Inflammatory myofibroblastic tumor is a histopathologically distinctive neoplasm of children and young adults. According to World Health Organization (WHO) classification, inflammatory myofibroblastic tumor is an intermediate-grade tumor, with potential for recurrence and rare metastasis. There are no definite histopathologic, molecular, or cytogenetic features to predict malignant transformation, recurrence, or metastasis. A 5-year retrospective study of histopathologically diagnosed inflammatory myofibroblastic tumors of various anatomic sites was conducted to correlate anaplastic lymphoma kinase-1 (ALK-1) expression with histological atypia, multicentric origin of tumor, recurrence, and metastasis. Clinical details of all the cases were noted from the clinical work station. Immunohistochemical stains for ALK-1 and other antibodies were performed. Statistical analysis was done using Fisher exact test. A total of 18 cases of inflammatory myofibroblastic tumors were found during the study period, of which 14 were classical. The female-male ratio was 1:1 and the mean age was 23.8 years. Histologically atypical (four cases) and multifocal tumors (three cases, multicentric in origin) were noted. Recurrence was noted in 30% of ALK-1 positive and 37.5% of ALK-1 negative cases, whereas metastasis to the lung, liver, and pelvic bone was noted in the ALK-1 positive group only. Overall, ALK-1 protein was expressed in 55.6% of inflammatory myofibroblastic tumors. There was no statistically significant correlation between ALK-1 expression, tumor type, recurrence and metastasis. However, ALK-1 immunohistochemistry is a useful diagnostic aid in the appropriate clinical and histomorphologic context.
1 June 2016 in Journal of Clinical and Diagnostic Research
Papillary cystadenocarcinoma is a rare malignant neoplasm of the salivary gland, characterized by noticeable cystic and solid areas with papillary endophytic projections. These tumours lack features that characterize cystic variants of several more common salivary gland carcinomas. It was first described in 1991 by World Health Organization as a separate entity and cystadenocarcinoma with or without papillary component in the AFIP classification. Most of these tumours occurred in the major salivary glands followed by minor salivary glands. Cystadenocarcinoma is the malignant counterpart of cystadenoma. We report a case of papillary cystadenocarcinoma of parotid. A 40-year-old lady presented with gradually progressive swelling below the right ear associated with occasional pain. Clinical and radiological features suggested benign neoplasm. Right lobe superficial parotidectomy was performed. The histopathologic diagnosis showed papillary cystadenocarinoma of the parotid gland. Histologic confirmation of stromal invasion is required to differentiate it from the benign lesion. Conservative wide local surgical excision is the treatment of choice.
1 February 2016 in Journal of Clinical and Diagnostic Research
Journal of Clinical and Diagnostic Research aims to publish findings of doctors at grass root level and post graduate students, so that all unique medical experiences are recorded in literature.
Malignant gastrointestinal neuroectodermal tumor (GNET), also referred to as clear cell sarcoma-like tumor of the GI tract is a rare mesenchymal tumor of the gastrointestinal tract. It has to be distinguished from various mimickers including gastrointestinal stromal tumor (GIST) due to its aggressive course and different natural history and therapeutic approach. Here we report a case of GNET arising in the small intestine with aberrant DOG1 expression posing a diagnostic challenge. In this context, the combination of clinical, histomorphological, immunohistochemical, and molecular features helped to establish a proper diagnosis.
Objectives The study was aimed to (1) evaluate the effectiveness of clinical examination, intraoperative finding, and contrast-enhanced computed tomography (CECT) to detect extranodal extension (ENE) in buccoalveolar complex squamous cell carcinoma (BAOSCC), (2) to know various factors influencing ENE, and (3) to evaluate survival outcome in patients with ENE. Materials and Methods This was a retrospective cohort study, which included 137 patients with BAOSCC who underwent curative treatment between May 2019 and April 2021. Collaborative findings suggestive of ENE were noted during preoperative clinical examination, CECT, and intraoperatively, and their efficacy was compared with postoperative histopathology. Also, the various factors associated with ENE were evaluated and compared. Statistical Analysis Univariate and multivariate analysis of parameters was done using multiple logistic regression analysis and significant correlation was determined using chi-square test between ENE positive and negative categories. Analysis of prognosis and survival was done by Kaplan–Meier curve plotting using regression analysis and its significance was compared. Results The overall prevalence of ENE was 18.98% and that of lymph node involvement was 40.88%. CECT (73.1%) was found to be more sensitive in detecting ENE compared to intraoperative examination (46.2%) and clinical examination (34.6%).In comparison with clinical examination (91.9%) or CECT (78.38%), intraoperative examination (93.7%) showed the highest specificity in detecting ENE. Clinical nodal size ≥ 3 cm (p ≤ 0.001), fixity (p ≤ 0.001), and clinical number of nodes (p ≤ 0.001) had significant association with ENE. The presence of thick nodal walls on CECT increased the probability of predicting ENE 15 times (p = 0.180, confidence interval: 0.3–765.4). After a mean follow-up of 18 months, subjects without nodal positivity had a survival advantage over patients with positive lymph nodes (86.4% vs. 53.3%) and those with ENE (86.4% vs. 23.2%), respectively. Conclusion The results demonstrated that clinical examination can be used as an adjuvant to radiological imaging for prediction of ENE preoperatively. Clinical finding suggesting size of node ≥ 3 cm and ≥ 2 nodes are strong predictor of ENE, in addition to other known predictors. Patients with ENE had an unfavorable prognosis when compared with subjects with metastatic nodes without ENE. Presence of ENE remains one of the strongest factors predicting recurrence and thus poor prognosis.