Recurrence of Primary Synovial Chondromatosis (Reichel’s Syndrome) in the Ankle Joint following Surgical Excision

Primary synovial chondromatosis, or Reichel’s syndrome, is a rare benign tumour arising from the synovial lining of a joint. We present the case of a 25-year-old male with Reichel’s syndrome of the ankle, with subsequent recurrence following open retrieval of loose bodies. The initial presentation was of lateral malleolus discomfort which limited moderately strenuous exercise. Clinical examination showed a mild effusion and pain on extremes of movement. Imaging confirmed the presence of multiple loose bodies within the anterior and anterolateral recesses of the ankle. Open removal of 27 loose bodies from the joint was performed, with good postoperative recovery. He represented with pain 9 months later, with imaging of the ankle showing reaccumulation of loose bodies to a lesser extent. A trial of conservative management was opted for. Reichel’s syndrome confined to the ankle is an exceedingly rare diagnosis, with few cases reported in the literature. This case saw the recurrence of the disease in a short time period despite successful surgery in the first instance. Management options to treat recurrence include repeat retrieval of foreign bodies, synovectomy, radiotherapy, or arthrodesis. While the prognosis is favourable, a low risk of malignant potential warrants adequate patient follow-up.

1033 Endobronchial Sclerotic Neurofibroma: A Case Report of a Very Rare Benign Tumour

Introduction Endobronchial neurofibromas are exceedingly rare benign lesions most commonly originating at the trachea. Primary pulmonary tumours of neurogenic origin such as peripheral nerve sheath tumours (PNSTs) are extremely rare, accounting for less than 0.2% of all lung tumours. Intrathoracic PNSTs are usually benign and are commonly found in the posterior mediastinum as schwannomas with female preponderance. We present the first reported occurrence of the sclerotic variant of endobronchial neurofibroma and the approach used in its definitive investigation and management. Case Report A 69-year-old Caucasian male with a 66 pack-year smoking history and a background of alcohol induced liver cirrhosis, peripheral vascular disease and dilated cardiomyopathy presented with dyspnoea and fatigue with severe normocytic anaemia. Computed Tomography (CT) chest, abdomen and pelvis revealed an indeterminate nodule at the secondary carina projecting into the bronchus intermedius (BI). Fibreoptic bronchoscopy showed a polypoid lesion with a vascular appearance. Under general anaesthetic, combined fibreoptic and rigid bronchoscopy was used to remove the lesion with rigid forceps. Gross histological appearance showed an 8x6x5mm pedunculated lesion with a 2mm diameter short stalk and histology of a sclerotic neurofibroma with fibroblasts and collagen, positively staining for S100 and LP10. This patient is due for follow up in 6-month with a CT chest to ensure no tumour recurrence. Conclusions Endobronchial neurofibromas appear highly vascular and demand extra caution at bronchoscopic intervention. Prompt, experienced thoracic surgical and intervention pulmonology input should always be sought. Combined approach of fibreoptic and rigid bronchoscopy allows better control of potential bleeding and the airway.

Atypical presentation of intravascular leiomyomatosis mimicking advanced uterine sarcoma: modified laterally extended endopelvic resection with preservation of pelvic neural structures

Intravascular leiomyomatosis is a rare, benign tumour of myometrial smooth muscle. Despite being non-invasive, these tumours can proliferate aggressively within vascular structures including pelvic vessels, the vena cava and the heart. We discuss a 77-year-old woman presenting with a 9 cm uterine mass extending into the right adnexa and ovarian vein. Following hysteroscopic biopsy, palliative radical surgical resection was performed for suspected stage IV leiomyosarcoma. Tumour extension into the pelvic sidewall and obturator fossa indicated a modified laterally extended endopelvic resection combined with skeletonisation and preservation of the pelvic neurovasculature, ultimately providing a curative procedure with minimal functional neurological morbidity. We present this unusual case to assist in the development of a consensus for optimal case management where formal guidelines are not yet available. We summarise current understanding of intravascular leiomyomatosis and highlight the value of advanced surgical techniques using knowledge of complex ontogenetic and pelvic neuroanatomy in its management.

1068 Cranial Fasciitis of the Temporal Bone

We present the case of a child with a rare benign tumour of the temporal bone. A nine-month-old girl was referred to her local paediatric hospital with a painless right-sided, post-auricular swelling increasing in size in the preceding two months. On examination there was a 2x2cm mass overlying the temporal bone posterior to the right ear. The mass was firm, immobile, non-tender and well circumscribed. Ultrasound demonstrated a 1.9cm subcuticular mass with evidence of intracranial extension through the suture. Vascular flow and hyperechoic foci were demonstrated within the lesion. The local paediatric team requested magnetic resonance imaging (MRI) and computed tomography (CT) of temporal bone prior to referral to tertiary care centre. Imaging displayed a 2cm expansile lesion of the temporal bone with cortical loss and effacement of underlying dural venous sinus. The patient proceeded to incisional biopsy for tissue diagnosis which was suggestive of cranial fasciitis. Following discussion at the regional multidisciplinary meeting, she underwent surgical excision of the tumour in a joint Neurosurgery/Otology case. The tumour was fully resected macroscopically, with the normal surrounding bone and mastoid air cells preserved. Final histopathology showed a highly cellular lesion composed of spindle cells arranged in fascicles, with areas of myxoid background and confirmed the tumour to be cranial fasciitis. The child has made a good recovery and will be kept under close clinical follow-up. This case demonstrates a rare benign tumour of the temporal bone that may present to an otolaryngologist and the importance of tissue diagnosis, imaging and multidisciplinary management.

Adult nasal chondromesenchymal hamartoma: a rare and benign tumour with aggressive malignant transformation

Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign tumour of the nasal cavity predominantly described in infants. We report a case involving a 48-year-old woman who had been diagnosed with NCMH a year earlier and now re-presented with a short history of progressive nasal blockage, recurrent epistaxis and orbital apex syndrome. Histopathology was suggestive of malignant transformation into sinonasal sarcoma. However, following multidisciplinary team (MDT) discussions, including second and third opinions from external departments, the histological diagnosis was revised to ‘NCMH with bizarre stromal cells’. Despite this, the lesion demonstrated malignant features of rapid, invasive growth and was treated with palliative radiotherapy. The patient later developed radiological evidence of lung and liver metastases with subsequent pulmonary emboli. Shortly after this, she passed away. This case is unique in its diagnostic challenge, with ambiguous histopathological findings, and highlights the importance of an MDT approach when managing complex sinonasal tumours.

Cervical Dumbbell Ganglioneuroma Causing Quadriplegia

Ganglioneuroma is a rare benign tumour that originates from the ganglion cells of the sympathetic nervous system. They are rare in cervical spine region and only 8 % of ganglioneuromas occur in the neck. The common sites of occurrence are in the posterior mediastinum, retroperitoneum and adrenal medulla, and as such, a cervical occurrence presenting with quadriplegia is a reportable event. We present a 26-year old young male with a two-year history of neck pain and progressive quadriplegia. He later became wheelchair-bound. Musculoskeletal examination revealed multiple generalized nodular skin swellings with café au lait macules. Magnetic resonance imaging showed a huge dumbbell tumour of the first two cervical vertebrae, to the right side of the spinal canal causing significant spinal cord compression. He had surgical intervention, aimed at complete tumour resection, postoperatively, power of the limbs improved to normal. Histological examination was consistent with ganglioneuroma. We present this report because the occurrence of ganglioneuroma is rare, secondly a cervical presentation is unusual and thirdly it presented as a rare cause of quadriplegia.

Rare Case of Childhood Lipoblastoma presenting as Tongue Mass

Introduction Lipoblastoma is a rare benign tumour arising from embryonic white fat been commonly noted in limbs and trunk, but tongue involvement is rare and has not yet been reported. Case Report A child with tongue lipoblastoma is reported, whose imaging reported an encapsulated, well-delineated, fat-containing tumour. Surgical excision was performed with no post-operative morbidities. Discussion Lipoblastoma is an uncommon childhood tumour, which rarely affects the tongue. It presents as a progressive painless swelling, rarely causing any symptom. MRI is helpful to assess the precise location and extent of the lesion. Although the ratio of fat to myxocollagenous tissue in the tumour is variable, the diagnosis can be suggested in most cases based on the imaging characteristics. Recommended treatment is complete surgical excision and confirmation of diagnosis by histopathological examination.

82 A Case of Recurrent Calcifying Aponeurotic Fibroma of the Hand: Managing a Rare Hand Tumour in an Evolving Healthcare Landscape

Introduction Calcifying Aponeurotic Fibroma (CAF) is a rare benign tumour originating from the aponeuroses of tendons and their bony insertions. Our case illustrates the technical challenges and considerations of removing a large, recurrent CAF of the hand. Case Report A 15-year-old student presented to his GP with a one-year history of a progressively enlarging painless swelling on the dorsum of the middle phalanx of the left middle finger. Plain radiographs identified a calcified soft tissue swelling with no bony involvement. The lesion was excised by the local paediatric orthopaedic service and recurred rapidly. Histology confirmed the diagnosis. He was referred to our specialist hand surgery service and the lesion was excised with the overlying skin (which demonstrated histological but not clinical disease). At six months, there was no clinical evidence of recurrence. Conclusions CAF may present atypically, and a high index of suspicion is warranted with calcified soft tissue hand lesions. Excision with conservative margins, and we newly suggest, the overlying skin, is recommended to preserve hand function but minimise recurrence (very common). Hand surgery provision in the UK is changing with adoption of the hub and spoke model and hand tumours may be more appropriately managed at specialist centres.

560 A Rare Case Report of Cartilaginous Choristoma In Base of Tongue

Introduction Choristomas are rare, benign, tumour-like growths with histologically normal morphology in an ectopic location. Choristomas can constitute of various tissue types including cartilage, bone and muscle. Chondroid tissue choristomas are referred to as cartilaginous choristomas. Case Presentation: A 69 year old gentleman presented with three weeks’ history of foreign body sensation in his throat. No other red flag symptoms were present. Fine nasal endoscopy was unremarkable. Magnetic resonance imaging (MRI) scan revealed mild bilateral prominence of the lingual tonsils. Patient underwent Panednoscopy and biopsy. This confirmed the presence of a cartilaginous choristoma in base of tongue. Discussion: This is a rare case of cartilaginous choristoma in base of tongue. It was investigated as per potential head and neck cancers. The majority of previous cases in the literature were managed using surgical resection of the lesion. In this particular case, the patient was discussed in multi-disciplinary team (MDT) meeting and managed conservatively due to the benign nature of the lesion and symptoms. Conclusions Cartilaginous choristomas are rare benign lesions, which can present similarly to malignant head and neck tumours. Their size and symptoms should be carefully considered before management. There is a role for conservative management in select cases.