Low-grade Myofibroblastic Sarcoma Research Articles

Low-grade myofibroblastic sarcoma of retroperitoneum: a rare case

Low-grade myofibroblastic sarcoma (LGMFS) is an uncommon tumor which develops mainly in the bone or soft tissues of the head and neck region, trunk, or extremities and extremely rarely found in the abdominal cavity. The rarity of the disease and its low-grade features make an accurate diagnosis difficult in most cases. We recently encountered a giant LGMFS which had developed in retroperitoneum and surgically resected with gratifying results. An 18 years old female presented with complaint of left sided abdominal pain since 8 months and left sided abdominal lump since 5 months. Imaging examinations revealed retroperitoneal soft tissue mass, and surgical treatment was scheduled. During operation, a tumor about 20x18x15cm in diameter with its anterior aspect covered with the pancreas, mesocolon and splenic flexure of colon with densely adhered to splenic vessels and left renal vessels was found. The tumor had firm adhesions to the surrounding tissues, and it was excised with concomitant distal pancreato-splenectomy, left nephrectomy and resection of splenic flexure of colon with colo-colic anastomosis. Histopathologically, fusiform cells were arranged in a complicated or storiform pattern, and immunohistochemical staining revealed that the tumor was positive for vimentin and focally positive for α-smooth muscle actin (SMA), negative for desmin, CD34, CD117, EMA, DOG-1 and S-100. Diagnosis of LGMFS was made. During 6 months follow up patient is asymptomatic and ultrasound abdomen is normal. In case of LGMFS, favorable prognosis can be attained by complete resection of the primary lesion and regular follow up of patient by physical examination and imaging modality.

Low-grade myofibroblastic sarcoma: a case report of a child

Introduction: Myofibroblastic sarcomas are malignant tumors characterized by the increased proliferation of myofibroblasts; they are rare and have been recently discovered. Observation: A 14-year-old adolescent had a large lesion in the inside of her right mandible. The diagnosis, after a difficult histological analysis, was low-grade myofibrosarcoma. The patient was treated with non-interruptive hemimandibulectomy. Discussion: Low-grade myofibroblastic sarcoma is one of four types of myofibroblastic sarcomas recognized by the WHO in the 2001 classification. This classification allows us to determine a prognosis based on histological characteristics of the lesion. Conclusion: In all cases of low-grade myofibroblastic sarcoma, the preferred treatment is curative surgical resection; but still poses a problem in the prosthetic rehabilitation of oral lesion.

Unilateral maxillary swelling: a varied spectrum

<p class="abstract"><strong>Background:</strong> Diverse disease entities may present with mass in the maxilla and include benign conditions and malignant tumours. A detailed evaluation including preoperative imaging is essential to plan approach to management of isolated maxillary sinus lesions optimally.</p><p class="abstract"><strong>Methods:</strong> We did a retrospective chart review of 14 patients with isolated maxillary swelling and involvement of maxillary sinus who presented to the Department of ENT in a tertiary care hospital over a period of 4 years. A detailed history and examination were done and investigations including contrast enhanced CT scan of the nose and paranasal sinuses was done in all patients. </p><p class="abstract"><strong>Results:</strong> A total of 14 patients were included in this study. 7 were male and 7 were female. The mean age of the patients was 52.6, the age group of patients varied from 6 to 77 years. In our series we had patients with benign lesions like dentigerous cyst, fibro-osseous lesions, mucocele, radicular cyst, aspergilloma, and mucormycosis. Malignant cases included Adenoid cystic carcinoma, Low grade myofibroblastic sarcoma, Diffuse B cell lymphoma and undifferentiated carcinoma. All patients were treated surgically, they underwent a combined endoscopic and Caldwell Luc approach.</p><p class="abstract"><strong>Conclusions:</strong> In patients with unilateral maxillary swelling, a systematic approach to diagnosis with preoperative contrast enhanced CT scan is essential with a high index of suspicion. Trans nasal endoscopic approach with sinus surgery is the preferred approach for these lesions, however, in cases of jaw cysts and odontogenic cysts extending laterally it can be combined with a Caldwel-Luc approach.</p>

Histopathological and Immunohistochemical Diagnosis of Low-Grade Myofibroblastic Sarcoma

Myofibroblastic sarcoma is a rare neoplasm of smooth muscle cell origin and, importantly, contains cells with myofibroblastic differentiation. A 27-year-old man was referred to the maxillofacial surgery service with the complaint of a bleeding nodule, with pain, and a 1-month evolution. A physical examination revealed an ulcerated nodule involving the left soft palate, measuring approximately 2 × 1 cm, of a rubbery consistency to palpation. After an incisional biopsy, the microscopic examination revealed a mesenchymal neoplasia composed of spindle to stellate-shaped cells arranged in intersecting fascicles, sheets, and storiform whorls. Immunohistochemical analysis showed negativity for S-100, laminin, CD34, ALK1, CK pool, and desmin; thus, neural, hematopoietic/vascular, lymphatic, and epithelial origin were excluded. However, positive results were obtained for SMA, calponin, collagen IV, and Ki-67 confirming the association with smooth muscle myofibroblastic differentiation and a high rate of cell proliferation. The diagnosis of low-grade myofibroblastic sarcoma was confirmed, and the patient was referred for medical evaluation.

Low-grade myofibroblastic sarcoma arising in the tip of the tongue with intravascular invasion: A case report.

Low-grade myofibroblastic sarcoma (LGMS) is a rare intermediate tumor, which rarely metastasizes and has myofibroblastic differentiation in various sites. It is particularly associated with the tongue in the head and neck region. The lack of any pathological features means it is difficult to make a conclusive diagnosis of LGMS. The immunohistochemical features and genomic rearrangements, including SS18-SSXs and MYH9-USP6s and the genetic mutations of cancer-associated genes, including APC, CTNNB1, EGFR, KRAS, PIK3CA and p53 were examined in a case of LGMS arising in the tip of the tongue. Immunohistochemically, the tumor cells were positive for alpha-smooth muscle actin and vimentin, as in previous reports. They demonstrated neither genomic rearrangements nor point mutations of cancer-associated genes. Although several tumor cells demonstrated intravascular invasion, the MIB-l labeling index of the cells was the same as the original lesion. To the best of our knowledge, this is the first case report of LGMS arising in the tip of the tongue with intravascular invasion.

Evaluation of pan-TRK immunohistochemistry in infantile fibrosarcoma, lipofibromatosis-like neural tumour and histological mimics.

Infantile fibrosarcoma is characterised by intersecting fascicles of spindle cells and ETV6-NTRK3 gene fusion in most cases. Given histological overlap with other spindle-cell tumours, the diagnosis can be challenging and often requires molecular confirmation. A recently developed pan-TRK antibody shows promise for identifying tumours with NTRK fusions. The purpose of this study was to evaluate the potential diagnostic utility of pan-TRK immunohistochemistry for infantile fibrosarcoma. We evaluated whole-tissue sections from 210 cases, including 15 infantile fibrosarcomas; five each lipofibromatosis-like neural tumour and lipofibromatosis; 10 each primitive myxoid mesenchymal tumour of infancy (PMMTI) and low-grade myofibroblastic sarcoma; 15 each fibrous hamartoma of infancy (FHI), myofibroma/myofibromatosis and desmoid-type fibromatosis; and 20 each low-grade fibromyxoid sarcoma, synovial sarcoma, spindle-cell rhabdomyosarcoma, malignant peripheral nerve sheath tumour, fibrosarcomatous dermatofibrosarcoma protuberans (F-DFSP) and nodular fasciitis. Immunohistochemistry was performed using a rabbit monoclonal pan-TRK antibody. Immunoreactivity for pan-TRK was observed in all 15 (100%) infantile fibrosarcomas, including diffuse immunoreactivity (>50% of cells) in 14 (93%) cases. Pan-TRK was positive in all five (100%) lipofibromatosis-like neural tumours. Of the 190 histological mimics, diffuse pan-TRK immunoreactivity was noted in 16 (8%) cases, including five PMMTI, five FHI (highlighting predominantly the primitive myxoid spindle-cell components), three F-DFSP, one low-grade myofibroblastic sarcoma, one myofibroma and one spindle-cell rhabdomyosarcoma. Diffuse pan-TRK immunoreactivity is a highly sensitive but not entirely specific diagnostic marker for infantile fibrosarcoma, and may be helpful in selecting patients for TRK-targeted therapy. As expected, lipofibromatosis-like neural tumours, which harbour NTRK1 fusions, also show diffuse pan-TRK immunoreactivity.

Low Grade Myofibroblastic Sarcoma of the Tongue: A Case Report in an Infant

A case of a 12-month-old male infant, who presented a painless swelling of the right anterolateral edge of the mobile tongue, is reported. The treatment consisted of a partial glossectomy with total surgical excision of the tumor. The diagnosis of low grade myofibroblastic sarcoma on the histological arguments (tumor architecture, cytology, mitotic index) and immunohistochemistry was retained.

Indolent growth of low-grade myofibroblastic sarcoma of the cheek mimics benign lesions: A case report and literature review.

Low-grade myofibroblastic sarcoma (LGMS) is a neoplasm of the soft tissue characterized by myofibroblastic differentiation. This type of tumor has been observed in various sites in the whole body, but frequently occurs in the head and neck region. It typically presents as a slow-growing painless mass, which is often mistaken for a benign lesion due to its indolent growth; however, LGMS is a malignant neoplasm. In the present study, a 43-year-old female presented with a 14-mm LGMS lesion in the buccal subcutaneous tissues of the buccinator muscle. The patient had initially noticed the lesion 2-months prior to presenting at the hospital. Following biopsy, the tumor was surgically resected and no recurrence or metastasis was observed during a follow-up time of 2 years. To the best of our knowledge, this case is the first report of LGMS located in the buccal subcutaneous tissue of the buccinator muscle. The present study a literature review of 55 cases of this tumor type in the head and neck region was conducted, revealing that the indolent growth of these lesions may contribute to a delay in diagnosis. The average time between the onset of clinical symptoms and hospital admission is 3.9 months, and this form of tumor is frequently misdiagnosed as a benign lesion. Therefore, the present study suggests that an incisional biopsy may be performed to rule out LGMS when clinicians encounter patients with the aforementioned indolent lesions anywhere in the body. In addition, the avoidance of radiotherapy is recommended following resection of the LGMS tumor, as it may induce LGMS recurrence.

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 sarcoma: A population-based study.

Low-grade myofibroblastic sarcoma (LGMS) is a rare entity that is described as having a predilection for occurring in the head and neck region. Here we analyze its demographics, clinic-pathologic, and survival characteristics. Retrospective database analysis. A cohort from the Surveillance, Epidemiology, and End Results Program database of cases with LGMS between 2001 and 2012. There were 49 cases with a 5-year overall survival of 71.6% and disease- specific survival of 76.3%. The majority of cases were in patients <60 years old, female, and white ethnicity. The most common sites were the extremities in 40.8% of cases followed by the head and neck region with 26.5% of cases. Multivariate analysis showed that only older age was significantly associated with worse survival (P < .05). LGMS is uncommon in the United States and occurs most commonly in the extremities followed by the head and neck region, despite an existing characterization of a predilection for the head and neck region. Treatment most commonly involves surgery, but the optimal surgical extent and/or radiotherapy needs to be further investigated. 2c Laryngoscope, 127:116-121, 2017.

Pediatric Fibroblastic and Myofibroblastic Tumors: A Pictorial Review.

Pediatric fibroblastic and myofibroblastic tumors are a relatively common group of soft-tissue proliferations that are associated with a wide spectrum of clinical behavior. These tumors have been divided into the following categories on the basis of their biologic behavior: benign (eg, myositis ossificans, myofibroma, fibromatosis colli), intermediate-locally aggressive (eg, lipofibromatosis, desmoid fibroma), intermediate-rarely metastasizing (eg, inflammatory myofibroblastic tumors, infantile fibrosarcoma, low-grade myofibroblastic sarcoma), and malignant (eg, fibromyxoid sarcoma, adult fibrosarcoma). Imaging has a key role in the evaluation of lesion origin, extent, and involvement with adjacent structures, and in the treatment management and postresection surveillance of these tumors. The imaging findings of these tumors are often nonspecific. However, certain imaging features, such as low or intermediate signal intensity on T2-weighted magnetic resonance images and extension along fascial planes, support the diagnosis of a fibroblastic or myofibroblastic tumor. In addition, certain tumors have characteristic imaging findings (eg, multiple subcutaneous or intramuscular lesions in infantile myofibromatosis, plaquelike growth pattern of Gardner fibroma, presence of adipose tissue in lipofibromatosis) or characteristic clinical manifestations (eg, great toe malformations in fibrodysplasia ossificans fibroma, neonatal torticollis in fibromatosis colli) that suggest the correct diagnosis. Knowledge of the syndrome associations of some of these tumors-for example, the association between familial adenomatous polyposis syndrome and both Gardner fibroma and desmoid fibromatosis, and that between nevoid basal cell carcinoma syndrome and cardiac fibroma-further facilitate a diagnosis. The recognition of key imaging findings can help guide treatment management and help avoid unnecessary intervention in cases of benign lesions such as myositis ossificans and fibromatosis colli. In this article, we describe the various types of fibroblastic and myofibroblastic tumors in children and the characteristic clinical manifestations, imaging features, and growth patterns of these neoplasms-all of which aid in the appropriate radiologic assessment and management of these lesions. (©)RSNA, 2016.

Low-grade myofibroblastic sarcoma of the larynx: A case report

Low-grade myofibroblastic sarcoma is a rare neoplasm, with an incidence of less than 1% and a predilection for the head and neck area.We report a case occurring in a 55 year-old male and in an unusual location: the anterior commissure of the larynx. The patient presented with short-term dysphonia. A nodular lesion on the right vocal cord was detected and a biopsy and subsequent resection of the mass was performed. The nodule consisted of a spindle cell fascicular proliferation in a dense collagenous matrix. Moderate atypia, mitotic activity (>10X10HPF) and a proliferation index of 10% were noted. Immunohistochemistry showed positivity for vimentin, muscle-specific actin (HHF35) and negativity fordesmin, ALK, p53, cytokeratins (AE1/AE3, 5/6 and 18), EMA, GFAP, S100, CD34, CD68 and CD99.

Pancreatic localization of a low-grade myofibroblastic sarcoma. A case report

Pancreatic localization of a low-grade myofibroblastic sarcoma. A case report

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.

In myofibroblastic sarcomas of the head and neck, mitotic activity and necrosis define grade: a case study and literature review

Low-grade myofibroblastic sarcoma (LGMFS) is considered a distinct entity in the World Health Organization classification of soft tissue neoplasms, defined as an atypical myofibroblastic proliferation with fibromatosis-like features and a predilection for the head and neck. A substantial subset of previously reported myofibroblastic sarcomas (MFS), particularly in the head and neck region, are associated with appreciable tumor-associated morbidity and mortality and should be differentiated from the more indolent LGMFS. However, no specific morphological criteria have been developed to define the entity of LGMFS. We have reviewed histological findings in conjunction with clinical follow-up information of previously reported MFS in the head and neck region in the English literature, with the addition of five new cases from our institution. We found that MFSs with 6 or more mitoses per 10 high power fields and/or presence of spontaneous necrosis were accompanied by a higher mortality rate that is statistically significant.

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.

Low-grade myofibroblastic sarcoma of the larynx: A rare entity with review of literature

Mesenchymal neoplasms of the larynx are rare and make up approximately 0.3% to 1.0% of all malignancies at this location. Low grade myofibroblastic sarcoma (LGMS) of larynx is a rare entity. We describe a rare case of LGMS of larynx who presented with complaint of hoarseness of voice. The patient was treated with total laryngectomy plus partial pharyngectomy followed by post-operative radiotherapy. Histopathologically, the lesion was composed of spindle cells that manifested variable cellular anaplasia and expressed smooth muscle actin (SMA) and focally S-100.One of the resection limits was involved so patient was given post-operative radiotherapy. The patient is alive and disease free 14 months after surgery. The characteristic clinical, histopathological features and treatment of this case are described with a literature review.

Low-grade myofibroblastic sarcoma of the distal femur

INTRODUCTIONLow-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 CASEA 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. A biopsy specimen from the intraosseous lesion showed a hypocellular area of spindle cell proliferation with dense collagen deposition, which is reminiscent of a histological feature of desmoplastic fibroma. However, histological examination of the extraosseous lesion indicated a slightly hypercellular area containing scattered spindle-shaped atypical cells with enlarged nuclei, suggestive of low-grade sarcoma. Spindle-shaped atypical cells were immunohistochemically positive for SMA. A final diagnosis of low-grade myofibroiblastic sarcoma of the bone was made from a surgically resected specimen. DISCUSSIONThe patient was alive and well with no evidence of disease at 15 months after the surgery without any additional therapy. CONCLUSIONExtensive sampling of a tumor may be necessary to determine the true nature of the tumor and to make an accurate diagnosis.

Low-grade myofibroblastic sarcoma of the palate

Low-grade myofibroblastic sarcoma (LGMS) is a rare, malignant tumor with myofibroblastic differentiation. Despite it being classified as a distinct entity by the World Health Organization, a few cases were reported in the oral and maxillofacial region. Here, a LGMS developed on the palate of a 73-year-old man who presented with a 1-cm tumor on the posterior border of the palate. Based on the histological and immunohistochemical features, a diagnosis of LGMS was established. The tumor was resected, and no recurrence was observed over 2 years. Although the tongue is the most preferred site for LGMS, it may occur in any region of the oral cavity.