Short-term comparative results of segmental resection versus radical resection in two patients with myxofibrosarcoma of the limbs - case series

AbstractIntroduction: Myxofibrosarcoma is a rare subtype of soft tissue sarcoma with a locally infiltrative behavior and ability to determine distant metastases.Materials and methods: We presented two myxofibrosarcoma cases who benefited from segmental or radical resection.Management and outcome: In the case of the 80-year-old woman, with grade 3 myxofibrosarcoma, we practiced radical surgery with scapulohumeral disarticulation followed by adjuvant radiotherapy.The therapeutic option for the 77-year-old man with grade 2 myxofibrosarcoma was segmental resection followed by adjuvant radiotherapy. After three months, the patient was in a good clinical condition with no sign of local recurrence, but with the presence of pulmonary metastases for the patient who benefited of segmental resection.Discussion: The radical resection had better short-term results, with no local or distant metastases at three months after surgery, although the tumor had a higher grade (G3), compared to segmental resection practiced for a lower grade tumor (G2), in which case the patient developed pulmonary metastasis at three months follow up.Conclusion: Myxofibrosarcoma represents a challenging situation regarding the management due to its unpredictive clinical course. Our cases raised the following question: should we consider treating it more aggressively in order to obtain good local control and reduce the risk of metastasis?

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The radiation therapy options of intracranial hemangiopericytoma: An overview and update on a rare vascular mesenchymal tumor

Oncology Reviews ◽

10.4081/oncol.2018.354 ◽

2018 ◽

Cited By ~ 2

Author(s):

Maria Paola Ciliberti ◽

Rosa D'Agostino ◽

Laura Gabrieli ◽

Anna Nikolaou ◽

Angela Sardaro

Keyword(s):

Surgical Resection ◽

Adjuvant Radiotherapy ◽

Therapeutic Option ◽

Treatment Options ◽

Distant Metastases ◽

Complete Resection ◽

Complete Surgical Resection ◽

Hypervascular Tumor ◽

The Central Nervous System ◽

Radiotherapy Treatment

Hemangiopericytoma (HPC) is an extremely rare hypervascular tumor of mesenchymal lineage. It tends to recur and to develop distant metastases even many years after primary surgical resection. The management of recurrent and metastatic disease is not always so well defined. A complete surgical resection does not eliminate the high risk of local recurrences that occur in the central nervous system, often in the same surgical bed. However, treatment with adjuvant radiotherapy even in cases of complete resection remains controversial. Because of its rarity, there is no standard for treatment. We focused on radiotherapy treatment options, analyzing the literature and making a base on conduct further studies to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease and to help specialists to take the most appropriate therapeutic option.

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Pathology-proven extradural ('distant') metastases of gliomas in adults in the Netherlands between 1971-2018: a systematic case series

Neuro-Oncology Practice ◽

10.1093/nop/npab006 ◽

2021 ◽

Author(s):

Sanne J den Hartog ◽

Anja van der Kolk ◽

Annette Bruggink ◽

Tatjana Seute ◽

Pieter Wesseling ◽

...

Keyword(s):

The Netherlands ◽

Lymph Nodes ◽

Survival Data ◽

Case Series ◽

Distant Metastases ◽

Lower Grade ◽

Diffuse Glioma ◽

Primary Tumors ◽

Paraffin Block ◽

Diffuse Gliomas

Abstract Background Diffuse gliomas are the most frequent primary tumors originating in the central nervous system parenchyma. Although the majority of these tumors are highly malignant, extradural metastases (EDM) are extremely rare. We aimed to perform a systematic review of patients with pathology-proven EDM of diffuse gliomas in the Netherlands. Methods From the Nationwide Network and Registry of Histo- and Cytopathology in the Netherlands information on all cases with EDM between 1971 and October 2018 was retrieved. Patients aged < 18 years or with a diagnosis of ependymoma or continuous tumor growth from intra- to extradural were excluded. Demographics, initial tumor diagnosis, treatment characteristics, location of the EDM and survival data were collected. IDH1 R132H immunohistochemistry was performed on cases of which a paraffin block of the metastatic tumor could be retrieved. Results Twenty-five patients with diffuse glioma and pathology-proven EDM were identified. Median age at diagnosis of glioma was 46 years (IQR;35-59); 21 patients (84%) were male. Histopathologic diagnosis was glioblastoma in 17 patients (68%) and lower grade tumor in eight patients. In three out of 12 patients of which a paraffin block could be retrieved immunohistochemistry revealed an IDH1-mutant glioma. Most frequent EDM locations were bone/bone marrow (14/25 patients;56%), and lymph nodes (6/25 patients;24%). Conclusion EDM of diffuse glioma are rare. They occur most frequently in patients with glioblastoma, however they can also originate from lower grade, IDH-mutant gliomas. In daily practice, EDM of diffuse glioma should be considered in patients with tumefactive lesions of the bone or lymph nodes.

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Golimumab as Rescue Therapy for Refractory Immune-Mediated Uveitis: A Three-Center Experience

Mediators of Inflammation ◽

10.1155/2014/717598 ◽

2014 ◽

Vol 2014 ◽

pp. 1-5 ◽

Cited By ~ 28

Author(s):

Miguel Cordero-Coma ◽

Vanesa Calvo-Río ◽

Alfredo Adán ◽

Ricardo Blanco ◽

Carolina Álvarez-Castro ◽

...

Keyword(s):

Retinal Thickness ◽

Rescue Therapy ◽

Therapeutic Option ◽

Case Series ◽

Tertiary Referral ◽

Short Term ◽

Complete Control ◽

Safety And Efficacy ◽

Immune Mediated ◽

Different Types

Objective. To evaluate, in three Spanish tertiary referral centres, the short-term safety and efficacy of golimumab (GLM) for treatment of immune-mediated uveitis resistant to previous immunosuppressive therapy.Methods. Nonrandomized retrospective interventional case series. Thirteen patients with different types of uveitis that were resistant to treatment with at least 2 previous immunosuppressors were included in this study. All included patients were treated with GLM (50 mg every four weeks) during at least 6 months. Clinical evaluation and treatment-related side effects were assessed at least four times in all included patients.Results. Eight men and 5 women (22 affected eyes) with a median age of 30 years (range 20–38) and active immune-mediated uveitides were studied. GLM was used in combination with conventional immunosuppressors in 7 patients (53.8%). GLM therapy achieved complete control of inflammation in 12/13 patients (92.3%) after six months of treatment. There was a statistically significant improvement in mean BCVA (0.60 versus 0.68,P=0.009) and mean 1 mm central retinal thickness (317 versus 261.2 μ,P=0.05) at the six-month endpoint when compared to basal values. No major systemic adverse effects associated with GLM therapy were observed.Conclusions. GLM is a new and promising therapeutic option for patients with severe and refractory uveitis.

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Gemcitabine-Containing Chemotherapy for the Treatment of Metastatic Myxofibrosarcoma Refractory to Doxorubicin: A Case Series

Current Oncology ◽

10.3390/curroncol28010078 ◽

2021 ◽

Vol 28 (1) ◽

pp. 813-817

Author(s):

Arielle Elkrief ◽

Suzanne Kazandjian ◽

Thierry Alcindor

Keyword(s):

Overall Survival ◽

Soft Tissue ◽

Soft Tissue Sarcoma ◽

Objective Response ◽

Case Series ◽

Progression Free Survival ◽

Distant Metastases ◽

First Line ◽

Pleomorphic Sarcoma ◽

Line Setting

Background: Myxofibrosarcoma is a type of soft-tissue sarcoma that is associated with high rates of local recurrence and distant metastases. The first-line treatment for metastatic soft-tissue sarcoma has conventionally been doxorubicin-based. Recent evidence suggests that myxofibrosarcoma may be molecularly similar to undifferentiated pleomorphic sarcoma (UPS), which is particularly sensitive to gemcitabine-based therapy. The goal of this study was to evaluate the activity of gemcitabine-containing regimens for the treatment of metastatic myxofibrosarcoma refractory to doxorubicin. Material and Methods: We retrospectively evaluated seven consecutive cases of metastatic myxofibrosarcoma at our institution treated with gemcitabine-based therapy in the second-line setting, after progression on doxorubicin. Baseline clinical and baseline characteristics were collected. Primary endpoints were objective response rate (ORR), progression-free survival (PFS) and overall survival (OS). Results: After progression on first-line doxorubicin, a partial, or complete radiological response was observed in four of seven patients who received gemcitabine-based chemotherapy. With a median follow-up of 14 months, median progression-free and overall survival were 8.5 months and 11.4 months, respectively. Conclusions: Gemcitabine-based chemotherapy was associated with encouraging response rates in this cohort, similar to those seen in UPS. Both entities could be studied together for novel gemcitabine-based regimens.

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Assessment of the value of adjuvant radiotherapy for treatment of gastric adenocarcinoma based on pattern of post-surgical progression

World Journal of Surgical Oncology ◽

10.1186/s12957-021-02304-4 ◽

2021 ◽

Vol 19 (1) ◽

Author(s):

Peng Wang ◽

Haihua Zhou ◽

Gaohua Han ◽

Qingtao Ni ◽

Shengbin Dai ◽

...

Keyword(s):

Local Recurrence ◽

Gastric Adenocarcinoma ◽

Adjuvant Radiotherapy ◽

Cox Regression ◽

Radical Resection ◽

Progression Rate ◽

Cox Regression Analysis ◽

T Stage ◽

Local Progression ◽

Degree Of Differentiation

Abstract Purpose To assess the value of adjuvant radiotherapy for treatment of gastric adenocarcinoma and to investigate subgroups of patients suitable for adjuvant radiotherapy. Methods and materials Data from 785 patients with gastric adenocarcinoma who had undergone D1/D2 radical resection and adjuvant chemotherapy were collected, the site of first progression was determined, and the relationship between the rate of local recurrence and clinicopathologic features was analyzed. Results By the end of the follow-up period, progression was observed in 405 patients. Local recurrence was observed as the first progression in 161 cases. The local recurrence rate was significantly lower than the non-local progression rate (20.5% vs 31.5%, p=0.007). Multivariate Cox regression analysis showed a significant relationship among degree of differentiation, T stage, N stage, and rate of local recurrence. Conclusions Not all patients with gastric carcinoma required adjuvant radiotherapy. However, patients with poorly differentiated cancer cells, advanced T stage (T3/T4), and positive lymph nodes, which included patients in the T4N1-2M0 subgroup, were recommended for adjuvant radiotherapy.

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Use of MitraClip system for severe mitral regurgitation in cardiogenic shock: results from a multicentre observational Italian experience (the MITRA-SHOCK study)

European Heart Journal ◽

10.1093/ehjci/ehaa946.2635 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

G Falasconi ◽

L Pannone ◽

F Melillo ◽

M Adamo ◽

F Ronco ◽

...

Keyword(s):

Mitral Regurgitation ◽

Hospital Mortality ◽

Cardiogenic Shock ◽

Therapeutic Option ◽

Strong Predictor ◽

Medical Emergency ◽

Short Term ◽

Italian Experience ◽

Procedural Success ◽

Mitraclip System

Abstract Background/Introduction Cardiogenic shock (CS) is a medical emergency and a frequent cause of death. CS can be complicated by mitral regurgitation (MR). The presence of at least moderate MR in the setting of shock was associated with about three-times higher odds of 1-year mortality. In the setting of refractory CS, percutaneous mitral valve repair (PMVR) can be a potential therapeutic option. Purpose The aim of the study was to evaluate the efficacy of percutaneous approach of severe MR in patients with CS assessing short-term clinical outcomes. Methods In this study we retrospectively included patients with CS and concomitant severe MR treated with Mitraclip system. We enrolled 28 patients from 5 Italian centers between 2012 and 2019. MitraClip implantation was performed according to each hospital standard care. CS was defined utilizing the Diagnostic Criteria of Cardiogenic Shock used in the SHOCK trial. Procedural success was defined as the presence of moderate or less MR after MitraClip implantation. Results All patients presented at least severe MR. All treated patients were at high surgical risk (STS mortality score 36.4±11.7%). Procedural success was obtained in 24 patients (86%). A mean of 1.71±0.76 clips per patients were implanted. In-hospital complications occurred in 13 patients (46%): 7 minor bleedings (25% of patients), 7 major bleedings (25%), 8 acute kidney injuries (28%). In-hospital mortality was 25% and the reported causes of death were cardiovascular in all patients. At Cox multivariate analysis procedural success was a strong predictor of in-hospital survival (HR 0.11, CI 95% 0.02–0.67, p=0.017). Conclusions PMVR with Mitraclip system in patients with CS and concomitant MR demonstrated high procedural success and acceptable safety. It can be considered a bailout option in this setting of patients with high short-term mortality. Larger prospective studies are needed. In-hospital mortality predictors Funding Acknowledgement Type of funding source: None

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Long-term outcome of children undergoing surgery for suspected perilymph fistula

The Journal of Laryngology & Otology ◽

10.1017/s0022215108003046 ◽

2008 ◽

Vol 123 (3) ◽

pp. 298-302 ◽

Cited By ~ 2

Author(s):

R J Sim ◽

A H Jardine ◽

E J Beckenham

Keyword(s):

Hearing Loss ◽

Case Series ◽

Outcome Data ◽

Rank Test ◽

Short Term ◽

Term Outcome ◽

Long Term Outcome ◽

Signed Rank ◽

Signed Rank Test

AbstractA number of authors have suggested that surgery for suspected perilymph fistula is effective in preventing deterioration of hearing and in improving hearing in some cases in the short term. We present long-term hearing outcome data from 35 children who underwent exploration for presumed perilymph fistula at The Children's Hospital, Sydney, Australia, between 1985 and 1992.Methods:The pre-operative audiological data (mean of 500, 1000, 2000 and 4000 Hz results) were compared with the most recently available data (range two to 15 years) and the six-month post-operative data.Results:The short-term results showed no significant change in hearing at six months, with a subsequent, statistically significant progression of hearing loss in both operated and non-operated ears (Wilcoxon signed rank test: operated ear, p < 0.017; non-operated ear, p < 0.009).Conclusion:In this case series, exploratory surgery for correction of suspected perilymph fistula did not prevent progression of long-term hearing loss.

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Calcaneal Reconstruction With Free Deep Circumflex Iliac Artery Osseocutaneous Flap Following Aggressive Benign Bone Tumor Resection

Foot & Ankle International ◽

10.1177/10711007211025280 ◽

2021 ◽

pp. 107110072110252

Author(s):

Mohamad Aizat Rosli ◽

Wan Faisham Wan Ismail ◽

Wan Azman Wan Sulaiman ◽

Nor Azman Mat Zin ◽

Syurahbil Abdul Halim ◽

...

Keyword(s):

Iliac Artery ◽

Donor Site ◽

Tumor Resection ◽

Case Series ◽

Benign Tumors ◽

Reconstruction Method ◽

Short Term ◽

Deep Circumflex Iliac Artery ◽

First Case ◽

Donor Site Pain

Background: Advances in limb-salvage techniques have made total calcanectomy and primary reconstruction possible in managing calcaneal aggressive benign tumors and selected cases of intraosseous malignant tumors. However, there is still no consensus on the operative approach, oncologic margin, and the best reconstruction method to date. These 2 cases describe our experience in calcaneal reconstruction with the free deep circumflex iliac artery (DCIA) osseocutaneous flap in benign aggressive calcaneal tumors. Methods: We reported 2 consecutive male and female patients, with an average age of 25 years (age 19 and 31, respectively), who underwent total calcanectomy and primary calcaneal reconstruction with the free DCIA osseocutaneous flaps for calcaneal chondroblastoma and giant cell tumor. A marginal resection of the entire calcaneus through the subtalar and calcaneocuboid joints (intra-articular approach) was performed in the first case and a wide local resection leaving 1 cm normal calcaneal bone margin anterosuperiorly (intraosseous approach) was performed in the second case. Results: The follow-up period averaged 48 months. Negative oncologic margins were achieved in both cases. The first case was complicated with venous thrombosis; however, the graft remained viable after emergency reexploration. Normal foot function was restored with good solid osseous union and bony hypertrophy observed. Both patients achieved good short-term functional and aesthetic outcomes with no donor site pain or disability. No local recurrence was reported either. Conclusion: Primary calcaneal reconstruction with the free DCIA osseocutaneous flap can lead to good short-term functional and aesthetic outcomes. Level of Evidence: Level IV, case series.

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