Extra-thoracic, extra-meningeal solitary fibrous tumours - A case series and service review.

BACKGROUND: Solitary fibrous tumours (SFT) are a type of mesenchymal tumour. Whilst the majority of cases follow an indolent course a significant proportion of patients suffer metastases or disease recurrence post-surgical excision. Due to the unpredictable clinical course follow up duration and intensity remains contentious. AIMS: We aimed to determine current outcomes of management of this tumour, apply and assess current risk recurrence models to determine if our standard of care could be improved upon. METHODS AND PATIENTS: A prospective database of patients treated at a regional musculoskeletal oncology service was assessed. Only extra-pleural, extra-meningeal SFTs were included in the study. Surgical outcome and post-operative investigations were scrutinised and the Pasquali and Demicco recurrence risk models were applied and assessed. RESULTS: From 2009 to 2019 12 patients were identified, 8 female and 4 males. Their age at diagnosis ranged from 21 to 76 years. 11 patients underwent surgery with curative intent and no patient suffered disease progression or recurrence, with a mean follow up time of 41 months. One patient presented with metastatic disease and was managed palliatively. CONCLUSIONS: Following this review of our case series and utilising risk recurrence models published in the literature we have changed our follow up protocol. In new cases of SFT the Pasquali prognostic model, with the addition of the presence or absence of necrosis, will be utilised. If a patient has benign features on initial biopsy we propose to not perform staging. Furthermore, if biopsy and final pathology results remain concordant, with no concerning features, and the patient has undergone complete excision reduced intensity follow up could be considered.Level of evidence Level IV, retrospective case series.

An update on mesenchymal tumours of the orbit with an emphasis on the value of molecular/cytogenetic testing.

Mesenchymal tumours of the orbit are uncommon. Beyond childhood primary sarcomas are extremely rare and the literature is limited to case reports and short case series. However there is a diverse assortment of benign and malignant soft tissue tumours that may involve the orbit. Techniques to identify tumour specific cytogenetic or molecular genetic abnormalities often resulting in over- expressed proteins are becoming an increasingly important ancillary technique for these tumours. This review focuses on 3 specific areas: 1. Orbital mesenchymal tumours where cytogenetics are important to reach the correct diagnosis. The majority of these are chromosomal translocations that often result in a fusion gene and protein product; 2. Orbital mesenchymal tumours where cytogenetics are important to identify patients who will do well versus those with a poorer prognosis. This is turn helps with therapeutic options. In some tumours e.g. synovial sarcoma the chromosomal translocations can occur with 2 different regions resulting in different fusion products that carry a different prognosis. Alternatively whilst the majority of alveolar rhadomyosarcomas are fusion positive a minority are fusion negative with a better prognosis; 3. Orbital mesenchymal tumours where the identification of specific cytogenetic abnormalities has resulted in overexpression of specfic proteins which are diagnostically useful biomarkers for immunohistochemistry.

Imaging in osteofibrous dysplasia, osteofibrous dysplasia-like adamantinoma, and classic adamantinoma.

Fibro-osseous lesions of the bone are well-recognized primary bone tumours. However, given the degree of overlap of imaging findings and variation in management of various sub-types, it is a widely accepted practice to perform a biopsy to obtain histopathological confirmation of the diagnosis. The following is a summary of the epidemiology, clinicopathological features, and review of the imaging features of fibro-osseous lesions, including osteofibrous dysplasia, osteofibrous dysplasia-like adamantinoma, adamantinoma, and lesions that closely mimic them. The illustrated examples are histologically proven cases that were presented to a tertiary referral teaching hospital and national bone and soft-tissue tumours unit. It is important that all radiologists are aware of the nature and imaging characteristics of these tumour sub-types, so that suspected lesions are recognized and appropriately referred to specialist bone tumour services for work-up and management.

Abdominal wall fibromatosis associated with previous laparoscopic hernia repair.

CASES: Two cases of desmoid-type fibromatosis developing after laparoscopic hernia repair are described: one in a young male 3 years after laparoscopic umbilical hernia repair and the other in a young female 1 year after laparoscopic incisional hernia repair. FINDINGS: The male patient presented with a slowly enlarging non-tender firm abdominal wall mass; the female patient had similar findings. Excision biopsy in the male and core biopsy in the female were consistent with fibromatosis. TREATMENT: The young male patient underwent resection of the fibromatosis, and the female patient has been managed conservatively. RELEVANCE TO CURRENT KNOWLEDGE: These are the first documented cases of fibromatosis developing after laparoscopic hernia surgery. Whilst the safety of hernia meshes has been assessed in animal studies, it may be that more detailed study of intraperitoneal placement of these meshes is required.

Paget sarcoma of the spine: Scottish Bone Tumor Registry experience.

STUDY DESIGN: Retrospective case study of 13 cases of Paget sarcoma of the spine accrued from a prospectively collected Tumor Registry database. OBJECTIVES: To analyze the clinical, radiologic, and histologic features of Paget sarcoma of the spine and to determine the factors influencing the prognosis. SUMMARY OF BACKGROUND DATA: Paget disease of bone is a common disorder with the spine being involved in over 50% of patients. However, sarcomatous degeneration in the vertebral column is an extremely rare complication. There is very little in the literature with regard to clinical presentation and prognosis of patients with Paget sarcoma affecting the vertebral column. METHODS: Between January 1944 and December 2003, 89 patients were registered with a diagnosis of Paget sarcoma in the Scottish Bone Tumor Registry. Thirteen patients with Paget sarcoma of the spine were analyzed with regard to their clinical, radiologic, and histopathologic features along with the prognostic predictors. RESULTS: The mean age was 66.9 years (range: 56-79 years). There were 10 males and three females. There were seven cases involving the sacral spine (63.6%), three cases involving lumbar vertebrae, two affecting the dorsal spine, and one with diffuse dorsolumbar involvement (D11-L3). The mode of presentation was progressively increasing low back pain (in all 13), unilateral sciatica (six; left-sided, five; right-sided, one), bilateral sciatica (two), lower limb weakness (eight), and autonomic dysfunction (four). Ten of 13 cases (76.9%) were osteosarcoma. The rest were chondrosarcoma (n = 1), fibrosarcoma (n = 1), and malignant fibrous histiocytoma (n = 1). Decompression laminectomy was performed in three patients with progressive neurologic deficit. Eight patients had received radiotherapy. The mean survival was 4.22 months. CONCLUSIONS: This series confirmed that Paget sarcoma of the spine has a very poor prognosis. We found a constellation of symptomatology in patients with sarcomatous Paget spine resulting from radiculomedullary compression, primarily lumbosacral involvement and predominantly osteosarcomatous histology. There was no significant difference observed on the overall prognosis of the patients with Paget sarcoma of the spine in the last 6 decades.

Sarcomatous change in the Pagetoid tibiae.

We reviewed 13 cases of tibial Paget's sarcoma constituting 14% of all registered Paget's sarcoma cases of the Scottish Bone Tumour Registry between January 1947 and June 2004. Eleven patients were male, and in ten patients the tumour involved the upper half of the tibia. In all cases, the main presenting feature was progressively worsening pain followed by a mass in six, a pathological fracture in five and tibial bowing in four. In nine patients, a lytic lesion was seen radiologically. Histologically, there were three osteosarcomas and ten malignant fibrous histiocytomas. Limb ablation was carried out in 11 patients, of whom nine had trans-femoral amputation. Six patients received adjuvant radiotherapy and/or chemotherapy. Post-operative complications included stump revision in two cases, non-union of a pathological fracture of the tibial tuberosity and a stress fracture. The median survival was 17 months.