Case Of Low-grade Myofibroblastic Sarcoma Research Articles

Surgical Management of a Rare Case of Low-Grade Myofibroblastic Sarcoma of the Larynx.

Low-grade myofibroblastic sarcoma (LGMS) of the larynx is an extremely rare entity. We report a case of LGMS in a 59-year-old man presenting with progressive hoarseness and a right laryngeal mass and there was no recurrence during the 6-month follow-up after the total laryngectomy.

Transoral Laryngeal Surgery in Low-grade Myofibroblastic Sarcoma of Larynx: A Plausible Treatment Option.

The laryngeal tumor type, location and disease extent are essential determinants in deciding the type of surgery and the potential voice and swallowing outcomes. The surgical options available are conservative laryngeal surgeries like transoral laser microsurgery (TLM) or open conservative laryngeal surgery and Total laryngectomy. We report an unusual case of low-grade Myofibroblastic sarcoma of vocal cord which was managed by TLM.

Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children

Low-grade myofibroblastic sarcoma (LGMS) is a mesenchymal tumor of myofibroblasts that occurs more frequently in adults. A series of three cases is presented to illustrate that LGMS may also occur within the oral cavity in children and adolescents. The first case (Case 1) occurred intra-osseously in the mandible, while the remaining two presented as gingival swellings and were purely restricted to soft tissue (Cases 2 and 3). The intra-osseous lesion arose in a 7-year-old girl, whereas the gingival lesions were observed in a 12-year-old girl (Case 2) and a 13-year-old boy (Case 3). Histopathologically, all cases were composed of spindle shaped cells arranged into long fascicles showing mild to moderate degree of nuclear atypia. Ki-67 (MIB-1) proliferation activity was relatively low, amounting to 3–5% in all cases. Immunohistochemically, all cases showed smooth muscle actin (SMA) positivity in spindle cells, while desmin, beta catenin, cytokeratin, and CD34 were negative, resulting in a diagnosis of LGMS. In conclusion, current series of three cases of LGMSs that occurred in the oral cavity in a child and two adolescent patients is presented to highlight an emerging disease that requires additional data for further characterization.

Myofibroblastic sarcoma in a sickle cell anemia patient: A case report from Sokoto, North-Western, Nigeria

Low-grade myofibroblastic sarcoma (LGMS) is a rare malignant tumor of soft tissue that commonly presents as a painless enlarging mass of the affected site. There are several reported cases of malignant cancers among sickle cell disease (SCD) patients, and to the best of our knowledge, there was no previously reported case of LGMS occurring in a known SCD patient in our environment. We report a 16-year- old patient with sickle cell anemia who presented with the left knee swelling that was initially managed as a benign infection, but a later histological examination revealed a diagnosis of LGMS.

Low-grade myofibroblastic sarcomas of the maxilla.

Low-grade myofibroblastic sarcoma (LGMS) is a distinct mesenchymal myofibroblastic malignancy. The tumor may occur at a variety of sites, but is particularly associated with the head and neck. Of the two maxillary sarcomas that were analyzed in the present study, one was misdiagnosed as an inflammatory myofibroblastic tumor during pre-operative excision biopsy, and later presented with a different immunophenotype upon recurrence. Representative paraffin blocks from formalin-fixed tissues were selected from each patient and designated as case 1 and case 2. Immunohistochemical studies were performed on 3-μm thick sections using primary antibodies against α-smooth muscle actin (α-SMA), muscle-specific actin (MSA), desmin, vimentin, calponin, h-caldesmon, fibronectin, cytokeratin, cluster of differentiation 34 (CD34), S-100 protein, anaplastic lymphoma kinase (ALK), epithelial membrane antigen (EMA) and Ki-67. Immunohistochemistry was performed using the streptavidin-biotin-peroxidase complex method. The tumor cells from the two maxillary LGMSs, including the recurrent lesion, were positive for vimentin and fibronectin, and negative for S-100 protein, CD34, EMA, h-caldesmon, ALK, MSA and calponin. The tumor cells from case 1 demonstrated positive staining for α-SMA protein and negative staining for desmin. By contrast, the tumor cells from the primary lesion in case 2 presented with negative staining for α-SMA and positive staining for desmin, while the cells of the recurrent lesion were α-SMA-positive and desmin-negative. The present study concluded that cases of LGMS with immunoprofile alterations are predictive of relatively poor prognoses.

Cardiac Metastasis of a Low‐Grade Myofibroblastic Sarcoma

We aim to present a rare case of low-grade myofibroblastic sarcoma arising in the inguinal region accompanied by cardiac metastasis. A 36-year-old male patient suffering from recurrent inguinal swelling was operated on and the initial histopathological evaluation mistakenly diagnosed the condition as benign. During follow-up, a recurrence of mass was detected in the same region and a pathological examination revealed a low-grade myofibroblastic sarcoma. Cardiac metastasis was diagnosed shortly before rapid disease progression and death. The learning points relevant to this case are as follows: (1) Echocardiographic screening in patients with noncardiac myofibroblastic sarcomas may be helpful in the detection of silent metastasis. (2) Low-grade myofibroblastic sarcomas in the inguinal region may be misdiagnosed as benign after enucleation. Thus, rigorous histopathological examination of myofibroblastic sarcomas is crucial. (3) According to our knowledge, this is the first report of a low-grade myofibroblastic sarcoma to have a potential for cardiac metastasis with potentially fatal course.

Long-term disease-free survival after radical local excision of low-grade myofibroblastic sarcoma of the vulva

•A second case of low-grade myofibroblastic sarcoma of the vulva.•Six-year follow-up with no evidence of disease after radical local excision.•Adequate margin resection is important.

Clinicopathological and immunohistochemical study of low-grade myofibroblastic sarcoma of the liver—One case report

Low-grade myofibroblastic sarcoma is a recently characterized tumor showing features of myofibroblastic differentiation that is part of the spectrum of malignant mesenchymal tumors. This extremely rare type of tumor occurs most commonly in superficial locations. The case we describe herein is that of a 60-year-old man with two large hepatic masses. The patient’s tumor was removed radically through an incision due to the inconclusive imaging findings. Follow-up computed tomography showed no recurrence and metastasis after 37 months, suggesting that enucleation was adequate for tumor eradication. To our knowledge, this is the first reported case of low-grade myofibroblastic sarcoma in the liver in the English language literature.

Differential Diagnosis of Inflammatory Myofibroblastic Tumour and Low-Grade Myofibroblastic Sarcoma: Two Case Reports with a Literature Review

Inflammatory myofibroblastic tumour (IMT) and low-grade myofibroblastic sarcoma (LGMS) have similar morpho logical and immunophenotypic features, but LGMS is more malignant than IMT and the treatment requires a wider surgical margin plus post-operative chemotherapy or radiotherapy. To date, only 28 cases of IMT and two cases of LGMS have been reported in the laryngopharynx. Recent studies have suggested that anaplastic lymphoma kinase (ALK) and cytokeratin are important markers for differentiating between the two tumours. Here, two cases involving different myofibroblastic tumours of the larynx are reported. Based on the histological and immunohistochemical results, case 1 was diagnosed as IMT involving the right arytenoepiglottic fold, while case 2 was diagnosed as LGMS involving the epiglottic-glossal surface. There was no recurrence or metastasis in either case after post-operative follow-up (12 and 14 months, respectively). It is difficult to distinguish IMT from LGMS; both morphological and immunohistological analyses are required.

A rare case of low-grade myofibroblastic sarcoma of the femur in a 38-year-old woman: a case report

IntroductionPrimary myofibroblastic sarcoma of the bone is a rare spindle cell tumour with, to the best of our knowledge, only eight cases reported in the available English language literature. The disease's rarity and its low-grade features make an accurate diagnosis difficult in most cases. The differential diagnoses of this unusual tumour include various benign entities as well as other sarcomas. Due to the difference in prognosis, a precise pathologic diagnosis is essential, which requires a combination of thorough morphologic examination, immunohistochemistry and electron microscopy wherever available.Case presentationWe report the case of a 38-year-old Indian woman with a lytic lesion in her left femur. The tumour was associated with cortical destruction and soft tissue extension. A biopsy from the soft tissue component showed features suggestive of a low-grade malignant mesenchymal tumour. Excision of the tumour was performed and histopathological examination showed a low-grade spindle cell sarcoma with collagenous stroma. Expressions of vimentin and smooth muscle actin were also noted. Ultrastructural examination confirmed its myofibroblastic nature. A final diagnosis of low-grade myofibroblastic sarcoma of the left femur was thus rendered.ConclusionLow-grade myofibroblastic sarcoma is one of the rarer osseous spindle cell sarcomas depicting a favourable prognosis in the cases reported so far. Its diagnosis requires ancillary techniques like immunohistochemistry and electron microscopy. To the best of our knowledge, we report the ninth case in the literature and the first case from our subcontinent.

Low-grade myofibroblastic sarcoma of the oral cavity

Two cases of low-grade myofibroblastic sarcoma (LGMS) are presented: one of lateral tongue, the other of lower buccal vestibule. LGMS represents a distinct atypical myofibroblastic tumor that occurs in several sites, primarily within the head and neck regions. A painless, enlarging mass is the most common clinical presentation, but a definitive diagnosis requires both histopathological and immunohistochemical analyses. Histologically, LGMS commonly presents as a cellular lesion composed of spindle-shaped tumor cells arranged primarily in fascicles with a diffusely infiltrative pattern. Immunohistochemically, LGMS shows positive staining for at least one myogenic marker, such as desmin, and muscle actin.

上顎に発生した低悪性型筋線維芽細胞肉腫の1例

上顎に発生した低悪性型筋線維芽細胞肉腫の1例

Myofibroblastic Tumor of the Lower Lip in a Patient With X-Linked Hypogammaglobulinemia and Isolated Growth Hormone Deficiency: A Case Report

Tumors of myofibroblastic origin are a relatively recently characterized group of conditions that are the subject of considerable controversy and confusion in the literature. Recognition of the myofibroblast as a distinct cell type with well-defined ultrastructural and immunohistochemical characteristics prompted reclassification of many stromal tumors and tumor-like proliferations as myofibroblastic. Although relatively uncommon, a number of these conditions may affect oral and maxillofacial regions and present considerable diagnostic challenge. Biological behavior of myofibroblastic lesions ranges from entirely benign to frankly malignant and, therefore, accurate histopathological diagnosis is paramount to patient management. We describe a case of low-grade myofibroblastic sarcoma of the lower lip in a patient with X-linked hypogammaglobulinemia and isolated growth hormone deficiency.