Reconstruction of the Scapula in Pediatric and Adolescent Patients After Total Scapulectomy. A Report of 10 Patients Treated by Extracorporeal Irradiation and Reimplantation of the Scapula.

BACKGROUND: Ewing sarcoma of the scapula is uncommon. Restoration of shoulder function after total scapulectomy is challenging. Extracorporeal irradiation and reimplantation is a well-known method of biological reconstruction in orthopaedic oncology; yet very few reports in the literature describe its specific use in tumors of the scapula. PATIENTS AND METHODS: Ten patients with the diagnosis of Ewing sarcoma of the scapula were surgically treated by total scapulectomy and extracorporeal irradiation and reimplantation of the scapula. The mean age at presentation was 10 years (3 to 15 y). Six patients were male individuals and 4 were female individuals. An extracorporeal single dose of 5000 cGy was applied to each graft. At reimplantation, internal fixation was used in the first 2 cases, whereas, in the following 8 patients, stabilization was carried out by resuturing of the glenohumeral and acromioclavicular joint capsules without internal fixation. An ipsilateral Latissimus Dorsi muscle flap was used in all patients to cover the irradiated scapula. RESULTS: The mean follow-up duration was 29.2 months (13 to 50 m). At the latest follow-up, 8 patients were continuously disease free and 2 patients had systemic relapse. No patient had local recurrence. The mean musculoskeletal tumor society functional score was 87% (66.6% to 100%). Eight of the 10 patients (80%) could actively elevate the upper limb up to and beyond 90 degrees in both the forward and scapular planes. Complications included wound gaping in 2 patients (20%), dislocation of the acromioclavicular joint in 3 patients (30%), and partial resorption of the scapular graft in 5 patients (50%). No patient showed any progressive deterioration of his or her shoulder function throughout the follow-up period. CONCLUSIONS: The irradiated scapular graft provides a stable biological fulcrum for the reattached muscles of the shoulder joint after total scapulectomy, thereby providing an adequate postoperative range of shoulder elevation. A longer follow-up study is needed to evaluate the progression and effect of graft resorption on the functional outcome. LEVEL OF EVIDENCE: Level IV.

Low-grade Nonrhabdomyosarcoma Soft Tissue Sarcoma: What is Peculiar for Childhood.

BACKGROUND: Nearly half of soft tissue sarcomas are nonrhabdomyosarcomas (NRSTSs). The low-grade (LG) form comprises a heterogenous group of diseases that rarely metastasize but are known for local recurrence. AIM OF THE STUDY: The aim of the study was to retrospectively evaluate pediatric LG-NRSTS with regard to demography, survival, and factors affecting outcome in Egyptian patients. PATIENTS AND METHODS: The study reviewed 66 NRSTS patients who presented to the Pediatric Oncology Department, National Cancer Institute, Cairo University, between January 2008 and December 2013. RESULTS: Out of the reviewed cases 32 patients had LG tumors and were eligible for analysis. The male to female ratio was 1:1 and the median age was 7.5 years (range, 1 mo to 18 y). Desmoid fibromatosis (N=18) showed frequent local recurrence and nearly half of this group was alive without disease. No recurrence of the disease occurred in the nonfibromatosis group (n=14) and all patients were alive and free of disease. The 5-year overall survival was 88% for the entire group of study patients versus 45% for event-free survival. Tumors >5 cm in diameter and fibromatosis histology subtype were associated with lower EFS. CONCLUSIONS: LG-NRSTS generally has good prognosis, with overall survival reaching 90%. However, aggressive fibromatosis usually runs a poorer course in the form of high incidence of local recurrence and lower survival rates. This needs to be further assessed in larger prospective studies including novel therapies in addition to the current conventional modalities.

Groin recurrence in patients with early vulvar cancer following superficial inguinal node dissection.

OBJECTIVE: To investigate the causes of groin recurrence in patients with vulval cancer who previously had negative nodes following superficial inguinal node dissection (SIND). MATERIAL AND METHODS: Forty-one patients with squamous cell carcinoma of the vulva (stage I or II) were operated upon. The primary treatment was wide local excision with 2 cm safety margin and superficial inguinal lymphadenectomy. Six patients had ipsilateral and one patient had bilateral groin recurrence. Those patients were subjected to deep inguinal node dissection (one patient required bilateral node dissection). RESULTS: The mean age at time of diagnosis was 59 years (range 51-68). The median follow-up period for all patients was 63 months (range 24-71) and that of the recurrent cases was 20 months (range 12-38). The mean depth of invasion of the recurrent cases was 5.5mm (range 5-5.9 mm) and the mean diameter of the primary tumor in recurrent cases was 3.8 cm (range 3-4.5 cm). All recurrent cases had a high grade of the primary tumor. The median interval to recurrence was 21 months (range 12-57). The groin recurrence rate after negative SIND was 17% (7/41 patients).The mean number of nodes resected per groin was eight (range 1-17). The nodes ranged in size from 0.2 to 4.0 cm. CONCLUSION: Carcinoma of the vulva with the following criteria (size of tumor is greater than 3 cm, depth of invasion greater than 5mm, and high grade tumors) is at high risk of recurrence.

Limb sparing surgical resection of groin sarcoma. Surgical approach and reconstructive options.

PURPOSE: The aim of this study is to evaluate limb sparing surgical resection and reconstructive options in a group of patients having soft tissue sarcoma of the groin and nearly most of them were previously subjected elsewhere to some sort of mismanagement. PATIENTS AND METHODS: Between 2001 and 2006, 14 patients having soft tissue sarcoma of the groin presented to National Cancer Institute with some sort of mismanagement elsewhere. Preoperative reevaluation included CT chest, MRI or MRA, Doppler US and angiography in some selected patients. According to the Enneking staging system, 9 patients had stage II, 4 had stage IIA and 1 patient had stage III. Limb sparing resection was done including wide resection of the tumor enbloc with the pubic bone or its rami and involved femoral vessels and nerve. Abdominal wall defect was reconstructed by mesh, skin defect was reconstructed by local myocutaneous flaps and vascular replacement was done by vascular prosthesis. RESULTS: The mean follow-up period was 31 months (range 25-53 months). Surgical margins were negative in 13 patients and microscopically positive in one patient. Femoral nerve was resected in 3 cases. Pubic bone resection was done in all patients. Vascular resection and prothetic replacement were done in 2 cases. Ten cases required myocutaneous flap reconstruction of skin defect, 2 cases required muscle flap only. All mobilized flaps showed no failure. Complications included seroma in all cases, superficial stitch gaping in 3 cases, wound breakdown and deep infection occurred in one case and chronic lymphedema in 5 cases. Limb sparing function according to MSTS functional score ranged from 92 % to 97 %. The 2 year local control rate was 92.8 % and the 2 years survival rate was 85.7 %. CONCLUSION: Patients having groin sarcoma with some sort of improper management may still have a chance of successful limb sparing surgical resection with a curative intent and achievement of good functional results. This requires proper preoperative reevaluation of the extension of the tumor and vascular involvement, wide retroperitoneal exposure to maximize tumor resection, vascular replacement if ileofemoral vessels are involved and local myocutaneous flap transfer to minimize the problems of wound healing and subsequent infection. KEY WORDS: Groin sarcoma - Limb salvage - Groin defect reconstruction.

Long term behavior of pedicled vascularized fibular grafts in reconstruction of middle and distal tibia after resection of malignant bone tumors.

OBJECTIVE: The aim of this study was to evaluate the long term behavior of pedicled vascualrized fibula graft in reconstruction of middle and distal tibia defects following malignant tumor resection with particular emphasis on success in limb salvage and the specific late related complications. PATIENTS AND METHODS: Between 1997 and 2006, 16 patients having malignant bone tumors of the middle tibia (10 cases) and distal tibia (6 cases) were candidate for wide resection of their tumors and reconstruction of the bony defect by ipsilateral vascularized pedicled fibular graft based on the peroneal vessels. There were 9 males and 7 females with a mean age of 13 years at time of surgery (range 11-23 years). Mean follow up period was 52 months (range 32 -110 months). Fixation was done by cast in 14 cases and by plate and screws in 2 patients. According to the Enneking staging system (1), 9 patients had stage IIB and 7 had stage IIA. Bony union and hypertrophy were assessed radio graphically on regular basis. Hypertrophy was estimated in a percentage. Functional limb results were also reported according to MSTS functional scores [2]. RESULTS: The mean length of bony gap bridged was 14 cm (range of 11.0-16.0 cm) and the mean length of fibula harvested was 15.5 cm (range 12-17 cm). Ninety seven percent of patients (15 cases) healed primarily at a mean time of 4.8 months (range 3.5-6 months). Hypertrophy was evident in all patients and ranged from 60 to 210% (mean 91% ). Increase in size of the hypertrophied fibula beyond the recipient bone was noticed in three cases (18.7% ) and we relate this to weight-bearing forces and not to the size of the recipient bone. The mean time of the fibula to double its size (10 cases) was 21 months (range 18-31 months). Young patients developed full hypertrophy earlier than older patients. Complication rate was low. One patient had non union at the proximal end of the fibulotibial junction and two patients had stress fracture of the fibula. CONCLUSION: Long term follow-up of pedicled vascularized fibula in reconstruction of bony defects of the middle or distal tibia after bone tumor resection showed that it is a useful tool in the limb salvage procedure. It is a short procedure, inexpensive, with low rate of late complications. It has a good outcome regarding the union, hypertrophy and the functional outcome.

Pedicled gastrocnemius flap: clinical application in limb sparing surgical resection of sarcoma around the knee region and popliteal fossa.

OBJECTIVE: To highlight on the versatility of superiorly based pedicled gastrocnemius muscle flap in the limb-sparing surgery for bone or soft tissue sarcoma around the knee and popliteal fossa. PATIENTS AND METHODS: A total of 30 patients with localized bone or soft tissue sarcoma around the knee and popliteal fossa were treated with limb-salvage procedure. The study included 5 cases with bone sarcoma of the distal femur, 15 cases having bone sarcoma of proximal tibia and 10 cases having soft tissue sarcoma around the knee region and popliteal fossa. Routine preoperative staging studies were done for every patient and included local plain radiography, local MRI, isotopic bone scan and CT chest. Local MRA or angiography was done in selected cases. According to the Enneking staging system, 19 patients had stage IIB and 11 had stage IIA. Patients having bone sarcoma of the proximal tibia were subjected to wide resection, endoprosthetic reconstruction and reconstruction of the extensor mechanism by the medial gastrocnemius muscle flap. Patients having bone sarcoma of the distal femur were subjected to wide resection, endo prosthetic reconstruction and coverage of the prosthesis and rebalance of the patellar tendon by the medial gastrocnemius flap. Patients having soft tissue sarcoma were subjected to wide resection and soft tissue coverage with either medial or lateral myocutaneous gastrocnemius flap or muscle flap with grafting. Limb function was evaluated according to MSTS functional scores. Adjuvant chemotherapy or radiotherapy was given according to nationally-agreed protocols. RESULTS: There were 18 males and 12 females with a mean age of 29 years at the time of surgery (range 11-44 years). The mean follow-up period was 52 months (range 25-72 months). Resection with a negative bony and soft tissue margins could be achieved in all cases. A total of 30 flaps were used and included medial gastrocnemius muscle flaps in 21 cases (15 cases had proximal tibia endoprothesis, 5 cases had distal femur endoprothesis, 2 cases had soft tissue sarcoma infiltrating the chin of tibia), myocutaneous gastrocnemius flaps in 8 cases having soft tissue sarcoma (5 had medial head flap and 3 cases lateral head flap). Flap survival was 100&. The success rate of limb salvage was 96.6% with high score functional results according to MSTS functional scores. The area of the skin island harvested in cases of myocutaneous flaps ranged from 11 to 18cm in length and from 5 to 8cm in width and all these cases required grafting of the donor site. Complications included partial wound dehiscence in one case, revision of the prosthesis in one case, amputation due to local recurrence in one case and significant extension lag in 2 cases. CONCLUSION: The unique vascularization of the gastrocnemius muscle (one pedicle to each head), the size of the muscle belly, the fact that it is situated in the dissection field and that its transfer does not affect the function of the spared limb too adversely, makes it particularly suitable for limb sparing procedures for sarcoma in the region of the knee and popliteal fossa. The medial head is the workhorse muscle flap for soft tissue coverage of knee endoprothesis and reconstruction of extensor mechanism. This decreases the rate of complications and improves the functional outcome. Reconstruction of the soft issue defect improves wound healing, protects exposed deep structures and subsequently prevents wound problems, delays adjuvant therapy and secondary amputation.

Reconstruction of the proximal humerus after wide resection of tumors: comparison of three reconstructive options.

PURPOSE: Assessment of the functional results and complications of three bone reconstructive procedures after resection of primary tumors of the proximal humerus. MATERIAL AND METHODS: Between 2000 and 2008, 32 patients having primary malignant, aggressive benign or metastatic tumors of the proximal humerous were selected for limb sparing surgery. Preoperative evaluation included CT chest, MRI. Limb sparing surgical resection was done including intraarticular or extraarticular wide resection of the tumor. Bone defect was reconstructed with fusion shoulder using free vascularized fibular graft (FFFG) in 11 patients and pedicled lateral scapular crest graft (PLSCG) in 8 patients and mobile shoulder reconstruction using proximal humerus prosthesis in 13 patients. Those patients were followed-up at regular interval to detect bone union and complications related to bone flaps or prosthesis. Functional results were assessed for every patient after one year postoperatively. RESULTS: The median age of the patients was 21 years and the follow-up period ranged from 19 months to 92 months. Postoperative resection margins were negative in all cases. The mean length of the resected humerus was 13cm. The mean operative time for prosthesis cases was 3.5 hours and that for FVFG was 6.5 hours and was 5 hours for PLSCG cases. The mean time of bone union proximally and distally for FVFG and PLSCG was 4.2 and 5.5 months accordingly. At 1 year follow-up, the functional results for the three reconstructive procedures were nearly the same with a mean functional score for FVFG cases was 73%, for PLSCG cases was 68% and was 71% for prosthesis cases. Hand and elbow functions were preserved in all types of reconstruction. The range of shoulder abduction and flexion was grossly limited with prosthesis cases while it showed marked improvement with fusion by FVFG and PLSCG. Complications for prosthesis cases were one case proximal migration and one case posterior sublaxation. Complications of bone flaps were nonunion of one end in 2 cases of PLSCG and one case of FVFG. Limb shortening was seen in 32 cases with PLSCG. CONCLUSION: Summarizing our experience in this small study, we can state that, limb sparing surgical resection of tumors of the proximal humerus is effective in local tumor control and preserving hand and elbow movements. MSTS functional score of the limb after reconstruction and shoulder fusion with FVFG or PLSCG is nearly the same as mobile shoulder reconstruction with endoprosthesis. Shoulder range of movement was grossly limited with prosthesis replacement while a wider range of movement was obtained with shoulder fusion. Pedicled lateral scapular crest graft is able to bridge defects only up to 12cm whereas the fibula can replace a longer defect. Selection between those reconstructive options should be individualized and is based on the age, the length of defect, the type of resection, intraarticular or extraarticular, preservation of deltoid and axillary nerve, the prognosis of the tumor. KEY WORDS: Proximal humerus tumors - Reconstruction - Limb salvage.

Detubularized sigmoid neobladder versus detubularized W-shaped ileal neobladder as a bladder substitute after radical cystectomy for carcinoma of the urinary bladder: a study of 60 patients.

OBJECTIVES: To compare the urodynamic parameters, continence rates, and complications between two different continent urinary reservoirs-the sigmoid neobladder (SN) and W-shaped ileal neobladder (IN). MATERIAL AND METHODS: Sixty patients with bladder cancer underwent radical cystectomy and were divided into two groups. The first group comprised 33 patients who underwent detubularized sigmoid neobladder surgery. The second group included 27 patients who underwent detubuarized W-shaped ileal neobladder surgery. RESULTS: Early post-operative complications occurred in 42% of patients in the sigmoid neobladder (SN) group and in 33% of patients in the ileal neobladder (IN) group. Most of these complications were minor, mainly due to wound infection or urinary leak, and were managed conservatively. No early post-operative mortality occurred in patients of either group. Day-time continence was achieved in 78.8% and 74% of patients in the SN group and the IN groups, respectively. Night-time continence was achieved in 45.5% of patients in the former group and 59.3% of patients in the latter. The mean neobladder capacity was 472.5cc in patients who underwent detubularized SN surgery and 463.8cc in patients who underwent detubularized IN surgery. The mean post-voiding residual volume was slightly lower in SN group (90.1cc) than in those of IN group (93.7cc). The pelvicalyceal systems were preserved in 94% of patients in the SN group and in 96.3% of patients in the IN group. No significant metabolic disturbances occurred in patients of either group. CONCLUSIONS: Both methods are good alternatives as a bladder substitute. Surgeon preference, ease of construction and length of mesentery are among the factors that must be considered when choosing between them.