scholarly journals Myofibroblastic sarcoma of the bone: two cases

2021 ◽  
Vol 9 (5) ◽  
Author(s):  
Esperanza moreno ◽  
Eduardo Ortiz-Cruz ◽  
José Juan Pozo-Kreilinger ◽  
Manuel Peleteiro-Pensado
Keyword(s):  
Head And Neck ◽  
Soft Tissues ◽  
Treatment Options ◽  
Bone Sarcoma ◽  
Initial Diagnosis ◽  
Low Grade ◽  
Histological Findings ◽  
Rare Tumour ◽  
Myofibroblastic Sarcoma

Low-grade myofibroblastic sarcoma (L-G MFS) is a rare tumour that commonly affects the soft tissues of the head and neck. Its location in bone is very rare and there are few cases published in the literature, which sometimes leads to a wrong initial diagnosis. The following report will give an overview of the clinical, radiological and histological findings in two patients with a very unusual bone sarcoma, which are more often found in the soft tissues. Treatment options and outcome will be discussed.

scholarly journals The chemotherapy and radiation in low-grade myofibroblastic sarcoma: is there a role?

2020 ◽  
Author(s):  
Yao Xu ◽  
Guijun Xu ◽  
Xin Wang ◽  
Min Mao ◽  
Haixiao Wu ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Surgical Treatment ◽  
Head And Neck ◽  
Radiation Treatment ◽  
Treatment Strategies ◽  
Neck Region ◽  
Low Grade ◽  
Myofibroblastic Sarcoma ◽  
Kaplan Meier ◽  
Cox Proportional Hazard Regression

Abstract Background: Low-grade myofibroblastic sarcoma (LGMS) is a rare entity with a predilection in the head and neck. There are still no optimal treatment strategies for LGMS. We aimed to investigate the role of chemotherapy and radiation treatment for LGMS. Survival estimate was performed and prognostic factors were identified.Methods: Based on the Surveillance, Epidemiology, and End Result (SEER) database, LGMS patients diagnosed between 2001 and 2015 were involved in our study. Kaplan-Meier curves and log-rank tests were used to estimate overall survival. Cox proportional hazard regression model was performed to identify prognostic factors.Results: A total of 96 eligible patients with LGMS were included, among which 86 (89.6%) received surgical treatment. Twenty-eight (29.2%) patients received radiation treatment while chemotherapy was offered to 20 (10.4%) patients. The median age was 55.0 years old with 22 cases occurred in head and neck region. The mean OS was 125.2 (95%CI 106.3-144.2) months while 1-, 3-, 5- and 10-year OS rates were 88%, 77%, 70% and 59%, respectively. Age older than 60 years, positive nodal status and no surgical treatment were independent prognostic factors for patients with LGMS. Chemotherapy and radiation were not independent prognostic factors for LGMS.Conclusions: Several prognostic factors for LGMS were revealed in this study. Surgical resection is the main therapy while chemotherapy and radiation showed limited effects on survival improvement. Thus, chemotherapy and/or radiation should not be routinely performed in LGMS.

scholarly journals LOW-GRADE MYOFIBROBLASTIC SARCOMA OF THE LARYNX: CASE REPORT AND REVIEW OF LITERATURE

2014 ◽  
Vol 57 (4) ◽  
pp. 162-164 ◽  
Author(s):  
Petr Kordač ◽  
Dimitar Hadži Nikolov ◽  
Katarína Smatanová ◽  
David Kalfeřt
Keyword(s):  
Case Report ◽  
Head And Neck ◽  
Male Patient ◽  
Neck Region ◽  
Clinicopathological Characteristics ◽  
Low Grade ◽  
Review Of Literature ◽  
Head And Neck Region ◽  
Myofibroblastic Sarcoma ◽  
Male Predominance

Low-grade myofibroblastic sarcoma (LGMS) is a very rare, atypical myofibroblastic tumor with fibromatosis-like features with predilection mostly in head and neck region. LGMS occurs primarily in adult patients with a slight male predominance. Only few cases of LGMS affecting the larynx have been reported in literature to this date. We describe a case of low-grade myofibroblastic sarcoma of the larynx in a 40-year-old male patient. The clinicopathological characteristics, immunohistochemical findings and treatment are discussed.

scholarly journals CYTO HISTOLOGIC CORRELATION OF A LOW GRADE FIBROMYXOID SARCOMAAT AN UNUSUAL LOCATION – A CASE REPORT

2021 ◽  
pp. 1-3
Author(s):  
Pooja Jain ◽  
Annu Nanda ◽  
Deepak Kumar Singh ◽  
Udita Singhal
Keyword(s):  
Soft Tissues ◽  
Correct Diagnosis ◽  
Subcutaneous Fat ◽  
Needle Aspiration ◽  
Low Grade ◽  
Difficult Case ◽  
Nuclear Pleomorphism ◽  
Rare Tumour ◽  
Spindle Cells ◽  
High Local Recurrence Rate

BACKGROUND Low grade fibromyxoid sarcoma (LGFMS) is a rare tumour of the deep soft tissues seen in young adults. The histologic features of LGFMS are well established, however the cytologic findings are scantily defined and the distinction from other benign and malignant myxoid soft tissue tumours is difficult. CASE DETAILS A 29 year old male presented with a superficial swelling in the left areolar region for two months. USG revealed a well-defined 2 by 2 cm hyperechoic lesion extending upto the subcutaneous fat. FNA yielded mucoid material and smears showed spindle cells having round to elongated nuclei with mild anisokaryosis, bland chromatin and wispy cytoplasm in a myxoid background. No significant nuclear pleomorphism or mitoses were noted. A diagnosis of myxoid mesenchymal lesion was made. The excised tumour was well circumscribed with grey white cut surface showing myxoid areas. Microscopically, the tumour displayed moderate cellularity with storiform, intersecting and parallel bundles of spindle cells showing mild nuclear pleomorphism. The myxoid areas showed cells in a haphazard fashion floating in abundant mucoid matrix associated with a capillary network. The mitotic index was low. IHC showed tumor cells positive for vimentin and negative for S-100 & SMA. CONCLUSION LGFMS is a rare low-grade malignancy with a high local recurrence rate and late metastasis. The diagnosis of LGFMS can be difficult to render from fine needle aspiration cytology (FNAC) alone because of morphological overlap with other spindle cell and myxoid lesions. Cytohistological correlation and IHC is helpful in arriving at a correct diagnosis.

scholarly journals Mesenchymal Chondrosarcoma of the Parotid Gland

2020 ◽  
Vol 26 (2) ◽  
pp. 158-160
Author(s):  
MA Matin ◽  
Md Abdullah Al Harun ◽  
Mohammad Saiful Islam ◽  
AKM Shaif Uddin ◽  
MA Sabur
Keyword(s):  
Parotid Gland ◽  
Head And Neck ◽  
Rare Case ◽  
Soft Tissues ◽  
Clinical Presentation ◽  
Mesenchymal Chondrosarcoma ◽  
Rare Tumour ◽  
Rare Tumours ◽  
Myxoid Matrix ◽  
Mesenchymal Chondrosarcomas

Mesenchymal Chondrosarcomas of the parotid are extremely rare tumours. They are broadly classified under extra skeletal form of mesenchymal chondrosarcomas and account for less than 1% of all sarcomas. It is characterized by a multinodular architecture, abundant myxoid matrix, and malignant chondroblast like cells arranged in cords. The tumor is an entity from chondrosarcomas of bones, commonly found in the soft tissues of the lower extremities at 80%. There are very limited reports of this tumor in the head and neck, especially in the parotid gland. We report a rare case of a parotid mesenchymal chondrosarcoma in a 55 years old man and discuss the pathogenesis, clinical presentation and management of such a rare tumour Bangladesh J Otorhinolaryngol; October 2020; 26(2): 158-160

scholarly journals Low-grade myofibroblastic sarcoma of retroperitoneum: a rare case

2019 ◽  
Vol 6 (2) ◽  
pp. 633
Author(s):  
Suresh K. Choudhary ◽  
Shalu Gupta ◽  
Somendra Bansal ◽  
Narender Kumar
Keyword(s):  
Splenic Flexure ◽  
Soft Tissues ◽  
Imaging Modality ◽  
Smooth Muscle Actin ◽  
Abdominal Cavity ◽  
Neck Region ◽  
Low Grade ◽  
Tissue Mass ◽  
Myofibroblastic Sarcoma

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.

scholarly journals Pediatric Chondrosarcoma of the Sternum Resected with Thorascopic Assistance

2017 ◽  
Vol 11 (1) ◽  
pp. 479-485 ◽  
Author(s):  
Harpreet S. Bawa ◽  
Drew D. Moore ◽  
Juan C. Pelayo ◽  
Nicole Cipriani ◽  
Grace Mak ◽  
...  
Keyword(s):  
Chest Wall ◽  
Soft Tissues ◽  
Thoracoscopic Surgery ◽  
Bone Sarcoma ◽  
Wide Resection ◽  
Malignant Neoplasms ◽  
Low Grade ◽  
Orthopaedic Oncology ◽  
Primary Bone Sarcoma ◽  
Primary Bone

Background: Chondrosarcomas are a heterogeneous group of malignant neoplasms that arise from bones, cartilage or other soft tissues that produce cartilage and are commonly seen in the middle decades of life. Despite being the most common primary bone sarcoma in adults, chondrosacromas are rare in pediatric patients. Case Report: We report the case of a six-year-old child with a painless enlarging sternal mass of which biopsy was consistent with low-grade surface chondrosarcoma. This is the first reported case of a chest wall chondrosarcoma in a young child. This unusual location in a young patient presented challenges to treatment. Resection of the manubrium was performed by a multidisciplinary team of orthopaedic oncology and pediatric general surgery. The patient underwent a wide resection of the sternal mass from an anterior approach performed by the orthopaedic oncology team using an oscillating saw under video-assisted thoracoscopic surgery to ensure adequate mass resection without injury to nearby structures. The patient was followed with quarterly physical exams and radiographs for 18 months postoperatively and did not have any pain or evidence of recurrence. Conclusion: Clinicians should consider utilizing multidisciplinary approaches to treat patients with chondrosarcomas of the chest wall.

Head and Neck Cancer with Lung Metastases: Treatment Challenges

2019 ◽  
Vol 6 (1) ◽  
pp. 48-53
Author(s):  
Selvamalar Vengathajalam ◽  
Norhafiza Mat Lazim
Keyword(s):  
Head And Neck Cancer ◽  
Head And Neck ◽  
Neck Cancer ◽  
Lung Metastases ◽  
Case Reports ◽  
Primary Tumour ◽  
Treatment Options ◽  
Case Series ◽  
Low Grade ◽  
First Case

Background:Head and neck cancer has predilection of metastasising to the lung, bones or liver. The site of metastasis usually depends on the primary tumour location, the staging and the regional spread of the tumour. Patients with distant metastasis are predicted to have a poor prognosis with low survival rate. Oligometastasis is the term used for an intermediate biologic state of restricted metastatic capacity with limited number and sites of organ with metastasis. It is also defined by 5 or less than 5 metastatic lesion in a disease with a controlled primary tumour.Case Reports:In this case series, we have reported three cases of head and neck carcinomas that pose treatment dilemmas because of lung metastases. First case is a gentleman with laryngeal carcinoma with multiple small lung metastases where the treatment options of surgery versus chemoradiation was debated. The second case is a gentleman with low grade mucoepidermoid carcinoma of the parotid gland with suspicious lung spread of disease. Lastly is a patient with papillary thyroid carcinoma with florid lung metastases who completed chemoradiation.Conclusion:The presence of lung metastases does not necessarily mean that the prospect of surviving is poor for the patient. It is necessary to determine the best choice of treatment yielding the best quality of life to maximize the survival period for these patients.

Low-grade myofibroblastic sarcoma of the sacrum

2010 ◽  
Vol 6 (3) ◽  
pp. 286-290 ◽  
Author(s):  
William E. Humphries ◽  
Krishna B. Satyan ◽  
Katherine Relyea ◽  
Eugene S. Kim ◽  
Adekunle M. Adesina ◽  
...  
Keyword(s):  
Soft Tissues ◽  
Neck Region ◽  
Benign Tumors ◽  
Low Grade ◽  
Rare Tumor ◽  
Head And Neck Region ◽  
Myofibroblastic Sarcoma ◽  
Total Sacrectomy ◽  
Fibroblastic Cells

Myofibroblastic tumors are soft-tissue neoplasms arising from myofibroblasts, ubiquitous cells sharing ultrastructural features of muscular and fibroblastic cells. Vasudev and Harris described a malignant counterpart of these benign tumors in 1978. Most reported cases of myofibroblastic sarcoma have arisen in the head and neck region and the soft tissues of the extremities. To the best of the authors' knowledge, there have been only 8 previous reports on primary myofibroblastic sarcoma of the bone. The authors report a new case of this rare tumor affecting the sacrum and ilium of a 15-year-old girl and discuss the role of total sacrectomy and lumbopelvic reconstruction for treatment of this disease.

Immunotherapy and potential predictive biomarkers in the treatment of neuroendocrine neoplasia

2020 ◽  
Author(s):  
Guanghui Xu ◽  
Yuhao Wang ◽  
Hushan Zhang ◽  
Xueke She ◽  
Jianjun Yang
Keyword(s):  
Current Knowledge ◽  
Checkpoint Inhibitors ◽  
Treatment Options ◽  
Predictive Biomarkers ◽  
The Body ◽  
Low Grade ◽  
Ablative Therapies ◽  
Cancer Types ◽  
New Treatment ◽  
Well Differentiated

Neuroendocrine neoplasias (NENs) are a heterogeneous group of rare tumors scattered throughout the body. Surgery, locoregional or ablative therapies as well as maintenance treatments are applied in well-differentiated, low-grade NENs, whereas cytotoxic chemotherapy is usually applied in high-grade neuroendocrine carcinomas. However, treatment options for patients with advanced or metastatic NENs are limited. Immunotherapy has provided new treatment approaches for many cancer types, including neuroendocrine tumors, but predictive biomarkers of immune checkpoint inhibitors (ICIs) in the treatment of NENs have not been fully reported. By reviewing the literature and international congress abstracts, we summarize the current knowledge of ICIs, potential predicative biomarkers in the treatment of NENs, implications and efficacy of ICIs as well as biomarkers for NENs of gastroenteropancreatic system, lung NENs and Merkel cell carcinoma in clinical practice.

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