Long-term outcomes of real world 'watch and wait' data for rectal cancer after neoadjuvant chemoradiotherapy.

AIM: A 'watch and wait' (W&W) strategy after neoadjuvant long-course chemoradiotherapy (NACRT) remains controversial. Whilst encouraging short-term data exist, the strategy will be judged on long-term data. We present long-term, real-world UK data from a single National Health Service trust. METHODS: An analysis was performed of a prospectively maintained W&W database over 9 years between 2010 and 2018. Outcome measures include incidence and time to regrowth and overall and disease-free survival. RESULTS: We diagnosed 563 rectal cancers in 9 years. In all, 283 patients underwent rectal resection (50.3%). NACRT was used in 155 patients for margin-threatened tumours on staging MRI. Forty-nine patients (31.6%) experienced either a 'near complete' or a complete clinical response (cCR) at their 10 weeks post-NACRT assessment (MRI and endoscopy). The median age was 69 years (range 44-83), and the male to female ratio was 32:17. The median follow-up was 38 months (range 12-96). The median tumour distance from the anal verge was 7 cm (1-15 cm). Twenty-two patients had a cCR on initial assessment and 27 patients had a 'near' cCR. Of those 27 who experienced a 'near' cCR, 17 (63%) progressed to cCR on repeat assessment and 10 (37%) did not. Of these 10 patients, seven underwent standard surgical resection and three were unfit for surgery. R0 for the seven with delayed resection was 100%. Of 39 patients (22 cCR and 17 'near' cCR who progressed to cCR) (25.2% of those receiving NACRT), six patients experienced local regrowth (15.4%). The median time to local regrowth was 29 months (15-60 months). One of these six patients underwent salvage abdominoperineal resection, one was advised to have contact radiotherapy and four opted against surgery and also had contact radiotherapy. The overall survival was 100% at 2 years and 90% at 5 years. Disease-free survival was 90.47% at 2 years and 74.8% at 5 years. CONCLUSION: A W&W treatment strategy was employed safely in this patient cohort with acceptable rates of local regrowth and survival.

Lipomatous hamartoma of the interatrial septum.

Four cases of lipomatous hamartoma of the interatrial septum (LHIAS) were diagnosed at autopsy, three in elderly women, two of whom were morbidly obese. One patient had signs of congestive cardiac failure, without other significant cardiopulmonary disease, which was thought to be a result of the interatrial lipomatous hamartoma, while in the others, the LHIAS was considered an incidental finding. This condition, previously considered a hypertrophic phenomenon, is now best described as hamartomatous, and is being recognized more frequently both by clinicians and pathologists.

Clinicopathological characteristics of peripheral primitive neuroectodermal tumour of skin and subcutaneous tissue.

AIMS: To study the clinical and pathological features of primary malignant peripheral primitive neuroectodermal tumours (PNETs) of the skin and subcutaneous tissue, to discuss the differential diagnosis, and to review the existing literature on these tumours. METHODS AND RESULTS: Eight cases of PNETs presenting in the skin and subcutaneous tissue were identified from the pathology records of the Christie Hospital, Manchester. Detailed immunohistochemical studies were performed on all cases and seven tumours were subjected to electron microscopic examination. Detailed clinical and follow-up information was obtained on seven cases. Six tumours occurred in children and adolescents and two were seen in young adults (age range, 8-36 years). No sex or site predilection was observed. Five tumours occupied the dermis and subcutis and three were entirely located in the subcutaneous tissue. Microscopically, they were composed of small round cells and seven tumours contained glycogen. Only one tumour focally exhibited Home-Wright rosettes and neuropil. Two tumours contained rhabdoid or plasmacytoid cells in places and all cases showed microcystic and pseudovascular spaces. Immunostains revealed MIC2 (8/8), NSE (7/8), PGP9.5 (7/8), beta 2 microglobulin (7/8), neurofilament protein (6/8), S100 protein (3/8), synaptophysin (2/8) and Leu-7 (1/8) positivity. Anomalous cytokeratin (4/8), desmin (2/8), myoglobin (2/8), NKIC3 (4/8) and GFAP (1/8) staining was also noted. Ultrastructurally, neuroendocrine granules were detected in five cases and one case exhibited microtubules in processes. Adequate follow-up information was available in four cases. One patient died of metastatic disease. One child developed axillary lymph node metastasis but is alive with no evidence of disease 96 months after treatment. Two other patients are alive with no residual or recurrent disease 44 and 52 months after excision and radio/chemotherapy. CONCLUSION: PNETs are rare malignant small round cell tumours of the skin and subcutaneous tissue which are probably underdiagnosed. A correct diagnosis can be made on light microscopic features, demonstration of neuroendocrine granules on electron microscopy and a combination of MIC2, beta 2 microglobulin, and more than one neural marker positivity. These neoplasms should be differentiated from other cutaneous neoplasms composed of small round cells. The number of cases of cutaneous PNETs studied so far is rather small, and no firm conclusion can be drawn about their behaviour but long-term survival is possible in some cases.

The nature of atypical multinucleated stromal cells: a study of 37 cases from different sites.

The morphology, immunophenotype and ultrastructure of atypical multinucleated stromal cells in 37 specimens from different anatomical sites were compared. Overall, cellular morphology was similar between sites but nuclear features, immunophenotype and ultrastructure varied and reflected that of adjacent normal mononuclear stromal cells. It is concluded that the atypical multinucleated stromal cells represent a reactive change in the indigenous stromal cells. In all the cases mast cells were seen as a prominent component of the inflammatory infiltrate and many of them were intimately related to the atypical stromal cells. It is suggested that reactive stromal cell changes may relate to an interaction with mast cells.

Variant Creutzfeldt-Jakob disease in an elderly patient.

We report a case of variant Creutzfeldt-Jakob disease(vCJD) in a 74-year old man in whom diagnosis was made at necropsy. The occurrence of vCJD in an individual in this age group is unlikely to be an isolated event. Doctors need to be aware that vCJD can arise in elderly patients so that appropriate investigations (including magnetic resonance imaging) can be done, and permission for neuropathological necropsy requested, in suspected cases. This case could also have important implications for public health policy decisions and surveillance programmes that target the younger age range of vCJD cases.