| Summary: | Background: Usually, hematolymphoid malignancies present as generalized lymphadenopathy, splenomegaly, and cytopenias. However, sometimes, this may not be the scenario, and that is where the challenge arises. These unusual scenarios are present either in extranodal lymphoma or extramedullary acute leukemia, especially occurring at unusual sites and these often lays down a diagnostic dilemma. Aims: This study aims to evaluate the utility of extensive workup with amalgamation of radiological imaging, immunohistochemical panel, and flow-cytometry to diagnose these unusual hematolymphoid malignancies. Materials and Methods: This is a retrospective observational study of results obtained from a series of nine patients with various unusual manifestations of extranodal lymphoma or acute leukemia at tertiary center of Northern India since the past 18 months. Results: All nine cases which showed variable and unusual clinical presentations were studied involving all available ancillary techniques in the form of immunohistochemistry (IHC), flow cytometry (FCM). These cases because of their unusual presentation in the form of recurrent pericardial/peritoneal effusion, abdominopelvic masses, chronic cervicitis, and subacute intestinal obstruction mimicked various neoplastic and nonneoplastic lesions. IHC and/or FCM evaluating CD34, MPO, CD117 along with Tdt and B/T-cell markers played a vital role for a definitive diagnosis of aleukemic myeloid sarcoma primarily involving intestine, T-cell lymphoblastic lymphoma (LBL) in ovary, diffuse large B-cell lymphoma in ovary, cervix, and intestine with peritoneal carcinomatosis. Two cases of mediastinal masses revealed primary thymic B-cell lymphoma and T-cell LBL, respectively. Conclusion: Cases have highlighted the unusual clinical presentation of hematolymphoid malignancies and elicit the importance of providing a definitive opinion, which is mandatory to give appropriate effective and timely management for the best outcome.
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