Myofibrosarcoma

INTRODUCTION: Low-grade myofibroblastic sarcoma (LGMS) is a rare tumor entity which occurs in the subcutaneous and deep soft tissues; it is less common in the bone with a predilection for the extremities and the head and neck region. As confirming the diagnosis is difficult and treatment strategies are not standardized, we aimed to identify patient and tumor characteristics, and to summarize treatment strategies and their clinical outcomes to guide surgeons. METHODS: Included were full articles reporting patients with histology of LGMS in the extremities, excluding tumors of the trunk...

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a highly aggressive malignant subtype of inflammatory myofibroblastoma (IMT) associated with poor prognosis. IMT can occur in various parts of the body, most frequently in the lungs, followed by the mesentery, omentum, retroperitoneum, and pelvis, among other areas; however, it is exceptionally rare in the stomach. Anaplastic lymphoma kinase (ALK) is a critical driver of lung cancer development and is currently the "gold standard" target for non-small cell lung cancer treatment...

Undifferentiated pleomorphic sarcoma (UPS), once termed as malignant fibrous histiocytoma, has always been diagnosed exclusively in clinical practice because it lacks any defined resemblance to normal mesenchymal tissue. Although myxofibrosarcoma (MFS) has been separated from UPS due to its fibroblastic differentiation with myxoid stroma, UPS and MFS are still identified as a sarcoma group in terms of molecular landscapes. In this review article, we will describe the associated genes and signaling pathways involved in the process of sarcoma genesis and make a summary of conventional management, targeted therapy, immunotherapy, and some novel potential treatments of UPS/MFS...

Cutaneous mesenchymal sarcomas are rare malignancies that include dermatofibrosarcoma protuberans, atypical fibroxanthoma, pleomorphic dermal sarcoma, cutaneous angiosarcoma, myofibrosarcoma, and leiomyosarcoma. These tumors lack consensus guidelines on staging and management. Treatment of local disease involves complete surgical removal but recurrence rates are higher compared with more common forms of nonmelanoma skin cancer. Cutaneous angiosarcoma, pleomorphic dermal sarcoma, and subcutaneous leiomyosarcoma have increased risk of metastatic spread and lower survival rate...

Soft tissue tumors with myxoid components are often a diagnostic challenge for the pathologist. We retrospectively reviewed 41 cases of soft tissue tumors with myxoid components diagnosed in our center over a five-year period. The most frequent diagnoses were myxofibrosarcoma and myxoid liposarcoma, followed by low-grade fibromyxoid sarcoma, low-grade fibromyxoid tumor and myxoid neurofibroma. Other diagnoses included were extraskeletal myxoid chondrosarcoma, myxoinflammatory fibroblastic sarcoma, low-grade myxoliposarcoma, myofibrosarcoma, fibromatosis, solitary fibrous tumor, non-ossifying variant of ossifying fibromyxoid tumor and ancient neurinoma with myxoid degeneration...

No abstract text is available yet for this article.

Cutaneous mesenchymal tumors (CMT) are rare tumors with wide clinicopathologic heterogeneity. Treatment of malignant cutaneous mesenchymal tumors traditionally includes wide local excision (WLE), though Mohs micrographic surgery (MMS) has been increasingly used. A PubMed literature review of articles from inception until September 2019 related to malignant CMT and surgical treatment with MMS or WLE was completed. Dermatofibrosarcoma protuberans treated with MMS recurred in 1.2% of patients with no reported metastasis...

No abstract text is available yet for this article.

Myofibroblastic, fibroblastic and/or myoid lesions are rare in the breast but comprise the majority of mammary mesenchymal spindle cell lesions. Whereas most have similar features to their counterparts at extramammary sites, pseudoangiomatous stromal hyperplasia is considered a breast-specific myofibroblastic proliferation on the same spectrum as myofibroblastoma. Other lesions with myofibroblastic/fibroblastic differentiation include fibromatosis and nodular fasciitis, as well as more aggressive tumors such as the rarely reported myofibrosarcoma, inflammatory myofibroblastic tumor and fibrosarcoma...

PURPOSE: To report a case of recurrent conjunctival myofibrosarcoma treated with wide surgical excision, cryotherapy, and triple sequential applications of episcleral brachytherapy. METHODS: A single case of recurrent conjunctival myofibrosarcoma. RESULTS: A 54-year-old man with a history of a renal transplant presented with a recurrent conjunctival tumor. Histopathologic diagnosis was established through immunohistochemistry. In total, 3 iodine radiation episcleral plaques were used over a period of 49 weeks...

Synovial sarcoma is a malignant mesenchymal tumor with variable epithelial differentiation, which is defined by the presence of a specific t(X;18)(p11.2;q11.2) chromosomal translocation that generates SS18-SSX fusion oncogenes. Synovial sarcoma typically arises within extremity deep soft tissue (particularly around large joints) of young adults, but has been shown to occur at almost any location. When it arises in more unusual sites, such as the abdomen, it can present a significant diagnostic challenge. We describe a case of intraabdominal monophasic synovial sarcoma that immunohistochemically showed strong expression of smooth muscle actin and calponin but only very scanty cytokeratin, and which showed morphologic and immunohistochemical overlap with other spindle cell neoplasms that can arise at this site, such as gastrointestinal stromal tumor and myofibrosarcoma...

BACKGROUND: Myofibroblastic sarcoma or myofibrosarcoma is a malignant tumor of myofibroblasts and known to develop rarely in the breast, but its underlying lesion and tumor cell origin have never been reported yet. CASE PRESENTATION: A 61-year-old female presented with a gradually growing breast mass with well-demarcated ovoid nodular shape. The tumor was histologically characterized by fascicular-growing spindle cell proliferation with large areas of hyalinized fibrosis and focally ductal epithelial remnants embedded in myxoid stroma, mimicking a fibroadenomatous lesion...

We report on construction of a neo-left atrium (LA) with 2 bovine pericardial patches after in toto removal of a huge mediastinal tumor invading the heart and lung. The procedure was performed on the beating heart using normothermic cardiopulmonary bypass (CPB) without cardioplegic arrest. Treatment consisted of tumor removal necessitating left pneumonectomy, excision of the LA, resection of the mitral annulus, excision of the tumor from the left ventricle, and construction of a new LA using 2 bovine pericardial patches and a new mitral bioprosthesis...

Malignant tumours of myofibroblasts are rare lesions. Post radiation sarcoma is a rare potential late sequel of ionizing radiation. We present the first case of myofibrosarcoma of the hypopharynx in a 67-year-old man, occurring 16 years after radiotherapy for laryngeal carcinoma.

INTRODUCTION: Spindle cell rhabdomyosarcoma of the head and neck is a very rare tumor in adults. We report on one case with long-term survival. CASE PRESENTATION: A 41-year-old nonsmoking Caucasian man presented in June 2007 with a painless swelling under his tongue. A diagnosis of a soft tissue sarcoma, and a myofibrosarcoma in particular, was made via biopsy. After multimodal treatment, including local and systemic therapy, our patient remained disease-free until September 2010...

OBJECTIVE: The current study presents our experience with accessory parotid gland masses and reviews the literature on accessory parotid tumor incidence and surgical management. Based on our results, we advocate a standard parotidectomy approach with routine facial nerve dissection at the time of excision. STUDY DESIGN: We performed a retrospective chart review and comprehensive literature review on incidence of accessory parotid gland neoplasms. METHODS: A retrospective chart review of all patients with mid-cheek masses treated by the senior author was conducted from January 2003 to January 2009...

INTRODUCTION: Low-grade myofibroblastic sarcoma (myofibrosarcoma) is described to be a distinct atypical myofibroblastic tumor often with fibromatosis-like features and predilection for head and neck. Low-grade myofibroblastic sarcoma of bone is extremely rare. PRESENTATION OF CASE: A 50-year-old woman was admitted to our hospital because she had experienced right knee pain for 2 years. Plain radiography showed a honeycombed lesion on the right distal femur, and computed tomography showed a bone tumor with cortex destruction invading the soft tissue...

We present a carcinosarcoma ex non-recurrent pleomorphic adenoma composed of a large cell neuroendocrine carcinomatous component and a spindle cell sarcoma with myofibroblastic differentiation. The tumor contained a hyalinized transition zone where the classical PA appeared to acquire two different histopathological patterns of malignant transformation of the epithelial component. The carcinomatous component was strongly and diffusely positive for low-molecular weight cytokeratins (AE1-3), synaptophysin, thyroid transcription factor-1 and focally positive for chromogranin A...

Some view ultrastructure as key to myofibrosarcoma diagnosis, whereas others argue that electron microscopy is too little used in contemporary practice to be considered an important diagnostic tool. These views are discussed in the context of 10 ultrastructurally confirmed cases of myofibrosarcoma, some occurring at rare sites such as skin and penis. Patient age ranged from 21 to 83 years, with a 6:4 male to female ratio. Size ranged from 2 to 7.5 cm and all had infiltrative margins. Histologically, all consisted of variably cellular fascicles of spindle cells with mild to moderately pleomorphic nuclei, small punctate nucleoli, and eosinophilic cytoplasm...

Low-grade myofibroblastic sarcoma is a malignant tumour from myofibroblasts, which has only recently become clearly defined. It represents a rare entity developing in the soft tissues of the head and neck. About 20 cases have been reported in the oral cavity, especially in the tongue and bone, while gingiva as the primary site has been described only once to date. Diagnostic methods include histology and immunohistochemistry. The present report concerns a case of a 37-year-old man who presented with a persistent gingival ulcerated swelling that was interpreted for a long time as a gingival epulis...