Orbital rhabdomyosarcoma (RMS) is a highly malignant tumor that originates mainly from mesenchymal tissue. It is considered a rare childhood malignancy; therefore, presentation in adulthood is scarce. In this case report, a 36-year-old male patient presented with a rapidly progressive RMS of the left orbit. The patient was treated in a different institution with radiochemotherapy with good response. However, relapse occurred after 2 months of completing the course, as rapid growing proptosis. Computed tomography scan revealed a lesion in the left orbit with invasion of the left ethmoid cells and the left maxillary sinus. Exenteration of the left orbit with the left partial maxillectomy and ethmoidectomy was performed. RMS is a highly aggressive malignancy that should be considered in any orbital mass that progresses rapidly. Surgery should be the first line in treatment. Delay in diagnosis and inappropriate treatment negatively affects the outcome.
T-cell/histiocyte-rich B-cell lymphoma is a high-grade, morphologic variant of diffuse large B-cell lymphoma. T-cell/histiocyte-rich B-cell lymphoma. It is rare as a primary splenic involvement and is usually reported as a second malignancy after hairy cell leukemia. Here, we report the first case that describes the occurrence of primary splenic T-cell/histiocyte-rich B-cell lymphoma in a patient with a previous diagnosis of recurrent hairy cell leukemia. A 53-year-old male patient was diagnosed with hairy cell leukemia in 1996 and achieved complete remission with Pentostatin. Then, recurrence of hairy cell leukemia was diagnosed in 2015 and treated with Cladribine. In 2016, he presented with B symptoms and hypersplenism. Therapeutic and diagnostic splenectomy was performed. Histopathological study with immunohistochemistry evaluation revealed the presence of T-cell/histiocyte-rich B-cell lymphoma. Therefore, second malignancies should be considered in patients with a previous neoplasm when symptoms recur or develop.
T-Cell Rich B-Cell Lymphoma (TCRBCL) is relatively a new entity, lately classified as a morphologic variant of Diffuse Large B-cell lymphomas (DLBCL). It consists (1-3) % of all B-cell lymphomas. The rate is far less when describing cases of primary splenic involvement with TCRBCL. Pathologically, TCRBCL is described as a limited number of scattered, large, atypical b-cells embedded in a background of abundant t-cells and frequently histiocytes. The similarity of this malignancy with other types makes it difficult to distinguish between them. Thus, it needs expertise in both clinical and pathological fields to make the right diagnosis. Here, we present a case of an adult male patient whose first presentation and previous medical history of renal colic misguided the initial diagnosis and suggested another colic episode as the underlying ailment. However, further physical, radiological and histopathological investigations uncovered the presence of primary TCRBCL within spleen with no involvement of other sites. Moreover, unusual pathologic finding of CD3 positivity was proved by immunohistochemistry.
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