Bronchial Dieulafoy's disease: A series of seven cases with review of the literature.

Bronchial Dieulafoy's disease (BDD), remains poorly understood, with only 88 cases reported globally. Herein, we present the largest case series (n = 7) from a single centre, between 2017 and 2023, retrospectively reviewed, detailing clinical presentations, diagnoses, management and up to 4-year follow-up outcomes. Diagnosis relied on characteristic lesions detected through white light bronchoscopy with or without endobronchial ultrasound (EBUS) or narrow band imaging (NBI), along with computed tomography (CT) scans or bronchial angiography. Identification of aberrant vessels beneath lesions and bronchoscopy details were documented. Treatment modalities and follow-up outcomes until December 2023 were noted. All patients were non-smokers. Review of imaging findings by an experienced radiologist was crucial in suspected cases due to risk of bleeding and often unconclusive results from biopsy. Management of BDD varied, with six patients undergoing bronchial artery embolization (BAE) and one requiring lobectomy; four patients received additional endobronchial therapy, one died due to malignancy, none experienced recurrence of haemoptysis. Identifying patients with large volume haemoptysis disproportionate to parenchymal disease in CT scans is important. A bronchoscopic surveillance is crucial to avoid biopsy; it can be confirmed using EBUS of NBI. While no established guidelines exist, BAE and endobronchial therapy emerge as valuable interventions, with surgical resection reserved for recurrent cases.

Therapeutic Response to PRRNT in a Rare Case of Metastatic Renal Neuroendocrine Carcinoma.

Neuroendocrine tumors (NETs) are a rare spectrum of neoplasms that are characterized by neuroendocrine and neural differentiation. The treatment can be challenging in view of the heterogeneity in differentiation and behavior. Primary renal origin NETs are rare and only a few cases have been reported in the literature. There is limited knowledge on their presentation and response to various lines of treatment. We report a case of a patient with a metastatic renal NET from a rare histological subtype of large cell neuroendocrine carcinoma, known to cause aggressive disease with poor prognosis. A multimodality treatment approach was followed. In spite of surgical management and second-line chemotherapy, the disease progressed. The patient subsequently received peptide receptor radionuclide therapy (PRRNT) using lutetium-177 DOTATATE, following which the patient demonstrated a remarkable clinical and radiological response and is stable to date. In a rare tumor with poor prognosis, the relevance of theranostics and the efficacy of targeted therapies like PRRNT are noteworthy.

Curative Resection for Metastatic Solid Pseudopapillary Neoplasm of Pancreas-a Case Series.

Solid pseudopapillary neoplasm (SPN) is an unusual tumor of the pancreas. Unlike ductal adenocarcinoma, SPN is commoner in young women and is indolent with better prognosis. Fifteen percent of patients, however, develop metastases, often synchronous and involving the liver or peritoneum. Owing to the paucity of cases, management of the metastatic disease is not clearly defined. Retrospective review of case notes of patients treated between 2006 and 2019. There were 53 patients with SPN of which 4 had hepatic metastases-3 synchronous and 1 metachronous. Two patients underwent simultaneous distal pancreatectomy and splenectomy with liver resection (right posterior sectionectomy and metastasectomy). One required right hepatectomy with metastasectomy for metachronous liver metastases. The other underwent a staged operation-remnant tumor excision with metastasectomy followed by right hepatectomy after portal vein embolization. All four patients are recurrence free on median follow-up of 38.5 months. In view of the excellent prognosis, we recommend radical resection of both the primary lesion and metastases for SPN.

Myofibroblast and pro-fibrotic cytokines in fibrosis of IgG4-related disease (IgG4-RD) patients from South Asia: preliminary data.

INTRODUCTION: Fibrosis is a typical pathological characteristic in IgG4-RD patients and often irreversible. There exists a lack of suitable markers for detection of earlier onset of fibrosis in various organs in IgG4-RD patients. Hence, this study aims at analysing ambispectively the myofibroblasts and the pro-fibrotic cytokines, IFN gamma and IL-33 involved in IgG4-RD associated fibrosis in South Asian patients. METHOD: Archived biopsy samples of definite/probable/possible cases of IgG4-RD, classified according to diagnostic criteria, taken from patients who attended the OPD and IPD of our tertiary care centre during January 2015-January 2020 were chosen for this study. The paraffin sections were examined qualitatively for fibrosis and the excessive collagen deposition by Hematoxylin & Eosin and Masson's Trichrome staining. Also, the presence of alpha-Smooth muscle actin (α-SMA) expressing myofibroblasts and the involvement of pro-fibrotic cytokines (IFN-gamma, IL-33) were assessed by Immunohistochemistry and scored semi-quantitatively (+mild, ++moderate, +++ severe). Serum IL-33 levels were analysed by indirect Elisa (R & D Systems). RESULTS: Myofibroblasts were present in 10/12 biopsy samples, in moderate levels in 4 (33%) and very high levels (+++) in 3 (25%) of the patients. IFN-gamma was expressed at low levels in 6 (50%) and absent in 6 (50%). All patients showed IL-33 expression with very high levels in tissue (6, 50%), as well as in serum samples. CONCLUSION: The findings of this study reinforce the role of myofibroblasts and profibrotic cytokines like IL-33 in fibrosis of Ig4-RD patients, pointing to their potential as earlier predictive markers of onset and extent of fibrosis.

Surgical management of sternal tumours-a decade of experience from a tertiary care centre in India.

Tumours of the sternum can be either primary or secondary with malignancy being the most common etiology. Wide local excision of these tumours results in a midline defect which pose a unique challenge for reconstruction. As limited data on the management of these tumours exists in the literature, we hereby report 14 consecutive patients who were treated at our institute between January 2009 to December 2020. Most of them were malignant with majority of them, 11 (78%) patients, with manubrial involvement requiring partial sternectomy. Overall, the average defect size was 75 cm2. Reconstruction of the chest wall defect was done using a semi-rigid fixation: mesh and suture stabilization in 3 (21%) or suture stabilization in 7 (50%) and without mesh or suture stabilization in 3 (21%) patients. Rigid fixation with polymethyl methacrylate (PMMA) was done for one patient (7%). Pectoralis major advancement flap was most commonly used for soft tissue reconstruction with flap necrosis noted in one patient (7%). There was no peri-operative mortality and one patient required prolonged post-operative ventilation. On a median follow-up of 37.5 months, one patient (7%) had a recurrence. Sternal defects after surgical resection reconstructed with semi-rigid fixation and suture stabilization render acceptable post-operative outcomes.

Single-Centre Experience With Low-Volume Plasma Exchange and Low-Dose Steroid to Treat Patients With Idiosyncratic Drug-Induced Acute Liver Failure.

Background: Idiosyncratic drug-induced liver injury (iDILI) causing acute liver failure (ALF) carries high short-term mortality and patients who meet King's College criteria for liver transplantation have 1-month survival of 34% without liver transplantation (PMID: 20949552). We present our experience with low-volume plasma exchange (PLEX-LV, 50% of estimated plasma volume exchanged per session) and low-dose steroid to treat iDILI ALF. Methods: We retrospectively analysed data of patients with iDILI (diagnosed as per RUCAM score), treated with PLEX-LV and low-dose steroid (prednisolone: 10 mg OD, with rapid taper) in our department from 2016 to 2022. Baseline and dynamic parameters (post-PLEX) were assessed as predictors of 1-month liver transplantation-free survival. Results: Twenty-two iDILI patients [probable: possible iDILI: 20:2, males: 9, age: 30 (14-84) years, median (range); MELD score: 30.5 (19-43)] underwent PLEX-LV for ALF during the study period. Causative agents were complementary and alternative medications (36%), antiepileptics (18%) antimicrobials (14%), antitubercular drugs (14%), antifungal drugs (9%) and others (9%). All patients had jaundice and encephalopathy; 9 patients also had ascites. None of the patients underwent liver transplantation. Study patients underwent 3 (1-7) PLEX sessions and 1.4 (0.6-1.6) litres of plasma was exchanged per session. One-month transplant-free survival was 59% (13/22) in the study population and 63% (12/19) among patients who fulfilled Kings College criteria for liver transplantation. Reduction of ≥25% in plasma von Willebrand factor (VWF) levels after PLEX-LV predicted improved survival (HR: 0.09, 95% CI: 0.01-0.65; AUROC: 0.81; 95% CI: 0.6-1.0). Conclusion: Low-volume PLEX and low-dose steroid appears a promising treatment option in patients with iDILI-induced ALF not opting for liver transplantation. Dynamic changes in VWF level after PLEX predict 1-month survival in these patients.

Focused panel sequencing points to genetic predisposition in non-cirrhotic intrahepatic portal hypertension patients in India.

OBJECTIVE: Non-cirrhotic intrahepatic portal hypertension (NCIPH), a portal microangiopathy affecting small portal vein radicles, is a disease of Indian sub-continent. NCIPH appears to be a complex disease with interactions between inherited and acquired factors, though the exact pathophysiological mechanism is unknown. We aimed at investigating the genetic variants that might contribute to susceptibility to NCIPH. METHODS: In this case-control study, we analyzed genes associated with microangiopathy-VWF-ADAMTS13 (von Willebrand factor and its cleavase enzyme - a disintegrin and matrix metalloprotease with thrombospondin type-1 motifs member 13) and alternative complement system vitamin B12 metabolism and with familial NCIPH. RESULT: Eighty-four Indian patients with liver biopsy-proven NCIPH (cases) and 103 healthy controls (matched for residential region of India) were included in the study. Targeted next-generation sequencing (NGS) panel, comprising 11 genes of interest, was done on 54 cases. Genotyping of selected variants was performed in 84 cases and 103 healthy controls. We identified variants in MBL2, CD46 and VWF genes either associated or predisposing to NCIPH. We also identified a single case with a novel compound heterozygous mutation in MBL2 gene, possibly contributing to development of NCIPH. CONCLUSION: In this first of a kind comprehensive gene panel study, multiple variants of significance have been noted, especially in ADAMTS13-VWF and complement pathways in NCIPH patients in India. Functional significance of these variants needs to be further studied.

Rib malignancies masquerading as benign mediastinal tumours.

Mediastinal tumours are commonly encountered in general thoracic surgery practice. Benign tumours like thymoma and teratoma often need direct surgical resection if resectable. Pre-operative biopsy is not recommended. We report 2 cases which were radiologically diagnosed as thymoma and teratoma turned out to be Ewing sarcoma and chondrosarcoma respectively after surgical excision. This has challenged the notion whether biopsy is really not needed. More elaborate studies are needed to find out tumour characteristics which would warrant a biopsy even if they are resectable. In our cases, pre-operative biopsy would have changed the line of management in both patients. This has led to a change in institutional protocol that now, we do biopsy for any mediastinal tumour more than 8 cm and abutting the chest wall.

Pulmonary sclerosing pneumocytoma-a case series.

Pulmonary sclerosing pneumocytoma (PSP) is a rare and interesting pulmonary tumour that presents a diagnostic challenge to the surgeon due to its bland symptomatology, indolent course, non-specific radiological features and ambiguous histopathological findings, which could lead to errors in management and prognostication. Herein, we present a series of 4 patients with PSP who were managed in our institution over a period of 8 years and briefly discuss relevant literature.

Pulmonary mucormycosis-a case series.

Pulmonary mucormycosis (PM) is a rare disease. Literature about the surgical management of PM in India is sparse. Recently, there has been a surge in the number of cases of mucormycosis in association with the coronavirus disease 2019 (COVID-19) pandemic, igniting the interest in PM. Hence, we endeavoured to analyse our surgical experience in treating PM prior to the pandemic, in a tertiary care centre, to provide insight into this disease. Data of 10 adult patients with isolated PM, who underwent surgery between the years 2009 and 2020, and maintained in our departmental database, were retrieved and analysed.

Giant cystic pulmonary hamartoma-images.

Hamartoma is the commonest benign tumor of the lung. They usually present as an asymptomatic solitary solid nodule of varying size, located peripherally in the lung parenchyma and picked up incidentally in a chest X-ray. Rarely, they may be intra-bronchial in location. A giant cystic variant of hamartoma is very rare, with only a few cases reported in literature so far. It may be misdiagnosed as any other cystic disease of the lung and thus get mismanaged. We herewith present the images of a patient with a giant cystic pulmonary hamartoma who presented with respiratory distress.

Elucidating the anatomy of a rare communicating bronchopulmonary foregut malformation (CBPFM) in a preschool child.

Communicating bronchopulmonary foregut malformations (CBPFMs) are complex and rare anomalies. Their characteristic feature is an anomalous communication between the respiratory system (trachea, lung or bronchus) on one side and the gastrointestinal tract (oesophagus or stomach) on the other. Though acquired CBPFMs are known, the large majority of them are congenital and single. CBPFMs often go undetected even at surgery and require more than one operation before they are successfully addressed. This is because the symptomatology of CBPFM resembles the more common oesophageal atresia (OA) with tracheoesophageal fistula, wherein it may coexist. We report a patient with OA who had a rare form of CBPFM where the upper lobe of the right lung communicated with the upper oesophagus. This account highlights a novel method of working out the uncertain anatomy, in such cases. There may be associated anomalies of the lung parenchyma and vasculature usually involving the pulmonary arterial supply to the affected lung. Clinical, radiological, endoscopic and pathological characterisation permit precise diagnosis in most instances, with an occasional case that defies definition.

Myositis ossificans-a rare tumor of the chest wall.

Myositis ossificans (MO) is the abnormal formation of benign heterotopic bone tissue in soft tissues or muscles, mostly in sites of trauma. Though it has been described in most parts of the body, less than a dozen cases involving the chest wall have been reported. It is known to resolve spontaneously and various medical treatments have been suggested to hasten its resolution. Large tumors, suspicion of malignancy, and presence of symptoms are indications for surgical intervention. The differential diagnoses include sarcomas, infections, callous, calcified hematomas, and cysts. We present the clinical, radiological, and pathological images of a post traumatic MO of the chest wall, arising from under the medial third of the clavicle and growing into the deeper surface of the pectoralis major muscle. The patient is doing well eight months after the excision of the same.

Case of functioning thoracic paraganglioma.

Functioning thoracic paraganglioma (PGL) is rare in clinical practice. We present a 33-year-old man with this pathology, who came with right-sided chest pain and was found to have a right-sided paravertebral mass. Fine needle aspiration cytology revealed a PGL. Urine normetanephrine was elevated and meta- iodobenzylguanidine scan showed increased tracer uptake in the right hemithorax, suggestive of a functioning neuroendocrine tumour. The patient was subjected to right PGL excision by video-assisted thoracoscopic surgery, after adequate preoperative preparations. The perioperative period was uneventful, except for a transient rise in blood pressure during the surgery. His blood pressure continued to be normal in the postoperative period. In any patient with a paravertebral mass, the possibility of PGL should be kept in mind even if the patient is normotensive. Making a preoperative diagnosis is important, because excision of functioning PGL without adequate preoperative preparation may be detrimental.

Role of Cross-sectional Imaging (CT/MRI) in Characterization and Distinguishing Benign from Malignant/Potentially Malignant Cystic Lesions of Pancreas.

OBJECTIVES: The aim of the study was to evaluate the accuracy of computed tomography/magnetic resonance imaging (CT/MRI) in characterizing cystic lesions of the pancreas and in differentiating between benign and malignant/potentially malignant lesions. MATERIAL AND METHODS: A retrospective study was performed on patients with pancreatic cystic lesions who underwent pre-operative imaging and surgery between October 2004 and April 2017 at a tertiary care teaching hospital. The images were reviewed for specific characteristics and diagnoses recorded independently by two radiologists who were blinded to the histopathological examination (HPE) report. Radiological diagnostic accuracy was assessed with HPE as reference standard. RESULTS: A total of 80 patients fulfilled the inclusion criteria (M: F = 27:53). The final HPE diagnoses were solid pseudopapillary neoplasm (32.5%), walled off necrosis/pseudocyst (27.5%), mucinous cystadenoma (15%), serous cystadenoma (11.25%), intraductal papillary mucinous neoplasm (8.75%), mucinous cystadenocarcinoma (2.5%), simple epithelial cyst (1.25%), and unspecified benign cystic lesion (1.25%). Observer1 correctly identified the diagnosis in 73.75% of cases while observer 2 did so in 72.5%. Sensitivity for distinguishing benign versus malignant/potentially malignant lesions was 85.1% for observer 1 and 80.9% for observer 2. On multivariate logistic regression analysis: Solid cystic morphology, presence of mural nodule, and female gender were associated with premalignant/malignant lesions. CONCLUSION: Cross-sectional imaging is a valuable tool for characterization of pancreatic cystic lesions within its limitations.

Atypical presentation of intrahepatic cholangiocarcinoma---Fever and ascites in a postpartum lady.

Intrahepatic cholangiocarcinoma is an uncommon malignancy which usually occurs in the 7th decade. Here we present a postpartum patient with fever, hepatomegaly, and ascites, who was diagnosed to have metastatic intrahepatic cholangiocarcinoma.

Organising pneumonia in Rhupus syndrome.

Interstitial lung disease (ILD) is seen in 17% of patients with Rhupus syndrome. Organising pneumonia (OP), a subtype of connective tissue disease-associated ILD, is rare but associated with good outcomes. Here, we present a patient with Rhupus who developed OP.