A 59-year-old woman presented with a painful, pruritic eruption that had commenced as an erythematous, dry patch on the upper back but progressed to erythroderma. Examination revealed orange-tinged erythroderma, scalp scaling, ectropion, palmoplantar keratoderma and nail changes. A diagnosis of type I adult-onset pityriasis rubra pilaris was made, and a subsequent skin biopsy was consistent with this. She was treated with a number of topical and systemic agents with minimal improvement or major side-effects. The patient was then treated with intravenous infliximab 5 mg/kg. She improved dramatically within 2 weeks and was no longer erythrodermic. Five further infusions resulted in additional improvement. Methotrexate was briefly added to the regime, but was ceased owing to nausea. Topical tar and keratolytics were used on the scalp. The patient was left with minimal disease activity and was maintained on emollients.
BACKGROUND:Much of the reluctance about using cytology specimens rather than histology specimens to assess programmed death ligand 1 (PD-L1) expression for guiding the use of immune modulating drugs in the management of non-small cell lung cancer (NSCLC) is based on the belief that the alcohol-based fixatives favored by cytopathologists might reduce the antigenicity of PD-L1 and lead to artifactually low expression levels and false-negative reporting. Therefore, this study was performed to determine whether there is any difference in PD-L1 expression between endobronchial ultrasound (EBUS)-guided aspirates of NSCLC fixed in alcohol-based fixatives and those fixed in neutral buffered formalin (NBF), the standard laboratory fixative for histology specimens. METHODS: The expression of PD-L1 was compared in 50 paired EBUS aspirates of NSCLC taken from the same lymph node during the same procedure. One aspirate of each pair was fixed in an alcohol-based fixative, and the other was fixed in NBF. RESULTS: In none of the 50 pairs was there any significant difference, qualitative or quantitative, in the strength, pattern, or extent of PD-L1 expression. In the great majority, the expression was identical, regardless of fixation. CONCLUSIONS: There is no evidence from this study showing that the use of alcohol-based fixatives has any effect on the expression of PD-L1 or its interpretation. Notwithstanding the general challenges in accurately assessing such expression in cytology specimens, pathologists should feel able to interpret them with confidence, and clinicians should feel able to rely on the results. Cancer Cytopathol 2020;128:100-106.
Background: Low-grade papillary adenocarcinomas of the sinonasal tract are rare neoplasms. Over recent years, little doubt remains that this tumour represents a separate entity based on morphology, ultrastructural features and behaviour. We outline a case of this rare entity displaying a not hitherto described immunophenotype.
Investigations completed in a 77-year-old ex-smoker presenting with weight loss showed raised CA 19-9 levels. The findings of chest radiograph, abdominal computed tomography (CT) and gastrointestinal endoscopic examinations were all normal. On follow-up, the patient developed left upper lobe collapse on chest radiograph with increasing CA 19-9 levels. Chest CT findings suggested the presence of a left upper lobe tumour. The results of a biopsy of the left upper lobe nodule seen on bronchoscopy suggested a diagnosis of amyloidosis; however, this was not the only diagnosis. The left upper lobe mass comprised a neuroendocrine tumour with amyloid deposition just beneath the bronchial epithelium and focally between the nests of the tumour cells. We report and discuss this uncommon association and presentation. Case ReportA 77-year-old woman who was an ex-smoker of 40 packyears presented with complaints of weight loss and a decreased appetite. She was known to have mild diverticular disease and experienced a disease flare up with an increased stool frequency of 2-3 bowel movements/day on admission. There was no history of haematochezia, malena, cough, expectoration or haemoptysis. She was known to have bronchial asthma and was receiving treatment with inhaled corticosteroids. On examination, she was not found to have any pallor, clubbing or lymphadenopathy. She had a body mass index of 16. Her heart rate was 80 beats/min and her blood pressure was 130/80 mmHg. The findings of a systemic examination were normal.The initial diagnoses considered included malignancy (possibly gastrointestinal), mal-absorption syndrome and thyroid dysfunction, and investigations were accordingly organised. Other than a serum albumin level of 2.7 g/dL, the patient's biochemical profile was normal. Liver function tests were within the normal range and showed a bilirubin level of 16 umol/L, an alkaline phosphatase level of 92 IU/L and an alanine aminotransferase level of 29 IU/L. A full blood count and thyroid function tests were normal. Chest radiograph findings were normal. Sigmoidoscopy and colonoscopy results were normal other than showing mild diverticular disease. Oesophagogastroduodenoscopy findings were normal, as was the subsequent report of a duodenal biopsy. Tumour marker tests showed that the levels of Carbohydrate Antigen 19-9 (CA 19-9) were raised at 300 ku/L (normal <40 ku/L). The tests were subsequently repeated, and the CA 19-9 level was found to be 326 ku/L. The Carcinoembryonic Antigen, Alpha-fetoprotein and Cancer Antigen 125 levels were normal. Ultrasound and subsequent computed tomography (CT) of the abdomen and pelvis were normal. The diarrhoea resolved, and the patient was feeling better. A dietary opinion was sought, and the patient was started on nutritional supplements. She was discharged three weeks from admission, as nothing specific was found, with plans to follow up with repeat CA 19-9 level measurements.Two months after discharge the patient was again admitted, as her symptoms of anorexia and weight lo...
Leiomyosarcomas are extremely rare in the head and neck; the common sites of origin are the skin and sinonasal tract. The overall prognosis is poor.
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