German Campuzano-Zuluaga

A 60-year-old man with chronic renal failure and a costal mass: a case report and review of the literature

IntroductionBrown tumors are a rare focal manifestation of osteitis fibrosa cystica, which results from hyperparathyroidism. Chronic kidney failure may lead to secondary or tertiary hyperparathyroidism and thus to osteitis fibrosa cystica and brown tumors.Case presentationA 60-year-old man with a history of diabetes mellitus and chronic kidney failure presented with a 15-day history of dyspnea, cough, malaise and fever. Initially, there was little correlation between his history and his physical examination. Various pulmonary, cardiac and infectious etiologies were ruled out. A chest X-ray showed a costal mass that was further verified by tomography and gammagraphy. The mass was suspected of being neoplastic. After a failed biopsy, the mass was removed surgically and on histopathology was compatible with a giant-cell tumor versus a brown tumor caused by hyperparathyroidism. Laboratory tests showed elevated calcium, phosphate and parathyroid hormone concentrations. The patient was diagnosed with a brown tumor secondary to refractory hyperparathyroidism.ConclusionTending towards a diagnosis because it is more frequent or it implies more risk for the patient may delay the consideration of other diagnostic options that, although rare, fit well into the clinical context. The patient presented here was suspected to have an osseous neoplasia that would have had major implications for the patient. However, reassessment of the case led to the diagnosis of a brown tumor. Brown tumors should be an important diagnostic consideration in patients with chronic kidney failure who have secondary or tertiary hyperparathyroidism and an osseous mass.

Epstein-Barr virus-positive follicular lymphoma

Epstein-Barr virus (EBV) -associated follicular lymphoma is only rarely reported. Herein, we report the largest series analyzing prevalence and clinicopathologic characteristics of EBV-associated follicular lymphoma occurring in unselected cases. Out of 382 analyzed cases, 10 EBV-positive follicular lymphomas were identified (prevalence=2.6%, 95% confidence interval 1.3–4.0%). All EBV-positive follicular lymphomas showed EBV-encoded small RNA-positive lymphoma cells present in a follicular distribution. Of these, eight also had tissue available for testing of expression of latent membrane protein 1 (LMP1), out of which six (75%) were positive. There was a significant association with grades 3A-3B follicular lymphoma (P<0.0001) and CD30 expression (P=0.0002). EBV-positive follicular lymphomas were otherwise morphologically and immunophenotypically indistinguishable from EBV-negative cases of similar grade. Nine of the EBV-positive follicular lymphomas occurred in patients with no known history of immunosuppression, while one patient had a history of hydroxychloroquine administration for Sjögrens syndrome. The mean age in the EBV-positive and -negative follicular lymphomas was 56 (range 31–83 years) and 49 years (range 25–92 years), respectively, with no statistically significant difference. Seven of the patients with EBV-positive follicular lymphoma had additional biopsies from different time points available for review, all of which showed progression of disease in the form of progression of tumor grade. Five of these progressed to diffuse large B-cell lymphoma, one of which had tissue available for testing and was EBV-positive. Our findings suggest that EBV infection may have a role in lymphomagenesis and/or disease progression in a subset of follicular lymphomas, thereby expanding the spectrum of recognized EBV-associated B-cell lymphomas.

Differential CD30 expression in adult T-cell leukemia-lymphoma subtypes

Adult T-cell leukemia-lymphoma (ATLL) is caused by HTLV-I, and is a disease with dismal prognosis urging new therapies. Brentuximab vedotin (SGN-35) is an anti-CD30 monoclonal antibody conjugated to a potent microtubule poisoning agent monomethyl auristatin E that is effective in the treatment of CD30-expressing lymphomas. There are conflicting reports on the frequency of ATLL tumors expressing CD30. At our institution we encounter a relatively large number of ATLL cases due to our patient demographics. Thirty-nine out of 156 ATLL cases identified so far have been checked for CD30 status. CD30 expression was evaluated by immunohistochemistry (IHC) performed on either tissue sections as part of the routine pathology workup or cytospins prepared from CD4+ lymphocyte-enriched peripheral blood specimens using 20% expression as a cut-off positive value. The proportion of CD30+ ATLLs was 36% (95% CI 11%-61%), including 47% in lymphomatous-type, 28% in acute-type, and 10% in indeterminate cases. Four of 12 (33%) acute-type ATLL cytospin cases were CD30+, however, a high expression of 80% was observed in only one case. One patient with CD30+ acute-type ATLL with diffuse skin involvement treated with brentuximab had an objective transient response. Our preliminary data show that 36% of ATLLs are CD30+ and therefore may be amenable to anti-CD30 therapy. However, the low percentage of CD30+ cells in acute-type ATLL may limit the response to anti-CD30 target therapies for this subtype. We are currently testing a larger number of tissue and leukemic ATLL specimens available for further immunophenotypic characterization and comprehensive analysis of clinical outcome.

Adult T-cell leukemia/lymphoma can be indistinguishable from other more common T-cell lymphomas. The University of Miami experience with a large cohort of cases

Adult T-cell leukemia/lymphoma, an aggressive T-cell neoplasm, is causally linked to human T-cell lymphotropic virus type 1 and based on this association has a distinct geographic distribution. In our United States-based practice, whose population is enriched for immigrants from human T-cell lymphotropic virus type 1 endemic areas, we have identified that a subset of adult T-cell leukemia/lymphoma, in the absence of human T-cell lymphotropic virus type 1 identification, are indistinguishable from other more common T-cell neoplasms. We retrospectively gathered serology results for anti-human T-cell lymphotropic virus type 1/2 antibody in patients diagnosed with T-cell neoplasms at our institution. A total of 220 human T-cell lymphotropic virus type 1/2 positive patients with T-cell neoplasms were identified; 199 (91%) were correctly classified as adult T-cell leukemia/lymphoma or provisionally as peripheral T-cell lymphoma (serology testing pending). Twenty-one cases (9%) were initially misclassified, including the following: 13 presenting with skin +/− peripheral blood involvement and misclassified as mycosis fungoides/Sezary syndrome; 7 with lymphomatous disease, absence of leukemic involvement, and diffuse CD30 expression, misclassified as ALK- negative anaplastic large-cell lymphoma; 1 thought to represent T-prolymphocytic leukemia with TCL-1 gene rearrangement and diffuse marrow involvement. We also present an example of adult T-cell leukemia/lymphoma, which mimicked lymphoepithelioid variant of peripheral T-cell lymphoma also with diffuse marrow involvement. A subset of adult T-cell leukemia/lymphoma can closely mimic a variety of other more common T-cell neoplasms. Due to its extreme clinicopathologic heterogeneity, identification of adult T-cell leukemia/lymphoma requires a high level of suspicion based on patient demographic alone, which should prompt anti-human T-cell lymphotropic virus type 1/2 serology testing in all T-cell neoplasms developing in patients of appropriate demographic. Absence of high level of suspicion, adult T-cell leukemia/lymphoma is easily misclassified.

Laryngeal Involvement of Multiple Myeloma

The objectives of this paper are to discuss a rare cause of laryngeal multiple myeloma, to review unique pathologic findings associated with plasma cell neoplasms, to discuss epidemiology, differential diagnosis, and treatment options for plasma cell neoplasms of the larynx. Laryngeal multiple myeloma, also noted in the literature as “metastatic” multiple myeloma, presenting as a de novo laryngeal mass is extremely rare with few reported cases. Laryngeal involvement of extramedullary tumors is reported to be between 6% and 18% with the epiglottis, glottis, false vocal folds, aryepiglottic folds, and subglottis involved in decreasing the order of frequency. We present the case of a 58-year-old male with a history of IgA smoldering myeloma who presented to a tertiary care laryngological practice with a two-month history of dysphonia, which was found to be laryngeal involvement of multiple myeloma. We review the classification of and differentiation between different plasma cell neoplasms, disease workups, pathologic findings, and treatment options.

Frozen section evaluation via dynamic real-time nonrobotic telepathology system in a university cancer center by resident/faculty cooperation team

Frozen section telepathology interpretation experience has been largely limited to practices with locations significantly distant from one another with sporadic need for frozen section diagnosis. In 2010, we established a real-time nonrobotic telepathology system in a very active cancer center for daily frozen section service. Herein, we evaluate its accuracy compared to direct microscopic interpretation performed in the main hospital by the same faculty and its cost-efficiency over a 1-year period. From 643 (1,416 parts) cases requiring intraoperative consultation, 333 cases (690 parts) were examined by telepathology and 310 cases (726 parts) by direct microscopy. Corresponding discrepancy rates were 2.6% (18 cases: 6 [0.9%] sampling and 12 [1.7%] diagnostic errors) and 3.2% (23 cases: 8 [1.1%] sampling and 15 [2.1%] diagnostic errors), P = .63. The sensitivity and specificity of intraoperative frozen diagnosis were 0.92 and 0.99, respectively, in telepathology and 0.90 and 0.99, respectively, in direct microscopy. There was no correlation of error incidence with postgraduate year level of residents involved in the telepathology service. Cost analysis indicated that the time saved by telepathology was

Anaphylactic reaction to platelet transfusion as the initial symptom of an undiagnosed systemic mastocytosis: a case report and review of the literature

19,691.00 over 1 year of the study period, whereas the capital cost for establishing the system was

A Limited Immunohistochemical Panel to Distinguish Basal Cell Carcinoma of Cutaneous Origin From Basaloid Squamous Cell Carcinoma of the Head and Neck.

8,924.00. Thus, real-time nonrobotic telepathology is a reliable and easy-to-use tool for frozen section evaluation in busy clinical settings, especially when frozen section service involves more than one hospital, and it is cost-efficient when travel is a component of the service.

Abstract LB-160: HDAC9 expression is deregulated in malignant B-cell lymphomas in particular in diffuse large B-cell lymphoma and mantle cell lymphoma

IntroductionThe association between anaphylactic reactions and systemic mastocytosis is well documented. However, platelet transfusion has not previously been reported as a potential elicitor of anaphylaxis in the context of systemic mastocytosis.Case presentationWe describe the clinicopathological findings of a 59-year-old Latin American man who presented to the emergency room with fatigue, leukocytosis, thrombocytopenia and mild hepatosplenomegaly. He developed two separate, temporally associated and severe anaphylactic reactions after receiving platelet transfusions. The result of a laboratory investigation for clerical errors and Coombs test was negative. Pre- and post-transfusion urine samples were negative for hemolysis. Bone marrow biopsy and aspirate smears performed demonstrated involvement by systemic mastocytosis, which had been previously undiagnosed.ConclusionsWe posit the transfusion reaction to be an anaphylactic reaction to transfused products as a result of heightened allergic sensitivity due to the underlying systemic mastocytosis. To the best of our knowledge, this is the first reported case of a severe anaphylactic-type reaction to blood products occurring in the setting of a previously undiagnosed systemic mastocytosis. Furthermore, it seems there are no published studies closely examining the relationship between hematopoietic neoplasms and transfusion reactions in general.

Clinical Utility of Morphological Evaluation of Day 14 Bone Marrow Biopsies in Acute Myeloid Leukemia Patients Undergoing Standard Induction Chemotherapy: Time to Change Practice?

Head and neck carcinomas with basaloid features can be diagnostically challenging. A common diagnostic issue is the distinction between a basaloid squamous cell carcinoma (bSCC) and a basal cell carcinoma (BCC) of cutaneous origin. This is particularly true in small biopsy specimens where classic architectural and histologic features may be difficult to appreciate. A specific diagnosis is essential because of significant differences in clinical outcome and therapeutic management. Ten resection cases of bSCC and BCC of the head and neck were selected based on primary location and the classic morphologic features that characterize these 2 entities. The following immunohistochemical markers were evaluated: epithelial membrane antigen (EMA), Ber-EP4, CD44, Bcl2, androgen receptor, SOX2, and p16. The strongest statistically significant differences in staining patterns were for EMA, p16, and SOX2. EMA was positive in all bSCCs and negative in all BCCs. SOX2 was positive in all bSCCs and in only 3 out of 10 BCCs. Staining was weak and peripheral in the SOX2-positive BCCs. p16 was positive in 8 out of 10 bSCCs and negative in all BCCs. We conclude that bSCC and BCC of the head and neck can be readily distinguished by a limited panel consisting primarily of EMA, and supported by SOX2 and p16.