Assessing IgG4-related ophthalmic disease and its mimics: a comparison of ACR/EULAR, organ-specific and revised comprehensive diagnostic criteria.

AIMS: Diagnosis of IgG4-related ophthalmic disease (IgG4-ROD) rests on the correlation of clinical features, serological testing and histopathology, using internationally accepted diagnostic criteria for objective interpretation; however, several mimickers of IgG4-RD overlap in clinical presentation and histopathology. We assess histopathological features in a series of presumptive IgG4-ROD cases, with emphasis on histopathological mimics and comparison of three IgG4-ROD diagnostic/classification criteria (organ-specific (OS), revised comprehensive diagnostic (RCD) and American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) criteria). METHODS: The histopathology database was screened for cases with clinical/histopathological suspicion of IgG4-ROD. Slides were reviewed, OS, RCD and ACR/EULAR criteria were applied, and the final clinicopathological diagnosis was recorded. RESULTS: 37 patients (24 females, 13 males; 19-73 years) were diagnosed as either IgG4-ROD (n=18) or non-IgG4-related disease (n=19). Non-IgG4-related disease group showed elevated serum IgG4 (55.5%), fibrosis (100%), dense lymphoplasmacytic inflammation (92.8%), with an increase in tissue IgG4+plasma cells (57.1%) and elevated IgG4:IgG+plasma cell ratio (14.3%). ACR/EULAR missed 50% (9/18, sensitivity-52.8%) of true IgG4-ROD cases, while OS and RCD criteria missed 11.1% (2/18, sensitivity-88.9%) of IgG-ROD cases. ACR/EULAR criteria mislabelled 7.14% (1/14, specificity-90.9%) while OS and RCD criteria wrongly categorised 71.4% (10/14, specificity-47.4%) and 50% (7/14, specificity-63.2%) specific non-IgG4-ROD cases as IgG4-ROD. Storiform fibrosis, obliterative phlebitis, increased IgG4:IgG+plasma cell ratio and elevated serum IgG were statistically significant in distinguishing IgG4-ROD from its mimics. CONCLUSION: ACR/EULAR criteria showed high specificity but were cumbersome and sensitivity was low, while RCD and OS criteria showed low specificity. Stringent clinicopathological correlation to exclude mimics is critical in avoiding diagnostic errors in IgG4-ROD.

Ulcerative colitis: a diagnostic odyssey through cutaneous polyarteritis nodosa and granulomatous hepatitis.

SummaryUlcerative colitis (UC), a chronic inflammatory bowel disease, can cause extraintestinal manifestations (EIMs) in approximately 40% of individuals. This case report discusses the diagnostic procedure of a woman in her 20s who initially had non-specific symptoms. The patient underwent a thorough evaluation, which initially pointed towards tuberculosis (TB) due to necrotic lymphadenopathy and granulomatous hepatitis. However, no microbiological evidence of TB was found, and her symptoms worsened despite antitubercular therapy. The patient developed painful nodular-ulcerative skin lesions consistent with cutaneous polyarteritis nodosa (cPAN) on biopsy. Eventually, a definitive diagnosis of UC was made, revealing the true nature of her multisystemic manifestations. Cutaneous vasculitis, including leucocytoclastic vasculitis and cPAN, is a rare EIM of UC, with only five reported cases in the literature. This case report highlights the clinical implications of EIMs and contributes to the expanding knowledge of rare EIMs such as cPAN and granulomatous hepatitis.

Benign Lesion of the Common Bile Duct Mimicking a Malignant Tumor: an Entity Commonly Overdiagnosed Clinically.

Adenomyomatous hyperplasia and adenomyoma are rare benign inflammatory pseudotumors of the gallbladder arising from Rokitansky-Aschoff sinuses. Occurrence of these hyperplastic conditions in the Vaterian and biliary system is extremely rare and is a concern for gastroenterologists and surgeons in distinguishing them from primary malignancies of the biliary system. Definitive diagnosis by imaging or cytopathological examination is difficult; thus, surgical resection becomes the only choice in such cases to relieve the obstruction. Here, we report two extremely rare cases of adenomyomatous hyperplasia of the extrahepatic bile duct after an extensive diagnostic workup, followed by Whipple's procedure.

Evaluation of the follicular patterned thyroid lesions based on the WHO 2022 criteria with an emphasis on the grey-zone lesions.

Follicular-patterned thyroid nodules (FPTN) are classified byWHO-2022 into benign, borderline and malignant categories. There are however, grey-zone lesions that pose a diagnostic challenge due to ambiguity in defining criteria and inter-observer variability. WHO-2022 has enumerated specific diagnostic criteria for these lesions. Accurate categorization of morphologically similar TNs is vital to reduce overtreatment of indolent lesions. In this study, we have reclassified FPTNs according to WHO-2022 criteria, emphasizing on grey-zone lesions. We studied the utility of immunohistochemistry (IHC)-CD56, HBME-1 and CK19 in distinguishing benign from malignant nodules and BRAFV600E IHC to better distinguish the (widely-invasive) encapsulated follicular variant of papillary thyroid carcinoma (FVPTC) from infiltrative FVPTC. Only those cases with dominant nodule having follicular pattern histology were included and re-evaluated for following histopathological features-focality, encapsulation, circumscription, nuclear PTC features, capsular-invasion, angio-invasion, papillae and necrosis. IHC findings for above-mentioned markers were noted. Seventy-nine cases met the inclusion criteria. Amendment of original diagnosis was done in 19 % cases. BRAFV600E IHC was positive in the two cases of infiltrative FVPTC while it was negative in all nine IE (invasive encapsulated) FVPTCs. Diffuse HBME1 was noted in most malignant nodules (61 %) while CD56 was expressed more often in benign lesions (70 %). CK19 was positive in lesions displaying nuclear PTC features (86 %). Using WHO 2022 criteria, we were able to re-classify follicular thyroid lesions with greater confidence. Appropriate IHC panel in adjunct to histology aids in categorizing challenging cases.

Disseminated multiloculated peritoneal inclusion cysts: Bubble trouble in belly.

ABSTRACT: Multiloculated peritoneal inclusion cysts, usually arise from peritoneal mesothelium lining the serous cavity of the abdomen, pelvis and retroperitoneum. These lesions can be incidentally found on imaging or during surgery, and confirmation of the diagnosis is done by radiological imaging, histomorphology and immunohistochemical findings. Although fewer than 200 cases of solitary peritoneal inclusion cysts have been reported, their occurrence in a disseminated fashion has hardly ever been described in literature. Herein, we report a case of multiloculated peritoneal inclusion cysts that involved the whole abdominal and pelvic cavity and were successfully treated with surgery.

A Curious Case of Omental Oxalate Crystals in a Patient With Struma Ovarii: Pointers to an Unusual Clinicopathological Scenario.

Percutaneous image-guided biopsies are becoming increasingly common in routine pathology practice, with the greater omentum emerging as a common target. We present herein an account of a middle-aged lady with a complex ovarian mass, omental thickening, and raised serum CA125; clinically suspected to have advanced ovarian malignancy. Fine needle aspiration cytology (FNAC) from the ovarian mass was inconclusive. Omental biopsy revealed only refractile, birefringent crystalline material with surrounding foreign body giant cell reaction; thus surprising the clinical team. Subsequent resection of the ovarian mass showed a teratoma composed exclusively of thyroid tissue, diagnosed as struma ovarii. The omental crystals, interpreted as calcium oxalate crystals, were possibly a consequence of colloid seeding during the ovarian mass FNAC.

Neuroendocrine carcinoma of ovary: Hitherto rare entity in primary ovarian tumors.

Neuroendocrine neoplasms (NEN) of the female genital tract are extremely uncommon. These tumors can be broadly divided into well differentiated (carcinoid) and poorly differentiated NEN (small cell and large cell carcinomas). Occurrence of neuroendocrine carcinomas (NECs) in ovary has rarely been reported. These high-grade malignant tumors have a fulminant clinical course with a short period of survival, even when diagnosed at an early stage. We hereby report two cases of primary neuroendocrine carcinoma of the ovary.

Gastric Metastasis of Primary Neuroendocrine Tumor of Skin: Rare Tumor with Rare Presentation.

Merkel cell carcinoma (MCC) is a rare primary neuroendocrine tumor of the skin. It has an aggressive biological behavior and shows early local and distant metastasis. Diagnosis of MCC is a challenge and requires confirmation by immunohistochemistry (IHC). However, metastasis of MCC to the stomach is extremely uncommon and is rarely reported in the literature. We hereby describe a patient with gastric metastasis of MCC, who presented with black tarry stool and was finally diagnosed on the basis of clinical history, histology, and IHC.

Amelanotic Melanoma: A Great Masquerader.

Malignant melanoma is an aggressive, notorious tumor showing great variability in morphological and immunohistochemical expression, thus commonly leading to an erroneous diagnosis. Within the melanoma group, amelanotic melanoma, with its wide clinical presentations, lack of pigmentation, and varied histological appearances, has taken on a new persona as a master masquerader. Use of immunohistochemistry in the diagnosis of malignant tumors, including melanoma, is primordial and indispensable. However, the problem gets compounded in scenario of aberrant antigenic expression. The present case posed multiple diagnostic challenges in form of atypical clinical presentation, variant morphology, as well as aberrant antigenic expression. Here, we present the case of a 72-year-old male who, upon his initial presentation, was thought to be sarcomatoid anaplastic plasmacytoma, but 5 months later another biopsy from a different site revealed the actual diagnosis of amelanotic melanoma.

Transformation of ameloblastoma to ameloblastic carcinoma in a 10-year-old child.

Ameloblastic carcinoma (AC) is a rare odontogenic malignant epithelial neoplasm of maxillofacial skeleton with a distinct predisposition of the mandible. It can occur in a wide range of age groups, with a sex predilection in males. It can arise either as a de novo lesion or from preexisting ameloblastoma. AC has a high propensity for local recurrence as well as distant metastasis (chiefly lungs), thus requiring an aggressive surgical approach and a strict surveillance. Owing to the rarity of publications describing AC, little is known about this entity in pediatric patients. We report a case of transformation of ameloblastoma into AC in a 10-year-old child.

Stakeholder engagement as a strategy to enhance palliative care involvement in intensive care units: A theory of change approach.

BACKGROUND: Adult patients admitted to intensive care units in the terminal phase experience high symptom burden, increased costs, and diminished quality of dying. There is limited literature on palliative care engagement in ICU, especially in lower-middle-income countries. This study explores a strategy to enhance palliative care engagement in ICU through a stakeholder participatory approach. METHODS: Theory of Change approach was used to develop a hypothetical causal pathway for palliative care integration into ICUs in India. Four facilitated workshops and fifteen research team meetings were conducted virtually over three months. Thirteen stakeholders were purposively chosen, and three facilitators conducted the workshops. Data included workshop discussion transcripts, online chat box comments, and team meeting minutes. These were collected, analysed and represented as theory of change map. RESULTS: The desired impact of palliative care integration was good death. Potential long-term outcomes identified were fewer deaths in ICUs, discharge against medical advice, and inappropriate admissions; increased referrals to palliative care; and improved patient and family satisfaction. Twelve preconditions were identified, and eleven key interventions were developed. Five overarching assumptions related to contextual factors influencing the outcomes of interventions. CONCLUSION: Theory of change framework facilitated the identification of proposed mechanisms and interventions underpinning palliative care integration in ICUs.

Same storm, different boat: The global impact of COVID-19 on palliative care.

OBJECTIVE: The COVID-19 pandemic has had a complex and profound impact on the provision of palliative care globally. To support learning from palliative care providers and researchers worldwide, the Education Subcommittee of International Psycho-Oncology Society (IPOS) Palliative Care Special Interest Group developed a webinar with presentations by and discussion with eight international palliative care leaders. METHODS: Presentations were content rich; the speakers used both quantitative (e.g., sharing recent statistical findings) and qualitative (e.g., narrative storytelling, anecdotal experiences) approaches to portray the effect of COVID-19 in their region. Subsequent to the webinar, the committee collectively identified five themes conveyed by the presenters through consensus. RESULTS: The themes included: (1) altered accessibility to palliative care, with socio-economic status impacting virtual health availability; (2) reduced opportunities to preserve dignity, as survival has been prioritized over preserving the humanity of patients and their loved ones; (3) complicated grief and bereavement arising from social distancing requirements; (4) greater awareness of the importance of sustaining health provider well-being; and (5) the development of valuable innovations across nations, institutions, disciplines, and communities. CONCLUSIONS: Overall, the webinar facilitated valuable connection for global learning and identified opportunities for research and clinical interventions. In an ongoing crisis that has exacerbated isolation, we will need to continue to learn and lean on one another as a global community to navigate ongoing challenges of the COVID-19 pandemic.

PENS approach for breaking bad news in the oncology outpatient setting: a real-world report.

PURPOSE: Breaking bad news (BBN) is a vital part of oncology practice. We conducted this study to assess an abbreviated PENS protocol [Patient preference, Explanation, Next appointment, and Support] for BBN in oncology outpatient (OP) settings. METHODS: This observational study was conducted in a university teaching hospital, including cancer patients who were unaware of their condition and willing to discuss their disease status. The duration of BBN was the primary outcome. After the BBN session, patients filled a validated questionnaire; response scores of ≤ 13 were classified as content with BBN. RESULTS: Fifty patients (mean age 53.7 years, range 28-76) were included in the study. The average duration of BBN was 6.1 (range 2-11) min. Assessed by the response score sum, 43 (86%) patients were satisfied with BBN. Only three (6%) of the discontented patients felt that the BBN duration was too short. Most (94%) of patients reported that they understood the information imparted during the BBN session. After the session, 36 (72%) patients admitted to either feeling the same or reassured compared to before the session. The oncologists also were comfortable with PENS. CONCLUSIONS: The PENS approach is a practical method for BBN, especially when the oncologists have higher OP workloads. More extensive trials are required to validate the protocol in other settings. TRIAL REGISTRATION: Clinical Trial Registry of India (CTRI/2021/07/034707).

A 14-Year-Old Female With Chest Mass, Shortness of Breath, and Hypertension.

BACKGROUND: A previously healthy 14-year-old girl was evaluated in the emergency department (ED) with 2 months of shortness of breath and fatigue. METHODS: She initially presented to her primary pediatrician, where a complete blood count was only significant for mild anemia, thyroid function tests were normal, and a chest x-ray revealed a chest mass. This prompted her primary pediatrician to order a computed tomography scan and refer to the ED. RESULTS: Her computed tomography scan confirmed a mediastinal mass versus necrotic lymphadenopathy. Her initial physical exam was unremarkable, except for hypertension. From the ED, she was admitted to the pediatric hospital medicine service. Infectious disease, oncology, and nephrology services were consulted to guide diagnostic evaluation. CONCLUSIONS: Eventually, during her hospitalization, further history and workup led to a definitive diagnosis.

COVID-19 Palliative and End-of-Life Care Plan: Development and Audit of Outcomes.

Objectives: Palliative care (PC) referral in serious and critical COVID-19 improves decision-making, health resource utilisation, end-of-life symptom management and family support. In this study, we explored developing a systematic decision-making matrix for PC referral in COVID-19 and audited its outcomes. Materials and Methods: A team of interdisciplinary experts developed a hospital COVID-19 PC plan. PC referral and outcomes of PC referral in hospitalised COVID-19 patients were audited. Results: Out of 1575 inpatients, 1066 (67.7%) had mild and 509 (32.3%) had serious and critical COVID-19 illness. Among 50 (3.1%) referred to PC, 5 (0.4%) had mild and 45 (8.8%) had serious and critical COVID-19 illness. Out of 45 serious and critical COVID-19 patients referred to PC, 38 (84%) received end-of-life care (EOLC), 4 (9%) self-discharged against medical advice and 3 (7%) recovered. Forty-seven (94%) were referred for goals-of-care discussion. About 78% received opioids, 70% benzodiazepines and 42% haloperidol for symptom management. Among 45 serious and critical COVID-19 patients referred to PC, foregoing life-sustaining treatment was documented in 43 (96%) but implemented only in 23 (53%). Out of 38 who received EOLC, ICU was the place of death in 31 (82%) and ward in 7 (18%). Conclusion: Despite interdisciplinary experts developing a hospital COVID-19 PC, low referral of serious and critical COVID-19 patients to PC was observed. PC referral enabled access to management of end-of-life symptoms and facilitated limitation of life-sustaining treatment in some COVID-19 patients with serious illness. Educating critical care physicians about the scope of PC in the COVID-19 setting might improve PC referral.

Retrospective histopathology audit of thymectomy specimens: A clinicopathological study of 303 cases spanning the non-neoplastic, benign and malignant spectrum.

Aims: Thymectomy specimens are uncommon in routine histopathology practice. However, awareness of various pathologic entities and definite reporting of these specimens is paramount to optimal patient management. Our objective was to determine the histomorphologic spectrum of thymectomy specimens spanning the non-neoplastic, benign to malignant spectrum. Methods and Results: Thymectomies received over an 8-year period were retrospectively analyzed by reviewing clinical details and histologic findings in detail, incorporating the latest World Health Organization (WHO) 2015 histologic classification. A total of 303 thymectomy specimens (179 males/124 females, mean age 45.3 years [3-84 years]) were included. Around 51.2% (n = 155) patients had associated myasthenia gravis (MG), while 17.5% (n = 53) had incidentally detected anterior mediastinal mass (AMM). Non-neoplastic and benign pathologies comprised 31% (n = 94) cases and showed stronger association with MG (P = 0.009). Thymic follicular hyperplasia (TFH) was the commonest non-neoplastic pathology (n = 32), while the benign tumor group included thymic hemangioma/lymphangioma, thymolipoma, and ectopic parathyroid adenoma. Thymic epithelial tumors (TETs) comprised 64.7% cases, with majority being thymomas (185/303; 61.1%). Thymoma type B2 was the commonest histologic subtype and Stage I/T1 was the most frequent stage. Type A and AB thymomas affected older patients (P = 0.005) and were in lower stage (both Masaoka and American Joint Committee on Cancer [AJCC]) than type B thymomas (P = 0.007). No significant association between MG and thymoma subtype, patient sex or Masaoka stage was seen (P > 0.05). Thymic carcinomas comprised 11 cases and showed no association with MG (0/11, P < 0.001); squamous cell carcinoma was the commonest histologic type (8/11; 72.7%). Conclusion: TETs are the commonest thymic lesions; however, a diverse spectrum of pathologic processes can affect the thymus.

IgG4 related sclerosing encapsulating peritonitis with cocoon formation: An unusual and undescribed presentation.

IgG4-related sclerosing mesenteritis is a rare disease of mesentery of an unknown etiology which shows a constellation of histopathologic findings of lymphoplasmacytic inflammation with IgG4-positive plasma cells and marked fibrosis. This chronic inflammatory condition of mesentery forming an abdominal cocoon has never been described before to the best of our knowledge. Here, we report a patient with a history of subacute small bowel obstruction who was found to have an intra-abdominal encapsulating mass in the right iliac fossa and was finally diagnosed as IgG4-related sclerosing encapsulating peritonitis (abdominal cocoon) based on peroperative findings, histology and immunohistochemistry.

Palliative and end-of-life care in intensive care units in low- and middle-income countries: A systematically constructed scoping review.

PURPOSE: Death is common in intensive care units, and integrating palliative care enhances outcomes. Most research has been conducted in high-income countries. The aim is to understand what is known about the type and topics of research on the provision of palliative care within intensive care units in low- and middle-income countries MATERIALS AND METHODS: Scoping review with nine databases systematically searched for literature published in English on palliative care in intensive care units in low- and middle- income settings (01/01/1990 to 31/05/2021). Two reviewers independently checked search results and extracted textual data, which were analyzed and represented as themes. RESULTS: Thirty papers reported 19 empirical studies, two clinical case reports and six discussion papers. Papers originated from Asia and Africa, primarily using observational designs and qualitative approaches, with no trials or other robust evaluative or comparative studies. No studies directly sought data from patients or families. Five areas of research focus were identified: withholding and withdrawing treatment; professional knowledge and skills; patient and family views; culture and context; and costs of care. CONCLUSIONS: Palliative care in intensive care units in low-and middle-income countries is understudied. Research focused on the specific needs of intensive care in low- and middle-income countries is required to ensure optimal patient outcomes.

"Building palliative care capacity in cancer treatment centres: a participatory action research".

INTRODUCTION: There is a significant lack of palliative care access and service delivery in the Indian cancer institutes. In this paper, we describe the development, implementation, and evaluation of a palliative care capacity-building program in Indian cancer institutes. METHODS: Participatory action research method was used to develop, implement and evaluate the outcomes of the palliative care capacity-building program. Participants were healthcare practitioners from various cancer institutes in India. Training and education in palliative care, infrastructure for palliative care provision, and opioid availability were identified as key requisites for capacity-building. Researchers developed interventions towards capacity building, which were modified and further developed after each cycle of the capacity-building program. Qualitative content analysis was used to develop an action plan to build capacity. Descriptive statistics were used to measure the outcomes of the action plan. RESULTS: Seventy-three healthcare practitioners from 31 cancer treatment centres in India were purposively recruited between 2016 and 2020. The outcome indicators of the project were defined a priori, and were audited by an independent auditor. The three cycles of the program resulted in the development of palliative care services in 23 of the 31 institutes enrolled in the program. Stand-alone palliative care outpatient services were established in all the 23 centres, with the required infrastructure and manpower being provided by the organization. Morphine availability improved and use increased in these centres, which was an indication of improved pain management skills among the participants. The initiation and continuation of education, training, and advocacy activities in 20 centres suggested that healthcare providers continued to remain engaged with the program even after the cessation of their training cycle. CONCLUSION: This program illustrates how a transformational change at the organizational and individual level can lead to the development of sustained provision of palliative care services in cancer institutes.