Giant cell tumor - distal end radius: Do we know the answer?

Background:

The distal end of the radius is one of the common sites of involvement in giant cell tumors (GCTs) with reportedly increased propensity of recurrence. The objective of the present analysis was to study the modalities of management of the different types of distal end radius GCTs so as to minimize the recurrence rates and retain adequate function.

Materials and methods:

Twenty-four patients of distal end radius GCTs treated between January 2000 and December 2004 were retrospectively reviewed. Nineteen cases were available for follow-up with an average follow-up of 37.5 months. There was one Campanacci Grade 1 lesion, nine Grade 2 and 14 Grade 3 lesions. Thirteen (54%) of these patients were treated elsewhere earlier and presented with recurrence. The operative procedures that were performed were: curettage and cementing (five), curettage and bone grafting (seven), excision and proximal fibular arthroplasty (two), excision and wrist arthrodesis (nine) and excision of soft tissue recurrence (one).

Results:

Functional status was evaluated using Musculo Skeletal Tumor Society scoring system which averaged 78%. The recurrence rate was 32%. Complications included local recurrence (six), nonunion at the graft bone junction (one), infection (one), deformity (two), stiffness (two), subluxation (two) and bony metastasis (one).

Conclusions:

The majority of patients undergoing curettage were either Campanacci Grade 1 or 2. Patients undergoing curettage and reconstruction had a better functional result (82%) as compared to arthrodesis or fibular arthroplasty (69%). Previous intervention did not appear to increase the recurrence rates. Even though complications occur, judicious decision-making and an appropriate treatment plan can ensure a satisfactory outcome in the majority of cases.     

Does the addition of cement improve the rate of local recurrence after curettage of giant cell tumours in bone?

We retrospectively compared the outcome after the treatment of giant cell tumours of bone either with curettage alone or with adjuvant cementation. Between 1975 and 2008, 330?patients with a giant cell tumour were treated primarily by intralesional curettage, with 84 (25%) receiving adjuvant bone cement in the cavity. The local recurrence rate for curettage alone was 29.7% (73 of 246) compared with 14.3% (12 of 84) for curettage and cementation (p = 0.001). On multivariate analysis both the stage of disease and use of cement were independent significant factors associated with local recurrence. The use of cement was associated with a higher risk of the subsequent need for joint replacement. In patients without local recurrence, 18.1% (13 of 72) of those with cement needed a subsequent joint replacement compared to 2.3% (4 of 173) of those without cement (p?=?0.001). In patients who developed local recurrence, 75.0% (9 of 12) of those with previous cementation required a joint replacement, compared with 45.2% (33 of 73) of those without cement (p = 0.044).     

Giant cell tumor of rib – rare location on the anterior aspect

Giant cell tumor rarely occurs in ribs, where it presents posteriorly. We present a report of a giant cell tumor of bone occurring anteriorly in the rib with a review of the literature. Received: 26 January 1999     

Treatment of giant cell tumor of bone: surgery or radiotherapy?]

The results of treatment for giant cell tumor of bone in 41 patients subdivided into 4 groups have been assessed. In group treated with radical surgery (amputations, extensive excision) 100% patients were cured. In group treated with curettage alone success rate was 33.3% and in radiotherapy group--72.3%. Curettage and radiotherapy combined rendered 95% success rate and this mode of treatment should be recommended.     

Giant Haemangioma of the Liver: Is Enucleation Better than Resection?

INTRODUCTION

Haemangioma is the most common liver tumour. Treatment is indicated for symptomatic tumours, rapid increase in size, rupture or doubt in diagnosis. There is continuing debate regarding the ideal method of surgical treatment for liver haemangiomas, with some surgeons favouring enucleation over liver resection.

PATIENTS AND METHODS

Retrospective analysis of prospectively compiled database of patients who were surgically treated for liver haemangioma.

RESULTS

Between 1987 and 2003, we operated on 21 patients with liver haemangioma. Pre-operative diagnosis on imaging was made in 16 patients (13 symptomatic, 3 had progressive increase in size). In five patients, the indication of surgery was uncertain diagnosis. Enucleation was performed in 9 patients and liver resection in 12. The size of the haemangioma was similar in the enucleation and resection groups (8.9 cm versus 10 cm; P = 0.85). The mean intra-operatiive blood loss was significantly less in the enucleation group (400 ml versus 1330 ml; P = 0.004). The mean operative time was significantly less in the enucleation group as compared to the resection group (170 min versus 230 min; P = 0.035). Five patients had major postoperative morbidity in the resection group as compared to none in the enucleation group (P = 0.045). The duration of hospital stay was significantly longer in the resection group.(9.9 days versus 5.6 days; P = 0.005).

CONCLUSIONS

Enucleation of liver haemangiomas is safer, quicker and associated with less morbidity than liver resection. Except for some situations, such as uncertain diagnosis or total replacement of a lobe, we recommend enucleation as the surgical procedures of choice for the treatment of hepatic haemangiomas.     

'Spindle cell oncocytoma' of the adenohypophysis: a tumor of folliculostellate cells?

We describe five primary tumors of the adenohypophysis featuring mitochondrion-rich spindle cells. The patient ages ranged from 53 to 71 years (mean 61.6 years); two were female. All presented with panhypopituitarism. Two also had visual field defect. On neuroimaging all tumors showed suprasellar extension and were indistinguishable from pituitary adenoma. None showed imaging or operative evidence of dural involvement. All were gross totally removed: four by transsphenoidal surgery and one by frontal craniotomy. Follow-up ranged from 2 to 68 months (mean 35.4 months). No recurrences were noted. The clinical workup was noncontributory in all but two patients: one (case no. 4) with an oncocytic thyroid adenoma and another (case no. 5) with squamous carcinoma of both the uterine cervix and of vocal cord. Histologically, the five tumors were composed mainly of fascicles of spindle cells with eosinophilic, granular cytoplasm. Mitoses were rare and necrosis was absent. Neoplastic cells were immunoreactive for vimentin, epithelial membrane antigen, S-100 protein, and galectin-3. Stains for pituitary hormones, synaptophysin, chromogranin, glial fibrillary acidic protein, cytokeratin CAM5.2, smooth muscle actin, CD34, and CD68 were negative. No thyroglobulin immunoreactivity was noted in the tumor of case no. 4. Ultrastructurally, the neoplastic cells contained numerous mitochondria with lamellar cristae. The neoplastic cells were linked by intermediate junctions and desmosomes. No secretory granules were noted. The histologic, immunohistochemical, and fine structural features of these tumors were unlike those of pituitary adenoma or any other primary sellar tumor. A derivation from adenohypophyseal folliculostellate cells is suggested.     

Sertoli cell proliferations of the infantile testis: an intratubular form of Sertoli cell tumor?

We report on six boys with intratubular Sertoli cell proliferations (ISCPs), studied by routine histologic methods, electron microscopy, and immunohistochemistry of anti-müllerian hormone (AMH), inhibin alpha-subunit, 3beta-hydroxysteroid dehydrogenase (3beta-HSD), proliferative cellular nuclear antigen, and p53, and carefully followed for extended periods with periodic clinical examinations, testicular ultrasonographies, and determinations of serum levels of AMH and inhibin B. Peutz-Jeghers syndrome was found in four of six patients, and gynecomastia occurred in five of six patients. One boy had isosexual pseudoprecocity. ISCPs were observed as multiple foci of seminiferous tubules with large and proliferated Sertoli cells replacing germ cells and limited by the basement membrane. Mitotic figures, atypia, and/or interstitial invasion were not observed. Bilateral ISCPs were the only pathologic finding in three patients (patient nos. 1-3) and were associated with a microscopic tumor that resembled a large-cell calcifying Sertoli cell tumor (LCCSCT) in a fourth patient (patient no. 4). In the two remaining patients (patient nos. 5 and 6) ISCPs and LCCSCT were found in both testes. Ultrastructural examination showed large Sertoli cells, with round nuclei, sparse organelles, and some glycogen. Inhibin alpha-subunit immunolocalization was positive in the five patients in whom it was determined (patient nos. 2-6), AMH was positive in those ISCPs associated with tumors (patient nos. 4-6) and negative in isolated ISCPs (patient nos. 2 and 3); 3beta-HSD and PCNA were variable, and p53 was negative in all ISCPs. Patient nos. 1-4 have been followed for 2-19 years. One of them is currently entering puberty, the other two have already completed puberty and have testes of normal size, and the remaining one is an adult with clinically normal testes and sperm production. None of these patients had evidence of tumor development during follow-up as shown by serial ultrasonographies and serum levels of AMH and inhibin B. Patient nos. 5 and 6 who had bilateral ISCPs and LCCSCT were orchidectomized and evolved for 2-10 years after surgery without tumor recurrence. The prognostic significance of ISCPs, particularly when they are the only pathologic finding in a testicular biopsy, is a matter of controversy. Based on the long normal evolution, we recommend a conservative approach to therapy. The bilateral and multicentric character of ISCPs and their association with Sertoli tumors and Peutz-Jeghers syndrome suggest that they represent either proliferative lesions with tumorigenic potential or the intraepithelial stage in the evolution of some testicular Sertoli cell tumors.     

Giant basal cell carcinomas: a result of neglect?

Although the occurrence of giant basal cell carcinoma in clinical practice is quite rare, there are some references to these malignancies in the literature. Our study includes 12 patients without previous treatment who had giant basal cell carcinoma with no evidence of metastatic dissemination. Depending on the anatomic region, we used a primary suture, local flap and split-thickness skin grafts for the reconstruction of the defect after the wide excision of the carcinoma. All patients have been followed up and no recurrence has been detected to date. Nine lesions diagnosed on the face (69%) were obvious, and therefore they should have been diagnosed at their early stage. In our series most of the patients had had tumors for more than 3 years (except 2 cases) with no history of radiation exposure or recurrence after previous treatment. Therefore our findings suggest to us that the primary reason for a tumor to achieve this giant size was neglect.     

Is neoadjuvant therapy the answer to adenocarcinoma of the esophagus?

The conflicting results of randomized studies have led to confusion over the proper management of patients with esophageal adenocarcinoma. Although there is no firm evidence that neoadjuvant chemoradiation improves survival, because of the shortcomings of these trials, this method of treatment is practiced at many centers. Without the results of another multiinstitutional randomized trial, the true answer may never be known.     

Giant cavernous hemangioma of the liver: is tumor size a risk factor for hepatectomy?

The aim of this study was to evaluate whether hepatic giant cavernous hemangioma (GCH) tumor size is a risk factor for hepatectomy. Twenty patients with GCH of the liver were treated by hepatic resection. Eleven patients with maximum resected specimen tumor size of >10 cm (mean tumor size, 18.5 cm; group 1) were compared with the 9 patients with tumor size. <10 cm (mean tumor size, 8.6 cm; group 2). The incidence of major hepatectomy in group 1 was significantly higher than that in group 2 (P = 0.0241). Although there were no significant differences in preoperative liver function, or in fibrinogen or platelet counts between the two groups, the level of preoperative fibrin degradation product (FDP) in group 1 was significantly higher than that in group 2 (P = 0.0116). Mean intraoperative hemorrhage volume, blood transfusion volume, and operation time in group 1 vs group 2 were 7003 ml vs 1092 ml (P = 0.0251), 2927 ml vs 556 ml (P = 0.0169), and 431 min vs 216 min (P < 0.0001), respectively. The incidence of postoperative complications in group 1 (45.5%) was higher than that in group 2 (22.2%), although not significantly so. There was no operative mortality in either group. Tumor size significantly correlated with intraoperative blood loss, operation time, weight of resected liver, intraoperative blood transfusion volume, and preoperative FDP levels. GCH tumor size is a significant risk factor for hepatectomy mainly because of the massive intraoperative blood loss and blood transfusion associated with major hepatic resection. More careful preoperative management to decrease tumor size may increase the safety of surgery for GCH of the liver. Received for publication on Jan. 21, 1999; accepted on May 24, 1999     

Denosumab in giant cell tumour of bone in the pelvis and sacrum: Long-term therapy or bone resection?

IntroductionSurgery of GCTB in sacrum and pelvis is challenging, with high rates of complications and local recurrence. Denosumab can consolidate the peripheral rim of the tumour, thus reducing the rate of morbidities of surgery. The aim of this paper is to evaluate the use of denosumab in pelvic/sacrum giant cell tumours of bone (GCTB).Patients and methodsWe retrospectively reviewed a cohort of 26 patients with aggressive GCTB in sacrum or pelvis treated with denosumab at two referral centres. Clinical response and local recurrence were recorded and the radiologic responses were evaluated with the MDA criteria.Results69% of the pelvic GCTB treated with denosumab presented partial or good radiologic responses (type 2A or 2B) after 49 weeks of treatment. Denosumab was administered as adjuvant therapy prior and after surgery in 11 patients (group A), and as the only treatment in 15 patients (group B). In group A, 62% of local recurrence was observed in patients treated with intralesional curettage. No recurrences were identified after en bloc resection. In group B, 9 patients were on continuous bimonthly long term denosumab administration with type 2A and 2B responses. Six patients stopped denosumab and 66% remained stable after 10 months of follow-up.ConclusionsLong-term denosumab therapy can be considered with curative intent for pelvic and sacrum GCTB. If surgical intervention is required wide resection may be advisable to reduce the risk of recurrence.     

Giant cell tumour of the cuboid – A case report

Giant cell tumours are uncommon benign osseous neoplasia. Most occur around the epiphysis of long bones after skeletal maturity and are very rarely seen in the foot. We present a case of a fifteen-year-old amateur basketball player who presented with a painful ‘peroneal spastic foot’ that was diagnosed to be due to a giant cell tumour of the cuboid bone. There was a good response to a curettage and bone grafting, with no recurrence at his thirty-month follow-up.     

Management of focal cartilage defects in the knee - Is ACI the answer?

Injuries to the articular cartilage of the knee are common. They alter the normal distribution of weightbearing forces and predispose patients to the development of degenerative joint disease. The management of focal chondral lesions continues to be problematic for the treating orthopaedic surgeon. Although many treatment options are currently available, none fulfill the criteria for an ideal repair solution: a hyaline repair tissue that completely fills the defect and integrates well with the surrounding normal cartilage. Autologous chondrocyte implantation (ACI) is a relatively new cell-based treatment method for full-thickness cartilage injuries that in recent years has increased in popularity, with early studies showing promising results. The current article reviews the nature of cartilage lesions in the knee and the treatment modalities utilized in their management, focusing on the role ACI plays in the surgical treatment of these complex injuries.     

Hürthle cell tumor of the thyroid: analysis of 188 cases

We reviewed 188 cases of Hürthle cell tumor of the thyroid (HCT) between 1982 and 1996. There were 160 women and 28 men with a mean age of 51.8 years. Thirty-one of the patients had cancer, and the others had adenoma. Age, size of the primary tumor, and preoperative thyroglobulin level were not significantly different in the cancer and adenoma patients. The gender ratio, however, was significantly different (p < 0.05). Recurrent HCT was observed in three patients with adenoma. Two patients had subcutaneous recurrence (suspected implantation), and the other patient had recurrence in the residual thyroid gland. All patients with recurrence of adenoma underwent partial lobectomy at the initial operation. Three cancer patients had recurrent disease. Locoregional recurrence was observed in one patient and distant metastases in two patients (lung in one, lung and bone in one). One of the patients with distant metastasis died from the disease, and the other is alive with the disease. Tumor implantation was observed in patients with adenoma, so intraoperative handling of the tumor requires care. It also means that this tumor, even though benign, is aggressive in terms of proliferative activity. All patients with Hürthle cell tumor should be treated by total lobectomy at least. The outcome of the cancer patients was not as poor as in previous reports.     

Infiltrating giant cell tumor in a case of Paget’s disease of bone

Giant cell tumor (GCT) of the bone, also called osteoclastoma, is a rare complication of Paget’s bone disease. We report a patient from Southern Italy who developed a GCT infiltrating the neighboring tissues. The natural history and the therapeutic outcomes of this unique complication of Paget’s bone disease are presented.