Perioperative, oncological, and survival outcomes of robotic radical cystectomy with urinary diversion in females: A single-center retrospective observational study.

Introduction: Robot-assisted radical cystectomy (RARC) is a standrad approach for surgical management of bladder cancer. Currently, most literature on RARC is in men, possibly due to the higher incidence of bladder cancer in males. We reviewed the perioperative, oncological and survival outcomes in 41 women who underwent RARC by a single surgeon at a tertiary health-care center. Methods: Out of 225 RARC and urinary diversion procedures performed from 2012 to 2020, a retrospective analysis of 41 women was performed. Baseline demographic and perioperative details, oncological data, and survival were recorded and analyzed. Kaplan-Meir analysis was done for survival outcomes and prognostic factors were assessed by log rank test. Results: Thirty-eight patients underwent intracorporeal urinary diversion, while three underwent extracorporeal diversion. One patient underwent organ preserving cystectomy. Clavien-Dindo 30-day postoperative complications were Grade I in 8 (19.5%), Grade II in 4 (9.8%), and Grade IIIa in 3 (7.3%) patients with no mortality. During the median follow-up of 34 months (range: 6-87 months), 7 patients died of disease recurrence. Five-year survival was 74% (95% confidence interval [CI]: 59-82) and 35% (95% CI: 10-91) in transitional cell carcinoma (TCC) and non-TCC group, respectively, with P = 0.04. There was no mortality in Stages 0 and 1 disease. Five-year survival was 78% in Stage 2 and 41% in Stage 3 and 4. Conclusion: Our study demonstrates acceptable clinical, perioperative, and oncological outcomes of robotic radical cystectomy in females, thus highlighting its safety and feasibility.

Mutational Analysis of EGFR Mutations in Non-Small Cell Lung Carcinoma-An Indian Perspective of 212 Patients.

Lung cancer is the world's leading cause of cancer-related deaths. Epidermal growth factor receptor (EGFR) is one of the critical oncogenes and plays a significant role in tumor proliferation and metastasis. Patients with sensitizing mutations in the EGFR gene have better clinical outcomes when treated with tyrosine kinase inhibitors (TKI). This study expands our knowledge of the spectrum of EGFR mutations among lung cancer patients in the Indian scenario. This is a retrospective descriptive study of all newly diagnosed patients with lung cancer in tertiary care hospital in India. All the samples were subjected to real-time PCR (q-PCR) analysis and confirmation of rare novel mutations was done using Sanger sequencing. Clinicopathological characteristics, mutational EGFR status, and location on the exon and metastatic sites were evaluated. An analysis of total 212 samples showed mutations in 38.67% of cases. Among these, five (5.9%) samples had mutations in exon 18, 41 (48.8%) samples had mutations in exon 19, 12 (14.28%) samples had mutations in exon 20, and 26 (30.95%) samples had mutations in exon 21. Eleven (13.41%) were found to be uncommon EGFR mutations. Additionally, six (21.4%) samples that had EGFR mutations were also positive for brain metastasis. Future testing on bigger panels will help to characterize the incidence of genetic mutations and to determine the appropriate targeted treatment choices for NSCLC patients.

A Difficult Case of Pyrexia of Unknown Origin.

This is the case of a 15-y-old boy who presented with fever and back pain with MRI features of spondylitis. A CT-guided vertebral biopsy showed acute and chronic inflammatory cells and grew Pseudomonas aeruginosa on aerobic culture. The child was treated for 2 wk with antibiotics with no response. Meanwhile, he developed new lung, liver, and splenic lesions on CT imaging. Empiric antitubercular therapy was then started and continued for 8 wk during which time there was progressive clinical deterioration. At this time the patient underwent bronchoscopy with lavage and endoscopic ultrasound-guided subcarinal lymph node and lung biopsy. The Xpert MTB/Rif ULTRA was "trace positive" in the bronchoscopic lavage with indeterminate rifampicin resistance, while it was negative in lymph node and lung biopsy. The lymph node and lung biopsy histopathology showed nonspecific inflammatory changes with no granulomas or malignant cells. In view of the positive Xpert ULTRA with indeterminate rifampicin resistance and no response to first-line drugs, treatment with second-line antitubercular drugs was initiated. The clinical condition continued to deteriorate; here the imaging findings were reviewed again and repeat aspiration cytology and biopsy from intra-abdominal nodes was carried out. This yielded the diagnosis of Hodgkin lymphoma. The patient had stage IVB disease. He responded well to standard chemotherapy and is currently in remission. The case illustrates the need to avoid empiric therapy, repeat invasive procedures if so needed, choose the site/method of biopsy appropriately and interpret results of investigations carefully when evaluating a patient with pyrexia of unknown origin.

Neonatal respiratory distress produced by a nasopharyngeal glioma with a persistent craniopharyngeal canal.

Nasopharyngeal gliomas are congenital masses of heterotopic glial tissue. Our case report describes an infant with respiratory distress produced by a nasopharyngeal glioma with a persistent craniopharyngeal canal (CPC). The identification of CPC is important for medical and surgical management of possible pituitary dysfunction and preventing operative and post-operative complications.

An Overview of Liver Transplant Pathology: Data from a Tertiary Referral Centre in Western India.

INTRODUCTION AND AIM: 1. Study of liver explants - Etiologic types of end-stage chronic liver disease (ESCLD) and acute liver failure (ALF) in adults and children. 2. Assessment of donor steatosis and incidental granulomas. 3. Post-transplant liver biopsies. MATERIAL AND METHODS: Specimens of 180 explant hepatectomies, 173 donor wedge and 30 core liver biopsies, and 58 post transplant liver biopsies received in our department from April 2013 to March 2017. RESULTS: 1. Most common causes of ESCLD in adults were: alcohol related (30.32%), hepatitis virus related (18.71%) and non-alcoholic steatohepatitis related (18.06%); and in children ≤ 12 years were: biliary atresia (27.27%), autoimmune disease (18.18%) and Wilson's disease (18.18%). Most common causes of ALF in adults and children were anti-tubercular therapy induced and idiopathic respectively. 2. Prevalence rate of moderate steatosis (between 30-60%) was 4.28%. Incidental granulomas were seen in 5 cases. 3. Most common diagnoses of post-transplant biopsies in adults included acute cellular rejection (ACR) (36.17%), recurrence of viral disease (8.51%) and moderate non-specific portal triaditis (8.51%). Among children ≤ 12 years, most common diagnoses included unremarkable liver parenchyma, ACR and ischemia/reperfusion injury. CONCLUSION: 1. Alcohol- and hepatitis- virus related ESCLD, and biliary atresia are leading indications for liver transplantation in adults and children respectively. 2. Prevalence of 4.28% of moderate steatosis, is much lower than that documented in western literature. Only 5 cases of incidental granulomas is unexpectedly low in a country endemic for tuberculosis. 3. Most common diagnoses of post-transplant liver biopsies in adults has been acute rejection, which is similar to the findings from much larger published series.

Immunoglobin G4-related disease: Two case reports and review of the recent literature.

IgG4-related disease (IgG4-RD) is a new, multiorgan and constantly evolving disease characterized by IgG4-positive plasma cells in the affected organ. This disease often affects the elderly. The pancreas is the main target organ affected, with almost all organs in the body being affected. A large amount of studies have been conducted to understand the pathogenesis of the disease and its spectrum. For accurate diagnosis of the condition, an adequate knowledge of imaging findings, clinical presentations and laboratory reports is essential. We report two cases of the IgG4-RD and review the recent literature about this increasingly recognized entity.

Interobserver variation is a significant limitation in the diagnosis of Burkitt lymphoma.

CONTEXT: The pathology of classic Burkitt lymphoma (BL) remains a challenge despite being a well-defined entity, in view of the significant overlap with atypical BL and B-cell lymphoma intermediate between DLBL (diffuse large B cell lymphoma) and BL. They are difficult to be segregated in resource-limited setups which lack molecular testing facilities. This is further affected by interobserver variability and experience of the reporting pathologist. AIMS: The aim of our study was to quantitate variability among a group of pathologists with an interest in lymphomas (albeit with variable levels of experience) and quantitate the benefit of joint discussions as a tool to increase accuracy and reduce interobserver variability of pathologists, in the diagnosis of BL in a resource-limited setup. MATERIALS AND METHODS: A set of 25 non-Hodgkin lymphoma cases in which a diagnosis of BL was entertained were circulated to 14 participating pathologist within the Mumbai lymphoma study group. A proforma recorded the morphologic and immunohistochemical features perceived during the initial independent diagnosis followed by a consensus meeting for discussion on morphology and additional information pertinent to the case. STATISTICAL ANALYSIS AND RESULTS: The concordance was poor for independent diagnosis among all the pathologists with kappa statistics (±SE) of 0.168 (±0.018). Expert lymphoma pathologists had the highest (albeit only fair) concordance (kappa = 0.373 ± 0.071) and general pathologists the lowest concordance (kappa = 0.138 ± 0.035). Concordance for morphological diagnosis was highest among expert lymphoma pathologists (kappa = 0.356 ± 0.127). Revision of diagnoses after consensus meeting was highest for B-cell lymphoma intermediate between DLB and BL. To conclude, interobserver variation is a significant problem in BL in the post WHO 2008 classification era. Experience with a larger number of cases and joint discussion exercises such as the one we conducted are needed as they represent a simple and effective way of improving diagnostic accuracy of pathologists working in a resource-limited setup.

Myxoid lipoblastoma.

A rapidly growing soft tissue mass in the axilla of an infant raises the suspicion of a lipoblastoma or a liposarcoma. Excisional/incisional biopsy is vital in confirming the diagnosis and hence avoiding aggressive extirpation. This case report highlights the role of histopathology and immunohistochemistry as the gold standard in differentiating a lipoblastoma from a liposarcoma. In some cases where the histopathology is inconclusive, genetic rearrangement of the PLAG1 (pleomorphic adenoma gene 1) oncogene on chromosome 8q12 helps in confirming the diagnosis of lipoblastoma.

Idiopathic myelofibrosis in an infant.

Idiopathic Myelofibrosis (MF) is an extremely rare condition in children. It has a very variable clinical spectrum. Cases of secondary myelofibrosis associated with Vitamin D deficiency and Systemic Lupus Erythematosus have been reported from India .In this case report, the authors describe clinical signs, laboratory findings and histologic features in a 6 month old infant with Idiopathic myelofibrosis.

Primary non-Hodgkin's lymphoma of the salivary gland: a spectrum of lymphoepithelial sialadenitis, low-grade B-cell lymphoma of mucosa-associated lymphoid tissue with transformation to high-grade lymphoma.

Lymphoid infiltrates of the salivary gland can be either reactive or neoplastic. The reactive lesion, lymphoepithelial sialadenitis (LESA) may be associated with Sjogren's syndrome (SS) or may occur as an isolated salivary gland enlargement. Patients with LESA/SS have a particularly high risk of subsequently developing lymphoma, which is a low-grade mucosa-associated lymphoid tissue (MALT) type lymphoma of the salivary gland. We document a rare case of primary non-Hodgkin's lymphoma of the parotid gland arising in the background of LESA and with a rare example of transformation from low grade to high-grade B cell lymphoma of MALT type.

Self inflicted, bilateral oleogranuloma of the breast: report of a bizarre case.

A 30-year-old male was admitted with bilateral painful breast enlargement of 6 months duration. There was a past history of surgery for similar bilateral swellings, which was then diagnosed as fibrocystic disease. On examination both breasts were firm to hard, tender & adherent to deeper structures. A single axillary lymph node was palpable on each side. The clinical diagnosis was sarcoma of the breast. After initial denial, the patient confessed to having injected gear oil into both the breasts,for the purpose of augmentation. He was a homosexual.

Granulocytic sarcoma of breast: an aleukemic presentation.

Granulocytic sarcoma is a rare extramedullary tumor composed of immature myeloid cells. It is usually associated with leukemia or other myeloproliferative disorders but can also occur without overt hematologic diseases. The breast is an uncommon site of presentation and requires a high index of suspicion for diagnosis. We report such a case in a 45-year-old female, who presented with nontender left breast lump of 6 months' duration. A peripheral smear and bone marrow examination at that time was normal. A lumpectomy was done. An H and E diagnosis of lobular carcinoma vs. non-Hodgkin's lymphoma was entertained. Immunostains, however, revealed myeloperoxidase, naphthol AS-D chloroacetate esterase and CD43 positivity, indicating a diagnosis of granulocytic sarcoma. It appears that early initiation of systemic AML-type chemotherapy is beneficial and may delay or avert the development of AML in bone marrow and blood. Eight months later, the patient presented with an orbital mass; bone marrow and peripheral smear involvement by AML.

Primary aneurysmal cyst: bone type in the breast.

A 60-year-old women presented with one month's history of a large breast lump. On examination she had irregular hard mass occupying the central and outer quadrants of the right breast with no palpable lymph nodes. Fine needle aspiration cytology (FNAC) diagnosis was a benign stromal lesion. In view of the large size, a simple mastectomy was performed. We are reporting this case of primary extrasseous aneurysmal cyst of the bone in the breast as the first case described in the literature.