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Background: Noninvasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) is considered an indolent neoplasm of the thyroid. Currently, this entity presents a diagnostic challenge on preoperative fine-needle aspiration (FNA) cytology.Methods: Cases of NIFTP and noninvasive encapsulated follicular variant of papillary thyroid carcinoma from January 1994 to August 2018 were retrieved from our institution's pathology databases, and their clinical and cytopathologic features were reviewed.

A 59-year-old hypertensive and diabetic man presented with bone pain, oliguria, and abdominal pain. Blood tests showed: hemoglobin, 8.7 g/dL; platelets, 69 3 10 9 /L; b-2 microglobulin, 54.23 nmol/L; serum IgA, 3450 mg/L; free l light chain, 8570 mg/L; serum calcium, 4.05 mmol/L; blood urea nitrogen, 24.28 mmol/L; and creatinine, 526.86 mmol/L. Imaging showed innumerable lytic lesions in the calvarium and a T7 vertebral body mass with extraosseous extension. Marrow biopsy showed sheets of abnormal plasma cells, with zones of pleomorphic, multinucleated cells alternating with more uniform malignant plasma cells (panels A-B; hematoxylin and eosin and Diff-Quik stains, respectively; original magnification 31000), both l-restricted by in situ hybridization. Fluorescence in situ hybridization showed del(17p13)(p53) (88% of cells), IGH/FGFR3 rearrangement (69% of cells), and del(13q14.3) (58% of cells) (panels C-E; original magnification 31000). Karyotyping revealed multiple abnormalities including t(1;19), chromosome 13 abnormalities, add(1)(q21), and near tetraploidy in 75% of the cells analyzed. The patient was treated with cyclophosphamide, bortezomib, and dexamethasone, and the follow-up free l light chain after 3 months was 147.7 mg/L. Anaplastic myeloma is a rare, treatment-resistant subtype with poor prognosis. It is more common in younger patients, associated with immunoglobulin A isotype, predisposition for the extramedullary sites, and with 1q21 amplification, 17p(p53) deletion, t(4;14), and/or chromosome 13 anomalies. Anaplastic morphology may present initially or as a feature of progression.For additional images, visit the ASH IMAGE BANK, a reference and teaching tool that is continually updated with new atlas and case study images. For more information visit http://imagebank.hematology.org.

BackgroundWaldenström macroglobulinemia (WM) is an incurable disease and, while treatable, can develop resistance to available therapies and be fatal. Chimeric antigen receptor (CAR) T cell therapy directed against the CD19 antigen has demonstrated efficacy in relapsed or refractory B lymphoid malignancies, and is now approved for B cell acute lymphoblastic leukemia and certain B cell lymphomas. However, CAR T therapy has not been evaluated for use in WM.Methods and resultsWe performed preclinical studies demonstrating CAR T cell activity against WM cells in vitro, and developed an in vivo murine model of WM which demonstrated prolonged survival with use of CAR T therapy. We then report the first three patients with multiply relapsed and refractory WM treated for their disease with CD19-directed CAR T cells on clinical trials. Treatment was well tolerated, and observed toxicities were consistent with those seen in CAR T treatment for other diseases, and no grade 3 or higher cytokine release syndrome or neurotoxicity events occurred. All three patients attained at least a clinical response to treatment, including one minimal residual disease-negative complete response, though all three eventually developed recurrent disease between 3 and 26 months after initial treatment.ConclusionsThis report summarizes preclinical and clinical activity of CD19-directed CAR T therapy in WM, demonstrating early tolerability and efficacy in patients with WM, and representing a possible treatment option in patients with heavily pretreated and relapsed or refractory WM. Larger studies evaluating CAR T therapy in WM are warranted, along with further evaluation into mechanisms of resistance to CAR T therapy.

The most common metastatic tumors to the umbilicus are from the adjacent organs with the gynecologic tract as the most frequent primary followed by the gastrointestinal system. Primary malignant tumors of the umbilical region are rarely identified in clinical practice.

: A morbidly-obese 57-year-old diabetic and hypertensive man with chronic kidney disease, diastolic heart failure, and bilateral hearing loss was found to have soft tissue masses/densities in the lateral aspect of both auditory canals on local examination and on imaging. He underwent biopsies of both ear canal masses and histologic examination revealed amyloid deposits in the dermis. These deposits were confirmed as AL (amyloid light chain) kappa-type amyloid by laser mass spectrometry. A systemic work-up showed plasma cell dyscrasia with 9% kappa light chain restricted plasma cells in the bone marrow as well as amyloid deposits on a kidney biopsy.Involvement of the external auditory canals is a rare manifestation of systemic amyloidosis with only 13 cases reported so far, of which four cases had bilateral external auditory canal involvement, associated with multiple myeloma. Interpretation of small biopsies can be challenging especially with early, scant amyloid deposits, but a sufficient biopsy to type amyloid by immunohistochemistry or laser mass spectrometry is imperative for further management. Early diagnosis and treatment of systemic light chain amyloidosis translates to better patient outcomes, while delay in management could lead to dismal prognosis.

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