Primary Testicular Presentation of ALK-1–negative Anaplastic Large Cell Lymphoma in a Pediatric Patient
Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, USA.Journal of Pediatric Hematology/Oncology (Impact Factor: 0.9). 06/2009; 31(5):330-2. DOI: 10.1097/MPH.0b013e3181979c5e
Anaplastic large cell lymphoma is a heterogeneous group of malignant non-Hodgkin lymphomas that occurs in up to 15% of all pediatric non-Hodgkin lymphomas. It is characterized by B-symptoms and involvement of extranodal sites such as skin, bone, and soft tissue. This brief report describes first reported case of pediatric primary testicular anaplastic large cell lymphoma in a 14-year-old boy. The presentation included acute testicular pain, fever, and vomiting. After chemotherapy and unilateral radical orchiectomy, patient continues in complete remission.
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ABSTRACT: The year 2010 commemorates the 25th year since the seminal publication by Karl Lennert and Harald Stein and others in Kiel, West Germany, describing an unusual large cell lymphoma now known as anaplastic large cell lymphoma (ALCL). Investigators at many universities and hospitals worldwide have contributed to our current in-depth understanding of this unique peripheral T-cell lymphoma, which in its systemic form, principally occurs in children and young adults. To summarize our current knowledge of the clinical and pathologic features of systemic and primary cutaneous ALCL. Particular emphasis is given to the biology and pathogenesis of ALCL. Search of the medical literature (Ovid MEDLINE In-Process & Other Non-Indexed Citations and Ovid MEDLINE: 1950 to Present [National Library of Medicine]) and more than 20 years of diagnostic experience were used as the source of data for review. Based on immunostaining for activation antigen CD30 and the presence of dysregulation of the anaplastic lymphoma kinase gene (2p23), the diagnosis of ALCL has become relatively straightforward for most patients. Major strides have been made during the last decade in our understanding of the complex pathogenesis of ALCL. Constitutive NPM-ALK signaling has been shown to drive oncogenesis via an intricate network of redundant and interacting pathways that regulate cell proliferation, cell fate, and cytoskeletal modeling. Nevertheless, pathomechanistic, therapeutic, and diagnostic challenges remain that should be resolved as we embark on the next generation of discovery.Archives of pathology & laboratory medicine 01/2011; 135(1):19-43. DOI:10.1043/2010-0507-RAR.1 · 2.84 Impact Factor
Article: Primary testicular lymphoma[Show abstract] [Hide abstract]
ABSTRACT: Primary testicular lymphoma (PTL) is a rare, clinically aggressive form of extranodal lymphoma. The vast majority of cases are histologically diffuse large B-cell lymphoma, but rarer subtypes are clinically important and must be recognised. In this review we discuss the incidence, clinical presentation and prognostic factors of PTL and present a summary of the recent advances in our understanding of the pathophysiology which may account for the characteristic clinical features. Although outcomes for patients with PTL have historically been poor, significant gains have been made with the successive addition of radiotherapy, full-course anthracycline-based chemotherapy, rituximab and CNS-directed prophylaxis. We describe the larger retrospective series, prospective clinical trials and critically examine the role of radiotherapy. Although 3-weekly RCHOP with intrathecal methotrexate and locoregional RT is the current international standard of care, a substantial minority of patients progress, representing an unmet medical need. Finally, we discuss new treatment approaches and recent discoveries which may translate into improved outcomes for patients with PTL.Blood 11/2013; 123(4). DOI:10.1182/blood-2013-10-530659 · 10.45 Impact Factor
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ABSTRACT: The judicious use of immunostains can be of significant diagnostic assistance in the interpretation of testicular neoplasms when the light microscopic features are ambiguous. A limited differential diagnosis by traditional morphology is required for the effective use of immunohistochemistry (IHC); otherwise, the inevitable occurrence of exceptions to anticipated patterns will lead to "immunoconfusion." The diagnosis of tumors in the germ cell lineage, the great majority of primary tumors of the testis, has been considerably facilitated over the past decade by IHC directed at developmentally important nuclear transcription factors, including OCT4, SALL4, SOX2, and SOX17, that are mostly restricted to certain tumor histotypes. In conjunction with other markers, a specific diagnosis can be achieved in most instances through a panel of 3 or 4 immunostains and often fewer. IHC among tumors in the sex cord-stromal group may produce a significant proportion of false-negative cases until more sensitive and equally specific markers are validated. The negativity of these tumors for the IHC stains used for germ cell tumors is key in the important distinction of neoplasms in these 2 general categories. In this review, the International Society of Urological Pathologists (ISUP) provides diagnostic guidelines in the form of algorithms to assist practicing pathologists confronting a differential diagnostic question concerning a testicular neoplasm. The goal of ISUP is to anticipate commonly encountered differential diagnoses and recommend an efficient and limited pattern of IHC stains to resolve the question.The American journal of surgical pathology 05/2014; 38(8). DOI:10.1097/PAS.0000000000000233 · 5.15 Impact Factor
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