scholarly journals Locally Aggressive Connective Tissue Tumors

2018 ◽  
Vol 36 (2) ◽  
pp. 202-209 ◽  
Author(s):  
Mrinal M. Gounder ◽  
David M. Thomas ◽  
William D. Tap

In this review, we highlight the complexities of the natural history, biology, and clinical management of three intermediate connective tissue tumors: desmoid tumor (DT) or aggressive fibromatosis, tenosynovial giant cell tumor (TGCT) or diffuse-type pigmented villonodular synovitis (dtPVNS), and giant cell tumor of bone (GCTB). Intermediate histologies include tumors of both soft tissue and bone origin and are locally aggressive and rarely metastatic. Some common aspects to these tumors are that they can be locally infiltrative and/or impinge on critical organs, which leads to disfigurement, pain, loss of function and mobility, neurovascular compromise, and occasionally life-threatening consequences, such as mesenteric, bowel, ureteral, and/or bladder obstruction. DT, PVNS, and GCTB have few and recurrent molecular aberrations but, paradoxically, can have variable natural histories. A multidisciplinary approach is recommended for optimal management. In DT and PVNS, a course of observation may be appropriate, and any intervention should be guided by symptoms and/or disease progression. A surgical approach should take into consideration the infiltrative nature, difficulty in obtaining wide margins, high recurrence rates, acute and chronic surgical morbidities, and impact on quality of life. There are similar concerns with radiation, which especially relate to optimal field and transformation to high-grade radiation-associated sarcomas. Systemic therapies must be considered carefully in light of acute and chronic toxicities. Although standard and novel therapies are promising, many unanswered questions, such as duration of therapy and optimal end points to evaluate efficacy of drugs in clinical practice and trials, exist. Predictive biomarkers and novel clinical trial end points, such as volumetric measurement, magnetic resonance imaging T2 weighted mapping, nuclear imaging, and patient-reported outcomes, are in development and will require validation in prospective trials.

Sarcoma ◽  
2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Thale M. Asp Strøm ◽  
Anette Torød Skeie ◽  
Ingvild Koren Lobmaier ◽  
Olga Zaikova

Background. Pediatric giant cell tumor (GCT) of bone is rare and the course of the disease in the immature skeleton is sparsely described. We performed a retrospective study addressing symptoms, treatment, and outcome in children with GCT.Methods. Review of medical records and images of patients with GCT. Patients were detected from our hospital prospective database and those with open epiphyseal cartilages were included.Results. 16 children (75% girls) from 6 to 15 years old were identified. Eight lesions (50%) were in long bones and 4 (25%) in flat bones. One lesion appeared to be purely epiphyseal. All patients had pain as the initial symptom. Local recurrence developed in 2 patients. 14 of 16 patients returned to normal activity with no sequelae. One patient developed anisomelia after surgery.Conclusions. The biological tumor behavior in children does not seem to differ from what is reported in adults. Lesions in flat bones are very unusual, but our data alone do not provide enough evidence to conclude that this is more common in the immature skeleton. Literature review showed only one previous case report describing a purely epiphyseal GCT. Intralesional curettage is appropriate treatment and gives good functional results with acceptable recurrence rates.


2015 ◽  
Vol 33 (15_suppl) ◽  
pp. 10521-10521 ◽  
Author(s):  
Heather Gelhorn ◽  
Sandra Tong ◽  
Grant Maclaine ◽  
John H. Healey ◽  
Susan V. Bukata ◽  
...  

2020 ◽  
Vol 10 (18) ◽  
pp. 6310
Author(s):  
Cosmin Ioan Faur ◽  
Ahmed Abu-Awwad ◽  
Daniel Laurențiu Pop ◽  
Carmen Lăcrămioara Zamfir ◽  
Daniela Gurgus ◽  
...  

Giant cell tumor (GCT) of bone is a benign aggressive bone lesion with significant recurrence rates following surgical curettage. Historically, these tumors were approached by performing an intralesional curettage of the tumoral tissue by filling the resulting cavity using morselized iliac bone autograft. The major problems of this therapy were the high recurrence rates of up to 40–50%. Several adjuvant treatments have been proposed in order to augment resection margins, including liquid nitrogen (LN), phenol, ethanol, hydrogen peroxide (H2O2) and bone cement (polymethyl methacrylate (PMMA)). LN can be used either to preserve tissues or for controlled necrosis depending on the cycles of freezing and thawing. Usually, a quick freeze followed by a slow thaw will lead to destruction of human cells. This article reviews the results of cryosurgery with LN associated with surgical resection and the additional use of PMMA in a small group of patients with a histopathological confirmation of bone GCT with different localizations (i.e., tibia, distal radius and iliac bone). Cryosurgery with LN of bone GCT proved to be an efficient tool to decrease the recurrence rate for this tumoral type. In our series of cases, there were no complications, oncological or otherwise, at the two-year minimum follow-up, with good and excellent functional results.


2020 ◽  
Vol 25 (02) ◽  
pp. 158-163 ◽  
Author(s):  
Hüseyin Bilgehan Çevik ◽  
Sibel Kayahan ◽  
Engin Eceviz ◽  
Seyit Ali Gümüştaş

Background: Tenosynovial giant cell tumor (TSGCT) is the second most common benign tumor of the hand. Even though it is a benign lesion there is still a high incidence of local recurrence (range, 7%–44%) according to data in published papers. In this study, the clinical and epidemiological features of 173 patients who underwent excision of localized TSGCT, the recurrence rates and possible reasons for recurrence were examined in the light of current literature. Methods: Medical records of 173 patients with TSGCT were reviewed. Data on demographic characteristics as well as clinical and intraoperative findings were collected. Patients were asked about the recurrence of the TSGCT and the QuickDASH scoring was applied at the final clinical evaluation after mean follow-up of 81 months. Results: Females were predominantly involved (73%). Patients aged mean 44 years at the time of surgery. There were 93 tumors in flexor zones and 80 tumors in extensor zones of the hand. Of the tumors with flexor zone localization, zone II was most predominantly involved with 46 tumors, and 18 of these were on the index finger. The extensor zones III and IV were mostly involved with 9 tumors each on the middle and ring fingers. A total of 12 recurrences (6.9 %) were determined over the mean follow-up period of 81 months. Conclusions: The characteristics of our patients identified were similar to the previous studies. Surgical excision provides good outcomes in the treatment of TSGCT especially when clear margins are obtained.


2017 ◽  
Vol 27 (6) ◽  
pp. 813-819 ◽  
Author(s):  
Costantino Errani ◽  
Shinji Tsukamoto ◽  
Giulio Leone ◽  
Manabu Akahane ◽  
Luca Cevolani ◽  
...  

2019 ◽  
Vol 20 (1) ◽  
Author(s):  
Hakan Ozben ◽  
Tamer Coskun

Abstract Background Giant cell tumor of the tendon sheath is the most common form of giant cell tumors and is the second most common soft tissue tumor of the hand region after ganglion cyst. Magnetic resonance imaging is the diagnostic tool of choice for both diagnosis and treatment planning. The current standard treatment of choice is simple excision. The main concern about the treatment is related to the high recurrence rates. Besides incomplete excision, there is no consensus concerning the effect of other risk factors on recurrence. The literature lacks detailed reports on surgical excision of these tumors with a standardized surgical treatment and an appropriate patient follow up. The aim of this study was to investigate the recurrence rate and the associated recurrence risk factors for giant cell tumor of tendon sheath of the hand following a standardized treatment. Methods The records of patients treated for giant cell tumor of tendon sheath of the hand treated by the same hand surgeon were evaluated retrospectively. The features obtained from preoperative magnetic resonance imaging, final physical examination, patients’ age and sex, anatomical site of the tumor, relationship of the tumor with bone, joint or neurovascular structures, bone invasion, recurrence after surgery and complications like skin necrosis, digital neuropathy or limitation in range of motion were documented. Chi-square test was used to compare categorical variables. Results Fifty patient were included in the study. The average follow-up time was 84 months. Three recurrences (6%) were recorded. The only significant risk factor for the recurrence was tumor adjacency to the interphalangeal joints of the fingers other than thumb. No major or minor complications were encountered in the postoperative period. Conclusion With adequate surgical exposure and meticulous dissection provided by the magnification loupes, we were able demonstrate one of the lowest recurrence rates in the literature. Well-designed studies combining the recurrence rates of several hand surgery centers implementing a standardized treatment are needed to better demonstrate the associated risk factors for recurrence.


1995 ◽  
Vol 16 (10) ◽  
pp. 617-623 ◽  
Author(s):  
Regis J. O'Keefe ◽  
Richard J. O'Donnell ◽  
H. Thomas Temple ◽  
Sean P. Scully ◽  
Henry J. Mankin

Giant cell tumor of bone has been shown to behave more aggressively when located in the wrist and hand. Although nearly 4% of giant cell tumors arise in the foot and ankle, biological features specific to this location have not been identified. In our experience with more than 300 cases of giant cell tumor, 12 arose in the foot and ankle and were followed for more than 2 years. These included nine females and three males ranging in age from 15 to 52 years (mean age, 29.5 years). All patients presented with pain of 5.0 months’ mean duration and 9 of 12 tumors demonstrated aggressive radiographic features, including bone erosion and destruction; five had either invasion of a joint or a soft tissue mass present. Unlike the hand, where metacarpal and phalangeal lesions are common, no tumors arose in the forefoot and nine of the tumors were present in the ankle region. Four patients were treated with resection (no recurrence), two with curettage and cement packing (one recurrence), and six with curettage and autologous bone graft (two recurrences), which resulted in an overall recurrence rate of 25%. None of the recurrent tumors have returned after additional treatment, which consisted of curettage and cement packing in two cases and resection in one case. Five tumors (four primary, one recurrent) were treated with local resection and reconstruction with no major complications and with no amputations performed. Thus, giant cell tumors of the foot and ankle can be treated with local procedures, which result in recurrence rates similar to those found in more common locations.


2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Ingo Schmidt

A 51-year-old female patient presented with a carpal giant cell tumor (GCT) of the right capitate bone. The lesion was initially misdiagnosed as having an osteomyelitis. First, the diagnosis of a benign GCT was confirmed by histological examination. Second, an intralesional curettage and packing of the cavity with cancellous iliac crest bone grafts combined with a fusion of the third carpometacarpal (CMC III) joint were carried out. Third, due to a secondary midcarpal osteoarthritis and a secondary scaphoid nonunion, the CMC III joint fusion plate was removed and the midcarpal joint completely excised. Fourth, in the absence of recurrence of GCT, a four-corner fusion (4CF) with a corticocancellous iliac crest bone graft and complete excision of the scaphoid bone had to be performed. Fifth, a total wrist arthroplasty (TWA) was performed due to hardware failure of 4CF with migration of a headless compression screw into radiocarpal joint which led to erosion of articular surface of the distal radius. At the 3-year follow-up that includes a 1-year follow-up after TWA, there was no recurrence of GCT, and the TWA was not failed. The patient reported that she would have the motion-preserving TWA again.


2021 ◽  
Vol 29 (2) ◽  
pp. 230949902110075
Author(s):  
Himanshu Kanwat ◽  
Roshan Banjara ◽  
Venkatesan Sampath Kumar ◽  
Abdul Majeed ◽  
Shivanand Gamnagatti ◽  
...  

Objectives: Both Zoledronic acid and denosumab have been utilized in neo-adjuvant setting for facilitating surgery and downsizing the lesion in Giant cell tumor (GCT). This study is aimed at comparing Zoledronic acid and Denosumab, when used in neo-adjuvant setting, in terms of radiological and clinical outcomes in GCT undergoing surgical intervention. Patients and Methods: Patients undergoing surgical intervention for GCT who received either denosumab or Zoledronic acid as neoadjuvant agents were retrospectively analyzed for reduction in tumor load radiologically, change in surgical plan after therapy, facilitation of surgery, therapy related complications, cost of treatment, rate of local recurrence and clinical outcomes. Results: Twenty patients received denosumab and 19 patients received Zoledronic acid as neoadjuvant agent. There was no significant difference in radiological outcomes, facilitation of surgery and clinical outcomes at end of follow-up. Zoledronic acid group had lower number of recurrences, however, not statistically significant. Therapy with Zoledronic acid was significantly cheaper (p = 0.001). Conclusion: Zoledronic acid is a cheaper alternative to denosumab in terms of solidification of lesion, reducing recurrence rates and improving clinical outcomes. Larger prospective studies required to further delineate this outcome with Zoledronic acid.


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