Cutaneous basal cell carcinosarcoma: case report and literature review.

Carcinosarcomas are malignant neoplasms with biphasic carcinomatous and sarcomatous or sarcoma-like components. In general, the sarcomatous component is accepted to be the result of divergent mesenchymal differentiation of the epithelial component. Although well characterized in some anatomic locations (e.g. uterus, upper aerodigestive tract and lung), carcinosarcomas of the skin are rare. Reported epithelial components include squamous, adnexal, neuroendocrine and basaloid. Including this case, only 47 cases of primary cutaneous basal cell carcinosarcoma have been reported in the literature to date. We performed an extensive immunophenotypic evaluation in our case, which confirmed the previously reported coexpression of p53 in both components, and revealed a hitherto unreported coexpression of p16 and p63. Additionally, this report reviews the clinical, pathologic, immunophenotypic characteristics and outcomes of the basal cell carcinosarcomas reported in the literature, in order to emphasize the overall uniform characteristics and clinical behavior of this neoplasm.

Listeria rhomboencephalitis in a patient on a tumor necrosis factor α inhibitor (etanercept).

We describe a case of an 87-year-old woman with rheumatoid arthritis on etanercept who developed subacute encephalopathy. Magnetic resonance imaging and blood cultures led to the diagnosis of Listeria monocytogenes rhomboencephalitis, which proved to be fatal. Postmortem examination of the brain revealed abscesses with extensive necrosis. Gram stain showed small gram-positive rods in the necrotic tissue and the wall lining the abscesses. While rhomboencephalitis is a rare entity, and clinical recognition may be hampered by immunosuppression such as in this case, early blood cultures, lumbar puncture, and empiric treatment with ampicillin should be initiated in all patients with suspected rhomboencephalitis if suggested by imaging and by a clinical concern for infection.

Case Report: Flavoring-Related Lung Disease in a Coffee Roasting and Packaging Facility Worker With Unique Lung Histopathology Compared With Previously Described Cases of Obliterative Bronchiolitis.

Occupational exposure to diacetyl, a butter flavor chemical, can result in obliterative bronchiolitis. Obliterative bronchiolitis is characterized by exertional dyspnea, fixed airflow obstruction, and histopathologic constrictive bronchiolitis, with bronchiolar wall fibrosis leading to luminal narrowing and obliteration. We describe a case of advanced lung disease with histopathology distinct from obliterative bronchiolitis in a 37-year-old male coffee worker following prolonged exposure to high levels of diacetyl and the related compound 2,3-pentanedione, who had no other medical, avocational, or occupational history that could account for his illness. He began working at a coffee facility in the flavoring room and grinding area in 2009. Four years later he moved to the packaging area but continued to flavor and grind coffee at least 1 full day per week. He reported chest tightness and mucous membrane irritation when working in the flavoring room and grinding area in 2010. Beginning in 2014, he developed dyspnea, intermittent cough, and a reduced sense of smell without a work-related pattern. In 2016, spirometry revealed a moderate mixed pattern that did not improve with bronchodilator. Thoracoscopic lung biopsy results demonstrated focal mild cellular bronchiolitis and pleuritis, and focal peribronchiolar giant cells/granulomas, but no evidence of constrictive bronchiolitis. Full-shift personal air-samples collected in the flavoring and grinding areas during 2016 measured diacetyl concentrations up to 84-fold higher than the recommended exposure limit. Medical evaluations indicate this worker developed work-related, airway-centric lung disease, most likely attributable to inhalational exposure to flavorings, with biopsy findings not usual for obliterative bronchiolitis. Clinicians should be aware that lung pathology could vary considerably in workers with suspected flavoring-related lung disease.

Diagnosis of Lymphoid Lesions in Limited Samples: A Guide for the General Surgical Pathologist, Cytopathologist, and Cytotechnologist.

OBJECTIVES: Advances in interventional techniques allow obtaining samples from most body sites through minimally invasive procedures that yield limited samples. We provide practical guidelines for diagnosis of lymphoid lesions in these samples. METHODS: Guidelines for selection of biopsy site and triage of the specimen according to results of rapid on-site evaluation (ROSE), and description of the advantages and limitations of currently available ancillary studies are described, based on the experience of the authors, complemented by a comprehensive review of the literature. RESULTS: Five diagnostic categories are observed at ROSE: (1) preponderance of small cells, (2) large cells, (3) mixed small and large cells, (4) blast-like cells, and (5) rare large pleomorphic cells. Detailed description of the diagnostic work-up and subsequent classification for each of these groups is provided. CONCLUSIONS: A definitive diagnosis of lymphoid neoplasms in limited samples is possible in most cases through correlation of morphology, ancillary studies, and clinical scenario.

Thrombocytosis in myelodysplastic and myelodysplastic/myeloproliferative syndromes.

Thrombocytosis at diagnosis is uncommon in myelodysplastic (MDS) and myelodysplastic/myeloproliferative (MDS/MPD) syndromes. We conducted a retrospective analysis to determine the clinical and haematopathological features of such patients, and the effect of thrombocytosis on prognosis. Of the 388 patients diagnosed with MDS from 1980 - 2006, 31 presented with thrombocytosis. The majority (71%) had low risk features and a low incidence of spontaneous bleeding or thrombo-embolic events. Compared to a case-matched control group of MDS and MDS/MPD patients without thrombocytosis of similar ages and IPSS scores, patients with thrombocytosis had a slightly lower probability of progression to a higher grade of MDS (P = 0.03), equivalent risk of transformation to acute myeloid leukemia (AML), and a trend (P = 0.07) towards longer overall survival (median 35.4 months compared to 27.6 months for controls).

Cerebrospinal fluid cytology in seasonal epidemic West Nile virus meningo-encephalitis.

The incidence of West Nile Virus (WNV) infection has progressively increased in North America since the first epidemic in 1999. Formal scholarly documentation of cerebrospinal fluid (CSF) cytology changes in patients with WNV infection is limited. We report our experience with CSF cytospins from a population of consecutive patients with documented CSF WNV-specific IgM. Thirty-two patients (12 male, 20 female) with a median age of 52 yr (range, 19-88) diagnosed with WNV meningo-encephalitis were studied. Symptoms were present for a mean of 5 days (range, 1-14) prior to lumbar puncture. CSF proteins were elevated in 94% of patients (30/32) with a mean value of 79 mg/dl (range, 36-185). CSF glucose was normal to elevated in all cases. All cytomorphologically adequate samples demonstrated a pleocytosis with a mean of 156 cells/mm3 (range, 13-683). Nearly, all (26/28) patients showed increased CSF neutrophils--mean 43% (range, 1-83). Mean lymphocyte and monocyte fractions were 44% (range, 8-85) and 14% (range, 2-27), respectively. Three cases showed 1-4% plasma cells. Mean total leukocyte counts (TLC) (197 cells/mm3) and mean neutrophil fractions (50%) were greater in patients sampled within the first 3 days of symptoms than in those sampled beyond day 3 (mean TLC, 126 cells/mm3; mean neutrophil fraction, 37%). Relative lymphocyte proportions increased from a mean of 39 to 48% after 3 days of illness. WNV should be considered as a potential etiology of infectious CSF pleocytosis in the North American late summer and early fall seasons.

Automated reticulocyte response is a good predictor of bone-marrow recovery in pediatric malignancies.

The authors attempted to establish the benefits of flow cytometry-based reticulocyte analysis over absolute neutrophil count (ANC) recovery. Serial hemograms of 18 pediatric cases of hematologic malignancies were analyzed until day 35 of chemotherapy. Immature reticulocyte fraction (IRF) showed early recovery in 44.4% of cases compared to ANC. Since reticulocyte fractions are not influenced by infections, they are a better parameter of bone marrow regeneration than ANC. The study shows that IRF can act as a harbinger of regenerating bone marrow activity in patients with persistent neutropenia and guide the modulation of antibiotic strategies in these patients.

Expression and phosphorylation of eukaryotic translation initiation factor 4E binding protein 1 in B-cell lymphomas and reactive lymphoid tissues.

CONTEXT: Cap-mediated messenger RNA translation controlled by the eukaryotic initiation factor 4F (eIF-4F) complex plays a key role in human cancer. eIF-4F activity is controlled by a repressor binding protein (4E-BP1), which promotes translation when phosphorylated. OBJECTIVE: To examine the level of expression and phosphorylation of 4E-BP1 in various subtypes of B-cell lymphoma and reactive lymphoid tissues. DESIGN: Archival formalin-fixed, paraffin-embedded B-cell lymphoma samples and reactive lymphoid tissues were immunostained and examined for expression of 4E-BP1 and phosphorylated 4E-BP1. Expression of components of the eIF-4F complex and unphosphorylated and phosphorylated 4E-BP1 was confirmed using Western immunoblotting on lysates of frozen lymphoma samples and reactive tissues. RESULTS: Immunohistochemical analysis demonstrated weak to undetectable 4E-BP1 staining within benign, reactive germinal centers (N = 10). In contrast, 4E-BP1 was consistently expressed (moderate to strong staining) in 98% of various subtypes of mature B-cell lymphoma (N = 50). 4E-BP1 expression was also demonstrable in all 4 lymph nodes with in situ or partial involvement by follicular lymphoma and in all 12 cases of BCL2-negative lymphoma. The level of phosphorylation of 4E-BP1 in lymphomas, evaluated by immunohistochemistry, was heterogeneous. CONCLUSIONS: The immunohistochemical expression pattern of 4E-BP1 exhibits regional and cellular specificity in reactive lymphoid tissues and may offer a diagnostic tool for distinguishing reactive follicles from neoplastic B-cell proliferations.

"Burned out" testicular seminoma presenting as a primary gastric malignancy.

In contrast to primary gastric adenocarcinomas, germ cell tumors are potentially curable even when metastatic. It is therefore essential for clinicians and pathologists to be aware of the spectrum of unusual manifestations of germ cell malignancies. Here we report on a 55-year-old man who presented with clinical and endoscopic features indicative of a primary gastric carcinoma. Surprisingly, the ulcerative mucosal lesion was found to be due to a metastasis from an occult, "burned-out" testicular seminoma. This case describes the radiological and pathological features that helped differentiate this rare situation from the much more common gastric adenocarcinoma, and extends the diagnostic possibilities that must be considered in patients presenting with gastric ulcers.

Site-specific morphologic differences in extranodal marginal zone B-cell lymphomas.

CONTEXT: Lymphoepithelial lesions (LELs) are a useful diagnostic feature of extranodal marginal zone B-cell lymphoma (EMZL); however, there is scant literature comparing their frequency and morphology at various sites. OBJECTIVE: To evaluate any diagnostically useful, site-specific, morphologic patterns in EMZLs. DESIGN: In this retrospective review, we evaluated 136 EMZLs from different sites for LEL pattern and other pathologic differences, including CD43 coexpression and plasma cell component features. RESULTS: Prominent and destructive LELs were most common in salivary and thyroid gland cases, and LELs were rare to absent in breast, skin, and ocular adnexa cases. An LEL pattern with lymphocytes "stuffing" glandular lumina was seen in lung, thyroid, and salivary gland cases. Monoclonal plasma cells were most common in breast, upper aerodigestive tract, skin, and salivary gland cases. CD43 coexpression was seen in 36% of cases, most commonly in salivary gland, stomach, and upper aerodigestive tract. CONCLUSIONS: The relative importance of LEL pattern, CD43 coexpression, and clonal plasma cell component in EMZLs is site-dependent, and the differences may aid in the diagnosis of EMZLs at different anatomic sites.

Poor correlation of supratherapeutic international normalised ratio and vitamin K-dependent procoagulant factor levels during warfarin therapy.

Patients with a supratherapeutic international normalised ratio (ST-INR) are at risk for bleeding. ST-INR is corrected by withholding warfarin therapy and often by supplementing vitamin K or providing vitamin K-dependent factors; the exact therapeutic decision is based on the extent of the prolonged INR. Currently, ST-INRs are frequently observed in clinical practice due to the use of sensitive recombinant tissue thromboplastin reagents and automation. However, there are scant data correlating an ST-INR with various vitamin K-dependent factors. This prospective cohort study, set in a large tertiary care teaching hospital for the University of Texas Southwestern Medical Center at Dallas, defined the relationship between ST-INR (>5.0) and measured vitamin K-dependent procoagulant factors. Prothrombin time, INR and vitamin K-dependent factors II, VII, IX and X were measured in 78 patients with an INR > 5.0 (ST-INR) who were on warfarin therapy for more than 2 months. There was no significant relationship between the ST-INR and levels of important vitamin K-dependent factors II and X. These data support the recent guidelines that the management of an INR > 5.0 should be driven by the clinical determinants rather than specific INR values per se.

Testicular extramedullary myeloid cell tumor: report of a case with unique clinicopathologic features and a brief review of the literature.

We report a case of testicular extramedullary myeloid cell tumor in a 37-year-old man who presented with an acute testicular hemorrhage. A pathologic examination revealed no gross tumor mass. A well-differentiated extramedullary myeloid cell tumor infiltrate was seen histologically, localized largely to the seminiferous tubules. The patient had no evidence of any past or concurrent myeloid disorders. The lack of the usual clinical features of a testicular mass and the presence of an intratubular pattern of infiltration can further compound the challenges in diagnosing this entity.

Acquired Glanzmann's thrombasthenia as part of multiple-autoantibody syndrome in a pediatric heart transplant patient.

Although advances in immunosuppressive therapy have allowed prolonged patient survival, immune dysregulation observed in these patients has increased. We report an 11-year-old female heart transplant recipient in whom Glanzmann thrombasthenia was identified as part of a "multiple autoantibody syndrome" manifesting sequentially as autoimmune hemolytic anemia, thrombocytopenia, and neutropenia.

Isolated esthesioneuroblastoma of sphenoid sinus.

Esthesioneuroblastoma is a rare neuroendocrine tumor that arises from the olfactory epithelium and accounts for approximately 3% of all intranasal tumors. The tumor involvement in the nasal cavity with extension to paranasal sinuses, orbit, and anterior cranial fossa, is reported in the literature. In this report, we present an interesting case of isolated sphenoid sinus esthesioneuroblastoma, which is the first case to be reported in the literature, and discuss the pathology, clinical manifestations, and various treatment options for this tumor.