Biliary tract rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Associazione Italiana Ematologia Oncologia Pediatrica

2018 ◽  
Vol 104 (3) ◽  
pp. 232-237 ◽  
Author(s):  
Katia Perruccio ◽  
Valerio Cecinati ◽  
Angela Scagnellato ◽  
Massimo Provenzi ◽  
Giuseppe Maria Milano ◽  
...  

Introduction: Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor frequent in children. Biliary duct localization is extremely rare, but it is the most common cause of malignant obstructive jaundice in pediatric patients. Methods: This report describes a series of 10 patients under 18 years of age with biliary tract rhabdomyosarcoma who were enrolled, from 1979 to 2004, in 3 consecutive Italian pediatric cooperative protocols that had been drawn up by the Soft Tissue Sarcoma Committee of the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP). Results: Considering initial and delayed surgery, tumor resection was achieved in 7 cases, 3 complete with free margins (2 liver transplants) and 4 with microscopic residual disease. Chemotherapy was given to all patients and radiotherapy to 3. At present, 5 patients survive in complete remission 90-200 months after diagnosis while 4 died of disease progression or relapse and 1 of liver transplant-related complications. Conclusions: Better outcomes in this series were associated with the feasibility of conservative surgery due to the favorable location of the tumor, in particular in the common bile duct. Chemotherapy and radiotherapy might obviate the need for demolitive surgery or liver transplant, which were linked to worse outcomes in our series.

2021 ◽  
Vol 5 (1) ◽  
pp. 3-7
Author(s):  
Wei-na Wu ◽  
Hai-ying Cao ◽  
Zhen Feng

Purpose: Patients who were diagnosed with soft tissue sarcoma (STS) of limbs and met the indications for amputation because of no effective soft tissue coverage after extensive tumor resection were treated with skin flap transplantation and therapeutic courses were observed.  Methods: A total of 23 patients, pathologically diagnosed as soft tissue sarcoma from January 2011 to June 2015 and whose effective soft tissue coverage was not guaranteed due to extensive tumor resections were included. They underwent extensive tumor excision and skin flap grafting. The postoperative follow-up, tumor control and functional recovery was evaluated as per the Musculoskeletal Tumor Society (MSTS) score.  Results: The follow-up time was from 8 to 60 months, with an average follow-up of 39.69 months. Of the 23 patients, 10(43.47%) died of tumor metastasis from 8th to 23rd months after surgery. In these 10 cases, 2 cases (8.70%) subsequently underwent amputation because of local tumor recurrence. The remaining 13 patients are recurrence free till date of follow up. The MSTS limb function score at 3 months after surgery of all patients were 7-28 points, with an average of 22.56 points, and the excellent and fair scores are observed in 91.30% of patients.  Conclusion: For patients who cannot achieve effective soft tissue coverage after extensive resection of soft tissue sarcoma and meet the indications of amputation can have limb preservation by extensive tumor resection combined with skin flap transplantation with a good functional results 


2012 ◽  
Vol 30 (2) ◽  
pp. 62 ◽  
Author(s):  
Jieun Lee ◽  
Young Je Park ◽  
Dae Sik Yang ◽  
Won Sup Yoon ◽  
Jung Ae Lee ◽  
...  

2018 ◽  
Vol 39 (1) ◽  
pp. 331-334 ◽  
Author(s):  
TOMOKI NAKAMURA ◽  
TORU OI ◽  
KUNIHIRO ASANUMA ◽  
TOMOHITO HAGI ◽  
AKIHIRO SUDO

2021 ◽  
Author(s):  
Zhongshan Liu ◽  
Yangzhi Zhao ◽  
Yunfeng Li ◽  
Xia Lin ◽  
Dongzhou Wang ◽  
...  

Abstract Purpose: Locally recurrent extremity soft tissue sarcoma remains a therapeutic challenge; conservative surgery alone results in an inferior local control rate. This study demonstrates a new interstitial (IS) brachytherapy (BT) technique in a patient with recurrent extremity soft tissue sarcoma. Patients and methods: A 53-year-old man with recurrent left thigh malignant fibrous histiocytoma underwent conservative surgery and adjuvant intensity-modulated radiotherapy (IMRT) after two surgical excisions. A magnetic resonance imaging (MRI) of the lower extremity after the conservative surgery and IMRT revealed a left thigh mass measuring 12 cm × 8 cm × 7 cm. An IS BT with 3 fractions of 8 Gy each and guided with three-dimensional (3D) computed tomography (CT) was administered. For this procedure, IS metal needles were inserted at a depth of 1 cm into the tumor as a preliminary implantation, and their direction and depth were adjusted repeatedly until a satisfactory distribution was achieved through multiple CT scans. Results: The course of the IS BT procedure was uneventful. No severe bleeding, infection, or other complications were observed. At 3, 12, and 24 months after the IS BT, lower extremity MRI scans showed a left thigh mass measuring 10 cm × 5 cm × 4, 8 cm × 3 cm × 2 cm, and 6 cm × 2 cm × 2 cm, respectively. Minimal fibrosis, local numbness, and edema in the treatment area were noted. The patient had an excellent quality of life. Conclusion: Favorable oncologic outcomes for locally recurrent extremity soft tissue sarcoma were achieved using 3D CT-guided IS BT. This BT technique may contribute to an excellent local control rate and offer an effective and safe therapeutic option in selected cases.


2018 ◽  
Vol 108 (2) ◽  
pp. 164-171
Author(s):  
G. Kask ◽  
I. Barner-Rasmussen ◽  
J. Repo ◽  
C. Blomqvist ◽  
E. Tukiainen

Background and Aims: The present standard of care in treating lower extremity soft tissue sarcomas is function-sparing, limb-preserving resection and reconstruction with or without oncological therapy. The aim of this pilot study was to test the suitability and adequacy of the Finnish translations of two functional outcome questionnaires (Toronto Extremity Salvage Score and Musculoskeletal Tumor Society score) and to perform a preliminary investigation of functional outcomes of Finnish lower-limb soft tissue sarcoma patients after operative treatment. Materials and Methods: Between June 2015 and December 2015, consecutive surgically treated outpatients were asked to participate in the study. Demographic, clinical, surgical, and oncological outcome data were collected. Two functional outcome questionnaires were used (Toronto Extremity Salvage Score and Musculoskeletal Tumor Society scores). A comparative analysis is presented. Results: A total of 19 lower-limb soft tissue sarcoma patients with a mean follow-up time of 2 years and 10 months were included. All (n = 19) invited patients participated in the study. Mean age was 62.3 years. In total, 13 had high-grade sarcomas. Eight wounds were closed directly, four used skin grafts, and five required flap reconstructions. One patient required a tumor prosthesis, and one required a rotationplasty. A total of 14 patients received oncological therapy. No problems or difficulties were reported in using and completing the Finnish versions of the Toronto Extremity Salvage Score or Musculoskeletal Tumor Society questionnaires. The overall Toronto Extremity Salvage Score and Musculoskeletal Tumor Society scores were 88 and 76, respectively. Conclusion: This pilot study suggests that the Finnish versions of the Toronto Extremity Salvage Score and Musculoskeletal Tumor Society questionnaires are suitable for measuring functional outcome after lower extremity soft tissue sarcomas treatment. Functional outcomes vary from moderate to excellent.


1994 ◽  
Vol 30 (3) ◽  
pp. 271-273 ◽  
Author(s):  
Roberto Zucali ◽  
Mirella Merson ◽  
Marco Placucci ◽  
Silvana Di Palma ◽  
Umberto Veronesi

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