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.

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.

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.

Ultrasonography for Interval Assessment in the Nonoperative Management of Hepatic Trauma

2005 ◽  
Vol 71 (10) ◽  
pp. 841-846 ◽  
Author(s):  
William C. Chiu ◽  
Jade J. Wong-You-Cheong ◽  
Aurelio Rodriguez ◽  
K. Shanmuganathan ◽  
Stuart E. Mirvis ◽  
...  
Keyword(s):  
Imaging Modality ◽  
Operative Management ◽  
Biliary Fistula ◽  
Low Grade ◽  
Trauma Patients ◽  
Hepatic Trauma ◽  
Abdominal Ultrasonography ◽  
Radiologic Study ◽  
Grade Ii

Abdominal ultrasonography (US) is gaining widespread acceptance as a valuable diagnostic tool in the initial evaluation of trauma victims. We investigated the utility of US as a follow-up radiologic study in nonoperative hepatic trauma. Patients with liver injury designated for non-operative management were prospectively studied over a 2-year period at our primary adult resource center for trauma. Computed tomography (CT) and radiologist-performed US were obtained at admission and at 1 week. The ability of US to detect lesions, fluid, and complications was evaluated by comparing with the corresponding CT. Twenty-five hepatic trauma patients in the study were successfully managed nonoperatively and had both initial and follow-up US and CT scans: 1 (4%) grade I, 5 (20%) grade II, 7 (28%) grade III, 7 (28%) grade IV, and 5 (20%) grade V. Four complications developed [biloma (3) and biliary fistula (1)] in 3 patients with grade IV injury and 1 with a grade II injury. Interval US appropriately detected a complication or confirmed the absence of complication in all (13/13, 100%) patients with low-grade (I–III) injury and only missed a small biloma in one patient with a grade IV injury. Interval US and CT agreement was 92 per cent for change in hemoperitoneum or parenchymal lesion. Ultrasonography is a convenient imaging modality in the evaluation of hepatic trauma. US is sufficient to detect or exclude complications in low-grade injuries. In high-grade injuries, US may be an adjunct to CT for definitive interval assessment.

scholarly journals A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

2020 ◽  
Vol 2020 ◽  
pp. 1-10
Author(s):  
Yihua Wang ◽  
Yu Wang ◽  
Rui Chen ◽  
Zhenrong Tang ◽  
Shengchun Liu
Keyword(s):  
Retrospective Analysis ◽  
Smooth Muscle Actin ◽  
Clinical Information ◽  
Dermatofibrosarcoma Protuberans ◽  
Surgical Excision ◽  
Low Grade ◽  
Pubmed Database ◽  
Storiform Pattern ◽  
Rare Malignancy

Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade fibroblastic mesenchymal tumor derived from the dermis. The aim of this retrospective analysis was to summarize the clinicopathological data from our cases and published cases to offer more evidence for the recognition of dermatofibrosarcoma protuberans (DFSP). A total of 6 breast DFSP patients who had received treatment in our hospital were retrospectively enrolled, and detailed clinicopathological data were gathered for analysis. The median age was 29.5 years (ranging from 17 to 42 years). Most cases presented a red or brown-red, mobile, well-circumscribed, protruding, breast mass (ranging from 1 to 3 cm). For histopathology, all cases (6/6) showed a storiform pattern of spindle cells that were positive for CD34 (6/6) and Vimentin (5/6) and negative for smooth muscle actin (0/6) and S-100 protein (0/6). The majority of patients (5/6) underwent wide local excision, with 2 cases treated with radiotherapy. With a median follow-up of 36 months, all 6 patients survived without recurrence or metastasis. The PubMed database was used to search for similar cases. Eventually, 36 cases were included in this review, while cases without detailed clinical information or not reported in English were excluded from the analysis. To summarize, DFSP of the breast is an extremely rare malignancy characterized by spindle tumor cells arranged in a storiform pattern and positivity for CD34. The core needle biopsy is one of the crucial methods for its preoperative diagnosis. Management of DFSP is mainly based on surgical excision. It is prone to local recurrence, so long-term follow-up is required.

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.

Low-grade fibromyxoid sarcoma of the parapharyngeal space: an unusual location

2021 ◽  
Vol 14 (5) ◽  
pp. e237083
Author(s):  
Muhammad Hammad Deewani ◽  
Muhammad Hassan Danish ◽  
Muhammad Sohail Awan ◽  
Nasir Ud Din
Keyword(s):  
Head And Neck ◽  
Parapharyngeal Space ◽  
Neck Region ◽  
Low Grade ◽  
Head And Neck Region ◽  
Myxoid Stroma ◽  
Spindle Cells ◽  
Fibromyxoid Sarcoma ◽  
Established Treatment

Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon soft-tissue malignancy. LGFMS preferentially affects trunks and extremities of young adults; however, occasional cases have been reported in different sites of head and neck region including oral cavity, larynx and oropharynx. LGFMS usually exhibit areas of collagenised and myxoid stroma with appearance of spindle cells in whorling pattern. It is a challenge to diagnose it accurately as most of the time it is misdiagnosed as benign neoplastic entity of spindle cells. There have been only few isolated cases of LGFMS reported in head and neck region and LGFMS originating from the parapharyngeal space has never been reported before. We recently experienced a case of low grade fibomyxoid sarcoma in parapharyngeal space of neck. LGFMS have the propensity to locally recur and to metastasise. Due to its rarity in head and neck region, there are no well-established treatment and follow-up guidelines.

scholarly journals Inflammatory Pseudotumor (Plasma Cell Granuloma) of the Temporal Bone

2010 ◽  
Vol 89 (7) ◽  
pp. E1-E13 ◽  
Author(s):  
Dare V. Ajibade ◽  
Iwao K. Tanaka ◽  
Kapila V. Paghda ◽  
Neena Mirani ◽  
Huey-Jen Lee ◽  
...  
Keyword(s):  
Temporal Bone ◽  
Plasma Cell ◽  
Inflammatory Pseudotumor ◽  
Soft Tissues ◽  
Plasma Cell Granuloma ◽  
Tissue Mass ◽  
Tegmen Tympani ◽  
The Right ◽  
Multiple Recurrences

We report the case of a 41-year-old man who presented with progressive right-sided ear pressure, otalgia, hearing loss, tinnitus, and intermittent otorrhea. Computed tomography and magnetic resonance imaging detected a soft-tissue mass in the right mastoid with intracranial invasion and erosion through the tegmen tympani and mastoid cortex. Histopathologic examination was consistent with an inflammatory pseudotumor (plasma cell granuloma). These lesions rarely occur in the temporal bone. When they do, they are locally destructive and can erode bone and soft tissues. Aggressive surgery is recommended as a first-line treatment, with adjunctive steroid or radiotherapy reserved for residual or refractory disease. Our patient subsequently experienced multiple recurrences, and his treatment required all of these modalities. At the most recent follow-up, he was disease-free and doing well.

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

2021 ◽  
Vol 8 (1) ◽  
pp. 1-9
Author(s):  
Primali Rukmal Jayasooriya ◽  
Chamara Athukorala ◽  
Manjula Attygalla ◽  
Balapuwaduge Ranjit Rigobert Nihal Mendis ◽  
Tommaso Lombardi
Keyword(s):  
Oral Cavity ◽  
Smooth Muscle Actin ◽  
Emerging Disease ◽  
Low Grade ◽  
Beta Catenin ◽  
Moderate Degree ◽  
Ki 67 ◽  
Myofibroblastic Sarcoma ◽  
First Case ◽  
Current Series

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.

scholarly journals Giant Low-Grade Fibromyxoid Sarcoma in the Neck

2020 ◽  
Vol 63 (9) ◽  
pp. 432-435
Author(s):  
Jong Min Park ◽  
Hye Rin Lim ◽  
Jo Heon Kim ◽  
Dong Hoon Lee
Keyword(s):  
Neck Region ◽  
Surgical Excision ◽  
Late Recurrence ◽  
Low Grade ◽  
Head And Neck Region ◽  
Long Term Follow Up ◽  
Fibromyxoid Sarcoma ◽  
Middle Aged Adults

Low-grade fibromyxoid sarcoma (LGFS) is a soft tissue tumor that rarely occurs in the head and neck region. It occurs mainly in the proximal extremities and the trunk and is prevalent in the young and middle-aged adults. In the present case, LGFS was present at an atypical location and at an unusual age. The treatment of choice for LGFS is radical wide surgical excision with a clear margin. Long-term follow-up is essential for all patients with LGFS, as it has the potential for late recurrence or metastasis.

scholarly journals Low-Grade Myofibroblastic Sarcoma (LGMS) of the Extremities – A Systematic Review and Case Report

2020 ◽  
Author(s):  
Astrid Schenker ◽  
Burkhard Lehner ◽  
Ewgenija Gutjhar ◽  
Gunhild Mechtersheimer ◽  
Leila Harhaus ◽  
...  
Keyword(s):  
Systematic Review ◽  
Local Recurrence ◽  
Distant Metastasis ◽  
Cochrane Library ◽  
Wide Resection ◽  
Clinical Aspects ◽  
Low Grade ◽  
Incomplete Resection ◽  
Myofibroblastic Sarcoma

Abstract Background: Low-grade myofibroblastic sarcoma (LGMS) is a rare subtype of soft tissue sarcoma of intermediate grade often representing fibromatosis-like features with a rare metastasing behavior. This type of tumor has a predilection for the head and neck region, but also occurs in the extremities. Confirming the diagnosis is difficult and treatment strategies have to be chosen individually.Methods: The objective of this study was to conduct a systematic review for LGMS of the extremities. The electronic databases PubMed and the Cochrane Library were searched for eligible studies. 141 abstracts were screened on PubMed, while 10 studies were identified as eligible. Cases were summarized in terms of clinical aspects, therapeutic regimen with the primary endpoint of follow-up controls regarding local recurrence or distant metastasis. In addition, we present the rare case and surgical management of a 28-year-old male patient with residual LGMS of the thumb after initial incomplete resection. Results: 33 cases of LGMS in the extremities were identified on PubMed. Cochrane library didn’t show any results. All of them were surgically resected. Only two cases of LGMS in the hand were described in literature so far. Treatment options varied from local excision to wide resection without exact definition of the safety distance. 26 cases provided follow-up information with local recurrence in 6 cases (23 %), while 4 cases (15 %) showed distant metastasis. Conclusions: Wide resection should be the surgical aim to avoid local recurrence and distant metastases. While the excision of tumors of the thumb and hand often require closer resection margins, due to the close proximity of anatomical structures, tumor-free margins are elementary even if tissue transfer from a donor site is needed for reconstruction.

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