Human Papillomavirus-Related Multiphenotypic Sinonasal Carcinoma with Unique HPV type 52 Association: A Case Report with Review of Literature

Abstract

Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC) is a recently described distinctive clinicopathologic entity defined by association to high risk HPV, localization to sinonasal tract and close histologic resemblance to salivary gland tumors. Lack of awareness of its pathologic features and biology among pathologists and oncologists make this entity susceptible to misdiagnosis and erroneous management. Herein, we illustrate a case of HMSC of the nasal cavity associated with heretofore unreported subtype HPV-52 and discuss the challenges associated with diagnosis and management of this rare tumor. A 48-year-old woman with intermittent epistaxis for 6 months presented with a nasal mass and underwent middle turbinectomy. Histology showed a tumor with features typical of adenoid cystic carcinoma (ACC) in the form of basaloid cells and cribriform architecture. However, careful inspection revealed findings uncommon in ACC; such as surface pagetoid tumor spread, areas of solid sheets of myoepithelial cells accompanied by increased mitotic figures which prompted immunohistochemistry. Multidirectional differentiation into ductal (CK7, AE1/AE3) and myoepithelial (p63, p40, S100, calponin) lineage together with strong and diffuse immunopositivity for p16 distinguished this tumor from ACC. HPV genotyping was positive for high risk HPV subtype HPV52, which confirmed the diagnosis of HMSC. HPV-related multiphenotypic sinonasal carcinoma is an under-recognized unique clinicopathologic entity that needs awareness to avoid mistaking it for commoner salivary gland tumors. Making accurate diagnosis of this newly-described tumor is imperative in order to understand its biology and to develop optimal therapeutic strategies.

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Post-traumatic bilateral vesicocutaneous fistula of thighs treated with buccal mucosa graft urethroplasty and cyanoacrylate glue instillation: a novel treatment for management of a complicated fistula

Post-traumatic vesicocutaneous fistula (VCF) is a rare variant of urinary fistulas. These fistulas may externally communicate to abdomen, perineum, buttocks, scrotum or very rarely thigh. These fistulas usually develop at a variable time duration after trauma and are usually preceded with thigh swelling or abscess formation followed by spontaneous rupture. We, hereby, report a case of VCF of bilateral thighs with associated penobulbar urethral stricture after road traffic accident which was managed with dual modality of buccal mucosa graft urethroplasty surgery and cyanoacrylate glue injection in the fistulous tracts. To the best of our knowledge, this is first report of bilateral VCFs communicating externally to thighs. In literature, very few cases of VCFs of thigh are reported and are rarely managed with adhesive glue application.

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Cushings reflex secondary to neck haematoma following thyroidectomy

Neck haematoma following thyroid surgery can present with respiratory distress which is generally attributed to airway obstruction. We recently had a 63-year-old female patient who underwent total thyroidectomy for toxic nodular goitre. However, within 4 hours of surgery, she developed sudden respiratory distress which was managed by prompt evacuation of the neck haematoma. Just before the haematoma evacuation, the patient had hypertension and bradycardia along with the distress. The arterial blood gas analysis sampled at that time was normal. Intraoperatively, the tracheal framework was found rigid and non-pliable. Considering the various clinical–biochemical findings observed, we think that the cause of the respiratory distress in the index case was transiently elevated intracranial pressure, secondary to bilateral internal jugular veins' compression. We hypothesise that in many patients with immediate postoperative neck haematoma, the Cushing's reflex would at least contribute partly, if not solely to the respiratory distress.

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Three differently timed presentations of dermatomyositis associated with advanced ovarian cancer

Each of the three patients reported in this article presented with dermatomyositis at various stages of their advanced ovarian cancer. Dermatomyositis was the presenting feature and preceded the diagnosis of ovarian cancer by several months in one patient. In another patient, dermatomyositis occurred just prior to the scheduled third cycle of palliative chemotherapy after surgical debulking for stage 4 disease. The third patient presented with pathognomonic diagnostic features of dermatomyositis after ovarian cancer recurrence. Diagnosis was delayed in at least two of these patients; however, once appropriately diagnosed, each patient responded well to immunomodulatory treatment. In one patient, initiation of oral prednisolone seemed to correlate with a steady improvement in her proximal myopathy. A pulsed methylprednisolone approach was used in another patient with conversion to a tapering dose of oral prednisolone to good effect. In the patient in whom the most severe myopathy affecting bulbar muscle groups was demonstrated, an infusion of 5 days of intravenous immunoglobulin produced an eventual improvement in her steroid-refractory myopathy.

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DNA as a self-antigen: nature and regulation

Chetna Soni | Boris Reizis

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Macrophage Activation Syndrome, Glomerulonephritis, Pericarditis, and Retinal Vasculitis as Initial Presentation of Systemic Lupus Erythematosus

Macrophage activation syndrome (MAS) is a rare manifestation of systemic lupus erythematosus (SLE) with potentially life-threatening consequences. To the best of our knowledge, this is the first case reported in literature for a constellation of MAS, glomerulonephritis, pericarditis, and retinal vasculitis as initial presentation of SLE. Despite extensive multisystem involvement of his disease, the patient responded well to initial steroid treatment, with mycophenolate mofetil successfully added as a steroid-sparing agent. Our case highlights the importance of multispecialty collaboration in the diagnosis and management of SLE with multisystem involvement.

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An international survey-based study on colorectal cancer pathology reporting—guidelines versus local practice

Abstract

Different guidelines for colorectal cancer (CRC) pathology reporting have been published. We aimed to identify differences between publicly available CRC reporting guidelines and to survey pathologists from different countries to establish the degree of guideline implementation in local routine practice. We compared all core and non-core items of CRC reporting guidelines to identify discrepancies. We then created a survey, which was sent out to 782 pathologists practicing in 30 different countries. It included questions on the demographics of the reporting pathologist as well as resection specimen handling and microscopic evaluation, grading, staging, and additional techniques, such as immunohistochemistry or molecular pathology. First, core and non-core items of five national CRC reporting guidelines were compared and 12 items were found to differ. Different items are considered core or non-core by different guidelines and more than one TNM staging edition was applied across guidelines. The survey was completed by 143 pathologists from 30 countries. We identified differences between local practice and guidelines with potential clinical impact, e.g., tumor budding was never reported by 28.7% of responders, although it has prognostic value for survival in stage II CRC. This is the first international study comparing CRC pathology reporting guidelines with real-world local practices. There are differences in CRC pathology reporting guidelines and in guideline implementation into local practice, both with potential impact on patient care. Harmonization of datasets, use of templates, and audits of local pathology practice are needed to ensure best possible quality of CRC pathology reporting.

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