore, co-overexpression of Skp2 and cyclin A identified highly lethal cases in the entire cohort [P < 0.0001 for disease-specific survival (DSS), P = 0.0004 for overall survival (OS)] and the lower-grade subset (Fédération Nationale des Centres de Lutte Contre le Cancer grade 1 and 2; P = 0.0006 for DSS, P = 0.0093 for OS). In multivariate analyses, Skp2 overexpression overshadowed most intrinsic clinicopathologic factors and independently correlated with worse metastasis-free survival (P = 0.0012), DSS (P = 0.0234), and OS (P = 0.0056). Notably, positive margins independently predicted inferior local recurrence-free survival (P = 0.0012) and also negatively affected metastasis-free survival (P = 0.0471), DSS (P = 0.0152), and OS (P = 0.0173). Reverse transcription-PCR showed up-regulation of Skp2 mRNA in four cases and Western blotting displayed a matched expression pattern of Skp2. CONCLUSIONS: Margin status and intrinsic property of myxofibrosarcomas both affect patient survival. Skp2 overexpression is highly representative of the biological aggressiveness of myxofibrosarcomas and plays an important prognostic role.

Adolescent↗