Extraordinary Case ReportALK-Negative Primary Cutaneous Anaplastic Large Cell Lymphoma With Systemic Involvement or Systemic ALCL With Cutaneous Lesion. A Diagnostic DilemmaGupta, Ishan*; Ramteke, Prashant MD*; Narwal, Anubhav MD*; Sharma, Mehar Chand MD*; Bakhshi, Sameer MD, DM†; Mallick, Saumyaranjan MD* Author Information Departments of *Pathology, and †Medical Oncology, Dr B. R. A. Institute Rotary Cancer Hospital (Dr. B. R. A. IRCH), All India Institute of Medical Sciences (AIIMS), New Delhi, India. Correspondence: Saumyaranjan Mallick, MD, Department of Pathology, All India Institute of Medical Sciences (AIIMS), Ansari Nagar East, New Delhi 11029, India (e-mail: [email protected]). Each author has contributed significantly to the work to take public responsibility for appropriate portions of the content; and have agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. S. Mallick: conception and design. I. Guptaishan and A. Narwal: acquisition of data, final approval. I. Guptaishan, and P. Ramteke: drafting the manuscript. M. C. Sharma: revising the manuscript. S. Bakhshi: Medical Oncologist, treatment of the patient and work up. The authors declare no conflicts of interest. The case submitted to European School of Hematology Lymphoid malignancy conference. The American Journal of Dermatopathology: May 2022 - Volume 44 - Issue 5 - p 376-379 doi: 10.1097/DAD.0000000000002168 Buy Metrics Abstract Primary cutaneous anaplastic large-cell lymphoma (C-ALCL) is a cutaneous CD30-positive lymphoproliferative disorder. The patients usually present with single or multiple cutaneous nodules or papules and about 10% cases present with extracutaneous manifestations, which are predominantly in the form of regional lymph nodal involvement. Visceral involvement especially pulmonary or hepatic involvement in C-ALCL is only rarely described in the scientific literature. Approximately 20%–42% cases show spontaneous regression, about 50% cases may recur; however, C-ALCL generally carries a good prognosis. We present a rare case of primary C-ALCL in a 66-year-old man with regional lymph nodal and hepatic involvement. Differential diagnostic entities are discussed in this report with the review of the literature. Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.