Pulmonary Mucoepidermoid Carcinoma With Multiple Paraneoplastic Syndromes.

Pulmonary mucoepidermoid carcinoma (PMEC) is rare and challenging to diagnose. Its association with paraneoplastic syndromes is poorly described. It is also uncommon for a patient with lung cancer to present with multiple paraneoplastic syndromes. We report a case of a patient with metastatic high-grade PMEC associated with three paraneoplastic syndromes, namely, humoral hypercalcemia of malignancy, ectopic ACTH syndrome, and paraneoplastic leukemoid reaction.

A Case of Inflammatory Pseudotumour Masquerading as Hepatocellular Carcinoma.

Inflammatory pseudotumors (IPTs) of the liver can mimic malignant lesions. As the name implies, they are usually associated with an inflammatory process and usually regress with the treatment of the underlying pathology. We report a case of a 67-year-old female who presented with right upper quadrant pain, deranged liver enzymes, elevated tumor markers [alpha-fetoprotein (AFP) and CA 19-9], and a large liver mass on imaging, suspected to be hepatocellular carcinoma (HCC). She was eventually diagnosed with IPT complicating the liver inflammation due to autoimmune hepatitis (AIH). She responded well to treatment with steroids and immunosuppressive therapy.

A Case of Pulmonary Nodular Lymphoid Hyperplasia Responding to Corticosteroid Treatment.

Pulmonary nodular lymphoid hyperplasia (PNLH) is a rare non-neoplastic disease that presents with mass lesions in the lung. It is radiologically difficult to differentiate it from adenocarcinoma of the lung or pulmonary lymphoma. There has been no consensus regarding the treatment of PNLH; however, in many case series, patients usually undergo surgical resection for diagnostic and therapeutic purposes. Here, we present the case of a 60-year-old Chinese male who presented with cough and hemoptysis. A computed tomography scan of the thorax revealed a mass-like lesion. A biopsy was performed which showed lymphocytic pneumonitis. He was treated with a tapering dose of corticosteroids with good clinical and radiological outcomes. Upon a subsequent review of the case, a diagnosis of PNLH was made. This case report suggests that corticosteroids may be an alternative therapy to surgical resection. They have the advantage of being non-invasive and can be used in patients who are otherwise not surgical candidates due to other comorbidities. However, further research is required before we can recommend corticosteroids as a treatment for PNLH.

Virtual Multidisciplinary Review of a Complex Case Using a Digital Clinical Decision Support Tool to Improve Workflow Efficiency.

OBJECTIVE: Integration of distinct clinical perspectives in multi-disciplinary tumor board meetings is critical to determine optimal patient care. Digital tools can support the data consolidation needed for meeting preparation and data sharing during complex case reviews. In this paper, we assessed the value of a clinical decision support tool on workflow efficiency and conducting a complex case review of a dermatofibrosarcoma protuberans (DFSP) tumor. METHODS: Case presentation was performed by each unique clinical specialty that had relevant information about the patient; an oncologist, a pathologist, and a radiologist. Virtual discussion was completed online with case presentation and documentation with NAVIFY Tumor Board. Workflow efficiency assessment was done through interviews and observation of the # of steps across different team members involved in preparing and conducting cancer multidisciplinary team (MDT) meetings before and after the implementation of the NAVIFY Tumor Board solution. RESULTS: Case review consisted of surgical and therapeutic intervention history, distinct histological and sequencing patterns representative of DFSP, with radiological review to determine areas for surgical intervention. Consolidation of clinical input led to a recommendation of a formal external hemipelvectomy with potential chemotherapy. Workflow assessment demonstrated a 46% total reduction in the # of steps for meeting preparation (from 69 to 37), with specific changes based on role: data manager (33 to 15), pathologist (26 to 13), radiologist (no change), and logistics (5 to 4). There was a 31% total reduction in the # of steps for conducting the meeting (from 51 to 35). CONCLUSION: Utilizing a digital clinical decision support tool helped to consolidate patient data and improved case presentation through workflow efficiency. This allowed for improved interdisciplinary discussion on a complex DFSP case and supported the determination of a clinical decision.

Isolated IgG4-related gastric disease presenting as diffuse gastric wall thickening with ulcer.

An 81-year-old male presented with loss of appetite, early satiety and iron deficiency anaemia. A computed tomography (CT) scan of the abdomen and pelvis during initial work-up revealed diffuse gastric mural thickening associated with a large ulcer and adjacent gastro-hepatic lymphadenopathy. The CT appearances, together with the clinical features, were highly suspicious for an infiltrative type of gastric malignancy. Endoscopic biopsy however showed erosive inflammation, IgG4 plasmacytosis and fibrosis, raising the possibility of IgG4-related disease. A serologic assay for IgG showed normal IgG4 and elevated IgG2 serum levels. After appropriate steroid treatment, endoscopy and CT scan showed resolution of the ulcer and gastric wall thickening. This case shows yet another possible appearance of gastric involvement in IgG4-related disease on the current evolving spectrum of this disease presentation. Greater awareness and education of this disease would help in patient care, ensuring earlier diagnosis, prevention of severe organ damage and morbidity, as well as unnecessary surgery.

Serum IgG2 and tissue IgG2 plasma cell elevation in orbital IgG4-related disease (IgG4-RD): Potential use in IgG4-RD assessment.

AIMS: To determine the role of serum and tissue IgG2 in orbital biopsies with the histological features of IgG4-related disease (IgG4-RD) in comparison with non-IgG4-related orbital inflammatory disorders (OID), including autoimmune disorders. METHODS: This is an international (Sheffield, UK, and Singapore) collaborative, retrospective case review of 69 patients (38 from Singapore National Eye Centre and 31 from Royal Hallamshire Hospital, Sheffield) with orbital inflammatory biopsies between 2002 and 2016. Clinical information and histology were reviewed and cases were classified into three groups: Group 1: IgG4-RD orbital inflammation (n=43); Group 2: idiopathic OID (n=12) and Group 3: autoimmune OID (n=14). Serum IgG1, IgG2, IgG3 and IgG4 levels were collated where available and immunohistochemistry (IHC) for tissue IgG2 plasma cells was performed. RESULTS: Dual IHC showed IgG2 plasma cells as a distinct population from IgG4 plasma cells. Significant (twofold) serum IgG2 elevation was noted among IgG4-RD (group 1), idiopathic (group 2) and autoimmune OID (group 3). Similarly, significant elevation of tissue IgG2 plasma cells was also seen among IgG4-RD (group 1), idiopathic and autoimmune OID (groups 2 and 3). CONCLUSIONS: Significant elevations of serum IgG2 and tissue IgG2 plasma cells are present in orbital IgG4-RD in comparison with non-IgG4 orbital inflammation (idiopathic and autoimmune OID), suggesting that IgG2 may play a role in IgG4-RD. A serum IgG2 cut-off >5.3 g/L was found to be 80% sensitive and 91.7% specific for orbital IgG4-RD, with an accuracy of 0.90. Tissue IgG2 and IgG4 subclass reporting may provide additional insight regarding the 'IgG4-RD' pathogenesis.

Histological features of Cytomegalovirus-related corneal graft infections, its associated features and clinical significance.

AIM: To describe the histological features of ITALIC! Cytomegalovirus (CMV)-related corneal graft infections, its associated features and clinical significance. METHODS: This was a retrospective histological study of 48 consecutive cases of failed repeat penetrating keratoplasty cases with a clinical diagnosis of allograft rejection from 2011 to 2013. CMV infection was confirmed with CMV antibody immunohistochemistry (IHC) and electron microscopy. Additional CD163 and CD68 IHCs for macrophages were also performed. Clinical data and previous graft histology were then reviewed. RESULTS: Mean incidence of CMV infection in corneal graft rejection buttons was 6.3% per year. 3/48 graft buttons were CMV antibody positive. Histological features of CMV graft infection include: (1) stromal keratocytes with cytopathic changes; (2) lack of inflammation, only occasional macrophages present and (3) absence of vascularisation. None of the patients had a history of active CMV infection. CONCLUSION: CMV infection is not limited as endotheliitis, but extends into the corneal stroma, and is a potential reservoir for graft infection, especially in partial thickness endothelial surgery. Clinical features are often non-specific, although glaucoma was present in our patients. CMV-infected grafts showed CD163-positive M2 macrophages in close association with the infected keratocytes, suggesting that the macrophage may be important in CMV graft infection. Histological examination with CMV IHC is a useful method to detect CMV infection postoperatively. Post penetrating keratoplasty, CMV systemic treatment with valganciclovir can prevent graft infection and failure. Boston keratoprosthesis may be a potential alternative surgery in active CMV infections that obviates the need for systemic therapy.

The association of lacrimal gland inflammation with alopecia areata.

PURPOSE: To describe the association of lacrimal gland inflammation with alopecia areata. METHODS: We reviewed the medical records of 4 patients diagnosed with lacrimal gland inflammation who had an antecedent or subsequent episode of alopecia. Data was collected on the presentation age, gender, medical history, disease onset, symptoms and signs, imaging, histopathology, systemic evaluation, management and outcome. Pathology and imaging results were correlated with clinical findings. RESULTS: Three patients were Asian and one Caucasian. Two developed alopecia after presentation for lacrimal inflammation. The remaining two had a history of alopecia totalis (2 years and 10 years). Three of the 4 patients presented or developed other systemic disorders, including seizures, thrombocytopenia, optic neuritis, ulcerative colitis, allergic rhinitis, lymphadenopathy, vasculitic rash and positive p-ANCA values. All received oral corticosteroids, with the addition of methotrexate therapy in one for relapsing inflammation. CONCLUSIONS: Lacrimal gland inflammation and alopecia areata are autoimmune processes that can be seen in association with each other.

'Prechronous' metastasis in clear cell renal cell carcinoma: a case report.

INTRODUCTION: Although metastatic carcinoma in the presence of an occult primary tumor is well recognized, underlying reasons for the failure of the primary tumor to manifest are uncertain. Explanations for this phenomenon have ranged from spontaneous regression of the primary tumor to early metastasis of the primary tumor before manifestation of a less aggressive primary tumor. We report a case of 'prechronous' metastasis arising from clear cell renal cell carcinoma, where metastatic disease initially manifested in the absence of a primary renal tumor, followed by aggressive growth of the primary renal lesion. CASE PRESENTATION: A 43-year-old Malay man initially presented to our facility with fever and cough. He subsequently underwent surgical resection of a 9 cm right-sided lung mass found on radiological examination. Histology showed a high-grade clear cell tumor with sarcomatoid differentiation, suggestive of a metastasis from clear cell renal cell carcinoma. However, no concurrent renal lesions were noted on computed tomographic evaluation at that time. Then, four months after lung resection, he presented with a subcutaneous mass in the left loin, as well as right loin discomfort. Computed tomography scanning revealed a 10 cm right renal mass, with renal vein and inferior vena cava invasion, as well as recurrent disease in the right thorax. Histological examination of the excised subcutaneous mass revealed a high-grade carcinoma consistent with clear cell renal cell carcinoma. CONCLUSIONS: This is the first reported case of prechronous metastasis of renal cell carcinoma, with metastatic disease manifesting prior to the development of the primary lesion. The underlying mechanism is uncertain, but our patient's case provides anecdotal support for the early dissemination model of metastasis.

Bilateral orbital varices: an approach to management.

The authors report a case of bilateral orbital varices in a 19-year-old man with a 7-year history of intermittent left proptosis and dystopia. CT demonstrated enhancing lesions with phleboliths and gadolinium-enhanced MRI showed characteristic hyperintense lesions. The asymptomatic right lesion was treated conservatively; the left lesion was excised following intralesional injection of cyanoacrylate. Bilaterality in orbital varices may not be obvious clinically and only diagnosed radiologically. A multidisciplinary approach involving the ophthalmologist and interventional radiologist using intraoperative fluoroscopy enabled accurate characterization, delineation, embolizationm, and excision of the lesion with good hemostatic control.

Epibulbar osseous choristoma: a report of two cases.

Epibulbar osseous choristoma occurs rarely and is commonly mistaken for a dermoid or dermolipoma. We report two cases: an 8-year-old boy and a 79-year-old lady with epibulbar osseous choristomas where computed tomography showed an extraocular lesion in the temporal quadrant and diagnosis was confirmed by excision biopsy. The osseous component was adherent to the sclera in the young patient, while it was loosely attached to the surrounding tissue in the older patient. We believe that the 79-year-old lady is the oldest reported patient with an epibulbar osseous choristoma.