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Myofibrosarcoma.

Myofibrosarcomas are malignant tumours of myofibroblasts, which have been recognised for many years, but have become clearly defined only recently. They are low- or high-grade sarcomas that arise in soft tissue or bone in adults or children. Low-grade myofibrosarcomas are infiltrative tumours, usually in deep soft tissue, with a predilection for the head and neck region, which display a range of microscopic appearances from fasciitis-like to fibrosarcoma-like; all cases at least focally display nuclear pleomorphism. They express smooth-muscle actin and calponin, and some express desmin, but most lack h-caldesmon. Low-grade myofibrosarcomas can recur but rarely metastasise. Their differential diagnosis is from benign myofibroblastic proliferations, such as fasciitis and fibromatosis, as well as from fibrosarcoma and leiomyosarcoma. Pleomorphic myofibrosarcomas are high-grade pleomorphic sarcomas (malignant fibrous histiocytoma), which show ultrastructural evidence of myofibroblastic differentiation. They closely resemble malignant fibrous histiocytoma clinically and morphologically, but are more frequently actin positive. This article describes the low- and high-grade variants of myofibrosarcoma and other malignant tumours with myofibroblastic differentiation.

Adult↗

Unusual sarcomatoid neoplasm of the lung suggesting a myofibrosarcoma.

Myofibrosarcoma is a rare neoplasm that occurs mainly in the head and neck region and extremities of middle-aged patients. It often appears as a low-grade sarcoma and rarely metastasizes. We report the case of a 47-year-old male patient with a malignant mesenchymal pulmonary tumor affecting almost the entire lower left lobe. Clinically suggestive for a lung carcinoma, the tumor showed typical features of a myofibrosarcoma. A major spindle cell component was observed being positive for smooth-muscle actin, calponin, and vimentin, while stainings for desmin, h-caldesmon, alkaline phosphatase (ALK), and extensively studied cytokeratins were negative. Striking was a strong infiltrate with neutrophilic and eosinophilic granulocytes. DNA cytometry revealed aneuploidy with a peak in the near triploid range. Comparative genomic hybridization demonstrated multiple DNA gains and losses correlating with an aggressive clinical course. Shortly after resection of the primary tumor, the patient showed multiple distant metastases in the contralateral lung, the mediastinal lymph nodes, the left adrenal gland, and the pectoral and deltoid muscle, which responded well to chemotherapy. The case report will discuss the evidence for the final diagnosis of a primary pulmonary myofibrosarcoma and the differential diagnosis of sarcomatoid tumors of the lung.

Diagnosis, Differential↗

Sphincter-splitting approach to the rectum.

This is a brief report of experience gained on our service with the sphincter-splitting approach to the rectum. The main indications for the approach are emphasized. Sphincter splitting is a new way to resolve surgical problems that have been difficult to solve before. It should be offered to suitable patients who otherwise would have to undergo more extensive operations that carry with them more inherent liabilities.

Adenoma↗

Treatment of human clonogenic tumor cells and bone marrow progenitor cells with bleomycin and peplomycin under 40.5 degrees C hyperthermia in vitro.

Tumor cells derived from 13 different individual human tumors were plated in a colony forming monolayer assay. The effect of bleomycin and peplomycin on colony formation was assessed in normothermic conditions and after a hyperthermic treatment at 40.5 degrees C for 2 h at the beginning of the culture. In three out of the 13 tumor samples (two colon carcinomas, one malignant melanoma), hyperthermic incubation resulted in a thermal enhancement of the effects of bleomycin and peplomycin. In addition, human bone marrow progenitor cells (CFU-C) were subjected to the same procedure. Peplomycin proved to be less toxic to CFU-C than bleomycin. In samples from eight different donors, homogeneous dose-response curves were observed. There was no difference between normo- and hyperthermic incubation.

Bleomycin↗

Myofibrosarcoma of the nasal bone.

Myofibrosarcoma is a recently recognized rare tumor that mainly occurs in adults. These tumors are composed of a collagenous stroma and pleomorphic stellate to spindle-shaped cells that resemble smooth muscle cells with eosinophilic cytoplasm and tapered nuclei. We present a case of myofibrosarcoma of the nasal bones in a 4-year-old girl who showed rapid enlargement of a painless glabellar swelling. Computed tomography and magnetic resonance imaging revealed an expanding solid mass with erosion of the surrounding nasal bones. After excision and histopathological examination, this tumor was identified as a myofibrosarcoma. This is the first report of such a tumor localized to the glabellar region. This case report contributes to better awareness of an extremely rare type of glabellar lesion in children.

Bone Neoplasms↗

The cDNA of mouse skeletal muscle transcribe for both isoforms 1 and 2 of acetylcholine receptor alpha subunit.

The mRNA isolated from mouse ocular and limb muscle and human rabdomyosarcoma cell line TE671 was subjected to reverse transcriptase polymerase chain reaction (RT-PCR) using a specific primer pair for the extracellular domain of AChR-alpha subunit that transcribes both for P3A- (isoform 1) and P3A+ (isoform 2). The cDNA synthesized from mRNA by reverse transcription, transcribed both isoforms 1 and 2 from mRNA of mouse limb and ocular muscle and human rabdomyosarcoma cell line TE671 as evidenced by agarose gel electrophoresis of polymerase chain reaction products.

Animals↗

Amplification of Echoviruses genomic regions by different RT-PCR protocols--a comparative study.

In the present report, the results of a comparative study in the detection of all Echoviruses reference strains as well as of 38 clinical isolates are presented. Using RT-PCR with already published primer pairs (UG(52)-UC(53), 292-222, 012-011 and EUG2a, 2b, 2c-EUC2) from the 5'UTR, the VP1 region as well as a long genomic fragment including the VP1 3' end, the entire coding sequence of 2A, 2B, and the 5' moiety of the 2C-coding region amplification was effective with all reference and clinical Echovirus isolates with primer pair UG(52)-UC(53) while with 292-222 and 012-011 were amplified 27/28 reference Echovirus strains and all clinical isolates. As far as EUG2a,2b,2c-EUC2 is concerned, the RT-PCR gave a positive result for 26/28 reference Echovirus strains and 34/38 clinical isolates. The sequence analysis of a large part of the 5'UTR has revealed that there is no correlation between 5'UTR identity and the currently recognized human enterovirus species. It has been suggested that part of VP1 coding sequence would correlate well with serotype since a number of important neutralization epitopes, as well as receptor recognition sequences, lie within the VP1 coding sequence. Therefore, UG(52)-UC(53) and 292-222 primer pairs seem to be the most appropriate for Echovirus detection and, moreover, UG(52)-UC(53) is useful for the classification of enteroviruses into genetic clusters (sub-groups) while 292-222 for the identification of enteroviruses by amplicon sequencing.

DNA Primers↗

Stent fabrication for graft immobilization following wide surgical excision of myofibroblastic sarcoma of the buccal mucosa: a clinical report.

Sarcomas of the head and neck are rare tumors derived from cells of mesenchymal origin. This article briefly discusses the epidemiology, etiology, and classification of head and neck sarcomas. Emphasis is placed on myofibrosarcoma, a malignant tumor of the myofibroblasts. Histologic criteria, prognostic factors, and the multidisciplinary management of these tumors are reviewed. A situation of a surgically excised myofibrosarcoma of the buccal mucosa is reported. In this patient, a stent was fabricated to stabilize a split-thickness skin graft used for the buccal mucosa reconstruction.

Adult↗

Myofibroblastic sarcoma of the nasal cavity and paranasal sinus: a clinicopathologic study of 6 cases and review of the literature.

OBJECTIVE: We describe the clinicopathologic features of 6 cases of myofibroblastic sarcoma (MS) occurring in the nasal cavity and paranasal sinus. STUDY DESIGN: The paraffin-embedded tissues of 6 cases of MS were stained immunohistochemically and examined by electron microscopy. RESULTS: Clinically, a painless enlarging mass was the most common symptom, followed by the nasal obstruction, epistaxis, copious rhinorrhea, and proptosis. Histologically, the tumors showed a diffusely infiltrative growth pattern and consisted mainly of spindle cells with abundant eosinophilic cytoplasm. The hypocellular myxoid areas and the hypercellular fibrous areas were identified. Immunohistochemically, all 6 tumors were positive for vimentin, alpha-smooth muscle actin, calponin, and fibronectin. Ultrastructural examination in 3 cases showed characteristic features of myofibroblast. Follow-up in 6 patients revealed high local recurrence rate (6 out of 6). CONCLUSION: Myofibroblastic sarcoma of the nasal cavity and paranasal sinus exhibit diverse histologic appearances and a strong aggressive behavior.

Actins↗

Low-grade myofibroblastic sarcoma of the vulva.

BACKGROUND: Vulvar sarcomas are rare tumors. A distinctive low-grade myofibroblastic sarcoma is described. CASE: A 46-year-old female presented with a progressively enlarging vulvar mass. Pathological evaluation revealed a low-grade myofibroblastic sarcoma. The immunophenotype is outlined and ultrastructural features are highlighted. Tumor cells were positive for p53 protein, smooth muscle actin, steroid receptors, and showed myofibroblastic differentiation on electron microscopy. The patient has been followed for >14 months without evidence of recurrence. CONCLUSION: The tumor was positive for p53, mitotically active, but was categorized as a low-grade malignancy. Immunohistochemical and ultrastructural criteria were utilized to distinguish this tumor from other neoplasms.

Female↗

Elevated (> or = 10%) MIB-1 proliferative index correlates with poor outcome in gastric stromal tumor patients: a study of 35 cases.

Mitotic activity and tumor size are currently regarded as the most powerful prognostic indicators for patients with gastrointestinal stromal tumor (GIST). This retrospective study evaluated the prognostic accuracy of MIB-1 proliferative index (PI) in combination with these two indicators in 35 GIST patients. Within a high-risk group, determined initially by tumor size and mitotic count, overall survival was significantly shorter for patients whose tumors had PI > or = 10% MIB-1 positive cells. When tumor location (gastric versus small intestine) was taken into account, a combination of tumor size, mitotic count, and PI > or = 10% identified a subgroup of patients with significantly shorter survival for gastric (but not small intestinal) GIST. Based on our results, MIB-1 immunostaining, when used in combination with tumor size and mitotic count, appears to be a powerful tool for identifying patients, especially those with gastric tumors, at high risk of recurrence and early tumor-related death.

Adult↗

Fibroblastic/myofibroblastic sarcoma of the skin: a report of five cases.

BACKGROUND: A number of malignant soft tissue tumors, particularly those of fibroblastic and fibrohistiocytic derivation, have been found to display myofibroblastic differentiation focally. The term myofibroblastic sarcoma, a controversial presumably distinctive entity, defines a malignant soft tissue tumor in which myofibroblasts are quantitatively the predominant cell type. METHODS: Five cases of cutaneous spindle-cell sarcomas showing fibroblastic-myofibroblastic differentiation with predominance of fibroblasts were retrieved from the files of three large centers of dermatopathology. Tumors were analyzed histopathologically, immunophenotypically, and, in two cases, ultrastructurally. Results were compared with those previously reported in fibrosarcoma, malignant fibrous histiocytoma, and myofibroblastic sarcoma. RESULTS: Immunophenotypic and ultrastructural profiles of the cases analyzed in this series were closer to fibrosarcoma and to malignant fibrous histiocytoma than to myofibroblastic sarcoma by virtue of quantitative predominance of fibroblasts over myofibroblasts. On the other hand, histopathologic findings were in keeping with those reported in myofibroblastic sarcoma. CONCLUSIONS: Our series highlights the intrinsic problems in attaching certain cutaneous sarcomas with fibroblastic-myofibroblastic differentiation to one of the recognized entities and gives support to the hypothesis that fibrosarcoma, malignant fibrous histiocytoma, and myofibroblastic sarcoma are related histogenetically.

Aged↗

Penetration failure and misdiagnosis of stereotactic biopsy caused by the uncommonly firm tissue of a gliomyosarcoma.

OBJECTIVE AND IMPORTANCE: We report the very rare case of a gliomyosarcoma that caused penetration failure in stereotactic biopsy and therefore led to misdiagnosis. This complication should be considered as a potential reason for diagnostic failure with uncommonly firm tumors in frame-based stereotactic biopsy. CLINICAL PRESENTATION: An 83-year-old women presented with a 4-week history of right hemiparesis. Computed tomography (CT) demonstrated a left precentral lesion of 1 cm in diameter with moderate contrast uptake and perifocal edema. INTERVENTION: Stereotactic biopsy was performed using the Cosman-Robert-Wells (CRW) system and a side-aspirating biopsy needle. Six tissue samples were taken; however, histopathologic examination remained non-diagnostic. Because the hemiparesis had worsened, a magnetic resonance tomography (MRT) was taken four weeks later and clearly demonstrated an increase in size of the lesion. Neuronavigation-guided open surgery revealed a very firm, well-delimited tumor that was classified in the pathologic examination as a gliomyosarcoma. Repeated recalculations of the target coordinates, analysis of the CT scan that was taken 4 days after the stereotaxy, and finally, recognition of the extraordinary firmness of this gliomyosarcoma allowed us to presume with certainty that we had not penetrated the lesion with the biopsy cannula, but rather had merely pushed it ahead of the instrument while the tissue samples were taken. CONCLUSION: The reported case is both unique for its histopathologic diagnosis and for the complication it caused in stereotactic biopsy. The case also supports the implementation of image-guided interventions for diagnostic biopsy, rather than frame-based stereotaxy in the future.

Aged↗