Pediatric extremity rhabdomyosarcoma treated with re-brachytherapy in recurrent setting.

Rhabdomyosarcomas (RMS) are pediatric soft-tissue sarcomas arising from immature mesenchymal cells that are intended to form striated skeletal muscles. Brachytherapy delivers high-dose of precised radiation to the target tissue with high conformity, sparing the nearby normal tissues, hence allowing dose escalation and reducing the likelihood of normal tissue toxicity. There is a scarcity of reports on the use of brachytherapy for extremity RMS. We report the case of pediatric extremity RMS treated with re-brachytherapy in recurrent setting. A 4-year-old boy diagnosed with RMS of right upper arm underwent local excision of the lesion. Postoperative magnetic resonance imaging showed suspicious residual lesion. Revision surgery followed by brachytherapy with 30 Gy in 10 fractions twice a day over 5 days was delivered. The child developed local recurrence after 12 months. Reexcision and re-irradiation with brachytherapy were done delivering 27 Gy in 9 fractions twice a day over 5 days. The child is disease-free 18 months posttreatment with no significant disparity in limb length suggestive of successful preservation of growth epiphysis. Re-irradiation with interstitial brachytherapy can be considered as an option for the treatment of recurrent pediatric extremity rhabdomyosarcoma, in conjunction with surgery and chemotherapy, despite treated previously with brachytherapy.

Extra digital glomus tumor causing scapulothoracic impingement: A case report.

Glomus tumor is a painful benign neoplasm which arises from the glomus body. The glomus body is neuromyoarterial receptor responsible for regulating the peripheral skin blood flow and local temperature. The classical clinical symptoms of the glomus tumor are acute local pain, pin point tenderness and hypersensitivity to cold. The commonest location of the glomus tumor is the subungual region of the distal phalanx of the fingers and toes. We hereby describe a very rare case of extradigital glomus tumor in the interspace between the scapula and the posterior chest wall causing paroxysmal severe pain on the movement of the right shoulder. Being deep in location, the tumor was not clinically palpable or identifiable. The lesion was eventually located on MRI. Surgical resection of the tumor led to complete resolution of patient's symptoms.

Epithelioid sarcoma of the foot with subsequent lesion in hand: metastatic lesion or second primary?

Epithelioid sarcomas (ES) are rare tumors of soft tissue that have a propensity to occur in the extremities. Epithelioid sarcomas are known to metastatise to draining lymph nodes and commonly to the lungs. Herein, a case of epithelioid sarcoma which recurred in an unusual site namely the distal phalanx of left middle finger, six months post amputation of the primary lesion in the left foot is being reported. The ipsilateral inguinal lymph node showed metastatic deposits. The tumor at both these sites had similar histology and an identical immunohistochemical (IHC) pattern showing reactivity to cytokeratin (CK), epithelial membrane antigen (EMA), vimentin (Vim) and CD34. This case is presented to record an unusual occurrence of ES in the distal phalanx of middle finger with an ES of foot. The metastasis of ES to the distal acral bones has not been documented till date.

Giant cell tumor of bone in children and adolescents.

BACKGROUND: There are very few series that document giant cell tumor of bone (GCT) in the immature skeleton, and the reported incidence in literature varies from 1.8% to 10.6%. The purpose of this study was to document the incidence of GCT in patients with open physis in the Indian population and study the course of the disease with respect to its adult counterpart to see if it behaved any differently. METHODS: Between January 2000 and December 2005, 17 (6%) of 285 surgically treated patients with histologically proven GCT had open physis on imaging. Treatment was directed toward local control without sacrificing joint function, with most lesions treated with intralesional curettage. RESULTS: Fourteen (82%) patients were girls. The most common site was around the knee (53%). Of 15 lesions in tubular bones, 13 were epiphysiometaphyseal in location. An open physis did not prevent GCT from penetrating the epiphyseal cartilage. Histologically, the tumors were typical of GCT. Of 15 patients available for follow-up, 3 (20%) developed local recurrence. CONCLUSIONS: Although the overall incidence of GCT may be higher in the Asian population, the percentage of skeletally immature patients or those nearing skeletal maturity is similar to that described in literature. The biological behavior of the disease is similar to that seen in adults, except a marked female preponderance, principles of treatment, recurrence patterns, and course of the disease mirror the behavior of its adult counterpart.

Limb salvage surgery for osteosarcoma: effective low-cost treatment.

Successful management of osteosarcoma with limb salvage surgery is a challenging problem in the developing world. We report our early results with low-cost chemotherapy (without methotrexate) and low-cost limb salvage surgery. We prospectively collected data for 135 patients with histologically proven high-grade osteosarcoma of the extremities given neoadjuvant chemotherapy and treated with limb salvage surgery between January 2000 and February 2004. A locally designed and fabricated stainless steel customized megaprosthesis was used in 78 patients. Disease status and function was evaluated at followup ranging from 24 to 72 months. Followup data were available for 120 of the 135 patients. There were eight infections, four prosthesis breakages and three loosenings. Eighteen patients had local recurrence; 17 of these patients also developed lung metastases. Seventy-three patients (61%) were disease-free at followup. The group of 34 patients with 100% necrosis had better disease-free survival (79%). According to a modified Enneking system, the average functional score was 25.5 of 30 (85%) for the lower extremity and 20 of 30 (66%) for the shoulder. Our preliminary results suggest osteosarcoma can be managed well in a developing country in a cost-effective way. Limb salvage surgery has now become the standard of care.

Rotationplasty for bone tumors: is there still a role?

We evaluated the disease status and functional results in 30 patients (range, 6-25 years) who underwent rotationplasty for bone sarcomas from January 2000 to February 2004. Plating was used for fixation in all 27 distal femur resections. In the proximal femur tumor, the distal femur was contoured and fixed to the ilium with cancellous screws. In the two cases involving the entire femur, the upper end of the tibia was articulated with the acetabulum in one case and an Austin Moore prosthesis was inserted in the upper end of the tibia in the other. Two patients underwent an amputation after postoperative vascular compromise. Other complications included venous congestion in two patients, who recovered after exploration, partially recovered nerve palsy in one, wound infection in two, local recurrence in one, and nonunion with subsequent bone grafting in one. Functional evaluation was documented in 26 patients with a followup ranging from 24 to 60 months. Using the Musculoskeletal Tumor Society scoring system, the score was 25 or greater in 20 of 26 patients. In appropriately indicated cases, rotationplasty provides good local disease control and good function.

Hemicortical excision for low-grade selected surface sarcomas of bone.

In malignant surface tumors, hemicortical excision in select cases may allow adequate margins with better function. We treated 10 of 22 malignant surface tumors with hemicortical excision and reconstruction from January 2000 to June 2003. There were eight conventional parosteal osteosarcomas, one high-grade surface osteosarcoma, and one surface chondrosarcoma. Six tumors occurred in the femur, two in the tibia and one each in the humerus and radius. Defects were reconstructed with allograft (five), fibula autograft (three), or autogenous iliac crest (two). Internal fixation as suitable was used. Margins were reported uninvolved in all cases. At followup ranging from 40 months to 61 months we observed no local recurrence. Using the MSTS scoring system, six patients had a maximum possible score of 30; the other four had a score of 29. None developed distant metastasis. Though a longer followup is ideal, in selected cases hemicortical excision appears a reasonable procedure that obviates joint replacement. Biological reconstruction with bone could help reduce the incidence of subsequent revision surgery that would likely occur in cases treated initially with conventional excision and replacement with megaprostheses or circumferential intercalary allografts.

Recurrent gossypiboma in the thigh.

Gossypiboma, an iatrogenic mass lesion caused by a retained surgical sponge is an extremely rare event following musculoskeletal procedures. This entity is therefore a very unusual experience and can create considerable confusion. Unsuspecting surgeons may thus be caught out by this unlikely presentation. We present our experience with a recurrent gossypiboma in the thigh occurring several years after surgical evacuation of a similar gossypiboma from the same anatomic location with interval resolution of symptoms. The purpose of this case report is to highlight the possibility of a "recurrent" soft tissue mass occurring for reasons other than a neoplasm. In the absence of a definitive biopsy diagnosis of tumor in patients who have undergone prior surgical procedures in that area, it may be more prudent to adopt a conservative surgical resection rather than a conventional radical resection as warranted by the dramatic clinical presentation mimicking a soft tissue sarcoma.

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.