The biological response to a failed extra-articular polyester ligament used for AC Joint reconstruction at the shoulder girdle: a retrieval analysis of five cases.

The LockDown device (previously called Surgilig) is a braided polyester mesh which is mostly used to reconstruct the dislocated acromioclavicular joint. More than 11,000 have been implanted worldwide. Little is known about the tissue reaction to the device nor to its wear products when implanted in an extra-articular site in humans. This is of importance as an adverse immunological reaction could result in osteolysis or damage to the local tissues, thereby affecting the longevity of the implant. We analysed the histology of five LockDown implants retrieved from five patients over the last seven years by one of the senior authors. Routine analysis was carried out in all five cases and immunohistochemistry in one. The LockDown device acts as a scaffold for connective tissue which forms an investing fibrous pseudoligament. The immunological response at the histological level seems favourable with a limited histiocytic and giant cell response to micron-sized wear particles. The connective tissue envelope around the implant is less organised than a native ligament.

Extra-abdominal desmoid fibromatosis--a sarcoma unit review of practice, long term recurrence rates and survival.

INTRODUCTION: Desmoid fibromatosis (DF) carries a significant morbidity and a recognised mortality. Despite this there are currently limited diagnostic or treatment algorithms specific to cases of extra-abdominal DF. Historically surgical excision has formed the cornerstone of treatment. Recently however a paradigm shift has meant many practitioners now adopt a more conservative approach, placing emphasis on active surveillance, function preserving resections, and non-surgical oncologic therapies. METHODS: We performed an 8-year retrospective review of all cases of extra-abdominal DF managed within our region to assess the consistency of diagnostics, management and long-term outcome. RESULTS: 47 eligible cases were identified. Mean age at diagnosis was 41.3 years (1-81 years). Disease location and speciality of diagnosing practitioners were varied. Management was generally inconsistent. Variation was seen in imaging, biopsy techniques, MDT involvement and management. At a median follow up of 4.9 years our local recurrence rate was 19%. DISCUSSION: The optimal management of DF is unknown. This has led to a lack of formalised guidance for practitioners managing this challenging condition, resulting in inconsistencies and areas for improvement in current management. We propose a diagnostic pathway which may improve consistency of care, reduce potentially unnecessary surgery and the associated morbidity, and significantly increase the rate of complete (R0) surgical resections when surgery is deemed appropriate whilst not significantly worsening oncological outcome. Specifically we propose all cases should be imaged appropriately (usually with MRI), undergo a planned biopsy (by radiologically guided core needle biopsy) and be managed centrally in conjunction with multidisciplinary sarcoma units.

Tumour-to-tumour metastasis of laryngeal leiomyosarcoma to an axillary hibernoma.

Tumour-to-tumour metastasis is a rare, but well-recognised occurrence. This case report documents the metastasis of a primary laryngeal leiomyosarcoma to a hibernoma. We believe that this is the first recorded case of leiomyosarcoma metastasising to another neoplasm, and the first recorded case of a hibernoma acting as a recipient tumour for metastasis. This case study emphasises the importance of re-imaging a known benign mass in the presence of new symptoms in a patient with underlying malignancy, to ensure prompt diagnosis and management of potentially treatable metastasis. The imaging findings including whole body magnetic resonance imaging (MRI) staging, macroscopic and histological features are presented.

Inadvertent surgical resection of soft tissue sarcomas.

BACKGROUND: The National Institute of Clinical Excellence (NICE) published Improving Outcome Guidance in 2006 defining urgent referral criteria for soft tissue sarcoma (STSs) with the twin aims of improving diagnostic accuracy and overall outcome. Despite these guidelines inadvertent excisions of soft tissue sarcomas continue to occur with alarming frequency, potentially compromising patient outcomes. OBJECTIVE: We reviewed the East Midlands Sarcoma Service experience of treating inadvertent excision of STSs and highlight the patient profile, referral pattern, subsequent management and oncological outcome associated with inadvertent resection. METHODS: Patients were identified from our sarcoma database and a retrospective case note review performed. RESULTS: Over a 3-year period, 42 patients presented to our specialist centre after unplanned excision of soft tissue sarcomas. There were 29 men and 13 women, with a mean age at presentation of 59 years (19-90). 50% of the tumours were located in lower extremity, 33% around the trunk and 17% in the upper extremity. The unplanned surgery was most commonly from general surgeons, plastic surgeons, orthopaedic surgeons, general practitioners followed by vascular surgeons. Re-resection was undertaken in 40 cases to achieve clear margins with residual tumour present in 74% of cases. Limb salvage surgery was not possible in 5 cases. CONCLUSION: Unplanned excision of sarcoma by non-oncologic surgeons remains a problem. It appears that it is equally prevalent in varied surgical community and general practitioners. Excision of large or deep solid soft tissue masses without tissue diagnosis is unacceptable.

Epithelioid perineurioma: an unusual variant.

An unusual epithelioid variant of perineurioma of the groin occurring in a 53-year-old man is described. The lesion appeared to be associated with a femoral nerve branch. The tumour was characterised by the presence of a syncytial proliferation of epithelioid cells, mimicking a meningioma of syncytial type. In addition there was a minor component of a conventional perineurioma. The tumour cells were EMA+, claudin-1+ and collagen type IV+. Bcl 2 was focally expressed. This case highlights the possibility of a common histogenetic pathway for meningiomas and perineuriomas. Although ultrastructural evidence of possible meningiomatous differentiation within an otherwise histologically typical perineurioma has been described, this is the first reported case of an unconventional epithelioid variant of perineurioma, histologically resembling meningioma.

Colonic angiomyolipoma with a monotypic expression and a predominant epithelioid component.

Angiomyolipomas are rare lesions, often arising in the kidney, and are part of a group of tumours with a diverse appearance and evidence of dual melanocytic and smooth muscle differentiation known as PEComas (tumours of perivascular epithelioid cell origin). This report describes an unusual case of a colonic PEComa in a 40 year old woman. Unlike most of the previous colonic angiomyolipomas/PEComas reported in the literature, this case formed a large, mainly extrinsic mass and was monotypic, and composed entirely of the myomatous component with no adipose tissue or typical vasculature.

Operation notes illustrated with digital images.

We would like to report on our experience of illustrating our operation notes with pre-, per- and post-operative digital images.

Extra-abdominal desmoid tumour of the breast: review of the primary management and the implications for breast reconstruction.

This case report illustrates the presentation and management of an extra-abdominal desmoid tumour of the breast. A review of the literature describing the aetiology, pathology and risk of recurrence was undertaken to determine how current understanding of this rare tumour may affect the management of patients, should they require breast reconstruction after radical excision of the primary tumour. The natural progression of the disease is variable and there are no markers predictive of recurrence or regression. Primary lesions should be assessed with respect to their anatomical site of origin (i.e. whether they arise within the breast or invade the breast from the underlying musculo-aponeurotic tissue) and the extent of local invasion. Radical excision of the tumour with clear histological margins decreases the likelihood of recurrence. Tumours arising from the musculo-aponeurotic system have increased risks of recurrence and of developing multifocal primary tumours in specific anatomical territories. Local recurrences should be assessed for extent and anatomical distribution, and radical excision performed as for a primary tumour. Radiotherapy can be used as an alternative treatment if radical excision of a primary or recurrent tumour would cause severe functional loss or mutilation. Radiotherapy can be used for positive histological margins following tumour excision. There is a higher risk of recurrence in the first 3 years after primary excision, and breast reconstruction may be best delayed for this period. Surgical trauma has been implicated in the aetiology of recurrence and the patient should be informed of this prior to breast reconstruction.

Malignant melanoma in a skin graft: burn scar neoplasm or a transferred melanoma?

Malignant melanomas (MM) arising in burn scars are rare with 16 cases previously reported. Malignant melanomas arising on skin grafts are even more rare with only two cases reported. We present the case of MM arising on a burned area that had been previously grafted with a split thickness skin graft. A 19-year-old patient sustained 20% burns in a road traffic accident. The burned areas were debrided and skin grafted. Six months later, the patient developed MM on the left calf (an area that was burned and grafted). The tumour was excised with wide margins. Six months following the excision of the MM, the patient started to develop multiple dysplastic naevi in the skin grafted burned areas. In the present case, the main question to be answered is whether the MM arose from the donor or the recipient site of the split thickness skin graft. After thorough discussion of the two options and reviewing the literature, the authors believe that the MM and the atypical naevi were transferred to the recipient site with the skin graft. Therefore, it is suggested that in the process of harvesting skin grafts, any pre-existing naevi should be avoided or removed, and if this is not feasible, should be recorded in detail in the operation notes. Also, patients at discharge should be advised that any change in the appearance of the grafts or any new lesions in the engrafted areas should be reported to their physicians.

Second primary malignant melanoma on a skin graft used to cover the defect of the first primary's wide excision.

The unique case of a second primary malignant melanoma developing on a split thickness skin graft that had been used for reconstruction of the defect following the wide excision of a first primary malignant melanoma is reported. A review of the literature revealed that no similar case has been reported before.

Evaluation of a statistically derived decision tree for the cytodiagnosis of fine needle aspirates of the breast (FNAB).

A decision tree for the diagnosis of FNAB was derived from defined human observations using a rule induction method, C4.5 (a derivative of the ID3 algorithm). This algorithm is an implementation of the top-down induction method where the tree is determined iteratively by adding those nodes and branches which maximize the information gain at each step. The tree was derived from a training set of 200 FNAB with known outcome using 10 defined features (from one observer) and patient age. The tree contained a total of seven nodes (six observable features and patient age) with eight endpoints (four benign, four malignant). The tree was applied to a test set of 400 further FNAB with observations from the training observer and produced a sensitivity of 95%, specificity of 93% and a positive predictive value (PPV) of a malignant result of 89%. Four trainee pathologists were given a training session on the observable features and then used the tree to determine outcome in a further 50 FNAB. The observers were blind to clinical details apart from age and the endpoints were coded with letters and not labelled benign or malignant. The results from these observers produced ranges of sensitivity 80-96%, specificity 64-92%, PPV 73-92% and kappa statistics (with known outcome) 0.6-0.8. Reported difficulties in using the tree included estimation of nuclear size. These results were worse than the performance of the observers on a further 50 cases without using the decision tree (sensitivity 80-100%, specificity 72-100%, PPV 78-100%, kappa 0.72-0.92). The original 50 case test set was rerandomized and the four trainee observers made all 10 defined observations on each specimen without using the decision tree; these observations were then used to derive decisions from the tree. The performance from this method was similar to that using selected features from the tree, suggesting that observation of all features together does not improve the reliability of each specific observation. The poor performance of this tree suggests that this methodology may be unsuitable for producing decision support aids for diagnostic or training purposes in this domain.

Postmortem examination of the lungs: a preservation technique for opening the bronchi and pulmonary arteries individually without transection problems.

The classic methods of examining both the pulmonary arteries and bronchi postmortem partly destroy the anatomy of one of these systems. A technique is described whereby the bronchi and pulmonary arteries are dissected and preserved. The principal difference of this technique is that most of the upper lobe vasculature is opened from the hilum. The exception to this is the lingula, which because it is the embryological homologue of the right middle lobe should be treated in the same way--that is, with the vessels opened from the pleural side and the bronchi from the hilum. One general practical point is that there is a great variation in the number of pulmonary arterial branches in each lobe, particularly in the upper lobes, and especially in the left upper lobe. Furthermore, there is some variation in the local anatomic relations of the vessels and bronchi and on occasion the technique will not work as described and will require some modification. However, most of the time this technique should allow the pathologist to preserve the anatomy of the bronchial tree and pulmonary arteries for better demonstration and photographic purposes when the need arises.

When is bacterial vaginosis not bacterial vaginosis?--a case of cervical carcinoma presenting as recurrent vaginal anaerobic infection.

Vaginal anaerobic infection is the most common cause of vaginal discharge in women. We present a case of recurrent vaginal anaerobic infection and cervical carcinoma and discuss the association of the two conditions. More frequent cytology/colposcopy may be indicated in women who give a history of recurrent or persistent vaginal anaerobic infection.

Gastric outflow obstruction caused by gall stones and leading to death by complex metabolic derangement.

A 67 year old woman was admitted with a three week history of vomiting, having become increasingly confused for three days. Investigations revealed deranged serum biochemistry consistent with a combination of a diabetic non-ketotic hyperosmolar state and a metabolic alkalosis consistent with gastric outflow obstruction. She was treated with intravenous saline, intravenous insulin, and subcutaneous heparin, but did not improve clinically and had an asystolic cardiac arrest the following day; she was transferred to the intensive care unit and despite treatment with inotropes she died 40 hours after admission. Necropsy revealed that the stomach was massively dilated with gas and stomach contents, and contained many small black faceted gall stones. In addition a large nonfaceted brown-yellow gall stone was wedged in the pyloric antrum causing total obstruction. The patient had died from a complex metabolic derangement including non-ketotic hyperosmotic diabetic coma and metabolic alkalosis precipitated by the acute gastric outflow obstruction complicated by previously undiagnosed type II diabetes mellitus.

Early measurement of interstitial fibrosis predicts long-term renal function and graft survival in renal transplantation.

This study investigated the relationships between renal allograft interstitial fibrosis, renal function and graft survival. A total of 107 consecutive renal transplant recipients immunosuppressed with cyclosporin were studied. Needle core transplant biopsies were performed before operation and at 1, 6 and 12 months after transplantation. Allograft fibrosis was assessed by histomorphometric analysis of graft interstitial volume fraction. Renal function was measured by isotopic glomerular filtration rate (GFR) measurement at the same time points. Interstitial volume fraction was already high in preperfusion biopsies, significantly increased with time but stabilized at 6 months after transplantation. GFR correlated negatively with interstitial volume fraction at 6 months (P = 0.05). Interstitial volume fraction at 1 month was not a useful predictor of subsequent graft survival but for allografts surviving to 6 months an interstitial volume fraction above 25 per cent predicted significantly poorer survival (P = 0.04). It provides an objective measure of chronic allograft damage and may prove to be a useful surrogate endpoint in the study of therapeutic intervention.

Pilomatrix carcinoma and multiple pilomatrixomas.

Pilomatrixoma is the only skin appendage tumour showing predominantly hair matrix differentiation. Rarely, aggressive or malignant variants are reported in the literature. We report a case of a 44-year-old male with multiple pilomatrixomas, one of which grew rapidly, recurring several times. Histological examination of this tumour showed predominantly hair matrix differentiation but also abortive hair follicle formation with pilar keratinization reminiscent of a trichoepithelioma. Numerous mitoses and moderate cellular pleomorphism were present, associated with infiltration of subcutaneous skeletal muscle and vascular invasion.

Analysis of necropsy request behaviour of clinicians.

AIM: To develop a necropsy related audit system to record accurate information in relation to necropsy requests, necropsy rates and coronial referrals. METHODS: A simple audit form was used to record detailed necropsy related data via an integrated questionnaire design and data entry system based on available optical image scanning technology. The system recorded the numbers and locations of deaths, referrals to the coroner, clinical necropsy requests, hospital and medicolegal necropsies, the grade of clinician involved in these processes, and the identity of the consultant in charge of the case. The overall, hospital and medicolegal necropsy rates were calculated by individual consultant, specialty and for the whole hospital. Necropsy request rates and coronial referral rates were also calculated and these data were related to the grade of clinician. All data were available on a monthly or an accumulative basis. RESULTS: Of 1398 deaths, 534 (38%) were discussed with the local coroner's office and 167 of these were accepted for further investigation. House officers and senior house officers referred over 80% of all cases, whereas consultants referred only 2%. There were no significant differences in case acceptance rates by grade of clinician. Clinicians made 307 hospital necropsy requests (overall hospital necropsy request rate 22%). House officers made 65% of all necropsy requests. Consultant necropsy requests represented 13% of all requests. There were no significant differences in necropsy request success rates by grade of clinician. CONCLUSIONS: The referral of cases to coroners and clinical necropsy requests are still being inappropriately delegated to the most junior clinicians. This study illustrates the type of useful information which can be produced for individual clinicians, specialty audit groups and pathology departments using a simple necropsy related audit system.