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 ◽  
...  

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.

2014 ◽  
Vol 57 (4) ◽  
pp. 162-164 ◽  
Author(s):  
Petr Kordač ◽  
Dimitar Hadži Nikolov ◽  
Katarína Smatanová ◽  
David Kalfeřt

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.


2021 ◽  
pp. 014556132110264
Author(s):  
Hyun-Ho Kwak ◽  
Ji-Hwan Park ◽  
Sung-Dong Kim ◽  
Kyu-Sup Cho

Chondromas are benign cartilaginous tumors that frequently occur in the long bones, pelvis, sternum, ribs, and scapula. They seldom develop in the head and neck region, and there have been rare reports of them arising in the nasal septum. Although the mainstay of management is surgery, surgical treatment strategies vary depending on the size, location, and extent of the disease. Herein, we describe a case of huge chondroma originated from the anterior nasal septum, which was completely removed by endoscopic septoplasty approach thorough modified Killian incision.


Cancer ◽  
1992 ◽  
Vol 70 (1) ◽  
pp. 169-177 ◽  
Author(s):  
Luir M. Tran ◽  
Rufus Mark ◽  
Robert Meier ◽  
Thomas C. Calcaterra ◽  
Robert G. Parker

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Tomohiro Minagawa ◽  
Takeshi Yamao ◽  
Ryuta Shioya

Spindle cell hemangioendothelioma (SCH) was originally described by Weiss and Enzinger (1986) as a low-grade angiosarcoma resembling both cavernous hemangioma and Kaposi's sarcoma. Recent studies suggest that SCH is a benign neoplasm or reactive lesion accompanying a congenital or acquired vascular malformation. Most SCHs present as one or more nodules affecting the dermis or subcutis of the distal extremities. Few reports describe SCH of the head and neck region; even fewer note intramuscular SCH. Here, we describe a case of SCH involving the temporal muscle mimicking soft tissue sarcoma, who had a successful surgical treatment with a coronal approach and zygomatic osteotomy.


2010 ◽  
Vol 28 (15_suppl) ◽  
pp. e16008-e16008
Author(s):  
N. R. Khanna ◽  
A. Patil ◽  
N. A. Jambhekar ◽  
A. K. Dcruz ◽  
D. Chaukar ◽  
...  

2004 ◽  
Vol 11 (S2) ◽  
pp. S63-S63
Author(s):  
V. M. Bondar ◽  
J. D. Ellenhorn ◽  
D. Qiah ◽  
S. Berndt ◽  
L. D. Wagman ◽  
...  

2021 ◽  
Vol 16 (3) ◽  
pp. 50-57
Author(s):  
F. Sh. Kamolova ◽  
L. Yu. Grivtsova ◽  
S. M. Samborskiy ◽  
V. B. Larionova ◽  
Yu. E. Ryabukhina ◽  
...  

Background. Extramedullary plasmacytoma (EP) of soft tissues, which in most cases affects the organs of the head and neck region, is a relatively rare malignant tumor. Until now, there are no consensus approaches to the diagnosis and treatment of EP. Differentiating EP from other types of non-Hodgkin’s lymphomas is difficult. There are difficulties in the differential diagnosis of EP and carcinomas in the head and neck region. Given the rare occurrence of this nosological form, the frequency of diagnostic errors is quite high, which dictates the need for a thorough description of each head and neck EP case.Objective of the study: analysis of possible difficulties and reasons for incorrect interpretation of diagnostic data, and treatment for head and neck EP.Materials and methods. Clinical and morphoimmunological data of 97 primary patients with B-cell non-Hodgkin’s lymphomas (B-NHLs) of the head-neck region were analyzed.Results. In our cohort we identified 2 tumor cases of a plasma cell nature, which amounted to 2 % among all B-NHLs. In one case, the process was located in the nasal cavity and clinically manifested itself with nosebleeds. The second case is a lesion of the mouth floor, primarily with the ulcer formation. In the first cases, at diagnosis, the immunohistochemistry (IHC) test was performed after patient’s chemotherapy and radiation treatment, which distorted the tumor immunophenotype. In the second cases with extensive process in maxillary sinuses, a complete and very detailed IHC test was carried out; however the data did not allow for a definitive diagnosis. Difficulties apparently arose in the interpretation of CD38 expression – main marker of plasmacytic line cells, as well as due to the unusual morphology.Conclusion. The described diagnostic situations dictate the need for a comprehensive algorithm in the diagnosis of head and neck tumors. It is advisable to perform an extended morpho-immunophenotypic study of the tumor (IHC, immunocytology, flow cytometry, etc.), if a tumor of a plasma cell nature is suspected, a morpho-immunological study of the bone marrow is indicated.


2019 ◽  
Vol 6 (2) ◽  
pp. 633
Author(s):  
Suresh K. Choudhary ◽  
Shalu Gupta ◽  
Somendra Bansal ◽  
Narender Kumar

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.


2021 ◽  
Author(s):  
Yuxin Ding ◽  
Runyi Jiang ◽  
Yuhong Chen ◽  
Jing Jing ◽  
Xiaoshuang Yang ◽  
...  

Abstract Background Previous studies have reported poorer survival in head and neck melanoma (HNM) than in body melanoma (BM). Individualized tools to predict the prognosis for patients with HNM or BM remain insufficient. Objectives To compare the characteristics of HNM and BM, and to establish and validate the nomograms for predicting the 3-, 5- and 10-year survival of patients with HNM or BM. Methods We studied patients with HNM or BM from 2004 to 2015 in the Surveillance, Epidemiology, and End Results (SEER) database. The HNM group and BM group were randomly divided into training and validation cohorts. We performed the Kaplan-Meier method for survival analysis, and used multivariate Cox proportional hazards models to identify independent prognostic factors. Nomograms for HNM patients or BM patients were developed via the rms package, and were measured by the concordance index (C-index), the area under the receiver operator characteristic (ROC) curve (AUC) and calibration plots. Results Of 70605 patients acquired, 21% (n = 15071) had HNM and 79% (n = 55534) had BM. The HNM group contained more older patients, male patients, and lentigo maligna melanoma, and more frequently had thicker tumors and metastases than the BM group. The 5-year CSS and OS rates were 88.1 ± 0.3% and 74.4 ± 0.4% in the HNM group and 92.5 ± 0.1% and 85.8 ± 0.2% in the BM group, respectively. Eight independent prognostic factors (age, sex, histology, thickness, ulceration, stage, metastases, and surgery) were identified to construct nomograms for HNM patients or BM patients. The performance of the nomograms were excellent: the C-index of the CSS prediction for HNM patients and BM patients in the training cohort were 0.839 and 0.895, respectively; in the validation cohort, they were 0.848 and 0.888, respectively; the AUCs for the 3-, 5- and 10-year CSS rates of HNM were 0.871, 0.865 and 0.854 (training), and 0.881, 0.879 and 0.861 (validation), respectively; of BM, the AUCs were 0.924, 0.918 and 0.901 (training) and 0.916, 0.908 and 0.893 (validation), respectively; and the calibration plots showed great consistency. Conclusions The characteristics of HNM and BM are heterogeneous, and we constructed and validated specific nomograms as practical prognostic tools for patients with HNM or BM.


2021 ◽  
Vol 14 (5) ◽  
pp. e237083
Author(s):  
Muhammad Hammad Deewani ◽  
Muhammad Hassan Danish ◽  
Muhammad Sohail Awan ◽  
Nasir Ud Din

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.


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