Impact of specialist management on survival from radiation-associated angiosarcoma of the breast.

BACKGROUND: Radiation-associated angiosarcoma of the breast (RAAS) is a rare complication of adjuvant radiotherapy associated with poor survival. The British Sarcoma Group guidelines recommend that all angiosarcomas are referred to a sarcoma multidisciplinary team, although there is no recommendation that patients are managed within a sarcoma service. The aims of this study were to compare survival, complete excision rates and local recurrence rates of patients managed within a sarcoma service and those managed within local hospitals. METHODS: All patients with RAAS referred to a regional sarcoma service between 1998 and 2015 were identified from prospective databases. Patient records, and radiology, pathology and operation notes were reviewed retrospectively. RESULTS: Thirty-six patients were operated on with curative intent; 26 were managed by the sarcoma service (of whom 21 underwent radical excision of the irradiated field followed by chest wall reconstruction) and ten were managed locally. Median age was 69·5 (range 43-85) years. Disease-specific survival was significantly longer in patients managed by the sarcoma service than in those managed locally: median 91·1 (range 69·2-113·0) versus 48·8 (18·6-79·1) months respectively (P = 0·012). Overall survival rates were similar (P = 0·112). There was no difference in complete excision rate (18 of 26 in sarcoma service versus 5 of 10 in local services; P = 0·456), although the local recurrence rate was significantly lower among patients managed by the sarcoma service (9 of 26 versus 8 of 10; P = 0·015). CONCLUSION: Specialist management of RAAS leads to fewer local recurrences and improved disease-specific survival. Early referral and management within specialist units is recommended.

Vascularized fibular epiphyseal transfer for proximal humeral reconstruction in children with a primary sarcoma of bone.

Aims: Preserving growth following limb-salvage surgery of the upper limb in children remains a challenge. Vascularized autografts may provide rapid biological incorporation with the potential for growth and longevity. In this study, we aimed to describe the outcomes following proximal humeral reconstruction with a vascularized fibular epiphyseal transfer in children with a primary sarcoma of bone. We also aimed to quantify the hypertrophy of the graft and the annual growth, and to determine the functional outcomes of the neoglenofibular joint. Patients and Methods: We retrospectively analyzed 11 patients who underwent this procedure for a primary bone tumour of the proximal humerus between 2004 and 2015. Six had Ewing's sarcoma and five had osteosarcoma. Their mean age at the time of surgery was five years (two to eight). The mean follow-up was 5.2 years (1 to 12.2). Results: The overall survival at five and ten years was 91% (confidence interval (CI) 95% 75% to 100%). At the time of the final review, ten patients were alive. One with local recurrence and metastasis died one-year post-operatively. Complications included seven fractures, four transient nerve palsies, and two patients developed avascular necrosis of the graft. All the fractures presented within the first postoperative year and united with conservative management. One patient had two further operations for a slipped fibular epiphysis of the autograft, and a hemi-epiphysiodesis for lateral tibial physeal arrest. Hypertrophy and axial growth were evident in nine patients who did not have avascular necrosis of the graft. The mean hypertrophy index was 65% (55% to 82%), and the mean growth was 4.6 mm per annum (2.4 to 7.6) in these nine grafts. At final follow-up, the mean modified functional Musculoskeletal Tumour Society score was 77% (63% to 83%) and the mean Toronto Extremity Salvage Score (TESS) was 84% (65% to 94%). Conclusion: Vascularized fibular epiphyseal transfer preserves function and growth in young children following excision of the proximal humerus for a malignant bone tumour. Function compares favourably to other limb-salvage procedures in children. Longer term analysis is required to determine if this technique proves to be durable into adulthood. Cite this article: Bone Joint J 2018;100-B:535-41.

Infected palmar pits: a rare presentation of Gorlin's syndrome.

This paper presents a rare hand presentation of Gorlin's Syndrome, also known as Naevoid Basal Cell Carcinoma Syndrome, which has not been reported previously. This condition is an autosomal dominant, multisystem condition whose diagnosis is important to ensure further surveillance and treatment of features of the condition not routinely dealt with by hand surgeons. However, hand surgeons should be aware of this condition and those parts of the syndrome which may present to them.

The management of hand injuries caused by sea urchin spines.

Injuries to the hand by sea urchin spines are not commonly seen in the United Kingdom. There are many varieties of sea urchins (Echinoidea) throughout the world. They have a spherical calcium carbonate exoskeleton covered with spines. Certain varieties may be venomous, in particular the flower urchin (Toxopneustes pileolus) found in the Indo-Pacific oceans. Injury may also be caused by the urchin spines or pedicellaria (delicate seizing organs equipped with jaws). A small number of hand injuries associated with sea urchin spines have been reported in the literature.

Redefining gigantomastia.

Gigantomastia is a rare but disabling condition characterised by excessive breast growth. Most definitions of gigantomastia refer to a particular weight of excess breast tissue. We speculate that in gigantomastia the weight of the breasts contributes significantly to the BMI, which has implications for healthcare rationing. This study aims to establish the contribution breast tissue makes to BMI in gigantomastia. In so doing, we propose a new definition of gigantomastia. Retrospective data was collected from the case notes of 68 females who underwent breast reduction or therapeutic mastectomy for gigantomastia. For the purposes of patient inclusion, gigantomastia is arbitrarily defined as excessive breast growth of over 1.5kg per breast. The difference between pre- and post-operative BMI is statistically significant (P<0.001). Mean pre-operative BMI is 38.7 with a mean specimen weight of 4506g. Mean contribution of specimen to body weight is 4.29%. There is no correlation between pre-operative body weight and the percentage contribution the breast resection specimen makes to body weight. Based on our data, we define gigantomastia as excess breast tissue that contributes 3% or more to the patient's total body weight, approximately one standard deviation below the mean. We suggest that the estimated excess breast tissue weight is taken into account when calculating pre-operative BMI in the gigantomastia population. The challenge of estimating excess breast weight pre-operatively may be met by 3D photography coupled with computer-assisted volumetry.

Management of radiation-induced sarcomas in a tertiary referral centre: a review of 25 cases.

INTRODUCTION: Radiation-induced sarcomas are a rare and late complication of radiotherapy for breast carcinoma which often have poor prognosis. METHODS: This study is a retrospective review of 25 patients referred to a regional sarcoma unit between 1978 and 2009. RESULTS: Radiation-induced sarcoma following the diagnosis and treatment of breast cancer occurred after a mean period of 156 months. Anatomical sites involved were the breast, chest wall, clavicle, scapula, humerus and axilla. Twenty one patients had wide local excision followed by chest wall reconstruction, latissimus dorsi flap cover or limb amputation. The estimated five years survival following the diagnosis of the radiation-induced sarcoma was 27% and the local recurrence rate 52%. CONCLUSION: Radiation-induced sarcoma following breast cancer has high local recurrence rate and poor prognosis. They should be managed in a multi-disciplinary setting. Long-term follow-up of patients treated with radiotherapy for breast cancer is therefore advisable.

The management of soft tissue sarcoma.

SUMMARY: Soft tissue sarcomas are a rare group of mesenchymal tumours that display cardinal signs, which can raise suspicion to their diagnosis. Management in a sarcoma treatment centre by its multidisciplinary team has improved outcome. Good local disease control with limb salvage and adjuvant radiotherapy has considerably reduced the morbidity of previous limb amputation. By the early involvement of a plastic surgeon, tissue reconstruction is optimised and wound complications reduced. This article looks at the contemporary management of soft tissue sarcoma and, in particular, its relevance to the plastic surgeon today.

Gigantomastia--a classification and review of the literature.

INTRODUCTION: Gigantomastia is a rare, psychologically and physically disabling condition characterised by excessive breast growth. To date, there is no universal classification or accepted definition for this condition. Many authors cite gigantomastia as breast enlargement that requires reduction of over 1500 g per breast. However, there is discordance in the literature with the weight of reduction ranging from 0.8 to 2 kg, or even a D cup bra size. Practically this is a postoperative definition which is of little use to the clinician in terms of patient management or prognosis. METHOD: We conducted a literature review and meta analysis of all published cases of gigantomastia. We combined this with seven cases managed at Selly Oak Hospital, Birmingham, to give us a series of 115 patients. In order to devise a universally accepted definition of gigantomastia, a survey of 150 plastic surgery consultants within Europe was conducted. Participants were e-mailed a questionnaire, requesting their definition of gigantomastia with an appropriate excision weight. They were also asked to comment on any preoperative measurements taken to establish this predicted weight. CONCLUSION: We propose a classification of gigantomastia based on the cause, management and prognosis of the disease.

Use of bone wax to aid harvesting of chondrocutaneous composite grafts.

For reconstruction of nasal defects, chondrocutaneous grafts harvested from ears are commonly used. Bone wax can be molded easily to create a three-dimensional form of the defect to be reconstructed. The mold then can be held against different parts of either ear to choose a site providing the best match of shape and size. The outline of the mold is drawn on the ear, and an appropriate chondrocutaneous graft can be easily harvested.

The management of compound leg injuries in the West Midlands (UK): are we meeting current guidelines?

The joint British Association of Plastic Surgeons and British Orthopaedic Association (BAPS/BOA) guidelines for the management of patients with open tibia fractures were published in 1991 and 1997 and provided a synopsis of injury epidemiology and best care. We present a retrospective review of 66 patients treated over a 2-year period (January 2001-February 2003) at a regional plastic surgery unit. There were 33 direct admissions and 33 transfers from other hospitals in which the average delay in transfer was 7.8 days (1-28) and in whom 26 (79%) patients had already undergone surgery. Plastic surgeons were involved in 46 (62%) of the 66 patient cohort and 27 (82%) transferred patients. The delay after admission until soft tissue cover was 3.8 days (0-15). Twenty-nine (44%) complications were recorded, 20 (69%) of these were in the transferred group and additional orthopaedic intervention was needed in 11 (42%) of patients who had been operated on in other hospitals. Despite widespread dissemination and teaching of the BAPS/BOA guidelines, complex extremity trauma is often not managed well in our region. There are unacceptable delays in admission, late communication, poor note keeping and follow up. The initial surgery/fixation is often sub-optimal and soft tissue reconstruction has to be considered at the least advantageous time period for the patient, leading to an increased hospital stay and complications.

Alternatives to thumb replantation in three cases of traumatic amputation of the thumb.

Three cases of complete amputation of the thumb are reported in which the amputated distal parts were not suitable for replantation. In all cases there were either complete or incomplete amputations of other digits. Two different techniques were used for thumb reconstruction: 1) pollicization of a partially amputated digit with transposition microsurgery in case 1; and 2) replantation of a less important amputated digit to the thumb stump for cases 2 and 3. These microsurgical efforts successfully restored thumb function in all three patients.