Cutaneous Richter syndrome: a better place to transform?

The development of high-grade lymphoma in patients with chronic lymphocytic leukaemia is known as Richter syndrome (RS) and is associated with a grave prognosis, with a mean survival of 8 months despite treatment. Cutaneous RS has been described in a handful of cases and may be associated with a better outcome than the more common extracutaneous variants. We review the literature with particular emphasis on pathogenesis, treatment and survival of RS. We postulate that the absence of B symptoms and a normal lactate dehydrogenase level, presumably reflecting localized or limited disease, and a lower tumour burden, may explain the apparently better survival in some patients with cutaneous RS than with extracutaneous variants.

Current literature and imaging techniques of aseptic lymphocyte-dominated vasculitis-associated lesions (ALVAL).

Aseptic lymphocyte-dominated vasculitis-associated lesions (ALVAL) are a recognized complication of metal-on-metal bearing hip prostheses. There is an impending concern regarding the future investigation and management of patients who have received such implants. The current literature is discussed, and the current guidelines for management of these patients in the UK are reviewed. The various imaging techniques available, such as computed tomography, metal artefact reduction magnetic resonance imaging, and ultrasound are discussed and evaluated with respect to the assessment of patients with suspected ALVAL. The histopathological findings are discussed with images of the tissue changes provided. Images of the radiological findings are also provided for all general radiological methods. ALVAL and its radiological presentation is an important issue that unfortunately may become a significant clinical problem.

Diagnosis and subclassification of thymoma by minimally invasive fine needle aspiration directed by endobronchial ultrasound: a review and discussion of four cases.

Thymomas have been classified by the World Health Organisation (WHO) into six groups, based on the morphology of epithelial cells and the ratio between epithelial cells and lymphocytes within the tumour. Among 1458 consecutive cases of endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA) performed in a tertiary referral centre between February 2008 and February 2012, we have encountered four cases of thymic neoplasms. We discuss the cytomorphological features of three cases of type B thymoma (one each of B1, B2 and B3 subtypes) and one case of thymic carcinoma diagnosed on EBUS-TBNA using cell blocks, immunocytochemistry and flow cytometry which allowed preoperative chemotherapy to be carried out in two cases, diagnosis to be made after unsatisfactory surgical mediastinoscopy in the third and diagnosis of lymph node metastasis of the thymic carcinoma in the fourth. The differential diagnosis and criteria for subclassification of thymomas are discussed; although subclassification of these cases was possible in these cases, and tumours other than thymoma excluded, additional cases would be necessary to assess the potential accuracy of EBUS-TBNA. These, to the best of our knowledge, represent the first cases of thymoma that were diagnosed and subclassified according to WHO criteria using multimodality evaluation of EBUS-derived cytological aspirates.

Desmoplastic fibroma of the vidian canal in a child: case report.

INTRODUCTION: To present a case of a child with a desmoplastic fibroma of the vidian canal, compressing the vidian nerve. CASE REPORT: A 12-year-old girl with several years' history of right-sided facial pain was referred to our institution. Magnetic resonance imaging and computed tomography scans showed an expansile mass involving the right vidian canal. The patient underwent a complete endoscopic surgical resection aided by the FusionTM ENT navigation system. This was performed through a transnasal, trans-septal, trans-sphenoidal route via the right nostril, and achieved macroscopic clearance with minimal peri-operative morbidity. A biopsy of the lesion showed a fibro-osseous lesion consistent with desmoplastic fibroma. CONCLUSION: Diagnosis and resection of this rare lesion at an earlier stage would have avoided delays in resolving the child's disabling pain. This emphasises the importance of early referral of unusual cases to tertiary centres.

Leiomyosarcoma of the esophagus.

Leiomyosarcomas of the esophagus are rare, malignant, smooth-muscle tumors. The presenting symptoms are indistinguishable from other esophageal neoplasms, though the history may be longer due to the slow growth of these tumors. Barium studies may show large intramural masses with ulceration or tracking, expansile intraluminal masses or areas of luminal narrowing. Endoscopic biopsies may give a high false negative rate especially in cases where the mucosa is intact. The treatment of choice is surgical excision. Synchronous and metachronous metastases do not preclude surgery, provided the metastases are also resectable. Prognosis is better than in patients with squamous esophageal cancer. The role of adjuvant radiotherapy and chemotherapy is controversial. We report a 40-year-old man who presented to us with dysphagia and was found to have a leiomyosarcoma of the esophagus. He was treated successfully with esophagectomy and is disease-free after 7 years. We review the literature on esophageal leiomyosarcomas and their management.

Hormone receptor status of breast cancer in India: a study of 798 tumours.

The objectives of this study were to document the oestrogen and progesterone receptor (ER & PR) status of breast cancer in the Indian population (as done by immunohistochemistry on paraffin blocks), and correlate the steroid receptor status of breast cancer with all relevant patient and tumour characteristics. Our current data have been compared with previously published data from other centres. In contrast to the higher rates reported in the Western literature, only 32.6% of our tumours were ER positive and 46.1% were PR positive. Tumours were separated into four categories: ER+PR+ (25%), ER+PR- (7.4%), ER-PR+ (21.1%) and ER-PR- (46.5%). ER and PR immunoreactivity increased with advancing age, and correlated with the presence of elastosis. Infiltrating lobular carcinoma, mucinous carcinoma, and mixed tumours were more frequently ER & PR positive. High-grade infiltrating duct carcinomas, pure comedo ductal carcinoma in situ, and medullary carcinoma were predominantly ER & PR negative. The presence of necrosis and lymphovascular invasion showed an inverse relationship with ER and PR reactivity.