scholarly journals Locally recurrent extraskeletal myxoid chondrosarcoma of the shoulder: a case of complete neoadjuvant radiotherapy response

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Luca Improta ◽  
Sergio Valeri ◽  
Rossana Alloni ◽  
Chiara Pagnoni ◽  
Francesco Mallozzi Santa Maria ◽  
...  

Abstract Background Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue tumor that typically affects the lower limbs of men between the ages of 50 and 60. EMC of the shoulder is rare with a high risk of local recurrence and distant metastasis. A planned surgical excision in sarcoma referral centers (SRCs) is mandatory to obtain the best outcome. The role of chemotherapy (CHT) and Radiotherapy (RT) on soft tissue chondrosarcoma is still controversial. Case presentation A 47-year-old man presented to our referral center with a history of EMC in the right shoulder excised with microscopic positive surgical margins in a non-referral center. Staging imaging exams did not reveal distant metastasis or residual disease, but during follow-up a local recurrence was detected. After a multidisciplinary discussion, preoperative radiotherapy was administered with a total dose of 50 Gy, and then the patient underwent wide surgical excision. Histological examination was negative for viable tumor cells. No relapse occurred in a 24-months post-operative follow up. Conclusions The case here described suggests the importance of patient’s management in SRCs. A planned combined treatments with both surgery and RT seems to be the best choice to improve local control. RT seems to be promising within this specific histotype. Further studies are needed to confirm if the observed efficacy of combined treatments reflects in a consistent survival benefit for EMC patients.

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Chiara Pagnoni ◽  
Luca Improta ◽  
Rossana Alloni ◽  
Francesco Mallozzi Santa Maria ◽  
Irene Aprile ◽  
...  

Abstract Background Myxofibrosarcoma (MFS) is a rare soft tissue sarcoma with a high recurrence rate and a low risk of distant metastasis. It occurs mainly in the extremities of elderly men. Head and neck MFS is extremely rare. Surgery is the cornerstone of treatment. The role of radiotherapy (RT) and chemotherapy (CHT) on MFS is still debated. Case presentation A 67-year-old Caucasian man presented to our sarcoma referral center (SRC) with a history of MFS of the neck excised with microscopic positive surgical margins in a non-referral center. Staging imaging exams did not reveal distant metastasis. After a multidisciplinary discussion, preoperative RT was administered with a total dose of 50 Gy followed by wide surgical excision. Histological examination was negative for viable tumor cells. No relapse occurred during the 24-month postoperative follow-up. Conclusions The case described suggests the importance of planned combined treatments with both RT and surgery for high-grade soft tissue sarcoma. RT seems to be promising within this specific histotype. Close follow-up is advisable in all cases. Further studies are needed to confirm if the observed efficacy of combined treatments results in a prolonged time of disease-free survival and overall survival.


2012 ◽  
Vol 132 (10) ◽  
pp. 1379-1386 ◽  
Author(s):  
Koichi Ogura ◽  
Tomohiro Fujiwara ◽  
Yasuo Beppu ◽  
Hirokazu Chuman ◽  
Akihiko Yoshida ◽  
...  

2003 ◽  
Vol 11 (1) ◽  
pp. 9-11
Author(s):  
Aleksandar Kiralj ◽  
Zlata Janjic ◽  
Mladen Jovanovic ◽  
Nada Vuckovic

BACKGROUND: The purpose of this study was to evaluate local recurrence of soft-tissue sarcomas as a prognostic factor reflecting adequate or inadequate excision. METHODS: We reviewed the cases of 53 patients who had soft-tissue sarcomas and were treated between 1991 and 2001. All patients were treated operatively, but 11 of them (20.75%), before being sent to us were operated elsewhere with inadequate surgical margins. The oncology status, including local recurrence and metastasis was determined at the follow-up evaluation. RESULTS: All of 11 patients treated with inadequate excision had palpably or histologically determined local recurrence. The most common histological diagnosis of local recurrence was dermatofibroma protuberans (7 patients, 63.63%). In patients who were treated with planned and adequate excision there were 4 (9.52%) recurrences. Five patients (45.45%) had metastases in the group of inadequate and only one patient (1.88%) in the group of adequate surgical margins. CONCLUSION: Our study demonstrated that excellent rates of survival and low rates of local recurrence and distant metastasis of soft-tissue sarcomas could be obtained with the use of carefully planned radical resection. The quality of operation is the most important factor.


2014 ◽  
Vol 3 (2) ◽  
pp. 45-48 ◽  
Author(s):  
Shyamal Chandra Debnath ◽  
Suman Kumar Roy ◽  
RR Kairy

Glomus tumor is a rare condition and constitutes only 1 % of all soft tissue tumor of body. Glomus tumor is familiar for its unusual presentation and long standing symptoms due to delay in diagnosis. Authors have presented the clinical behavior and treatment of 7 patients with subungual soft tissue mass. Following surgical excision, all patients cured of symptoms, at the end of follow up, no recurrence occurred; post operative nail deformity was insignificant. DOI: http://dx.doi.org/10.3329/bdjps.v3i2.18250 Bangladesh Journal of Plastic Surgery July 2012, 3(2): 45-48


Author(s):  
Deepak Sharma ◽  
Sachin Lamba ◽  
Aakash Pandita ◽  
Sweta Shastri

Klippel–Trénaunay syndrome (KTS or KT) is an infrequently seen dermatological syndrome, which is often viewed as a triad of vascular malformation (capillary malformations or port-wine brands), venous varicosity, and soft tissue and/or bony hypertrophy. We report a case of a 12-year-old male who presented to us with the symptoms of varicose plaques over both lower limbs and was diagnosed as a case of KTS. Management is normally conservative and includes stockings for compression of the branches to reduce edema because of chronic venous insufficiency; modern devices that cause on and off pneumatic compression; and rarely, surgical correction of varicose veins with lifelong follow-up. The orthopedic abnormalities are treated with epiphysiodesis in order to prevent (stop) overgrowing of limb and correction of bone deformity.


2013 ◽  
Vol 7 (9-10) ◽  
pp. 651 ◽  
Author(s):  
Gabriele Guglielmetti ◽  
Paolo De Angelis ◽  
Paolo Mondino ◽  
Carlo Terrone ◽  
Alessandro Volpe

Perivascular Epithelioid Cell tumour (PEComa) is rare. We describe a 39-year-old man who underwent a left radical orchidectomy and adjuvant radiation therapy for a stage IA classical testicular seminoma. He was diagnosed with a mass lateral to the right common iliac artery that was considered suspicious for late lymph node relapse after 3 years of follow-up. Due to the unusual location of the mass and the equivocal findings of percutaneous biopsy, a laparoscopic pelvic lymphadenectomy was performed. Final pathology revealed PEComa of soft tissue. The patient is disease free after 38 months of follow-up without adjuvant treatment. The presence of rare soft-tissue neoplasm should be considered in differential diagnosis of retroperitoneal masses during follow-up of germ cell tumours. Suspicious isolated recurrences of these neoplasms in unusual locations can require surgical excision to confirm diagnosis and avoid inappropriate treatment.


2020 ◽  
Vol 40 (2) ◽  
pp. 1035-1039
Author(s):  
YUSUKE MINAMI ◽  
SEIICHI MATSUMOTO ◽  
KEISUKE AE ◽  
TAISUKE TANIZAWA ◽  
KEIKO HAYAKAWA ◽  
...  

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Arshad S. Khan ◽  
Girish D. Bakhshi ◽  
Aftab Shaikh ◽  
Ashraf A. Khan ◽  
Adil A. Khan ◽  
...  

Extraskeletal myxoid chondrosarcoma (ESMC) is a rare tumor seen more often in men. It is seen to arise from soft tissue of lower extremity or buttocks. We report a case of soft tissue swelling of left labium majus in a 66-year-old female. Patient underwent wide excision with uneventful postoperative course. Histopathology of specimen confirmed it to be ESMC. Patient refused adjuvant therapy. Followup of 1 year has shown her to be disease- and symptom- free. Only two cases arising from vulva have been reported in literature . This is the third case and first from Indian subcontinent. A brief review of clinical features, diagnosis, treatment, and outcome of patients with extraskeletal chondrosarcoma is presented.


Rare Tumors ◽  
2019 ◽  
Vol 11 ◽  
pp. 203636131984728
Author(s):  
Cristian Pavelescu ◽  
Alexandra Pavelescu ◽  
Cristian Surcel ◽  
Cristian Mirvald ◽  
Mario Alvarez-Maestro ◽  
...  

Urachal adenocarcinoma represents the third most common histological type of non-urotelial bladder cancer. A very low incidence of this disease and the lack of prospective studies have led to a rich and heterogeneous treatment history. Currently, the standard of care for these patients is represented by partial cystectomy en bloc with resection of the urachal ligament and total omphalectomy. The aim of this article is to present our experience and results in the management of patients with urachal adenocarcinoma. Between 2005 and 2015, 16 patients have undergone surgical treatment for urachal adenocarcinoma in “Fundeni” Clinical Institute and Madrid University Hospital “Infanta Sofia.” Partial cystectomy was performed in 11 (68.76%) patients, while radical cystectomy en bloc with omphalectomy was performed in 5 (31.25%) patients, which were not amendable to a limited resection. The Sheldon classification was used, as it provides appropriate disease staging and is the most commonly utilized. Postoperative pathological results showed that 7 (43.75%) patients had localized tumors, and more than one-third (37.5%) of the patients had locally advanced Sheldon III disease, while 3 patients had distant metastasis at the time of surgery. Lymph node involvement was present in 3 patients (18.75%). Mean follow-up time was 2.5 years, ranging from 4 months to 7.6 years. Three patients (18.75%) were lost to follow-up, without any documented signs of local or systemic recurrence and were cancer free at the time of the last evaluation. In cases with lymph node involvement, local recurrence or distant metastasis, patients underwent cisplatin- or 5-fluorouracil-based salvage chemotherapy. Surgical treatment represents the gold standard, while adjuvant chemotherapy has a limited impact on overall survival. The utility of navel resection is questionable due to the rarity of direct invasion or local recurrence.


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