Concurrent Liver and Mesentery Primary Extraosseous Multiple Myeloma on 18F-FDG PET/CT.

ABSTRACT: Extramedullary multiple myeloma (EMM) is a subset of multiple myeloma with a poor prognosis. We report a rare case with biopsy-proven concurrent liver and mesentery primary EMM at the time of initial staging after serologic diagnosis of multiple myeloma. 18F-FDG PET/CT is valuable in detection of EMM when the patient has no osseous lesions and a negative bone marrow biopsy.

Histopathologic Evaluation of Flexor Tenosynovium in Recurrent Carpal Tunnel Syndrome.

BACKGROUND: The authors' purpose was to evaluate the histopathology of flexor tenosynovium in true, idiopathic recurrent carpal tunnel syndrome for the presence of abnormal inflammatory or pathologic findings that might explain causation or that differ from those previously described for primary, idiopathic carpal tunnel syndrome. METHODS: Thirty-five patients (19 women and 16 men; mean age, 72 years) underwent open revision carpal tunnel release a mean 13 years (range, 0.5 to 30 years) after primary carpal tunnel release. Recurrence was confirmed by recurrent symptoms, positive provocative tests, and electrodiagnostic testing. All patients underwent tenosynovial biopsy, including Congo red staining for amyloid. RESULTS: Histopathologic findings demonstrated noninflammatory, fibrous connective tissue in 31 of 35 patients (89 percent); and mild, chronic inflammation (without granulomas) in four of 35 patients (11 percent). Nine of 35 patients (26 percent) had positive results for amyloid, with a statistically higher incidence in men (p = 0.03) and advanced age (p = 0.02). Subtyping performed in eight of nine amyloid-positive specimens confirmed seven cases of transthyretin-type amyloid typically seen in localized (senile) amyloidosis and one case of light-chain amyloid in a patient who was subsequently diagnosed with myeloma. CONCLUSIONS: Flexor tenosynovium in patients with recurrent carpal tunnel syndrome does not appear to be substantially different histologically from that previously described in primary idiopathic carpal tunnel syndrome, although a slightly higher prevalence of amyloid was seen in this group (especially older men). No patients have developed systemic amyloidosis. Routine biopsy of tenosynovium in idiopathic, recurrent carpal tunnel syndrome is unnecessary.

A phase 2 study of rituximab, cyclophosphamide, bortezomib and dexamethasone (R-CyBorD) in relapsed low grade and mantle cell lymphoma.

In this phase 2 trial, we sought to evaluate the efficacy and safety of rituximab, cyclophosphamide, bortezomib, and dexamethasone (R-CyBorD) in patients with low-grade NHL. The regimen included rituximab on day 1 with weekly cyclophosphamide, dexamethasone, and bortezomib 1.3 mg/m2 IV in a 28-day cycle. Twenty one patients were enrolled on the study. Median age was 69 years (range 51-80) and 17 (81%) patients had two or more prior treatments. Histologies included FL (n = 8), MCL (n = 8), and LPL/WM (n = 5). Hematologic toxicity and peripheral sensory neuropathy were the most common adverse events. With a median follow-up of 38.1 months, ORR was 13/21 (62%), with 4 (19%) CR. The ORR was 7/8 (88%) in FL and was 4/5 (80%) in LPL/WM. Median PFS and OS were 11.6 months and 54.8 months, respectively. R-CyBorD is an effective regimen in relapsed FL and LPL/WM patients with an acceptable safety profile.

An Organization Model to Assist Individual Physicians, Scientists, and Senior Health Care Administrators With Personal and Professional Needs.

Working as a physician, scientist, or senior health care administrator is a demanding career. Studies have demonstrated that burnout and other forms of distress are common among individuals in these professions, with potentially substantive personal and professional consequences. In addition to system-level interventions to promote well-being globally, health care organizations must provide robust support systems to assist individuals in distress. Here, we describe the 15-year experience of the Mayo Clinic Office of Staff Services (OSS) providing peer support to physicians, scientists, and senior administrators at one center. Resources for financial planning (retirement, tax services, college savings for children) and peer support to assist those experiencing distress are intentionally combined in the OSS to normalize the use of the Office and reduce the stigma associated with accessing peer support. The Office is heavily used, with approximately 75% of physicians, scientists, and senior administrators accessing the financial counseling and 5% to 7% accessing the peer support resources annually. Several critical structural characteristics of the OSS are specifically designed to minimize potential stigma and reduce barriers to seeking help. These aspects are described here with the hope that they may be informative to other medical practices considering how to create low-barrier access to help individuals deal with personal and professional challenges. We also detail the results of a recent pilot study designed to extend the activity of the OSS beyond the reactive provision of peer support to those seeking help by including regular, proactive check-ups for staff covering a range of topics intended to promote personal and professional well-being.

An Unusual Suspect: Lymphoepithelial Cyst of the Pancreas.

Lymphoepithelial cysts (LECs) of the pancreas are benign, rare pancreatic cysts that are found predominantly in men. These cysts can present as a diagnostic conundrum given their rarity and difficulty of distinguishing these cysts from those with malignant potential. We present an incidental case of a LEC in a middle-aged man.

Secondary Philadelphia chromosome and erythrophagocytosis in a relapsed acute myeloid leukemia after hematopoietic cell transplantation.

The acquisition of the Philadelphia chromosome (Ph) as a secondary change during the course of hematopoietic malignancies is rare and is associated with poor prognosis. Few cases of secondary Ph have been reported after hematopoietic cell transplantation (HCT). A secondary Ph at relapse is of clinical importance because it provides a therapeutic target for tyrosine kinase inhibitors along with or in replacement of chemotherapy. We describe a case of relapsed acute myeloid leukemia (AML) after HCT that developed a BCR-ABL1 translocation along with erythrophagocytosis by blasts as a secondary change at the time of relapse. The progression of this patient's myeloid neoplasm from myelodysplastic syndrome to AML to relapsed AML after HCT was accompanied by a stepwise cytogenetic evolution: A deletion 20q abnormality subsequently acquired a deletion 7q and, finally, at relapse after HCT, a secondary Ph was gained. The relationship between the secondary Ph and the erythrophagocytosis by blasts is not clear. We review the possible pathogenesis and cytogenetic associations of erythrophagocytosis by blasts, a rare feature in acute leukemias.

Update on intravenous leiomyomatosis: report of five patients and literature review.

The objective of this study was to review management and results of surgical therapy of intravenous leiomyomatosis (IVL). A retrospective review of five patients treated at the Mayo Clinic between 2002 and 2012 and a literature review from 1970 to the present were performed. IVL is a rare condition, often overlooked, misdiagnosed or inadequately treated. Despite its benign histological features, invasion of large vessels and cardiac extension can occur and be fatal. Appropriate diagnosis and a radical surgical approach to IVL provide optimal outcomes. Incomplete resection and/or microscopic foci of IVL can lead to recurrence. Surgery should always aim for complete tumor excision and include hysterectomy and bilateral salpingoophorectomy. Radical parametrectomy and intravenous tumor resection may be necessary.

Bladder wall fat on computed tomography with pathologic correlation.

OBJECTIVES: To determine the prevalence and significance of fat in the urinary bladder wall detectable by computed tomography (CT). METHODS: Retrospective review of 200 consecutive patients with noncontrast CT. Cystectomy specimen CT/pathologic correlation. RESULTS: CT-detectable fat in 30 (15%) patients was significantly more predominant in men (P=.003) and in patients with past renal stones (P<.001). Urinary tract infections were significantly less common in patients with bladder wall fat (P=.05). Pathologically, CT-detectable fat was submucosal. CONCLUSION: CT-detectable bladder wall fat is more common in men than women and in patients with a history of renal stones and urinary tract infections.

A novel role for cyclooxygenase-2 in regulating vascular channel formation by human breast cancer cells.

INTRODUCTION: Cyclo-oxygenase (COX)-2 expression correlates directly with highly aggressive and metastatic breast cancer, but the mechanism underlying this correlation remains obscure. We hypothesized that invasive human breast cancer cells that over-express COX-2 have the unique ability to differentiate into extracellular-matrix-rich vascular channels, also known as vasculogenic mimicry. Vascular channels have been associated with angiogenesis without involvement of endothelial cells, and may serve as another mechanism by which tumor cells obtain nutrients to survive, especially in less vascularized regions of the tumor. METHODS: To determine whether COX-2 regulates vascular channel formation, we assessed whether treatment with celecoxib (a selective COX-2 inhibitor) or silencing COX-2 synthesis by siRNA inhibits vascular channel formation by breast cancer cell lines. Cell lines were selected based on their invasive potential and COX-2 expression. Additionally, gene expression analysis was performed to identify candidate genes involved in COX-2-induced vascular channel formation. Finally, vascular channels were analyzed in surgically resected human breast cancer specimens that expressed varying levels of COX-2. RESULTS: We found that invasive human breast cancer cells that over-express COX-2 develop vascular channels when plated on three-dimensional matigel cultures, whereas non-invasive cell lines that express low levels of COX-2 did not develop such channels. Similarly, we identified vascular channels in high-grade invasive ductal carcinoma of the breast over-expressing COX-2, but not in low-grade breast tumors. Vascular channel formation was significantly suppressed when cells were treated with celecoxib or COX-2 siRNA. Inhibition of channel formation was abrogated by addition of exogenous prostaglandin E2. In vitro results were corroborated in vivo in tumor-bearing mice treated with celecoxib. Using gene expression profiling, we identified several genes in the angiogenic and survival pathways that are engaged in vascular channel formation. CONCLUSION: Antivascular therapies targeting tumor cell vasculogenic mimicry may be an effective approach to the treatment of patients with highly metastatic breast cancer.

Sentinel lymph node metastases detected by immunohistochemistry only do not mandate complete axillary lymph node dissection in breast cancer.

BACKGROUND: The significance of breast cancer sentinel lymph node (SLN) metastases detected only by immunohistochemistry staining (IHC) remains poorly understood. This study attempted to quantify the risk of non-SLN metastases. METHODS: A prospectively collected database of 750 consecutive SLN biopsy procedures in breast cancer patients was reviewed. Medical records were reviewed to supplement the database. RESULTS: SLNs were identified in 738 (98.4%) of these procedures in 723 patients. Of these, 151 patients (20.5%) had metastases detected by hematoxylin and eosin staining (H&E), and 33 (4.6%) of the 718 with known IHC staining results had metastases detected by IHC only. Twenty-eight (84.8%) of 33 patients with IHC-detected metastases underwent complete axillary lymph node dissection (CALND). The median primary tumor size was 2.0 cm among those undergoing CALND and 0.9 cm among the five patients treated without CALND (P = .10). Two of the 28 patients (7.1%) had additional metastases detected with CALND. These patients had a T3 or T4 invasive lobular primary tumor. Of 24 patients with T1 or T2 primary tumors and IHC-detected metastases who underwent CALND, none had additional metastases detected. Median follow-up was 14.5 months. All patients with IHC-detected SLN metastases were treated with adjuvant systemic therapy. None of the five patients with IHC-detected metastases not undergoing CALND has subsequently manifested clinical axillary disease. CONCLUSIONS: CALND could have been or was safely omitted in 29 of 29 patients with T1 or T2 primary tumors and metastases detected by IHC. Such patients should be counseled about this low risk before CALND is recommended.